Endocrine Abstracts

Introduction: Endocrine issues in Sickle Cell Disease (SCD): dysglycaemia, growth failure, hypogonadism, thyroid, and adrenal insufficiency (1) are often worsened by iron overload from transfusions (2). Data on their prevalence in children are limited, and screening practices vary (3,4). Improving screening can lead to early detection and treatment. We aimed to audit adherence to guidance in our unit and assess dysglycaemia rates in transfused CYP with SCD.

Methods: We reviewed electronic records from October 2021 to October 2024, following 2021 BSH guidelines (4). Recommendations included pubertal assessment from age 10; annual OGTT from puberty or age 10 if family history; yearly vitamin D, cortisol, thyroid, and pubertal assessment; biannual height/weight; and biennial DEXA scans.

Inclusion: Diagnosed with SCD or rare anaemia, on regular transfusions, aged at diagnosis and treatment start.

Results: Our cohort included 8 patients; median age at diagnosis was 10 years, treatment started at 20 years, with a median treatment duration of 10 years. Gender was evenly split; ethnicity included Pakistani (n,2 (25%), Black Caribbean (n,3 (38%), Black African (n2=,2 (25%), and others (n,1(12%). All received chelation. Screening adherence varied: vitamin D (100%), thyroid tests (60–100%), OGTT improved from 25% to over 60% over the years, while height measurements were lowest (25–50%). adherence was for height measurements, with compliance rates between 25% and 50%. Data for OGTT): Median age at first OGTT was 15 years (range 12–24) with Median follow-up duration was 5.5 years (range 2–9). The median number of OGTTs per patient was 3.5 (range 2–7). During follow-up, 2 patients showed impaired fasting glucose, which resolved later. One patient initially had impaired glucose tolerance, which also resolved.

Conclusion: Screening adherence was inconsistent, particularly for height. Dysglycaemia was rare and transient. A larger multicentre study is needed to better understand endocrine issues in SCD.