Path quick facts Flashcards
1
Q
Wire looping appearance on light microscopy of renal bx
A
Lupus nephritis
2
Q
Crescent formation in the glomeruli
A
Rapid progressive glomerulonephritis
3
Q
Anti-GBM abs, hematuria, hemoptysis
A
Goodpasture syndrome (rapid crescentic GN)
4
Q
Deposits of IgA in the mesangium
A
Post-streptococcal glomerulonephritis (remember subepithelial humps)
5
Q
Lumpy-bumpy deposits of IgG, IgM, and C3 in the mesangium
A
Acute post-streptococcal GN
6
Q
Linear pattern of IgG deposition on immunofluorescence of kidney bx
A
Goodpasture syndrome (hemoptysis + hematuria)
7
Q
Cola-colored urine + periorbital edema
A
Acute Post-strep GN
8
Q
Crescents of fibrin and plasma proteins on bx + c-ANCA
A
Granulomatosis with polyangitis
9
Q
Crescents of fibrin and plasma proteins on bx + p-ANCA
A
microscopic polyangitis
10
Q
Renal pathology associated with Henoch-Schonlein purpura
A
IgA nephropathy (berger dz)
11
Q
Eye problems (retinopathy, lens dislocation) + glomerulonephritis + sensorineural deafness
A
Alport syndrome (defect in Type IV collagen)
12
Q
Nephritic syndrome and nephrotic syndrome, Tram-track appearance of GBM, subendothelial IC deposits, associated with hep B, hep C, lupus, subacute endocarditis
A
Membranoproliferative glomerulonephritis
13
Q
Most common cause of nephrotic syndrome in kids, associated with recent infection, immunization or immune stimulus, tx with corticosteriods
A
minimal change disease
14
Q
Associated with HIV, massive obesity, higher incidence in AA and Hispanics, effacement of foot processes
A
Focal segmental glomerulosclerosis
15
Q
GBM thickening, “spike and dome” + subepithelial deposits, can be seen with SLE
A
Membranous nephropathy
16
Q
deposits in mesangium, show apple-green birefringence with Congo red stain
A
Amyloidosis (seen in MM)
17
Q
Eosinophilic nodular deposits = Kimmelstiel-Wilson lesions, mesangial expansion
A
Diabetic glomerulonephropathy (most common cause of end stage renal dz in US)
18
Q
RBC casts
A
glomerulonephritis
19
Q
WBC casts
A
acute pyelonephritis
20
Q
Granular “muddy brown” casts
A
acute tubular necrosis
21
Q
Waxy casts
A
end-stage renal disease
22
Q
Radiopaque, envelope shaped kidney stone
A
Calcium oxalate
23
Q
What are calcium oxalate stones associated with?
A
Chron’s dz (malabsorption), vitamin C abuse, ethylene glycol ingestion, vit C abuse, low citrate with low urine pH
-most common stone
24
Q
Tx for calcium kidney stones
A
thiazides –> cause Ca2+ to be retained in serum
25
What infections are ammonium magnesium stones associated with?
Proteus mirabilis, Staph saprophyticus, Klebsiella
26
Radiopaque coffin lid shaped kidney stone
AMP (struvite)
27
Radioluscent rhomboid or rossette shaped kidney stone
uric acid stone
28
What are uric acid stones associated with?
hyperuricemia --> gout, Lesch-Nyhan
29
Radioluscent hexagonal kidney stone
cysteine stones
30
What are cysteine stones associated with?
Hereditary condition that prevents reabsorption of cysteine in the proximal tubule, begins in childhood
31
Most common childhood kidney tumor
Wilms tumor
32
What is the WAGR complex?
Wilms tumor, Aniridia (absent iris), Genitourinary malformations, Mental Retardation (deletion in WT1 gene)
33
Risk factors associated with transitional cell carcinoma
smoking, napthylamine dyes, aniline dyes, cyclophosphamide
most common tumor of urinary tract system
34
Risk factors for squamous cell carcinoma of the bladder
chronic cystitis, smoking, chronic nephrolithiasis, Schistosoma haematobium
35
Most common renal malignancy
Renal cell carcinoma - originates from PCT cells
36
Risk factors for renal cell carcinoma
smoking, men 50-70
37
What are the paraneoplastic syndromes associated with RCC?
2' Polycythemia - excess EPO
| secrete excess PTHrP --> bone resorption
38
Sources of ectopic EPO production and 2' polycythemia
"Potentially Really High Hematocrit" - Pheocromocytoma, Renal cell carcinoma, Hepatocellular carcinoma, Hemangioblastoma
39
Causes of polycythemia
Hypoxia - lung dz, high altitude
Ectopic EPO
Primary Polycythemia
Down syndrome
40
Blistering cutaneous photosensitivity, tea colored urine, hypertrichosis, facial hyperpigmentation, can be associated with alcohol or Hep C (increased LFTs)
Porphyria cutanea tarda
41
Defective enzyme in porphyria cutanea tarda
Uroporphyrinogen decarboxylase - converts uroporphyrinogen III to coprophyrinogen
42
Painful abdomen, port-wine colored urine, polyneuropathy, psychological disturbances, precipitated by drugs
Acute intermittent porphyria
Tx = heme and glucose to inhibit ALA synthase
43
Defective enzyme in Acute intermittent porphyria
Porphobilinogen deaminase - converts porphobilinigen to hydroxymethylbilane
44
Rate limiting step in heme synthesis
Delta-aminolevulinic acid synthase (ALA synthase) - requires B6 cofactor
45
Enzymes inhibited by lead in the heme synthesis pathway
Ferrochelatase, Delta-aminolevulinic acid dehydratase
46
Blue lines on gum, hyperluscent lines on long bones in X-ray, abdominal pain, growth impairment, HA, hearing problems, wrist/foot drop, encephalopathy, basophilic stippling
Lead poisoning
Tx - succimer + dimercaprol in kids, EDTA in adults
47
Acanthocyte (spur cell)
Abetalipoproteinemia, liver dz
48
Basophilic stippling
lead poisoning
49
Teardrop cell (dacrocyte)
Bone marrow infiltration
50
Bite cell (degmacyte)
G6PD def.
51
Echinocyte (burr cell)
Liver dz, renal dz, pyruvate kinase def
52
Elliptocyte
hereditary elliptocytosis
53
Ringed sideroblast
disorders of heme synthesis - found on BM bx
54
Schistocytes
DIC, TPP/HUS, HELLP syndrome - fragments of RBCs
55
Spherocyte
Hereditary spherocytosis
56
Target cells
"HALT at target" - HbC dz, Asplenia, Liver dz, Thalassemia
57
Heinz bodies
G6PD
| caused by oxidized Hb molecules that precipitate, can become bite cells
58
Howell-Jolly bodies
Asplenia
| -nuclear remnants found in RBCs, only 1/cell
59
Causes of hypochromic, microcytic anemia
iron deficiency, lead poisoning, thalassemia
60
Skull x-ray shows hair-on-end appearance
Beta thalassemia, sickle cell disease
61
decreased serum iron, increased TIBC/transferrin, decreased ferritin, decreased transferrin saturation
IDA
62
decreased serum iron, decreased TIBC/transferrin, increased ferritin (high stores)
AOCD
63
increased serum iron, increased ferritin, increased transferrin saturation
Hemochromatosis
64
Causes of microcytic anemia
Iron deficiency, late anemia of chronic dz, thalassemias, lead poisoning, sideroblastic anemia
65
Alpha-globin gene deletions, Asian and African populations, form excess beta globin chains and form HbH (four beta globin chains pair)
Alpha thalassemia
66
Beta-globin absent or underproduced, increase in HbF, crew cut look on x-ray due to marrow expansion, target cells, Mediterranean populations, chipmunk face
Beta thalassemia - tx with blood transfusions
minor = increased HbA2
67
What is the genetic cause of sideroblastic anemia?
defect in delta-ALA synthase gene (also caused by drugs, alcohol, B6 def., isoniazid)
tx - B6 supplement (cofactor for ALA synthase)
68
Microcytic anemia + swallowing difficulty + glossitis
Plummer-Vinson syndrome (iron deficiency with esophageal webs)
69
Causes of megaloblastic anemia (hypersegmented neutrophils)
Folate deficiency, B12 deficiency, orotic aciduria
-can be decreased intake, increased requirement (preggers), mtx or TMP-SMX
70
Neuro sx, increased homocysteine, neuro sx, megaloblastic anemia, increased methylmalonic acid, hypersegmented neutrophils
B12 deficiency
-can be seen in strick vegans, malabsorption, Diphyllobothrium latum
71
Increased homocysteine, megaloblastic anemia, normal methylmalonic acid, hypersegmented neutrophils
Folate deficiency
72
Causes of non-megaloblastic macrocystic anemia
Liver disease, alcoholism
73
Mechanism of AOCD
inflammation --> increases hepcidin which binds all ferroportin in macs and intestinal cells --> can't transport iron to bone marrow for RBC synthesis
74
Drugs that cause aplastic anemia
Chloramphenicol, Cancer drugs, Benzene, Zidovudine (HIV tx)
75
Causes of aplastic anemia
```
Radiation
Fanconi anemia
Drugs
Infections - EBV, Parvo B19, HIV, hepatitis
Idiopathic
```
76
Pancytopenia + hypocellular bone marrow with fatty infiltrate
Aplastic anemia
| -can tx with GM-CSF
77
Elevated creatine + normocytic anemia
renal failure (can't make EPO)
78
Spherocytes on smear, splenomegaly, increased MCHC, increased RDW, + osmotic fragility test
Hereditary spherocytosis
| -tx = splenomegaly
79
Hemolytic anemia in a newborn
think about pyruvate dehydrogenase def.
80
Glutamic acid --> lysine mutation in Beta-globin gene
HbC disease - milder than sickle cell dz
81
point mutation in beta-globin gene glutamic acid --> valine
Sickle cell anemia
82
Precipitating factors for HbS to polymerize in sickle cell anemia
low O2, high altitude, dehydration, acidosis
83
Treatment for sickle cell anemia
Hydroxyurea - increases concentration of HbF
84
Complications of sickle cell anemia
aplastic crisis with ParvoB19, autosplenectomy (vax for S. pneumo and Hib), splenic infarcts, Salmonella osteomyelitis, painful crisis in hands and feet or acute chest syndrome (vaso-occlusion), Renal papillary necrosis
85
Causes of Warm AI hemolytic anemia
congenital immune issues, CLL, EBV, HIV, SLE
| -IgG ab
86
Causes of Cold AI hemolytic anemia
EBV mononucleosis, Mycoplasma pneumonia, EBV, CLL
| -IgM ab + complement destruction
87
Test for AI hemolytic anemia
Coombs
- direct = testing if RBCs are coated with Ig
- indirect = testing for presence of anti-RBC Ig in serum
88
Painful cyanosis in fingers and toes with hemolytic anemia
Cold AI hemolytic anemia
89
Red urine in the morning and fragile RBCs, hemosiderinuria thrombosis
Paroxysmal nocturnal hemoglobinuria
90
Labs seen with von Willebrand disease
increased bleeding time and PTT
91
What is the defect in von Willebrand disease?
defect in vWF which normally protects Factor VIII (intrinsic pathway) --> increase PTT
defect in vWF which helps platelets form plug --> increase bleeding time
92
most common inherited bleeding disorder
von Willebrand disease
93
Lab used to diagnose von Willebrand disease
ristocetin cofactor assay - won't see platelet aggregation
94
Treatment for von Willebrand disease
desmopressin - causes vWF to be released
95
Labs seen with DIC
schistocytes, increase in D-dimer, decrease in fibrinogen,
| increase in BT, PT, PTT, decreased platelets
96
Causes of DIC
"STOP Making Thrombin" - Sepsis (gram -), Trauma, Obstetric complications (placental abruption, amniotic fluid embolism), Pancreatitis (acute), Malignancy, Transfusions
97
Clinical signs of a platelet disorder
microhemorrhages - petechiae, purpura, epistaxis, mucous membrane bleeding
increased bleeding time
98
Symptoms of TTP
"FAT RN" - Fever, Anemia (microangiopathic hemolytic), Thrombocytopenia, Renal Failure, Neuro symptoms
99
Defect in Thrombotic thrombocytopenic purpura
inhibition of deficiency in ADAMTS 13 - can't break down large vWF multimers --> they bind platelets --> platelets aggregate and thrombosis
-generally an acute disease
100
Defect in Immune Thrombocytopenia
anti-GpIIb/IIIa antibodies --> splenic macs consume platelet/ab complexes --> decreased platelets
- often follows viral illness
- see megakaryocytes on BM bx
- tx with steroids or IVIG
101
Defect in Glanzman thrombocytopenia
defect in GpIIb/IIIa --> can't form platelet-platelet aggregation
-normal platelet count, just increased BT
102
Defect in Bernard-Soulier syndrome
defective GpIb --> platelets can't bind vWF
| -normal platelet count, increased BT
103
Triad of HUS
thrombocytopenia, renal failure, microangiopathic hemolytic anemia
-milder form of TTP
104
What is a common illness that affects children before they have HUS?
E. coli O157:H7 infection causes diarrhea - produces shiga toxin
