Usmle

Front 1

Autosomal recessive diseases (11)

Back 1

1. CF 2. albinism 3. alpha 1 antitrypsin deficiency 4. phenylketonuria 5. thalassemias 6. sicke cell anemia 7. glycogen storage diseases 8. mucopolysaccharidoses (except Hunter's) 9. sphingolipidoses (except Fabry's) 10.infant polycystic kidney disease 11. hemochromatosis

Front 2

_____are associated with low golic acid intake during pregnancy.

Back 2

Neural tube defects

Front 3

90% of adult polycystic kidney disease cases are due to mutation in _____.

Back 3

APKD1 (on chromosome 16)

Front 4

95% of Down's syndrome cases are due to what?

Back 4

meiotic nondisjunction of homologous chromosomes (4% due to Robertsonian translocation and 1% due to Down mosaicism)

Front 5

A patent ductus arteriosus is maintained by what 2 things?

Back 5

PGE synthesis and low oxygen tension

Front 6

Abnormalities associated with Marfan's syndrome:

Back 6

1. Skeletal: tall with long extremities, hyperextensive joints, long tapering fingers and toes 2. Cardiovascular: cystic medial necrosis of the aorta, aortic incompetence, aortic dissection, aortic aneurysm, floppy mitral valve Ocular: subluxation of lenses

Front 7

Adult polycystic kidney disease is associated with what other diseases or disorders?

Back 7

polycystic liver disease BERRY ANEURYSMS mitral valve prolapse

Front 8

Babies with Fetal Alcohol Syndrome are at higher risk for developing what other problems?

Back 8

pre and postnatal developmental retardation microcephaly facial abnormalities limb dislocation heart and lung fistulas

Front 9

Becker's muscular dystrophy is due to____.

Back 9

dystrophin gene mutations (not deletions) Becker's is less severe.

Front 10

Besides pulmonary infections, what are some other consequences of CF?

Back 10

infertility in males fat-soluable vitamin deficiencies (A,D,E,K)

Front 11

Causes of female pseudohermaphroditism:

Back 11

excessive and inappropriate exposure to androgenic steroids during early gestation (i.e., congenital adrenal hyperplasia or exogenous administration of androgens during pregnancy)

Front 12

Characteristic murmur with a patent ductus arteriosus.

Back 12

continuous, 'machine-like'

Front 13

Characteristics of Adult polycystic kidney disease:

Back 13

always bilateral massive enlargement of kidneys due to multiple large cysts patients present with pain, hematuria, HTN, and progressive renal failure

Front 14

Characteristics of Duchenne's MD:

Back 14

onset before age 5 weakness begins in the pelvic girdle muscles and progresses superiorly pseudohypertrophy of calf muscles due to fibrofatty replacement of muscle cardiac myopathy use of Gower's maneuver

Front 15

Characteristics of female pseudohermaphroditism:

Back 15

ovaries present but external genitalia are virilized or ambiguous

Front 16

Characteristics of Fragile X syndrome:

Back 16

macro-orchidism (enlarged testes), long face with a large jaw, large everted ears, and autism

Front 17

Characteristics of Hereditary Sperocytosis:

Back 17

spheroid erythrocytes hemolytic anemia increased MCHC splenectomy is curative

Front 18

Characteristics of male pseudohermaphroditism:

Back 18

testes present, but external genitalia are female or ambiguous.

Front 19

Children may do this to increase venous return with R-to-L shunt.

Back 19

squat

Front 20

Compare the cholesterol levels of heterozygores and homozygotes with familial hyperchlosterolemia:

Back 20

Heterozygotes (1 : 500) cholest. levels around 300mg/dL Homozygotes (very rare) cholest. levels over 700 mg/dL.

Front 21

Complications associated with homozygous familial hypercholesterolemia:

Back 21

severe atherosclerotic disease early in life tendon xanthomas (classically in the Achilles tendon) Myocardial Infarction before age 20

Front 22

Congenital heart defects are often due to which infection?

Back 22

rubella

Front 23

Cri-du-chat syndrome results from a congenital deletion on which chromosome?

Back 23

short arm of chromosome 5 46 XX or XY, 5p-

Front 24

Define Meningocele:

Back 24

meninges herniate through spinal canal defect picture on p. 229

Front 25

Define Meningomyelocele:

Back 25

meninges and spinal cord herniate through spinal canal defect picture on p.229

Front 26

Define pseudohermaphroditism:

Back 26

disagreement between the phenotypic (external genitalia) and gonadal (testes vs. ovaries) sex.

Front 27

Define Spina bifida occulta:

Back 27

failure of bony spinal canal to close, but no structural herniation. (usually seen at lower vertebral levels) picture on p. 299 (2002 edition)

Front 28

Describe a true hermaphrodite:

Back 28

46 XX or 47 XXY both ovary and testicular tissue present; ambiguous genitalia

Front 29

Describe Eisenmenger's syndrome:

Back 29

Uncorrected VSD, ASD, or PDA leads to progressive pulm. HTN. As pulm. resistance increases, the shunt changes from L to R to R to L, which causes late cyanosis (clubbing and polycythemia).

Front 30

Does coarctation of the aorta affect males or females most commonly?

Back 30

3:1 males to females

Front 31

Down's syndrome is associated with increased or decreased levels of AFP?

Back 31

decreased

Front 32

Elevated ___ in amniotic fluid is evidence of a neural tube defect.

Back 32

AFP (alpha fetal protein)

Front 33

Explain the adult type of coarctation of the aorta and give some associated symptoms.

Back 33

aortic stenosis distal to ductus arteriosus (postductal) aDult is Distal to Ductus associated with notching of the ribs, hypertension in upper extremities, weak pulses in lower extermities (check femoral pulse)

Front 34

Explain the infantile type of coarctation of the aorta. What is it commonly associated with?

Back 34

aortic stenosis proximal to insertion of ductus arteriosus (preductal) 'INantile, IN close to the heart.' associated with Turner's syndrome

Front 35

Explain the pathogenesis of eisenmeinger's syndrome

Back 35

In fetal period, shunt is R to L (normal). In neonatal period, lung resistance decreases and shunt becomes L to R with subsequent RV hypertrophy and failure (abnormal).

Front 36

Explain the transposition of the great vessels.

Back 36

Aorta leaves RV (anterior) and pulmonaryt trunk leaves LV (posterior) this leads to separation of systemic and pulmonary circulations.

Front 37

Familial Adenomatous Polyposis features:

Back 37

Colon becomes covered with adenomatous polyps after puberty 'FAP' F= five (deletion on chromosome 5) A= autosomal dominant inheritance P= positively will get colon cancer (100% without resection)

Front 38

Findings in Von Recklinghausen's disease:

Back 38

café-au-lait spots, neural tumors, Lisch nodules (pigmented iris hamartomas), skeletal disorders (scoliosis), and increased tumor susceptibility

Front 39

Findings of Cri-du-chat syndrome:

Back 39

microcephaly, severe Mental Retardation, high pitched crying/mewing -(Cri-du-chat is French for cry of the cat), cardiac abnormalities

Front 40

Findings with Huntington's disease:

Back 40

depression progressive dementia choreiform movements caudate atrophy dec. levels of GABA and Ach in the brain

Front 41

Frequency of L-toR shunts:

Back 41

VSD>ASD>PDA

Front 42

Gender identity is based on what two things?

Back 42

1. external genitalia 2. sex of upbringing

Front 43

Genetic anticipation of Fragile X syndrome may be shown by what?

Back 43

Triplet repeat (CGG)n

Front 44

Highest risk of development of fetal alcohol syndrome at __ to ___ weeks.

Back 44

3 to 8

Front 45

How does CF present in infancy?

Back 45

Failure to thrive

Front 46

How is CF diagnosed?

Back 46

increased concentration of Cl- ions in sweat test

Front 47

How is MD diagnosed?

Back 47

muscle biopsy increased serum CPK

Front 48

Huntington's disease manifests between the ages of :

Back 48

20 and 50

Front 49

Incidence and characteristics and Turner's syndrome:

Back 49

1 in 3000 births short stature, ovarian dysgenesis, webbed neck, coarction of the aorta

Front 50

Incidence and characteristics of double Y males:

Back 50

1 in 1000 births phenotypically normal, very tall, severe acne, antisocial behavior (seen in 1-2%)

Front 51

Incidence and characteristics of Edward's syndrome:

Back 51

1 in 8000 births severe MR, rocker bottom feet, low-set ears, micrognathia, congenital heart disease, clenched hands (flexion of fingers), prominent occiput. Death usually occurs within 1 year of birth.

Front 52

Incidence and characteristics of Klinefelter's syndrome:

Back 52

1 in 850 births testicular atrophy, eunuchoid body shape, tall, long extremities, gynecomastia, female hair distribution

Front 53

Incidence and characteristics of Patau's syndrome:

Back 53

1 in 6000 births severe MR, microphthalmia, microcephaly, cleft lip/palate, abnormal forebrain structures, polydactly, congenital heart disease Death usually occurs within 1 year of birth.

Front 54

Marfan's is due a mutation in which gene?

Back 54

fibrillin

Front 55

Mechanism of Fetal Alcohol Syndrome may be :

Back 55

inhibition of cell migration

Front 56

Most common form of male pseudohermaphroditism is ____.

Back 56

testicular feminization (androgen insensitivity) results from a mutation in the androgen receptor gene (X linked recessive); blind-end vagina

Front 57

Name 3 examples of L-to-R shunts. (late cyanosis) 'blue kids'

Back 57

1. VSD (ventricular septal defect) 2. ASD (atrial septal defect 3. PDA (patent ductus arteriosus)

Front 58

Name 3 examples of R-to-L shunts. (early cyanosis) 'blue babies'

Back 58

The 3 T's' 1. Tetralogy of Fallot 2. Transposition of great vessels 3. Truncus arteriosus

Front 59

Name 7 common congenital malformations

Back 59

1. heart defects 2. Hypospadias (when the urethral canal is open on the undersurface of the penis or on the perineum) 3. Cleft lip w/ or w/out cleft palate 4. congenital hip dislocation 5. Spina Bifida 6. Anencephaly 7. Pyloric stenosis

Front 60

Name 8 autosomal-dominant diseases:

Back 60

1. Adult polycystic kidney disease 2. Familial hyperchloresterolemia (type IIA) 3. Marfan's syndrome 4. Von Recklinghausen's disease (NFT1) 5. Von Hippel-Lindau disease 6. Huntington's disease 7. Familial Adenomatous Polyposis 8. Hereditary Sperocytosis

Front 61

Name an X-linked recessive muscular disease that leads to accelerated muscle breakdown.

Back 61

Duchenne's Muscular Dystrophy

Front 62

Name the 4 components of Tetralogy of Fallot.

Back 62

PROVe' 1. Pulmonary Stenosis 2. RVH (right ventricular hypertrophy) 3. Overriding aorta (overrides the VSD) 4. VSD (ventricular septal defect)

Front 63

Neural tube defects (spina bifida and anencephaly) are associated with increased levels of ___ in the amniotic fluid and maternal serum.

Back 63

AFP (alpha fetal protein)

Front 64

Newborns of mothers who consumed significant amounts of alcohol (teratogen) during pregnancy are at risk for _______.

Back 64

fetal alcohol syndrome (the number one cause of congenital malformations in the U.S.)

Front 65

Patau's syndrome = trisomy ___

Back 65

13 (hint: Puberty - 13)

Front 66

Pathogenesis of Cystic Fibrosis:

Back 66

defective Cl- channel --> secretion of abnormally thick mucus that plugs lungs, pancreas, and liver --> recurrent pulmonary infections (Pseudomonas species and Staph aureus), chronic bronchitis, bronchiectasis, pancreatic insufficiency (malabsorption and steatorrhea), meconium ileus in newborns.

Front 67

Patients with Tetralogy of Fallot often suffer _________.

Back 67

cyanotic spells

Front 68

Pyloric stenosis is associated with______.

Back 68

polyhydramnios; projectile vomiting

Front 69

T/F: Trisomy 21 is associated with advanced maternal age.

Back 69

True (from 1 in 1500 births in women<20 to 1 in 25 births in women>45)

Front 70

Tetralogy of Fallot leads to early cyanosis from a R-to-L shunt across the ____.

Back 70

VSD

Front 71

The defect in Von Recklinghausen's disease is found on which chromosome?

Back 71

17 (hint: 17 letters in Recklinghausen's)

Front 72

The gene responsible for Huntington's disease is located on chromosome__.

Back 72

4 ; triplet repeat disorder

Front 73

The incidence of neural tube defects is decreased by maternal ingestion of what?

Back 73

folate

Front 74

Transposition of great vessels is a common congenital heart disease in offspring of _____ mothers.

Back 74

diabetic

Front 75

Transposition of great vessels is not compatible with life unless what is present?

Back 75

a shunt that allows adequate mixing of blood (VSD, PDA, or patent foramen ovale)

Front 76

Trisomy 18 is also known as______.

Back 76

Edward's syndrome (hint: Election age = 18)

Front 77

Turner's syndrome is the most common cause of what?

Back 77

primary amenorrhea

Front 78

Von Hippel-Lindau disease characteristics:

Back 78

hemangioblastomas of retina/cerebellum/medulla about half of affected individuals develop multiple bilateral renal cell carcinomas and other tumors

Front 79

Von Hippel-Lindau disease is associated with the deletion of what gene?

Back 79

VHL gene (tumor suppressor) on chromosome 3 (3p)

Front 80

What are some findings of Down's syndrome?

Back 80

mental retardation flat facial profile prominent epicanthal folds simian crease duodenal atresia congenital heart disease (most common malformation is endocardial cushion defect) Alzheimer's disease in individuals over 35 increased risk of ALL

Front 81

What does a heart with Tetralogy of Fallot look like on x-ray?

Back 81

boot-shaped due to RVH

Front 82

What gender genetic disorder has been observed with increases frequency among inmates of penal institutions?

Back 82

Double Y males (XYY)

Front 83

What is different about the juvenile form of polycystic kidney disease?

Back 83

it is recessive

Front 84

What is Gower's maneuver?

Back 84

requiring assistance of the upper extermities to stand up (indicates proximal lower limb weakness)

Front 85

What is the cause of Duchenne's MD?

Back 85

a deleted dystrophin gene (hint: 'D' for deletion)

Front 86

What is the cause of Tetralogy of Fallot?

Back 86

anterosuperior displacement of the infundibular septum

Front 87

What is the incidence of Down's syndrome?

Back 87

1 in 700 births

Front 88

What is the most common cause of early cyanosis?

Back 88

Tetralogy of Fallot

Front 89

What is the most common chromosomal disorder and cause od congenital mental retardation?

Back 89

Down's syndrome (trisomy 21)

Front 90

What is the most common congenital cardiac anomaly?

Back 90

VSD

Front 91

What is the most common lethal genetic disease of Caucasians?

Back 91

Cystic Fibrosis

Front 92

What is the second most common cause of mental retardation?

Back 92

Fragile X syndrome

Front 93

What is the underlying cause of Cystic Fibrosis?

Back 93

Autosomal - recessive defect in CFTR gene on chromosome 7

Front 94

What is the underlying cause of Familial hypercholesterolemia?

Back 94

defective or absent LDL receptor leading to elevated LDL

Front 95

What is used to close a patent ductus arteriosus? What is used to keep it open?

Back 95

Indomethacin PGE (may be necessary to sustain life in conditions such as transposition of the great vessels)

Front 96

Which gene is affected in Fragile X?

Back 96

methylation and expression of the FMR 1 gene is affected in this X-linked disorder

Front 97

Which genetic gender disorder has an inactivated X chromosome (Barr body)?

Back 97

Klinefelter's syndrome

Front 98

Why are female carriers of X-linked recessive disorders rarely affected?

Back 98

because of random inactivation of X chromosomes in each cell

Front 99

X-linked recessive disorders (10)

Back 99

1. Fragile X 2. Duchenne's MD 3. hemophilia A and B 4. Fabry's 5. G6PD deficiency 6. Hunter's syndrome 7. ocular albimism 8. Lesch-Nyhan syndrome 9. Bruton's agammaglobulinemia 10. Wiscott-Aldrich syndrome

Front 100

XO =

Back 100

Turner's syndrome (think: 'hugs and kisses' -XO- from Tina Turner)

Front 101

XXY =

Back 101

Klinefelter's syndrome one of the most common causes of hypogonadism in males

Front 102

Approximately what percentage of brain tumors arise from metastasis?

Back 102

0.5

Front 103

Are basal cell carcinomas invasive?

Back 103

Locally invasive but rarely metastasize

Front 104

Are Ewing's sarcomas likely to metastasize?

Back 104

Yes. They are extremely aggressive with early metastasis. However, they are responsive to chemotherapy.

Front 105

Are meningiomas resectable?

Back 105

Yes

Front 106

Are squamous cell carcinomas of the skin invasive?

Back 106

Locally invasive but rarely metastasize

Front 107

Are the majority of adult tumors supratentorial or infratentorial?

Back 107

Supratentorial

Front 108

Are the majority of childhood tumors supratentorial or infratentorial?

Back 108

Infratentorial

Front 109

Common histopathology often seen in squamous cell carcinomas of the skin?

Back 109

Keratin pearls

Front 110

Define anaplasia

Back 110

Abnormal cells lacking differentiation; like primitive cells of the same tissue. Often equated with undifferentiated malignant neoplasms. Tumor giant cells may be formed.

Front 111

Define dysplasia

Back 111

Abnormal growth with loss of cellular orientation, shape, and size in comparison to normal tissue maturation. It is reversible but is often a preneoplastic sign.

Front 112

Define hyperplasia

Back 112

Increase in the number of cells (reversible)

Front 113

Define metaplasia

Back 113

One adult cell type is replaced by another (reversible). It is often secondary to irritation and/or environmental exposure (e.g. squamous metaplasia in the trachea and bronchi of smokers)

Front 114

Define neoplasia

Back 114

Clonal proliferation of cells that is uncontrolled and excessive

Front 115

Do oncogenes cause a gain or loss of function?

Back 115

Gain of function

Front 116

Do tumor suppressor genes cause a gain or loss of function?

Back 116

Loss of function. Both alleles must be lost for expression of disease

Front 117

Does a melanoma have a significant risk of metastasis?

Back 117

Very significant risk! The depth of the tumor often correlates with the risk of metastasis.

Front 118

From what cells do meningiomas most commonly arise?

Back 118

Arachnoid cells external to the brain

Front 119

Give 2 examples of a benign tumor of epithelial origin.

Back 119

1. Adenoma 2. Papilloma

Front 120

Give 2 examples of a malignant tumor of mucosal epithelial origin.

Back 120

1. Adenocarcinoma 2. Papillary carcinoma

Front 121

Give 2 examples of malignant tumors of blood cell (mesenchymal) origin.

Back 121

1. Leukemia 2. Lymphoma

Front 122

Give an example of a benign tumor of blood vessel (mesenchymal) origin.

Back 122

Hemangioma

Front 123

Give an example of a benign tumor of bone (mesenchymal) origin.

Back 123

Osteoma

Front 124

Give an example of a benign tumor of more than one cell type.

Back 124

Mature teratoma

Front 125

Give an example of a benign tumor of skeletal muscle (mesenchymal) origin.

Back 125

Rhabdomyoma

Front 126

Give an example of a benign tumor of smooth muscle (mesenchymal) origin.

Back 126

Leiomyoma

Front 127

Give an example of a malignant tumor of blood vessel (mesenchymal) origin.

Back 127

Angiosarcoma

Front 128

Give an example of a malignant tumor of bone (mesenchymal) origin.

Back 128

Osteosarcoma

Front 129

Give an example of a malignant tumor of more than one cell type.

Back 129

Immature teratoma

Front 130

Give an example of a malignant tumor of skeletal muscle (mesenchymal) origin.

Back 130

Rhabdomysarcoma

Front 131

Give an example of a malignant tumor of smooth muscle (mesenchymal) origin.

Back 131

Leiomyosarcoma

Front 132

Give an example of a neoplasm associated with Down's Syndrome.

Back 132

Acute Lymphoblastic Leukemia (ALL)

Front 133

How are tumor markers used?

Back 133

Tumor markers are used to confirm diagnosis, to monitor for tumor recurrence, and to monitor the response to therapy. They should not be used as a primary tool for diagnosis.

Front 134

How is prostatic adenocarcinoma most commonly diagnosed?

Back 134

Digital rectal exam (detect hard nodule) or by prostate biopsy

Front 135

How often do primary brain tumors undergo metastasis?

Back 135

Very rarely

Front 136

In what population is osteochondroma most often found?

Back 136

Usually men under the age of 25

Front 137

In which age group is prostatic adenocarcinoma most common?

Back 137

Men over the age of 50

Front 138

Is malignant transformation in osteochondroma common?

Back 138

Malignant transformation to chondrosarcoma is rare

Front 139

Name 1 common tumor staging system.

Back 139

TNM system T= size of tumor, N=node involvement, and M=metastases

Front 140

Name 3 herniation syndromes that can cause either coma or death when the herniations compress the brainstem

Back 140

1. Downward transtentorial (central) herniation 2. Uncal herniation 3. Cerebellar tonsillar herniation into the foramen magnum

Front 141

Name 4 factors that predispose a person to osteosarcoma.

Back 141

1. Paget's disease of bone 2. Bone infarcts 3. Radiation 4. Familial retinoblastoma

Front 142

Name 4 possible routes of herniation in the brain

Back 142

1. Cingulate herniation under the falx cerebri 2. Downward transtentorial (central) herniation 3. Uncal herniation 4. Cerebellar tonsillar herniation into the foramen magnum

Front 143

Name 5 primary brain tumors with peak incidence in adulthood.

Back 143

1. Meningioma 2. Glioblastoma multiforme 3. Oligodendroglioma 4. Schwannoma 5. Pituitary adenoma

Front 144

Name 5 primary brain tumors with peak incidence in childhood.

Back 144

1. Medulloblastoma 2. Hemangioblastoma 3. Ependymomas 4. Low-grade astrocytoma 5. Craniopharyngioma

Front 145

Name 5 sites from which tumor cells metastasize to the brain.

Back 145

1. Lung 2. Breast 3. Skin (melanoma) 4. Kidney (renal cell carcinoma) 5. GI

Front 146

Name a common histopathological sign of basal cell carcinoma nuclei

Back 146

The nuclei of basal cell tumors have 'palisading' nuclei

Front 147

Name a population at a greater risk for melanoma.

Back 147

Fair-skinned people (blue eyes and red hair have also been considered as factors)

Front 148

Name the 5 primary tumors that metastasize to the liver

Back 148

1. Colon 2. Stomach 3. Pancreas 4. Breast 5. Lung

Front 149

Name two of the most common sites of metastasis after the regional lymph nodes

Back 149

The liver and the lung

Front 150

Name two presenting sequelae of a pituitary adenoma.

Back 150

1. Bitemporal hemianopsia (due to pressure on the optic chiasm) 2. Hypopituitarism

Front 151

On which chromosome is the p53 gene located?

Back 151

17p

Front 152

On which chromosome is the Rb gene located?

Back 152

13q

Front 153

On which chromosomes are the BRCA genes located?

Back 153

BRCA 1 is on 17q and BRCA 2 is on 13q

Front 154

Out of the 6 primary tumors that metastasize to bone, which two are the most common?

Back 154

Metastasis from the breast and prostate are the most common

Front 155

What 2 cancers are associated with EBV?

Back 155

1. Burkitt's lymphoma 2. Nasopharyngeal carcinoma

Front 156

What 2 neoplasms are associated with AIDS?

Back 156

1. Aggressive malignant lymphomas (non-Hodgkins) 2. Kaposi's sarcoma

Front 157

What 2 neoplasms are associated with Autoimmune disease (e.g. Hashimoto's thyroiditis, myasthenia gravis, etc.)?

Back 157

Benign and malignant thymomas

Front 158

What 2 neoplasms are associated with Paget's disease of bone?

Back 158

1. Secondary osteosarcoma 2. Fibrosarcoma

Front 159

What 2 neoplasms are associated with Tuberous sclerosis (facial angiofibroma, seizures, and mental retardation)?

Back 159

1. Astrocytoma 2. Cardiac rhabdomyoma

Front 160

What are 2 characteristic findings in carcinoma in situ?

Back 160

1. Neoplastic cells have not invaded the basement membrane 2. High nuclear:cytoplasmic ratio and clumped chromatin

Front 161

What are 2 characteristic findings of an invasive carcinoma?

Back 161

1. Cells have invaded the basement membrane using collagenases and hydrolases 2. Able to metastasize if they reach blood or lymphatic vessels.

Front 162

What are 2 neoplasms associated with Xeroderma pigmentosum?

Back 162

1. Squamous cell carcinoma of the skin 2. Basal cell carcinoma of the skin

Front 163

What are 3 disease findings associated with Alkaline Phosphatase?

Back 163

1. Metastases to bone 2. Obstructive biliary disease 3. Paget's disease of bone

Front 164

What are 6 primary tumors that metastasize to bone?

Back 164

1. Kidney 2. Thyroid 3. Testes 4. Lung 5. Prostate 6. Breast

Front 165

What are a common histopathological finding of meningiomas?

Back 165

Psammoma bodies. These are spindle cells concentrically arranged in a whorled pattern.

Front 166

What are ependymomas?

Back 166

Ependymal cell tumors most commonly found in the 4th ventricle. May cause hydrocephalus

Front 167

What are the steps in the progression of neoplasia?

Back 167

1. Normal 2. Hyperplasia 3. Carcinoma In Situ/Preinvasive 4. Invasion

Front 168

What are two signs of bone metastases in prostatic adenocarcinoma?

Back 168

An increase in serum alkaline phosphatase and PSA (prostate-specific antigen)

Front 169

What are two useful tumor markers in prostatic adenocarcinoma?

Back 169

Prostatic acid phosphatase and prostate-specific antigen (PSA)

Front 170

What can be associated with the risk of melanoma?

Back 170

Sun exposure

Front 171

What cancer is associated with HBV and HCV (Hep B and C viruses)?

Back 171

Hepatocellular carcinoma

Front 172

What cancer is associated with HHV-8 (Kaposi's sarcoma-associated herpes virus)?

Back 172

Kaposi's carcinoma

Front 173

What cancer is associated with the HTLV-1 virus?

Back 173

Adult T-cell leukemia

Front 174

What cancers are commonly associated with HPV (human papilloma virus)?

Back 174

Cervical carcinoma, penile, and anal carcinoma

Front 175

What causes the local effect of a mass?

Back 175

Tissue lump or tumor

Front 176

What causes the local effect of a nonhealing ulcer?

Back 176

Destruction of epithelial surfaces (e.g. stomach, colon, mouth, bronchus)

Front 177

What causes the local effect of a space-occupying lesion?

Back 177

Raised intracranial pressure in brain neoplasms. Also seen with anemia due to bone marrow replacement.

Front 178

What causes the local effect of bone destruction?

Back 178

Pathologic fracture or collapse of bone

Front 179

What causes the local effect of edema?

Back 179

Venous or lymphatic obstruction

Front 180

What causes the local effect of hemorrhage?

Back 180

Caused by ulcerated area or eroded vessel

Front 181

What causes the local effect of inflammation of a serosal surface?

Back 181

Pleural effusion, pericardial effusion, or ascites

Front 182

What causes the local effect of obstruction in the biliary tree?

Back 182

Jaundice

Front 183

What causes the local effect of obstruction in the bronchus?

Back 183

Pneumonia

Front 184

What causes the local effect of obstruction in the left colon?

Back 184

Constipation

Front 185

What causes the local effect of pain?

Back 185

Any site with sensory nerve endings. Remember that tumors in the brain are usually painless.

Front 186

What causes the local effect of perforation of an ulcer in the viscera?

Back 186

Peritonitis or free air

Front 187

What causes the local effect of seizures?

Back 187

Tumor mass in the brain.

Front 188

What causes the localized loss of sensory or motor function?

Back 188

Compression or destruction of nerve (e.g. recurrent laryngeal nerve by lung or thyroid cancer causes hoarseness)

Front 189

What causes the paraneoplastic effect gout?

Back 189

Hyperuricemia due excess nucleic acid turnover (secondary to cytotoxic therapy of various neoplasms)

Front 190

What causes the paraneoplastic effect of Cushing's disease?

Back 190

ACTH or ACTH-like peptide (secondary to small cell lung carcinoma)

Front 191

What causes the paraneoplastic effect of hypercalcemia?

Back 191

PTH-related peptide, TGF-a, TNF-a, IL-2 (secondary to squamous cell lung carcinoma, renal cell carcinoma, breast carcinoma, multiple myeloma, and bone metastasis)

Front 192

What causes the paraneoplastic effect of Lambert-Eaton syndrome?

Back 192

Antibodies against presynaptic Ca2+ channels at NMJ (Thymoma, bronchogenic carcinoma)

Front 193

What causes the paraneoplastic effect of Polycythemia?

Back 193

Erythropoietin (secondary to renal cell carcinoma)

Front 194

What causes the paraneoplastic effect of SIADH?

Back 194

ADH or ANP (secondary to small cell lung carcinoma and intracranial neoplasms)

Front 195

What chemical carcinogen is commonly associated with the centrilobar necrosis and fatty acid change?

Back 195

CCL4

Front 196

What chemical carcinogen is commonly associated with the esophagus and stomach?

Back 196

Nitrosamines

Front 197

What chemical carcinogen is commonly associated with the lungs?

Back 197

Asbestos (Causes mesothelioma and bronchogenic carcinoma)

Front 198

What chemical carcinogen is commonly associated with the skin (squamous cell)?

Back 198

Arsenic

Front 199

What chemical carcinogen(s) are commonly associated with the liver?

Back 199

Aflatoxins and vinyl chloride

Front 200

What is a chondrosarcoma?

Back 200

Malignant cartilaginous tumor.

Front 201

What is a common genetic finding in Ewing's sarcoma?

Back 201

11;22 translocation

Front 202

What is a common gross pathological sign seen in Ewing's sarcoma?

Back 202

Characteristic 'onion-skin' appearance of bone

Front 203

What is a common origin of a chondrosarcoma?

Back 203

May be of primary origin or from osteochondroma

Front 204

What is a common sign found on the x-ray of a person with osteosarcoma?

Back 204

Codman's triangle (from elevation of periosteum)

Front 205

What is a craniopharyngioma?

Back 205

Benign childhood tumor. Often confused with pituitary adenoma because both can cause bitemporal hemianopsia. Calcification of the tumor is common.

Front 206

What is a Ewing's sarcoma?

Back 206

Anaplastic small cell malignant tumor.

Front 207

What is a giant cell tumor?

Back 207

Locally aggressive benign tumor around the distal femur, proximal tibial region.

Front 208

What is a gross pathological sign of basal cell carcinoma?

Back 208

Pearly papules

Front 209

What is a helpful mnemonic to remember the neoplasm associated with Down's Syndrome?

Back 209

We ALL go DOWN together.

Front 210

What is a helpful mnemonic to remember the site of metastasis to the brain?

Back 210

Lots of Bad Stuff Kills Glia

Front 211

What is a helpful mnemonic to remember the types of cancer that metastasize to the liver?

Back 211

Cancer Sometimes Penetrates Benign Liver

Front 212

What is a helpful mnemonic to remember what tumors metastasize to bone?

Back 212

BLT with a Kosher Pickle

Front 213

What is a Hemangioblastoma?

Back 213

Most often a cerebellar tumor. Associated with von Hippel Lindau syndrome when found with retinoblastoma.

Front 214

What is a low-grade astrocytoma?

Back 214

Diffusely infiltrating glioma. In children, it is most commonly found in the posterior fossa.

Front 215

What is a medulloblastoma?

Back 215

Highly malignant cerebellar tumor. A form of primitive neuroectodermal tumor (PNET). Can compress 4th ventricle causing hydrocephalus

Front 216

What is a neoplasm associated with actinic keratosis?

Back 216

Squamous cell carcinoma of the skin

Front 217

What is a neoplasm associated with Barrett's esophagus (chronic GI reflux)?

Back 217

Esophageal adenocarcinoma

Front 218

What is a neoplasm commonly associated with chronic atrophic gastritis, pernicious anemia, and postsurgical gastric remnants?

Back 218

Gastric adenocarcinoma

Front 219

What is an oligodendroglioma?

Back 219

A relatively rare, slow growing, benign tumor.

Front 220

What is CEA (carcinoembryonic antigen)?

Back 220

Very nonspecific antigen produced by 70% of colorectal and pancreatic cancers and by gastric and breast carcinoma

Front 221

What is considered a precursor to squamous cell carcinoma?

Back 221

Actinic keratosis

Front 222

What is considered to be a precursor to malignant melanoma?

Back 222

Dysplastic nevus

Front 223

What is meant by the term tumor grade?

Back 223

Histologic appearance of the tumor. Usually graded I-IV based on degree of differentiation and number of mitoses per high-power field.

Front 224

What is meant by the term tumor stage?

Back 224

Based on site an size of primary lesion, spread to regional lymph nodes, and presence of metastases.

Front 225

What is the characteristic appearance of a giant cell tumor on an x-ray?

Back 225

Characteristic 'double bubble' or 'soap bubble' appearance

Front 226

What is the common histopathology associated with Ependymomas?

Back 226

Characteristic perivascular rosettes. Rod-shaped blepharoblasts (basal ciliary bodies) found near the nucleus.

Front 227

What is the common histopathology associated with Hemangioblastoma?

Back 227

Foamy cells and high vascularity are characteristic. Can produce EPO and lead to polycythemia.

Front 228

What is the common histopathology associated with medulloblastomas?

Back 228

Rosettes or perivascular pseudorosette pattern of cells

Front 229

What is the common histopathology associated with oligodendrogliomas?

Back 229

Fried egg' appearance of cells in tumor. Often calcified.

Front 230

What is the common histopathology associated with schwannoma?

Back 230

Antoni A=compact palisading nuclei; Antoni B=loose pattern

Front 231

What is the common histopathology found in Glioblastoma multiforme?

Back 231

Pseudopalisading' tumor cells border central areas of necrosis and hemorrhage

Front 232

What is the differentiation pattern of normal cells?

Back 232

Basal to apical differentiation

Front 233

What is the histopathology commonly associate with giant cell tumors?

Back 233

Spindle-shaped cells with multi-nucleated giant cells.

Front 234

What is the most common benign bone tumor?

Back 234

Osteochondroma

Front 235

What is the most common location of basal cell carcinoma of the skin?

Back 235

Usually found in sun-exposed areas of the body.

Front 236

What is the most common location of osteosarcoma?

Back 236

Commonly found in the metaphysis of long bones

Front 237

What is the most common organ to 'send' metastases?

Back 237

The lung is the most common origin of metastases. The breast and stomach are also big sources.

Front 238

What is the most common organ to receive metastases?

Back 238

Adrenal glands. This is due to their rich blood supply. The medulla usually receives metastases first and then the rest of the gland.

Front 239

What is the most common population to have chondrosarcoma?

Back 239

Men age 30-60 years old

Front 240

What is the most common primary brain tumor?

Back 240

Glioblastoma multiforme (grade IV astrocytoma)

Front 241

What is the most common primary malignant tumor of bone?

Back 241

Osteosarcoma

Front 242

What is the most common type of pituitary adenoma?

Back 242

Prolactin secreting

Front 243

What is the most likely population to have Ewing's sarcoma?

Back 243

Boys under 15 years old.

Front 244

What is the origin of a craniopharyngioma?

Back 244

Derived from the remnants of Rathke's pouch

Front 245

What is the origin of a Pituitary adenoma?

Back 245

Rathke's pouch

Front 246

What is the origin of the Schwannoma?

Back 246

Schwann cell origin. Often localized to the 8th cranial nerve (acoustic schwannoma). Bilateral schwannoma found in NF2.

Front 247

What is the peak incidence of giant cell tumor?

Back 247

20-40 years old

Front 248

What is the peak incidence of osteosarcoma?

Back 248

Men 10-20 years old

Front 249

What is the prognosis for Glioblastoma multiforme?

Back 249

Prognosis is grave. Usually only have a year life expectancy.

Front 250

What is the second most common primary brain tumor?

Back 250

Meningioma

Front 251

What is the third most common primary brain tumor?

Back 251

Schwannomas

Front 252

What neoplasias are associated with a-fetoprotein?

Back 252

Hepatocellular carcinoma and nonseminomatous germ cell tumors of the testis.

Front 253

What neoplasias are associated with B-hCG?

Back 253

Hydatidiform moles, Choriocarcinomas, and Gestational trophoblastic tumors.

Front 254

What neoplasias are associated with CA-125?

Back 254

Ovarian and malignant epithelial tumors

Front 255

What neoplasias are associated with S-100?

Back 255

Melanoma, neural tumors, and astrocytomas

Front 256

What neoplasm is associated with Cirrhosis (due to alcoholism, Hep B, or Hep C)

Back 256

Hepatocellular carcinoma

Front 257

What neoplasm is associated with Dysplastic nevi?

Back 257

Malignant melanoma

Front 258

What neoplasm is associated with Immunodeficiency states?

Back 258

Malignant lymphomas

Front 259

What neoplasm is associated with Plummer-Vinson syndrome (atrophic glossitis, esophageal webs, and anemia; all due to iron deficiency)

Back 259

Squamous cell carcinoma of the esophagus

Front 260

What neoplasm is associated with ulcerative colitis?

Back 260

Colonic adenocarcinoma

Front 261

What oncogene is associated with breast, ovarian, and gastric carcinomas?

Back 261

erb-B2

Front 262

What oncogene is associated with Burkitt's lymphoma?

Back 262

c-myc

Front 263

What oncogene is associated with colon carcinoma?

Back 263

ras

Front 264

What oncogene is associated with Follicular and undifferentiated lymphomas (inhibits apoptosis)?

Back 264

bcl-2

Front 265

What tumor marker is associated with Prostatic carcinoma?

Back 265

PSA (Prostatic acid phosphatase)

Front 266

What tumor suppressor gene is associated with Retinoblastoma and osteosarcoma?

Back 266

Rb gene

Front 267

What type of metastases are common in the late stages of prostatic adenocarcinoma?

Back 267

Osteoblastic metastases in bone

Front 268

What type of neoplasm is associated with Acanthosis nigricans (hyperpigmentation and epidermal thickening)

Back 268

Visceral malignancies (stomach, lung, breast, and uterus)

Front 269

What type of skin cancer is associated with excessive exposure to sunlight or arsenic exposure?

Back 269

Squamous cell carcinoma

Front 270

Where are chondrosarcomas usually located?

Back 270

Pelvis, spine, scapula, humerus, tibia, or femur.

Front 271

Where are Ewing's sarcomas most commonly found?

Back 271

Diaphysis of long bones, pelvis, scapula, and ribs

Front 272

Where are Glioblastoma multiformes found?

Back 272

Cerebral hemispheres

Front 273

Where do giant cell tumors most commonly occur?

Back 273

At epiphyseal end of long bones

Front 274

Where do meningiomas most commonly occur?

Back 274

Convexities of hemispheres and parasagital region

Front 275

Where do oligodendrogliomas most often occur?

Back 275

Most often found in the frontal lobes

Front 276

Where do osteochondromas commonly originate?

Back 276

Long metaphysis

Front 277

Where do squamous cell carcinomas most commonly occur?

Back 277

Hands and face

Front 278

Where does prostatic adenocarcinoma most commonly arise?

Back 278

From the posterior lobe (peripheral zone) of the prostate gland

Front 279

Which has more prognostic value: tumor stage or grade?

Back 279

Stage

Front 280

Which is more common: metastasis to bone or primary tumors of bone?

Back 280

Metastatic bone tumors are far more common than primary tumors

Front 281

Which is more common: metastasis to the liver or primary tumors of the liver?

Back 281

Metastasis to the liver is more common

Front 282

Which tumor suppressor gene is associated with most human cancers and the Li-Fraumeni syndrome?

Back 282

p53

Front 283

Which tumor suppressor genes are associated with breast and ovarian cancer?

Back 283

BRCA 1 and 2

Front 284

___% of African-Americans carry the HbS trait, and ___% have the disease.

Back 284

8%; 0.2

Front 285

________ = defect of platelet aggregation

Back 285

Glanzmann's thrombasthenia

Front 286

________ disease = defect of platelet adhesion

Back 286

Bernard-Soulier

Front 287

_____= activation of coagulation cascade leading to microthrombi and global consumption of platelets, fibrin, and coagulation factors.

Back 287

DIC (Disseminated intravascular coagulation)

Front 288

2 therapies for sickle cell anemia:

Back 288

1. Hydroxyurea (increased HbF) 2. bone marrow transplantation

Front 289

50% of Hodgkin's cases are associated with which virus?

Back 289

EBV

Front 290

7 causes of normocytic normochromic anemia:

Back 290

1. hemorrage 2. enzyme defects (e.g., G6PD deficiency, PK deficiency) 3. RBC membrane defects (e.g., hereditary spherocytosis) 4. Bone Marrow disorders (e.g., aplastic anemia, leukemia) 5. Hemoglobinopathies (e.g., sickle cell) 6. Autoimmune hemolytic anemia 7. Anemia of chronic disease

Front 291

Antiplatelet antibodies and increased megakaryocytes are seen in ____.

Back 291

ITP

Front 292

Auer rods, myeloblasts, adults =

Back 292

AML

Front 293

bcl-2 activation is associated with which translocation and which lymphomas?

Back 293

t(14;18) - Follicular lymphomas

Front 294

bcr-abl hybrid is associated with which translocation and which leukemia?

Back 294

t(9;22) - CML

Front 295

Blood smear of a Multiple Myeloma patient would show what?

Back 295

RBCs stacked like poker chips (rouleau formation)

Front 296

Burkitt's lymphoma: '______' appearance associated with what virus? endemic where?

Back 296

starry sky' EBV Africa

Front 297

c-myc activation is associated with which translocation and which lymphoma?

Back 297

t(8;14) - Burkitt's

Front 298

Causes of aplastic anemia:

Back 298

radiation, benzene, chloramphenicol, alkylating agents, antimetabolites, viral agents (HCV,CMV,EBV, herpes zoster-varicella), Faconi's anemia, idiopathic (immune-mediated, primary stem cell defect)

Front 299

Causes of platelet abnormalities:

Back 299

ITP, TTP, drugs, DIC

Front 300

CFU-GM (colony forming unit-granulocyte-monocyte) gives rise to which cells?

Back 300

monocytes, neutrophils and basophils

Front 301

CLL is very similar to what lymphoma?

Back 301

SLL (small lymphocytic lymphoma)

Front 302

Coagulation factor defects (macrohemorrage) cause: (3)

Back 302

1. Hemarthroses (bleeding into joints) 2. easy bruising 3. prolonged PT and/or PTT

Front 303

Common cause of macrocytic megalobalstic anemia:

Back 303

Vit. B12/folate deficiency

Front 304

common in children, lymphoblasts, most responsive to therapy =

Back 304

ALL

Front 305

Compare Multiple Myeloma with Waldenstrom's macroglobinemia:

Back 305

Waldenstrom's also has an M spike, but large amounts of IgM are produced (not IgA or IgG), no lytic lesions

Front 306

Complications often seen in homozygotes (sickle cell disease) include:

Back 306

aplastic crisis (due to B19 parvovirus infection) autosplenectomy inc. risk of encapsulated organism infection Salmonella osteomyelitis painful crisis (vaso-occlusive) and splenic sequestration crisis

Front 307

Describe the RBCs of a parient with Hereditary spherocytosis.

Back 307

RBCs are small, round, with no central pallor less membrane therefore increased MCHC

Front 308

FAB classification L1:

Back 308

ALL-null, ALL-common

Front 309

FAB classification L2:

Back 309

ALL- T

Front 310

FAB classification L3:

Back 310

ALL- B

Front 311

FAB classification M1:

Back 311

AML (without maturation)

Front 312

FAB classification M2:

Back 312

AML (with maturation)

Front 313

FAB classification M3:

Back 313

acute promyelocytic leukemia

Front 314

FAB classification M4:

Back 314

acute myelomonocytic leukemia

Front 315

FAB classification M5:

Back 315

acute monocytic leukemia

Front 316

FAB classification M6:

Back 316

acute erythroleukemia

Front 317

FAB classification M7:

Back 317

acute megakaryocytic leukemia

Front 318

General considerations of leukemias:

Back 318

increased number of circulating leukocytes in blood bone marrow infiltrates of leukemic cells leukemic cell infiltrates in liver, spleen, and lymph nodes also common

Front 319

Genetics associated with Burkitt's lymphoma:

Back 319

t(8;14) c-myc gene moves next to heavy chain Ig gene (14)

Front 320

Hb Barts =

Back 320

Gamma 4 tetramers, lacks all 4 alpha globin genes

Front 321

Hb Barts results in what?

Back 321

hydrops fetalis and intrauterine fetal death

Front 322

Hb H =

Back 322

Beta 4 tetramers, lacks 3 alpha globin genes

Front 323

Hereditary spherocytosis causes intrinsic, extravascular hemolysis due to a _____ defect.

Back 323

spectrin

Front 324

Hereditary spherocytosis is associated with what other problems?

Back 324

gallstones, splenomegaly, anemia and jaundice

Front 325

Hereditary spherocytosis is distinguished from warm antibody hemolysis by what test?

Back 325

Direct Coomb's test (Hereditary spherocytosis is Coomb's negative)

Front 326

Hodgkin's or NHL: which is associated with HIV & immunosuppression?

Back 326

NHL

Front 327

Hodgkin's or NHL: which is associated with mediastinal lymphadenopathy?

Back 327

Hodgkin's

Front 328

Hodgkin's or NHL: which one displays a bimodal distribution?

Back 328

Hodgkin's (young and old)

Front 329

How does Multiple Myeloma affect calcium levels?

Back 329

destructive bone lesions cause hypercalcemia (punched-out lytic bone lesions can be seen on x-ray)

Front 330

In _____, the alpha globin chain is underproduced.

Back 330

alpha thalassemia

Front 331

In _____, the beta chain is underproduced.

Back 331

Beta-minor thalassemia (heterozygote)

Front 332

In ______, the beta chain is absent.

Back 332

Beta-major thalassemia (homozygote)

Front 333

In anemia of chronic disease, are these values increased or decreased? 1. TIBC 2. ferritin 3. serum iron 4. storage iron in marrow macrophages 5. % sat.

Back 333

1. dec. 2. inc. 3. dec. 4. inc. 5. normal

Front 334

In Beta Thalassemia, cardiac failure is often due to what?

Back 334

secondary hemochromatosis (due to transfusions)

Front 335

In which hemorrhagic disorder is PT increased?

Back 335

DIC

Front 336

In which hemorrhagic disorders is PTT increased? (4)

Back 336

1. Hemophilia A 2. Hemophilia B 3. von Willibrand's disease 4. DIC

Front 337

increased or decreased in iron deficiency anemia? 1. TIBC 2. ferritin 3. serum iron

Back 337

1. increased 2. decreased 3. decreased

Front 338

Is Hodgkin's more common in men or women?

Back 338

men (except nodular sclerosing type)

Front 339

Lab findings of DIC:

Back 339

increased PT and PTT, increased fibrin split products (D-dimers), decreased platelet count

Front 340

Lymphoblastic lymphoma commonly presents with what?

Back 340

ALL and mediastinal mass

Front 341

Lymphomas derived from the B cell lineage:

Back 341

ALL (B cell) Lymphoblastic lymphoma, CLL (B) Lymphocytic lymphoma, Follicular center cell lymphoma, Immunoblastic lymphoma (B), Plasmacytoid lymphocytic lymphoma and myeloma

Front 342

Lymphomas derived from the T cell lineage:

Back 342

ALL (T cell) Lymphoblastic lymphoma, CLL (T) Lymphocytic lymphoma, Immunoblastic lymphoma (T), Sezary syndrome and mycosis fungoides

Front 343

Macrocytic anemia is defined as MCV>____.

Back 343

100

Front 344

Marrow failure due to leukemia can lead to (3)

Back 344

1. anemia (dec. RBCs) 2. infections (dec. WBCs) 3. hemorrhage (decreased platelets)

Front 345

Microcytic, hypochromic anemia = MCV<____.

Back 345

80

Front 346

most commonly associated with Philadelphis chromosome, myeloid stem cell proliferation, may accelerate to AML =

Back 346

CML

Front 347

Name 3 coagulopathies:

Back 347

1. Hemophilia A 2. Hemophilia B 3. von Willibrand's disease

Front 348

Name 3 etiologies of microcytic, hypochromic anemia:

Back 348

1. iron deficiency 2. Thalassemias 3. lead poisoning

Front 349

Name 5 hemorrhagic disorders that exhibit increased bleeding time.

Back 349

1. Qualitative platelet defects 2. Vascular bleeding 3. Thrombocytopenia 4. Von Willibrand's disease 5. DIC

Front 350

Name 5 types of NHL:

Back 350

1. Small lymphocytic lymphoma 2. Follicular lymphoma (small cleaved cell) 3. Diffuse large cell 4. Lymphoblastic lymphoma 5. Burkitt's lymphoma

Front 351

Name 9 chronic myeloid leukemias:

Back 351

CML, Polycythemia rubra vera, CML, Myelofibrosis, idiopathic thrombocythemia, (chronic monocytic, chronic myelomonocytic, eosinophilic, chronic erythroid) *last 4 are rare*

Front 352

Nodal involvement and spread of Hodgkin's vs. NHL:

Back 352

Hodgkin's: localized, single group of nodes, extranodal rare, contiguous spread NHL: multiple, peripheral nodes, extranodal involvement common, noncontiguous spread

Front 353

Normal values: 1. TIBC 2. Serum iron 3. % sat.

Back 353

1. TIBC 250-400 micro grams/ dl 2. 50-150 3. 20-50 %

Front 354

older adults, lymphadenopathy, hepatosplenomegaly, few symptoms, indolent course, increased smudge cells in peripheral blood smear, warm Ab autoimmune hemolytic anemia =

Back 354

CLL

Front 355

Other causes of macrocytic anemia (2)

Back 355

1. drugs that block DNA synthesis (e.g., sulfa drugs, AZT) 2. marked reticulocytosis

Front 356

Pathologic features of aplastic anemia:

Back 356

pancytopenia with normal cell morphology, hypocellular bone marrow with fatty infiltration

Front 357

Peak incidence of NHL between what ages?

Back 357

20-40

Front 358

Philadelphia chromosome=

Back 358

t(9;22), bcr-abl

Front 359

Plasma cells in Multiple Myeloma produce large amounts of what?

Back 359

IgG (55%) and IgA (25%)

Front 360

Platelet abnormalities (microhemorrage) lead to: (4)

Back 360

1. mucous membrane bleeding 2. petechiae 3. purpura 4. prolonged bleeding time

Front 361

PMNs are hypersegmented in ____ .

Back 361

Vit. B 12 and folate deficiencies

Front 362

PT and PTT: which one measures the intrinsic and which one measures the extrinsic path?

Back 362

PT (extrinsic) PTT (intrinsic)

Front 363

Rank the prognoses of the different types of Hodgkin's:

Back 363

NS and LP = excellent MC = intermediate LD = poor

Front 364

Schistocytes are characteristic of ___.

Back 364

TTP

Front 365

Sickle cell heterozygotes (sickle cell trait) are relatively ____-resistant.

Back 365

malaria; (balanced polymorphism)

Front 366

Symptoms of aplastic anemia:

Back 366

fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection

Front 367

Thalassemia is prevalent in which populations?

Back 367

Mediterranean (hint: thalassa=sea. Think, thalaSEAmia)

Front 368

The lymphoid stem cell gives rise to: (2)

Back 368

T cells and B cells

Front 369

The majority of NHL involve T cells or B cells?

Back 369

B cells (except lymphoblastic T cell origin)

Front 370

Treatment of aplastic anemia:

Back 370

withdrawal of offending agent, allogenic bone marrow transplantation, RBC and platelet transfusion, G-CSF or GM-CSF

Front 371

What are 2 indications of hemolysis?

Back 371

1. decreased serum haptoglobin 2. increased serum LDH

Front 372

What are constitutional signs/symptoms?

Back 372

(mostly seen in Hodgkin's) low grade fever, night sweats, weight loss

Front 373

What are some other causes of DIC?

Back 373

gram-negative sepsis, transfusion, trauma, malignancy, acute pancreatitis, and nephrotic syndrome

Front 374

What genetics are involved with follicular lymphoma (small cleaved cell)?

Back 374

t(14;18) bcl-2 expression

Front 375

What is another Beta chain mutation in which patients have a milder disease than Hb SS patients?

Back 375

HbC defect. patients can be HbC or HbSC (1 of each mutant gene)

Front 376

What is aplastic anemia?

Back 376

pancytopenia characterized by severe anemia, neutropenia, and thrombocytopenia caused by destruction of multipotent myeloid stem cells, with inadequate production or release of differentiated cell lines.

Front 377

What is compensatorily increased in both forms of Beta Thalassemia?

Back 377

fetal hemoglobin (it is inadequate, however)

Front 378

What is found in the urine of patients with Multiple Myeloma?

Back 378

Ig light chains (Bence Jones protein)

Front 379

What is it called when CML --> AML?

Back 379

blast crisis

Front 380

What is the characteristic cell of Hodgkin's lymphoma?

Back 380

Reed-Sternberg cell (decreased numbers of RS cells indicates a better prognosis)

Front 381

What is the M spike?

Back 381

the monoclonal immunoglobin spike on serum electrophoresis

Front 382

What is the most common bleeding disorder?

Back 382

von Willibrand's disease

Front 383

What is the most common cause of DIC?

Back 383

obstetric complications

Front 384

What is the most common primary tumor arising within bone in adults?

Back 384

Multiple Myeloma

Front 385

What is the name of a chronic T cell leukemia?

Back 385

Sezary syndrome

Front 386

What mutation causes sickle cell anemia?

Back 386

a single AA replacement in the Beta chain (normal glutamic acid with valine)

Front 387

What other problems result from Multiple Myeloma?

Back 387

renal insufficiency, increased susceptibility to infections, anemia, and amyloidosis

Front 388

What precipitates sickling of cells?

Back 388

low oxygen or dehydration

Front 389

What test is used to confirm Hereditary spherocytosis?

Back 389

osmotic fragility test

Front 390

What test is used to distinguish between immune vs. non-immune RBC hemolysis?

Back 390

Direct Coomb's test

Front 391

What type of cell is cancerous in Multiple Myeloma and what does it resemble?

Back 391

Monoclonal plasma cell, 'fried egg' appearance

Front 392

Which 2 hemorrhagic disorders have decreased platelet counts?

Back 392

1. thrombocytopenia 2. DIC

Front 393

Which 2 types of NHL occur in children?

Back 393

lymphoblastic lymphoma, and Burkitt's lymphoma (20% of diffuse large cell type too)

Front 394

Which Beta Thalassemia results in severe anemia?

Back 394

Beta Thal. major, (requires blood transfusions)

Front 395

Which coagulation factors are a part of the intrinsic and which are a part of the extrinsic path?

Back 395

(extrinsic) = Factors II, V, VII, and X (intrinsic) = all factors except VII and XIII

Front 396

Which coagulation factors are deficient in each of the Hemophilias?

Back 396

Hemophilia A (factor VIII deficiency) Hemophilia B (factor IX deficiency)

Front 397

Which one is associated with neurological problems, folate deficiency or Vit. B12 deficiency?

Back 397

Vit. B12

Front 398

Which type of Hodgkin's accounts for 6% of cases and which type is the most rare.

Back 398

LP (lymphocyte predominant) - 6% LD (lymphocyte depleted) - rare

Front 399

Which type of Hodgkin's commonly affects males under 35?

Back 399

LP

Front 400

Which type of Hodgkin's commonly affects older males and is associated with disseminated disease?

Back 400

LD

Front 401

Which type of Hodgkin's has the most Reed Sternberg cells?

Back 401

Mixed Cellularity

Front 402

Which type of Hodgkin's is characterized by collagen banding?

Back 402

NS (nodular sclerosing)

Front 403

Which type of Hodgkin's is the most common? (65-75%)

Back 403

NS (nodular sclerosing)

Front 404

Which type of Hodgkin's is the second most common? (25%)

Back 404

MC (mixed cellularity)

Front 405

Which type of Hodgkin's primarily affects young adults, women>men?

Back 405

NS

Front 406

Which type of NHL clinically presents like CLL?

Back 406

small lymphocytic lymphoma

Front 407

Which type of NHL is difficult to cure?

Back 407

follicular lymphoma

Front 408

Which type of NHL is most common in children?

Back 408

Lymphoblastic lymphoma (very aggressive)

Front 409

Which type of NHL is the most common type in adults?

Back 409

follicular lymphoma (small cleaved cell)

Front 410

Which types of NHL involve T cells?

Back 410

Lymphoblastic lymphoma (immature T cells) 20% of Diffuse large cell NHL (mature T cells)

Front 411

With iron overload (hemosiderosis) are values increased or decreased? 1. TIBC 2. serum iron 3. %sat.

Back 411

1. TIBC normal 2. Serum iron increased 3. % sat. increased (100%) -see charts on p.238 of 2002 edition-

Front 412

A patient with gallstones may present with Charcot's triad. What comprises the triad?

Back 412

(1)epigastric/RUQ pain (2)fever (3)jaundice

Front 413

Failure of copper to circulate in what form causes Wilson's disease?

Back 413

ceruloplasmin

Front 414

How can one distinguish between Dubin-Johnson syndrome and Rotor's syndrome?

Back 414

Rotor's syndrome presents similarly, except less severely and no black liver(as seen in Dubin-Johnson)

Front 415

How does Budd-Chiari syndrome progress(in the liver)?

Back 415

Congestive liver disease

Front 416

How does cirrhosis/portal hypertension(HTN) affect liver histology?

Back 416

diffuse fibrosis, destroying normal structure, with nodular regeneration

Front 417

How does Hirschsprung's disease first present?

Back 417

Chronic constipation early in life

Front 418

How does one diagnose gallstones?

Back 418

ultrasound

Front 419

How does one differentiate liver nodules, in the case of cirrhosis?

Back 419

micronodular(<3mm and uniform) macronodular(>3mm and varied)

Front 420

How does one treat gallstones?

Back 420

cholecystectomy

Front 421

How is achalasia evidenced on a Barium swallow?

Back 421

Bird beak'--dilated esophagus with an area of distal stenosis.

Front 422

How is hepatocellular carcinoma spread?

Back 422

like renal cell carcinoma, hematogenously

Front 423

How is the hyperbilirubinemia in Dubin-Johnson syndrome different than in the other 2 hyperbilirubinemias(Gilbert's or Crigler-Najjar(type 1))?

Back 423

Dubin-Johnson syndrome-- conjugated hyperbilirubinemia (due to defective liver excretion)

Front 424

How would you expect a Gilbert's syndrome patient to present?

Back 424

Asymptomatically, with an elevated unconjugated bilirubin

Front 425

In PUD, how can H.pylori be treated?

Back 425

triple therapy (metronidazole, bismuth salicylate, amoxicillin or tetracycline with or without a proton pump inhibitor

Front 426

In what fatal childhood hepatoencephalopathy is there an association with viral infections(VZV,influ.B) and salicylates?

Back 426

Reye's syndrome

Front 427

In Wilson's disease, where does copper accumulate(3)?

Back 427

(1)Liver (2)Brain (3)cornea

Front 428

Is chronic pancreatitis strongly associated with alcoholism?

Back 428

YES

Front 429

Is the dilation proximal, at, or distal to the aganglionic segment?

Back 429

Proximal (results in a 'transition zone')

Front 430

Low-fiber diets are associated with which of the following?

Back 430

diverticulosis

Front 431

To what cancer is a chronic gastritic patient predisposed?

Back 431

Gastric carcinoma

Front 432

What 3 common findings are evident in a Reye's patient?

Back 432

(1)fatty liver (2)hypoglycemia (3)coma

Front 433

What are 2 anti-androgen effects of liver cell failure?

Back 433

gynecomastia loss of sexual hair

Front 434

What are 2 extraintestinal manifestations of Crohn's?

Back 434

migratory polyarthritis erythema nodosum

Front 435

What are 2 main symptoms and a histological sign of Duodenal ulcers?

Back 435

(1)pain Decreases with meals, (2)weight gain, hypertrophy of Brunner's glands

Front 436

What are 2 main symptoms of Gastric ulcers?

Back 436

pain Greater with meals, weight loss

Front 437

What are 2 signs of Crigler-Najjar syndrome(other than hyperbilirubinemia)?

Back 437

jaundice kernicterus(bilirubin deposition in the brain)

Front 438

What are 2 types of Inflammatory Bowel Disease?

Back 438

Crohn's disease Ulcerative colitis

Front 439

What are 2 types of peptic ulcer disease(PUD)?

Back 439

gastric ulcer duodenal ulcer

Front 440

What are 3 associations of Budd-Chiari Syndrome?

Back 440

(1)pregnancy (2)polycythemia rubra vera (3)hepatocellular carcinoma

Front 441

What are 3 neuro effects of liver cell failure?

Back 441

(1)asterixis, (2)scleral icterus, (3)coma

Front 442

What are 3 types of gallstones?

Back 442

(1)Cholesterol stones (2)Mixed stones (3)Pigment stones

Front 443

What are 4 potential complications of PUD?

Back 443

(1)bleeding, (2)penetration, (3)perforation, (4)obstruction

Front 444

What are 4 signs of congestive liver disease?

Back 444

(1)hepatomegaly (2)ascites (3)abdominal pain (4)eventual liver failure

Front 445

What are 5 GI and 2 GU effects of portal HTN?

Back 445

(1)esophageal varices(->hematemesis), (2)melena, (3)splenomegaly, (4)caput medusae, (5)ascites and (1)testicular atrophy, (2)hemorrhoids

Front 446

What are 5 possible consequences of acute pancreatitis?

Back 446

(1)DIC (2)ARDS (3)Diffuse fat necrosis (4)hypocalcemia (5)pseudocyst formation

Front 447

What are possible causes of acute pancreatitis(GET SMASHeD)?

Back 447

Gallstones Ethanol Trauma Steroids Mumps Autoimmune disease Scorpion sting Hyperlipidemia Drugs

Front 448

What are possible etiologies of hemochromatosis?

Back 448

primary(autos. Recessive) secondary to chronic transfusion therapy

Front 449

What are risk factors for esophageal cancer(ABCDEF)?

Back 449

Achalasia, Barrett's esophagus, Corrosive esophagitis, Diverticuli, Esophageal web, Familial

Front 450

What are some complications of Crohn's(4)?

Back 450

(1)strictures, (2)fistulas, (3)perianal disease, (4)malabsorption-nutritional depletion

Front 451

What are some complications of ulcerative colitis(3)?

Back 451

(1)severe stenosis, (2)toxic megacolon, (3)colorectal carcinoma

Front 452

What are the 2 types of chronic gastritis?

Back 452

Type A(fundal) Type B(antral)

Front 453

What are the 3 forms of Diverticular disease?

Back 453

(1)diverticulum, (2)diverticulosis, (3)diverticulitis

Front 454

What are the 4 risk factors for gallstone development?

Back 454

(1)Female (2)Fat (3)Fertile (4)Forty

Front 455

What are the ABCD characteristics of Wilson's?

Back 455

Asterixis Basal ganglia degeneration Cirrhosis, Ceruloplasmin decrease, Corneal deposits(Kayser-Fleischer rings), Carcinoma(hepatocell.), Choreiform movements Dementia

Front 456

What are the characteristic 4 A's of type A gastritis?

Back 456

Autoimmune disorder characterized by Autoantibodies to parietal cells, pernicious Anemia, Achlorhydria

Front 457

What are the respective etiologies of Crohn's and Ulcerative colitis(UC)?

Back 457

infectious(Crohn's) autoimmune(UC)

Front 458

What can achalasia arise from and lead to?

Back 458

A secondary form can arise from Chagas' disease; can lead to progressive dysphagia.

Front 459

What can hemochromatosis lead to(2)?

Back 459

(1)CHF (2)hepatocellular carcinoma

Front 460

What cell tumor marker is elevated in hepatocellular carcinoma?

Back 460

Alpha FetoProtein(AFP)

Front 461

What disorder is characterized by increased iron deposition in many organs(up to 50g)?

Back 461

hemochromatosis

Front 462

What happens to hepatocytes as a result of alcoholic hepatitis?

Back 462

they become swollen and necrotic

Front 463

What happens when cholesterol and bilirubin overwhelm solubilizing bile acids and lecithin?

Back 463

Gallstones

Front 464

What histological changes, other than to hepatocytes, does one see in liver hepatitis?

Back 464

neutrophil infiltration, Mallory bodies(hyaline), increased fat, and sclerosis around the central vein

Front 465

What is a mnemonic for Barrett's esophagus?

Back 465

BARRett's = Becomes Adenocarcinoma, Results from Reflux.

Front 466

What is Barrett's esophagus?

Back 466

The replacement of glandular stratified squamous with gastric columnar epithelium in distal esophagus.

Front 467

What is failure of lower esophageal sphincter relaxation due to?

Back 467

Achalasia is due to the loss of the myenteric plexus.

Front 468

What is hepatic vein or IVC occlusion with centrilobular congestion and necrosis?

Back 468

Budd-Chiari syndrome

Front 469

What is recommended for patients over 50, in terms of CRC screening?

Back 469

screen these patients over 50 with stool occult blood test

Front 470

What is the cause of diverticulosis?

Back 470

increased intraluminal pressure and focal weakness in the colonic wall

Front 471

What is the classic triad of hemochromatosis?

Back 471

(1)micronodular pigment cirrhosis (2)'bronze' diabetes (3)skin pigmentation

Front 472

What is the discerning characteristic for type B gastritis?

Back 472

Type B is caused by a Bug(H. pylori)

Front 473

What is the embryonic cause of Hirschsprung's disease?

Back 473

failure of neural crest migration

Front 474

What is the etiology of duodenal ulcers?

Back 474

H.pylori(100%)--lower mucosal protection or increased gastric acid secretion

Front 475

What is the etiology of gastric ulcers?

Back 475

H.pylori(70%), NSAIDS both lower mucosal protection vs. gastric acid

Front 476

What is the gross morphology of Crohn's?

Back 476

transmural inflamm. COBBLESTONE mucosa, creeping FAT, bowel wall thickening(string sign on x-ray), linear ulcers, fissures

Front 477

What is the gross morphology of ulcerative colitis?

Back 477

mucosal inflamm. Friable mucosal pseudopolyps with freely hanging mesentery

Front 478

What is the incidence of diverticulosis in the elderly?

Back 478

over 60y/o, 50%

Front 479

What is the microscopic morphology of Crohn's?

Back 479

noncaseating granulomas

Front 480

What is the microscopic morphology of ulcerative colitis?

Back 480

crypt abscesses and ulcers

Front 481

What is the mnemonic for Crohn's?

Back 481

For Crohn's, think of a FAT old CRONE SKIPping down a COBBLESTONE road.

Front 482

What is the most common clinical sign of acute pancreatitis?

Back 482

epigastric abdominal pain radiating to the back

Front 483

What is the most common primary malignant tumor of the liver in adults?

Back 483

hepatocellular carcinoma

Front 484

What is the most common type of gallstone?

Back 484

mixed stones

Front 485

What is the primary cause of macronodules in the liver?

Back 485

significant liver injury leading to hepatic necrosis(e.g. Infections, drug-induced)

Front 486

What is the primary cause of micronodules in the liver?

Back 486

metabolic(e.g. Alcohol)

Front 487

What is the prognosis for pancreatic adenocarcinoma?

Back 487

~6months (very aggressive--often already spread at presentation)

Front 488

What is the term for inflamm. of diverticula?

Back 488

diverticulitis

Front 489

What is the term used for congenital megacolon characterized by loss of parasympathetic ganglion cells?

Back 489

Hirschsprung's disease

Front 490

What is the term used to describe breath that smells like a freshly opened corpse, as seen in liver cell failure?

Back 490

fetor hepaticus

Front 491

What is the term used to describe having many diverticula?

Back 491

diverticulosis

Front 492

What is the treatment for Crigler-Najjar syndrome?

Back 492

plasmaphoresis phototherapy

Front 493

What is the treatment of hemochromatosis?

Back 493

repeated phlebotomy deferoxamine

Front 494

What is the treatment of Wilson's disease(1 drug)?

Back 494

penicillamine

Front 495

What is the typical presentation of a patient with pancreatic adenocarcinoma(5 signs)?

Back 495

(1)Abdominal pain radiating to the back (2)Weight loss (3)Anorexia (4)Migratory thrombophlebitis(Trousseau's Sd) (5)Pancreatic duct obstruction(palpable gallbladder)

Front 496

What is the usual location of Crohn's?

Back 496

Terminal ileum, small intestine, colon (but any part can be affected)-- often SKIP lesions, rectal sparing

Front 497

What is the usual location of ulcerative colitis?

Back 497

colon (with continuous lesions and rectal involvement)

Front 498

What lab values are characteristic in alcoholic hepatitis?

Back 498

SGOT(AST)/SGPT(ALT) > 1.5,usually (A Scotch and Tonic=AST elevation)

Front 499

What lab values are characteristic in hemochromatosis?

Back 499

increased ferritin and transferrin saturation

Front 500

What labs are elevated in acute pancreatitis?

Back 500

amylase lipase(higher specificity)

Front 501

What part of the GI tract is most frequently involved in diverticulosis?

Back 501

sigmoid colon

Front 502

What risk group has an increased incidence of PUD by 2X?

Back 502

smoking

Front 503

What type of stones are seen in patients with RBC hemolysis,alcoholic cirrhosis,biliary infection?

Back 503

pigment stones

Front 504

When do patients with Crigler-Najjar syndrome(type I) usually present?

Back 504

early in life(often die within a few years)

Front 505

Where are pancreatic tumors most often located?

Back 505

pancreatic head (with obstructive jaundice)

Front 506

Where is the pain associated with diverticulitis?

Back 506

left lower quadrant

Front 507

Which of the following are effects of liver cell failure? Anemia, hypercoagulation,spider nevi, jaundice, gynecomastia,bleeding tendency, ankle edema

Back 507

all, except hyper coagulation

Front 508

Which of the following are risk factors for colorectal cancer(CRC): age,smoking,personal and family history of colon cancer, low-fiber diet, hereditary non-polyposis CRC?

Back 508

all, except for smoking

Front 509

Which of the following are risk factors for colorectal cancer: colorectal villous adenomas, chronic inflamm.bowel disease, familial adenomatous polyposis,Peutz-Jeghers?

Back 509

all, except for Peutz-Jeghers

Front 510

Which of the following is a blind pouch leading off the GI tract lined by mucosa, muscularis, serosa? Diverticulosis, diverticulum, diverticulitis

Back 510

diverticulum

Front 511

Which of the following is a common association between cholesterol stones and pigment stones: obesity,Crohn's,cystic fibrosis,age,clofibrate, estrogens,multiparity,rapid weight loss?

Back 511

advanced age

Front 512

Which of the following is associated with perforation, peritonitis, abscesses, or bowel stenosis?

Back 512

Diverticulitis

Front 513

Which of the following is associated with stress:Gilbert's, Dubin-Johnson syndrome, or Crigler-Najjar(type 1)?

Back 513

Gilbert's

Front 514

Which of the following syndromes have a mildly decreased UDP-glucuronyl transferase: Gilbert's or Crigler-Najjar(type 1)?

Back 514

Gilbert's(Crigler-Najjar has an absence of UDP-glucuronyl transferase)

Front 515

Which type of liver nodule is associated with an increased risk of hepatocellular carcinoma?

Back 515

macronodules

Front 516

With what 6 diseases does hepatocellular carcinoma have an association?

Back 516

(1)Hepatitis B (2)Hepatitis C (3)Wilson's (4)Hemochromatosis (5)alpha 1 antitrypsin deficiency (6)alcoholic cirrhosis

Front 517

With what cancer is achalasia associated?

Back 517

Increased risk for esophageal cancer.

Front 518

An FEV1/FVC ratio greater than 80% indicates what form of pulmonary disease?

Back 518

Restrictive lung disease

Front 519

Are bronchogenic carcinoma metastases common?

Back 519

Yes, very common

Front 520

Decreased FEV1/FVC ratio are the hallmark of what kind of pulmonary disease?

Back 520

COPD

Front 521

How does interstitial fibrosis create a restrictive lung disease?

Back 521

It causes increased recoil (decreased compliance), thereby limiting alveolar expansion.

Front 522

How does lung cancer commonly present? (5)

Back 522

- Cough - Hemoptysis - Bronchial obstruction - Wheezing - Pneumonic 'coin' lesion on x-ray

Front 523

How does surfactant deficiency cause NRDS?

Back 523

It leads to an increase in surface tension, resulting in alveolar collapse

Front 524

How to you treat NRDS?

Back 524

- Maternal steroids before birth - Artificial surfactant for infant

Front 525

Identify: ivory-white pleural plaques in the lung.

Back 525

Ferruginous bodies

Front 526

In COPD, are lung volumes increased, decreased, or normal?

Back 526

Increased (increasedTLC, increased FRC, increased RV)

Front 527

In restrictive lung disease, are lung volumes increased, decreased, or normal?

Back 527

Decreased

Front 528

In what occupations is asbestosis most commonly seen? (2)

Back 528

Shipbuilders and plumbers

Front 529

Name three 'triggers' of asthma.

Back 529

- Viral URIs - Allergens - Stress

Front 530

Name three characteristics of Horner's syndrome?

Back 530

- Ptosis - Miosis - Anhidrosis

Front 531

Name two extrapulmonary (poor breathing mechanics) causes of restrictive lung disease.

Back 531

- Poor muscular effort: polio, myasthenia gravis - Poor apparatus: scoliosis

Front 532

Name two pulmonary (poor lung expansion) causes of restrictive lung disease.

Back 532

- Defective alveolar filling: pneumonia, ARDS, pulmonary edema - Interstitial fibrosis

Front 533

Patients with asbestosis are at increased risk for what? (2)

Back 533

Pleural mesothelioma and bronchogenic carcinoma

Front 534

T/F Bronchiectasis is associated with bronchial obstruction, cystic fibrosis, and poor ciliary motility.

Back 534

TRUE

Front 535

T/F In obstructive (not restrictive) lung disease, FEV1 and FVC are reduced.

Back 535

False, FEV1 and FVC are reduced in both

Front 536

T/F Restricted lung expansion causes decreased total lung capacity and increased vital capacity.

Back 536

False, decreased VC and TLC

Front 537

T/F Smokers with asbestosis have a decreased risk of developing cancer.

Back 537

False, it increases synergistically

Front 538

What are asbestos fibers coated with hemosiderin in the lung?

Back 538

Ferruginous bodies

Front 539

What are the characteristics of lobar pneumonia?

Back 539

Intra-alveolar exudate -> consolidation; may involve entire lung

Front 540

What are the clinical findings of chronic bronchitis? (3)

Back 540

- Wheezing - Crackles - Cyanosis

Front 541

What are the clinical findings of emphysema? (4)

Back 541

- Dyspnea - Decreased breath sounds - Tachycardia - Decreased I/E ratio

Front 542

What are the symptoms and complications of interstitial lung fibrosis?

Back 542

- Symptoms: gradual progressive dyspnea and cough - Complications include cor pulmonale (can be seen in diffuse interstitial pulmonary fibrosis and bleomycin toxicity)

Front 543

What bronchogenic carcinoma is associated with ectopic hormone production (ADH, ACTH) and may lead to Lambert-Eaton syndrome?

Back 543

Small cell carcinoma

Front 544

What bronchogenic carcinoma is associated with ectopic PTH-related peptide production?

Back 544

Squamous cell carcinoma

Front 545

What bronchogenic carcinoma is most common?

Back 545

Adenocarcinoma

Front 546

What bronchogenic carcinoma is thought not to be related to smoking?

Back 546

Bronchioalveolar carcinoma

Front 547

What bronchogenic carcinomas are clearly linked to SSmoking?

Back 547

SSquamous cell carcinoma and SSmall cell carcinoma

Front 548

What bronchogenic carcinomas usually express tumors that arise centrally? (2)

Back 548

- Squamous cell carcinoma - Small cell carcinoma

Front 549

What bronchogenic carcinomas usually express tumors that arise peripherally? (3)

Back 549

- Adenocarcinoma - Bronchioalveolar carcinoma - Large cell carcinoma--undifferentiated

Front 550

What carcinoma occurs in the apex of the lung and may affect the cervical sympathetic, causing Horner's syndrome?

Back 550

Pancoast's tumor

Front 551

What causes bronchiectasis?

Back 551

Chronic necrotizing infection of bronchi

Front 552

What causes neonatal respiratory distress syndrome (NRDS)?

Back 552

Surfactant deficiency

Front 553

What cells make surfactant and when is it made most abundantly in fetuses?

Back 553

Type II pneumocytes most abundantly after 35th week of gestation

Front 554

What COPD has a productive cough for greater than three months in two years and hypertrophy of mucus-secreting glands in the bronchioles (Reid index greater than 50%)?

Back 554

Chronic bronchitis

Front 555

What COPD is characterized by dilated airways, purulent sputum, recurrent infections, and hemoptysis?

Back 555

Bronchiectasis

Front 556

What COPD is due to an enlargement of air spaces and decreased recoil resulting from destruction of alveolar walls?

Back 556

Emphysema

Front 557

What COPD is due to bronchial hyperresponsiveness which causes reversible bronchoconstriction?

Back 557

Asthma

Front 558

What does inhaled asbestos do to the lungs?

Back 558

It causes diffuse pulmonary interstitial fibrosis

Front 559

What is the composition of surfactant:

Back 559

Dipalmitoyl phosphatidylcholine

Front 560

What is the difference in FEV1/FVC ratios between obstructive and restrictive lungs diseases?

Back 560

FEV1 and FVC are reduced in both, but in obstructive the FEV1 is more dramatically reduced, resulting in a decreased FEV1/FVC ratio

Front 561

What is the leading cause of cancer death?

Back 561

Lung cancer

Front 562

What is the SPHERE (acronym) of complications associated with lung cancer?

Back 562

Superior vena caval syndrome Pancoast's tumor Horner's syndrome Endocrine (paraneoplastic) Recurrent laryngeal symptoms (hoarseness) Effusions (pleural or pericardial)

Front 563

What kind of pulmonary diseases are caused by a inhibition of air flow resulting in air trapping in the lungs?

Back 563

Obstructive lung diseases (COPD)

Front 564

What organisms are is the most frequent cause of lobar pnuemonia?

Back 564

Pneumococcus

Front 565

What organisms are the most frequent cause of bronchopnuemonia? (4)

Back 565

- S. aureus - H. flu - Klebsiella - S. pyogenes

Front 566

What organisms are the most frequent cause of interstitial (atypical) pnuemonia? (3)

Back 566

- Viruses (RSV, adenoviruses) - Mycoplasma - Legionella

Front 567

What specific type of emphysema is caused by alpha-1-antitrypsin deficiency?

Back 567

Panacinar emphysema (and liver cirrhosis)

Front 568

What specific type of emphysema is caused by smoking?

Back 568

Centriacinar emphysema

Front 569

What test is used to measure in utero lung maturity?

Back 569

The lecithin-to-sphingomyelin ratio in the amniotic fluid, usually less than 1.5 in neonatal respiratory distress syndrome

Front 570

What type of lung cancer can cause carcinoid syndrome?

Back 570

Carcinoid tumor

Front 571

What type of pneumonia is characterized by acute inflammatory infiltrates from bronchioles into adjacent alveoli with a patchy distribution affecting more than one lobe?

Back 571

Bronchopneumonia

Front 572

What type of pneumonia is characterized by diffuse patchy inflammation localized to interstial areas at alveolar walls and involves more than one lobe?

Back 572

Interstitial (atypical) pneumonia

Front 573

Where does bronchogenic carcinoma commonly metastasize and how does it present? (3)

Back 573

- Brain (epilepsy) - Bone (pathologic fracture) - Liver (jaundice, hepatomegaly)

Front 574

Why does alpha-1-antitrypsin deficiency cause emphysema?

Back 574

Increased elastase activity

Front 575

Define epilepsy.

Back 575

Epilepsy is a disorder of recurrent seizures.

Front 576

Define syrinx.

Back 576

Tube, as in syringe

Front 577

Describe a myoclonic seizure.

Back 577

Quick,repetitive jerks

Front 578

Describe a tonic-clonic seizure.

Back 578

Alternating stiffening and movement (grand mal)

Front 579

Describe a tonic seizure.

Back 579

Stiffening

Front 580

Describe an absence seizure.

Back 580

A blank stare (petit mal- it's in 1st aid this way!!)

Front 581

Describe an atonic seizure.

Back 581

drop' seizures

Front 582

Describe Broca's aphasia.

Back 582

Broca's is nonfluent aphasia with intact comprehension. BROca's is BROken speech.

Front 583

Describe Horner's syndrome.

Back 583

Sympathectomy of face (lesion above T1). Interruption of the 3-neuron oculosympathetic pathway.

Front 584

Describe Wernicke's aphasia.

Back 584

Wernicke's is fluent aphasia with impaired comprehension. Wernicke's is Wordy but makes no sense.

Front 585

How do patients present with a subarachnoid hemorrhage?

Back 585

Worst headache of my life'

Front 586

How do pts present with MS?

Back 586

-Optic neuritis (sudden loss of vision) - MLF syndrome (internuclear ophthalmoplegia) -Hemiparesis -Hemisensory symptoms -Bladder/bowel incontinence

Front 587

How does it spread?

Back 587

Through the bloodstream to the CNS

Front 588

How does Werdnig-Hoffman disease present?

Back 588

At birth as a 'floppy baby'

Front 589

How is Huntington's disease inherited?

Back 589

Autosomal dominant

Front 590

How is the polio virus transmitted?

Back 590

Fecal-oral route

Front 591

How is the prevalence of MS geographically distributed?

Back 591

Higher prevalence with greater distance from the Equator

Front 592

In what persons is subdural hemorrhage often seen?

Back 592

Elderly individuals, alcoholics, and blunt trauma

Front 593

T/F. Partial seizures can not generalize.

Back 593

False- Partial seizures can generalize.

Front 594

What are 2 common organisms that target the brain in AIDS pts?

Back 594

1. Toxo!Toxo!Toxo! 2. Cryptococcus

Front 595

What are 2 degenerative diseases of the cerebral cortex?

Back 595

1. Alzheimer's 2. Pick's disease

Front 596

What are 2 degenerative diseases that affect the basal ganglia and brain stem?

Back 596

1. Huntington's disease 2. Parkinson's disease

Front 597

What are 3 degenerative disorders of the motor neuron?

Back 597

1. Amyotrophic lateral sclerosis (ALS) 2. Werdnig-Hoffman disease 3. Polio

Front 598

What are associated with Guillain-Barre?

Back 598

1. Infections (herpesvirus or C. jejuni) 2. Inoculations 3. Stress

Front 599

What are neurofibrillary tangles?

Back 599

Abnormally phosphorylated tau protein

Front 600

What are some demyelinating and dysmyelinating diseases?

Back 600

1. Multiple sclerosis (MS) 2. Progressive multifocal leukoencephalopathy (PML) 3. Postinfectious encephalomyelitis 4. Metachromatic Leukodystrophy 5. Guillain-Barre syndrome

Front 601

What are the 4 types of intracranial hemorrhages?

Back 601

1. Epidural hematoma 2. Subdural Hematoma 3. Subarachnoid hemorrhage 4. Parenchymal hematoma

Front 602

What are the 5 types of generalized seizures?

Back 602

1. Absence 2. Myoclonic 3. Tonic-clonic 4. Tonic 5. Atonic

Front 603

What are the clinical symptoms of Huntington's disease?

Back 603

Dementia, chorea

Front 604

What are the clinical symptoms of Parkinson's disease?

Back 604

TRAP= Tremor (at rest) cogwheel Rigidity Akinesia Postural instability (you are TRAPped in your body)

Front 605

What are the clinical symptoms of Tabes dorsalis?

Back 605

-Charcot joints -Shooting pain -Argyll-Robertson Pupils -Absence of deep tendon reflexes

Front 606

What are the common causes of seizures in adults?

Back 606

-Tumors -Trauma -Stroke -Infection

Front 607

What are the common causes of seizures in children?

Back 607

-Genetic -Infection -Trauma -Congenital -Metabolic

Front 608

What are the common causes of seizures in the elderly?

Back 608

-Stroke -Tumor -Trauma -Metabolic -Infection

Front 609

What are the lab findings in Guillain-Barre syndrome?

Back 609

Elevated CSF protein with normal cell count ('albumino-cytologic dissociation')

Front 610

What are the lab findings in poliomyelitis?

Back 610

-CSF with lymphocytic pleocytosis with slight elevation of protein -Virus recovered from stool or throat

Front 611

What are the pathological signs of glioblastoma multiforme (GBM)?

Back 611

-Necrosis -Hemorrhage -Pseudo-palisading

Front 612

What are the signs of LMN lesions seen in poliomyelitis?

Back 612

-Muscle weakness and atrophy -Fasciculations -Fibrillation -Hyporeflexia

Front 613

What are the symptoms of Horner's?

Back 613

1. Ptosis 2. Miosis 3. Anhidrosis and flushing of affected side of face

Front 614

What are the symptoms of poliomyelitis?

Back 614

-Malaise -Headache -Fever -Nausea -Abdominal pain -sore throat

Front 615

What area of the brain is affected by generalized seizures?

Back 615

Diffuse area

Front 616

What artery is compromised in an epidural hematoma?

Back 616

Middle meningeal artery

Front 617

What blood vessels are affected in subdural hemorrhages?

Back 617

Rupture of bridging veins

Front 618

What causes a parenchymal hematoma?

Back 618

-HTN -Amyloid angiopathy -Diabetes Mellitus -Tumor

Front 619

What causes poliomyelitis?

Back 619

Poliovirus

Front 620

What chemical can Parkinson's disease be linked to?

Back 620

MPTP, a contaminant in illicit street drugs

Front 621

What clinical symptoms are present with syringomyelia?

Back 621

Bilateral pain and temperature loss in the upper extremities with preservation of touch sensation

Front 622

What clinical symptoms are present?

Back 622

-Symmetric ascending muscle weakness beginning in the distal lower extremities -Facial diplegia in 50% of cases -Autonomic fx may be severely affected

Front 623

What congenital malformation is often associated with syringomyelia?

Back 623

Arnold Chiari Malformation

Front 624

What damage does cryptococcus cause in the brain?

Back 624

Periventricular calcifications

Front 625

What damage does toxoplasma cause in the brain?

Back 625

Diffuse (intracerebral) calcifications

Front 626

What diseases are berry aneurysms associated with?

Back 626

-Adult polycystic kidney disease -Ehlers-Danlos syndrome -Marfan's syndrome

Front 627

What do partial seizures affect?

Back 627

One area of the brain

Front 628

What does rupture of a berry aneurysm lead to?

Back 628

Stroke

Front 629

What does the spinal tap show in a subarachnoid hemorrhage?

Back 629

Bloody or xanthochromic

Front 630

What event is the rupture of the middle meningeal artery secondary to?

Back 630

Temporal bone fracture

Front 631

What genes is the familial form of Alzheimer's associated with?

Back 631

Genes are chromosomes 1, 14, 19 and 21

Front 632

What is a complex partial seizure?

Back 632

Impaired awareness

Front 633

What is a degenerative disorder of the Spinocerebellar tract?

Back 633

Friedrich's ataxia (olivopontocerebellar atrophy)

Front 634

What is anhidrosis?

Back 634

Absence of sweating

Front 635

What is another name for Guillain-Barre syndrome?

Back 635

Acute idiopathic polyneuritis

Front 636

What is another symptom of Werdnig-Hoffman disease?

Back 636

Tongue fasciculations

Front 637

What is another term for Broca's aphasia?

Back 637

Expressive aphasia

Front 638

What is another term for Wernicke's aphasia?

Back 638

Receptive aphasia

Front 639

What is miosis?

Back 639

Pupil constriction

Front 640

What is PML associated with?

Back 640

JC virus

Front 641

What is ptosis?

Back 641

Slight drooping of the eyelids

Front 642

What is the classic triad of MS?

Back 642

SIN 1. Scanning speech 2. Intention Tremor 3. Nystagmus

Front 643

What is the common name for ALS?

Back 643

Lou Gehrig's disease

Front 644

What is the course of a subdural hemorrhage?

Back 644

Venous bleeding (less pressure) with delayed onset of symptoms

Front 645

What is the course of MS?

Back 645

In most pts, the course is remitting and relapsing

Front 646

What is the incidence of brain tumors in adults?

Back 646

Metastases> Astrocytoma (including glioblastoma)> Meningioma

Front 647

What is the incidence of brain tumors in children?

Back 647

Astrocytoma> Medulloblastoma> Ependymoma

Front 648

What is the most common cause of dementia in the elderly?

Back 648

Alzheimer's disease

Front 649

What is the most common complication of a berry aneurysm?

Back 649

Rupture of the aneurysm

Front 650

What is the most common site for a berry aneurysm?

Back 650

The bifurcation of the anterior communicating artery

Front 651

What is the pathogenesis of Guillain-Barre syndrome?

Back 651

Inflammation and demyelination of peripheral nerves and motor fibers of ventral roots (sensory effect less severe than motor)

Front 652

What is the pathogenesis of Tabes dorsalis?

Back 652

Degeneration of the dorsal columns and dorsal roots due to tertiary syphilis.

Front 653

What is the pathology of Alzheimer's?

Back 653

Associated with senile plaques (beta-amyloid core) and neurofibrillary tangles

Front 654

What is the pathology of Huntington's disease?

Back 654

Atrophy of the caudate nucleus

Front 655

What is the pathology of MS?

Back 655

-Periventricular plaques -Preservation of axons -Loss of oligodendrocytes -Reactive astrocytic gliosis -Increased protein (IgA) in CSF

Front 656

What is the pathology of Parkinson's disease?

Back 656

Associated with Lewy bodies and depigmentation of the substantia nigra

Front 657

What is the pathology of Pick's disease?

Back 657

Associated with Pick bodies, intracytoplasmic inclusion bodies

Front 658

What is the pathology of poliomyelitis?

Back 658

Destruction of anterior horn cells, leading to LMN destruction

Front 659

What is the pathology of syringomyelia?

Back 659

Softening and cavitation around the central canal of the spinal cord.

Front 660

What is the prognosis for a pts diagnosed with a GBM?

Back 660

Very poor

Front 661

What is the second most common cause of dementia in the elderly?

Back 661

Multi-infarct dementia

Front 662

What is the shape of GBMs?

Back 662

Butterfly' glioma

Front 663

What neural deficits are present in Tabes dorsalis?

Back 663

Impaired proprioception and locomotor ataxia

Front 664

What neural tracts are damaged?

Back 664

Crossing fibers of the spinothalamic tract

Front 665

What neurons are affected in ALS?

Back 665

Both the upper and lower motor neurons

Front 666

What neurons are affected in Polio?

Back 666

Lower motor neurons only

Front 667

What seizures are categorized as simple partial?

Back 667

Awareness intact -Motor -Sensory -Autonomic -Psychic

Front 668

What tumor is Horner's syndrome associated with?

Back 668

Pancoast's tumor

Front 669

Where are most brain tumors located in adults?

Back 669

70% are supratentorial (cerebral hemispheres)

Front 670

Where are most childhood brain tumors located?

Back 670

70% below tentorium (cerebellum)

Front 671

Where do berry aneurysms occur?

Back 671

At the bifurcations in the Circle of Willis

Front 672

Where does it initially replicate?

Back 672

The oropharynx and small intestine

Front 673

Where does the 3 neuron oculosympathetic pathway project from?

Back 673

The hypothalamus

Front 674

Where does the 3 neuron oculosympathetic pathway project to?

Back 674

1. Interomediolateral column of the spinal cord 2. Superior cervical (sympathetic) ganglion 3. To the pupil, smooth muscles of the eyelids and the sweat glands

Front 675

Where is Broca's area located?

Back 675

Inferior frontal gyrus

Front 676

Where is Pick's disease specific for?

Back 676

The frontal and temporal lobes

Front 677

Where is the aopE-4 allele located?

Back 677

Chromosome 19

Front 678

Where is the most common site of syringomyelia?

Back 678

C8-T1

Front 679

Where is the p-App gene located?

Back 679

21

Front 680

Where is Wernicke's area located?

Back 680

Superior Temporal Gyrus

Front 681

Which demyelinating disease is seen in 2-4% of AIDS patients?

Back 681

PML

Front 682

Define Ankylosing spondylitis?

Back 682

Chronic inflammatory disease of spine & large joints, sacroilitis, uveitis, & aortic regurgitation

Front 683

Define Ankylosing spondylitis?

Back 683

Chronic inflammatory disease of spine & large joints, sacroilitis, uveitis, & aortic regurgitation

Front 684

Define Celiac sprue

Back 684

Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea.

Front 685

Define Celiac sprue

Back 685

Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea.

Front 686

Define Gout.

Back 686

Precipitation of monosodium urate crystals into joints due to hyperuricemia.

Front 687

Define Gout.

Back 687

Precipitation of monosodium urate crystals into joints due to hyperuricemia.

Front 688

Define Scleroderma

Back 688

Excessive fibrosis & collagen deposition throughout the body; commonly sclerosis of the skin, but also of CV & GI systems & kidney

Front 689

Define Scleroderma

Back 689

Excessive fibrosis & collagen deposition throughout the body; commonly sclerosis of the skin, but also of CV & GI systems & kidney

Front 690

Define Sicca syndrome.

Back 690

dry eyes, dry mouth, nasal & vaginal dryness, chronic bronchitis, reflux esophagitis

Front 691

Define Sicca syndrome.

Back 691

dry eyes, dry mouth, nasal & vaginal dryness, chronic bronchitis, reflux esophagitis

Front 692

In what population is ankylosing sponsylitis more commonly found?

Back 692

males (10-30 year old)

Front 693

In what population is ankylosing sponsylitis more commonly found?

Back 693

males (10-30 year old)

Front 694

In what population is Celiac sprue more commonly found?

Back 694

Assoc. w/ people of northern European descent

Front 695

In what population is Celiac sprue more commonly found?

Back 695

Assoc. w/ people of northern European descent

Front 696

In what population is Goodpasture's syndrome more commonly found?

Back 696

Men 20-40 y/o

Front 697

In what population is Goodpasture's syndrome more commonly found?

Back 697

Men 20-40 y/o

Front 698

In what population is gout more commonly found?

Back 698

Men

Front 699

In what population is gout more commonly found?

Back 699

Men

Front 700

In what population is Osteoarthritis more commonly found?

Back 700

Common in older patients

Front 701

In what population is Osteoarthritis more commonly found?

Back 701

Common in older patients

Front 702

In what population is pseudogout more commonly found?

Back 702

> 50 y/o, both sexes equally

Front 703

In what population is pseudogout more commonly found?

Back 703

> 50 y/o, both sexes equally

Front 704

In what population is Reiter's syndrome more commonly found?

Back 704

Strong predilection for males

Front 705

In what population is Reiter's syndrome more commonly found?

Back 705

Strong predilection for males

Front 706

In what population is Rheumatoid arthritis more commonly found & what the common autoimmune factor present?

Back 706

- Common in females - 80% of RA pt's have positive rheumatoid factor (anti-IgG Ab)

Front 707

In what population is Rheumatoid arthritis more commonly found & what the common autoimmune factor present?

Back 707

- Common in females - 80% of RA pt's have positive rheumatoid factor (anti-IgG Ab)

Front 708

In what population is sarcoidosis more commonly found?

Back 708

black females

Front 709

In what population is sarcoidosis more commonly found?

Back 709

black females

Front 710

In what population is scleroderma more commonly found?

Back 710

75% female

Front 711

In what population is scleroderma more commonly found?

Back 711

75% female

Front 712

In what population is Sjogren's syndrome more commonly found?

Back 712

females between the ages of 40 & 60

Front 713

In what population is Sjogren's syndrome more commonly found?

Back 713

females between the ages of 40 & 60

Front 714

In what population is SLE more commonly found?

Back 714

90% are female & between ages 14 & 45. More common & severe in black females

Front 715

In what population is SLE more commonly found?

Back 715

90% are female & between ages 14 & 45. More common & severe in black females

Front 716

What are the 2 major categories of scleroderma & what findings are they assoc w/?

Back 716

Diffuse scleroderma: widespread skin involvement, rapid progression, early visceral involvement. Assoc. w/ anti-Scl-70 Ab CREST syndrome: Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly & Telangiectasia; limited skin involvement, often confined to fingers & face. More benign clinical course - assoc w/ anticentromere Ab

Front 717

What are the 2 major categories of scleroderma & what findings are they assoc w/?

Back 717

Diffuse scleroderma: widespread skin involvement, rapid progression, early visceral involvement. Assoc. w/ anti-Scl-70 Ab CREST syndrome: Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly & Telangiectasia; limited skin involvement, often confined to fingers & face. More benign clinical course - assoc w/ anticentromere Ab

Front 718

What are the associated sx's & risks for Sjogren's syndrome?

Back 718

- Parotid enlargement - incr risk of B-cell lymphoma - Assoc. w/ RA

Front 719

What are the associated sx's & risks for Sjogren's syndrome?

Back 719

- Parotid enlargement - incr risk of B-cell lymphoma - Assoc. w/ RA

Front 720

What are the characteristic findings in Celiac sprue?

Back 720

Blunting of villi, lymphocytes in the lamina propria, & abnormal D-xylose test

Front 721

What are the characteristic findings in Celiac sprue?

Back 721

Blunting of villi, lymphocytes in the lamina propria, & abnormal D-xylose test

Front 722

What are the common characteristics of Sarcoidosis?

Back 722

immune-mediated, widespread noncaseating granulomas & elevated serum ACE levels

Front 723

What are the common characteristics of Sarcoidosis?

Back 723

immune-mediated, widespread noncaseating granulomas & elevated serum ACE levels

Front 724

What are the common gross findings in Goodpasture's syndrome?

Back 724

pulmonary hemorrhages, renal lesions, hemoptysis, hematuria, crescentic glomerulonephritis

Front 725

What are the common gross findings in Goodpasture's syndrome?

Back 725

pulmonary hemorrhages, renal lesions, hemoptysis, hematuria, crescentic glomerulonephritis

Front 726

What can cause gout?

Back 726

Lesch-Nyan disease, PRPP excess, decreased excretion of uric acid, or G6PD deficiency. Also assoc. w/ the use of thiazide diuretics which competitively ingibit the secretion of uric acid.

Front 727

What can cause gout?

Back 727

Lesch-Nyan disease, PRPP excess, decreased excretion of uric acid, or G6PD deficiency. Also assoc. w/ the use of thiazide diuretics which competitively ingibit the secretion of uric acid.

Front 728

What causes pseudogout?

Back 728

deposition of calcium pyrophosphate crystals w/in the joint space

Front 729

What causes pseudogout?

Back 729

deposition of calcium pyrophosphate crystals w/in the joint space

Front 730

What CV disease state can be caused by SLE?

Back 730

SLE causes LSE (Libman-Sacks Endocarditis): vavular vegetations found on both sides of valve (mitral valve stenosis) & do not embolize

Front 731

What CV disease state can be caused by SLE?

Back 731

SLE causes LSE (Libman-Sacks Endocarditis): vavular vegetations found on both sides of valve (mitral valve stenosis) & do not embolize

Front 732

What drugs can induce a commonly reversible SLE-like syndrome?

Back 732

- procainamide - INH - phenytoin - hydralazine

Front 733

What drugs can induce a commonly reversible SLE-like syndrome?

Back 733

- procainamide - INH - phenytoin - hydralazine

Front 734

What immune marker aids in dx?

Back 734

90% of cases are assoc w/ B27 (gene which codes for HLA MHC-I)

Front 735

What immune marker aids in dx?

Back 735

90% of cases are assoc w/ B27 (gene which codes for HLA MHC-I)

Front 736

What is characteristic about gout crystals?

Back 736

needle-shaped & negatively berefringent.

Front 737

What is characteristic about gout crystals?

Back 737

needle-shaped & negatively berefringent.

Front 738

What is characteristic about pseudogout crystals?

Back 738

basophilic, rhomboid crystals

Front 739

What is characteristic about pseudogout crystals?

Back 739

basophilic, rhomboid crystals

Front 740

What is Reiter's syndrome?

Back 740

a seronegative spondyloarthropath w/ a HLA-B27 link

Front 741

What is Reiter's syndrome?

Back 741

a seronegative spondyloarthropath w/ a HLA-B27 link

Front 742

What is the 'classic triad' for Reiter's syndrome?

Back 742

1. Urethritis (Can't pee) 2. Conjunctivities & ant. uveitis (Can't see) 3. Arthritis (Can't climb a tree)

Front 743

What is the 'classic triad' for Reiter's syndrome?

Back 743

1. Urethritis (Can't pee) 2. Conjunctivities & ant. uveitis (Can't see) 3. Arthritis (Can't climb a tree)

Front 744

What is the 'classic triad' for Sjogren's syndrome?

Back 744

1. dry eyes (conjunctivitis, xerophthalmia) 2. dry mouth (dysphagia, xerostomia) 3. arthritis

Front 745

What is the 'classic triad' for Sjogren's syndrome?

Back 745

1. dry eyes (conjunctivitis, xerophthalmia) 2. dry mouth (dysphagia, xerostomia) 3. arthritis

Front 746

What is the classic pathology for Osteoarthritis?

Back 746

Mechanical: wear & tear of joints leads to destruction of articular cartilage, subchondral bone formation, sclerosis, osteophytes, eburnation, & Heberden's nodes (DIP)

Front 747

What is the classic pathology for Osteoarthritis?

Back 747

Mechanical: wear & tear of joints leads to destruction of articular cartilage, subchondral bone formation, sclerosis, osteophytes, eburnation, & Heberden's nodes (DIP)

Front 748

What is the classic pathology for Rheumatoid arthritis?

Back 748

Autoimmune: inflammatory d/o affecting synovial joints, w/ pannus formation in joints (MCP, PIP), subcutaneous rheumatoid nodules, ulnar deviation, subluxation.

Front 749

What is the classic pathology for Rheumatoid arthritis?

Back 749

Autoimmune: inflammatory d/o affecting synovial joints, w/ pannus formation in joints (MCP, PIP), subcutaneous rheumatoid nodules, ulnar deviation, subluxation.

Front 750

What is the Classic presentation for Osteoarthritis?

Back 750

pain in weight-bearing joints after use (e.g.- at the end of the day), improving w/ rest. No systemic sx's

Front 751

What is the Classic presentation for Osteoarthritis?

Back 751

pain in weight-bearing joints after use (e.g.- at the end of the day), improving w/ rest. No systemic sx's

Front 752

What is the classic presentation for Rheumatoid arthritis?

Back 752

morning stiffness improving w/ use, symmetric joint involvement & systemic symptoms: fever, fatigue, pleuritis, pericarditis

Front 753

What is the classic presentation for Rheumatoid arthritis?

Back 753

morning stiffness improving w/ use, symmetric joint involvement & systemic symptoms: fever, fatigue, pleuritis, pericarditis

Front 754

What is the common immunologic finding for Goodpasture's syndrome?

Back 754

Anti-glomerular basement membrane antibodies produce linear staining on immunofluorescence

Front 755

What is the common immunologic finding for Goodpasture's syndrome?

Back 755

Anti-glomerular basement membrane antibodies produce linear staining on immunofluorescence

Front 756

What is the common tx for gout?

Back 756

allopurinol, probenecid, colchicine, & NSAID's.

Front 757

What is the common tx for gout?

Back 757

allopurinol, probenecid, colchicine, & NSAID's.

Front 758

What is the common tx for pseudogout?

Back 758

no tx

Front 759

What is the common tx for pseudogout?

Back 759

no tx

Front 760

What is the descriptive acrynym for Sarcoidosis?

Back 760

GRAIN Gammaglobulinemia Rheumatoid arthritis ACE incr. Interstitial fibrosis Noncaseating granulomas

Front 761

What is the descriptive acrynym for Sarcoidosis?

Back 761

GRAIN Gammaglobulinemia Rheumatoid arthritis ACE incr. Interstitial fibrosis Noncaseating granulomas

Front 762

What is the useful memory tool for Goodpasture's syndrome?

Back 762

there are TWO Good Pastures for this disease: Glomerulus & Pulmonary. Also, a type II (TWO) hypersensitivity disease

Front 763

What is the useful memory tool for Goodpasture's syndrome?

Back 763

there are TWO Good Pastures for this disease: Glomerulus & Pulmonary. Also, a type II (TWO) hypersensitivity disease

Front 764

What sx's is sarcoidosis commonly associated w/?

Back 764

restrictive lung disease, bilateral hilar lypmphadenopathy, erythema nodosum, Bell's palsy, epithelial granulomas containing microscopic Schaumann & asteroid mobies, uveoparotitis, & hypercalcemia (due to elevated conversion of vit. D to its active form in epithelioid macrophages)

Front 765

What sx's is sarcoidosis commonly associated w/?

Back 765

restrictive lung disease, bilateral hilar lypmphadenopathy, erythema nodosum, Bell's palsy, epithelial granulomas containing microscopic Schaumann & asteroid mobies, uveoparotitis, & hypercalcemia (due to elevated conversion of vit. D to its active form in epithelioid macrophages)

Front 766

What the common pattern of psudogout presentation?

Back 766

Usually affects large joints (classically the knee)

Front 767

What the common pattern of psudogout presentation?

Back 767

Usually affects large joints (classically the knee)

Front 768

Where is gout commonly manifested?

Back 768

Asymmetric joint distribution. Favored manifestation is painful MTP joint in the big toe (podagra). Tophus formation (often on external ear or Achilles tendon)

Front 769

Where is gout commonly manifested?

Back 769

Asymmetric joint distribution. Favored manifestation is painful MTP joint in the big toe (podagra). Tophus formation (often on external ear or Achilles tendon)

Front 770

Addison's disease is characterized by what (remember 4 A's)?

Back 770

Adrenal Atrophy and Absence of hormone production; involves All three cortical divisions

Front 771

All MEN syndromes have what mode of inheritance?

Back 771

Autosomal Dominant

Front 772

An increased risk of carcinoma with atypical cells is seen in which type of fibrocystic breast disease?

Back 772

Epithelial hyperplasia

Front 773

Blood filled, 'chocolate cysts' are seen in what condition?

Back 773

Endometriosis

Front 774

Carcinoid tumors result in what recurrent symptoms? (4)

Back 774

- diarrhea - cutaneous flushing - asthmatic wheezing - right-sided valvular disease

Front 775

Cretin means 'Christ-like,' why is that name used?

Back 775

Those affected were considered so mentally retarded as to be incapable of sinning.

Front 776

Diabetic ketoacidosis (DKA) is caused by what?

Back 776

Excess fat breakdown (usually due to an increase in insulin requirements) and increased ketogenesis from the increase in free fatty acids, which are then made into ketone bodies

Front 777

Do leiomyosarcomas derive from other known tumors?

Back 777

No, they usually arise de novo

Front 778

From where do leiomyosarcomas often protrude?

Back 778

Cervix

Front 779

How do you treat carcinoid syndrome?

Back 779

Treat with octreotide

Front 780

How do you treat postmenopausal osteoporosis?

Back 780

Estrogen replacement therapy

Front 781

Hydatiform moles result in an increase in what hormone?

Back 781

Beta-HCG

Front 782

Increase in what substance is seen in the urine due to carcinoid tumors?

Back 782

5-HIAA

Front 783

Is ACTH increased or decreased when increased cortisol is due to a primary adrenal hyperplasia or neoplasia?

Back 783

Decreased

Front 784

Is ACTH increased or decreased when increased cortisol is due to an iatrogenic etiology?

Back 784

Decreased

Front 785

Is ACTH increased or decreased when increased cortisol is due to Cushing's disease?

Back 785

Increased

Front 786

Is ACTH increased or decreased when increased cortisol is due to ectopic ACTH production (e.g., carcinoid)?

Back 786

Increased

Front 787

Is plasma renin low or high in primary hyperaldosteronism?

Back 787

Low

Front 788

Is plasma renin low or high in secondary hyperaldosteronism?

Back 788

High

Front 789

Leiomyomas are sensitive to what?

Back 789

Estrogen

Front 790

Name 3 common tests for evaluating DM?

Back 790

- Fasting serum glucose - Glucose tolerance test - HbA1c

Front 791

Name 3 specific example of large vessel atherosclerosis due to DM?

Back 791

- Coronary artery disease - Peripheral vascular occlusive disease and gangrene - Cerebrovascular disease

Front 792

Name five possible causes of secondary hyperaldosteronism?

Back 792

1. Renal artery stenosis 2. Chronic renal failure 3. CHF 4. Cirrhosis 5. Nephritic syndrome

Front 793

Name four causes of SIADH.

Back 793

Ectopic ADH CNS disorders/head trauma Pulmonary disease Drugs

Front 794

Name four common presenting symptoms of benign prostatic hyperplasia.

Back 794

1. Increased frequency of urination 2. Nocturia 3. Difficulty starting and stopping the stream of urine 4. Dysuria

Front 795

Name four important chronic manifestations of DM.

Back 795

- Small vessel disease - Large vessel atherosclerosis - Neuropathy - Cataracts, glaucoma

Front 796

Name four risk factors of endometrial carcinoma.

Back 796

1. Prolonged estrogen use 2. Obesity 3. Diabetes 4. HTN

Front 797

Name four treatment modalities for polycystic ovarian syndrome.

Back 797

- Weight loss - OCPs - Gonadotropin analogs - Surgery

Front 798

Name six risk factors of breast disease.

Back 798

1. Gender 2. Age 3. Early first menarche (under 12) 4. delayed first pregnancy (over 30) 5. Late menopause (over 50) 6. Family history of first degree relative with breast cancer at a young age.

Front 799

Name some of the acute manifestations of Diabetes Mellitus (DM)? (7)

Back 799

- Polydipsia - Polyuria - Polyphagia - Weight loss - DKA (type1) - Hyperosmolar coma (type 2) - Unopposed secretion of GH and Epi (exacerbating hyperglycemia)

Front 800

Name syndrome: increased LH due to peripheral estrogen production leads to anovulation and may manifest in amenorrhea, infertility, obesity, and hirsutism.

Back 800

Polycystic ovarian syndrome

Front 801

Name the autoimmune hyperthyroidism with TSH receptor antibodies, opthalmopathy, pretibial myxedema, and diffuse goiter.

Back 801

Graves' disease

Front 802

Name the benign breast tumor: most common tumor under 25 years; small, mobile, firm mass with sharp edges.

Back 802

Fibroadenoma

Front 803

Name the benign breast tumor: tumor of lactiferous ducts; presents with nipple discharge.

Back 803

Intraductal papilloma

Front 804

Name the benign breast tumor: large, bulky mass of connective tissue and cysts; breast surface has 'leaflike' appearance.

Back 804

Cystosarcoma phyllodes

Front 805

Name the disease caused by primary deficiency of aldosterone and cortisol due to adrenal atrophy?

Back 805

Addison's disease

Front 806

Name the histologic type of fibrocystic breast disease: fluid-filled.

Back 806

Cystic

Front 807

Name the histologic type of fibrocystic breast disease: hyperplasia of breast stroma.

Back 807

Fibrosis

Front 808

Name the histologic type of fibrocystic breast disease: increase in number of epithelial cell layers in terminal duct lobule.

Back 808

Epithelial hyperplasia

Front 809

Name the histologic type of fibrocystic breast disease: increased acini and intralobular fibrosis.

Back 809

Sclerosing

Front 810

Name the histologic type of malignant breast disease: cheesy consistency of tumor tissue due to central necrosis.

Back 810

Comedocarcinoma

Front 811

Name the histologic type of malignant breast disease: eczematous patches on nipple.

Back 811

Paget's disease

Front 812

Name the histologic type of malignant breast disease: lymphatic involvement; poor prognosis.

Back 812

Inflammatory

Front 813

Name the histologic type of malignant breast disease: most common carcinoma; firm, fibrous mass.

Back 813

Infiltrating ductal

Front 814

Name three characteristics of inappropriate ADH secretion (SIADH).

Back 814

1. Excessive water retention 2. Hyponatremia 3. Serum hypo-osmolarity with urine osmolarity > serum osmolarity

Front 815

Name three fractures commonly seen due to osteoporosis.

Back 815

1. Vertebral crush fractures 2. Distal radius (Colle's) fractures 3. Vertebral wedge fractures

Front 816

Pheochromocytomas may be associated with what 3 diseases?

Back 816

1. Neurofibromatosis 2. MEN type II 3. MEN type III

Front 817

T/F A partial hydatiform mole is commonly triploid or tetraploid.

Back 817

TRUE

Front 818

T/F DKA is common in Type 1 DM.

Back 818

TRUE

Front 819

T/F DKA is common in Type 2 DM.

Back 819

False, rare

Front 820

T/F Endometriosis often manifests with severe menstrual-related pain and often with infertility?

Back 820

TRUE

Front 821

T/F Fibrocystic breast disease usually does not indicate increased risk of carcinoma.

Back 821

TRUE

Front 822

T/F Genotype of a complete hydatiform mole is 46, XX and is purely maternal in origin.

Back 822

False, it is 46, XX and Purely Paternal in origin

Front 823

T/F Glucose intolerance in Type 1 DM is severe.

Back 823

TRUE

Front 824

T/F Insulin is always necessary to treat Type 1 DM.

Back 824

TRUE

Front 825

T/F Insulin is always necessary to treat Type 2 DM.

Back 825

False, sometimes

Front 826

T/F Leiomyomas often present with multiple tumors.

Back 826

TRUE

Front 827

T/F Leiomyomas often transform into malignant tumors.

Back 827

False, it is rare

Front 828

T/F Leiomyomas progress to leiomyosarcomas.

Back 828

FALSE

Front 829

T/F Leiomyosarcomas: highly aggressive, have a tendancy to recur, and have an increased incidence in blacks.

Back 829

TRUE

Front 830

T/F Risk of breast disease is increased by fibroadenoma and nonhyperplastic cysts.

Back 830

FALSE

Front 831

T/F Type 1 diabetes is often associated with obesity.

Back 831

FALSE

Front 832

T/F Type 1 diabetes is polygenic and strongly due to genetic disposition?

Back 832

False. It is polygenic and only weakly associated with genetic disposition, whereas Type 2 is strongly associated.

Front 833

T/F Women with endometrial hyperplasia are at increased risk for endometrial carcinoma which tends to manifest with vaginal bleeding?

Back 833

TRUE

Front 834

Urinary VMA levels and plasma catecholamines are elevated due to what neoplasms?

Back 834

Pheochromocytoma

Front 835

What are the clinical effects of increased cortisol? (9)

Back 835

- hypertension - weight gain - moon facies - truncal obesity - buffalo hump - hyperglycemia (insulin resistance) - skin changes (thinning striae) - osteoporosis - immune suppression

Front 836

What are the clinical effects of primary hyperaldosteronism? (4)

Back 836

- Hypertension - Hypokalemia - Metabolic alkalosis - Low plasma renin

Front 837

What are the episodic hyperadrenergic symptoms (5 P's) due to pheochromocytomas?

Back 837

- Pressure - Pain (headache) - Perspiration - Palpitations - Pallor/diaphoresis

Front 838

What are the primary hormonal causes of DM?

Back 838

Insulin deficiency (or inefficiency) and glucagon excess

Front 839

What are the six 'Rule of 10's' associated with pheochromocytomas?

Back 839

- 10% Malignant - 10% bilateral - 10% extraadrenal - 10% calcify - 10% kids - 10% familial

Front 840

What are theorized causes for Types 1 and 2 DM?

Back 840

Type 1 - viral or immune destruction of pancreatic beta cells Type 2 - Increased resistance to insulin