avascualr necrosis of femoral head, osteomyelitis=salmonella
Derm: SCA findings
skin ulcers of low extr=teens typically
Immune: SCA findings
pneumococcus, haemophilus influenzae, and encapsulated organisms are ususally responsible. Bacterial sepsis and meningitis are the most life threatening.
Crises: findings w/sca
acute chest syndrome
acute splenic sequestration
painful, transient, ischemic tissue damage which may be triggered by dehydration, infection, stress
Dactylitis (hand-foot syndrome) �
often 1st sign, fever may be present, Abdominal and musculoskeletal pain are also common=occurs in 50% in age of children b4=3yo
Acute chest syndrome, CVA
Acute Splenic Sequestration �
massive enlargement of spleen due to pooling of rbc�s rapid drop hgb/hct=shock or death. Seen in infants & young children following febrile illness.
sudden shutdown of bone marrow & drop in hct. May be life threating & follows parvovirus b19 infection.
Labs: normocytic or macrocytic anemia, increased platelets, reticulocytes. Other findings: sickle cells, Howell-Jolly bodies, target cells
Dx: hemoglobin electrophoresis
Treatment a. Health Maintenance: nutrition, baseline lab studies, immunizations, education & referreals=folic acid 1mgperday, pneumonocal, infleuza vaccines; delayed puberty, short stature & recognize disease sx=fever & pallor. Opthalomogy for retinopathy, echo for tricuspid regurg; nephrology based upon renal fx tests & pulmonalgy for tests
Infections: prevention & early recognition (prophylactic abx)=3mo�s to 2yo they should receive pencillin=125mg ; 2-5 should be 250mg.
Pain: analgesics, hydration, oxygen, abx, transfusion? Problem at capillary level
5. Skeletal � lead lines on radiographs of long bones
1. Screening � all children below 6 yo should be screened at least one time. Screening should begin at 9-12 mos and be more regimented if child is high risk. Capillary (finger) vs. Venous blood (might have to stick twice). Below 9 for cap=nothing above 9 confirm w/venous