A 16-year-old boy with BBS was on immune suppression therapies from the age of 9 years had a normal oral glucose tolerance test (OGTT) 18 months prior to presentation. He had presented with an incidental finding of high blood glucose level on routine screening at a renal outpatient clinic. He reported only a history of polydipsia, for which he had been drinking large quantities of sugary drinks. On examination, we found him to be overweight (body mass index (BMI): 30.3 kg/m2; BMI SDS: 2.62), and the patient exhibited mild tachycardic with a heart rate of 100 bpm but normal capillary refill and blood pressure of 121/69 with moist mucus membranes and was passing urine. His renal function was deranged. The patient was initially administered with normal saline 10 mL/kg followed by replacement of his estimated fluid deficit of 5% over 48 h, plus maintenance fluids intravenously. The patient was initially administrated paediatric unit 0.05 units/kg/h insulin and reduced depending on the response. Once blood glucose and osmolarity readings had improved, the patient was changed to subcutaneous insulin and was discharged home on twice daily biphasic isophane insulin. In the 12 months since his presentation, his glycatedhaemoglobin (HbA1c) has improved from 12.1% to 6.4%. Following initial twice daily treatment, he was changed to daily glarginemonotherapy. Eight months after diagnosis, allowing time for an initial improvement in HbA1c using insulin, the patient was switched to long-term metformin only. GH treatment has been discontinued—there were concerns about the possible link between GH therapy and T2DM, particularly in such a high-risk patient. Following risk-benefit analysis, the family felt they were happy with his height outcome, and in discussion with the endocrine team, GH treatment was stopped.
Published On: June 2019
Online ISSN: 2456-8163