Sickle-cell Anaemia

Sickle-cell Anaemia

Sickle-cell Anaemia

Outline .

  • Course objectives .
  • Definition .
  • Pathophysiology
  • Epidemiology .
  • Pathology .
  • Clinical presentation.

Definition

Sickle Cell Disease:

  • A genetic hemoglobinopathy
  • Results in deformation, increased rigidity, and destruction of RBCs
  • Chronic multiorgan system disease
  • Severe morbidity and early mortality

Epidemiology

Worldwide:

  • 200,000 children born yearly in Africa with Sickle Cell Disease
  • Areas of Prevalence
  • Seen commonly irn sub-Saharan Africa but also occur in the Mediterranean, India, and the Arabian Peninsula
  • Corresponds to the distribution of malaria
  • Highest rates in Ghana, Nigeria, Uganda In Uganda,
  • Highest rates in Bundibugyo, in the west.

Pathophysiology

Sickling Mechanism De-oxygenation of Hgb S results in polymers which precipitate in RBCs as long, rigid fibers resulting into RBCs taking on a sickle shape.

Chronic organ damage

  • • Gall bladder: Cholelithiasis due to bilirubin gallstones
  • • Lungs: Pulmonary venous hypertension
  • • Eyes: Retinopathy
  • • Kidney: Renal failure
  • • Spleen: Autosplenectomy
  • • Extremities: Chronic leg ulcerations
  • • Heart: Myocardial infarction and cardiomyopathy

Maintenance of health

  • • Prevent infection
  • — Antimalarial prophylaxis, ITN
  • — Daily oral Penicillin V, monthly parenteral benzathine penicillin
  • — Vaccinate against S. pnuemoniae and H. influenza
  • • Treat pain
  • — Provide paracetamol for home use
  • • Supplement nutrition
  • — Daily folate and zinc supplementation
  • • Prevent painful crises — Hydroxyurea reduces frequency of painful crises and Improves quality of life.
  • • Family and patient education
  • • Join sickle-cell societies
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