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44 Cards in this Set
- Front
- Back
pediatr inheritance and biochem
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AR, glutamic acid is replaced with valine
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pediatr sickling during the stress
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infect, fever,hypoxia, acidosis
microvascular occlusoin |
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pediatr when kid is symptomatic
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2-4 month old, when hemog F is replaced with HEm A
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pediatr when will be functional asplenia
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by 5-6 years od age
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pediatr clinical
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acute dactilitis ( necrosis of small bones is most common initial presentation
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pediatr most common initial presentation
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acute dactilitis ( necrosis of small bones is most common initial presentation
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pediatr second most common presentation
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acute splenic sequestration
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pediatr
how the symptoms progression |
progress more centrally with age
chest- acute chest syndrme head- stroke back-vertebral infarction abdomen penis- priapism |
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pediatr mcc cause of death
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acute chest syndrome and sepsis
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pediatr
ds best initial test |
periferal smear showing sickle cells
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pediatr
best confirmatory test |
Hb electrophoresis
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pediatr
treatment |
hydroxyurea-- maintsain, if fail- erythropoietin
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hydroxyurea-- the main tx but if fail what to give-
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erythropoietin
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immnisat
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regular immun + pneumococcal vaccination
add penicillin for profil at age 2moths to 5 years folate supplement |
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recom screening tests
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monitor cerebral blood flow with transcranial doppler annually to access the stroke risk
annual retinal examination |
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what is most likely diagnosis
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look for black with sudden severe pain in the chest, back, tights + fever
it is rare in adult to present with an acute crisis without clear history of sickle cell disease |
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common manifestation of sickel cell di
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bilirubin gallstones from const elevated bilirubin level
increased infection from auto splenoectome encapsulated org!! osteomyelitis from salmon retinopathy stroke enlarged heard with hyper dynamic features and a systolic murmur lower extremity skin ulcer avascular necrosis of the femoral head |
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why papillary necrosis
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due to chronic kidney damage
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best initial test
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per smear
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most accurate test
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hb electrophores
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sickle cell trait
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doesn't give sickle cells
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what is on the smear
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howell holey- precipitated remnants of nuclear material seen inside the cells in P without spleen
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tx
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1 start with O2 , hydra. analg
2if fever or wite cell count higher that usual-- give Antibiot---cefriaxone levofloaxacin moxifloaxacin folic acid hydroxeurea prevent cells from cycling by increasing hemog F |
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when to use exange transfusion
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if there is severe vaso occlusove crisis ---
presented with acute chest synd priapism stroke visual disturban from retinal infarc |
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aplastic crisis.... if P admitted with acute pain crisis what to order
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reticulocytes .. will be high level due to chronic compens hemolysis
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what could cause acute apl crisis
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parvocirus 19 freezes the growth of marrow
nothing will be on smear first clue is sudden drop in reticulocytes or it could be in folate def |
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what is most accurate test for parvovirus
what is the best initial therapy |
PCR for DNK
it is more accurate than IgM level// immunoglob |
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sickle cell trait- genetic
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heterozyg for sickel gene
manifestation will be |
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sickle cell trait-manifestation will be
tx? |
manifestation will be only --defect in the ability to concentrate the urine or isosthenuria
sometime may be hematuria but normal CBC and smear no tx! |
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trait - not anemic
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if it is- search fr other reason
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symptoms in SSa
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chronic compensated hemol anemia
hb= 6.5-10 retic- 10-25%, mild jandice, increased indirect bilirubin |
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why megaloblastic crisis in SSA
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folate def
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screening- sickle cell preparation-
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with sodium metabisulphate
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tx steps
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1- genetic concel
prenatal ds with fetal DNa analysis 2in childhood- -prof Pen VC - immuniza is critical+ hem = pneumoc - consider chronic transfusion 3 vasooclusive crisis- analg tx infec oral and iv fluid oxygen if hypoxic considet hydroxyurea or hyper transfusion if frequent 3aplastic crisis-- check reticul( should be elevated in SSD) 5 splenic sequestration: Tc as any signif bleed last resort- resect transfuse |
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The most likely hemolysis question to be asked will concern sickle cell anemia
The question will describe |
The most likely hemolysis question to be asked will concern sickle cell anemia.The
question will describe pain in the chest, hack, and thighs that is very severe. |
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Possible findings of the physical include the following:
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Possible findings of the physical include the following:
• HEENT: Retinal infarction • CV: Plow murmur from anemia • Abdomen: Splenomegaly in child ren: absence of spleen in adults Chest: Rales or consolidation from infection or infarction Extremities: Skin ulcers (unclear etiology in sickle cell) and aseptic necrosis of hip Neurological: Stroke. current or previo |
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when fever is present for a question of "Most urgent next step?"
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for a question of "Most urgent next step?" answer antibiotics
when fever is present. This is more important than waiting for results of testing. |
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When do I answer "exchange transfusion " in sickle cell disease
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When do I answer "exchange transfusion " in sickle cell disease
"when • E ye~: Visual disturbance from retinal infarction • lung : Pulmonary infarction reading to pleuritic pain and abnormal X-ray • Penis: Priapism from infarction of prostatic plexus of veins • Brain : Stroke |
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HEMOGl.OBIN SICKLE CELL (SC) DISEASE
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HEMOGl.OBIN SICKLE CELL (SC) DISEASE
HEMOGl.OBIN SICKLE CELL (SC) DISEASE This condition is like a mild version of sickle cell disease with fewer crises. Visual distu rbance is frequent. Painful crises do not occur. Renal problems are the only Significant manifestation. including the following: Hematuria • Isosthenuria (inability to concentr ate or dilute the urine) • Urinar y tra ct infections |
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tx of hemoglobin SC disease.
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There is no specific th erapy for hemoglobin SC disease.
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what could be in hemoglobin sickle cell disease
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more eye complcations
aseptic necrosis of femoral head splenic sequestr crisis seen in kids who still have spleen uncontrolled hemorrhage in to the spleen |
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when to order hydroxyurea
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after 3 crisis in year
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Sickle cell disease develops in
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Sickle cell disease develops in persons who are homozygous for the sickle gene (HbSS), in
whom 70% to 98% of hemoglobin is of the S type. About 0.2% of African Americans have sickle cell anemia. Risk factors that predispose to painful crises include a hemoglobin level greater than 8.5 g/dl, pregnancy, cold weather, and a high reticulocyte count. |
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sickle trait .. what will be increased
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urinary tract infect
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