Histo Test 2: 9 In '09

Front 1

X-linked Myotubular Myopathy

Back 1

females are carriers
Maps to Xq28, mutation in MTM1 gene which encodes Myotubularin protein lipid phosphatase
centrally located nuclei in skeletal muscle, which resembles the fetal stage of development
delayed muscular developmental milestones
floppy male

Front 2

Duchenne’s muscular dystophy

Back 2

X-linked recessive of Xp21
mutation in gene that encodes Dystrophin, results in weakness b/c actin cant anchor to extrecellular matrix
increased creatine kinase levels in blood
disrupts the sarcolemma, allowing Ca2+ into muscle cell, causing necrosis
onset in males 3/5 years of age

Front 3

Botulism

Back 3

food poisoning caused by Clostridium Botulinum Toxin
inhibits ACh release
causes paralysis, vomiting, nausea

Front 4

Myasthenia gravis

Back 4

Autoimmune disease, ACh receptor antibodies produced
bind and block access of ACh receptor, blocking normal nerve/muscle interaction
causes muscle weakness, ptosis and diplopia

Front 5

Rhabdosarcoma

Back 5

Cancer in striated muscle
3 types:
Embryonal: occurs in head, neck, genitalia
Alveolar: arms, legs, chest, abdomen, anal
Anaplastic: rare in children
causes lumps/swelling that increase in size

Front 6

Central Core Disease

Back 6

mutation in Ryanodine receptor
poor muscle tone and weakness in infants, skeletal deformaties
central abnormality in myofibrils

Front 7

Myocardial Infarction

Back 7

Irreversible necrosis of cardiac muscle due to prolonged ischemia
Lactic Dehydrogenase-1 and creatine kinase detectable in serum

Front 8

McArdle’s Disease

Back 8

affects the breakdown of glycogen via Myophosphorylase in glucose in muscle
not progressive
causes cramping, muscle weakness and pain during exercise

Front 9

Pompe’s Disease

Back 9

Acid Maltase Deficiency, leads to inability to process carbs
glycogen accumulates and is not converted to glucose
S/S: progressive weakness in respiratory muscles, upper legs, arms and shoulders (also cardiac in children)

Front 10

Sinusitis

Back 10

inflammation of ethmoid sinus or maxillary sinus
paralysis of ciliary elevator, deviated septum or upper respiratory infection leads to blockage of drainage
S/S: fever, nasal congestion, pain

Front 11

Cystic Fibrosis

Back 11

nasal polyps appear
increases viscosity of secretions of exocrine glands
decrease in Cl- secretions and increase in Na+ and H2O causes accumulation of thick, viscous mucous in lungs
S/S: pulmonary lesions, thickening of bronchiole walls, increase in # of resp. infections

Front 12

Resp. Distress Syndrome

Back 12

decrease in synthesis of surfactant, increasing surface tension in lungs
failure of type II neumocytes to produce
S/S: Cyanosis and labored breathing
thickening of hyaline membrane lining alveoli

Front 13

Alpha1 Antitrypsin Deficiency leading to Emphysema

Back 13

Alpha 1 functions to maintain elastin by inhibiting neutrophil elastase
smoking--> inflammation--> elastin in wall degraded--> Alpha 1 antitrypin inactivates neutrophile elastase (cant produce more elastin)

Front 14

Adult Respiratory Distress Syndrome

Back 14

due to water in lungs or from smoke inhalation
type II cells become type I to return to normal

Front 15

Atelectasis

Back 15

Loss of lung volume due to inadequate expansion of the air spaces (collapse)
May occur with CF or Emphysema

Front 16

Early Stages of Acute Pneumonia

Back 16

Alveoli fill with exudates containing WBC’s and RBC’s, referred to as hepatization, because the lung resembles the liver.
The lung has enlarged caps giving it a red color
The lung lacks alveoli or alveoli that are functional because they are swollen or filled with PMN’s, RBC’s or fibrin

Front 17

Mesothelioma

Back 17

malignant tumor originating in the mesothelial lining of serous membranes
due to long term exposure to absestos
can metastisize to any organ
S/S= pleural effusion (abnormal liquid in the pleural space), chest pain, and dyspnea

Front 18

Bronchiectasis

Back 18

Permanent dilation of the bronchi and bronchioles due to dystruction of cartilage and elastic tissue by an infection
Causes CF, TB, obstuction by a carcionoma, and primary ciliary dyskinesia
Most common in the lower lobes

Front 19

Atherosclerosis

Back 19

Initial finding is atherosclerotic lesions (fatty streak)
S/S: ischemic heart disease, MI, stroke, and gangrene of limbs
lesions develop on intima
accumulation of lipid causes loss of integrity of endothelium

Front 20

Atherosclerosis with Ischemic Heart Disease

Back 20

Ischemic heart disease is described as the imbalance between the supply and demand of the heart for oxygenated blood
Ischemic events are characterized by anginal pain associated with the loss of oxygenated blood to the region of the heart supplied by the affected vessel.
Coronary artery thrombosis usually precedes and precipitates a MI

Front 21

Marfans Syndrome

Back 21

Fibrillin disorder
Most common cause of death with Marfans is aortic dissection
Affects 3 cardiac structures:
1) Tunica Media- vessels rupture
2) Mitral Valve- prolapse or redundant
3) Chordae Tendinae- changes in them

Front 22

Vessel Aneurysm

Back 22

A localized or diffuse dilation of an artery with a diameter at least 50% greater than the normal size of the artery, due to the weakening of the vessel wall, followed by dilation and a tendency of rupture
Causes= Atherosclerosis and bacterial/fungal infections

Front 23

Thrombosis

Back 23

An intravascular mass attached to a vessel wall composed of variable portions of RBC’s, coagulation factors and platelets.
Can be caused by endothelial damage, esp arterial thrombi

Front 24

Embolism

Back 24

A detached mass (fat, clot, gas…) carried in the body to a distant site
These lodge in various places atrium, pulmonary system, microvasculature throughout the body

Front 25

Hypertension

Back 25

High BP: sustained diastolic P >90 or sustained systolic P > 140
Smooth muscle cells increase in lipid
Tunica intima thickness occurs in fat-free diet
Cardiac muscle cells increase in size and number (hypertropy). Hypertrophy makes the walls less elastic and the heart must work harder

Front 26

Aschoff Body

Back 26

Characteristic lesion of acute rheumatic myocarditis (appear in rheumatic fever)
The Anitschkow cell is the characteristic cell of the body
It is called a cardiac histiocyte

Front 27

Raynaud’s Phenomenon

Back 27

Reversible ischemia of peripheral arterioles usually involving fingers and toes
It’s a vasospasm involving arterioles
It is associated with other illnesses or secondary to another disease and the most common is an autoimmune disease

Front 28

Lymphedema

Back 28

A defect in the transport of lymph because of abnormal lymph development or damaged lymphatic vessels
Chylothorax is the accumulation of high fat containing lymph or chyle in the thorax

Front 29

Peptic Ulcers

Back 29

Open sores on the lining of the stomach, SI and/or esophagus
Can result in internal bleeding and peritonitis (if it penetrates the organ wall)
S/S= burning pain from breastbone to navel; worse when the stomach is empty, improves with food that buffers the stomach contents

Front 30

Pernicious Anemia

Back 30

Disruption of formation of RBC’s in bone marrow due to deficiency in vitamin B12
Can be caused by an autoimmune gastritis and the lack intrinsic factor

Front 31

Zollinger-Ellison Syndrome

Back 31

Gastrin-secreting tumors (aka gastrinomas) of pancreas
Complications include: fulminant (sudden onset) of stomach ulceration, diarrhea (gastrin causes inhibition of water and electrolyte absorption in intestine), Statorrhea (inability to absorb fat due to inactivation of pancreatic lipase by low pH) and hypokalemia
High acid secretion independent of food ingestion

Front 32

Gastic Reflux (Barrett’s Esophagus)

Back 32

Due to extensive gastroesophageal reflux
S/S: burning sensation below & behind breastbone
TX: antacids and acid blocking drugs. No improvement by blocking acid secretion

Front 33

Gluten Enteropathy

Back 33

Results from destructive effects of certain glutens on intestinal villi
Reduces SA available for absorption
TX: elimination of wheat and rye products from diet
Seen as failure to thrive in infants

Front 34

Inflammatory Bowel Disease

Ulcerative Colitis

Back 34

Includes Ulcerative Colitis and Crohn’s Disease
S/S: diarrhea, pain and periodic prolapses
Ulcerative Colitis can affect mucosa of LARGE INTESTINES

Front 35

Inflammatory Bowel Disease

Crohn’s Disease

Back 35

Crohn’s disease affects ANY segment of intestinal tract (small & large)
Crohn’s disease is a chronic inflammatory process (may be autoimmune) with immune system cells producing cytokines damaging intestinal mucosa, progressing into submucosa and muscularis externa
S/S: Diarrhea, chills, fever, nausea, weakness, anorexia and weight loss

Front 36

Colorectal Carcinoma

Back 36

Second highest cause of cancer death in US (3rd most common in men, 2nd most common in women) usually affect 55 or older
Usually arises from adenomatous polyps
May be asymptomatic for years, rectal bleeding frequently present
Probably diet related (high fat, refined carbs and low fiber)
TX: surgery with or without chemo and radiation

Front 37

Salivary Gland Tumors

Back 37

Approx 80% are benign, most originate from Parotid glands (but may come from submandibular or sublingual or even minor glands)
Most are pleomorphic (65%), they are characterized by epithelial tissue containing ductal and myoepithelial cells with areas of gs from CT.
Pts present with painless swelling of the involved gland and may also have numbness or tingling of the innervated muscles because of the nerve envolvment
TX: surgical removal of the tumor and sometimes the gland. Postoperative radiation is often necessary.

Front 38

Sjogren Syndrome

Back 38

S/S: Xerstomia (dry mouth) and dry, gritty eyes. Component of systemic disease
DX: part of the diagnosis is an inner lip biopsy. Positive if there are aggregates of 50 lymphocytes adjacent to the mucous acini/4mm2 gland. Lymphycytes eventually replace the acini

Front 39

Acute Pancreatitis

Back 39

An inflammatory condition of the exocrine pancreas that results from injury to the acinar cells. Acinar cell injury and duct obstruction are the major initiators.
Some causes:
secretion against obstruction (gallstone)
Inappropriate activation of proenzymes
AIDS
Ethanol
Most common causes are alcohol abuse and bile duct obstruction

Front 40

Cystic Fibrosis

Back 40

80% of CF pts have visible secretory abnormalities of the pancreas
CF causes mucous inspissation in the ducts and secondary atrophy of exocrine gland (due to blocking the lumens with dehydrated secretions)

Front 41

Centrilobular Necrosis

Back 41

Hepatocytes in zone 3 undergo ischemic necrosis, this may occur with CHF when they might not receive adequate oxygenation.
No change in cells in zone 1 and 2 (because these zones receive blood first from the sinusoids)

Front 42

Alcoholic Hepatitis

Back 42

Histological changes seen:
Swollen (balloon cells) hepatocytes in centrilobular region
Mallory bodies in hepatocytes
PMN’s present
Collagen deposited (fibrosis) around the central vein
Some hepatocytes become fat cells

Front 43

Cirrhosis

Back 43

Death of hepatocytes leading to scarring or increased production of collagen destroying the normal architecture (hepatocytes are replaced by CT)

Front 44

Cholecystitis

Back 44

Gallstone impacted in cystic duct leading to: Thickened muscular layer (due to trying to overcome the pressure)
Impaired break down of fat
3) High pressure in GB rearranges the mucosa

Front 45

Role of Hepatic Stellate Cells in Portal Hypertension

Back 45

Hepatic stellate change function/type to lay down matrix and affect sinusoids
Portal hypertension – caused by changes in the hepatic stellate cell. Lose storage function and begin to produce collagen and ECM material; also convert to myofibroblasts and constrict sinusoids. Matrix increase and constriction leads to portal hypertension.