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Quick associations :) Flashcards

1
Q

What is the most common cause of nephrotic syndrome in blacks and hispanics?

A

FSGS

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2
Q

Which nephrotic syndrome is associated with obesity, heroin use, IF tx, CKD due to congenital absence or surgical removal?

A

FSGS

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3
Q

Which nephrotic syndrome is associated with Hodgkin lymphoma?

A

MCD

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4
Q

Which nephrotic syndrome is associated with chronic conditions (multiple myeloma, TB, RA)?

A

Amyloidosis

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5
Q

Which nephrotic syndrome is associated with an Ab to PLA2-R, drugs, infections, SLE, or solid tumors?

A

Membranous nephropathy

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6
Q

Which nephrotic syndrome has a “spike and dome” appearance on EM?

A

Membranous nephropathy

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7
Q

Which nephrotic syndrome is associated with HBV and HCV?

A

Type I membranoproliferative GN

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8
Q

Which nephrotic syndrome is associated with C3 nephritic factor Ab?

A

Type II membranoproliferative GN

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9
Q

Which nephrotic syndrome has a “tram track” appearance?

A

Type I membranoproliferative GN

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10
Q

Which nephrotic syndrome has eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesions) on LM?

A

Diabetic glomerulonephropathy

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11
Q

What is the most common cause of nephrotic syndrome in Caucasian adults?

A

Membranous glomerulonephropathy

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12
Q

Which kidney pathology is associated with “crescents?”

A

RPGN (nephritic)

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13
Q

What do the crescents of RPGN consist of?

A

Fibrin and plasma proteins with glomerular parietal cells, monocytes, and macrophages

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14
Q

What disease processes can lead to RPGN?

A

Goodpasture syndrome, Wegener granulomatosis, Microscopic polyangiitis, Churgg-Strauss

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15
Q

What is the most common cause of death in SLE?

A

Diffuse proliferative glomerulonephritis

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16
Q

What is the most common cause of nephropathy worldwide?

A

IgA nephropathy (Berger disease)

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17
Q

What is Alport syndrome?

A

Mutation in type IV collagen –> thinning and splitting of glomerular BM. X linked. Isolated hematuria. Glomerulonephritis, deafness, and eye problems.

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18
Q

IgA nephropathy is seen with what disease?

A

Henoch-Schonlein purpura

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19
Q

What kind of kidney stones look like coffin lids?

A

Struvite

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20
Q

What kind of kidney stones look hexagonal?

A

Cystine

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21
Q

What kind of kidney stones look rhomboid or rosette-lke?

A

Uric acid

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22
Q

Ethylene glycol, VitC abuse, or Crohn disease can lead to what kind of kidney stones?

A

Ca oxalate

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23
Q

What is the tx for recurrent calcium kidney stones?

A

Thiazides and citrate

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24
Q

What causes struvite kidney stones?

A

UTI with urease positive bugs

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25
Q

Renal oncocytomas arise from which cells?

A

Collecting duct cells

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26
Q

What is the WAGR complex?

A

Wilms tumor, Aniridia, Genitourinary malformation, mental Retardation

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27
Q

Transitional cell carcinoma (kidney) arises from what cells?

A

Urothelial cells

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28
Q

Drug-induced interstitial nephritis (tubulointerstitial nephritis) can progress to what?

A

Renal papillary necrosis

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29
Q

How does Drug-induced interstitial nephritis present?

A

Either asymptomatic or with fever, rash, hematuria, oliguria, CVA tenderness about 2 weeks after starting drug

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30
Q

What parts of the kidney are highly susceptible to ischemic injury?

A

PCT and thick ascending limb

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31
Q

What part of the kidney is highly susceptible to nephrotoxic injury?

A

PCT

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32
Q

What is renal papillary necrosis associated with?

A

DM, acute pyelonephritis, chronic phenacetin use (acetaminophen), HbS/SS

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33
Q

How does renal papillary necrosis present?

A

Gross hematuria and proteinuria

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34
Q

Hypersegmented PMNs are seen in what?

A

B12/folate deficiency

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35
Q

What kind of gene malfunctioning leads to alpha-thalassemia?

A

Alpha globin gene deletion

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36
Q

What kind of gene malfunctioning leads to bet-thalassemia?

A

Point mutations in splice sites and promoter sequences

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37
Q

What blood disease presents with a crew cut appearance on xray and chipmunk facies?

A

Beta thalassemia major

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38
Q

Beta thalassemia major predisposes to aplastic crisis by which bug?

A

Parvovirus B19

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39
Q

What leads to basophilic stippling of RBCs?

A

Lead poisoning, anemia of chronic disease, EtOH abuse, lead poisoning, and thalassemias

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40
Q

What is first line treatment for lead poisoning?

A

Dimercaprol and EDTA

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41
Q

Orotic aciduria leads to what kind of anemia?

A

Megaloblastic macrocytic anemia

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42
Q

What is the pathophysiology of orotic aciduria?

A

Defect in UMP synthase; cannot convert orotic acid to UMP in de novo pyrimidine synthase pathway. Tx is UMP.

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43
Q

What is Fanconi anemia?

A

DNA repair defect

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44
Q

What are the lab findings of anemia of chronic disease?

A

Low Fe, low TIBC, high ferritin

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45
Q

What is the treatment for hereditary spherocytosis?

A

Splenectomy

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46
Q

What is HbC defect?

A

Glutamic acid –> lysine mutation at residue 6 in beta globin. Patients with HbSC have milder disease than those with HbSS.

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47
Q

Patients with Paroxysmal nocturnal hemoglobinuria have increased risk for what disease?

A

AML - 10% develop this

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48
Q

What is the pathophysiology of paroxysmal nocturnal hemoglobinuria?

A

Impaired GPI anchor for decay-accelerating factor that protects RBC from complement –> more complement destruction.

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49
Q

What are the clinical findings of paroxysmal nocturnal hemoglobinuria?

A

Coombs negative hemolytic anemia, pancytopenia, and venous thrombosis

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50
Q

What are the lab findings of paroxysmal nocturnal hemoglobinuria?

A

CD55/59 negative RBCs on flow cytometry

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51
Q

What is the tx of paroxysmal nocturnal hemoglobinuria?

A

Eculizumab

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52
Q

What is the mutation of HbS?

A

Substitution of glutamic acid with valine at position 6 of globin gene

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53
Q

What is the treatment of HbSS?

A

Hydroxyurea (increases fetal Hb)

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54
Q

Is transferrin production high or low in pregnancy and OCP use?

A

High (primary)

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55
Q

What are the presenting symptoms of acute intermittent porphyria?

A

5 Ps: Painful abdomen, Port wine-colored urine, Polyneuropathy, Psych disturbances, Precipitated by drugs, alcohol, starvation

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56
Q

What is the treatment of acute intermittent porphyria?

A

Glucose and heme which inhibit ALA synthase

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57
Q

What is the affected enzyme and the presenting symptom of porphyria cutanea tarda?

A

Uroporphyrinogen decarboxylase; blistering cutaneous photosensitivity

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58
Q

What is the presentation of Wiskott-Aldrich syndrome?

A

Thrombocytopenic purpura, eczema, recurrent infections

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59
Q

What is the presentation of Job syndrome?

A

FATED: course Facies, cold staphylococcal Abscesses, retained primary Teeth, hyper IgE, Derm problems (eczema)

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60
Q

What is the treatment of vWF disease?

A

DDAVP which releases vWF stored in endothelial W-P bodies

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61
Q

What are the lab findings of Antithrombin III deficiency?

A

No direct effect on PT, PTT, or thrombin time, but diminishes increase in PTT following heparin administration because heparin works on antithrombin

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62
Q

If a patient has skin and subcutaneous tissue necrosis after warfarin administration, what disease might they have?

A

Protein C or S deficiency

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63
Q

Packed RBCs are used to treat what?

A

Acute blood loss, severe anemia

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64
Q

Fresh frozen plasma is used to treat what?

A

DIC, cirrhosis, warfarin OD, exchange transfusions in TTP/HUS (increases coag factor levels)

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65
Q

What does cryoprecipitate contain? What does it treat?

A

Fibrinogen, factor VIII, factor XIII, vWF, and fibronectin; Coag factor deficiencies involving fibrinogen and factor VIII

66
Q

What is a leukemoid reaction?

A

Acute inflammatory response to infection. Increased WBC count with increased neutrophils and neutrophil precursors such as band cells (left shift); increased leukocyte ALP. Contrast with CML (also increased WBC count with left shift, but less leukocyte ALP).

67
Q

What type of cancer presents with constitutional “B” signs and contiguous spread?

A

Hodgkin lymphoma

68
Q

What are the CD markers of Reed-Sternberg cells (B cell origin)?

A

CD15 and CD30

69
Q

Which form of Hodgkin lymphoma has the best prognosis?

A

Lymphocyte-rich

70
Q

Which form of Hodgkin lymphoma has the worst prognosis?

A

Lymphocyte-mixed or depleted forms

71
Q

Which form of Hodgkin lymphoma is the most common?

A

Nodular sclerosing

72
Q

EBV can lead to what kind of lymphomas?

A

Burkitt lymphoma and Hodgkin lymphoma

73
Q

Describe the genetics of Burkitt lymphoma

A

t(8;14) translocation of c-myc (8) and heavy-chain Ig (14)

74
Q

Describe the genetics of diffuse large B cell lymphoma

A

t(14;18) or sporadic

75
Q

Describe the genetics of Mantle cell lymphoma

A

t(11;14) - translocation of cyclin D1 (11) and heavy chain Ig (14)

76
Q

Describe the genetics of Follicular lymphoma

A

t(14;18) - translocation of heavy-chain Ig (14) and bcl-2 (18)

77
Q

Which lymphoma is CD5+?

A

Mantle cell

78
Q

Follicular lymphoma can progress to what?

A

Diffuse large B cell lymphoma

79
Q

How does adult T-cell lymphoma present?

A

Cutaneous lesions, lytic bone lesions, hypercalcemia

80
Q

What is mycosis fungoides/Sezary syndrome?

A

Mature T cell neoplasm; Adults present with cutaneous patches/plaques/tumors with potential to spread to lymph nodes and viscera. Circulating malignant cells seen in Sezary syndrome.

81
Q

Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic precursor to what?

A

Multiple myeloma

82
Q

What is Pseudo-pelger-huet anomaly?

A

Neutrophils with bilobed nuclei typically seen after chemotherapy

83
Q

Which cancer has smudge cells in the blood smear?

A

Small lymphocytic lymphoma/chronic lymphocytic leukemia

84
Q

Which leukemia stains TRAP positive?

A

Hairy cell leukemia

85
Q

What is the treatment for hairy cell leukemia?

A

Cladribine (2-CDA)

86
Q

What is the median age of onset of AML?

A

65 YO

87
Q

Auer rods are seen where?

A

In AML

88
Q

The philadelphia chromosome (t[9;22] bcr-abl) is associated with what neoplasm?

A

CML

89
Q

CML responds to what treatment?

A

Gleevac (imatinib)

90
Q

How does Langerhans cell histiocytosis present?

A

In a child as lytic bone lesions and skin rash or as recurrent otitis media with a mass involving the mastoid bone.

91
Q

The cells of Langerhans cell histiocytosis express what?

A

S-100 and CD1a

92
Q

How does Langerhans cell histiocytosis look on EM?

A

Birbeck granules (tennis rackets)

93
Q

Chronic myeloproliferative disorders are associated with what mutation?

A

JAK2

94
Q

How does PV present?

A

Intense itching after a hot shower

95
Q

Which endocrine hormones use cGMP for signaling?

A

ANP, NO

96
Q

Which endocrine hormones use IP3 for signaling?

A

GnRH, oxytocin, ADH (V1), TRH, histamine (H1), AT-II, gastrin

97
Q

Which endocrine hormones use a steroid receptor for signaling?

A

VitD, estrogen, testosterone, progesterone, T3/T4, cortisol, aldosterone

98
Q

Which endocrine hormones use an intrinsic tyrosine kinase for signaling?

A

Insulin

99
Q

Which endocrine hormones use cAMP for signaling?

A

Glucagon, GHRH, calcitonin, PTH, MSH, ADH (V2), hCG, CRH, TSH, ACTH, FSH/LH

100
Q

Which endocrine hormones use a receptor-associated tyrosine kinase for signaling?

A

Prolactin, immunomodulators, GH

101
Q

What is the enzyme of thyroid peroxidase?

A

Oxidation and organification of iodide as well as coupling of monoiodotyrosine (MIT) and di-iodotyrosine (DIT)

102
Q

What is the Wolff-Chaikoff effect?

A

Excess iodine temporarily inhibits thyroid peroxidase leading to less iodine organification and less T3/T4

103
Q

What is Cushing disease?

A

ACTH-secreting pituitary adenoma

104
Q

How does cortisol suppress the immune system?

A

Inhibits histamine, IL-2, and PLA2

105
Q

What is Conn syndrome?

A

Aldosterone-secreting adrenal adenoma

106
Q

What is Waterhouse-Friderichsen syndrome?

A

Acute primary adrenal insufficiency due to adrenal hemorrhage associated with Neisseria meningitidis septicemia, DIC, and endotoxic shock

107
Q

What is the most common tumor of the adrenal medulla in children?

A

Neuroblastoma -

108
Q

What is the most common tumor of the adrenal medulla in adults?

A

Pheochromocytoma - from chromaffin cells - rule of 10s

109
Q

Hashimoto thyroiditis has an increased risk of what malignancy?

A

non-Hodgkin lymphoma

110
Q

What are the findings of cretinism (congenital hypothyroidism)?

A

Pot-bellied, pale, puffy face, protruding umbilicus, protuberant tongue, poor brain development

111
Q

What is subacute thyroiditis (de Quervain)?

A

Self-limited hypothyroidism often following a flu-like illness. Granulomatous inflammation. High ESR, jaw pain, tender thyroid.

112
Q

What is Riedel thyroditis?

A

Thyroid replaced by fibrous tissue (hypothyroid). Fibrosis may extend to local structures, mimicking anaplastic carcinoma. Fixed, hard and painless goiter.

113
Q

What is the most common thyroid cancer?

A

Papillary carcinoma. Excellent prognosis. Orphan Annie eyes. Psammoma bodies, nuclear grooves, increased risk with ret and BRAF mutations, childhood irradiation.

114
Q

Medullary carcinoma of the thyroid arises from what cells?

A

Parafollicular “C cells” - produces calcitonin. Sheets of cells in an amyloid stroma. Hypocalcemia. Neuroendocrine.

115
Q

What is osteitis fibrosa cystica?

A

Cystic bone spaces filled with brown fibrous tissue. A consequence of hyperparathyroidism. Stones, bones, groans, and pyschiatric overtones.

116
Q

What is renal osteodystrophy?

A

Renal disease –> less VitD –> hypocalcemia –> secondary hyperparathyroidism –> bone lesions

117
Q

Describe pseudohypoparathyroidism

A

Aka Albright hereditary osteodystrophy - aut dom. Unresponsiveness of kidney to PTH. Hypocalcemia, shortened 4th/5th digits, short stature.

118
Q

What are the findings of SIADH?

A

Excessive water retention, hyponatremia with continued urinary Na excretion, urine osmolarity > serum osmolarity

119
Q

What is the pancreatic histology in DM Type 2?

A

Islet amyloid polypeptide (IAPP) deposits

120
Q

What is the most common malignancy of the small intestine?

A

Carcinoid tumor

121
Q

Where are Zollinger-Ellison tumors usually found?

A

Pancreas or duodenum

122
Q

Which MEN is associated with Marfanoid habitus?

A

MEN2B

123
Q

Which fungi is acquired through bird or bat droppings?

A

Histoplasmosis

124
Q

What is the prophylaxis treatment for PCP?

A

TMP-SMX

125
Q

How does Sporothrix present?

A

Spores traumatically introduced into skin, local pustule or ulcer with nodules along draining lymphatics (ascending lymphangitis). Little systemic illness. Tx: Itraconazole or potassium iodide.

126
Q

How does congenital toxoplasmosis present?

A

Chorioretinitis, hydrocephalus, and intracranial calcifications

127
Q

What disease does Naegleria fowleri cause?

A

Rapidly fatal meningoencephalitis, primary amebic encephalitis (PAM)

128
Q

What is the MOA of chloroquine?

A

Blocks Plasmodium heme polymerase

129
Q

If resistant to chloroquine, which meds are used for malaria?

A

Mefloquine or atovaquine/proguanil. If life-threatening, use IV quinidine.

130
Q

If infected with vivax/ovale, which medication is added on in addition to regular ones?

A

Primaquine for hypnozoite

131
Q

How does Babesiosis present?

A

Fever and hemolytic anemia. NE US. Maltese cross.

132
Q

How is Babesiosis treated?

A

Atovaquone and azithromycin.

133
Q

How does visceral Leishmaniasis present?

A

Spiking fevers, hepatosplenomegaly, pancytopenia. 100% fatal if untreated. Macs contain amastigotes.

134
Q

What is the pathophysiology of osteopetrosis?

A

Failure of normal bone resorption due to defective osteoclasts. Thick dense bones that are prone to fracture.

135
Q

What is the most common mutation involved with osteopetrosis?

A

Mutation in carbonic anhydrase II - osteoclast can’t create acidic environment it needs

136
Q

Which intracranial hemorrhage can cross suture lines and which cannot?

A

Subdural can, epidural cannot

137
Q

Which intracranial hemorrhage can cross falx and tentorium and which cannot?

A

Epidural can, subdural cannot

138
Q

Intraparenchymal hemorrhages usuall occur where?

A

Basal ganglia and internal capsule

139
Q

What is a Charcot-Bouchard microaneurysm?

A

Associated with chronic hypertension; affects small vessels like in the basal ganglia and thalamus

140
Q

What event leads you to be “wet wobbly and wacky?”

A

Normal pressure hydrocephalus

141
Q

Tabes dorsalis affects what part of the spinal cord?

A

Demyelination of dorsal columns and roots

142
Q

MS affects what part of the spinal cord?

A

Random asymmetric lesions; mostly white matter of cervical region

143
Q

AML affects what part of the spinal cord?

A

Both UMN and LMN, ant horns and lateral corticospinal tract

144
Q

VitB12 or VitE deficiciency affects what part of the spinal cord?

A

Demyelination of dorsal columns, lateral corticospinal tracts, and spinocerebellar tracts; ataxic gait, paresthesia, impaired position and vibration sense

145
Q

Which CN passes through the cribriform plate?

A

CN1

146
Q

Which CNs pass through the middle cranial fossa and through the sphenoid bone?

A

CNs II-VI

147
Q

What passes through the optic canal?

A

CN II, ophthalmic artery, central retinal vein

148
Q

Which CNs pass through the superior orbital fissure?

A

CN III, IV, V1, VI, ophthalmic vein, sympathetic fibers

149
Q

Which CNs pass through the foramen rotundum?

A

CN V2

150
Q

Which CNs pass through the foramen ovale?

A

CN V3

151
Q

What passes through the foramen spinosum?

A

Middle meningeal artery

152
Q

Which CNs pass through the posterior cranial fossa and through the temporal or occipital bones?

A

CN VII-XII

153
Q

Which CNs pass through the internal auditory meatus?

A

CNs VII and VIII

154
Q

What passes through the jugular foramen?

A

CN IX, X, XI jugular vein

155
Q

Which CNs pass through the hypoglossal canal?

A

CN XII

156
Q

Which CNs pass through the foramen magnum?

A

Spinal roots of VN XI, brain stem, vertebral arteries

157
Q

What passes through the cavernous sinus?

A

CN III, IV, V1, V2, VI and postganglionic sympathetic fibers en route to the orbit

158
Q

Uvula deviation indicates what?

A

CN X lesion - uvula deviates away from side of lesion

159
Q

Jaw deviation indicates what?

A

CN V motor lesion - jaw deviates toward side of lesion

160
Q

What are the 3 muscles of mastication?

A

Masseter, temporalis, medial pterygoid

161
Q

What is a Marcus Gunn pupil?

A

Afferent pupillary defect. Due to optic nerve damage or severe retinal injury.

162
Q

What is scotoma?

A

Loss of central vision

Step 1 (20 decks)

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