Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
44 Cards in this Set
- Front
- Back
|
pediatr inheritance and biochem
|
AR, glutamic acid is replaced with valine
|
|
|
pediatr sickling during the stress
|
infect, fever,hypoxia, acidosis
microvascular occlusoin |
|
|
pediatr when kid is symptomatic
|
2-4 month old, when hemog F is replaced with HEm A
|
|
|
pediatr when will be functional asplenia
|
by 5-6 years od age
|
|
|
pediatr clinical
|
acute dactilitis ( necrosis of small bones is most common initial presentation
|
|
|
pediatr most common initial presentation
|
acute dactilitis ( necrosis of small bones is most common initial presentation
|
|
|
pediatr second most common presentation
|
acute splenic sequestration
|
|
|
pediatr
how the symptoms progression |
progress more centrally with age
chest- acute chest syndrme head- stroke back-vertebral infarction abdomen penis- priapism |
|
|
pediatr mcc cause of death
|
acute chest syndrome and sepsis
|
|
|
pediatr
ds best initial test |
periferal smear showing sickle cells
|
|
|
pediatr
best confirmatory test |
Hb electrophoresis
|
|
|
pediatr
treatment |
hydroxyurea-- maintsain, if fail- erythropoietin
|
|
|
hydroxyurea-- the main tx but if fail what to give-
|
erythropoietin
|
|
|
immnisat
|
regular immun + pneumococcal vaccination
add penicillin for profil at age 2moths to 5 years folate supplement |
|
|
recom screening tests
|
monitor cerebral blood flow with transcranial doppler annually to access the stroke risk
annual retinal examination |
|
|
what is most likely diagnosis
|
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 |
|
|
common manifestation of sickel cell di
|
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 |
|
|
why papillary necrosis
|
due to chronic kidney damage
|
|
|
best initial test
|
per smear
|
|
|
most accurate test
|
hb electrophores
|
|
|
sickle cell trait
|
doesn't give sickle cells
|
|
|
what is on the smear
|
howell holey- precipitated remnants of nuclear material seen inside the cells in P without spleen
|
|
|
tx
|
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 |
|
|
when to use exange transfusion
|
if there is severe vaso occlusove crisis ---
presented with acute chest synd priapism stroke visual disturban from retinal infarc |
|
|
aplastic crisis.... if P admitted with acute pain crisis what to order
|
reticulocytes .. will be high level due to chronic compens hemolysis
|
|
|
what could cause acute apl crisis
|
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 |
|
|
what is most accurate test for parvovirus
what is the best initial therapy |
PCR for DNK
it is more accurate than IgM level// immunoglob |
|
|
sickle cell trait- genetic
|
heterozyg for sickel gene
manifestation will be |
|
|
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! |
|
|
trait - not anemic
|
if it is- search fr other reason
|
|
|
symptoms in SSa
|
chronic compensated hemol anemia
hb= 6.5-10 retic- 10-25%, mild jandice, increased indirect bilirubin |
|
|
why megaloblastic crisis in SSA
|
folate def
|
|
|
screening- sickle cell preparation-
|
with sodium metabisulphate
|
|
|
tx steps
|
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 |
|
|
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. |
|
|
Possible findings of the physical include the following:
|
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 |
|
|
when fever is present for a question of "Most urgent next step?"
|
for a question of "Most urgent next step?" answer antibiotics
when fever is present. This is more important than waiting for results of testing. |
|
|
When do I answer "exchange transfusion " in sickle cell disease
|
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 |
|
|
HEMOGl.OBIN SICKLE CELL (SC) DISEASE
|
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 |
|
|
tx of hemoglobin SC disease.
|
There is no specific th erapy for hemoglobin SC disease.
|
|
|
what could be in hemoglobin sickle cell disease
|
more eye complcations
aseptic necrosis of femoral head splenic sequestr crisis seen in kids who still have spleen uncontrolled hemorrhage in to the spleen |
|
|
when to order hydroxyurea
|
after 3 crisis in year
|
|
|
Sickle cell disease develops in
|
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. |
|
|
sickle trait .. what will be increased
|
urinary tract infect
|
