Endocrinopathies are amongst the most common complications of thallasaemia, which is a hereditary disorder of haemoglobin synthesis and excessive iron deposition is thought to be the main reason.
Our hospital serves a multiethnic population and consequently we see a substantial number of patients with thallasaemia who are screened for endocrine complications. Those found to have endocrine problems are reviewed in our joint thallasaemia-endocrine clinic. Between 2003 and 2005, 14 patients were reviewed in the clinic. 8 were males. Their mean age was 32 years (range 2543). 12 were of Asian origin, 1 patient was Afro-Caribbean and 1 Caucasian. Their mean ferritin level was 2376 ng/ml (normal range 20300).
Six patients (42%) had diabetes while 2 patients had impaired glucose tolerance as defined by the WHO criteria. 6 patients (42%) had secondary hypogonadism with suppressed gonadotropins and subnormal testosterone levels. 2 patients (14%) had hypoparathyroidism. 1 patient had primary hypothyroidism. 4 patients (28%) had vitamin D deficiency. 5 patients (35%) had osteoporosis as evidenced by a T score of less than 2.5 by bone mineral density measurement.
The prevalence of endocrine complications is quite high, particularly in our multi-ethnic thallasaemic population. In particular, diabetes management is a big challenge given the ethnicity and the multi-disciplinary input required. Determining the exact prevalence of endocrine dysfunction in patients with thallasaemia is difficult because of the heterogeneity of the population and differences in age of first exposure to chelation therapy. However, the high prevalence of endocrinopathies in thallasaemia makes regular follow up essential for the early detection and appropriate treatment of associated complications. Thus, treatment of various endocrine complications along with improvements in protocols of transfusion regime and chelation therapy should improve the quality of life of these patients.
01 - 05 Apr 2006
European Society of Endocrinology