Deck 4 Flashcards
What factors does PT time measure
VII, X, V (extrinsic)
What factors does PTT measure
XIII, IX, XI, XII and X, V, prothrombin, fibrinogen
What drug do you not take with digoxin?
Quinidine- decreases digoxin clearance and displaces it from tissue binding sites
What drug increases fetal hemoglobin to prevent sickle cell attackes?
Hydroxyurea
Features of DiGeorge?
CATCH 22- Cleft palate, abnormal facies, thymic dysplasia, cardiac defects, hypocalcemia (loss 3rd/4th pharyngeal pouch and thymus/PTH gland)
What embronic structure does the thymus come from?
3rd pharyngeal pouch
Why do you use desmopressin with vWF dz?
releases vWF stored in endothelium and incr. factor VIII activity
Child with viral infection that has low platelets?
ITP - most common cause of thrombocytopenia in children
Thrombotic thrombocytopenic purpura is caused by Abs against what protease?
ADAMTS13 (vWF metalloprotease that cleaves vWF)
Symptoms of TTP?
FAT RN: Fever, microangiopathic hemolytic Anemia (schistocytes), Thrombocytopenia, Renal problems, Neurologic problems
When would you see an IgM cold hemolytic anemia?
After EBV infection, HIV, Mycoplasma pneumonia, leukemia - HELM
When you see target cells, HALT..?
Hemoglobin C, Asplenia, liver disease, thalassemia
von Hippel Lindau dz characteristics?
Autosomal dominant Chr. 3- hemangioblastomas (retina, cerebellum, medulla), ataxia, polycythemia, Incr. risk of bilateral renal cell carcinoma.
What tumors does Turcot syndrome cause?
Colon- APC gene, Brain - glioblastoma multiforme
Gardner syndrome = ?
Variant of FAP with colorectal polyposis, osteomas, bone and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium, dental abnormalities
Symptoms of tuberous sclerosis?
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
Fanconi anemia disorder
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.
What does warfarin block?
Inhibits epoxide reductase –> blocks gamma-carboxylation of vitamin K dependent clotting factors
What tumors are psammoma bodies found in?
PSMMa vody = papillary carcinoma of thyroid, Serous papillary cystadenocarcinoma of ovary, Meningioma, Mesothelioma
Tumor in brain- whorled pattern, seizure, headache, visual field defect
Meningioma
What are Bence Jones proteins from multiple myeloma?
Antibody light chains produced by plasma cells. Result in renal insufficiency by forming large eosinophilic casts
What is the mutation in Li fraumeni and what cancers are caused?
Pp53 tumor suppressor gene. Osteosarcomas, soft tissue sarcomas, breast cancer, adrenocortical tumors, leukemias. Autosomal dominant
Howell Jolly bodies seen in what?
Sickle cell
Paraneoplastic syndromes from small cell lung carcinoma?
SIADH - hyponatremia, Cushing - ACTH, Lambert eaton myasthenic syndrome from host antibody response that cross reacts with Ca channels at neuromuscular junction
5 P’s for acute intermittent porphyria?
Painful abdomen, port wine colored urine, polyneuropathy, psychological distrubance, precipitated by drugs
AFP marker with what two cancers?
Yolk sac germ cell carcinoma, hepatocellular carcinoma (hep C)
Cancer associated with NF-2?
Vestibular schwannoma. Also blurry vision and meningiomas
What condition with kid with large flank mass and abnormal enlargement of one side of body?
Wilm’s tumor. Deletion of tumor suppressor gene- WT1 on chromosone 11. Wilms tumor arises from neoplastic embryonic renal cells of metanephros
MEN 1?
Pituitary (GnRH, prolactinoma), pancreatic (ZE, VIPomas, insulinomas), PTH
MEN 2A?
2A = pheo, medullary of thyroid, PTH
MEN 2B?
2B = pheo, medullary of thyroid, marfanoid habitus
Most common cause of osteomyelitis? In sickle cell patients?
1) Staph aereus 2) Salmonella
What things is intereferon alpha used for?
leukocytic effects for kaposi, Hep B, Hep C, leukemias, malignant melanoma
How does factor V Leiden lead to a hypercoaguable state?
Normally activated protein C cleaves factor five terminating clotting cascade, but factor Va is resistant to cleavage
Difference between vWF and hemophilia lab values?
vWF has increased BLEED TIME and PTT. Hemo has just increased PTT and normal BT
What is Zollinger Ellison?
Gastrinoma- gastrin secreting tumor of pancreas. Check for multiple duodenal ulcers
Trousseau syndrome?
pancreatic adenocarcinoma with migratory thrombophlebitis
Ewing sarcoma
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
How does diarrhea affect metabolic labs?
Bicarb lost in GI is exchanged for Cl- leading to hyperchloric non anion gap metabolic acidosis.
Why do statins cause renal failure?
Rhabdomyolysis. Myoglobin and heme damage. Heme precipitates and induces ATN. BUN:Cr <15:1.
