Monitoring

Iron load:

  • Consistent rising serum ferritin levels or intermittent transfusions given at a volume likely to require chelation (e.g., 6 to 8 transfusions of 15 mL/kg each) should trigger R2 (1/T2) or R2* (1/T2*) MRI assessment of LIC.[37][38] Caution is advised in interpreting serum ferritin level, as it is not a reliable indicator of total body iron burden or as a parameter for the monitoring of chelator activity[40]

  • Cardiac MRI T2* annually.

Cardiovascular:

  • MRI for assessment of function (echocardiography with tissue Doppler if MRI not possible) annually

  • Holter monitoring annually.

Hepatobiliary:

  • Bilirubin levels and AST/ALT monitoring at each transfusion visit if on deferasirox or if active hepatitis B virus or hepatitis C virus infection; less frequently if on deferoxamine

  • Hepatitis B virus and hepatitis C virus serology annually.

Renal:

  • BUN and serum creatinine at each transfusion visit if on deferoxamine or deferasirox

  • Urinalysis for proteinuria, a toxic effect of deferasirox.

Endocrine:

  • Thyroid and parathyroid function annually

  • Bone density annually

  • Glucose tolerance annually

  • hGH, testicular, and ovarian function based on age and clinical indication.

Other:

  • HIV serology annually

  • Vision and hearing annually if on deferoxamine

  • Pulmonary function every 2 years.

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