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DIT/Boards > June 8th > Flashcards

June 8th Flashcards

1
Q

WAGR Complex

A

Wilms Tumor
Aniridia
GU malformations
Retardation (motor and mental)

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2
Q

Metabolic panel in young asthmatic

A

They’re on a Beta agonist which would shift K into cells

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3
Q

IF: Granular pattern of immune complex deposition LM: diffuse capillary thickening

A

Membranous glomerulonephritis
Diffuse Proliferative glomerulonephritis (Lupus nephritis)
Remember membranous = thickening of BM & diffuse proliferative!

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4
Q

IF: Granular pattern of immune complex deposition LM: hyper cellular glomeruli

A

Acute PSGN

Hypercellularity = inflammatory cells

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5
Q

LM: segmental sclerosis and hyalinosis

A

Focal Segmental Glomerulonephritis

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6
Q

IF: IgG, IgM, IgA and C3 deposition in the mesangium

A

Acute PSGN

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7
Q

Anticoagulant during pregnancy

A

Heparin/LMWH

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8
Q

Heparin antidote

A

Protamine sulfate

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9
Q

Warfarin antidote

A

Fresh frozen plasma

Vit K oral

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10
Q

Fibrinolytic agent (streptokinase,urokinase, alteplase) antidote

A

Aminocaproic Acid

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11
Q

Veteran describing war in detail with no emotion

A

Isolation

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12
Q

A child abuser was himself abused as a child

A

Identification

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13
Q

Underlies all other defense mechanisms

A

Repression

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14
Q

May lead to multiple personalities

A

Dissociation

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15
Q

Deficiency of Aldolase B

A

Fructose intolerance

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16
Q

Deficiency of cystathione synthase

A

Homocysteinuria

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17
Q

Galactose 1 phosphate uridyltransferase deficiency&raquo_space; intellectual disability, hepatosplenomegaly and cataracts

A

Classic Galactosemia

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18
Q

Deficient in tyrosine

A

Albinism

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19
Q

Protein responsible for biconcave shape and flexibility of RBCs

A

Spectrin

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20
Q

Anisocytosis

A

RBC of varying sizes

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21
Q

Poikilocytosis

A

RBC of varying shapes

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22
Q

Reticulocyte

A

Immature RBC

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23
Q

Acanthocyte

A

Spur cells
Liver disease & Abetalipoproteinemia
Irregular spiked

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24
Q

Basophilic stippling

A

Lead poisoning

Inhibition of RNA breakdown

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25
Degmacyte
Bite cell | G6PD deficiency
26
Echinocyte
Burr cell Uremia (renal failure) Regular spikes
27
Ringed sideroblasts
Excessive iron due to disorders of heme synthesis found in bone marrow
28
Schistocyte
Helmet cell Fragmented RBCs DIC TTP/HUS HELLP Mechanical hemolysis
29
Dacrocyte
Teardrop cell | Myelofibrosis and bone marrow infiltration
30
Target cells
``` "THAL" Thalassemia Hemoglobin C disease Asplenia Liver disease ```
31
Heinz bodies
Oxidized Hb fragments G6PD deficiency Many in one cell >> "Heinz 57 ketchup"
32
Howell-Jolly bodies
Basophilic nuclear remnant found in RBCs only 1 per cell Asplenic patients Sickle cell disease
33
How does mom/fetus transfer O2?
Fetal Hb (alpha2gamma2) has less affinity for 2,3-DPG which gives it a higher affinity for O2 allowing the Fetal Hb to extract O2 from the maternal Hb (alpha2beta2)
34
Rate limiting enzyme of heme synthesis
ALA-synthease
35
Acute Intermittent Porphyuria
Defect in porphobilinogen deaminase/uroporphyrinogen I synthase
36
Porphyria Cutaenous Tarda
Defect in URO decarboxylase
37
Sites of erythropoiesis in embryo/fetal?
``` "Young Livers Synthesize Blood" Yolk sac Liver Spleen Bone marrow ```
38
Causes of polycythemia
EPO producing tumors "Potentially Really High Hematocrit" (Pheo, RenalCC, Hepatic CC, Hemangioblastoma) Polycythemia vera Trisomy 21 Elevated altitude (chronic hypoxia)
39
Lead poisoning Sx
``` Encephalopathy Memory loss Renal failure Wrist and foot drop Lead lines in bones and gums Basophilic stippling and macrocytic anemia ```
40
Hexokinase vs. Glucokinase
``` Hexokinase find everywhere Insulin independent Low Km/ Low Vmax Glucokinase only in liver and B cells of pancreas Induced by insulin High Km/High Vmaz ```
41
Inhibitor of alcohol dehydrogenase
Fomepizole
42
Inhibitor of aldehyde dehydrogenase
Disulfram
43
Iron poisoning inhibits which enzymes in Heme synthesis?
ALA dehyrdataes | Ferrochelatase
44
Test to dx beta thalassemia minor
Hb electrophoresis | Increased HbA2
45
Iron deficiency anemia in pt >50
Rule out colon cancer
46
Which has neuro deficits B12/B9?
B12 has peripheral neuropathy | Folate does not
47
Megaloblastic anemia with increased methylmalonic acid
B12 deficiency
48
Megaloblastic anemia with no hyperammonemia
Orotic aciduria | Refractory to B12 and folate
49
Hypo cellular bone marrow with fatty infiltration
Aplastic anemia
50
Causes of aplastic anemia
Radiation Drugs (benzene, chloramphenicol, alkylating agents) Viral (parvo B19, HIV, EBV) Following acute hepatitis
51
Cause of anemia of chronic disease
Increased inflamm >> increased hepcidin >> binds ferroportin on intestinal mucosa and macrophages inhibiting iron transport
52
Labs for anemia of chronic disease
decreased serum iron decreased TIBC normal or increased ferritin normal transferrin saturation >18%
53
Hair on end skull appearance
Beta thalassemia Sickle cell (due to marrow proliferation)
54
Microcytic anemia reversible with B6
Sideroblastic anemia
55
Anemia in Mediterranean population
Beta thal
56
Anemia in African/Asian
Alpha thal
57
Beta thalassemia major increased type of Hb
HbF (ay)
58
Beta thalassemia minor increased type of Hb
HbA2 (alpha delta)
59
Thalassemia with target cells
Beta thalassemia
60
Difficulty swallowing Glossitis Microcytic anemia
Plummer Vinson Syndrome | Iron deficiency anemia
61
Alpha thalassemia type
Missing 1 allele = Asx Missing 2 allele = Alpha thal trait >> minimal anemia Missing 3 allele = HbH disease very little alpha, excess B forms B4 = HbH Missing 4 alleles = Hb Barts no alpha globin so forms y4, hydrops fetalis >> incompatible with life
62
Hb Barts
Alpha thal with 4 allele deletion | y4
63
HbH disease
Alpha thal with 3 allele deletion | B4
64
RLS of beta oxidation of fatty acids
Carnitine acyltransferase 1
65
Cofactors for pyruvate dehydrogenase | What other enzyme uses the same cofactors
``` "TLC For Nancy" TPP Lipoid acid CoA FAD NAD+ Also needed for alpha ketoglutarate ```
66
Result of glycolytic enzyme deficiency
Hemolytic anemia
67
Result of pyruvate dehydrogenase
Neuro deficits
68
Tx of sickle cell anemia
Hydroxyurea
69
Hams test +
Paroxysmal nocturnal hemoglobinuria | increases pH to increase complement and cause red cell lysis
70
+ osmotic fragility test
hereditary spherocytosis
71
Causes of sickle cell crisis
Low O2 Acidosis Dehydration
72
Sickle cell osteomyelitis
Salmonella
73
Sickle cell mutation
Glutamic acid (Glu) >> Valine (Val) at position 6 for the B chain
74
HbC mutation
Glutamic acid (Glu) >> Lysine (Lys) at position 6 for the B chain
75
Cold agglutinins Ab
IgM
76
Warm agglutinins Ab
IgG
77
Causes of microangiopathic hemolytic anemia
DIC TTP/HUS SLE Malignant HTN
78
Causes of macroangiopathic hemolytic anemia
Prosthetic heart valve | Aortic stenosis
79
Direct coombs test
Checking for Ab on the RBC with Ab which cause RBC agglutination
80
Indirect coombs test
Checking for Ab in the serum to normal RBC | Test blood prior to transfusion
81
Cause of cold agglutinins
EBV Mycoplasma CLL
82
Cause of warm agglutinins
HIV/EBV SLE CLL & NH Lymphoma Congenital immune abnormalities
83
Waterhouse Freidrichson caused by | What does it lead to
Caused by meningococcemia | Leads to adrenal insufficiency (after the adrenal hemorrhage)
84
Causes of hemolytic anemia in G6PD
``` "Spleen Purges Nasty Inclusions From Damaged Cells" Sulfonamides Primiquine Nitrofuratoin (UTI) INH (TB) Fava beans Dapsone (Leprosy) Chloroquine ```
85
Causes of hemolytic anemia in G6PD
``` "Spleen Purges Nasty Inclusions From Damaged Cells" Sulfonamides Primiquine Nitrofuratoin (UTI) INH (TB) Fava beans Dapsone (Leprosy) Chloroquine ```
86
Pentad of TTP
``` "Nasty Fever Torched His Kidneys" Neuro Sx Fever Thorombocytopenia Hemolysis/Anemia Kideny insufficiency ```
87
Triad of HUS
Thrombocytopenia Hemolysis Renal insufficiency
88
Triad of HUS
Thrombocytopenia Hemolysis Renal insufficiency
89
Causes of DIC
``` "STOP Making Thrombi" Sepsis (gram -) Trauma Obstetric complications (amniotic fluid emboli and abruptio placenta) acute Pancreatitis Malignancy Transfusion (Nephrotic syndrome has been excluded) ```
90
Labs in DIC and PT/PTT/BT/PC
PT/PTT/BT are all increased PC is decreased increased fibrin split products (D-dimer) Decreased fibrinogen***
91
Labs in DIC and PT/PTT/BT/PC
PT/PTT/BT are all increased PC is decreased increased fibrin split products (D-dimer) Decreased fibrinogen***
92
2 yr old girl with increase in abdominal girth, failure to thrive and skin/hair depigmentation
Kwashikor
93
Enzyme deficient in Essential Fructosuria
Fructokinase
94
Enzyme deficient in Classic Galactosemia
Galactose 1 phosphate uridytransferase
95
Thyroid cells with optically clear nuclei
Papillary cell cancer of the thyroid
96
Hemosiderinuria + Thrombosis
Paroxysmal nocturnal hemoglobinuria
97
Hemosiderinuria + Thrombosis
Paroxysmal nocturnal hemoglobinuria
98
Cancer most commonly associated with noninfectious fever
Hodgkin lymphoma
99
Large B cells with bilobed nuclei and prominent owl eye inclusions
Reed Sternberg cells of Hodgkin lymphoma
100
Most common lymphoma in US
Diffuse Large B Cell Lymphoma
101
Most common lymphoma in kids
Lymphoblastic lymphoma
102
Age of Hodgkin lymphoma
Bimodal distribution 20/65
103
Associated with long term celiac disease
Enteropathy/Intestinal T Cell Lymphoma
104
Associated with Sjogrens, Hashimoto's thyroiditis and H. pylori
MALToma (B cell)
105
Starry sky pattern
Burkitts
106
Follicular lymphoma mutation
14,18 BCL-2 on 18
107
Burkitt lymphoma mutation
8,14 | c-myc on 8
108
Mantle cell lymphoma mutation
11,14 | Cyclin D1 on 11

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