Background: Hyperglycaemic hyperosmolar state (HHS) is a life-threatening condition rarely seen in paediatrics. It is however becoming increasingly recognised with the growing incidence of childhood type 2 diabetes mellitus (T2DM).
Objective and hypotheses: We present a child with BardetBiedel syndrome (BBS), with new-onset T2DM presenting in HHS, and discuss the dilemmas encountered in his management due to multiple co-morbidities, including renal replacement therapy, and GH deficiency.
Method: A 16-year-old Asian boy with BBS presented with hyperglycaemia (45.7 mmol/l) on routine 6-weekly follow-up. Medical history included chronic renal impairment, requiring a renal transplant, isolated GH deficiency and obesity. GH treatment was suspended peri-transplant and restarted 4-month post-transplant. He was also on prednisolone and tacrolimus. His father and brother had T2DM.
Results: Further investigations revealed HHS (ketones 0.1 mmol/l, pH 7.38, osmolarity 311 mOsmol/kg). Acute management included fluid resuscitation and i.v. insulin. He is now stable on daily glargine. HbA1c is now 6.9%, he has lost weight, renal function is stable and he is off GH therapy.
Conclusion: Our patient had multiple risk factors for T2DM: a predisposing syndrome, obesity, family history of T2DM, a high-risk ethnic background, steroid/tacrolimus treatment, on-going puberty, and GH therapy. There was significant family anxiety about the influence of GH in triggering the T2DM, and the impact of the high HbA1c on the transplanted kidney. While the influence of GH on the development of T2DM remains controversial, in this particular case, given the significant risk factors for T2DM, re-analysis of the risk-benefit analysis of continuing GH treatment post-transplant was warranted. Patients with syndromes predisposing to metabolic syndrome, like BBS, require cautious management. In complex cases with significant T2DM risk, including where diabetogenic medications are prescribed, screening for diabetes is essential, to ensure early treatment and preserve renal function in what is likely to be a precious kidney.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology