Quick associations :) Flashcards
1
Q
What is the most common cause of nephrotic syndrome in blacks and hispanics?
A
FSGS
2
Q
Which nephrotic syndrome is associated with obesity, heroin use, IF tx, CKD due to congenital absence or surgical removal?
A
FSGS
3
Q
Which nephrotic syndrome is associated with Hodgkin lymphoma?
A
MCD
4
Q
Which nephrotic syndrome is associated with chronic conditions (multiple myeloma, TB, RA)?
A
Amyloidosis
5
Q
Which nephrotic syndrome is associated with an Ab to PLA2-R, drugs, infections, SLE, or solid tumors?
A
Membranous nephropathy
6
Q
Which nephrotic syndrome has a “spike and dome” appearance on EM?
A
Membranous nephropathy
7
Q
Which nephrotic syndrome is associated with HBV and HCV?
A
Type I membranoproliferative GN
8
Q
Which nephrotic syndrome is associated with C3 nephritic factor Ab?
A
Type II membranoproliferative GN
9
Q
Which nephrotic syndrome has a “tram track” appearance?
A
Type I membranoproliferative GN
10
Q
Which nephrotic syndrome has eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesions) on LM?
A
Diabetic glomerulonephropathy
11
Q
What is the most common cause of nephrotic syndrome in Caucasian adults?
A
Membranous glomerulonephropathy
12
Q
Which kidney pathology is associated with “crescents?”
A
RPGN (nephritic)
13
Q
What do the crescents of RPGN consist of?
A
Fibrin and plasma proteins with glomerular parietal cells, monocytes, and macrophages
14
Q
What disease processes can lead to RPGN?
A
Goodpasture syndrome, Wegener granulomatosis, Microscopic polyangiitis, Churgg-Strauss
15
Q
What is the most common cause of death in SLE?
A
Diffuse proliferative glomerulonephritis
16
Q
What is the most common cause of nephropathy worldwide?
A
IgA nephropathy (Berger disease)
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.
18
Q
IgA nephropathy is seen with what disease?
A
Henoch-Schonlein purpura
19
Q
What kind of kidney stones look like coffin lids?
A
Struvite
20
Q
What kind of kidney stones look hexagonal?
A
Cystine
21
Q
What kind of kidney stones look rhomboid or rosette-lke?
A
Uric acid
22
Q
Ethylene glycol, VitC abuse, or Crohn disease can lead to what kind of kidney stones?
A
Ca oxalate
23
Q
What is the tx for recurrent calcium kidney stones?
A
Thiazides and citrate
24
Q
What causes struvite kidney stones?
A
UTI with urease positive bugs
25
Renal oncocytomas arise from which cells?
Collecting duct cells
26
What is the WAGR complex?
Wilms tumor, Aniridia, Genitourinary malformation, mental Retardation
27
Transitional cell carcinoma (kidney) arises from what cells?
Urothelial cells
28
Drug-induced interstitial nephritis (tubulointerstitial nephritis) can progress to what?
Renal papillary necrosis
29
How does Drug-induced interstitial nephritis present?
Either asymptomatic or with fever, rash, hematuria, oliguria, CVA tenderness about 2 weeks after starting drug
30
What parts of the kidney are highly susceptible to ischemic injury?
PCT and thick ascending limb
31
What part of the kidney is highly susceptible to nephrotoxic injury?
PCT
32
What is renal papillary necrosis associated with?
DM, acute pyelonephritis, chronic phenacetin use (acetaminophen), HbS/SS
33
How does renal papillary necrosis present?
Gross hematuria and proteinuria
34
Hypersegmented PMNs are seen in what?
B12/folate deficiency
35
What kind of gene malfunctioning leads to alpha-thalassemia?
Alpha globin gene deletion
36
What kind of gene malfunctioning leads to bet-thalassemia?
Point mutations in splice sites and promoter sequences
37
What blood disease presents with a crew cut appearance on xray and chipmunk facies?
Beta thalassemia major
38
Beta thalassemia major predisposes to aplastic crisis by which bug?
Parvovirus B19
39
What leads to basophilic stippling of RBCs?
Lead poisoning, anemia of chronic disease, EtOH abuse, lead poisoning, and thalassemias
40
What is first line treatment for lead poisoning?
Dimercaprol and EDTA
41
Orotic aciduria leads to what kind of anemia?
Megaloblastic macrocytic anemia
42
What is the pathophysiology of orotic aciduria?
Defect in UMP synthase; cannot convert orotic acid to UMP in de novo pyrimidine synthase pathway. Tx is UMP.
43
What is Fanconi anemia?
DNA repair defect
44
What are the lab findings of anemia of chronic disease?
Low Fe, low TIBC, high ferritin
45
What is the treatment for hereditary spherocytosis?
Splenectomy
46
What is HbC defect?
Glutamic acid --> lysine mutation at residue 6 in beta globin. Patients with HbSC have milder disease than those with HbSS.
47
Patients with Paroxysmal nocturnal hemoglobinuria have increased risk for what disease?
AML - 10% develop this
48
What is the pathophysiology of paroxysmal nocturnal hemoglobinuria?
Impaired GPI anchor for decay-accelerating factor that protects RBC from complement --> more complement destruction.
49
What are the clinical findings of paroxysmal nocturnal hemoglobinuria?
Coombs negative hemolytic anemia, pancytopenia, and venous thrombosis
50
What are the lab findings of paroxysmal nocturnal hemoglobinuria?
CD55/59 negative RBCs on flow cytometry
51
What is the tx of paroxysmal nocturnal hemoglobinuria?
Eculizumab
52
What is the mutation of HbS?
Substitution of glutamic acid with valine at position 6 of globin gene
53
What is the treatment of HbSS?
Hydroxyurea (increases fetal Hb)
54
Is transferrin production high or low in pregnancy and OCP use?
High (primary)
55
What are the presenting symptoms of acute intermittent porphyria?
5 Ps: Painful abdomen, Port wine-colored urine, Polyneuropathy, Psych disturbances, Precipitated by drugs, alcohol, starvation
56
What is the treatment of acute intermittent porphyria?
Glucose and heme which inhibit ALA synthase
57
What is the affected enzyme and the presenting symptom of porphyria cutanea tarda?
Uroporphyrinogen decarboxylase; blistering cutaneous photosensitivity
58
What is the presentation of Wiskott-Aldrich syndrome?
Thrombocytopenic purpura, eczema, recurrent infections
59
What is the presentation of Job syndrome?
FATED: course Facies, cold staphylococcal Abscesses, retained primary Teeth, hyper IgE, Derm problems (eczema)
60
What is the treatment of vWF disease?
DDAVP which releases vWF stored in endothelial W-P bodies
61
What are the lab findings of Antithrombin III deficiency?
No direct effect on PT, PTT, or thrombin time, but diminishes increase in PTT following heparin administration because heparin works on antithrombin
62
If a patient has skin and subcutaneous tissue necrosis after warfarin administration, what disease might they have?
Protein C or S deficiency
63
Packed RBCs are used to treat what?
Acute blood loss, severe anemia
64
Fresh frozen plasma is used to treat what?
DIC, cirrhosis, warfarin OD, exchange transfusions in TTP/HUS (increases coag factor levels)
65
What does cryoprecipitate contain? What does it treat?
Fibrinogen, factor VIII, factor XIII, vWF, and fibronectin; Coag factor deficiencies involving fibrinogen and factor VIII
66
What is a leukemoid reaction?
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
What type of cancer presents with constitutional "B" signs and contiguous spread?
Hodgkin lymphoma
68
What are the CD markers of Reed-Sternberg cells (B cell origin)?
CD15 and CD30
69
Which form of Hodgkin lymphoma has the best prognosis?
Lymphocyte-rich
70
Which form of Hodgkin lymphoma has the worst prognosis?
Lymphocyte-mixed or depleted forms
71
Which form of Hodgkin lymphoma is the most common?
Nodular sclerosing
72
EBV can lead to what kind of lymphomas?
Burkitt lymphoma and Hodgkin lymphoma
73
Describe the genetics of Burkitt lymphoma
t(8;14) translocation of c-myc (8) and heavy-chain Ig (14)
74
Describe the genetics of diffuse large B cell lymphoma
t(14;18) or sporadic
75
Describe the genetics of Mantle cell lymphoma
t(11;14) - translocation of cyclin D1 (11) and heavy chain Ig (14)
76
Describe the genetics of Follicular lymphoma
t(14;18) - translocation of heavy-chain Ig (14) and bcl-2 (18)
77
Which lymphoma is CD5+?
Mantle cell
78
Follicular lymphoma can progress to what?
Diffuse large B cell lymphoma
79
How does adult T-cell lymphoma present?
Cutaneous lesions, lytic bone lesions, hypercalcemia
80
What is mycosis fungoides/Sezary syndrome?
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
Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic precursor to what?
Multiple myeloma
82
What is Pseudo-pelger-huet anomaly?
Neutrophils with bilobed nuclei typically seen after chemotherapy
83
Which cancer has smudge cells in the blood smear?
Small lymphocytic lymphoma/chronic lymphocytic leukemia
84
Which leukemia stains TRAP positive?
Hairy cell leukemia
85
What is the treatment for hairy cell leukemia?
Cladribine (2-CDA)
86
What is the median age of onset of AML?
65 YO
87
Auer rods are seen where?
In AML
88
The philadelphia chromosome (t[9;22] bcr-abl) is associated with what neoplasm?
CML
89
CML responds to what treatment?
Gleevac (imatinib)
90
How does Langerhans cell histiocytosis present?
In a child as lytic bone lesions and skin rash or as recurrent otitis media with a mass involving the mastoid bone.
91
The cells of Langerhans cell histiocytosis express what?
S-100 and CD1a
92
How does Langerhans cell histiocytosis look on EM?
Birbeck granules (tennis rackets)
93
Chronic myeloproliferative disorders are associated with what mutation?
JAK2
94
How does PV present?
Intense itching after a hot shower
95
Which endocrine hormones use cGMP for signaling?
ANP, NO
96
Which endocrine hormones use IP3 for signaling?
GnRH, oxytocin, ADH (V1), TRH, histamine (H1), AT-II, gastrin
97
Which endocrine hormones use a steroid receptor for signaling?
VitD, estrogen, testosterone, progesterone, T3/T4, cortisol, aldosterone
98
Which endocrine hormones use an intrinsic tyrosine kinase for signaling?
Insulin
99
Which endocrine hormones use cAMP for signaling?
Glucagon, GHRH, calcitonin, PTH, MSH, ADH (V2), hCG, CRH, TSH, ACTH, FSH/LH
100
Which endocrine hormones use a receptor-associated tyrosine kinase for signaling?
Prolactin, immunomodulators, GH
101
What is the enzyme of thyroid peroxidase?
Oxidation and organification of iodide as well as coupling of monoiodotyrosine (MIT) and di-iodotyrosine (DIT)
102
What is the Wolff-Chaikoff effect?
Excess iodine temporarily inhibits thyroid peroxidase leading to less iodine organification and less T3/T4
103
What is Cushing disease?
ACTH-secreting pituitary adenoma
104
How does cortisol suppress the immune system?
Inhibits histamine, IL-2, and PLA2
105
What is Conn syndrome?
Aldosterone-secreting adrenal adenoma
106
What is Waterhouse-Friderichsen syndrome?
Acute primary adrenal insufficiency due to adrenal hemorrhage associated with Neisseria meningitidis septicemia, DIC, and endotoxic shock
107
What is the most common tumor of the adrenal medulla in children?
Neuroblastoma -
108
What is the most common tumor of the adrenal medulla in adults?
Pheochromocytoma - from chromaffin cells - rule of 10s
109
Hashimoto thyroiditis has an increased risk of what malignancy?
non-Hodgkin lymphoma
110
What are the findings of cretinism (congenital hypothyroidism)?
Pot-bellied, pale, puffy face, protruding umbilicus, protuberant tongue, poor brain development
111
What is subacute thyroiditis (de Quervain)?
Self-limited hypothyroidism often following a flu-like illness. Granulomatous inflammation. High ESR, jaw pain, tender thyroid.
112
What is Riedel thyroditis?
Thyroid replaced by fibrous tissue (hypothyroid). Fibrosis may extend to local structures, mimicking anaplastic carcinoma. Fixed, hard and painless goiter.
113
What is the most common thyroid cancer?
Papillary carcinoma. Excellent prognosis. Orphan Annie eyes. Psammoma bodies, nuclear grooves, increased risk with ret and BRAF mutations, childhood irradiation.
114
Medullary carcinoma of the thyroid arises from what cells?
Parafollicular "C cells" - produces calcitonin. Sheets of cells in an amyloid stroma. Hypocalcemia. Neuroendocrine.
115
What is osteitis fibrosa cystica?
Cystic bone spaces filled with brown fibrous tissue. A consequence of hyperparathyroidism. Stones, bones, groans, and pyschiatric overtones.
116
What is renal osteodystrophy?
Renal disease --> less VitD --> hypocalcemia --> secondary hyperparathyroidism --> bone lesions
117
Describe pseudohypoparathyroidism
Aka Albright hereditary osteodystrophy - aut dom. Unresponsiveness of kidney to PTH. Hypocalcemia, shortened 4th/5th digits, short stature.
118
What are the findings of SIADH?
Excessive water retention, hyponatremia with continued urinary Na excretion, urine osmolarity > serum osmolarity
119
What is the pancreatic histology in DM Type 2?
Islet amyloid polypeptide (IAPP) deposits
120
What is the most common malignancy of the small intestine?
Carcinoid tumor
121
Where are Zollinger-Ellison tumors usually found?
Pancreas or duodenum
122
Which MEN is associated with Marfanoid habitus?
MEN2B
123
Which fungi is acquired through bird or bat droppings?
Histoplasmosis
124
What is the prophylaxis treatment for PCP?
TMP-SMX
125
How does Sporothrix present?
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
How does congenital toxoplasmosis present?
Chorioretinitis, hydrocephalus, and intracranial calcifications
127
What disease does Naegleria fowleri cause?
Rapidly fatal meningoencephalitis, primary amebic encephalitis (PAM)
128
What is the MOA of chloroquine?
Blocks Plasmodium heme polymerase
129
If resistant to chloroquine, which meds are used for malaria?
Mefloquine or atovaquine/proguanil. If life-threatening, use IV quinidine.
130
If infected with vivax/ovale, which medication is added on in addition to regular ones?
Primaquine for hypnozoite
131
How does Babesiosis present?
Fever and hemolytic anemia. NE US. Maltese cross.
132
How is Babesiosis treated?
Atovaquone and azithromycin.
133
How does visceral Leishmaniasis present?
Spiking fevers, hepatosplenomegaly, pancytopenia. 100% fatal if untreated. Macs contain amastigotes.
134
What is the pathophysiology of osteopetrosis?
Failure of normal bone resorption due to defective osteoclasts. Thick dense bones that are prone to fracture.
135
What is the most common mutation involved with osteopetrosis?
Mutation in carbonic anhydrase II - osteoclast can't create acidic environment it needs
136
Which intracranial hemorrhage can cross suture lines and which cannot?
Subdural can, epidural cannot
137
Which intracranial hemorrhage can cross falx and tentorium and which cannot?
Epidural can, subdural cannot
138
Intraparenchymal hemorrhages usuall occur where?
Basal ganglia and internal capsule
139
What is a Charcot-Bouchard microaneurysm?
Associated with chronic hypertension; affects small vessels like in the basal ganglia and thalamus
140
What event leads you to be "wet wobbly and wacky?"
Normal pressure hydrocephalus
141
Tabes dorsalis affects what part of the spinal cord?
Demyelination of dorsal columns and roots
142
MS affects what part of the spinal cord?
Random asymmetric lesions; mostly white matter of cervical region
143
AML affects what part of the spinal cord?
Both UMN and LMN, ant horns and lateral corticospinal tract
144
VitB12 or VitE deficiciency affects what part of the spinal cord?
Demyelination of dorsal columns, lateral corticospinal tracts, and spinocerebellar tracts; ataxic gait, paresthesia, impaired position and vibration sense
145
Which CN passes through the cribriform plate?
CN1
146
Which CNs pass through the middle cranial fossa and through the sphenoid bone?
CNs II-VI
147
What passes through the optic canal?
CN II, ophthalmic artery, central retinal vein
148
Which CNs pass through the superior orbital fissure?
CN III, IV, V1, VI, ophthalmic vein, sympathetic fibers
149
Which CNs pass through the foramen rotundum?
CN V2
150
Which CNs pass through the foramen ovale?
CN V3
151
What passes through the foramen spinosum?
Middle meningeal artery
152
Which CNs pass through the posterior cranial fossa and through the temporal or occipital bones?
CN VII-XII
153
Which CNs pass through the internal auditory meatus?
CNs VII and VIII
154
What passes through the jugular foramen?
CN IX, X, XI jugular vein
155
Which CNs pass through the hypoglossal canal?
CN XII
156
Which CNs pass through the foramen magnum?
Spinal roots of VN XI, brain stem, vertebral arteries
157
What passes through the cavernous sinus?
CN III, IV, V1, V2, VI and postganglionic sympathetic fibers en route to the orbit
158
Uvula deviation indicates what?
CN X lesion - uvula deviates away from side of lesion
159
Jaw deviation indicates what?
CN V motor lesion - jaw deviates toward side of lesion
160
What are the 3 muscles of mastication?
Masseter, temporalis, medial pterygoid
161
What is a Marcus Gunn pupil?
Afferent pupillary defect. Due to optic nerve damage or severe retinal injury.
162
What is scotoma?
Loss of central vision
