peds37 Flashcards
kasabach-erritt syndrome
enlarging hemangioma, microangiopathic hemolytic anemia, thrombocytopenia, and consumptive coagulopathy
glanzmann’s thomboasthenia
aut recess disorder characterized by decr ability of platelets to aggregate and form clot; def glycoprotein Iib/IIIa on platelet cell membrane
bernard-soulier syndrome
aut recessive; decr platelet adhesion as a result of absence of plateelt membrane glycoproteins; large unusualy platelets seen on blood smear
protein C
vit K dependent factor that is the most potent anticoag protein known
protein C def clinical features
hypercoagulability; homozygotes at birth get pupura fulminans; hets present later with DVT or stroke
treatment of protein C def
heparin, FFP, and warfarin; purified concentrates of protein C have been used
protein S def
hypercoagulability
antithrombin III def
hypercoagulability
factor V leiden def
hypercoagulability
kawasaki disease
autoimmune vasculitis in kids; most serious effect is on heart, where it can cause coronary artery aneurysms; more common in asians
mild neutropenia
1000-1500 cells
moderate neutropenia
500-1000 cells; infection involving skin and mucous membranes
severe neutropenia
less than 500 cells; severe infections like pneumonia, sepsis, meningtiis; gram neg bacteria are typical organisms
most common cause of neutropenia in childhood
infections that suppress bone marrow, marginate neutrophils, or exaust bone marrow reserves
chronic benign neutropenia of childhood
common cause of neutropenia in kids less than 4 yo; noncyclic neutropenia is the only abnormality
clinical features of benign neutropenia of childhood
kids at incr risk for mild infections
labs in benign neutropenia of childhood
low absolute neutrophil count, with slightly low total WBC, bone marrow shows immature neutrophil precursurs (development of mature neutrophils is arrested)
prognosis of benign neutropenia of childhood
most kids recover in months to a year
severe congenital agranulocytosis
aka Kostmann syndrome; aut recessive; frequent life-threatening pyogenic bacterial infections; ANC is VERY low
cyclic neutropenia
regular episodes of neutropenia with resultant infections; cycles last 21 days
chediak higashi syndrome
aut recess; oculocutaneous albinism, neutropenia, blond or brown hair w silver streaks; large blue-gray granules in neutrophils
cartilage-hair hypoplasia syndrome
aut reces; short staturs, immundef, fine hair, and neuropenia
schwachman-diamond syndrome
exocrine pancreatic insuff, short stature caused by metaphyseal chondrodysplasia; and neutropenia; FTT and recurrent infections
hypersplenism can cause neutropenia how?
increased destruction
autoimmune neutropenia
anti-neutrophil antibodies are produced in response to infection