BACKGROUND: Beta-thalassaemia major is a common medical problem worldwide. There is little data dealing with the nature and prevalence of different endocrine disorders in this disease in the Sultanate of Oman. OBJECTIVES: To establish the prevalence and times of occurrence of endocrine disorders in patients with beta-thalassaemia major. METHODS: This cross-sectional study was conducted during Jan-Jul 2008 and dealt with 30 Omani patients with transfusion-dependent homozygous beta-thalassaemia major who were consulting Thalassaemia Clinic, Royal Hospital. They included 15 males and 15 females, aged 16 to 32 years with median of 21 years and mean ± SD of 21.23 ± 3.42 years. The medical records of these patients were reviewed and their endocrine functions were assessed. This assessment included pituitary and gonadal function, thyroid function, bone profile (including Parathyroid Hormone), morning cortisol and fasting glucose. These profiles were reviewed to exclude hypogonadism, hypothyroidism, hypoparathyroidism, hypoadrenalism or diabetes mellitus. RESULTS: Hypogonadism was reported in 22 (73.3%) patients (13 female, 9 male). Low levels of Follicle-Stimulating Hormone (FSH) and low Luteinizing Hormone (LH) with low estradiol (in females) or testosterone (in males) was noted in 15 (50.0%) patients (7 female, 8 male). Normal (but inappropriately low) levels of FSH and LH with low estradiol (in females) or testosterone (in males) was noted in 7 (23.3%) patients (6 female, 1 male). Primary hypothyroidism was present in only 1 (3.3%) patient (female) who Hypoparathyroidism was found in 3 (10.0%) patients (2 female, 1 male). Diabetes mellitus with high fasting glucose was noted in 8 (26.7%) patients (2 female, 6 male). Morning cortisol levels for all patients were within the reference range with no suspicion of hypoadrenal cortical function. Eight (26.7%) patients had no endocrine disorder, 12 (40.0%) patients had one disorder, 8 (26.7%) patients had 2 disorders, and 2 (6.7%) patients had 3 endocrine disorders. There was no significant difference (p>0.050) in mean serum ferritin in thalassaemics with or without endocrinopathy, regardless of the number of endocrinopathy. CONCLUSION: There is high prevalence of endocrine disorders among Omani beta-thalassaemic adult patients. This signifies the importance of awareness for their development and monitoring for early detection and replacement therapy. No relationship between serum ferritin and development of endocrinopathy was noted.
BACKGROUND:Beta-thalassaemia major is a common medical problem worldwide. There is little data dealing with the nature and prevalence of different endocrine disorders in this disease in the Sultanate of Oman. OBJECTIVES: To establish the prevalence and times of occurrence of endocrine disorders in patients with beta-thalassaemia major. METHODS: This cross-sectional study was conducted during Jan-Jul 2008 and dealt with 30 Omani patients with transfusion-dependent homozygous beta-thalassaemia major who were consulting Thalassaemia Clinic, Royal Hospital. They included 15 males and 15 females, aged 16 to 32 years with median of 21 years and mean ± SD of 21.23 ± 3.42 years. The medical records of these patients were reviewed and their endocrine functions were assessed. This assessment included pituitary and gonadal function, thyroid function, bone profile (including Parathyroid Hormone), morning cortisol and fasting glucose. These profiles were reviewed to exclude hypogonadism, hypothyroidism, hypoparathyroidism, hypoadrenalism or diabetes mellitus. RESULTS:Hypogonadism was reported in 22 (73.3%) patients (13 female, 9 male). Low levels of Follicle-Stimulating Hormone (FSH) and low Luteinizing Hormone (LH) with low estradiol (in females) or testosterone (in males) was noted in 15 (50.0%) patients (7 female, 8 male). Normal (but inappropriately low) levels of FSH and LH with low estradiol (in females) or testosterone (in males) was noted in 7 (23.3%) patients (6 female, 1 male). Primary hypothyroidism was present in only 1 (3.3%) patient (female) who Hypoparathyroidism was found in 3 (10.0%) patients (2 female, 1 male). Diabetes mellitus with high fasting glucose was noted in 8 (26.7%) patients (2 female, 6 male). Morning cortisol levels for all patients were within the reference range with no suspicion of hypoadrenal cortical function. Eight (26.7%) patients had no endocrine disorder, 12 (40.0%) patients had one disorder, 8 (26.7%) patients had 2 disorders, and 2 (6.7%) patients had 3 endocrine disorders. There was no significant difference (p>0.050) in mean serum ferritin in thalassaemics with or without endocrinopathy, regardless of the number of endocrinopathy. CONCLUSION: There is high prevalence of endocrine disorders among Omani beta-thalassaemic adult patients. This signifies the importance of awareness for their development and monitoring for early detection and replacement therapy. No relationship between serum ferritin and development of endocrinopathy was noted.
Authors: N F Olivieri; D G Nathan; J H MacMillan; A S Wayne; P P Liu; A McGee; M Martin; G Koren; A R Cohen Journal: N Engl J Med Date: 1994-09-01 Impact factor: 91.245
Authors: Patrick B Walter; Eric A Macklin; John Porter; Patricia Evans; Janet L Kwiatkowski; Ellis J Neufeld; Thomas Coates; Patricia J Giardina; Elliott Vichinsky; Nancy Olivieri; Daniele Alberti; Jaymes Holland; Paul Harmatz Journal: Haematologica Date: 2008-05-10 Impact factor: 9.941