Usmle Step 1

Front 1

Wiskott-Aldrich Syndrome

Back 1

X-linked recessive disease characterized by recurrent sinopulmonary infections, thrombocytopenia, hepatosplenomegaly, epistaxis (hemorrage from the nose) and eczema. Pts have low serum IgM levels but increased IgG, IgA, and IgE levels. Pts have a defective response to polysaccaride antigens which is due to a cytoskeletal defect in T cells that inhibits their binding to B cells. Triad of sx includes recurrent pyogenic infections, thrombocytopenic purpura and eczema. Death usually occurs before age 6. Pts are inclined towards developing lymphoid malignancies

Front 2

Polycystic Kidney Disease (PKD)

Back 2

Autosomal dominant. Presents in 4th decade with hematuria, hypertension, and/or papable flank masses. Results in chronic renal failure. Due to mutations in the PKD-1 gene that encodes polycystin 1, a transmembrane protein involved in cell-matrix interaction. Extra renal manifestations include liver cysts, circle of Willis berry anurysms and mitral valve prolapse

Front 3

Aplastic anemia

Back 3

Pancytopenia with normal cell morphology. Hypocellular bone marrow with fatty infiltration. Hereditary (Fanconi's anemia-20%- defect in DNA repair leading to bone marrow damage) or aquired (acute hepatitis- most common, busulfan, vincristine, chloramphenicol, carbazamine, phenytoin, phenylbutazone, gold, benzene, insecticides). Often preceded by viral infections such as CMV, parvovirus and EBV. Tx: BM transplant, RBC and platelet transfusion, G-CSF or GM-CSF.

Front 4

Reidel thyroiditis

Back 4

Autoimmune thyroiditis characterized by dense fibrosis that destroys the adjacent muscle and connective tissue of the neck

Front 5

Angiosarcoma of the liver

Back 5

Caused by exposure to vinyl chloride used in the rubber industry

Front 6

Heaves

Back 6

Heard with ventricular hypertrophy

Front 7

S3 heart sound

Back 7

Low pitched sound heard at the end of rapid ventricular filling. Caused by early rapid ventricular filling of a volume-overloaded ventricle. Strongly suggests CHF. Can also occur in mitral or tricuspid regurgitation or in some young athletes

Front 8

Multiple myeloma

Back 8

Monoclonal neoplastic proliferation of plasma cells. "Fried-egg" appearance. Arises in the bone marrow. Produces large amounts of IgG (55%) or IgA (25%). monoclonal immunoglobulin spike (M spike) on serum protein electroporesis. Ig light chains (Bence-Jones proteins) in urine. Blood smear shows RBCs stacked like pocker chips (rouleaux formation). Destructive bone lesions in vertebral column, skull, ribs, pelvis and other bones. "Punched out" lesions are apparent as osteolytic areas on x-ray. Pts suffer spontaneous fractures and hypercalcemia. Renal insufficiency, increased suseptibility to infection and anemia.

Front 9

Vascular derivatives of pharyngeal arch 1

Back 9

Maxillary artery

Front 10

Vascular derivatives of pharyngeal arch 2

Back 10

Stapedial artery

Front 11

Vascular derivatives of pharyngeal arch 3

Back 11

Internal carotids

Front 12

Vascular derivatives of pharyngeal arch 4

Back 12

Arch of the aorta and right subclavian

Front 13

Pharyngeal arch 5

Back 13

Degenerates

Front 14

Vascular derivatives of pharyngeal arch 6

Back 14

Pulmonary arteries and ductus arteriosus

Front 15

Familial hypercholesterolemia

Back 15

Autosomal dominant. Mutations in LDL receptor. High cholesterol and xanthomas

Front 16

Carotid and aortic arch baroreceptors

Back 16

Increase firing rate with increasing blood pressure

Front 17

Werdnig-Hoffman disease

Back 17

Spinal muscular atrophy type 1. Due to defect in gene that normally turns off perinatal apoptosis. Results in lower motor nerve degenertion

Front 18

Conn Syndrome

Back 18

Primary aldosteronism. Characterized by hypertension secondary to sodium retention, hypokalemia and decreased serum renin. Clinical manifestations include episodic weakness, parathesias, transiant paralysis, tetany and personality disturbances

Front 19

Subfalcine herniation

Back 19

Cerebral hemisphere herniates along the midline into the cingulate gyrus

Front 20

Uncal herniation

Back 20

Subdural or epidural hematomas can force the medial aspect of the temporal lobe (uncus) under the free edge of the tentorium cerebelli. Can compress the oculomotor nerve, affecting peripheral areas of the nerve first, which carry parasympathetic innervation. Can also damage the medial temporal lobe or the midbrain.

Front 21

Arnold-Chiari type 2 malformation

Back 21

Cerebellar vermis and medulla herniate downward through the foramen magnum leading to obstruction of CSF flow and hydrocephalus. Associated with a small posterior fossa, myelomeningocele and syringomyelia.

Front 22

Urinary 5-hydroxyindoleacedic acid (5-HIAA)

Back 22

Seritonin metabolite. Characteristic of carcinoid syndrome.

Front 23

Carcinoid syndrome

Back 23

Diarrhea, flushing and abdominal pain

Front 24

Ki-67

Back 24

Nuclear factor whose expression correlates with neoplastic replicative activity

Front 25

Peutz-Jeghers syndrome

Back 25

Widespread spots of melanin pigmentation. Associated with the development of carcinomas of the pancreas, colon, stomach, small intestine, breast, ovary, uterus, cervix and lung

Front 26

Frothy urine

Back 26

Proteinuria

Front 27

Aortic Stenosis

Back 27

Systolic Crescendo-decrescendo murmer, ejection click, S4, low pulse pressure, sustained apical impulse, delayed pulse peak and reduced or delayed A2

Front 28

Berger disease

Back 28

IgA nephropathy. Develops after a respiratory infection. Causes microscopic hematuria and may progress to renal failure

Front 29

Beuger disease

Back 29

Vasculitis that occurs in smokers

Front 30

Acclimatization

Back 30

Decreased arterial O2 pressure and saturation but normal O2 content due to increased hemoglobin levels

Front 31

Neurofibromatosis 1 (von Recklinghausen disease)

Back 31

Autosomal dominant neurocutaneous syndrome. High penetrance but variable expressivity. Cafe-au-lait spots, axillary freckles, white Lisch nodules in the iris, optic gliomas, sphenoid bone dysplasia, thining of long bone cortex, pseudoarthrosis, and multiple cutaneous and subcutaneous tumors and neurofibromas. Due to nonsense or frameshift mutation in the neurofibromin gene on chromosome 17q11.2, causing a truncated nonfunctional ras protein leading to the proliferation of fibroblasts or Schwann cells in the peripheral nerves.

Front 32

Neurofibromatosis 2

Back 32

Bilateral acoustic neuromas, cafe-au-lait spots, axillary freckles, white Lisch nodules in the iris, and multiple cutaneous and subcutaneous tumors. The NF2 gene is a tumor suppressor gene on chromosome 22 that encodes a membrane cytoskeletal protein called merlin or schwannomin that appears to be involved in actin-cytoskeletal organization. Can have gait problems and ataxia due to acoustic neuromas

Front 33

Testicular feminization syndrome

Back 33

Testosterone is produced but the body fails to respond to it.

Front 34

Craniopharyngioma

Back 34

Adamantinoma/ ameloblastoma. Histologically resembles tooth enamel organ which contains ameloblasts

Front 35

Intraparenchymal hemorrhage

Back 35

Due to hypertension. Most common cause of death from stroke. Half of all cases involve the basal ganglia and internal capsule. Frequently dissect through the brain parenchyma into the ventricular system

Front 36

Menetrier disease

Back 36

Thickened rugal folds, hyperplasia of mucous glands, and protein losing eneteropathy. Not associated with gastric carcinoma

Front 37

CD14

Back 37

Endotoxin receptor on macrophages

Front 38

Wallenberg syndrome

Back 38

Lateral medullary syndrome. Occlusion of the posterior inferior cerebellar artery (PICA). Ipsilateral limb ataxia, ipsilateral loss of pain and temp sensation of the face, contralateral pain and temp sensation of the body, ipsilateral Horners syndrome, hoarsness, dysphasia, N/V, vertigo, and nystagmus

Front 39

Renal cell carcinoma

Back 39

Increased production of erythropoietin and parathyroid hormone related peptide (PTHrP) which causes hypercalcemia

Front 40

Polyarteritis nodosa

Back 40

Systemic necrotizing vasculitis of small to medium sized arteries that can affect any organ except the lungs but has a predelection for the GI tract and kidneys. Fibrosis of arteries can lead to the development of anurysms. Other sx include weight loss, malaise, low grade fever, abdominal pain, diarrhea, GI bleeding, arthralgias, and myalgia. Patients are typically young males. Can occur sporatically or as a complication of hepatitis B or C. 30% of pts have HBV.

Front 41

Chylous effusion

Back 41

Exudative lymphatic pleural effusion. Damage to the thoracic duct by a large tumor in the chest can cause leakage of lymphatic fluid into the pleural cavity

Front 42

Empyema

Back 42

Exudative purulent effusion resulting from an infection

Front 43

Ito cells

Back 43

Fat containing mesenchymal cells in the space of Disse. Store vitamin A

Front 44

Meconium ileus

Back 44

Manifestation of CF due to abnormally viscous pancreatic secretions. Failure to pass thickened meconium in the first few days of life. Abdominal distension and vomiting. Can cause intestinal perforation with peritonitis and intraperitonal calcifications or fistula formation into the bladder or vagina

Front 45

Kawasaki syndrome

Back 45

Mucocutaneous lymph node syndrome. Characterized by fever greater than 10 days, desquamative rash of fingers and toes, cervical lymphadenopathy, bilateral conjunctivitis, polymorphous rash, oral lesions, fissured/dry lips, pharyngeal erythema, and strawberry tongue. Affects small, medium and large arteries with transmural inflammation and variable necrosis. 20% of affected children have damage to coronary vessels and some develop coronary artery anurysms. Sudden death may occur from anurysm rupture. Leading cause of aquired heart disease in children in the US

Front 46

Mumps

Back 46

Moderate fever, headache, achiness, NO RASH, bilateral parotid gland swelling. Orchitis can develop in one or both testicles 4-7 days later. Strictly human virus spread by saliva

Front 47

Euthyroid sick syndrome

Back 47

Low T3 levels with normal T4 and TSH. Occurs with certain systemic illnesses such as pneumonia or septicemia, after a major surgery, and with malnutrition or starvation. The peripheral conversion of T4 to T3 is impaired due to a decrease in the activity of 5'-monodeiodinase activity in the liver and kidneys

Front 48

Exophthalmos

Back 48

A bulging of the eye anteriorly out of the orbit. Often seen in Graves' Disease

Front 49

resin T3 binding test

Back 49

To the sample of serum to be tested, labelled T3 is added and then a resin as secondary binder. if thyroid binding globulins are already occupied less of radioactive T3 will bind to it and more of it will be available to bind to senondary binder. The amount of T3 bound to resin is then measured. So in case of hyperthyroidism we will have high T3 resin uptake (because more T4 is bound to TBG) and in hypothyroidism we will have low T3 resisn uptake

Front 50

Primary biliary cirrosis

Back 50

antimitochondrial antibodies

Front 51

PIZZ allele

Back 51

Mutated form of the alpha 1 antitrypsin gene

Front 52

Minimal change disease

Back 52

aka nil disease or lipoid nephrosis. Peak incidence at 2-3 years of age. Associated with food allergies, medications, hematologic malignancies or can be idiopathic. Abnormal secretion of lymphokines by T-cells reduces the production of anions in the glomerular basement membrane, increasing glomerular permiability to positively charged albumin. Epithelial foot process fusion is also seen.

Front 53

Defective androgen receptor

Back 53

High levels of LH because the androgen receptors on the hypothalamic and pituitary tissue are also defective, impairing negative feedback and elevating testosterone levels

Front 54

Drugs that can cause hemolytic anemia in G6PD deficiency

Back 54

Sulfonamides, nitrofurantoin, dapsone, primaquin, and quinine

Front 55

Nerves that emerge from the cerebellopontine angle

Back 55

Facial (VII) and vestibulocochlear (VIII)

Front 56

Nerves that emerge from the post-olivary sulcus

Back 56

Glossopharyngeal (IX) and Vagus (X)

Front 57

Nerve that emerges from the anterolateral surface of the pons

Back 57

Trigeminal (V)

Front 58

Libman-Sacks endocarditis

Back 58

Non-infectious form of endocarditis associated with lupus. Small, granular vegetations consisting of fibrin develop on the undersurface of the leaflets of the mitral and aortic valves. Fragments of the vegetations may detach, resulting in embolism. Eventually, vegitations heal by organization of fibrin and fibrosis, leading to distortion of valve leaflets and resultant insufficiency or stenosis. Probably related to thrombotic diathesis

Front 59

Sarcoidosis

Back 59

Multisystem disease characterized by non-caseating granulomas. May be symptomatic with respiratory symptoms, erythema nodosum, uveitis, and arthritis or may be discovered incidentally. BILATERAL HILAR LYMPHADENOPATHY. ACE is elevated in 60% of cases

Front 60

Ziehl-Neelsen stain

Back 60

Special bacteriological stain used to identify acid-fast organisms, mainly Mycobacteria.

Front 61

Shape of epidural hematoma vs subdural hematoma

Back 61

Lenticular vs crescent

Front 62

Target cells

Back 62

RBCs with a peripherally dense rim of hemoglobin surrounding a central area of pallor. They are a result of an increase in the area of the red cell membrane and/or a decrease in hemoglobin. Most commonly seen in thalassemias, hemoglobin C disease, asplenia, and liver disease

Front 63

Syngenic graft

Back 63

Transfer of tissue between genetically identical individuals

Front 64

C3

Back 64

Removes immune complexes from the circulation

Front 65

Diffuse renal cortical necrosis sparing the medulla

Back 65

DIC (septic shock)

Front 66

Annular pancreas

Back 66

Bifid ventral pancreatic bud that rotates around both the right and left side of the second part of the duodenum, causing duodenal stenosis. Pt presents with projectile billous vomiting. Radiologic findings are the same seen in duodenal atresia: large air fluid level in the stomach and a smaller one in the first portion of the duodenum ("double-bubble sign")

Front 67

2 degree AV nodal block Mobitz type 1 "Wenckebach"

Back 67

PR interval progressively lengthens with RR interval shortening before the blocked beat. Caused by drugs (beta blockers, digoxin, and calcium channel blockers) or decreased vagal tone

Front 68

2 degree AV nodal block Mobitz type 2

Back 68

Intermittent non-conducted atrial beats not preceeded by lengthening AV conduction. Caused by organic lesion in the conduction pathway. May require a pacemaker.

Front 69

FTA-ABS

Back 69

Fluorescent treponemal antibody absorption test. For syphillis antibodies in the serum or CSF.

Front 70

Causes of rapidly progressing glomerulonephritis

Back 70

Goodpastures and systemic vasculitises

Front 71

Subacute thyroiditis

Back 71

Due to a viral infection of the thyroid gland causing follicular destruction and inappropriate release of thyroid hormone with resulting hyperthyroidism. Later, the distruction can proceed to the point that hypothyroidism occurs. Thyroid will be extremely painful

Front 72

Loeffler endocarditis

Back 72

Endomyocardial fibrosis with myocyte necrosis and a prominent eosinophilic infiltrate. Result of the direct toxicity to the heart by eosinophilic proteins (ribonuclease and major basic protein). The underlying eosinophilia is often idiopathic. Death can result from heart failure, arrythmias, or massive emboli. Treatment is resection of the fibrous heart tissue.

Front 73

Nerve that passes through the foramen rotundum

Back 73

V2

Front 74

Orthoclone (OKT3)

Back 74

anti-CD3 monoclonal antibody against T-cells. Used for immunosuppression in transplants

Front 75

Language at age 2

Back 75

200 words and 2 word sentences

Front 76

Genetic heterogeneity

Back 76

The presence of a variety of genetic defects that cause the same disease

Front 77

Tuberous sclerosis

Back 77

Autosomal dominant. Facial lesions, hypopigmented "ash leaf spots" on skin, cortical and renal hamartomas, seizures, mental retardation, renal cysts, angiomyolipomas, cardiac rhabdomyomas, astrocytomas. Mutations in TSC1 and TSC2 which encode for the proteins hamartin and tuberin, tumor supressors.

Front 78

DCC oncogene

Back 78

18q; gastric and colon carcinomas

Front 79

DPP tumor supressor

Back 79

18q; pancreatic cancer

Front 80

BRCA-1 gene

Back 80

17q; breast and ovarian cancer

Front 81

NF-1 gene

Back 81

17q; neurofibromatosis type 1

Front 82

NF-2 gene

Back 82

22q; neurofibromatosis type 2

Front 83

p53

Back 83

17p

Front 84

Borders of the inguinal triangle

Back 84

Medial- Rectus abdominis Lateral- Inferior epigastric arteries
Inferior- inguinal ligament

Front 85

Direct inguinal hernia

Back 85

Enter the inguinal canal by tearing through the transversalis fascia of the abdominal wall and through the inguinal (Hesselbach's) triangle into the superficial inguinal ring.Covered only by the external spermatic fascia.
More common in males

Front 86

Indirect inguinal hernias

Back 86

Covered by all three layers of spermatic fascia and pass lateral to the inferior epigastric arteries through the superficial and deep inguinal rings. Occurs in infants owing to the failure of the process vaginalis to close

Front 87

Femoral hernias

Back 87

Exits the abdominal cavity through the femoral ring to enter the femoral canal. The femoral ring is bounded medially by the lacunar ligament, laterally by the femoral vein, anteriorly by the inguinal ligament, and posteriorly by the pubis. Passes inferior and lateral to the pubic tubercle. More common in females

Front 88

anti-microsomal antibody

Back 88

anti-thyroid peroxidase antibody; found in Hashimoto's thyroiditis

Front 89

Persistent Truncus Arteriosis

Back 89

Results from the failure of the conotruncal ridges to form the articopulmonary septum. As a consequence, the ascending aorta and pulmonary trunk arise from a common tube, receiving blood from both the right and left ventricles, allowing oxygenated blood to mix with deoxyginated blood, causing cyanosis. Since the conotruncal ridges also participate in the formation of the membranous interventricular septum, a VSD is also evident

Front 90

Charcot-Marie-Tooth disease

Back 90

Hereditary sensory motor neuropathy type 1. Damage to the deep peroneal nerve results in wasting of the muscles of the anterior compartment of the lower limb. These children are clumsy and suffer from foot drop and pes planus (flat feet).

Front 91

Chevostek's sign and Troussaeus sign

Back 91

HypOcalcemia

Front 92

Fick's equation for cardiac output

Back 92

CO=O2 consumption/ arterial O2 content-venous O2 content

Front 93

Reed-Sternberg cells

Back 93

Binucleate or bilobed nuclei cells with prominent "owls-eye" nucleoli cells. Found in Hodgkin's lymphoma. Derived from germinal center or post-germinal center B lymphocytes. CD30 and CD15+. Display somatic hypermutation.

Front 94

Auer rods

Back 94

Cytoplasmic inclusions found in promyelocytic leaukemia cells in AML. Composed of azurophilic granules. Cause DIC if released

Front 95

Types of bile stones

Back 95

Cholesterol- yellow, radiolucent; composed of cholesterol monohydrate crystals
Black stones- radioopaque; calcium salts and unconjugated bilirubin
Brown stones- unconjugated bilirubin, calcium soaps, and cholesterol crystals

Front 96

Paroxymal Nocturnal Hemoglobinuria

Back 96

Red-brown to black urine, esp in the morning. Aquired red cell defect causing defective synthesis of phosphatydlinositol glycan A (PIG-A), located on the X chromosome. This deficiency causes RBCs to be very sensitive to lysis by complement, esp in an acidic environment such the respiratory acidosis that occurs during the night. Intravascular hemolysis. Leads to hemoglobinuria (increased urine hemosiderin). Can also be triggered by infection, menstration, surgical procedures, exposure to cold, and vaccines. Pts are prone to venous thromboses which may be a source of abdominal pain. The intrahepatic veins are a favorite site of thrombosis, leading to Budd-Chiari syndrome. Other complications include pigment gall stones (secondary to chronic hemolysis), and granulocytopenia leading to frequent infections. Pts can also develop aplastic anemia and leukemias. Diagnosis is by a sugar-water test and the Ham test in which serum is acidified to activate complement and then added to the pts RBCs resulting in lysis. Decay accelerating factor (CD 55 and CD 59) which blocks compliment mediated RBC lysis is also decreased in these pts. Tx: eculizamab (Ab to C5)

Front 97

Wilms tumor

Back 97

Childhood cancer. Presents as a large well demarcated abdominal mass containing embryonic glomerular and tubular structures imbedded in a spindle-cell stroma which may also contain muscle, bone cartilage, fat and fibros tissue. Usually unilateral on the kidney but may be bilateral if familial. Caused by a deletion of tumor suppressor gene WT1 on chromosome 11

Front 98

WAGR syndrome

Back 98

Wilms tumor, aniridia, genital anomalies, and mental retardation

Front 99

Alopecia acreta

Back 99

Autoimmune attack of hair follicles leading to hair loss in localized patches, the entire scalp (alopecia totalis), or the entire body surface (alopecia universalis). Strong association with HLA DQ3. Also DQ7 and DR4 less frequently

Front 100

Equation for clearance

Back 100

CL = 0.7xVd / t1/2

Front 101

Cords of Billroth

Back 101

Red pulp of the spleen

Front 102

Ito cells

Back 102

Hepatic adipose cells in the space of disse that store vitamin A

Front 103

Kupffer cells

Back 103

Phagocytes in the liver sinusoids

Front 104

Median umbillical ligament

Back 104

Adult derivative of the urachus. Surrounded by the medial umbillical fold. Also known as the median umbillical fold

Front 105

Lateral umbillical fold

Back 105

Fold of pariental peritoneum that covers the inferior epigastric artery and vein

Front 106

Burkitt lymphoma

Back 106

Occurs most often in children. B-cells. "Starry-sky" pattern (small lymphocytes with interspersed macrophages surrounded by clear spaces). Epidemic form- Africa, jaw lesion, associated with EBV Sporatic form- involves the pelvic and abdominal organs, 10% associated with EBV in US. t(8;14) translocation places c-myc in front of the IgH promotor.

Front 107

Follicular lymphoma

Back 107

Most common non-Hodgkin's lymphoma in adults. B-cells. t(14,18), bcl-2 (inhibits apoptosis. Difficult to cure. Indolent course. Marrow involvment.

Front 108

Managment of diabetic ketoacidosis

Back 108

Fluid, insulin, monitor potassium (can drop quickly since potassium is cotransported into cells with glucose in the presence of insulin)

Front 109

Occupational asthma

Back 109

Direct release of bronchoconstrictor substances. Triggered by inhalation of a number of chemicals such as epoxy resins, plastics, cotton fibers, toulene, formaldehyde and penicillin products

Front 110

Non-atopic/ nonreaginic asthma

Back 110

Enhanced vagal stimulation initiated by upper respiratory viral infections that lower the threshold of respiratory musoca to parasympathetic (vagal) stimulation

Front 111

Innervation of the posterior half of the external auditory meatus

Back 111

Auricular branch of the vagus nerve

Front 112

Innervation of the anterior half of the external auditory meatus

Back 112

Auriculotemporal nerve

Front 113

Innervation of the cranial surface of the upper auricle

Back 113

Lesser occipital nerve

Front 114

Innervation of the lower part of the auricle

Back 114

Greater occipital nerve

Front 115

Epinephrine reversal

Back 115

Administration of phentolamine blocks alpha 1 and 2 receptors of vascular smooth muscle leaving only beta receptors to be stimulated by epinephrine, which normally causes vasoconstriction by agonizing alpha > beta recptors, causing vasodilation

Front 116

TORCH

Back 116

Acronym for microorganisms causing specific pathologic changes during fetal or neonatal infection. T= Toxoplasma O= others (syphilis and TB) R= rubella C= CMV H= HSV

Front 117

Congenital syphilis

Back 117

Cleft palate, rhinitis, desquamating rash, Saddle nose (flattening of the nose), Hutchinson's teeth (notched incisors), mulberry molars (deformed molars), saber shins (anterior bowing of the anterior tibias), deafness, and interstitial keritinitis (vascularization of the corneas). Pts will have a positive VDRL (nonspecific antibodies that flocculate mamallian cardiolipin)

Front 119

Hutchinson's triad

Back 119

Deafness, interstitial keritinitis, and notched incisors. Congetital syphilis.

Front 120

Ecthyma Gangrenosum

Back 120

Lesion caused by Pseudomonas. Black necrotic center with raised red edges. Exotoxin A inhibits protein synthesis

Front 121

Kernicterus

Back 121

Caused by infantile unconjugated hyperbilirubinemia which damage the basal ganglia. Associated with opisthotonus (a state of a severe hyperextension and spasticity in which an individual's head, neck and spinal column enter into a complete "bridging" or "arching" position. This abnormal posturing is an extrapyramidal effect and is caused by spasm of the axial muscles along the spinal column), a high-pitched cry, an abnormal Moro reflex, and abnormal eye movements. Can be caused by mom taking sulfomamide antibiotics during the second or third trimester

Front 122

Viruses with segmented genomes that can undergo genetic shift

Back 122

Influenza A, Rotavirus

Front 123

Secretory endometrium

Back 123

Strips of endometrium bearing long, narrow, coiled glands lined by a single layer of columnar epithelium showing regular, uniform, small nuclei and clear apical (secretory) vessels

Front 124

Proliferative endometrium

Back 124

Smaller, noncoiled glands lined with columnar epithelium without apical (secretory) vesicles

Front 125

Simple endometrial hyperplasia

Back 125

Cystically dilated glands without cellular atypia

Front 126

Complex endometrial hyperplasia without atypia

Back 126

Complex branching glands without cellular aypia

Front 127

Atypical endometrial hyperplasia

Back 127

Complex glands lined by cells showing features of atypia, such as cytomegaly, increased nuclear to cytoplasmic ratio, prominent nucleoli and increased mitotic index. Can progress to endometrial cancer

Front 128

Falicform ligament

Back 128

Remnant of the umbilical vein

Front 129

Ataxia-telangictasia syndrome

Back 129

AR. Ataxia refers to poor coordination and telangiectasia to small dilated blood vessels Presents between the ages of 2 and 5 with symptoms such as lack of balance, slurred speech, and increased infections. Associated with a DNA repair enzyme defect on chromosome 11 and an increased number of translocations, especially involvind the T-cell receptor loci, thereby decreasing production of IgA and weakening the immune system, leading to respiratory disorders and increased risk of cancer.

Front 130

Anti-Scl-70 antibodies

Back 130

Scleroderma

Front 131

Atrial myxoma

Back 131

Most common primary adult cardiac tumor. Benign. Typically occurs as a single lesion in the left atrium that may intermitently obstruct the mitral valve ("ball-valve obstruction"). Comprised of scattered mesenchymal cells in a prominent myxoid background.

Front 132

Psamomma bodies

Back 132

Laminated, concentric, calcific spherules seen in papillary thyroid cancer, serous papillary cystadenoma of the ovary, meningiomas, and malignant mesothelioma

Front 133

Anitschkow cells

Back 133

Activated histiocytes found in rheumatic heart disease

Front 134

Roth's spots

Back 134

Pale retinal spots surrounded by hemorrage. Seen in endocarditis

Front 135

Yersinia enterocolitica

Back 135

Important cause of mini epidemics of pediatric diarrhea in day care centers. May be complicated by severe dysetery, pseudoappendicitis, mesenteric lymphadinitis, or chronic relapsing ileocolitis

Front 136

Typhoid fever

Back 136

Caused by Salmonella enterica subspecies typhi. Characterized by fever, abdominal rash and splenomegaly

Front 137

Immune cells involed in atypical pneumonia

Back 137

Macrophages and T-cells

Front 138

Salicylate toxicity

Back 138

Respiratory alkalosis by stimulation of the respiratory center in the brain stem causing hyperventilation and hyperapnea (increased depth of ventilation). Metabolic acidosis by reversion to anaerobic glycolysis causing lactic acidosis. Death is usually due to dehydration and hypokalemia.

Front 139

Acetominophen toxicity

Back 139

N/V, abdominal pain, shock and irreversible hepatic failure. Can administer N-acetylcysteine to counteract.

Front 140

Mercury poisoning

Back 140

CNS atrophy, gingivitis, gastritis and necrosis of the gastrointestinal epithelium, and renal tubular necrosis

Front 141

Lead poisoning

Back 141

Inhibits ferrochelatase and ALA dehydrase, enzymes involved in the synthesis of heme. Microcytic anemia with basophilic stippling of RBCs, neuropathy and abdominal pain. Urine shows accumulation of coproporphyrin and ALA

Front 142

Staphlococcus epidermidis

Back 142

Coagulase negative, non-hemolytic and novobiocin-sensitive. Common cause of subacute bacterial endocarditis particularly following infection of an intravenous catheter or shunt

Front 143

Optochin resistence

Back 143

Differentiates between alpha hemolytic streptococci. S. pneumoniea is optochin and bile sensitive. S. viridians is optochin and bile resistant.

Front 144

Only two microorganisms that produce cogulase

Back 144

Staph aureus and Yersinia pestis

Front 145

Bacitracin resistance

Back 145

Distinguishes S. agalactiae which is bacitracin resistant from S. pyogenes which is bacitracin sensitive

Front 146

Novobiocin resistance

Back 146

Distinguishes Staph aureus which is novobiocin resistant from Staph epidermidis which is novobiocin sensitive

Front 147

Crew haircut

Back 147

Distinctive radiological change on skull X-ray seen in sickle cell anemia. Develops as a result of marrow expansion with resorption of bone and secondary new bone formation. Pts also have exrtamedullary hematopoiesis in the liver or spleen

Front 148

Punched out lytic bone lesions

Back 148

Multiple myeloma

Front 149

Inulin

Back 149

A substance that is filtered but not secreted or reabsorbed. Clearance is equal to GFR

Front 150

Para-aminohippuric acid (PAH)

Back 150

A substance that is both freely filtered and secreted. The clearance of PAH equals effective renal plasma flow (RPF) unless the secreting mechanism is saturated (by increased plasma concentration)

Front 151

Jarisch-Herxheimer reaction

Back 151

Reaction to treatment of Treponema pallidum. As the bugs die, they release large amounts of endotoxin which can cause endotoxic shock. Endotoxin binds to CD14 on macrophages and stimulates overproduction and release of IL-1, IL-6, and TNF-alpha.

Front 152

Interferon gamma

Back 152

Produced by active T-helper cells and serves to activate macrophages

Front 153

Recurrent laryngeal nerve

Back 153

Branch of the vagas nerve that innervates all of the laryngeal muscles except for the cricothyroid muscle which is innervated by the external laryngeal nerve

Front 154

Muscles that cause adduction of the vocal ligament

Back 154

Cricothyroid and lateral cricoarytenoid

Front 155

Muscle that cause abduction of the vocal ligament

Back 155

Posterior cricoarytenoid

Front 156

Muscle that causes loosening of the vocal ligament

Back 156

Thyroarytenoid

Front 157

Muscle that causes tension of the vocal ligament

Back 157

Vocalis

Front 158

Mesosome

Back 158

Complex invaginations of the bacterial cell membrane that serve in cell division

Front 159

Muscle that accomplishes the backward movement of the jaw during mastication

Back 159

Temporalis

Front 160

Muscle that causes forward motion of the jaw during mastication

Back 160

Lateral pterygoid

Front 161

Muscles that elevates the lower jaw

Back 161

Medial pterygoid and masseter

Front 162

Muscles that depress the lower jaw

Back 162

Digastric and myohyoid

Front 163

Structures that drain into the medial side of the horizontal chain of superficial inguinal ligaments

Back 163

Penis, vagina, anal canal

Front 164

Lymph nodes that drain the colon

Back 164

Mesenteric nodes

Front 165

Regions of the brain affected in OCD

Back 165

Increased activity in the caudate nucleus and prefrontal cortex

Front 166

anti-glutamic acid decarboxylase antibodies

Back 166

Antibodies to beta islet cells seen in DM1

Front 167

Nerve that passes through the pronator teres

Back 167

Median nerve

Front 168

Nerve that passes through the flexor carpi ulnaris

Back 168

Ulnar nerve

Front 169

Nerve that passes through the coracobrachialis

Back 169

Musculocutaneous

Front 170

Nerve that passes through the supinator

Back 170

Radial

Front 171

Sturge-Weber disease

Back 171

Spontaneous congenital neurocutaneous syndrome. Associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is caused by an arteriovenous malformation that occurs in the cerebrum of the brain on the same side as the port wine stain.

Front 172

Friedrich's ataxia

Back 172

An inherited disease that causes progressive damage to the nervous system resulting in symptoms ranging from gait disturbance and speech problems to heart disease. Autosomal recessive disease that is caused by a trinucleotide repeat expansion (GAA). Defect in the frataxin gene. The ataxia of Friedreich's ataxia results from the degeneration of nerve tissue in the spinal cord, in particular sensory neurons essential (through connections with the cerebellum) for directing muscle movement of the arms and legs. The spinal cord becomes thinner and nerve cells lose some of their myelin sheath.

Front 173

Pharyngeal arch 1 derivatives

Back 173

Mandible, Malleus, Incus, muscles of mastication, anterior 2/3 of the tongue. Innervated by CN V2 and V3

Front 174

Pharyngeal arch 2 derivatives

Back 174

Stapes, Styloid process, muscles of facial expression, stapedius, stylohyoid. Innervated by CN VII

Front 175

Pharyngeal arch 3 derivatives

Back 175

Greater horn of hyoid, stylopharyngeus, posterior 1/3 of the tongue. Innervated by CN IX

Front 176

Pharyngeal arch 4 derivatives

Back 176

Thyroid cartilage, pharyngeal constrictor muscles, cricothyroid, levator veli palatini, posterior 1/3 of the tongue. Innervated by the superior laryngeal branch of the vagus. Swallowing.

Front 177

Pharyngeal arch 6 derivatives

Back 177

Thyroid cartilage, all intrinsic muscles of the larnyx except the cricothyroid. Innervated by the recurrent laryngeal branch of the vagus. Speaking

Front 178

Pharyngeal cleft devivatives

Back 178

1st cleft- external auditory meatus 2-4th clefts- temporary cervical sinus which is obliterated by the 2nd pharyngeal arch.

Front 179

Persistent cervical sinus

Back 179

Brachial cleft cyst within the lateral neck

Front 180

Derivatives of the 1st pharyngeal pouch

Back 180

Middle ear cavity, eustachian tube, mastoid hair cells

Front 181

Derivatives of the 2nd pharyngeal pouch

Back 181

Epithelial lining of palantine tonsil

Front 182

Derivatives of the 3rd pharyngeal pouch

Back 182

Thymus and inferior parathyroids. Descend and end up below the 4th pouch structures

Front 183

Derivatives of the forth pharyngeal pouch

Back 183

Thymus and superior parathyroids

Front 184

Derivatives of the 5th pharyngeal pouch (ultimobrachial body)

Back 184

C-cells of the thyroid

Front 185

Aberrant development of the 3rd and 4th pharyngeal pouches

Back 185

DiGeorge syndrome- T-cell deficiency (thymic aplasia) and hypocalcemia (faliure of parathyroid development). Pts also have abnormal facies such as low-set ears, hypertelorism (wide-set eyes), and a shortened philtrum

Front 186

Development of the thyroid gland

Back 186

Arises from the floor of the primative pharynx and descends through the foramen cecum and thyroglossal duct into the neck

Front 187

Cleft lip

Back 187

Failure of fusion of the maxillary and medial nasal processes

Front 188

Cleft palate

Back 188

Failure of fusion of the lateral palantine processes, the nasal septum and/or the median palantine process

Front 189

CD 19

Back 189

B-cells

Front 190

CD 18

Back 190

Integrin required for leukocyte margination and diapedesis. Absent in leukocyte adhesion deficiency

Front 191

CD 40-L

Back 191

Ligand of T-cells that allows for B-cell class switching. Mutated in hyper-IgM immunodeficiency.

Front 192

Clinical findings in a pt with an atrial septal defect

Back 192

Right ventricular cardiac impulse, systolic ejection murmer heard in the pulmonic area and allow the left sternal border, fixed splitting of the second heart sound, and right atrial dilation

Front 193

Equation for renal plasma flow (RPF)

Back 193

RBF x hematocrit
If RPF decreases due to an increase in Hct or decrease in RBF, GFR will decrease because water is being removed from a smaller volume resulting in a steep rise in oncotic pressure along the glomerular capillary

Front 194

Graft vascular disease

Back 194

Aka graft atherosclerosis. Complication that develops years after a heart transplantation. Due to intimal thickening of coronary arteries due to proliferation of fibroblasts and myocytes without associated atheroma formation or significant inflammation. Leads to progressive stenosis of the lumen. Since transplanted hearts are denervated, chest pain does not accompany the ischemic injury so sudden death can occur.

Front 195

HLA-A3

Back 195

Hemochromatosis

Front 196

Hemochromatosis

Back 196

Excess iron is deposited in tissues which can discolor the skin and damage the pancreas ("bronze diabetes"). Also produces liver damage/ cirrosis, arthropathy, splenomegaly, hypogonadism, and cardiomyopathy. Prussian blue staining shows iron deposition. Strong association with HLA-A3

Front 197

Reticulin staining

Back 197

Used to identify cirrosis by demonstrating fibrosis

Front 198

Sudan staining

Back 198

Used for staining fat such as in steatorrhea

Front 199

Gamma glutamyl-transferase (GGT)

Back 199

Enzyme secreted from the SER of hepatocytes in alcoholics

Front 200

Hemosiderin-laden macrophages

Back 200

Found in the lungs of pts with CHF

Front 201

Complete hyatidiform mole

Back 201

46,XX. No fetal tissue. Characteristic grapelike, multicystic appearance of the chorionic villi. Carry a 2% increased risk of choriocarcinomas

Front 202

Partial hyatidiform mole

Back 202

Fetal tissue is present. Triploid with 69,XXX or 69,XXY. Rarely develops into choriocarcinoma

Front 203

Thyrotoxic crisis

Back 203

Occurs in pts with poorly controlled Graves disease. Abrupt onset triggered by stressors such as infection, radio-iodine treatment, and childbirth. Fever, profuse sweating, tachycardia, tremor, delirium, N/V, and abdominal pain. Can progress to congestive heart failure, pulmonary edema and death.

Front 204

Necrotizing enterocolitis

Back 204

Acute abdominal distress, GI bleeding, large intestinal perforation, and sepsis in the first week of life. Common in premature and low birth weight infants. Multifactorial origin with intestinal ischemia, microbial agents and poor GI immune response being major contributing factors

Front 205

"Pearly papules"

Back 205

Basal cell carcinoma

Front 206

Histology of basal cell carcinoma

Back 206

Palisading nuclei (nuclei lined up like a fence) at the edges of clusters of cells penetrating into the dermis

Front 207

Pseudopalisading nuclei

Back 207

Nuclei lined up with necrosis next to them. Seen in glioblastomas

Front 208

Molluscum contagiosum

Back 208

Viral infection of the skin

Front 209

Fetal alcohol syndrome

Back 209

Epicanthal folds, short palpebral fissures, a smooth philtrum, small teeth, a thin upper lip, poorly formed ears, limited joint movement, mental retardation, microcephaly, ASDs, and VSDs

Front 210

Maternal lithium

Back 210

Abnormal formation of the tricuspid valve

Front 211

Seborrheic keratosis

Back 211

Common benign skin lesions of middle aged and older people. Well circumscribed tan-brown lesions, slightly raised with a rough surface and .5-1.5 cm on diameter. Peel away easily. Histologically show hyperplasia of the epidermis with a basaloid apperance. Pseudo-horn cysts- small cystic spaces with keratin formation. May mimic the apperance of basal or squamous cell carcinoma.

Front 212

Thrombotic thrombocytopenic purpura (TTP)

Back 212

Aka Meschowitz disease. Characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure, fever, and neurologic symptoms. Pts have microhemorrhages (mucous membrane bleeding, epistaxis (nose bleeds) petechiae and purpura) and show an increased bleeding time but a normal PT and PTT. Other findings include purpura, microthrombi, elevated indirect bilirubin, high lactate dehydrogenase, schistocytes/ helmet cells, and akinocytes/ burr cells. Autoimmune. More common in young women. Caused by a deficiency in vWF-cleaving metalloprotease that causes huge vWF multimers and increased platelet aggregation when endothelial damage occurs. May be precipitated by the use of estrogens or pregnancy.

Front 213

DIC

Back 213

Massive activation of the coagulation cascade leading to microthrombi and global consumption of platelets, fibrin, and coagulation factors. Clotting and bleeding at the same time. Microangiopathic anemia with schistocytes/ helmet cells on smear. Similar to TPP but the PT, PTT and thrombin time are also prolonged, fibrinogen levels are reduced and fibrin split products (D-dimers) are present. Caused by sepsis, trauma, obstetric complications, acute pancreatitis, malignancy, nephrotic syndrome, and transfusion reactions. Tx: RBCs and platelets

Front 214

Hemolytic uremic syndrome

Back 214

Microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Like TTP but without mental status changes. Schistocytes/ helmet cells and akinocytes/ burr cells. Caused by a toxin that damages the endothelium.

Front 215

CV effects of autonomic agonists

Back 215

NE- decreased HR, increased BP
Iso- increase HR, decreased BP
Epi- Increase HR, increase BP

Front 216

CV effects of autonomic antagonists

Back 216

Atropine- Increase HR, no effect on BP
Phentolamine (alpha)- Increase HR, decrease BP
Propranolol- decrease HR, decrease BP

Front 217

Erythroblastosis fetalis

Back 217

Immune attack of the mother's Rh antibodies on her fetus. Causes hydrops, anemia, cardiac decompensation, edema, and high serum bilirubin levels

Front 218

S4 heart sound

Back 218

Occurs during late rapid ventricular filling when the aorta contract just before S1. Heard in pts with conditions that increase resistence to ventricular filling such as hypertension, CAD, aortic stenosis, and cardiomyopathy

Front 219

Locked-in syndrome

Back 219

Pts are conscious and cognitively intact but are unable to move their extremities or face. Caused by large lesions of the brain stem, particularly at the base of the pons, produced by thrombotic stroke to the basilar artery.

Front 220

Parinaud's syndrome

Back 220

Headaches, paralysis of upward gaze, bilateral papilladema, and pupils that react to accomidation but not to light. Caused by a pineal tumor compressing the superior colliculus and pretectal area of the dorsal midbrain, the region that contains the vertical gaze center. Can also cause compression of the cerebral aqueduct causing noncommunicating hydrocephalus

Front 221

MOA of theophylline

Back 221

Inhibits phosphodiesterase and antagonizes adenosine receptors. Narrow therapeutic window. Can cause sinus arrythmias and tachycardia

Front 222

Metastatic carcinoma of the brain

Back 222

Most common adult brain cancer. Usually located at the junction of cortical gray and white matter and is round in apperance.

Front 223

Paramyxovirus

Back 223

Causes mumps. Negative-sense RNA viruses that spread from cell-to-cell via syncytia formation. Antibodies are innefective and cell-mediated immunity is necessary to clear the virus. Hence, MMR is a live, attenuated vaccine.

Front 224

Poliovirus

Back 224

naked single stranded positive-sense RNA virus

Front 225

Ether inactivation

Back 225

Enveloped viruses

Front 226

Dense deposits in the kidney

Back 226

Type II membranoproliferative glomerulonephritis

Front 227

IgA nephropathy

Back 227

Most common nephritic syndrome but presents as a nephrotic syndrome. Increased synthesis of IgA. ICs deposit in mesangium. Often follows a URI. Common in children.

Front 228

Type I membranoproliferative glomerulonephritis

Back 228

Subendothelial deposits of immune complexes. "tram-track" apperance on EM due to GBM splitting caused by mesangial ingrowth. Associated with HBV

Front 229

Membranous glomerulonephritis

Back 229

Most common adult nephrotic syndrome. GBM thickening and "spike and dome" appearance on EM. Caused by drugs (captopril), infections (HBV, syphilis), malignancies, and SLE

Front 230

Rapidly progressive glomerulonephritis

Back 230

Crescent shaped casts. Poor prognosis. Associated with Goodpastures, Wegener's granulomatosis, and microscopic polyarteritis

Front 231

Diffuse proliferative glomerulonephritis

Back 231

90% progress to CRF. Most common cause of death in SLE. Subendothelial IC deposits. "Wire-looping" of capillaries

Front 232

Minimal change disease

Back 232

Nephrotic syndrome in children. Foot-process effacement

Front 233

Diabetic glomerulonephropathy

Back 233

Nonenzymatic glycosylation of GBM. Kimmelstiel-Wilson "wire-loop" lesions. Progesses to CRF

Front 234

Focal segmental glomerulosclerosis

Back 234

Aggressive nephrotic syndrome seen in HIV patients and IV drug abusers. Responds poorly to steroids. Progresses to CRF

Front 235

Calculating GFR using creatinine clearance

Back 235

GFR= Creatinine Clearance = (rate of urine flow x urine creatinine)/ plasma creatinine

Front 236

Nasopharyngeal carcinoma

Back 236

Caused by EBV

Front 237

Hormones with a common alpha subunit

Back 237

TSH, LH, FSH, hCG

Front 238

Somatostatin

Back 238

Inhibits GH and TSH secretion

Front 239

Regulation of prolactin

Back 239

DA inhibits, TRH stimulates
PRL inhibits GnRH (amenorrhea with prolactinomas)

Front 240

21 alpha-hydroxylase deficiency

Back 240

Most common form of CAH. Decreased cortisol, decreased mineralcorticoids, increased sex hormones. Sx: virulization, hypotension, hyperkalemia

Front 241

11 beta-hydroxylase deficiency

Back 241

Decreased cortisol, decreased aldosterone, increased sex hormones. Sx: virulization, hypertension and hypokalemia (due to excess of 11-deoxycorticosterone, a mineralocorticoid)

Front 242

17 alpha-hydroxylase deficiency

Back 242

Decresed sex hormones and cortisol, increased mineralocorticoids. Sx: hypertension, hypokalemia, males- pseudohermaphroditism (externally female bc no DHT but no internal female reproductive structures due to intact MIF)

Front 243

Causes of decreased Mg2+

Back 243

Diarrhea, aminoglycosides, diuretics, alcohol abuse

Front 244

Desmolase

Back 244

Converts cholesterol into pregnenolone. Activated by ACTH, inhibited by ketoconazole

Front 245

Endocine hormones that signal through Gq

Back 245

Oxytocin, ADH (V1), GnRH, GHRH, TRH

Front 246

Conn syndrome

Back 246

Primary hyperaldosteronism. Caused by an aldosterone secreting tumor. Hypertension, hypokalemia, metabolic alkalosis and low plasma renin. Tx: spironolactone

Front 247

Addison's disease

Back 247

Primary deficiency of aldosterone and cortisol due to adrenal atrophy or destriction by disease. Hypotension, hyperkalemia, and skin hyperpigmentation (increased ACTH metabolized to melanin)

Front 248

Waterhouse-Friderchsen syndrome

Back 248

Acute adrenocortical insufficiency due to adrenal hemorrhage associated with meningiococcal septicemia

Front 249

Test for pheochromocytoma

Back 249

Urinary VMA (breakdown product of NE)

Front 250

Treatment for pheochromocytoma

Back 250

Phenoxybenzamine (a nonselective irreversible alpha blocker)

Front 251

Rule of 10s for pheos

Back 251

10% malignant, 10% bilateral, 10% extraadrenal, 10% calcify, 10% kids, 10% familial

Front 252

Neuroblastoma

Back 252

Most common tumor of the adrenal medulla in kids. Can occur anywhere along the sympathetic chain. HVA (breakdown product of dopamine) in urine. N-myc oncogene. Homer-Wright pseudorosettes are circles of tumor cells with central young nerve fibers arising from the tumor cell.

Front 253

MEN type 1 (Wermer's syndrome)

Back 253

3 P's
Parathyroid
Pancreas
Pituitary

Front 254

MEN Type 2A (Sipple's syndrome)

Back 254

2 P's
Pheo
Parathyroid
Medullary thyroid
Associated with ret gene

Front 255

MEN Type 2B

Back 255

1 P
Pheo
Medullary thyroid
Oral/intestinal ganglioneuromatosis
Associated with ret gene

Front 256

Hurthle cells

Back 256

Enlarged epithelial cells with abundant eosinophilic granular cytoplasm as a result of altered mitochondria.[2] They generally stain pink and are prominently found in histological sections of thyroid glands affected with Hashimoto's

Front 257

Toxic multinodular goiter

Back 257

Multiple nodules that overproduce thyroid hormone. Can occur when an iodine deficient person is made iodine repleate (Jod-Basedow phenomenon)

Front 258

Papillary thyroid cancer

Back 258

Most common thyroid cancer, excellent prognosis. Papillary bodies, "orphan anne" (empty or "ground glass") nuclei, psammoma bodies, nuclear grooves, increased risk with childhood radiation

Front 259

Cretinism

Back 259

Due to severe fetal hypothyroidism. Can be due to a lack of dietary iodine or defect in thyroid or T4 formation. Sx: pot-bellied, pale, puffy-faced, protruding umbillicus, protuberant tongue, jaundiced, mental retardation

Front 260

Dx and Tx of acromegaly (excess GH in adults)

Back 260

Dx: increased serum IGF-1 and failure to supress serum GH following an oral glucose tolerance test.
Tx: Pituitary adenoma resection followed by octreotide

Front 261

Hyperparathyroidism

Back 261

Primary- adenoma. "Bones, stones and groans"- osteitis fibrosa cystica, renal stones, constipation, hypercalcemia, hypophosphatemia
Secondary- decreased Ca++ absorption from gut usually due to chronic renal disease and decreased production of vit D. Hypocalcemia, hyperphosphatemia. Renal osteodystrophy

Front 262

Osteitis fibrosa cystica (von Recklinghausen's syndrome)

Back 262

Cystic bone spaces filled with brown fibrous tissue. Causes bone pain. Associated with hyperparathyroidism

Front 263

Hypoparathyroidism

Back 263

Due to accidental surgical excision of parathyroid glands during thyroid surgery, autoimmune distruction or Di George syndrome. Hypocalcemia and tetany

Front 264

Pseudohypoparathyroidism

Back 264

Autosomal dominant. Kidney unresponsive to PTH. Hypocalcemia, short stature, short 4th and 5th digits

Front 265

Hypercalcemia

Back 265

Caused by calcium ingestion, hyperPTH, hyperthyroid, multiple myeloma, paget's disease, addison's disease, neoplasms, Zollinger-Ellison syndrome, excess vitamin D, excess vitamin A, sarcoidosis

Front 266

Prolactinoma

Back 266

Most common pituitary adenoma. Amenorrhea, galactorrhea, low libido, infertility, bitemporal hemianopia. Shrink with bromocriptine or cabergolide (DA agonists)

Front 267

Treatment of nephrogenic diabetes insipidus

Back 267

Hydrochlorothiazide, indomethacin or amiloride

Front 268

Histiocytosis X

Back 268

Langerhans histiocytosis (LHC). Proliferative disorder of dendritic (Langerhans) cells. Cells express S-100 and CD1a. Birbeck granules ("tennis rackets") on EM. Seen in young adults who smoke. Associated with central DI. The least severe is eosinophilic granuloma. 60-80%. Usually occurs in children aged five to 10 years. The bones are involved 50–75% of the time, which includes the skull, or mandible, and the long bones. If the bone marrow is involved, anemia can result. With skull involvement, growths can occur behind the eyes, bulging them forward. The lungs are involved less than 10% of the time, and this involvement signals the worst prognosis. Next in severity is Hand-Schuller-Christian disease, a chronic, scattered form of histiocytosis. It occurs most commonly from the age of one to three years and is a slowly progressive disease that affects the softened areas of the skull, other flat bones, the eyes, and skin. Letterer-Siwe disease is the acute form of this series of diseases. It is generally found from the time of birth to one year of age. It causes an enlarged liver, bruising and skin lesions, anemia, enlarged lymph glands, other organ involvement, and extensive skull lesions.

Front 269

Causes of nephrogenic DI

Back 269

Hereditary or secondary to hypercalcemia, lithium, demeclocycline (ADH antagonist)

Front 270

Carcinoid syndrome

Back 270

Caused by carcinoid tumors (neuroendocrine tumors) especially small bowel tumors that secrete high levels of serotonin. Not seen if tumor is limited to GI tract (5-HT undergoes first pass metabolism). Results in recurrent diarrhea, flushing, asthmatic wheezing, and right-sided valvular disease. Most common tumor of the appendix. Increased 5-HIAA in urine. Tx: octreotide (somatostatin)

Front 271

Acute intermittent porphyria

Back 271

Autosomal dominent metabolic disorder of porphyrin synthesis more common in young women. Due to a deficiancy in Uroporphyrinogen I synthase. 6 P's- Painful abdomen, Pink urine, Peripheral neuropathy, Paralysis, Psychological disurbances, Precipitated by drugs. Other symptoms include fever, nausea, vomiting, constipation, agitation, restlessness, and diaphoresis may occur. Laboratory findings include leukocytosis, elevated urine porphobilinogen and delta aminolevulinic acid (ALA). Can treat with glucose loading- glucose inhibits ALA synthase

Front 272

Tumors associated with increased erythropoietin

Back 272

HCC, renal cell carcinoma, hemangioblastoma, pheochromocytoma, and uterine myomata

Front 273

Polycythemia vera

Back 273

Primary polycythemia. Acquired disorder of the bone marrow that causes the overproduction red blood cells and occasionally white blood cells and platelets. Occurs more frequently in men than women. Sx include headache, vertigo, hepatosplenomegaly, pruritis, particularly after exposure to warm water, gouty arthritis, peptic ulcer disease, and erythromelalgia-a sudden, severe burning pain in the hands or feet, usually accompanied by a reddish or bluish coloration of the skin.
Patients with polycythemia vera are prone to the development of blood clots leading to heart attack, stroke, deep venous thrombosis, or Budd-Chiari syndrome. Associated with mutations in JAK2. Tx- phlebotomy

Front 274

Reactive polycythemia

Back 274

Occurs in cases of tissue hypoxia, commonly seen in patients who smoke or have COPD

Front 275

Chromosomal inversions

Back 275

Chromosomal rearrangment that occurs when a central segment created by 2 double strand breaks inverts before the breaks are repaired. Pericentric inversions include the centromere and produce viable gametes. Paracentric inversions do not include the centromere and produce an acentric fragment and a dicentric bridge that will not complete meiosis

Front 276

Medullary thyroid cancer

Back 276

Part of MEN type IIa and IIb. Polygonal cells. Local production of amyloid formed from precipitated calcitonin. Genetic link to ret gene on chromosome 10.

Front 277

Rate of nerve regeneration

Back 277

1mm/day. Determined by the speed of slow axonal transport.

Front 278

Parvovirus

Back 278

Causes fifth disease, aplastic crisis in pts with chronic hemolytic diseases, and still birth or hydrops fetalis

Front 279

Coxsackie virus

Back 279

Coldlike illness, herpangina (painful mouth blisters), myocarditis and meningitis

Front 280

Sodium thiosulfate

Back 280

Antidote for cyanide poisoning

Front 281

Mesna

Back 281

Used to prevent hemorrhagic cystitis in pts taking cyclophosphamide or ifofamide

Front 282

Leucovorin

Back 282

Used to prevent myelosuppression in patients taking methotrexate

Front 283

Amifostine

Back 283

Used to prevent nephrotoxicity in pts taking cisplatin

Front 284

Beta-2 microglobulin

Back 284

Amyloid deposits in joints, muscle, and ligaments following long term hemodialysis

Front 285

Transthyretin

Back 285

Primary amyloidosis. Deposits in cardiac tissue

Front 286

Calcitonin precursors

Back 286

Amyloid deposition in medullary thyroid tumors

Front 287

Amyloid light chains

Back 287

Primary amyloidosis. Produced in the bone marrow and lymph nodes by plasma cell and B-cell prolifetations (e.g. multiple myeloma). Bence Jones proteins. Most common form of amyloidosis in the US. Systemic deposition including heart and kidneys

Front 288

Heinz bodies

Back 288

RBC inclusions of oxidized and denatured hemoglobin. Causes damage to cell membranes ("bite cells"). Seen in acute hemolysis and G6PD deficiency

Front 289

Pammenheimer bodies

Back 289

Iron-containing dark blue granules in RBCs seen in sideroblastic anemia

Front 290

Sideroblasts

Back 290

Microcytic hypochromic nucleated RBCs with iron granules in their cytoplasm that form a ring around the nucleus. Seen in alcoholism, lead or zinc poisoning, drugs (isoniazid, chloramphenicol, cycloserine), pyridoxine or coppor deficiency, or ALA synthase deficiency.

Front 291

Sideroblastic anemia

Back 291

Abnormal production of RBCs in due to an inability to form heme. Characterized by sideroblasts- microcytic hypochromic nucleated RBCs with iron granules in their cytoplasm that form a ring around the nucleus. Caused by lead or zinc poisoning, drugs (isoniazid, chloramphenicol, ethanol, cycloserine), pyridoxine or coppor deficiency, or ALA synthase deficiency. Can progress to hematologic malignancies, especially AML

Front 292

Basophilic stippling

Back 292

Blue ribosomal precipitate granules in the cytoplasm of RBCs. Seen in thalassemias, alcohol abuse, lead or other heavy metal poisoning, and iron deficiency

Front 293

Polychromasia

Back 293

Describes lots of proerythroblasts that have lots of RNA so they stain blue

Front 294

Corrected reticulocyte count

Back 294

(hematocrit/45) x retic count
If polychromasia is present, divide by 2

Front 295

Myeloid lineage

Back 295

Promyelocyte- primary granules
Myelocyte- secondary granules
Metamyelocyte- indented nucleus
Band cell- horseshoe nucleus

Front 296

Anisocytosis

Back 296

Varying RBC sizes

Front 297

Poikilocytosis

Back 297

Varying RBC shapes. Seen with microvascular damage.

Front 298

Basophil

Back 298

Bilobate nucleus. Basophilic granules contain heparin (anticoagulant), histamine (vasodilator) and other vasoactive amines, and leukotrienes

Front 299

Eosinophil

Back 299

Bilobate nucleus. Eosinophilic granules contain major basic protein and histiminase and arylsulfatase to help limit reaction following mast cell degranulation

Front 300

Hypersermented neutrophils

Back 300

Seen in B12/ folate deficiency

Front 301

Neutrophils

Back 301

Multilobed nucleus. Azurophilic granules contain myeloperoxidase, lysozyme, and lactoferrin. Turn-over every 1-2 days

Front 302

Monocyte

Back 302

Kidney shaped nucleus. Live 3-5 days in blood

Front 303

Macrophages

Back 303

Tissue monocytes. Activated by gamma IFN. Can live in tissue for days-months. APC cell.

Front 304

Protein C

Back 304

Vitamin K dependent factor. Inactivates Va and VIIIa. Activated by thrombin and protein S (regulation)

Front 305

Factor V leidin

Back 305

Hereditary thrombolytic disease. Mutant factor V can't be degraded by protein C

Front 306

Platelet aggregation

Back 306

TxA2 released by platelets activated by thrombin increases platelet aggregation (aspirin inhibits TxA2 synthesis). PGI2 and NO released by endothelial cells decreases platelet aggregation
Binding of ADP released by activated platelets to receptors on other platelets allows platelet aggregation (clopidogrel blocks ADP receptors)
ADP binding leads to signaling cascade that produces GpIIb/IIIa receptors which allow binding between platelets using fibrinogen (Abciximab blocks GpIIb/IIIa receptors)

Front 307

Epoxide reductase

Back 307

Activates vitamin K. Warfarin inhibits epoxide reductase

Front 308

Antithrombin III

Back 308

Inactivates factors II, VII, IX, X, XI. Heparin enhances activity of antithrombin III

Front 309

Kallikren

Back 309

Activates plasmin which cleaves fibrin. Indirectly activated by high molecular weight kininogen (HMWK) which activates factor XIIa, which activated kallikren. Kallikren also activates bradykinin from HMWK which increases vasodilation, permiability and pain

Front 310

Bombay blood type

Back 310

No fucose (H-substance) or sugar moieties (A or B) on RBCs. Have anti-H substance antibodies. Can only accept Bombay blood.

Front 311

Acanthocyte/ spur cell

Back 311

Spiny RBCs seen in liver disease and abetalipoprotinemia

Front 312

Normocytic anemia

Back 312

Decreased response of bone marrow to EPO, lack of EPO production, hypo and hyperthyroidism

Front 313

HbS mutation

Back 313

A single amino acid replacement in the beta globin chain (valine for glutamic acid)

Front 314

alpha thalassemia

Back 314

Underproduction of the alpha globin chain. HbH- tetramers of beta chains (lacks 3 alpha globin genes). Hb Barts- tetramers of gamma chains (lacks all 4 alpha chains); results in hydrops fetalis. Prevalent in Asia and Africa. Africans have alpha -/ alpha - genotypes. Asians have alpha globin mutations on the same gene (alpha alpha/ - -). If baby gets - -/ - -, hydrops.

Front 315

beta thalassemia

Back 315

Minor- beta globin chain is underproduced (heterozygote)
Major- aka Cooley's anemia; beta globin chain is absent (homozygote). Cardiac failure due to hemochromotosis secondary to blood transfusions. Marrow expansion from increased erythropoiesis gives skeletal abnormalities ("crew cut" on skull x-ray)

Front 316

Autoimmune hemolytic anemia

Back 316

Warm agglutin (IgG)- 70%. chronic anemia seen in SLE, CLL, or with certain drugs (e.g alpha-methyl dopa). Extravascular hemolysis in liver Kupffer cells and spleen. Leads to jaundice.
Cold agglutin (IgM)- 30%. Acute anemia triggered by cold. Seen with mycoplasma pneumoniae on mono infections. Intravascular hemolysis. Leads to hemoglobinuria.

Front 317

Direct Coombs test

Back 317

anti-IgG antibodies are added to a pts RBCs. RBCs agglutinate if coated with IgG.

Front 318

Indirect Coombs test

Back 318

Normal RBCs are added to pts serum. RBCs agglutinate if serum has anti-RBC IgGs.

Front 319

Schistocytes (helmet cells)

Back 319

Due to mechanical distruction of RBCs passing through obstructed or narrowed vessel lumina. Seen in tramatic hemolysis or in intravascular microangiopathic anemias in DIC, TTP/HUS, SLE, or malignant hypertension

Front 320

Idiopathic Thrombocytopenia Purpura (ITP)

Back 320

Autoimmune thrombocytopenia. Abs against platelets. Bleeding disorder causing microhemorrhage- mucous membrane bleeding, epistaxis (nose bleeds), petechiae, purpura. Bleeding time is increased.

Front 321

Hemophilia

Back 321

A- factor VIII deficiency
B- factor IX deficiency
Causes macrohemorrhages- hemarthroses (bleeding into joints), easy brusing. Increased PT and PTT.

Front 322

von Willebrand's disease

Back 322

Mild, most common bleeding disorder. Deficiency of vWF. Defect in platelet aggregation (increased bleeding time) and low factor VIII (increased PT and PTT). Type I, II, and III with I being the most severe.

Front 323

Hodgkin's lymphoma

Back 323

40% of lymphomas. Localized, single group or nodes usually mediastinal. Extra nodal involvment is rare. Constitutional "B" signs- low grade fever, night sweats, weight loss, and generalized pruritis. 50% of cases are associated with EBV. Bimodal distribution- young and old. Reed-sternberg cells. Increased lymphocyte to RS ratio correlates with a good prognosis. Types:
Non-sclerosing (65-75%)- excellent prognosis, collagen banding, women>men.
Mixed cellularity (25%)- males > 50, numerous RS cells, eosinophils, and plasma cells, intermediate prognosis
Lymphocyte predominent (6%)- males < 35, popcorn cells, usually asymptomatic, excellent prognosis
Lymphocyte depleted (rare)- older males with disseminated disease, poor prognosis

Front 324

Non-Hodgkin's lymphoma

Back 324

60% of lymphomas. Multiple peripheral nodes. Extranodal involvment is common. May be associated with HIV and immunosuppression. No "B" signs. Peak incidence is 20-40.

Front 325

Small lymphocytic lymphoma

Back 325

Adults. B-cells. Like CLL but a focal mass. Low grade.

Front 326

Diffuse large cell lymphoma

Back 326

Usually older adults but 20% occur in children. 80% B-cells 20% T-cells. Aggressive but curable.

Front 327

Mantle cell lymphoma

Back 327

Adults. B-cells. t(11;14). Poor prognosis.

Front 328

Lymphoblastic lymphoma

Back 328

Mostly children. T-cells. Commonly presents with ALL. Mediastinal mass. Very aggressive.

Front 329

Extramarginal zone lymphoma

Back 329

Caused by H. pylori. Can cure with antibiotics.

Front 330

M3 type of AML

Back 330

t(15;17). Responsive to all-trans retinoic acid.

Front 331

t(11;22)

Back 331

Ewing's sarcoma

Front 332

MGUS

Back 332

Monoclonal gammopathy of undetermined significance. Asymptomatic.

Front 333

Waldenstrom's macroglobulinemia

Back 333

M spike = IgM. Hyperviscosity symptoms. No lytic bone lesions.

Front 334

ALL

Back 334

Children. Increased lymphoblasts in marrow. TdT+ (marker of pre-T and pre-B cells), CD10+, CALLA+. Very responsive to therapy. May spread to CNS and testes.

Front 335

CLL

Back 335

Older adults. Lymphadenopathy, hepatosplenomegaly, fever and night sweats. Indolent course. "Smudge cells" (pink, crushed lymphocytes) on smear- reflect the fragility of the B-CLL cells. Warm antibody immune hemolytic anemia. Very similar to small lymphocytic lymphoma.

Front 336

Hairy cell leukemia

Back 336

Mature B-cell leukemia in the elderly. Presents with splenomegaly, abdominal fullness, and fatigue. Cells have filamentous, hair like projections. Stains TRAP positive. No lymphadenopathy. Diagnosis is made by a positive tartrate-resistant acid phosphatase test.

Front 337

Polycystic ovarian syndrome (PCOS)

Back 337

Enlarged bilateral cystic ovaries. Tonic estrogen levels cause pulse generator to be turned up too high and increases LH secretion leading to anovulation and hyperandrogenism. Amenorrhea, infertility, acanthosis nigracans, obesity and hirsutism. Associated with insulin resistance. Increased risk of endometrial cancer.

Front 338

Yolk sac tumor

Back 338

2nd most common germ cell derived tumor. Aggressive malignancy in ovaries or testes in children ages 10-30. Bad prognosis. Produces alpha fetoprotein. Schiller-Duval bodies

Front 339

Granulosa cell tumor

Back 339

Ovarian tumor that secretes estrogen. Causes precocious puberty, endometrial hyperplasia or carcinoma. Call-Exner bodies- small follicles filled with eosinophilic secretions

Front 340

Benign breast tumors

Back 340

Fibroadenoma- small, mobile and firm. Most common < 25. Increase size and tenderness with increased estrogen
Intraductal papilloma- small tumor that grows in lactiferous ducts beneath the areola. Serous or bloody nipple discharge
Cystosarcoma phyllodes tumor- large bulky mass of connective tissue and cysts. "Leaf-like projections". Most common in 6th decade.

Front 341

Malignant breast tumors

Back 341

Ductal carcinoma in situ (DCIS)- fills ductal lumen
Invasive ductal- firm fibrous mass with small glandular duct-like cells. Most common (76%). Worst and most invasive.
Invasive lobular- orderly rows of cells. Often multiple and bilateral.
Medullary- fleshy cellular lymphatic infiltrate. Good prognosis.
Comedocarcinoma- ductal caseous necrosis
Inflammatory- dermal lymphatic invasion by breast carcinoma. Peau d'orange. 50% 5 yr survival.
Paget's disease- inflammation, pigmentation and eczematous patches on the nipples. Large cells with clear halo. Suggests underlying adenocarcinoma which can spread as isolated cells through the breast lymphatics.

Front 342

Ovarian cysts

Back 342

Follicular cyst- distention of unruptured grafian follicle. May be associated with endometrial hyperplasia.
Corpus luteum cyst- hemorrhage into persistent corpus luteum
Theca-lutein cyst- often bilateral/ multiple. Due to gonadotropin stimulation. Associated with choriocarcinoma and moles
Chocolate cyst- blood containing cyst from endometriosis.

Front 343

Ovarian germ cell tumors

Back 343

Dysgerminoma- Equivalent to male seminoma. Malignant. Sheets of uniform cells. hCG.
Choriocarcinoma- Malignant. Large hyperchromatic syncytiotrophoblasts. hCG.
Yolk sac tumor- 2nd most common. Malignant. Aggressive. Worst survival. Ages 10-30. Shiller-Duval bodies. alpha fetoprotein.
Teratoma- most common (90%). Mature- most frequent. Benign, Immature- aggressive. Malignant. Struma ovarii- contains functional thyroid tissue.

Front 344

Ovarian non-germ cell tumors

Back 344

Serous cystadenoma- 20%. Bilateral. Lined with fallopean like epithelium. Benign.
Serous cystadenocarcinoma- 50%. Malignant. Bilateral. Associated with BCRA-1 and HNPCC.
Mucinous cystadenoma- Multiloclar cyst lined by mucous secreting epithelium. Benign.
Mucinous cystadenocarcinoma- Malignant. Pseudomyxoma peritonei.
Brenner tumor- Benign. Looks like bladder.
Fibromas- bundles of spindle-shaped fibroblasts. Meigs' syndrome- triad of ovarian fibroma, ascites, and hydrothorax. Pulling sensation in groin.
Granulosa cell tumor- secretes estrogen. Precocious puberty, endometrial hyperplasia, carcinoma. Call-Exner bodies- small follicles filled with eosinophilic secretions.
Krukenberg tumor- mets from stomach adenocarcinoma. Signet cells.

Front 345

Vaginal carcinomas

Back 345

Squamous cell- secondary to cervical SCC
Clear cell adenoma- exposure to DES in utero
Sarcoma botryoides- affects girls < 4. Spindle shaped tumor cells that are desmin positive

Front 346

Myometrial tumors

Back 346

Leiomyoma- fibroid. Most common of all tumors in females. increased incidence in blacks. Benign smooth muscle tumor. estrogen sensitive. peak occurance is 20-40. May cause abnormal uterine bleeding. Does not progress to leiomyosarcoma. Whorled pattern of smooth muscle bundles.
Leimyosarcoma- Malignant. Bulky irregularly shaped tumor with areas of necrosis and hemorrhage. Increased incidence in blacks. Highly aggressive with a tendency to recur. May protrude from cervix and bleed.

Front 347

Fibrocystic disease

Back 347

Fibrosis- hyperplasia of breast stroma
Cystic- fluid filled. blue dome.
Sclerosing adenosis- increased acini and intralobular fibrosis
Epithelial hyperplasia- increased number of epithelial cells in terminal duct lobule. Increased risk of carcinoma with atypical cells. Women > 30.

Front 348

Fat necrosis

Back 348

A benign painless lump in the breast. Occurs as a result of injury to breast tissue.

Front 349

Drugs that cause gynocomastia

Back 349

Estrogen, marijuana, heroin, psychoactive drugs, spironolactone, digitalis, cimentidine, alcohol, ketoconazole

Front 350

Testicular germ cell tumors

Back 350

95% of all testicular tumors.
Seminoma- Most common testicular tumor. Malignant. Painless testicular enlargment. Males 15-35. Grossly- large, gray-tan, bulky mass. Microscopically- large cells in lobules with watery cytoplasm and a "fried egg" apperance. Elevated placental alkaline phosphatase. Radio sensitive. Excellent prognosis.
Embryonal carcinoma- Malignant. Painful. Worse prognosis. Bulky mass with hemorrhages and necrosis. Glandular/ papillary morphology. Can differentiate into other tumors. AFP and beta hCG may be elevated.
Yolk sac tumor- Analogous to ovarian yolk sac tumor. Schiller-duval bodies. Primitive glomeruli. alpha fetoprotein. Poor prognosis. AFP is elevated.
Choriocarcinoma- Malignant. beta hCG is elevated.
Teratoma- unlike in females, mature teratomas are malignant.

Front 351

Testicular non-germ cell tumors

Back 351

5% of testicular tumors. Mostly benign.
Leydig cell- Benign. Contains Reinke crystals. Androgen producing- precoucious puberty or gynogomastia
Sertoli cell- Benign. Androblastoma from sex cord tumor
Testicular lymphoma- most common testicular cancer in older men

Front 352

Penile tumors

Back 352

Bowen's disease- gray, solitary, crusty plaque, usually on the shaft of the penis or on the scotum. peak incidence in 5th decade. progresses to SCC in < 10% of cases.
Erythroplasia of Queyrat- red velvety plaques usually involving the glans penis. otherwise similar to Bowen's disease
Bowenoid papulosis- multiple papular lesions. affects younger age groups. usually does not become invasive.
Squamous cell carcinoma- rare in uncircumscribed males. uncommon in US. More common in Asia, Africa and SA. Commonly associated with HPV.

Front 353

Peyronie's disease

Back 353

Bent penis due to aquired fibrous tissue formation

Front 354

Causes of brain abscesses

Back 354

Otitis media- 60%. Cerebellar, frontal and temporal tobes
Sinusitis- Frontal and temporal lobes

Front 355

CAMP test

Back 355

Additional zone of beta hemolysis that appears in an arrowhead formation is seen on a blood agar plate growing Group B strep. S. agalactiae produces a polypeptide that complements the sphingomyelinase of Staph aureus and produces this additional zone of beta hemolysis

Front 356

Causes of endometritis

Back 356

Chlamydia infection, postpartum endometrial changes, and IUDs (Actinomyces)

Front 357

Replication of single stranded negative sense RNA viruses

Back 357

Carry an RNA-dependent RNA polymerase within their nucleocapsid so that they can convert their genome into a positive sense RNA (mRNA) that can be translated using the hosts ribosomes
Example: RSV

Front 358

Replication of single stranded positive sense RNA viruses

Back 358

Carry a reverse transcriptase (RNA-dependent DNA polymerase) within their nucleocapsid to create double stranded DNA that can be incorporated into the host genome
Examples: retroviruses (HIV)

Front 359

Replication of double stranded DNA viruses

Back 359

Use host cell's DNA-dependent RNA polymerase present in the nucleus to transcribe its genome into mRNA. Poxviruses complete this step in the cytoplasm using a viron-associated DNA-dependent RNA polymerase

Front 360

Replication of single stranded positive sense RNA viruses

Back 360

Do not require a polymerase- can be directly transcriped on host's ribosomes

Front 361

Replication of double stranded RNA viruses

Back 361

Negative sense strand is used as a template by virus core enzymes to synthesize individual mRNAs.
Examples: reovirus or rotavirus

Front 362

Lines of Zahn

Back 362

White laminations on a premortum clot composed primarily of platelets

Front 363

Prolactin-growth hormone family

Back 363

Prolactin and Growth hormone

Front 364

ACTH family

Back 364

ACTH and melanocyte stimulating hormone (MSH)

Front 365

Glycoprotein hormone family

Back 365

FSH, LH, TSH

Front 366

Superior vena cava syndrome

Back 366

Obstruction of venous return from the head, neck and upper extremities. Over 85% of cases are related to malignancy. Broncogenic carcinomas (small cell and squamous cell carcinoma) account for over 80% of these cases. Lymphomas are uncommon causes of SVC syndrome. Infectious etiologies include TB, syphilis and histoplasmosis. Can also occur as a result of an enlarged goiter and from thrombus formation caused by an indwelling intravenous line or pacemaker wires.

Front 367

Aneurysms of the aortic root

Back 367

Syphilis

Front 368

Most common cause of sudden cardiac death

Back 368

Ischemic heart disease. Non-atherosclerotic causes should be considered in young victims

Front 369

Drainage of the right adrenal and right gonadal vein

Back 369

IVC

Front 370

Drainage of the left adrenal and left gonadal vein

Back 370

Left renal vein

Front 371

Erythema multiforme

Back 371

Erythematous patches with pale, vesicular, or eroded centers known as "target lesions". Caused by immune complex deposition in dermal blood vessels and CD8 mediated destruction. 50% of cases are idiopathic. Other causes include infections (HSV, enteroviruses, mycoplasma pneumoniae, chlamydia, histoplasmosis), drugs (penicillin, sulfomamides, phenytoin, aspirin, corticosteroids, cimentidine, allopurinol, OCPs), neoplasma, sarcoidosis, and foods (notablt emulsifiers in margarine. Stevens-Johnson syndrome is the more severe, life threatening version of erythema multifrome.

Front 372

Cystinuria

Back 372

An autosomal recessive defect of the renal tubulesin which the amino acid cysteine cannot be reabsorbed. The cysteine precipitates in the urine, forming yellow-brown hexagonal crystals in the urine and radioopaque stones in the bladder or renal pelvis. A positive nitroprusside cyanide test on urine will confirm the diagnosis. Alkanalization of the urine increases the solubility of cysteine and is used with high fluid intake as the mainstay of therapy. However, the eventual development of end-stage renal failure is common.

Front 373

Homocysteinuria

Back 373

Elevation is plasma homocysteine levels, a product of methionine metabolism. Promote oxidative damage, inflammation and endothelial dysfunction. Pts are at risk for occlusive vascular disease. Other symptoms include displacement of the lens of the eye, skeletal abnormalities, osteoporosis, mental retardation, tall stature and kyphosis. Caused by deficiencies in folate, B12, B6 or congenital deficiencies in cystathionine beta-synthase, the enzyme that converts homocysteine to cystathione. Elevated homocysteine levels in pregnant women are associated with NTDs.

Front 374

Albinism

Back 374

Defect in tyrosine metabolism results in a deficiency in the production of melanin. Can result in partial or complete absence of pigment from the hair, eyes, and skin. Can be AR, AD or X-linked. Complete albinism results from a deficiency tyrosinase activity. Pts also have vision defects, photophobia and are at a high risk for skin cancer

Front 375

Ketogenic aa's

Back 375

Leu and Lys (both essential)

Front 376

Ketogenic and glucogenic aa's

Back 376

Nonessential: Tyr
Essential: Ile, Phe, Trp

Front 377

Branched chain aa's

Back 377

Ile, Leu, Val

Front 378

PKU

Back 378

Due to a deficiency in phenylalanine hydroxylase (PAH) which hydrolyzes Phe into Tyr (requires BH4). Mental retardation, hypopigmentation and a mousey odor. Treatment is with dietary restriction of Phe and replacement with BH4, L-DOPA and 5-HT

Front 379

Alkaptonuria

Back 379

Deficiency in homogentisic acid oxidase, enzyme that converts tyrosine into fumerate. Results in the accululation of homogentisic acid. Sx: Urine turns black on standing due to homogenticic oxidation, large joint arthritis and black ochronotic pigmentation of collagen, cartilage, sclera and ear cartilege.

Front 380

Fanconi's syndrome

Back 380

Proximal tubular function of the kidney is impaired, resulting in decreased reabsorption. Results in glucosuria, phosphaturia, aminoaciduria, and bicarb wasting. The reduced reabsorption of bicarbonate results in Type 2 (proximal) renal tubular acidosis. Causes by cystinosis, Wilson's disease, galactosemia, glycogen storage diseases, fructose intolerance, lead poisoning or injesting expired tetracyclines

Front 381

Wilson's disease

Back 381

Inadequate copper excretion by the biliary system due to mutations in the copper transporting ATPase coded for by the ATP7B gene on chromosome 13. Hepatolenticular degeneration characterized by liver cirrhosis and neurologic symptoms (due to copper depositing in the caudate and putamen and causing atrophy of the basal ganglia) such as extrapyramidal movement disorders and psychiatric disorders. Copper deposition in Descemet's membrane in the cornea produces the pathognomonic Kayser-Fleisher rings which can be seen with slit-lamp examination of the eyes. Low serum ceruloplasmin levels and increased copper in the urine are also seen. Tx: penicilliamine

Front 382

Prussian blue staining

Back 382

Iron deposits in hemachromatosis

Front 383

Boerhaave syndrome

Back 383

Esophageal rupture due severe periods of retching. Seen in alcoholics

Front 384

MOA of chloramphenicol

Back 384

Inhibits ribosomal peptidyl transferase (which forms peptide bonds). Used for treatment of serious Salmonella infections

Front 385

MOA of tetracyclines

Back 385

Prevents binding of aminoacytl-tRNA to ribosomes. Used to treat Chlamydia, mycoplasma, H. pylori, rickettsia, brucella, vibrio, and acne

Front 386

MOA of Macrolides

Back 386

Interfere with translation translocation

Front 387

MOA of aminoglycosides

Back 387

Cause misreading at translation initiation. Used for treating gram negative rods

Front 388

Hepatoduodenal ligament

Back 388

Portion of the lesser omentum that connects the live to the duodenum. Contains the Portal triad (hepatic artery, portal vein and common bile duct) as well as their branches (r. and l. hepatic arteries, cystic artery, etc). May be compressed between thumb and index finger placed in the omental foramen (epiploic foramen of Winslow) to control bleeding. Connects the greater and lesser sacs.

Front 389

Gastrocolic ligament

Back 389

Connects the greater curvature of the stomach to the transverse colon. Part of the greater omentum. Contains the gastroepiploic artries.

Front 390

Gastrohepatic ligament

Back 390

Connects the liver to the lesser curvature of the stomach. Contains the right and left gastric arteries. Separates the right greater and lesser sacs. May be cut during surgery to access the lesser sac.

Front 391

Splenorenal ligament

Back 391

Connects the spleen to the posterior abdominal wall. Contains the splenic artery and vein.

Front 392

Gastrosplenic ligament

Back 392

Connects the greater curvature of the stomach to the spleen. Contains the short gastric arteries.

Front 393

Pneumonic to remember receptors and their G-protein classes

Back 393

QISS (kiss) and QIQ (kick) til you're SIQ (sick) of SQS (sex)
alpha 1- q (Vasoconstriction, mydriasis)
alpha 2- i (Decrease sympathetic outflow, decrease insulin release)
beta 1- q (Increase HR, increase contractility, renin release, lipolysis)
beta 2- q (Vasodilation, bronchodilation, increase HR, increase contractility, lipolysis, glucagon release, decrease uterine tone)
M1- q (CNS and enteric nervous system)
M2- i (Decrease heart rate and contractility of atria)
M3- q (Exocrine gland secretion, gut peristalsis, bladder contraction, bronchoconstriction, miosis, accomodation)
D1- s (Relaxes renal vascular smooth muscle)
D2- i (NT release)
H1- q (Nasal and bronchial mucus production, contracts bronchioles, pain and pruritus)
H2- s (Gastric acid secretion)
V1- q (Vasoconstriction)
V2- s (H2O permiability and reabsorption in the CTs of the kidney)

Front 394

Maternal diabetes

Back 394

Macrosomia and transposition of the great arteries

Front 395

Ectopic ductal tissue

Back 395

Coarctation of the aorta

Front 396

Tetralogy of Fallot

Back 396

VSD, overriding aorta, pulmonic stenosis, and right ventricular hypertrophy

Front 397

Endocardial cushion defect

Back 397

Failure of neural crest cells to migrate. Common in Downs.

Front 398

Scabies

Back 398

Transmissible ectoparasitic infection from the mite Sarcoptes scabei. The female mite burrows into the stratum corneum to lay her eggs and itchy papules on the wrists, elbows, web spaces of the fingers, buttocks, and the groin form as a result of type IV hypersensitivity to the female mite, her feces, or her eggs. Diagnosis is made by finding the burrow, scraping its edges, covering the scrapings with oil and and examining the scrapings under a microscope. Treat with topical permethrin cream. Sulfer in petrolatum can be used in pregnancy.

Front 399

VDRL

Back 399

Venereal disease research laboratory. Serology test for syphilis. A non-treponemal test that detects antibodies that cross react with a mamallian cardiolipid called reaginic antibodies. This test will become positive after the specific test and its titer will fall late in infection, with or without treatment

Front 400

FTA-ABS

Back 400

Fluorescent treponemal antibody-absorbed test is a specific serologic test for syphilis in which the organism is labeled with fluorescent antibodies. The levels begin to rise early in titer and stay elevated throughout the lifetime of the host or at least until long after drug therapy has been completed

Front 401

Total lung capacity (TLC)

Back 401

Inspiratpry reserve capacity (IRC) + functional residual capacity (FRC)

Front 402

Myotonic dystrophy

Back 402

Trinucleotide expansion of CTG. Chronic, slowely progressive, multisystem disease primarily characterized by muscle wasting. Associated with cataracts and heart conduction defects.

Front 403

22q11 deletion

Back 403

Variable presentation including Velocardiofacial syndrome (palate, facial and cardiac defects) and DiGeorge syndrome

Front 404

Williams syndrome

Back 404

Deletion of the long arm of chromosome 7 which includes the elastin gene. "Elfin" facies, mental retardation but well-developed verbal skills, cheerful disposition, extreme friendliness with strangers, and cardiovascular problems.

Front 405

Cri-du-chat syndrome

Back 405

Deletion of the long arm of chromosome 5. Microcephaly, moderate to severe mental retardation, high pitched crying/ mewing, epicanthal folds, cardiac abnormalities

Front 406

Consanguinity

Back 406

Autosomal recessive condition acuired by a brother and sister mating

Front 407

Staghorn struvite stones

Back 407

Large renal stones composed of struvite or magnesium ammonium phosphate that can fill the renal pelvis. Since ammonium comes from the hydrolysis of urea, repeated infections with urease + bacteria such as Proteus can cause these stones.

Front 408

gamma delta T-cells

Back 408

Do not require selection in the thymus. Have a T-cell receptor that contains gamma and delta chains instead of alpha and beta chains. Make up the majority of the cells in the submucosa and intraepithelial spaces and is hypothesized that they play a role against a limited repertoire of antigens that enter the body across epithelial barriers.

Front 409

Maintenance dose equation

Back 409

MD = (clearance x plasma concentration at steady state)/ bioavailability

Front 410

Trousseau syndrome

Back 410

Migratory thrombophlebitis associated with pancreatic, lung, colon and gastric cancer. Due to tumor secretion of platelet aggregating factors and procoagulents.

Front 411

Heparin sulfate

Back 411

Negatively charged barrier in the basement membrane of the glomerulus

Front 412

Tubuloglomerular feedback mechanism

Back 412

NaCl activates the macula densa to constrict the afferent arteriole

Front 413

Myogenic response

Back 413

Increased blood pressure in the afferent arteriole causes vasoconstriction of the afferent arteriole

Front 414

Filtered load

Back 414

FL = GFR x plasma conc.

Front 415

Equation for renal clearance

Back 415

Clearance = (conc. in urine x urine flow)/ conc. in plasma
units: ml/min

Front 416

Normal GFR

Back 416

125 ml/min

Front 417

Normal RBF

Back 417

1.2 L/min

Front 418

Normal RPF

Back 418

600 ml/min

Front 419

PAH

Back 419

Used to estimate RPF
Underestimates by 10-15%

Front 420

Inulin

Back 420

Used to measure GFR bc it is freely filtered, not reabsorbed, not sercetes, not metabolised, and is biologically inert

Front 421

Creatinine

Back 421

Used to measure GFR. Slightly overestimates bc some is secreted

Front 422

Range of urine concentration

Back 422

50-1200 mOsm/kg H2O

Front 423

Asbestos

Back 423

Ferrugenous bodies ("dumb bells")

Front 424

Calculation for free water clearance

Back 424

1- (urine osmolality/ plasma osmolality) x vol of urine per unit time

Front 425

Calculation for free water reabsorption

Back 425

(urine osmolality/ plasma osmolality) - 1 x vol of urine per unit time

Front 426

Where is ADH made?

Back 426

Supraoptic and paraventricular nuclei of the hypothalamus

Front 427

What hormone stimulates the thirst center of the hypothalamus?

Back 427

Angiotensin II

Front 428

Structures of the anterior mediastinum

Back 428

Thymus

Front 429

Structures of the middle mediastinum

Back 429

Heart and the roots of the great vessels

Front 430

Structures of the posterior mediastinum

Back 430

Descending aorta, azygous and hemiazygous vein, the thoracic duct and the esophagus

Front 431

Structures of the superior mediastinum

Back 431

Ascending aorta, aortic arch, branches of the aortic arch, descending aorta, superior vena cava, thoracic duct, brachiocephalic veins, thymus, trachea and esophagus

Front 432

Neuropeptide Y

Back 432

Stimulates feeding. Secreted by the gut and hypothalamus

Front 433

IL-8

Back 433

Secreted by macrophages. Chemotactic for neutrophils

Front 434

IL-10

Back 434

Secreted by TH2 cells. Inhibits TH1 cells. Activates B-cells

Front 435

Osteosarcoma

Back 435

Malignant neoplasm of the bone. Predominently affects children and adolescents but a second peak of incidence occurs in middle age with predisposing factors such as Paget's disease, bone infarcts and ionizing radiation. 50% of osteosarcomas in children arise around the knee in the distal femur or proximal tibia. Osteoid matrix with an eosinophilic hyaline apperance

Front 436

Cells with Fc receptors

Back 436

Macrophages, neutrophils and eosinpohils

Front 437

Heterotopic rests

Back 437

Small areas of normal tissue in abnormal sites. Gastric heterotopias can occur in the small intestine and produce enough acid to cause an ulcer in adjacent tissues which can be a source of gastrointestinal bleeding

Front 438

Medial medullary syndrome

Back 438

Caused by occlusion of the anterior spinal artery. Contralateral hemiparesis of the lower extremities and trunk due to corticospinal tract involvement. Medial lemniscus involvement leads to diminished proprioception on the contralateral side and ipsilateral paralysis of the tongue is caused by damage to the hypoglossal nerve

Front 439

ELEK test

Back 439

Used to differentiate Corynebacterium diptheriae from normal oral flora. Involves diffusion in a gel and a precipitation reaction that will appear as white lines on the gel occurs when toxin produced by the pathogen precipitates with antitoxin from a soaked strip place on the gel. The organism aquires the diptheria toxin from lysogenic bacteriophage conversion.

Front 440

"Coin" lesions in the lung

Back 440

Adenocarcinoma, bronchioalveolar carcinoma, and large cell lung cancer

Front 441

Myeloid metaplasia with myelofibrosis

Back 441

A type of myeloproliferative disorder where the bone marrow space is replaced with fibrosis and hematopoiesis moves to extramedullary sites such as the liver and spleen causing hepatosplenomegaly. Can be preceeded by polycythemia vera or CML.

Front 442

Rubella

Back 442

Fine, non-blotchy rash that begins on the hairline and spreads to the torso. Cervical and auricular lymphadenopathy. NO Koplik spots on buccal mucosa. Child is not very ill.

Front 443

Congenital rubella

Back 443

Ocular problems (cataracts, retinopathy, microphthalmos, glaucoma), cardiovascular problems (PDA, VSD, pulmonic stenosis), deafness, thrombocytopenic purpura, hepatosplenomegaly, CNS problems and bony lesions

Front 444

Measles

Back 444

Blotchy, maculopapular, erythematous rash that begins on the face and spreads downward. Koplik spots can be seen on the buccal mucosa. Cough, coryza, conjunctivitis, high fever. Children are much sicker than they are with Rubella. Complications include pneumonia, encephalitis, SSPE

Front 445

Roseola

Back 445

Abrupt onset of URI symptoms and high fever. Fever falls rapidly then a fine macular rash develops on the trunk and spreads to the extremities

Front 446

Prosopagnosia

Back 446

The inability to recognize faces. Usually caused by a bilateral lesion of the visual association cortex.

Front 447

Agnosia

Back 447

The person has normal perception but the perception is devoid of meaning.

Front 448

Alexia

Back 448

Impairment in reading caused by defects in central language processing. Can occur in isolation or in conjunction with agraphia (due to damage to the splenum of the corus callosum caused by an infarction of the left posterior cerebral artery)

Front 449

Agraphia

Back 449

Impairment in writing

Front 450

Anosognosia

Back 450

A defect in cognition about one's illness or the lack of awareness that one is suffering from a condition. Related to large lesions in the nondominant parietal lobe which causes the patient to be confused and unaware of motor and sensory deficits

Front 451

Asomatognosia

Back 451

Lack of knowledge about one's body. Hemiasomatognosia can occur with hemineglect and pts deny that half of their body actually belongs to them.

Front 452

Confabulation

Back 452

Refers to the fabrication of stories and events that never happened in order to fill memory gaps. Seen mostly in dementia

Front 453

Ganser syndrome

Back 453

Refers to the phenomenon of giving approximate answers instead of exact ones. Associated with amnesia, disorientation, and perceptual disturbances. Seen most commonly in prison inmates

Front 454

Ideomotor apraxia

Back 454

The inablility to carry out an action after receiving a verbal command. Cannot imitate movements either

Front 455

Common variable immunodeficiency

Back 455

Normal levels of B and T cells. Decreased levels of plasma cells due to a defect in B-cell maturation so decreased levels of Igs. Presents in 20s and 30s

Front 456

Job's syndrome

Back 456

Failure of IFN-gamma production by helper T cells. Neutrophils are not attracted to infection sites. Presents with coarse facies, noninflammed staphlococcus abcesses, retained primary teeth, Increased IgE levels, and eczema

Front 457

Leukocyte adhesion deficiency syndrome

Back 457

Defect in LFA-1 integrin (CD18) proteins on phagocytes. Presents early with recurrent bacterial infections, absent pus formation, neutrophilia, and delayed separation of the umbilicus

Front 458

Chediak-Higashi syndrome

Back 458

AR disorder involving a defect in microtubular function and lysosomal emptying of phagocytic cells. Presents with recurrent pyogenic infections by staph and strep, partial albinism, and peripheral neuropathy

Front 459

Chronic mucocutaneous candidiasis

Back 459

T-cell dysfunction against Candida albicans

Front 460

Plummer-Vinson syndrome

Back 460

Atrophic glossitis, esophageal webs and iron deficiency anemia. Pts are at an increased risk of developing squamous cell carcinoma of the esophagus

Front 461

Hirchsprung disease

Back 461

Congenital cause of constipation caused by an absence of ganglion cells in both the submucosa and myenteric plexi of a segment of the bowel. The aganglionic bowel segment is narrowed because of the lack of peristalsis to keep stool moving from into the segment. The distal rectum is always involved and the lesion can extend proximally anywhere from a few centemeters all the way up to the small intestine. Males, who are most commonly affected, usually have a short segment nvolved with proximal megacolon. Long-segment aganglionosis still involving the rectum would be more likely seen in female pts. Rectal exam can dilate the narrowed segment and temporarily allow passage of stool. Tx involves resection of the affected segment of bowel.

Front 462

erb-B2

Back 462

HER2/nu. Associated with breast, ovarian and gastric carcinomas. Inhibited by Trastuzumab (herceptin)

Front 463

HIV encephalitis

Back 463

Cerebral atrophy with multifocal lymphohistiocytic infiltrates with numerous microglial nodules and scattered MULTINUCLEATED GIANT CELLS.

Front 464

CMV encephalitis

Back 464

Frequent opportunistic infection affecting AIDS pts. Found in ependymal and periventricular locations. Cytomegalic cells contain large purple intranuclear inclusions and granular cytoplasmic inclusions. Lymphocytic infiltration, microglial nodules, and neuronophagia.

Front 465

Vacuolar myelopathy

Back 465

A noninfectious complication of obscure pathogenesis involving the spinal cord. Very similar to subacute combined degeneration pathologically. May be related to vitamin B12 deficiency. Ascending sensory tracts in the posterior columns and descending motor tracts in the lateral columns degenerate leading to sensory loss, ataxia, and spastic paraplegia

Front 466

Thyroid (follicular) adenoma

Back 466

Common benign lesion consisting of a single large nodule of thyroid tissue the histology of which is similar to normal thyroid tissue. The nodule is deliminated by bands of fibrous tissue. Pts are usually euthyroid.

Front 467

Diffuse nontoxic goiter and multinodular goiter

Back 467

Other benign masses in the thyroid

Front 468

Thyroid cyst

Back 468

Benign cystic space that develops in some thyroids

Front 469

t(15;17)

Back 469

AML M3. Auer rods. Treated with all-trans retinoic acid.

Front 470

t(4;11)

Back 470

ALL

Front 471

t(6;9)

Back 471

AML with basophilia (M1, M2, and M4)

Front 472

t(8;21)

Back 472

AML M2 and M4

Front 473

Bullous pemphigoid

Back 473

Autoimmune disorder with IgG antibody against hemidesmosomes in the epidermal basement membrane. Similar to but less severe than pemphigus vulgaris. Affects skin but spares oral mucosa. Negative Nikolosky's sign (separation of epidermis upon manual stroking of the skin)

Front 474

Pemphigus vulgaris

Back 474

Potentiallt fatal autoimmune disorder with IgG antibodies to desmosomes in the epidermis. Intraepidermal bullae involving the skin and oral mucosa. Positive Nikolsky's sign (separation of the epidermis upon manual stroking of the skin). Mortality rate is 5-15%

Front 475

Dermatitis herpetiformis

Back 475

Pruritic papules and vesicles seen on the forearm of pts with celiac disease. Deposits of IgA at the tips of dermal papillae

Front 476

Lichen plantus

Back 476

Pruritic, purple, scaly, volaceous polygonal flat topped papules usually located on the wrists or oral mucosa of pts with hepatitis C. Sawtooth infiltrate of lymphocytes at the dermal-epidermal jxn

Front 477

Erythema nodosum

Back 477

Inflammatory lesions of subcutaneous fat usually on the anterior shins. Associated with coccidiomycosis, histoplasmosis, TB, leprosy, streptococcal infections, and sarcoidosis

Front 478

Acanthosis nigricans

Back 478

Verrucoid pigmented lesions commonly located in the axilla. Hyperplasia of the stratum spinosum. Associated with hyperlipidemia and visceral malignancies (MEN2b and stomach adenocarcinomas)

Front 479

Pityriasis rosea

Back 479

Single oval scaly plaque on the trunk ("herald patch") followed days later by a papular eruption on the trunk following lines of cleavage ("christmas tree distribution"). Remits spontaneously after 2-10 wks. Unknown cause.

Front 480

Hereditary angioedema

Back 480

Inadequate C1 esterase inhibitor activity. causes episodes of swelling (edema) in parts of the body. It is due to a genetic defect of chromosome 11.

Front 481

"Ground-glass" hepatocytes

Back 481

Seen in hepatitis B infection. Finely granular eosinophilic cytoplasm.

Front 482

Chronic active hepatitis

Back 482

Jaundice, periportal fibrosis (compared to bridging fibrosis in cirrhosis), ground-glass hepatocytes, necrosis of hepatocytes surrounding the portal tract (limiting plate) and necrosis extening into the lobule with individual hepatocyte distruction (piecemeal necrosis)

Front 483

Adenovirus infections

Back 483

Naked icosahedral DNA viruses. Cause pharyngoconjunctivitis. Can be aquired from contaminated swimming pools. Can also cause watery, non-bloody diarrhea.

Front 484

Atherosclerotic occlusive disease

Back 484

Intermittent claudication and impotence

Front 485

External otitis

Back 485

"Swimmer's ear". Inflammation of the outer ear and ear canal.

Front 486

Suprachiasmatic nucleus

Back 486

Responsible for controlling endogenous circadian rhythms, circadian body temperature, and nocternal secretion of melatonin by the pineal gland. It is pine cone shaped and smaller than a pea and interacts with many other regions of the brain. It contains several cell types and several different peptides (including vasopressin and vasoactive intestinal peptide) and neurotransmitters. The SCN is situated in the anterior hypothalamus immediately dorsal and superior (hence supra) to the optic chiasm on either side of the third ventricle.

Front 487

Locus coeruleus

Back 487

A nucleus in the brain stem located within the dorsal wall of the rostral pons in the lateral floor of the fourth ventricleinvolved with physiological responses to stress and panic. The principal site for synthesis of norepinephrine in the brain. Melanin granules inside the LC contribute to its blue color ("the blue spot"). The locus coeruleus is studied in relation to clinical depression, panic disorder, and anxiet and some antidepressant medications are believed to act on neurons in this area. This area of the brain is also intimately involved in REM sleep. The locus coeruleus is activated by stress, and will respond by increasing norepinephrine secretion, which in turn will increase cognitive function (through the prefrontal cortex), increase motivation (through nucleus accumbens), activate the hypothalamic-pituitary-adrenal axis, and increase the sympathetic discharge/inhibit parasympathetic tone (through the brainstem). Specific to the activation of the hypothalamo-pituitary adrenal axis, norepinephrine will stimulate the secretion of corticotropin-releasing factor from the hypothalamus, which induces adrenocorticotropic hormone release from the anterior pituitary and subsequent cortisol synthesis in the adrenal glands.Opioids inhibit the firing of neurons in the locus coeruleus. When opioid consumption is stopped, the increased activity of the locus coeruleus contributes to the symptoms of opiate withdrawal. The alpha2 adrenoceptor agonist clonidine is used to counteract this withdrawal effect by decreasing adrenergic neurotransmission from the locus coeruleus.

Front 488

Fabry's disease

Back 488

Due to the acculumlation of globosides due to deficient alpha-galactosidase A. X-LINKED. Reddish purple rash, peripheral neuropathy, angiokeratomas, CV and renal disease.

Front 489

Gaucher's disease

Back 489

Most common lysosomal storage disease. Accumulation of glucocerebroside due to a deficiency in beta-glucocerebrocidase (aka lysosomal hydrolase). AR. Hepatosplenomegaly, asceptic necrosis of the femur, bone crisis, Gaucher's cells (macrophages that look like crumpled tissue paper). Increased incidence in Ashkenazi Jews.

Front 490

Niemann-Pick disease A and B

Back 490

Accumulation of sphingomyelin due to a deficiency in sphingomyelinase. AR. Progressive neurodegeneration, hepatosplenomegaly, cherry-red spot on macula, foam cells. Increased incidence in Ashkenazi Jews.

Front 491

Tay-Sachs disease

Back 491

GM2 ganglioside accumulation due to a deficiency in hexoaminidase A. AR. Progressive neurodegeneration, developmental delay, cherry-red spot on macula, lysosomes with onion skin, and shell-like inclusion bodies. Increased incidence in Ashkenazi Jews.

Front 492

Krabbe's disease

Back 492

Accululation of galactocerebroside due to a deficiency in galactocerebrosidase. AR. Peripheral neuropathy, developmental delay, optic atrophy, globoid cells

Front 493

Metachromatic leukodystrophy

Back 493

Accumulation of cerebroside sulfate due to a deficiency in arylsulfatase A. SR. Central and peripheral demyelination with ataxia and dementia.

Front 494

Von Gierke's disease (type I glycogen storage disease)

Back 494

Deficiency in glucose-6-P. Cannot convert G-6-P released by glycogen phosphorylase into free glucose. Also cannot complete the last step in gluconeogenesis. AR. Severe fasting hypoglycemia, increased glycogen in liver, hepatomegaly, increased blood lactate

Front 495

Pompe's disease (type II glycogen storage disorder)

Back 495

Deficiency in lysosomal alpha-1,4-glucosidase (acid maltase). Leads to accululation of glycogen in the lysosome. Cardiomegaly and systemic findings leading to early death.

Front 496

Cori's disease (type III glycogen storage disease)

Back 496

Due to a deficiency in debranching enzyme. Milder from of type I (Von Gierke's) with normal blood lactate levels because gluconeogenesis is intact.

Front 497

McArdle's disease (type V glycogen storage disease)

Back 497

Deficiency in skeletal muscle glycogen phosphorylase. Relativelt benign. Liver glycogen is normal. Increased glycogen in muscle but cannot break it down leading to painful muscle cramps and myoglobinuria with strenuous exercise. No rise in blood lactate levels.

Front 498

Viruses with hemagglutin/ neuraminidase

Back 498

Measles- HA
Mumps- HA/ NM
Influenza- HA/ NM
Parainfluenza- HA/ NM

Front 499

Mammilary bodies

Back 499

Part of the hypothlamus/ limbic system. Involved with the processing of recognition memory and olfactory sensations.

Front 500

Limbic system

Back 500

A set of brain structures including the hippocampus, amygdala, anterior thalamic nuclei, mammilary bodies and limbic cortex, which support a variety of functions including emotion, behavior, long term memory, and olfaction

Front 501

Salmonella enteridis

Back 501

Osteomyelitis in sickle cell

Front 502

Examples of type IV hypersensitivity

Back 502

Type I diabetes, Hashimotos, Guillain-Barre, MS, GvHD, contact dermatitis

Front 503

Examples of type III hypersensitivity reactions

Back 503

PSGN, RA, SLE, hypersensitivity pneumonitis, Arthus reaction, glomerulonephritis

Front 504

Chancroid

Back 504

Sexually transmitted disease characterized by painful bleeding ulcers on the genitalia. Caused by Haemophilus ducreyi.

Front 505

Paternalism

Back 505

Any action, decision, rule, or policy made by a physician or other care-giver, or a government, that dictates what is best for the patient(s) without considering the patient’s own beliefs and value system and does not respect patient autonomy.

Front 506

HAV vaccine

Back 506

Live, attenuated virus. Activates CD4 T-cells to elicit an antibody response

Front 507

Actions of angiotensin II

Back 507

Acts at ATII receptors on vascular smooth muscle- Increases BP
Constricts efferent arterioles- Increases filtration fraction and GFR
Induces release of aldosterone
Induces release of ADH
Increases proximal tubule Na+/H+ activity- can lead to metabolic alkalosis
Stimulates thirst center in the hypothalamus
Increases PG release- Dialate afferent arterioles

Front 508

Atretic follicle

Back 508

Degenerated ovarian follicle

Front 509

Giemsa's stain

Back 509

Borrelia, Plasmodium, trypanosomes, Chlamydia

Front 510

India Ink

Back 510

Cryptococcus

Front 511

Silver stain

Back 511

Fungi, Legionella

Front 512

Chocolate agar with factors V (NAD) and X (hematin)

Back 512

H. influenzae

Front 513

Thayer-Martin media

Back 513

N. gonorrhoeae

Front 514

Bordet-Gengou (potato) agar

Back 514

B. pertussis

Front 515

Tellurite plate, Loffler's media

Back 515

C. diptheriae

Front 516

Lowenstein-Jensen agar

Back 516

M. tuberculosis

Front 517

Eaton's agar

Back 517

M. pneumoniae

Front 518

Eosin-methylene blue (EMB) agar (blue black colonies with metallic sheen)

Back 518

E. coli

Front 519

Pink colonies on MacConkey's agar

Back 519

Lactose fermenting enterics

Front 520

Charcoal yeast extract agar buffered with iron and cysteine

Back 520

Legionella

Front 521

Sabourand's agar

Back 521

Fungi

Front 522

Quelling reaction

Back 522

Test for encapsulated bugs. Will swell when anticapsular antiserum is added

Front 523

Protein A

Back 523

Staph aureus. Binds Fc region of Ig. Disrupts opsonization and phagocytosis.

Front 524

IgA protease

Back 524

S. pneumoniae, H. influenzae, Neisseria

Front 525

M protein

Back 525

Group A strep. Helps prevent phagocytosis

Front 526

Superantigens

Back 526

Activate T-cells to release IFN-gamma and IL-2.
S. aureus- TSST-1, food poisoning, exfoliatin (scalded-skin syndrome)
S. pyogenes- ethrogenic toxin causes Scarlet fever

Front 527

ADP ribosylating A-B toxins

Back 527

C. diptheriae- inactivates EF-2
V. cholerae- ADP ribosylates Gs, stimulating AC, increasing cAMP, and increase pumping of Cl- into gut and decreasing Na+ absorption
E. coli- Heat-labile toxin stimulates AC. Heat-stable toxin stimulates GC.
B. pertussis- Inhibits Gi, increasing cAMP. Inhibits chemokine receptor causing lymphocytosis

Front 528

alpha toxin

Back 528

C. perfringens

Front 529

Edema factor

Back 529

B. anthracis. An AC- increases cAMP

Front 530

Shiga toxin

Back 530

Shigella and E. coli O157:H7. Cleaves host cell rRNA (inhibits production of the 60S ribosome). Also enhances cytokine release causing HUS.

Front 531

Streptolysin O

Back 531

S. pyogenes. Hemolysin. Antigen for ASO antibody

Front 532

Endotoxin (lipid A)

Back 532

Activates macrophages- IL-1 (fever), TNF (fever, hemorrhagic tissue necrosis), NO (hypotension)
Activates complement- C3a (hypotension, vascular permiability), C5a (neutrophil chemotaxis)
Activates Hageman factor (VII)- DIC

Front 533

Generalized transduction

Back 533

LYTIC phage cleaves bacterial DNA and packages parts of the DNA in the viral capsid.

Front 534

Specialized transduction

Back 534

LYSOGENIC phage incorporates viral DNA into bacterial genome. When phage DNA is excised, flanking bacterial genes may be excised with it.

Front 535

Transposition

Back 535

"Jumping" DNA segment can transfer genes from the genome to a plasmid which can be transferred to another bacteria.

Front 536

5 bacterial toxins aquired by specialized phage transduction

Back 536

ABCDE
ShigA-like toxin
Botulism toxin
Cholera toxin
Diptheria toxin
Erythrogenic toxin of S. pyogenes

Front 537

Marfan's syndrome

Back 537

Genetic condition caused by a mutation in the gene that encodes fibrillin. Pts are tall with long legs and tapering fingers. Laxity of joints os present. Chest and spinal cord deformities may be present. Most frequent CV anomalies are aortic incompetence, aortic dissection, and mitral valve prolapse. Characteristic ocular changes are ectopia lentis (dislocation of the lens), retinal tears and detachment and glaucoma. Most deaths are due to ruptures of aortic dissections.

Front 538

Congenital herpes simplex 2

Back 538

Neonatal encephalitis. Mortality rate is 65%. Sepsis, skin vescicles, conjunctivitis, seizures, cranial nerve palsies, lethargy and death.

Front 539

Congenital CMV

Back 539

"Blueberry-muffin" disease. Thrombocytopenic purpura, jaundice, hepatosplenomegaly, and intracerebral calcifications.

Front 540

Congenital toxoplasmosis

Back 540

Retinochoroiditis, hydrocephalus and intercerebral calcifications

Front 541

Adjuvent drugs for the treatment of EPSEs caused by antipsychotics

Back 541

anti-muscarinics

Front 542

Solar elastosis

Back 542

Seen in actinic keratosis

Front 543

Munro abcesses

Back 543

Psoriasis

Front 544

Elongated eosinophilic intracytoplasmic neuronal inclusions in the hippocampus and cerebellar cortex

Back 544

Negri bodies (rabies)

Front 545

Abnormally large cells with a large intranuclear purple inclusion and granular basophilic inclusions in the cytoplasm

Back 545

Cytomegalic cells (CMV encephalopathy)

Front 546

Hemorrhage necrosis of the temporal lobes

Back 546

Herpes encephalitis

Front 547

Normal vaginal flora of young children and postmenopausal women

Back 547

Staphylococcus epidermidis

Front 548

Normal vaginal flora of women in childbearing years

Back 548

Lactobacillus, Candida and Steptococcus species

Front 549

Bizarre pleomorphic tumor cells accompanied by necrosis and hemorrhage

Back 549

Glioblastoma multiforme
In cerebral cortex of adults
In brainstem of children

Front 550

Papillary masses with perivascular rosettes

Back 550

Ependymoma
Commonly arise in children in the fourth ventricle

Front 551

Secondary syphilis

Back 551

Bronzing maculopapular rash that covers the entire body including the soles of the feet, palms of the hands and mucous membranes. Condylomata lata- flat wartlike growths on the perineal skin. Initial test with VDRL. Confirm with FTA-ABS or microhemagglutinin.

Front 552

Diagnostic test for tertiary syphilis

Back 552

Initial test with FTA-ABS. Confirm with microhemagglutinin.

Front 553

Sympathetic cholenergic neurons

Back 553

Sympathetic postganglionoc neurons that secrete Ach instead of NE. Innervate sweat glands.

Front 554

17q

Back 554

Breast and ovarian cancer (BRCA-1)

Front 555

3p

Back 555

von Hippel-Lindau disease (VHL gene)

Front 556

5q

Back 556

Colon cancer (APC gene)

Front 557

17p

Back 557

Li-Fraumeni syndrome (p53)

Front 558

18q

Back 558

Pancreatic cancer (DPC)

Front 559

Equation relating MAP, TPR, and CO

Back 559

MAP = CO x TPR
"ABC" rule:
pressure (P) = flow (Q) x resistence (R)

Front 560

Reactive systemic amyloidosis

Back 560

Secondary amyloidosis. Cytokines released in inflammatory conditions cause the liver to produce and secrete serum amyloid-associated (SAA) protein. SAA is enzymatically degraded into amyloid-associated (AA) protein and deposits in the kidneys, spleen, liver, heart and GI tract

Front 561

Russel bodies

Back 561

Accumulations of Ig in the plasma cells of multiple myeloma

Front 562

Desmoplasia

Back 562

Excessive fibrous tissue formation in the stroma of a tumor

Front 563

Sirolimus

Back 563

Inhibits T-cell proliferation by binding a specific serine-threonine kinase (mTOR) that is necessary for cell cycle progression. Approved for use in renal transplantation.

Front 564

Tacrolimus

Back 564

Fungal derived macrolide that inhibits calcineurin-mediated transcription of IL-2, thereby inhibiting T-cell activation

Front 565

I-cell disease

Back 565

A genetic disease that results from an abnormal N-acetyl-glucoseaminotransferase that is not able to add the necessary recognition marker mannose phosphate to enzymes destined to enter the lysosome. A complete deficiency in this enzyme results in death early in life while partial deficiencies produce a milder disease (pseudo-Hurler syndrome). Sx include "gargoyle facies", hepatomegaly, and multiple skeletal, cardiac, and CNS malformations. Serum levels of various enzymes will be elevated.

Front 566

Enterococcus faecalis

Back 566

A gram positive, catalase negative bacteria that grows on bile-esculin and does not produce nitrite from nitrate but nonetheless causes UTIs. Dipstick test will be negative.

Front 567

Monckeberg arteriosclerosis

Back 567

aka medial calcific sclerosis. A disease of the elderly in which ring-like calcifications form in the media of medium to small muscular arteries. Not a vasculitis because no inflammation occurs. Not clinically significant because there is no narrowing of the vessel lumen.

Front 568

Cystic medial necrosis

Back 568

Secondary to Marfans or hypertension. Predisposes for dissecting aortic anurysms. Small cleftlike separations between elastic and and fibromuscular elements filled with extracellular matrix of connective tissue without inflammation.

Front 569

Wegener granulomatosis

Back 569

Medium and large cell vasculitis characterized by focal necrotizing vasculitis and necrotizing granulomas. Affects the lungs, upper airways and kidneys. Often occurs in middle aged males who present with bilateral pulmonary nodules and cavitary lesions. Pts may also have chronic sinusitis, nasopharyngeal ulcerations and focal necrotizing glomerulonephritis.

Front 570

Psammoma bodies

Back 570

Small, laminated, concentric, calcific spherules seen in papillary thyroid cancer, serous papillary cystadenocarcinoma of the ovary, meningioma and malignant mesothelioma

Front 571

Malignant mesothelioma

Back 571

Due to asbestos exposure. Malignant cells develop in the mesothelium, a protective lining that covers most of the body's internal organs. Its most common site is the pleura but it may also occur in the peritoneum, the heart, the pericardium, or tunica vaginalis.

Front 572

von Hippel-Lindau disease

Back 572

Autosomal dominant disorder characterized by retinal angiomas, capillary hemangioblastomas of the cerebellum, renal carcinomas, and angiomatous or cystic lesions of the kidneys, liver, pancreas, lung, skin and epididymis. Hemangioblastomas often produce erythropoietin and therefore lead to polycythemia.

Front 573

Mycosis fungoides

Back 573

A cutaneous T-cell lymphoma in which the malignant cells show trophism for the epidermis. These cells invade the epidermis and superficial dermis producing red, scaly plaques and nodules

Front 574

Which band of the sarcomere does not shorten with contraction and contains only myosin?

Back 574

A band

Front 575

Which band of the sarcomere contains only actin filaments?

Back 575

I band

Front 576

Decidualized stroma with inactive glands

Back 576

Histological apperance of the endometrium of a person on birth control. Have asynchronous glands and stroma.

Front 577

Asynchronous secretory endometrium

Back 577

Mismatch of 2 or more days between the age of the glands and the stroma. Causes dysfunctional ovulatory bleeding and presents with infertility. Usually due to an inadequate luteal phase bc the corpus luteum is not producing enough progesterone even though ovulation has occured.

Front 578

Proliferative endometrium with stromal breakdown

Back 578

Associated with anovulation. Estrogenic stimulation causes the endometrium to proliferate but a lack of progesterone prevents desidualization. The endometrium eventually breaks down and bleeds even though there is no secretory change present and the stroma is not menstrual.

Front 579

Occludin

Back 579

A transmembrane linker protein in tight junctions

Front 580

Reid index

Back 580

The ratio of the mucous gland depth to the total thickness of the bronchial wall. Increased in pts with chronic bronchitis.

Front 581

Bitemporal hemanopsia

Back 581

A type of partial blindness where vision is missing in the outer half of both the right and left visual field (e.g. no peripheral vision). It is usually associated with lesions of the optic chiasm

Front 582

Lower homonymous quadrantanopsia

Back 582

Loss of visual input from the contralateral lower quarter of the visual field. Caused by lesions in the parietal (medial) optic radiations.

Front 583

Upper homonymous quadtranaopsia

Back 583

Loss of visual imput from the contralateral upper quarter of the visual field. Caused by lesions in the temporal (lateral) optic radiations of Meyer's loop. Can be caused by occlusion of a branch of the middle cerebral artery.

Front 584

Contralateral homonymous hemianopsia

Back 584

Cortical lesions. Usually spares the macula

Front 585

Homonymous hemanopsia

Back 585

A type of partial blindness resulting in a loss of vision in the same visual field of both eyes. Lesions of the optic tract, optic rediations and lateral geniculate body of the thalamus. Associated with occlusion of a branch of the posterior cerebral artery.

Front 586

Arnold-Chiari type 1 malformation

Back 586

More frequent than type 2. Cerebellar tonsils herniate through the foramen magnum. Frequently asyptomatic and an incidental finding autopsy finding. If severe, can obstruct CSF flow and lead to hydrocephalus

Front 587

Dandy-Walker malformation

Back 587

Failure of the cerebellar vermis to form. Abnormally large posterior fossa. The vermis is replaced by a large emendyma-lined midline cyst that represents an expanded 4th ventricle. Brain-setm nuclei are also abnormal.

Front 588

Medulloblastoma

Back 588

Tumor arising from the cerebellar vermis in children. Composed of small, primitive-appearing neoplastic cells

Front 589

Mean circulatory filling pressure (MCFP)

Back 589

The pressure that exists in all parts of the circulation when the heart has been stopped and the blood volume has become redistributed in the system until all pressures are at an equilibrium

Front 590

Lymphatic drainage of colon (rectal) cancer

Back 590

Above the pectineal line- internal iliac
Below the pectineal line- superficial inguinal

Front 591

Tartrate-resistant acid phosphatase

Back 591

Marker of osteoclastic activity

Front 592

Medial umbilical ligaments

Back 592

Derivatives of the umbillical arteries that branch off the internal iliac arteries

Front 593

Cell types that derive from neural crest origin

Back 593

Pseudounipolar cells of spinal and cranial nerve ganglia, Schwann cells, multipolar ganglion cells of autonomic ganglia, chromaffin cells of the adrenal medulla, ondontoblasts, melanocytes, and cells that make up the leptomeminges (pia and arachnoid)

Front 594

Postrenal azotemia

Back 594

High ratio of serum urea nitrogen/ serum creatinine ratio (>12-20) with an elevated creatinine. Due to postrenal obstruction or prerenal azotemia superimposed on renal disease

Front 595

Prerenal azotemia

Back 595

High serum urea nitrogen/ serum creatinine ratio (>12-20) with normal creatinine levels. Due to CHF.

Front 596

Causes of low serum urea nitrogen/ serum creatinine ratio

Back 596

Acute tubular necrosis, low protein intake or severe liver disease

Front 597

Acidophils of the pituitary

Back 597

Take up eosin dye. somatotrophs (secrete GH) and mammatrophs (secrete prolactin)

Front 598

Basophils of the pituitary

Back 598

Take up hematoxylin dye. Corticotrophs (secrete ACTH), gonadotrophs (secrete LH and FSH), and thyrotrophs (secrete TSH)

Front 599

Drugs for open angle glaucoma

Back 599

Dorzolamide, Epinephrine, Latanoprost, and Timolol

Front 600

Drugs for closed angle glaucoma

Back 600

Acetazolamide

Front 601

Congenital chlamydial infection

Back 601

Conjunctivitis, pneumonitis, and eosinophilia

Front 602

Tumors of the endometrium

Back 602

Leiomyoma (fibroid)- most common of all tumors in women. Benign neoplasms that develop from the smooth muscle cells of the myometrium and are estrogen dependent. Can cause abnormal uterine bleeding. Well demarcated with a whorled pattern

Front 603

Meigs syndrome

Back 603

Unusual combination of hydrothorax (often right sided), acites, and ovarian fibroma. Low back pain and a stretching or pulling sensation in the groin are characteristic

Front 604

Reye syndrome

Back 604

Fatty liver with encephalopathy. Seen in children. Precipitated by varicella or influenza infections or aspirin use.

Front 605

Preeclampsia before week 20

Back 605

Hyadidiform mole

Front 606

Glucagon excess

Back 606

Sx like DM, migratory erythematous skin rashes, alopecia, hyperpigmentation of the skin and glossitis. Due to pancreatic cancer.

Front 607

P450 Inducers

Back 607

Phenytoin, Rifampin, Griseofulvin, Phenobarbitone

Front 608

P450 Inhibitors

Back 608

Macrolides (Erythromycin, Clarythromycin), Ketoconazole, Cyclosporin, HIV protease inhibitors, grapefruit juice

Front 609

Number needed to harm

Back 609

1/ absolute risk increase

Front 610

Methacholine

Back 610

Test to induce asthma. Muscarinic agonist that causes bronchoconstriction

Front 611

Bone resorption

Back 611

RANK-ligand (produced by osteoblasts) interacts with RANK receptors on osteoclasts and along with monocyte colony-stimulating factor (also produced by osteoblasts) activate osteoclasts. Secretion of RANK-ligand and monocyte colony stimulating factor is stimulated by PTH

Front 612

Bone building

Back 612

OPG secreted by osteoblasts decreases RANK-ligand interaction with osteoclast RANK receptors and inactivates osteoclasts.

Front 613

Path of the ureters

Back 613

From the kidney, travel under the gonadal vessels and course through the uterosacral ligament medial to the gonadal vessels and lateral to the internal iliac vessels

Front 614

t(11;14)

Back 614

Mantle cell lymphoma

Front 615

t(14;18)

Back 615

Follicular lymphoma

Front 616

bcl-2

Back 616

Follicular lymphoma

Front 617

17q

Back 617

NF-1 (frameshift mutation resulting in a truncated ras protein)

Front 618

Deletion on chromosome 11

Back 618

Wilms tumor

Front 619

Deletion on chromosome 7

Back 619

Williams syndrome

Front 620

Chromosome 11

Back 620

Ataxia-telangictasia (DNA repair enzyme defect)

Front 621

Effect of disease prevalence on PPV and NPV

Back 621

PPV increases with an increase in prevalence
NPV increases with a decrease in prevalence

Front 622

Power

Back 622

Power = 1- beta
beta is a type II error (stating that there is no difference between groups when one truely exists)

Front 623

Subperiosteal thinning

Back 623

Hyperparathyroidism

Front 624

Fenlodopam

Back 624

D1 receptor agonist used in hypertensive crises. Causes arteriolar dilation and improves renal profusion.

Front 625

Metabolism of very long chain FAs and FAs with branch points at odd numbered carbons

Back 625

In peroxysomes

Front 626

Treatment to prevent meningitis in contacts

Back 626

Rifampin

Front 627

Hemibalism

Back 627

Wild flinging movements on one side of the body. Due to damage of the subthalamic nucleus that can occur with a lacunar stroke 2/2 to HTN

Front 628

DA agonists

Back 628

Bromocriptine, pergolide, pramipexole and ropinerole

Front 629

Equation for filtration fraction

Back 629

FF = GFR/ RPF

Front 630

alpha-glucosidase inhibitors

Back 630

Acarbose and Miglitol. Used in DM2. Decrease the absorption of sugar by inhibiting disaccharidases in the brush border

Front 631

Amlodipine

Back 631

Calcium channel blocker used in HTN that can lead to flusking and peripheral edema

Front 632

Foot drop

Back 632

Damage to the common peroneal nerve

Front 633

Classifications of AML

Back 633

M0- Minimally differentiated and M1- AML w/o differentiation- few or no Auer rods
M2- AML w/ maturation- Auer rods, peroxidase +, t(8;21)
M3- Acute promyelocytic leukemia- Many Auer rods, peroxidase +, t(15;17), young, DIC
M4- Acute myelomonocytic leukemias and M5- Acute monocytic leukemia- monoblasts and promonocytes, peroxidase -, nonspecific esterase +, poor prognosis
M6- Acute erythroleukemia- PAS + erythroblasts
M7- Acute megakaryocytic leukemia- platelet peroxidase +, rare, poor prognosis

Front 634

13q deletion

Back 634

CLL

Front 635

Tx of acute mania

Back 635

Mood stabilizer (lithium, valproate, or carbamazepine) + an atypical antipsychotic (olanzapine)

Front 636

Neurofibromas

Back 636

Tumors of Schwann cells which are derived from the neural crest. Cutaneous neurofibromas are skin-colored or pink nodules with a rubbery texture that "buttonhole" (push down through the skin with gentile pressure)

Front 637

2 standard deviations of the mean

Back 637

95%

Front 638

Nephrotic syndrome

Back 638

Massive proteinuria, hypoalbuminemia, generalized edema, hyperlipidemia and lipiduria

Front 639

Craniopharyngiomas

Back 639

Calcified cystic tumors filled with thick brown fluid rich in cholesterol

Front 640

Flumazenil

Back 640

Used for benzodiazepine overdose

Front 641

95% confidence interval

Back 641

Mean +/- 1.96SD/ square root of n

Front 642

99% confidence interval

Back 642

Mean +/- 2.58SD/ square root of n

Front 643

Clozapine

Back 643

Atypical antipsychotic. Acts on D4 receptors. ADR: agranulocytosis

Front 644

Pulsus paradoxis

Back 644

Radial pulse disappears during inspiration (systolic pressure decreases 20mmHg or more during inspiration as compared to expiration). Associated with cardiac tamponade

Front 645

Lambert-Eaton myasthenic syndrome (LEMS)

Back 645

Disorder of the neuromuscular jxn manifesting with muscle weakness in the form of gait alteration, difficulty arising from a chair or climbing stairs, and ptosis. Caused by antibodies against Ca++ channels on the presynaptic terminal that participate in Ach release. Half of pts with LEMS have a malignancy, classically small cell carcinoma

Front 646

TCA overdose

Back 646

Quinidine-like effect on cardiac conduction causing QRS and QT prolongation and arrythmias. Due to inhibition of fast Na+ channels. Treat with sodium bicarbonate

Front 647

Neurophysins

Back 647

Carrier proteins for oxytocin and ADH produced in the paraventricular and supraoptic nuclei of the hypothalamus and released by the posterior pituitary

Front 648

Aldolase reductase

Back 648

Converts glucose into sorbitol

Front 649

Sorbitol dehydrogenase

Back 649

Converts sorbitol into fructose. In DM, sorbitol bulids up in the lens, retina and Schwann cells which have low activities of sorbitol dehydrogenase. Osmotic influx of water into these structures causes cellular damage and contributes to the neuropathy, retinopathy and cataracts seen in DM

Front 650

Acute interstitial nephritis (AIN)

Back 650

Increased BUN and creatinine with eosinophilia. Caused by drug hypersensitivity and/or toxicity (penicillins and cephalosporins, NSAIDs, and sulfonamides

Front 651

Complication of chronic lymph edema

Back 651

Angiosarcomas

Front 652

Phlegmasta alba dolens

Back 652

Painful white leg, "milk leg". Consequence of iliofemoral venous thrombosis occuring in peripartum women. Venous statis is due to the pressure of the uterus on the deep pelvic veins

Front 653

Lupus anticoagulent

Back 653

anti-phospholipid antibodies produce a hypERcoaguable state. Can react with cardiolipin producung a false VDRL. Associated with venous and arterial thromboembolism and fetal loss.

Front 654

Cilostazol and Dipyridamole

Back 654

PDE inhibitors used in pts with intermittent claudication. Cilostazol also dilates arterioles

Front 655

Pancoast syndrome

Back 655

Caused by a tumor on the apex of the lung in the superior sulcus (groove fromed by the subclavian vessels). Can cause pain in the sholder region that radiates to the axilla and scapula and arm paresthesias, weakness and muscle atrophy (due to compression of the lower brachial plexus), Horner's syndrome due to involvment of the cervical sympathetic ganglia, compression of the subclavian vessels causing edema of the upper extremity and extension of the tumor into the intervertebral foramina leading to spinal cord compression and paraplegia

Front 656

Mucicarmine stain

Back 656

Stains the polysaccharide capsule of Cryptococcus red

Front 657

Ticlopidine

Back 657

Like clopedogrel

Front 658

Hashimoto's thyroiditis

Back 658

Type IV hypersensitivity destruction of the thyroid gland leading to hypothyroidism. Mononuclear infiltration conststing of lymphocytes and plasma cells. Several germinal centers are often present. Thyroid follicular epithelial cells undergo a metaplastic change leading to the formation of large, oxyphilic cells with granular cytoplasm called "Hurthle cells"

Front 659

Hurthle cells

Back 659

Seen in Hastimoto's thyroiditis. Thyroid follicular epithelial cells undergo a metaplastic change leading to the formation of large, oxyphilic cells with granular cytoplasm

Front 660

Heteroplasmy

Back 660

Presence of both nromal and mutated mtDNA resulting in variable expression in mitochondrial inherited diseases

Front 661

Pleitropy

Back 661

1 gene has multiple phenotypes (ex: CF on lungs and pancreas)

Front 662

Locus heterogeneity

Back 662

Mutations at different loci can produce the same phenotype

Front 663

Uniparental disomy

Back 663

Offspring receive 2 copies of a chromosome from one parent with no copies from the other parent

Front 664

Mutation caused by trinucleotide repeat expansion

Back 664

Hypermethylation of DNA leading to gene inactivation

Front 665

I band

Back 665

Region of the sarcomere where actin does not overlap with myosin. Decreases in length during contraction.

Front 666

H band

Back 666

Distance between two actin filaments in a sarcomere. Decreases in length during muscle contraction.

Front 667

A band

Back 667

Length of the myosin molecules in the sarcomere. Does NOT decrease in length during muscle contraction

Front 668

Z line

Back 668

Line to which actin is attached

Front 669

M line

Back 669

Line to which myosin is attached

Front 670

Southwestern blotting

Back 670

Used to detect DNA binding proteins

Front 671

Polymyositis

Back 671

Immune mediated inflammatory myopathy possibly triggered by viral antigens. Muscle autoantibodies are taken up by macrophages and presented to CD4 T-cells which secrete IFN-gamma which activates macrophages and causes them to release proinflammatory cytokines such as IL-1 and TNF-alpha. Muscle cells also increase expression of MHC class I molecules which are recognized by CD8 T-cells and destroyed. Cellular infiltration of the endomysium is seen. Characterized by proximal muscle weakness, increased creatinine kinase and anti-Jo-1 antibodies. Associated with an increased risk of malignancy.

Front 672

anti-Jo-1 antibodies

Back 672

Polymyositis

Front 673

Polymyalgia rheumatica

Back 673

Bilateral stiffness of shoulders and pelvic girdle muscles, fever, weight loss and increased ESR. Associated with temporal arthritis

Front 674

Why is ampicillin included in tx of meningitis?

Back 674

Listeria

Front 675

Dx of central DI

Back 675

10% or greater increase in urine osmolality following SDH administration during a water deprivation challenge

Front 676

DX of complete central DI

Back 676

50% or greater increase in urine osmolality following SDH administration during a water deprivation challenge

Front 677

Periodic acid shiff (PAS) stain

Back 677

Stains unsecreted globules of alpha 1 anti-trypsin reddish pink in periportal hepatocytes

Front 678

Acetylcholinesterase in amniotic fluid

Back 678

NTDs

Front 679

Blood/gas partition coefficient

Back 679

Anesthesias are transfered to the brain only after the blood is saturated (the partial pressure of the anesthetic in the blood = the partial pressure in the brain). This is dependant on the solubility of the anesthetic in the blood. Anesthetics that are highly soluble have a high blood/gas partition coefficient and more of the gas can be taken up before the gas is saturated. If the anesthetic is poorly soluble it has a low blood/gas partition coefficient and the amount needed to saturate the blood is small and transfer to the brain occurs quickly (ex NO).

Front 680

Restrictive lung diseases

Back 680

Associated with decreased lung volumes and increased peak expiratory flow rates due to increased elastic recoil pressure and radial traction exerted on the conducting airways by the fibrotic lung.

Front 681

Isoniazid

Back 681

Chemically similar to pyridoxine (vitamin B6) and can compete with B6 in the synthesis of neurotransmitters causing peripheral neuropathy. Isoniazid also increases the excretion of B6.
B6 should be supplimented with isoniazid treatment.

Front 682

Bronchioalviolar carcinoma

Back 682

< 10% of lung cancers. Subtype of lung adenocarcinoma. Occurs in nonsmokers. Arises from the alveolar epithelium at the periphery of the lung. Well-differentiated, composed of tall, columnar cells that line the alveolar septa w/o vascular or stromal invasion. CXR shows pneumonia-like consolidation.

Front 683

Osler-Weber-Rednu syndrome

Back 683

Hereditary hemorrhagic telangictasia. Skin and mucosal telangictasias and severe nose bleeds

Front 684

IgA nephropathy

Back 684

Painless hematuria 2-3 days after a URI. Subsides and reoccurs every few months.

Front 685

Henoch-Schonlein Purpura

Back 685

IgA nephropathy accompanied by extra renal symptoms such as abdominal pain and purpuric skin lesions.

Front 686

Bug with which Guillain-Barre is most strongly associated

Back 686

Campylobacter jejuni

Front 687

Examples of type II hypersensitivity reactions

Back 687

Goodpastures, Dresslers syndrome, rheumatic fever, poststreptococcal glomerulonephritis, transfusion rxns, hemolytic disease of the newborn

Front 688

Caudal regression syndrome

Back 688

Agenesis of the sacrum and lumbar spine, flaccid paralysis of the legs, loss of ankle reflexes and urinary incontinence. Can range from anal atresia to sirenomelia (fusion of the legs. Related to poorly controlled maternal diabetes

Front 689

Adeno-to-carcinoma sequence

Back 689

Mutations of APC, K-ras, p53 and DCC

Front 690

Lead-time bias

Back 690

An artificial increase in survival time among tested pts who actually have an unchanged prognosis. Pts screened with a more sensitive test will appear to live longer only bc the disease was detected earlier. The overall length of time from disease onset to death remains the same.

Front 691

Acute hemolytic transfusion reaction

Back 691

Occurs within minutes of an unmatched blood transfusion. Chills, SOB, fever, hypotension, DIC, renal failure and hemoglobinuria.

Front 692

Hereditary non-polyposis colon cancer (HNPCC)

Back 692

aka Lynch syndrome. AD. Inherited mutation in a gene responsible for DNA mismatch repair. Predominantly right sided cancers

Front 693

Pure red cell aplasia (PRCA)

Back 693

Form of bone marrow failure characterized by marked hypoplasia of marrow erythroid elements in the setting of normal granulo- and thrombopoiesis. Can be caused by a thymoma.

Front 694

Coenzymes required by dehydrogenases

Back 694

CoA, FAD, lipoic acid, NAD, thiamine pyrophosphate

Front 695

Difference b/t systolic and diastolic heart failure

Back 695

Diastolic- Increases EDP only
Systolic- Increases EDP and EDV

Front 696

Ebstein's anomaly

Back 696

Caused by lithium exposure in utero. Characterized by apical displacement of the tricuspid valve leaflets, increased volume of the right ventricle and atrialization of the right ventricle

Front 697

Ortner syndrome

Back 697

Mitral stenosis causes left atrial dilation that impinges on the left recurrent laryngeal nerve causing hoarsness

Front 698

Sodium cyanide-nitroprusside test

Back 698

Cysteinuria

Front 699

Absolute risk reduction

Back 699

Event rate in the placebo group - event rate in the treatment group

Front 700

Important ADR of hydralazine and procanimide

Back 700

Lupus! Both drugs are N-acetylated

Front 701

Dimercaprol

Back 701

Antidote for mercury poisoning

Front 702

Subacute sclerosing panencephalitis (SSPE)

Back 702

Caused by the measels virus. Autoantibody destruction. 6 yr interval b/t infection and SSPE. Behavioral changes, mild intellectual deterioration, clumbsiness, jerky movements.

Front 703

11p

Back 703

Wilms tumor

Front 704

13q

Back 704

RB, osteosarcoma, and BRCA-2

Front 705

17q

Back 705

p53

Front 706

22q

Back 706

NF-2

Front 707

ADR of metformin

Back 707

Lactic acidosis

Front 708

Central pontine myelinolysis

Back 708

Demyelination of the central pons following overly rapid correction of severe hyponatremia

Front 709

single stranded positive sense RNA viruses

Back 709

Call Pico and Flo To Come Right away:
Calcivirus
Picornaviruses
Flavavirus
Togavirus
Coronavirus
Retrovirus
PEECoRnA viruses:
Polio
Entero
Echo
Coxsackie
Rhino
hepatitis A

Front 710

double stranded DNA viruses

Back 710

Poxvirus, herpesvirus, and Adenovirus

Front 711

double stranded RNA viruses

Back 711

Reovirus

Front 712

single stranded DNA viruses

Back 712

Parvovirus

Front 713

single stranded negative-sense RNA viruses

Back 713

Orthomyxoviruses

Front 714

Rosenthal fibers

Back 714

Pilocytic astrocytomas

Front 715

t(11;21)

Back 715

Ewings sarcoma

Front 716

1p deletion

Back 716

Leiomyosarcoma

Front 717

t(2;13)

Back 717

Alveolar rhabdomyosarcoma

Front 718

t(12;22)

Back 718

Clear cell sarcoma

Front 719

t(X;18)

Back 719

Synovial sarcoma

Front 720

Cowdry type A bodies

Back 720

Intranuclear inclusions seen in HSV infection

Front 721

Pentamidine

Back 721

Tx of pneumocystis carinii

Front 722

ADR of metformin

Back 722

Lactic acidosis. Contraindicated in pts with heart failure, liver disease, severe hypoxia, and renal insufficiency

Front 723

Contraindications for sulfonyluras

Back 723

Hepatic disfunction. (undergo hepatic clearance

Front 724

ADRs of Thiazolidinediones

Back 724

Liver toxicity, exacerbations of CHF and peripheral edema

Front 725

Congenital mumps

Back 725

Endocardial fibroelastosis, flattened trabeculae, stenosed valves, respiratory distress thrombocytopenia and splenomegaly

Front 726

Drug-induced lupus

Back 726

Procainamide, hydralazine, chlorpromazine, isoniazid, methyl dopa, and quinidine

Front 727

Methyl dopa

Back 727

A central alpha-2 receptor agonist that leads to alpha-2 receptor-negative feedback to the SNS allowing peripheral sympathetic nervous system tone to decrease. Used in HTN

Front 728

Lateral nucleus

Back 728

Induces eating when stimulated

Front 729

Ventromedial nucleus

Back 729

Inhibits eating when stimulated