2-yr-old with rare diabetes gets new lease of life

WRS results in recurrent episodes of acute liver failure. It is one of the common reason that leads to neonatal diabetes or early-onset of diabetes in patients whose parents suffer from diabetes.

At 34 weeks, the baby was born pre-term to diabetic parents. The symptoms became evident when the child reached was two-months-old and had other symptoms including fever, vomiting, diarrhoea. The boy was brought to Gleneagles Global Health City when he was one-and-a-half with similar symptoms.

On evaluation, doctors found that the child had jaundice and dry-coated tongue. Initial investigations revealed that the boy had diabetic ketoacidosis. Based on the clinical features and further laboratory investigations, a clinical diagnosis of acute liver failure with acute kidney injury was also made.

The doctors initially kept the child on IV fluids, insulin andantibiotics and decided to perform genetic analysis in the view of multi-organ dysfunction and history of diabetes in parents. The genetic analysisrevealed that the child was suffering from Wolcott – Rallison Syndrome.

Most of the patients with WRS get diabetes usually before they reach six months and skeletal dysplasia is diagnosed within the first year or two.

“In the first few months of life in patients with neonatal diabetes, the frequency of liver disease has been reported to be around a maximum of 85 per cent. In this case, the boy was managed conservatively and hepatic failure was prevented. He gradually improved with treatment and was later discharged when he was stable,” said Dr Perumal Karnan, a senior consultant in Clinical Lead Paediatric with Gleneagels Global Health City.