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PedAM

Pediatric Disease Annotations & Medicines




Disease hypoadrenalism
Phenotype C0039730|thalassaemia
Sentences 2
PubMedID- 23634994 Because iron overload is thought to be the primary cause of adrenal insufficiency, td children with hbe/ss-thalassaemia are considered likely candidates for the development of adrenal insufficiency, while the adrenal function of ntd children is generally considered to be normal.
PubMedID- 25286743 However, the prevalence of adrenal insufficiency in children with hbe/β-thalassaemia was recently found to be 50% for transfusion-dependent patients and 53·5% for non-transfusion-dependent patients 77. it was also recently shown that vitamin d deficiency was significantly more common in children with non-transfusion-dependent hbe/β-thalassaemia than those with transfusion dependency (33·3% vs. 12·2%, p = 0·01) 78. a total of 23% of the optimal care study cohort had osteoporosis, a common complication in β-ti, secondary to bone marrow expansion and vitamin d deficiency 23,79. transfusion therapy, along with appropriate iron chelation, may be beneficial in paediatric patients to minimize endocrine problems and the development of osteoporosis later in life.

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