Figure 2: Overnight blood glucose following WMHMS administration.
Currently, at 4 years of age, the hypoglycemia is well-controlled on her current regimen. She continues to exhibit central hypotonia, mild ataxia, and moderate global developmental delay. Owing to these features, a non-contrast brain MRI was performed which demonstrated subtle white matter loss in the parietal region without evidence of cystic change favoured to be sequelae of previous ischemic insult. Investigations for metabolic causes of developmental delay returned negative including serum ammonia, copper, ceruloplasmin, homocysteine, amino acid and acylcarnitine profiles, as well as urine organic acids, oligosaccharides, sialic acid, uric acid, mucopolysaccharide screen, and creatine. In view of the constitutive insulin pathway activation, serial abdominal ultrasounds have been performed for tumour surveillance which have been unremarkable including liver and kidneys normal in size for her age.