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NBME 16 Review Flashcards

1
Q

Postnatal derivative of the ductus areteriosus

A

Ligamentum arteriosum

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2
Q

postnatal derivative of the ductus venous

A

ligamentum venosum

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3
Q

Postnatal derivative of the notochord

A

Nucleus pulposus

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4
Q

Postnatal derivative of the umbilical vein

A

Ligamentum teres hepatis/ Round ligament of the liver

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5
Q

Postnatal derivative of the Umbilical arteries

A

(from aorta/ low O2) median umbilical ligament

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6
Q

postnatal derivative of the allantois

A

urachus (between bladder and umbilicus)

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7
Q

Multiple open reading frames that are translated into several proteins; found in bacteria

A

polycistronic mRNA; eukaryotes are monocistronic

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8
Q

Killed or inactivated Vaccines

A

Rabies virus, Influenza virus (injection), polio virus, and Hepatitis A (RIP Always); humoral response due to maintaining epitope

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9
Q

Subunit vaccines

A

HBV, HPV (6,11, 16, 18), acellular pertussis, N Meningitidis, S pneumo, H Influe type B

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10
Q

Toxoid Vaccine

A

C Tetani, Diphtheria

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11
Q

Phosphorylation of serine and threonine residues on the insulin receptors by serine kinases ultimately leads to Insulin resistance. Which factors initiate this insulin resistance

A

TNF-Alpha, Catecholamines, glucocorticoids and glucagon

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12
Q

artery, vein, nerve, and lymph drainage above the pectinate line

A

visceral innervation, superior rectal artery (of IMA), to portal system via the superior rectal vein, internal iliac nodes

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13
Q

Artery, vein, nerve and Lymph drainage below the pectinate line

A

Somatic innervation (internal br of pudendal n), inferior rectal artery (of pudendal artery), inferior rectal vein to IVC, and superficial iliac nodes

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14
Q

Glycogen storage disease associated with Severe fasting hypoglycemia, increased glycogen in the liver, increased lactate, increased TGs, and UA

A

Von Gierke’s Disease- Type I ; impaired gluconeogenesis, glycolysis; tx with frequent glucose and cornstarch

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15
Q

Deficiency in Von Gierkes

A

Glucose 6 phosphate deficiency (last step)

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16
Q

Glycogen storage disease associated with Cardiomegaly, hypertrophic cardiomyopathy, hypotonia, exercise intolerance,

A

Pompe Disease (Type II)

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17
Q

Deficiency in Pompe Disease

A

Alpha Glucosidase or Acid Alpha Glucosidase deficiency

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18
Q

Glycogen storage disease with limit dextrin accumulation in the cytosol, mild hypoglycemia, mild increase in glycogen stores in the liver, mild increase in TGs and UA, No lactic acidosis

A

Cori Disease (Type III)

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19
Q

Deficiency in Cori Disease

A

Debranching enzyme

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20
Q

Glycogen storage disease associated with painful muscle cramps, myoglobinuria with strenuous exercise, and electrolyte abnormalities; Histo will show increased glycogen stores in muscle

A

McArdles Disease

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21
Q

Enzyme Deficiency in McArdles Disease

A

Glycogen phosphorylase

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22
Q

Murmur heard in a VSD

A

holosystolic murmur at the left sternal border

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23
Q

Murmur heard with Aortic Regurgitation

A

Early Diastolic murmur heard at the left sternal boarder

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24
Q

Murmur heard with an ASD

A

Wide-Split S2

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25
Q

Murmur heard with Aortic Stenosis

A

Systolic Crescendo-Decrescendo murmur at the left sternal border.

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26
Q

N-Acetylglucosaminyl-1-phosphotransferases deficiency is associated with what disease

A

I-Cell Disease; inability of the golgi to phosphorylate mannose residues which leads to failure of the protein to be sent to the lysosome for destruction, instead proteins meant to be destructed accumulate extracellularly

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27
Q

Peroxisome biogenesis disorder: hypotonia, seizures, hepatomegaly, early death due to mutation in PEX genes

A

Zellweger syndrome

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28
Q

Peroxisome deficiency in Alpha-Oxidation: you cant convert phytanic acid to prostatic acid; scaly skin, ataxia, cataracts/ night blindness, shortening of the 4th toe, epiphyseal dysplasia

A

Refsum Disease

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29
Q

Deficiency of peroxisome; X-linked recessive disorder of Beta oxidation which leads to very long chain fatty acid accumulation in adrenal glands, white matter of the brain and testes; progressive disease that leads to adrenal gland crisis, coma, death

A

Adrenoleukodystrophy

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30
Q

Causes of ATN

A

Intrinsic renal injury- from hypo perfusion; muddy brown casts; precipitated by ahminoglycosides, heme products in the blood, IV contrast, and rhabdomyolysis

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31
Q

Causes of Renal papillary necrosis

A

Chronic NSAID use, sickle cell disease, Acute pyelonephritis, and DM; microvascular damage of the renal papilla that leads to necrosis- associated with prolonged experience with a stressor

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32
Q

Causes of AIN

A

NSAIDs, furosemide/ diuretics, penicillin, sulfa drugs; characterized by a hypersensitivity reaction– WBC casts with eosinophilia.

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33
Q

Most common cause of prostatitis

A

Old men: E Coli; young Men–> C Trachomatis, N Gonorrhea

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34
Q

Bacterial that undergoes normal transformation

A

S pneumo, H influe Type B, and Niesseria Species; import short pieces of environmental naked bacterial chromosomal DNA

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35
Q

Frequency of Diseases and frequency of risk-related factors are measured in the present

A

Cross-sectional study

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36
Q

Compares a group of people with diseases to a group of people without disease; looks to see if the odds or a prior risk factor or exposure differs by disease state

A

Case-Control Study

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37
Q

Compares a group with a given exposure or risk factor to a group without that exposure or risk factor

A

Cohort Study

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38
Q

Para aortic LN receive drainage from:

A

nodes draining the testes, ovaries, kidneys and uterus

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39
Q

The Inferior mesenteric LN receive drainage from:

A

the colon from the splenic flexure to the upper rectum

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40
Q

The popliteal LN receive drainage from:

A

LN draining of the dorsolateral foot and posterior calf

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41
Q

Superior mesenteric LN receive drainage from:

A

Ln drainage of the anal canal below the pectinate line, the scrotum and the vulva; skin below umbilicus

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42
Q

The internal Iliac nodes receive drainage from:

A

the anal canal above the pectinate line, bladder, middle 3rd of the vagina, cervix, and prostate

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43
Q

The superior mesenteric Ln receive drainage from

A

the lower duodenum, jejunum, ileum, and the colon all the way to the splenic flexure.

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44
Q

The celiac nodes receive drainage from

A

liver, stomach, spleen, upper duodenum, and pancreas

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45
Q

Gq pathway

A

Phospholipase C–PIP2–> DAG—> Protein Kinase C; IP3—> Increase Ca2+

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46
Q

Gs pathway

A

Adenylate Cyclase –> cAMP–> + PKA; increase Ca in heart, inhibits myosin light chain kinase in smooth muscle

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47
Q

Cafe au lait spots with ragged edges, polyostic fibrous dysplasia, and at least one endocrinopathy

A

McCune Albright Syndrome

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48
Q

Mutation Associated with McCune Albright syndrome

A

G-coupled protein signaling mutation thats either due to somatic mosaicism or gondal mosaicism

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49
Q

autism, mitral valve prolapse, long face with a large jaw, macroorchidism, and large everted ears

A

Fragile X syndrome

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50
Q

inheritance pattern and mutation associated with fragile x

A

X-linked dominant inheritance pattern; Trinucleotide repeat expansion of CGG_n

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51
Q

Trinucleotide repeated associated with huntington’s

A

CAG; AD

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52
Q

Trinucleotide Repeat associated with myotonic muscular dystrophy

A

CTG repeat expansion in the DMPK gene leading to abnormal expression of myotonia protein kinase; Autosomal Dominant

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53
Q

Trinucleotide repeat associated with Friedreich Ataxia

A

GAA; Autosomal Recessive

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54
Q

Renal reabsorption defect that results in increased excretion of amino acids, glucose, PO4, Bicarb, and all substances absorbed at the PCT

A

Fanconi Syndrome; associated with hereditary defects like Wilsons disease, tyrosinemia, glycogen storage disease, ischemia, MM, lead poisoning, etc)

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55
Q

Resorption defect affecting the TALoH ; metabolic alkalosis, hypokalemia, hypercalciuria

A

Bartter Syndrome; NaKCl2 defect: AR

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56
Q

resorption defect affecting the DCT; metabolic alkalosis, hypo magnesia, hypokalemia, hypocalciuria

A

Gitelman Syndrome; NaCl defect; AR

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57
Q

Gain of function mutation that results in increased absorption of Na at the CT; metabolic alkalosis, hypokalemia, hypertension, and decreased levels of aldosterone

A

Liddle syndrome:AD

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58
Q

Hereditary deficiency in 11-beta hydroxysteroid dehydrogenase deficiency leading to increased cortisol–> increased mineralcortcois activity that cause metabolic alkalosis, hypokalemia, hypertension and decreased serum aldosterone

A

Syndrome of Apparent Mineral Corticoid Excess (SAME); AR

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59
Q

Triad of eczema, thrombocytopenia, and low B and T lymphocytes leading to chronic infections

A

Wiskott-Aldrich Syndrome

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60
Q

mutation associated with Wiskott Aldrich

A

WASp gene: leukocytes and platelets unable to reorganize actin cytoskeleton –> defective antigen presentation; XLR

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61
Q

Mutation associated with Ataxia telangiectasia

A

ATM gene; failure to detect DNA damage-> fail to halt progression of cell cycle–> Accum of mutations

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62
Q

Cerebellar defects (ataxia), telangiectasia, and decrease IG

A

Ataxia-Telangiectasia

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63
Q

yellow firm adnexal mass with a microfollicular pattern around pink eosinophilic center

A

granulosa tumor that secretes estrogen

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64
Q

newborn with lethargy, poor feeding and bilious vomiting forming fibrous ladd bands

A

Malrotation of the midgut

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65
Q

bilious vomiting and abdominal distention in the first days of life; Double bubble sign on X-ray (dilated stomach and proximal duodenum)

A

Dudondenal Atresia (failure of gut recanalization)

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66
Q

Failure to obliterate the omphalomesenteric duct

A

mocker’s diverticulum

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67
Q

Brittle kinky hair, growth retardation, hypotonia

A

Menke Disease

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68
Q

Deficiency in Menke Disease

A

Impaired Cu absorption (ATP7A), Cu is a necessary co-factor for lysl oxidase, the enzyme that causes cross linking of collagen

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69
Q

Hyperextensibility of the skin, joints, and tendency to easily bruise

A

Ehlers danlos

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70
Q

Mutation associated with Ehlers Danlos

A

COL5A1 and COL5A2–> mutation in Type V collagen; leads to a problem with cleavage of disulfide rich terminal regions of pro-collagen or cross-llinking issues due to lysine hydroxylase

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71
Q

Mutation associated with Marfans

A

FBN1; fibrillar a glycoprotein that forms sheath around elastin; AD

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72
Q

nerves of the anterior compartment of the lower leg

A

Deep perineal n

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73
Q

Nerve of the posterior compartment of the lower leg

A

tibial

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74
Q

Mutation associated with PAH and its effects

A

BMPR2 which leads to vascular smooth muscle proliferation that kick starts the whole thing; young patients diagnosed with this

75
Q

absent apoB, very low TG, chylomicrons, VLDLS; Deficiency in fat soluble vitamins; late–> neuroabnormalities and acanthosis; histo shows lipid laden Mos

A

Abetalipoproteinemia mutation in MTP gene

76
Q

infant with macrocytic, megaloblastic anemia that is refractory to B12 and folate; elevated orotic acid levels, developmental delay

A

hereditary Orotic Aciduria

77
Q

Deficiency in hereditary orotic Aciduria and treatment

A

UMP synthase; Uridine supplementation

78
Q

Physicians receive a set amount per patient assigned to them per period of time regardless of how much the patient uses the healthcare system

A

Capitation Payment

79
Q

Patient pays for all expenses associated with a single incident of care with a single payment. Most commonly used for elective surgeries.

A

Global Payment

80
Q

Patients can see providers outside their network; higher copays, deductibles for out-of network services

A

Point of service health insurance plan

81
Q

Healthcare organization receives a set amount per service, regardless of ultimate cost, to be divided among all providers and facilities involved

A

Bundled payment

82
Q

BMT recipient develops maculopapular rash, jaundice, diarrhea, hepatosplenomegaly

A

Graft Vs Host Disease

83
Q

MOA of GVHD; name type of HS reaction

A

grafted immunocompetent T cells proliferate and reject host cell; Type IV

84
Q

liver transplant recipient develops thrombosis of graft vessels leading to ischemia and necrosis requiring removal of the graft

A

Hyperacute Transplant rejection

85
Q

MOA of Hyperacute Transplant Rejection; Type of HS reaction

A

Occurs in minutes and is due to pre-existing Ab to donor antigen; Type II HS reaction

86
Q

Liver transplant recipient develops vasculitis of graft vessels with dense interstitial lymphocytic infiltrate. Can be prevented or reversed with immunosuppressants.

A

Acute Transplant Rejection

87
Q

MOA of Acute Transplant Rejection and Type of HS reaction

A

Occurs weeks to months after transplant and is caused by either DONOR CD8+ or CD4+ T cells that become activated against donor MHCs; Type IV- Ab develop after transplant

88
Q

Liver transplant patient develops histological findings consistent with vascular smooth muscle proliferation, parenchymal atrophy and interstitial fibrosis of the grafted vessel. Dominated by Arteriosclerosis; Bronchiolitis obliterans, Accelerate atherosclerosis in the hear, nephropathy, vanishing bile duct syndrome (depends which organ is transplanted)

A

Chronic transplant rejection

89
Q

MOA of Chronic Transplant Rejection; Type of HS Reaction ?

A

CD4+ T cells respond to recipient APCs presenting donor peptides; Type II and IV HS Rxn

90
Q

outflow tract defects that occur due to failure of neural crest migration

A

transposition of the great vessels, tetralogy of Fallot, and Persistent truncus arteriosus

91
Q

Steroid Hormones- Binds directly to DNA

A

progesterone, cortisol, aldosterone

92
Q

Hypopigmented patches, hypoplastic thumbs, pancytopenia, increased risk of malignancy (AML), short stature

A

Fanconi Anemia

93
Q

Defect in Fanconi Anemia and Ataxia Telangiectasia

A

Non-homologous DNA end joining

94
Q

Patient with myopathy, lactic acidosis, and CNS disease; Muscle Biopsy shows red ragged fibers

A

Mitochondrial Myopathy: Mitochondrial inheritance pattern

95
Q

myotonia, muscle wasting, cataracts, testicular atrophy, frontal balding, arrhythmia

A

Myotonic Dystrophy Type I

96
Q

Symptoms appear between the ages of 1-4 and are characterized by regression, motor, or verbal and cognitive abilities, ataxia, seizures, growth failure, stereotypes hand-wringing

A

Rett Syndrome; Girls only

97
Q

Main function of ADH

A

Regulates Serum osmolality (secreted in low volume states) also increases Urea reabsorption in the collecting tubule to maintain the corticopapillary osmotic gradient

98
Q

Cancers have developed chemo evasion by developing what sort of system

A

ATP dependent transporter that pumps Chemo Rx out of the cell; Mutation in the human Multi-drug resistance gene

99
Q

Spirometry seen with morbid obesity

A

decreased FVC, Decreased FEV1, decreased expiratory reserve volume, normal residual volume, and decreased total lung capacity

100
Q

podocyte effacement

A

Minimal change disease, FSGS

101
Q

Spike and dome appearance on EM and Granular IF

A

Membranoglomerulonephritis / membranous nephropathy

102
Q

Wilms Tumor, Aniridia (absence of iris), Genitourinary malformations, mental retardation; WT1 mutation

A

WAGR complex- nephroblastoma

103
Q

Wilms tumor, diffuse mesangial sclerosis (Nephrotic syndrome, dysgenesis of gonads (pseudohermaphroditism), WT1 mutation

A

Denys Drash Syndrome

104
Q

Wilms tumor, macroglossia, organomegaly, hemihyperplasia (WT2)

A

Beckwith-Wiedemann syndrome

105
Q

Achilles Tendon Xanthoma with cholesterol 700+; increased LDL and Cholesterol

A

familial Hypercholesterolemia Type IIA- Defect or Absent LDL Receptor in hepatocytes (homozygous)

106
Q

Achilles Tendon Xanthoma with Cholesterol around 300, increased LDL, VLDL, and Cholesterol

A

Familial Hypercholesterolemia Type IIB (Heterozygous); Decreased LDL R and Increased ApoB

107
Q

Defective ApoE resulting in Palmar xanthomas

A

Dysbetalipoproteinemia

108
Q

Pancreatitis, overproduction of VLDL

A

Hypertriglyceridemia

109
Q

Decreased Lipoprotein lipase or defective Apo-CII with elevated Chylomicrons; Pancreatitis, hepatosplenomegaly, eruptive/ pruritic xanthomas

A

Hyperchylomicronemia

110
Q

Fat Malabsorption, steatorrhea, failure to thrive in an infant; later develop retinitis pigmentosa, spinocerebellar degeneration due to Vitamin E deficiency, progressive ataxia, and acanthocytosis

A

Abetalipoproteinemia

111
Q

Defect in Abetalipoproteinemia

A

ApoB48 and ApoB-100

112
Q

Thyroid nodule, biopsy show overlapping cells with large nuclei, containing sparse, finely dispersed chromatin and psammoma bodies

A

Papillary Thyroid Carcinoma

113
Q

Large follicles with diffuse scalloping

A

Hyperthyroidism- Graves Disease

114
Q

MOA of Graves

A

thyroid-stimulating Ig stimulates TSH receptor on thyroid: + fibroblasts–> GAGs-> pretrial myxedema; Infiltration of T cells in periorbital space causing exopthalamos,

115
Q

HLA Association in Graves

A

HLA-DR3 and HLA-B8

116
Q

Features of hyperthyroidism: Hyperfunctioning follicular cells distended with colloid working independently of TSh

A

Toxic multi nodular goiter

117
Q

Causes of thyroid storm and treatment

A

untreated hyperthyroidism or stress; 4 Ps: Propylthiouracil, propranolol, prednisone, potassium iodide

118
Q

MOA of Hashinmoto Thyroiditis

A

Anti-bodies against Thyroid peroxidase (Antimicrosomal,) and anti-thyroidglobulin Ab

119
Q

HLA associated in Hashimotos

A

HLA-DR3

120
Q

Histology of Hashimoto’s

A

Hurthle cells with lymphoid aggregates forming germinal center in thyroid

121
Q

Hypothyroidism following viral illness/ flu; may present as hyperthyroidism early in course. TENDER thyroid

A

Subacute granulomatous thyroiditis ( de Quervain)

122
Q

Histology of De Quervain Thyroiditis

A

granulomatous inflammation

123
Q

hypothyroidism that can manifest with IgG systemic diseases (autoimmune pancreatitis, retroperitoneal fibrosis, noninfectious aortitis); hard as a rock goiter that is pain less

A

Riedel Thyroiditis

124
Q

Malignancy of the thyroid: Thyroid tissues whose follicles bust through the thyroid capsule

A

Follicular Carcinoma– must remove thyroid to distinguish from thyroid adenoma

125
Q

Sheets of cells in amyloid stroma, elevated calcitonin, low calcium

A

Medullary Carcinoma

126
Q

Mutations associated with medullary carcinoma

A

MEN2A and MEN2B

127
Q

Mutations associated with Papillary Thyroid carcinoma

A

RET/PTC rearrangements, BRAF mutations

128
Q

Compares a group with a given exposure or rick factor to a group without such exposure. Looks to see if exposure or risk factor is associated with later development of disease

A

Cohort Study

129
Q

Looks at frequency of disease and frequency of risk-related factors in the present, asking what is happening

A

Cross-sectional Study

130
Q

Compares a group of people with disease to a group of people without disease

A

Case (diseased)-Control (without disease) Study.

131
Q

Narcolepsy is associated with what in the CSF

A

Hypocretin-1

132
Q

Creutzfeld Jacob is associated with what protein in the CSF

A

14-3-3 protein

133
Q

chorioretinitis, hydrocephalus, intracranial calcifications in an infant

A

Congenital Toxoplasmosis is caused by in-uteron infection- thats why pregnant women aren’t allowed to change kitty liter.

134
Q

hypo pigmentation of the skin, hair, eyes, and brain nuclei, severe intellectual disability, musty body odor, seizures,

A

PKU

135
Q

Albinism is due to a defect in which enzyme

A

Tyrosinase

136
Q

Type I collagen disorder, cant glycosylate collagen

A

osteogenesis imperfecta COL1A1 and 1A2

137
Q

Features of MEN1

A

Pituitary tumors, Pancreatic endocrine tumors (ZE, insulinoma, VIPomas, glucagonomas,etc)

138
Q

MEN2A features

A

Medullary Thyroid Carcinoma, Pheochromocytoma, parathyroid hyperplasia

139
Q

MEN2B features

A

Medullary thyroid carcinoma, Pheochromocytoma, MUCOSAL NEUROMAS, and marfanoid habits because RET mutation is involved.

140
Q

Holosystolic murmurs

A

Mitral Regurg, Tricuspid Regurg and VSD

141
Q

Diarrhea, weight loss, fever in an HIV + patient that has a CD4+ <50; Elevated Alk phos and LDH; treat with Azithromycin

A

Mycobacterium Avium- Mockingbird in cage in the azithromycin sketch

142
Q

The placement of this filter is meant to prevent propagation of DVT, especially in folks with recurrent PE

A

IVC filter placed in the IVC.

143
Q

Amino Acids required for the generation of Propionyl-CoA

A

VIMT: valine, isoleucine, methionine, threonine generates Propionyl-CoA–> Methylmalonyl-CoA–> Succinyl- CoA and enters the TCA

144
Q

Common causes of Bronchiectasis

A

obstruction, impaired immunity, recurrent infection, or cystic fibrosis

145
Q

Complications of OSA

A

Pulmonary HTN, Afib, coronary artery disease, and increased risk of sudden cardiac death.

146
Q

Regulation of heart rate in Afib

A

AV node refractory period

147
Q

Pt with truncal and limb ataxia, dysarthria, dizziness, and visual disturbances dies after unsuccessful treatment of a lung mass. Autopsy of the brain shows cerebellar purkinje cell degeneration. Antibodies Anti-Hu, anti-Yo and Anti-P/Q

A

Autoimmune disease triggered by neoplastic cells of the cancer (Paraneoplastic cerebellar degeneration)- different from mets which would present with HA, and decreased consciousness due to mass affect

148
Q

When is the confidence interval of the Mean between two groups not statistically significant?

A

When the CI includes 0

149
Q

When is the confidence interval of the odds ratio or relative risk not statistically significant

A

When the CI includes 1

150
Q

When is the CI between two groups statistically significant

A

with the confidence intervals dont over lap.

151
Q

What happens to sensitivity and specificity as prevalence changes

A

Nothing; independent of prevalence

152
Q

What happens to PPV and NPV as you decrease prevalence

A

Decrease PPV, increase NPV

153
Q

Shifting down the cut off point of a bimodal distribution results in what changes in sensitivity, specificity, NPV and PPV

A

Increases sensitivity, decreases specificity, decreases PPV, increases NPV

154
Q

Shifting up the cut off point of a bimodal distribution results in what changes to sensitivity, specificity, NPV, PPV

A

decrease in sensitivity, increase in specificity, increase in PPV, and decreased in NPV.

155
Q

Subject or investigator improves because they know they are being studied; Patient in an exercise study exercises more than usual

A

Hawthorne Effect- Type of Confounding

156
Q

Investigator believes in the efficacy of a treat and therefore skews results; Observer expecting signs of recovery is more like to document positive outcomes

A

Pygmalion effect/ Observer Expectancy Bias

157
Q

Prospective studies that lose patients to follow up

A

Attrition Bias

158
Q

Clinical trial that is not representative of actual practice

A

sampling bias

159
Q

Selection bias that occurs when hospitalized patients are chosen as the treatment or control arm

A

Berkson’s Bias

160
Q

Factor is related to both the exposure and outcome, but is not on the causal pathway and therefore can confuse or distort the effects of the exposure on the outcome

A

Confounding bias

161
Q

Four ways to reduce bias

A

Randomization, matching in case control studies, Crossover Study (placebo-washout–drug), and Blinding

162
Q

Looking at the effects of Drug Ex on DVT– stratify group further into smokers and non-smokers and find a higher RR associated with the smokers

A

Effect modification; When a 3rd factor modifies the relationship between the exposure and the outcome

163
Q

Shrinkage of neuronal body, deep eosinophilia of the cytoplasm, pykinosis (condensation of chromatin) of the nucleus with loss of the Nissl substance (stains RER)

A

Irreversible Cell Injury

164
Q

Anterior horn cell that show cell body rounding, displacement of the nuclei and dispersion of Nissl substance to the periphery of cells

A

Wallerian Degeneration- Axonal Reaction to injury

165
Q

Mutation associated with HbC

A

Missense; Glutamic Acid–> Lysine

166
Q

Mutation associated with HbS

A

Missense: Glutamic Acid–> Valine

167
Q

Mechanism of nephropathy in DM

A

Endoneural hyalinization–> Sorbitol accumulation in Schwann cells-> Osmotically active–> protein compression–> hyaline formation–> Nephropathy

168
Q

Fever, joint pain, urticarial rash, fatigue, heptosplenomegaly, elevated LFTs; Multiple sexual partners

A

Acute Hep B infection

169
Q

Subacute vision loss in teens/ young adults; inheritance pattern

A

Leber hereditary Optic Neuropathy= mt inheritance pattern

170
Q

Delusions, severe agitation, disorientation, sensitivity to light, pharyngospasm, excessive salivation, eventual coma and death

A

Rabies

171
Q

MOA of rabies

A

Binds to nAChR (smoking cigar) travels retrograde to the DRG, replicates in motor neurons (puppies under motor), then it spreads to the brain injecting purkinje cells leading to development of eosinophilic inclusions (pink tree) and infecting pyramidal cells of the hippocampus (boat with seahorse and pyramid)

172
Q

Infarct in the pons at the level of the middle cerebral peduncle

A

Trigeminal nerve dysfunction (motor to muscles of mastication and sensory); contralateral hemiparesis (+ babinski- corticospinal tracts), dysdiodochokinesis (ataxic hemiparesis) and dysarthria

173
Q

complication of Alcohol induced pancreatitis that results in what kind of cyst

A

pancreatic pseudocyst which is a cyst full of granulation tissue and fibroblasts because enzymes cause damage–> inflammation –> wound healing

174
Q

Endochondral ossification resulting in hyaline cartilage deposition by chondrocytes: mutation

A

Achondroplasia FGFR3 mutation

175
Q

Impaired osteoblast synthesis of Type I collagen (One-Bone) in bones

A

osteogenesis imperfecta

176
Q

inheritance pattern of OI

A

Autosomal Dominant COL1A1, COL1A2

177
Q

jaundice, vomiting, hepatomegaly, renal dysfunction, E coli infection, cataract and hemolytic anemia

A

Galactose-1-phosphate UDP transferase Deficiency

178
Q

swollen gums, poor wound healing, petechiae, anemia, hemarthrosis, perifollicular and subperiosteal hemorrhages

A

Ascorbic Acid (Vitamin C) Deficiency; more common in adults; Vit K more common in babies

179
Q

pt with ear pain that develops lightheadedness and dizziness with inspection of the ear

A

vasovagal syncope due to innervation of the external ear canal by the vagus nerve

180
Q

Patient presenting with fever, uterine tenderness, and cerivical discharge that is foul smelling

A

Staph Aureus caused by termination of pregnancy which results in retrained products

181
Q

Silver staining bugs

A

Fungi (Coccidiomycosis, Pneumocystis) Legionella, Helicobacter

182
Q

Type of tremor that improves with alcohol use

A

essential tremor

183
Q

S virdidans binds to what on valves

A

platelet-fibrin deposits

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