Endocrine Abstracts

Introduction: Treatment of β thalassemia major results in excessive iron deposition in most tissues including the endocrine glands.

Aim: To find out the prevalence of endocrine complications in our thalassemia-endocrine clinic as set out by the UK thalassemia society guidelines.

Methods: Clinic notes and electronic results of all patients attending Birmingham City Hospital for the 2-monthly thalassemia-endocrine MDT clinic (2010–2012).

Results: Of 21 patients (mean age 29 years, 11 M:10 F, 19 South Asian, one Chinese, and one Cypriot), 20 were transfusion dependant and two post allogenic BMT. 4/19 (21%) were currently non-compliant with chelation (19/21 patients).

Multiple endocrinopathies were common: three (14%) had one endocrinopathy, nine (42%) had two, four (14%) had three and five (23%) had four or more endocrinopathies (older, mean age 35 years).

The commonest endocrinopathies were: hypogonadism (16/21–76%), hypoparathyroidism (9/21–43%), osteoporosis (9/21–43%), diabetes mellitus (8/21–38%) and hypothyroidism (8/21–38%). 9/21 (43%) had cardiac overload with LV systolic dysfunction.

Mean fructosamine in diabetics improved over time (377 in 2010 vs 336 in 2012). Osteoporosis was associated with hypogonadism (8/9), hyperparathyriodism (7/9), diabetes (3/9), low vitamin D (9/9) and low calcium levels in (6/9) patients. 50% showed improvement in serial DEXA scanning (uptake only 50%) following treatment. Hypothyroidism (mostly asymptomatic) had poor relationship to serum ferritin or compliance with chelation. Delayed puberty and amenorrhoea were the commonest manifestations of hypogonadism.

Conclusion: High prevalence of endocrinological problems in thalassemia patients makes regular surveillance essential both for early detection and treatment of complications especially since thalassemics are living longer. Compliance with chelation and high DNA rates were particularly noted in those with hypogonadism (testosterone therapy) and diabetes (BM monitoring, retinopathy screening and clinic attendance). Regular follow-up, on going education and multi-disciplinary support remains vital for improving prognosis in these patients.