ABU DHABI, A multidisciplinary team of physicians at Sheikh Shakhbout Medical City (SSMC), has successfully treated an extremely rare and uncommon case of complex congenital hyper-insulinemic hypoglycaemia in a four-month-old girl from Socotra Islands in Yemen, who was transferred to the SSMC from another healthcare facility.
The SSMC is one of the UAE’s most prominent hospitals for severe and complex care and a joint-venture between Mayo Clinic and Abu Dhabi Health Services Company (SEHA).
Dr. Gamal Ahmed, Division Chair of the Paediatric Intensive Care Unit at the SSMC, said, “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 controlling the convulsions with unremarkable brain sequelae or complications by MRI and EEG, the baby was diagnosed with congenital hyper-insulinemic hypoglycaemia, a rare endocrine disease. A multidisciplinary team discussed management and care of the patient to ensure the best approach and long-term outcomes.”
Congenital hyperinsulinism is the most severe cause of persistent hypoglycaemia in newborns and children. If hypoglycaemia is prolonged, it can be the most common cause of irreversible but preventable brain damage. Hyper-insulinemic babies usually need up to five times more glucose than children with typical glucose requirements. Nearly 60 percent of babies with hyperinsulinism develop hypoglycaemia within the first month of life. Of the remaining 40 percent, almost all will develop hypoglycaemia before a year.
In most countries, hyperinsulinism has an incidence of 1 to every 25,000 to 50,000 births. For those with hyperinsulinism, the pancreas, responsible for insulin secretion, is blind to the blood glucose level and makes insulin even when blood sugar levels are not high, causing severe, and often prolonged, hypoglycaemia.
“After we confirmed the diagnosis of the patient’s condition, 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 it reached a steady state of blood glucose. By the end of the treatment course, we observed no hypoglycaemic episode,” Dr. Ahmed continued.
Physicians were, however, conscious that treating a four-month-old baby with refractory hypoglycaemia with uncommon medication that may not be available in her home country did pose its own challenges. They had to receive compliance from the baby’s parents on management in their home country, educate and train the parents on home blood glucose monitoring and provide them with a glucometer.
The primary approach the multidisciplinary team at the SSMC had to decide upon was whether to pursue medical versus surgical treatment. They consulted with colleagues at the parent partner company, Mayo Clinic, for a second opinion, and other international experts to determine whether a medical approach was the better course.
“This case was a success and a prime example of how we exercise our model of care at the SSMC. Both the parents were highly appreciative of the compassionate care shown and the treatment provided to their baby,” Dr. Ahmed said in conclusion.
Source: Emirates News Agency