Table II.
Schedule of Measurements including Laboratory details
| TEST | AGES | FREQUENCY (if normal) |
|---|---|---|
| Genotype | All | Once |
| Transplant Evaluation / HLA typing | All | Once |
| CBC with differential, LFTs, Chem | All | Q 6 months, monthly if transfused |
| Hepatitis, HIV | All | Annual |
| αFP/liver ultrasound | Hepatitis B/C | Annual |
| Transfusion/Chelation history | All | Monthly |
| LIC | All | Annual |
| Echocardiogram & Cardiac MRI T2* | ≥ 10 years | Annual |
| Ferritin | All | Q 3 months |
| FSH, LH, testosterone, estradiol | ≥ 10 years | Annual |
| Menstrual history | ≥ 8 years | Annual |
| Reproductive health | ≥ 14 years | Annual |
| Tanner Stage | 10 - 20 years | Q 6 months |
| Anthropometrics (height, truncal height, weight, head circumference, growth velocity) | All | Annual, Q 3-6 months during puberty and chronic transfusion |
| TSH or fT4, PTH, Vitamin D (1,25 and 25-OH) | ≥ 6 years | Annual |
| Bone Mineral Density | ≥ 10 years | Annual |
| Fasting ascorbate | All | Annual |
| Fasting glucose | ≥ 10 years | Annual |
| Chelation History and Toxicity Monitoring | All | Annual |
Abbreviations: CBC: complete blood count; FSH: follicle-stimulating hormone; HLA: human leukocyte antigen; LFT: liver function tests; LH: luteinizing hormone; HIV: human immunodeficiency virus; PTH: parathyroid hormone; TSH: thyroid stimulating hormone; fT4: free thyroxine