4/2 - UW 39 Flashcards
Subacute sclerosing panencephalitis is a rare complication of what disease?
Measles
What is another name for galactosyl-beta-1,4-glucose?
Lactose
How does factor V Leiden cause thrombophilia?
Factor Va has resists protein C cleavage
What disorder causes DNA hypersensitivity to ionizing radiation?
Ataxia-telangiectasia (autosomal recessive)
What disorder causes DNA hypersensitivity to UV radiation?
Xeroderma pigmentosum
What disorder causes DNA hypersensitivity to cross linking agents?
Fanconi anemia
What disorder is characterized by generalized chromosomal instability?
Bloom syndrome
What disorder causes defects in DNA mismatch-repair?
Hereditary nonpolyposis colorectal cancer (HNPCC)
What changes do you see in Ataxia-telangiectasia?
Cerebellar atrophy (causing ataxia), oculocutaneous telangiectasia, severe immunodeficiency, cancer risk
What is the Schilling test?
- Oral radiolabeled vit B12
- IM injection unlabeled B12
Normal urinary B12 = normal absorption, thus any deficiency is due to dietary def.
Low urinary B12 = absorption issue
If B12 def is determined to be an absorption issue, how to differentiate between pernicious anemia and malabsorption?
Pernicious anemia: autoimmune destruction of parietal cells in atrophic gastritis, so giving B12 with IF would result in normal urinary B12.
Malabsorption: pancreatic insufficiency, bacterial overgrowth, short gut syndrome, so even B12 with IF would not give normal urinary excretion
With what drug should AIDS patients be prophylactically treated for PCP?
TMP-SMX (first line)
Dapsone (in case of allergy, but must check G6PD level!)
What are some common precipitating factors of G6PD deficiency?
- Infx
- Drugs (dapson, antimalarials, sulfonamide abx)
- DKA
- Fava beans
What is suggested by anemia, elevated LDH and indirect bilirubin, and a positive osmotic fragility test?
Hemolytic anemia with spherocytes
How does chronic hemolysis cause pigmented gallstones?
Increased bilirubin precipitate as calcium bilirubinate.
Folate deficiency requires supplement of what AA?
Thymidine!
What segment of the GI tract is affected by Hirschsprung disease?
Colon: migration of neural crest cells goes proximal to caudal, so rectum is always involved.
Where is airway resistance the highest?
In the medium sized bronchi
What are some common presentations of CMV in AIDS patients?
Retinitis
Esophageal ulcers
Colitis