UAE: Abu Dhabi hospital successfully treats newborn with rare disease

Doctors at Abu Dhabi’s Sheikh Shakhbout Medical City have treated a rare and complex case of congenital hyperinsulinemic hypoglycaemia in a four-month-old baby girl from Yemen.

The baby from Socotra Islands was transferred to SSMC from another healthcare facility.

Dr Gamal Ahmed, division chair of the paediatric intensive care unit at SSMC, said that a multidisciplinary team discussed the management and care of the patient to ensure best outcome.

“The young patient came to us, and after a few days, developed convulsions that were associated with refractory hypoglycaemia, which her laboratory workup confirmed. After we managed to control the convulsions with unremarkable brain sequelae or complications by MRI and EEG, the baby was diagnosed with congenital hyperinsulinemic hypoglycaemia, a rare endocrine disease.”

Hypoglycaemia is a condition in which blood sugar (glucose) level is lower than normal. Congenital hyperinsulinism is the most severe cause of persistent hypoglycaemia in new-born babies and children. If hypoglycaemia is prolonged, it can be the most common cause of irreversible but preventable brain damage. Hyperinsulinemic babies usually need up to five times more glucose than children with typical glucose requirements do.

SSMC is a joint venture between Mayo Clinic and Abu Dhabi Health Services Company (Seha). The multidisciplinary team at SSMC consulted colleagues at Mayo Clinic and other international experts to determine before going for a medical approach rather than a surgical one.

“After the diagnosis of the patient’s condition was confirmed, we used quite an uncommon treatment, but one that is recommended in such a case of refractory hypoglycaemia. The patient’s blood glucose continued to fluctuate for a few days with the escalation of treatment until a steady state of blood glucose was reached. By the end of the treatment course, we observed no hypoglycaemic episode.” Dr Ahmed said.

“This case was a success and a prime example of how we exercise our model of care at SSMC. We had a collegiate and cooperative multidisciplinary team who took consultation from international expertise, including our colleagues at Mayo Clinic, and we had the patient’s parents who were involved in the patient’s care at every step of the way.”

In most countries, hyperinsulinism has an incidence of 1 in every 25,000 to 50,000 births. There are many genetic, transient, and metabolic causes of hyperinsulinism, but it’s very hard to diagnose. Signs and symptoms of hypoglycaemia to look out for are lethargy or lack of energy, irregular body temperature, a weak or high-pitched cry, seizures, fast heart rate, among others.

“Both the parents were highly appreciative of the compassionate care shown and the treatment provided to their baby. The referring hospital shared their appreciation for the successful management of the baby’s condition and for carrying out the best treatment suitable to the uncommon condition,” Dr Ahmed added.

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