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Step 1 Rx > Deck 4 > Flashcards

Deck 4 Flashcards

1
Q

What factors does PT time measure

A

VII, X, V (extrinsic)

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2
Q

What factors does PTT measure

A

XIII, IX, XI, XII and X, V, prothrombin, fibrinogen

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3
Q

What drug do you not take with digoxin?

A

Quinidine- decreases digoxin clearance and displaces it from tissue binding sites

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4
Q

What drug increases fetal hemoglobin to prevent sickle cell attackes?

A

Hydroxyurea

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5
Q

Features of DiGeorge?

A

CATCH 22- Cleft palate, abnormal facies, thymic dysplasia, cardiac defects, hypocalcemia (loss 3rd/4th pharyngeal pouch and thymus/PTH gland)

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6
Q

What embronic structure does the thymus come from?

A

3rd pharyngeal pouch

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7
Q

Why do you use desmopressin with vWF dz?

A

releases vWF stored in endothelium and incr. factor VIII activity

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8
Q

Child with viral infection that has low platelets?

A

ITP - most common cause of thrombocytopenia in children

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9
Q

Thrombotic thrombocytopenic purpura is caused by Abs against what protease?

A

ADAMTS13 (vWF metalloprotease that cleaves vWF)

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10
Q

Symptoms of TTP?

A

FAT RN: Fever, microangiopathic hemolytic Anemia (schistocytes), Thrombocytopenia, Renal problems, Neurologic problems

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11
Q

When would you see an IgM cold hemolytic anemia?

A

After EBV infection, HIV, Mycoplasma pneumonia, leukemia - HELM

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12
Q

When you see target cells, HALT..?

A

Hemoglobin C, Asplenia, liver disease, thalassemia

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13
Q

von Hippel Lindau dz characteristics?

A

Autosomal dominant Chr. 3- hemangioblastomas (retina, cerebellum, medulla), ataxia, polycythemia, Incr. risk of bilateral renal cell carcinoma.

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14
Q

What tumors does Turcot syndrome cause?

A

Colon- APC gene, Brain - glioblastoma multiforme

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15
Q

Gardner syndrome = ?

A

Variant of FAP with colorectal polyposis, osteomas, bone and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium, dental abnormalities

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16
Q

Symptoms of tuberous sclerosis?

A

Autosomal dominant. Intellectual disability, seizures, CNS tumors, angiomyolipomas of kidneys, leptomeningeal tumors, skin lesions such as ash-leaf spots and shagreen patches. Infantile spasms common

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17
Q

Fanconi anemia disorder

A

Autosomal recessive disorder resulting in marrow hypofunction and eventually pancytopenia. Hypoplastic bone abnormalities. Hallmark = incr. chromosomal breaking in response to DNA-damaging agents. More sensitive to cyclophosphamide and chemo agents. Incr. risk of AML and myelodyplastic syndrome.

18
Q

What does warfarin block?

A

Inhibits epoxide reductase –> blocks gamma-carboxylation of vitamin K dependent clotting factors

19
Q

What tumors are psammoma bodies found in?

A

PSMMa vody = papillary carcinoma of thyroid, Serous papillary cystadenocarcinoma of ovary, Meningioma, Mesothelioma

20
Q

Tumor in brain- whorled pattern, seizure, headache, visual field defect

A

Meningioma

21
Q

What are Bence Jones proteins from multiple myeloma?

A

Antibody light chains produced by plasma cells. Result in renal insufficiency by forming large eosinophilic casts

22
Q

What is the mutation in Li fraumeni and what cancers are caused?

A

Pp53 tumor suppressor gene. Osteosarcomas, soft tissue sarcomas, breast cancer, adrenocortical tumors, leukemias. Autosomal dominant

23
Q

Howell Jolly bodies seen in what?

A

Sickle cell

24
Q

Paraneoplastic syndromes from small cell lung carcinoma?

A

SIADH - hyponatremia, Cushing - ACTH, Lambert eaton myasthenic syndrome from host antibody response that cross reacts with Ca channels at neuromuscular junction

25
Q

5 P’s for acute intermittent porphyria?

A

Painful abdomen, port wine colored urine, polyneuropathy, psychological distrubance, precipitated by drugs

26
Q

AFP marker with what two cancers?

A

Yolk sac germ cell carcinoma, hepatocellular carcinoma (hep C)

27
Q

Cancer associated with NF-2?

A

Vestibular schwannoma. Also blurry vision and meningiomas

28
Q

What condition with kid with large flank mass and abnormal enlargement of one side of body?

A

Wilm’s tumor. Deletion of tumor suppressor gene- WT1 on chromosone 11. Wilms tumor arises from neoplastic embryonic renal cells of metanephros

29
Q

MEN 1?

A

Pituitary (GnRH, prolactinoma), pancreatic (ZE, VIPomas, insulinomas), PTH

30
Q

MEN 2A?

A

2A = pheo, medullary of thyroid, PTH

31
Q

MEN 2B?

A

2B = pheo, medullary of thyroid, marfanoid habitus

32
Q

Most common cause of osteomyelitis? In sickle cell patients?

A

1) Staph aereus 2) Salmonella

33
Q

What things is intereferon alpha used for?

A

leukocytic effects for kaposi, Hep B, Hep C, leukemias, malignant melanoma

34
Q

How does factor V Leiden lead to a hypercoaguable state?

A

Normally activated protein C cleaves factor five terminating clotting cascade, but factor Va is resistant to cleavage

35
Q

Difference between vWF and hemophilia lab values?

A

vWF has increased BLEED TIME and PTT. Hemo has just increased PTT and normal BT

36
Q

What is Zollinger Ellison?

A

Gastrinoma- gastrin secreting tumor of pancreas. Check for multiple duodenal ulcers

37
Q

Trousseau syndrome?

A

pancreatic adenocarcinoma with migratory thrombophlebitis

38
Q

Ewing sarcoma

A

in boys <15 years old. Aggressive malingnant tumor of bone, commonly in diaphysis. Lamellated “onion skin” periosteal reactions. Uniform sheets of anaplastic small blue cells

39
Q

How does diarrhea affect metabolic labs?

A

Bicarb lost in GI is exchanged for Cl- leading to hyperchloric non anion gap metabolic acidosis.

40
Q

Why do statins cause renal failure?

A

Rhabdomyolysis. Myoglobin and heme damage. Heme precipitates and induces ATN. BUN:Cr <15:1.

Step 1 Rx (10 decks)

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