pathology Flashcards

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1
Q

which is the achalasia pathogenesis?

A

Loss of neurons in the myenteric plexus – failure of LES to relax
*Secondary: Chagas disease, extraesophageal mass

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2
Q

what is the defect of the potassium and sodium channel in long QT syndrome?

A
  • Delayed closed of sodium channel

- Decrease activity of outward (delayed) rectifying potassium channel

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3
Q

Which is the thyroid disease that produce thyroid gland pain and self limited thyroid hormones alterations?

A

Subacute granulomatous thyroiditis (de
Quervain)——–Self-limited disease often following a flu-like illness (eg, viral infection).
- May be hyperthyroid early in course, followed by hypothyroidism.

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4
Q

Which is the explanation of sensory lost in Guillain sx?

A

GB syndrome–> Immune response against MYELIN due to molecular mimickry between the bug and myelin. (against gangliosides of myelin mostly)

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5
Q

Main characteristics of Alport syndrome?

A
  1. X linked recessive
  2. Hematuria, deafness and progressive renal failure
  3. mutation in collagen type IV
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6
Q

Malignant pigmented cells that involved which layer of skin, is associated with greater risk of unfavorable prognosis?

A
  • subcutaneous tissue——–the deeper it goes the dangerous it is.
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7
Q

which are tha characteristics of Pemphigus vulgaris and Bullous pemphigoid?

A

Pemphigus vulgaris— IgG antibody against desmoglein (component ofdesmosomes, which connect keratinocytes in the stratum spinosum)– oral mucosa is also involved. Type II hypersensitivity reaction– nikolsky +.
- Bullous pemphigoid—-Involves IgG antibody against hemidesmosomes (epidermal
basement membrane—Nikolsky sign ⊝.

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8
Q

which is the main component of lewy bodies?

A

α-synuclein—(intracellular eosinophilic inclusions)

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9
Q

Tell five characteristics of abetalipoproteinemia

A
  1. Autosomal recessive
  2. Deficiency in ApoB48, ApoB100—- Chylomicrons, VLDL, LDL absent.
  3. severe fat malabsorption, steatorrhea, failure to thrive.
  4. Later manifestations—retinitis pigmentosa, spinocerebellar degeneration due to vitamin E
    deficiency, progressive ataxia, acanthocytosis.
  5. Treatment: restriction of long-chain fatty acids, oral vitamin E.
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10
Q

urine sediment findiing in acute tubular necrosis

A

muddy brown casts

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11
Q

What is the molecular function of the neurothropic tyrosine kinase (Trka) receptors and which type of ligands bind?

A
  1. Autophosphorilation and down stream phosphorylation of MAPK pathway members
  2. Neurotrophins—- nerve growth factors
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12
Q

which is the clinical presentation of Trka gene mutation?

A

insensitivity to pain, anhydrosis, self-mutilation behavior, congenital retardation and cancer.

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13
Q

whats is mixing study in secondary hemostasis disorders?

A

is the study to differentiate between hemophilia A and coagulation factor inhibitor.

hemophilia A (ptt CORRECTS when mixing patient plasma with normal plasma)
coagulation factor inhibitor (ptt DOES NOT CORRECT)--because is a lot antibodies.
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14
Q

Which is the reason that in von Willebrand disease, PTT is increased?

A

because vWf stabilizes factor VIII

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15
Q

which is the result of Ristocetin test in vWF disease ans why?

A

is an abnormal test.

because normally ristocetin induces vWf binding to GpIb receptor.

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16
Q

which is the best screening test for DIC?

A

D-dimer

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17
Q

difference between DIC and fibrinolysis disorders

A
  • DIC— D dimer elevated
  • FD: normal D dimer (because there is no any thrombi
  • DIC: thrombocytopenia
  • FD: normal platelet count
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18
Q

which is the cause of DIC in pregnant women or during delivery?

A

amniotic fluid produce tissue thromboplastin— activate coagulation cascade

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19
Q

Which are the criteria to diagnose AIDS?

A
  • cd4 <200 or <14%

- HIV with AIDS defecting condition (pneumocystis pneumoniae)

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20
Q

Ischemic lesion in hippocampus (bilateral)

A

Anterograde amnesia—inability to make new

memories.

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21
Q

Primary hypothyroidism

2dary Hypothyroidism

A

1ary: Failure in pituitary gland
2dary: failure in thyroid gland

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22
Q

which are the nucleus that are affected mainly in wernicke korsakoff syndrome?

A
  • medial dorsal nucleus of thalamus

- mammillary bodies

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23
Q

peutz Jeghers syndrome

A

AD
chromosome 19— mutation in serine/threonine kinase 11 gene.
Pigmented mucocutaneous macules and hamartomatous polyps in GI tract —- abdominal pain and GI bleeding tract

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24
Q

Liposarcomas

Lipomas

A

Contain lipoblast— this lipids causing identations and scalloping of nuclear membrane (pleomorphism)
- Most soft tissue sarcomas in adults.
- Multiple mitosis
Lipomas: not pleomorphism and multiple mitosis neither

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25
Q

Chronic alcoholics— developed cerebellar atrophy

A

because thiamine deficiency

loss of purkinje cells y anterior lobes and cerebellar vermis—— ataxia, truncal istability and intention tremor

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26
Q

Hyperthrophic cardiomyopathy

A

AD— cause sudden death in adolescents and young adults

  • mutation in beta myosin heavy chain protein— inappropiate hypertrophy in left ventricle — dyastolic disfunction and outflow obstruction
  • increase cardiac work cause myocardial infarction, coronary arteries unaffected
  • systolic murmur in left sternal border
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27
Q

IgA deficiency characteristics

A
  1. sinopulmonar and GI tract infections
  2. High risk for autoimmune diseases and allergic reactions
  3. anti IgA-IgE antibodies can cause anaphylaxis reaction in patients that are exposed to IgA in blood transfusions
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28
Q

LYNCH SYNDROME

A

germline mutation involving mismatch repair genes MSH 2, 6, MLH 1, PMS2, leading to microsatellite instability of cell DNA: carcinogenesis results from impairment of genes that control cell growth and apoptosis.
Colorectal, ovarian and endometrial cancer

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29
Q

Cyanotic congenital heart disease (5ts)

A
  • Tetralogy of fallot
  • transposition of great arteries
  • truncus arteriosus
  • total anomalous pulmonary venous connection
  • Tricuspid atresia
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30
Q

Pinealomas

A

pineal gland tumors—- obstruction of cerebral aqueduct— hydrocephalus
could cause mass effect in superior colliculus — parinaud sx: upward gaze palsy, abscent light pupillary reflex and impaired comvergence

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31
Q

Kluver - bucy syndrome

A

lesion in temporal lobe mainly in amygdala, relate with HVS-1 encephalitis.
Inappropiate sexual behavior, hyperphagia, amnesia, agnosia and dementia.

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32
Q

Uncal herniation

A

The uncus (medial part of hippocampal gyrus) is pressed through the tentorium cerebelli into the infratentorial space, temporal herniated lobe exerts pressure on oculomotor nerrve (III)

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33
Q

Von hipple lindau disease

A

AR
Mutation in VHL tumor suppresor gene on chromosome 3
hemangioblastomas in cerebellum, cyst in pancreas and kidneys, pheochromocytoma and renal cell carcinoma (clear cell)

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34
Q

Dysplastic nevus syndrome

A

melanoma susceptibility locus CDKN2A on chromosome 9p21, encodes cyclin dependent kinase inhibitor —- cell inappropiately advance in cell cycle

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35
Q

P57 protein

A

a complete mole is distinguished for a partial mole by negative P57, is a product of a paternal imprinted but maternal expressed gene.

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36
Q

Pontine hemorrhage

A

cause pinpoint pupils, loss of horizontal gaze, cuadriparesis, decerebrate posturing, rapidly coma (reticular activating system disruption)

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37
Q

Urinary incontinence

A
  • stress—- decreased tone of urethral sphinchter: leakage with coughing, sneezing
  • Urge—- detrusor hyperactivity
  • Overflow: impaired for detrusor contractility: incomplete empyting and continue dribbling
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38
Q

Frontal lobe syndromes

A

Left: apathy and depression
Right: Desinhibited behavior

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39
Q

Cligger najjar sx
Dubin johnson sx
Gilbert sx

A
  • Absent UDP glucurosyltransferase — indirect
  • Defective liver excretion (black liver), Rotor is similar mild presentation
  • Midly UDP glucorosyltranferase defect— hyperbillirubinemiaonly in stress situations
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40
Q

Patent ductus arteriosus

A

Left to right shunt

splitting S2

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41
Q

Hamartoma

A

“pulmonary chondroma” most common location

Is a excessive growth of tissue type native to the organ of involvement

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42
Q

Annular pancreas

A

abnormal migration of ventral pancreatic bud

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43
Q

small cell carcinoma

A

smoking history
locally central
Histology: round or oval cell with scant cytoplasm and large hyperchromatic nuclei
neuroendocrine markers: enolase, chromogranin and synaptophisin

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44
Q

Trisomy 13

Trisomy 18

A
  • Both have Rocket bottom feet
  • 13: patau, midline defect, GI abnormalities, polydanctilia
  • 18: Edwards, hypertonia, clenched hand and overllaping fingers, Cardiac abnormalities, GI and genitourinary defects.
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45
Q

Hyperacusis

A

lesion in facial nerve —- stapedius nerve a branch– innervates the stapedius muscle

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46
Q

Systemic mastocytosis

A

Mutation in the KIT, receptor of tyrosine kinase
clonal mast cell proliferation: bone marrow, skin and other organs
Excessive histamine secreted by mastocytes: flushing, syncope, hypotension, pruritus, urticaria, gastric acid secretion. the excess of acid inhibits pancreatic enzyme causing diarrhea.

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47
Q

gallstones in crohn disease

A

inflammation of the terminal ileum causing decreased bile acid reabsorption—- supersaturation of bile— gallstones.

48
Q

Microcytic anemia

A
  • thalasemia
  • iron deficiency
  • Chronic disease
  • sideroblastic
49
Q

why gastrectomy cause decrease iron absorption

A

gastrectomy—– decreased acid——- decrease Fe+2 (easier absorpted) —- decreased absortiption

50
Q

when Ferritin goes down…

A

TIBC goes up—– because the mos and liver interpret there is no iron— trying to increase transferrin

51
Q

plummer vinson sx

A

Glossitis+ esophageal web+iron deficiency anemia

52
Q

Kimmelstiel wilson nodules

A

diabetic glomerulopathy—-nodular glomerulosclerosis

thickening of glomerular basal membrane

53
Q

Electrophoresis in major B thalassemia

A

Lilttle or no HBA, shows HbA2 and HbF

54
Q

Acute intermittent porphyria

A
AD
Porphobylinogen deaminase deficiency
Abdominal pain
neurologic symptoms 
port wine colored urine 
Treatment: inhibit ALA synthase, acute attack--- dextrose and IV heme
55
Q

Krukenberg tumor

A

first gastric cancer that has metastasized in ovaries—- Histologic: signet ring cells (nucleus displaced by mucin)

56
Q

Good pasture syndrome

A

autoantibodies against alpha 3 chain of type IV collagen found in glomerular basement membrane and pulmonary capillary membrane.

57
Q

E6 and E7 HPV proteins

A
  • Inhibition of cell cycle regulation
    E6– binds to p53: ubiquination and proteosome degradation— unable to activate apoptosis if DNA persists damage beyond repair
    E7— binds to Rb — promote unregultated DNA replication
58
Q

BCR-ABL

A

Oncogen
Chronic myelogenous leukemia
T- 9, 22
With tyrosine kinase activity

59
Q

Spherocytosis

A

No central pallor= hyperchromia = increased men corpuscular hemoglobin
Molecular defects in genes that encodes proteins of cell membrane: spectrin, ankyrin, band 3 and protein 4.2

60
Q

Myasthenia gravis

A
  • main affected muscles innervated by nerves of brainstem—- symptoms improve with rest, get worst with exercise
  • autoantibodies against postsynaptic acetyl choline nicotinic receptors
  • Damaged motor end plate due to complement fixation
61
Q

Multiple endocrine neoplasia (MEN)

A

MEN 1: pituritary adenomas, pancreatic neoplasia and parathyroid adenomas (MEN1 mutation)
MEN 2A: pheochromocytoma, medullary thyroid cancer (parafollicular C cells and Parathyroid hyperplasia
MEN2B: Same 2A buy with mucosal neuromas and marfanoid habitus (RET protoncogen mutation)

62
Q

Lead poisoning

A

lead bind to sulhydryl groups on proteins —- replaces calcium in Ca+2 dependent cellular functions inhinitory enzymes in heme synthesis
Cognitive impairment, irritability, constipation, abdominal pain, interstitial nephritis and anemia.
Dx; blood lead level and increaed urine gamma aminolevulinic acid

63
Q

Asbesto exposition is related with?

A
  • bronchogenic carcinoma mainly
  • pleural mesothelioma: but is rare
  • pulmonary fibrosis
64
Q

Colon villous adenoma

A

Long glands with villi like projections extending from the surface (cauliflower like projections)
Dysplastic changes
most likely to undergo malignant transformation
Occult or visible bleeding

65
Q

Hyperplastic polyps

A

more common asymptomatic
not dysplastic changes
well differentiated mucosal cells that form glands and crypts, maybe large but no dysplastic

66
Q

Aquired Q-T prolongation

A
hypoK+ and HypoMg++
Drugs: Antiarrhythmics class IA (quinidine) and class III (sotalol) 
macrolides, fluoroquinolones, haloperidol
67
Q

Myocardial infarction changes

A

1-4 hours: nothing
4h-12 h: elongated myocytes
12-24h: hypereosinophylia and pyknotic nuclei
1-3 days: coagulation necrosis, neutrophylic infiltrate
3-7 days: macrophages infiltration
7-10 days: robust phagocyte of death myocytes by mos, beggining formation of granulation tissue
10-14 days: granulation tissue and neovascularization
2 weeks to 2 months: scar formation, progressive collagen deposition

68
Q

Polymyositis

A

proximal muscle weakness
autoantibodies: anti Jo 1- anas
Biopsy: endomysial mononuclear infiltrate, patchy necrosis, cd8
- Complications: interstitial lung disease and myocarditis

69
Q

Familial disbetalipoproteinemia

A

AR
Xanthomas and premature coronary disease
Defect in Apo E3 and 4—– found in Triglyceride, chylomicrones and VLDL
** without apolipoproteins the liver cannot remove chylomicrones and vldl of circulation.

70
Q

Which are the uterine findings in ectopic pregancy?

A

dilated, coiled endometrial glands and vascularized edematous stroma.

71
Q

Increased serum levels of prolactin…

A

Supressed GnRh secretion—– decreased LH and FSH

72
Q

IgA nephropaty

A

LM– mesangial proliferation
EM— mesangial IC deposits
IF: IgA based deposits in mesangium
Renal pathology of henoch scholein purpura
Episode of painless gross hematuria, occurs after an upper respiratory infection

73
Q

Hypothyroidism Myopathy

A

slow of calcium reabsorption by sarcoplasmic reticulum
Mialgia, proximal muscle weakness ans cramping
CKmb elevated

74
Q

Which is the meaning of overlaping skin retractions in breast cancer?

A

involvement of suspensory ligaments of the breast (cooper ligament)

75
Q

Colitis associated carcinoma is more likely to…

A

Affected young patients
Progress from flat and non polypoid dysplasia
Histollogic appears mucinous or/and signet ring morphology
Develop early P53 mutation and LATE APC mutation,
multifocal in nature

76
Q

pathogenesis of pulmonar hypertension due to systemic arterial pressure

A

diastolic left ventricular failure (due to concentric hypertrophy)—————–increase the pulmonary capillary and arterial pressure secondary to pulmonary venous congestion.

77
Q

Intraductal papilloma

A

most common cause of bloody nipple discharge
typically no breast mass or skin changes
Histology: papillary cells with fibrovascular core

78
Q

CREST vs systemic sclerosis

A

CREST: anti-centromere
Systemic: anti- topoisomerase I (Scl -70)

79
Q

pseudogout

A

Crystals of calcium pyrophosphate (romboid shape)
Knee compromise > 50% of cases
synovial fluid: neutrophils

80
Q

poststreptoccocal glomerulonephritis

A
  • All glomeruli are enlarged and hypercellular—– due to leukocyte infiltration and proliferation of endothelial and mesangial cells
  • EM: electron dense deposits (humps) on basement membrane
  • Immunofluorescence: granular deposits of C3 and IgG—“lumpy-bumpy” appearance
81
Q

Mixomatous changes

A

pathologic weakening of connective tissue in the media of large arteries (medial degeneration).
— fragmentation of elastic tissue (cystic medial degeneration)—— predispose to aortic aneurysms
Example: marfan syndrome

82
Q

Hypothyroidism

A

most common cause: hashimoto thyroiditis (autoimmune destruction of thyroid gland)
increase TSH , t4 decrease and in initially stage T3 is normal (because as hort half life, t4-t3 conversion is variable)

83
Q

Restrictive cardiomyopathy

A

idiopathic of infiltrative disorders (amyloidosis (transthyretin-TTR) , sarcoidosis and hemochromatosis)——–diastolic heart failure with preserved ejection fraction

84
Q

Amyloid and DM2

A

islet amyloid polypeptide (amylin)is one factor responsible for beta cell dysfunction

85
Q

Endometriosis vs adenomiosis

A

endometrial glands and stroma ouside of the uterus
most common location is ovaries : chocolate cyst

  • Adenomiosis: endometrial tissue and stroma in myometrium
86
Q

The majority of cancers in head and neck are:

A

squamous cell carcinoma: oral cavity, lower lip, tongue, soft palate and gingiva

87
Q

Pseudohypoparathyroidism

A

Resistance to PTH: damaged in pth receptors or signaling pathway.
Hypocalcemia
Hyperphosphatemia despite elevated level of PTH
Type 1, albright hereditary osteodystrophy: defect in GNAS1, codes alpha subunit of G protein for PTH
Skeletal defects

88
Q

which is the reason of increased gastrin secretion in pernicious anemia?

A

autoimmune destruction of gastric mucosa ———–resulting in atrophic gastritis— causing profound hypochlorhydia– leading to compensatory increase in serum gastrin levels (by G cells, nomally inhibited hy Hydrochloric acid)

89
Q

Age related macular degeneration

A

dry: > 80%, deposition of extracellular material (treat with polivitaminand antioxidant supplement)
Wet: bleeding secondary to choroidal neovascularization. (treat: anti VEGF, ranibizumab)

90
Q

Alcohol induced hepatic steatosis

A

decreased in free fatty acid oxidation secondary to excess NADH production by alcohol dehydrogenase and aldehyde dehydrogenase

91
Q

Aflatoxins

A

aspergillus fumigatus; A1,B2,G1,G2

B1 is mos toxic————- mutation of p53 (G:C——T:A, transversion) = Hematocellular carcinoma

92
Q

Haptoglobin

A

acute phase reactant that bind with free hb to preserved the iron levels
Decreased in hemolytic anemia

93
Q

Henoch sholein purpura

A

small vessel vasculitis
the ag of infection stimulates production of IgA abs (immune complexes)
GI tract (abdominal pain, bleeding), kidneys (IgA nephropathy, Berger disease—-mesangual proliferation and crescent formation, Skin (palpable puruoura in buttocks and lower extremities), Joints (self limited arthalgias).

94
Q

polycystic kidney disease

A

AR: utation PKHD1 —- codes fibrocystin (kidney and liver)
olygohydramnios ——— potter sequence– flattened facies, limb deformities, pulmonary hypoplasia.
AD: cerebral aneurism common complication

95
Q

Orotic aciduria

A
AR
novo pyrimidine synthesis 
physical and mental retardation
megaloblastic anemia
elevated urinary orotic acid
defect in uridine 5´ monophosphate (UMP) synthase 
treatment: uridine supplementation
96
Q

Wallenberg syndrome

A
lateral medulla
V-VIII-IX-V-VII 
spinotalamic tract, sympathetic fibers
inferior cerebellar peduncle 
PICA
** vomiting, vertigo, nystagmus, decrease pain and temperature sensation ipslateral face and contralateral body 
dysphagia, hoarseness 
, decrease gag reflex and ipslat horner sx
97
Q

Pulsus paradoxus in absence of pericardial disease?

A

asthma or COPD exacerbation

98
Q

Classic laboratory abnormalities in multiple myeloma?

A

Normocytic, Nomochromic anemia
Bence jones proteins in urine
Erythrocyte rouleaux formation

99
Q

Atheroclerotic plaque cause angina until they obstruct?

A

75% of lumen— causing imbalance between myocardial oxygen supply and demand

100
Q

Acute rheumatic fever: hystologic finding

A

Pancarditis: interstitial fibrosis with lymphocyte and mos, multinucleated giant cells.
Myocardial granuloma or aschoff body (pathognomonic of myocarditis by ARF)

101
Q

Familial dyslipidemias

A

I: Hyper chylomicronemia (Lipoprotein lipase defect), pancreatitis, xanthomas
IIa: Hypercholesterolemia (LDL receptor defect): accelerate atherosclerosis, MI before 20, xanthomas (achilles tendom)
IV: Hypertriglyceridemia (herpatic over production of VLDL) acute pancreatitis

102
Q

Congenital adrenal Hyperplasia

A
  • *21 hydroxylase: decrease cortisol, aldosterone. Increase testosterone and 17 hydroxyprogesterone: ambiguous genitalia in girls, salt wasting (hypotension, decrase sodium and increase potassium)
    • 11 beta hydroxylase: decrease cortisol and aldosterone, increase testosterone and 11 deoxycorticosterone (weak minerlocorticoid)————-result in increase blood pressure. Ambiguous genitalia in girls.
    • 17 alpha hydroxylase: decrase cortisol, testosterone, increase mineralocorticoids, increase corticosterone (weak glucorticoid)——— all patient like female and hypertension.
103
Q

The presence of nephrotic sx and malignancy suggest?

A

membranous glomerulopathy

104
Q

Membranous glomerulopathy causes

A

Systemic disease: DM, cancer (solid tumors), immunologic disease
Drugs: gold, penicillamine, NSAIDS
Infections: hep B, C, malaria and syphilis
** Thickening of glomerualr capillary wall without and increase in cellularity. In EM showa protruiones “spikes” granular deposits between basement membrane and epitelial cells of IgG and C3

105
Q

Microscopic finding of osteonecrosis

A

dead of bonytrabeculae (empty lacunae) surround fat necrosis

106
Q

Wolff Parkinson white triad

A

pr < 120 mseg
Delta wave
widened QRS complex

107
Q

Rett Sx

A

neurodevelopment disorder in girls
1st normal until 5-18 months —- loss of motor and language skills, stereotipic movements in hands, deceleration of head growth, seizures, autistic features, breathing abnormalitis, de novo mutation x linked HECP2 gene

108
Q

Which is the reason of immune trigger for minimal change disease?

A

systemic T cells dysfunction— leads to production of glomerulus permeability factor (cytokine) ————damage podocyte

109
Q

Kallman sx

A

failure GnRh secreting neurons to migrate from their origin in olfatory placode —-mutation Kal-1 gene or fibroblast growth factor receptor-1 genere

110
Q

Delayed puberty

A

no testis enlargement in boys: 14 years

No breast enlargement in girls: 12 years

111
Q

Distal duodenal ulcer and increased/ normal gastrin level

A

zollinger ellison syndrome (tumor secreting gastrin)

Gatrin rise in responde to administration of secretin

112
Q

Most common cause of death in friederich ataxia?

A

cardiomyopathy: hyperthophic

113
Q

polycystic ovarian syndrome LH and FSH

A

increase both ——- alter hypothalamic hormonal feedback response.

114
Q

Gerstmann Sx

A

ischemic stroke in angular gyrus (integrate mulrisensory, visual, tactile and verbal) in dominant parietal lobe
Middle cerebral artery
agraphia, acalculia, finger agnosia, left- right desorientation

115
Q

Paroxysmal nocturnal hemoglobinuria

A

Complement mediated hemolyisis
Mutated phosphatidylinositol glycan class A (PIGA) gene, Attached cell surface proteins (CD55)– normally inactivated complement.
Leads to uncontrolled complement mediated hemolysis
Manifestations: hemolysis, hemoglobinuria, thrombosis, pancytopenia (due to stem cell injury). Chronic hemolyisis — leads to iron deposition in kidney (hemosiderosis)— damaged the proximal tubule, interstitial scarring and cortilcal infarcts.

116
Q

Hereditary hemorrhagic telangiectasia sx

A

inherited disorder of blood vessels

telangiectasias on skin and mucous membranes, epistaxis, arteriovenous malformations, GI bleeding and hematuria