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11 Cards in this Set
- Front
- Back
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Osteomyelitis
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Bacteria thrive in sites of previous infarction
Classic organism is Salmonella -(Most common organism in patients without sickle cell disease: Staph aureus) Will have: local tenderness, warmth, swelling, fever, elevated WCC, left shift, positive blood cultures, positive aspiration cultures |
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Osteonecrosis
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Thrombosis of end-arterial vessels
Can lead to joint destruction -Femoral head -Humoral head Adults can require surgery Pain from joint, bone destruction |
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Fat embolization syndrome
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Bone marrow infarcts and necroses
Fat from the bone marrow gets released into the bloodstream Filtered by the lungs Acts like infarction Diagnose on bronch with fat-laden macrophages Treat supportively |
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Pulmonary hypertension
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Defined as pulmonary artery pressure > 25 mm Hg
Multifactorial: -Chronic desaturation -Lung damage from recurrent acute chest syndrome -High pulmonary blood flow from anemia Diagnose on right heart cath Treat with medical management: vasodilators, Ca channel blockers, oxygen |
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Strokes
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Increased risk for thrombotic stroke during childhood and adulthood
-Usually in large intracranial arteries (around Circle of Willis) -Children have higher cerebral blood flow there -Sludging and occlusion by sickle cells occurs -Flow-related hemodynamic injury to endothelial cells of intracranial arteries, causing sickle cells to stick even more -Moyamoya vessels develop -25% of children have silent ischemic lesions seen on MRI -24% of patients had a clinically overt stroke by age 45 -Leading cause of death in children and adults -Associated with neurocognitive deficits even with silent lesions Hemorrhagic strokes are also a risk, primarily in patients 20-29 years of age |
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Stroke screening and treatment
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Screen:
-Ages 2-16 years old -Transcranial Doppler ultrasound Transcranial Doppler: -The smaller the diameter of the artery, the greater the flow -Focal areas of increased flow: stricture Treatment: -Chronic transfusions |
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Non-proliferative eye
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Due to local vaso-occlusive events
Turns normal smooth vessels into fragmented vessels Iris atrophy Retinal hemorrhages Retinal pigment changes Can be seen on dilated ophthalmoscopic exam Rarely affect vision |
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Proliferative eye
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Retinal arterial occlusions: ischemia, stimulates angiogenesis
Results in growth of abnormal “vascular fronds” -Increases risk for vitreous hemorrhage and retinal detachment due to mechanical traction on retina -“sea fan” changes Even children need routine ophthalmologic screening exams Treatment: -May include different modalities, including laser photocoagulation to involute neovascular lesions |
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Hydroxyurea
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Ribonucleotide reductase inhibitor
-Inhibits ribonucleotide diphosphates to deoxyribonucleotide diphosphates Non-specific inhibitor of DNA synthesis -Decreases bone marrow proliferation Hydroxyurea causes erythroid precursors to commit to Hb-F production (exact mechanism unknown) Hb-F decreases Hb S concentrations and inhibits polymerization of Hb S Observation was made that adults with Hb-SS and high Hb-F had less complications, and infants are protected Side effects: -myelosuppression -nausea and vomiting -teratogenicity |
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Children prophylaxis
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PCN VK 125 mg po bid < 3 years old
PCN VK 250 mg po bid 3-5 years old No data for improvement > 5 years old If PCN allergic, -Erythromycin 20 mg/kg po bid Will prevent ~80% of life-threatening episodes of S. pneumo sepsis in children -Decreasing morbidity and mortality This is why we screen for the disease now |
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Pregnancy
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Pregnancy is NOT contraindicated
30% of mothers will have an acute drop in their Hb by 30% of baseline -Can be associated with an increase in symptoms Higher rate of spontaneous abortion Babies have lower gestational age and lower birth rates Preeclampsia (higher risk): -HTN (Blood pressure > 140/90) -Proteinuria (>300 mg /2 hrs) -Edema -Eclampsia (seizures) -Cause for increased risk unknown -Monitor blood pressure closely -Treat supportively; unclear if transfusions will help |
