BSPED2018 Poster Presentations Miscellaneous Endocrinology (12 abstracts)
A 10 year experience of the management of severe hypocalcaemia associated with thymus transplantation in a United Kingdom tertiary centre
Nicole Goff 1 , Harshini Katugampola 1 , Elena Monti 1 , Katherine Taylor 1 , Rakesh Amin 1, , Peter Hindmarsh 1, , Catherine Peters 1 , Pratik Shah 1, , Helen Spoudeas 1 , Mehul Dattani 1, , Jeremy Allgrove 1 & Caroline Brain 1
1Great Ormond Street Hospital, London, UK; 2UCL Great Ormond Street Institute of Child Health, London, UK; 3University College London Hospital, London, UK.
Background: Thymus transplantation is undertaken for conditions associated with severe immunodeficiency. These comprise various genetic and syndromic associations including 22q deletion syndrome, CHARGE association, diabetic embryopathy, and other rarer conditions. Some of these conditions are associated with hypoparathyroidism and therefore hypocalcaemia. There are no established guidelines on the management and prevention of hypocalcaemia during the transplant period.
Case Series: 29 patients underwent thymus transplantation in our centre between 2009–2018 (age 2 months-2 years, 9 female). The underlying diagnoses included 22q11.2 (n=17, 1 only phenotypically 22q11.2), CHARGE association (n=8), diabetic embryopathy (n=2), FOXN1 mutation (n=1), and TBX1 mutation (n=1). 93% had hypoparathyroidism prior to transplant. 79% had hypocalcaemia (defined as corrected calcium (cCa)<2.0mmol/L) during admission. The mean nadir in the entire cohort was cCa=1.7 mmol/L (1.2–2.4 mmol/L). This occurred from 45 days pre-transplant to 35 days post-transplant (mean=day +1 post-transplant). 55% of patients required intravenous calcium during admission, and 35% required continuous calcium infusions. A diagnosis of 22q11.2 was associated with a slight increase in likelihood of requiring intravenous calcium (Likelihood Ratio=1.4, 63% of patients with 22q11.2 compared to 46% with alternate diagnosis). The mean duration of intravenous treatment was 4.7 days (1–39 days) and calcium requirements varied from 0.7–2.4 mmol/Kg/day (mean=0.7 mmol/Kg/day.) Associated complications included prolonged length of stay [median=28 days (11–255)], admission to intensive care (24%), hypocalcaemic seizures (14%), nephrocalcinosis (20% of those who underwent sonographic evaluation), infection (68%), mortality (10%).
Conclusion: This cohort is at significant risk of hypocalcaemia due to transplant conditioning, hypoparathyroidism, surgery itself and post-operative reduced enteral absorption. This case series highlights the variability of severe hypocalcaemia in patients undergoing thymus transplantation. Our practice has evolved over time to include prophylactic intravenous calcium infusions in patients with borderline hypocalcaemia at the start of conditioning. Further studies are warranted to evaluate whether early pre-operative intravenous calcium therapy reduces hypocalcaemia related post-operative complications. The lack of standardised evidence-based guidelines for managing these patients has important implications for morbidity, mortality and healthcare cost.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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