PROTECTING
THE FRAGILE

Expert doctors at BLK assure a healthy life for a newborn with Neonatal Hypoglycemia

A one day old full-term male baby was admitted to BLK Centre for Child Health with complaints of fast breathing, low blood sugar and jitteriness. The baby also had undescended testes and a small penis. On examination, the baby was found to be tachypneic with low set ears, had a depressed nasal bridge with a high arched palate and empty scrotum without hyperpigmentation and stretched penile length of 1.7 cm. Critical blood sample (when blood sugar was 37 mg/dl) was taken, and treatment was started in the form of intravenous glucose. High flow nasal oxygen was given in view of the respiratory distress.

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Additional evaluation revealed low serum cortisol, low thyroid profile suggestive of central Hypothyroidism. Possibility of multiple pituitary hormone deficiency was suspected as the baby was hypoglycemic with micropenis, low cortisol and central Hypothyroidism. Further investigation revealed low

serum growth hormone level, low gonadotropin and testosterone levels.

Fluorescence in situ hybridization report showed the male genotype and 17- OHP levels were normal. MRI brain showed small sized sella with significantly hypoplastic anterior pituitary gland with absent infundibulum and ectopic posterior pituitary seen posterior to optic chiasm with normal brain parenchyma suggesting of pituitary stalk interruption syndrome. Most cases of neonatal hypoglycemia are easy to manage as the baby improves over a few days to weeks. However, persistent hypoglycemia due to growth hormone deficiency is very difficult to treat because of lifelong injection and regular monitoring.

The patient was started on supplementary growth hormone injections and hydrocortisone replacement with improvement in his blood sugar levels. He was found fit to be weaned off intravenous fluids and was able to maintain his dextrose levels with just oral feeding. The patient was discharged on growth hormone; hydrocortisone and thyroid hormone supplement with close follow up of endocrine and neurology. At the 6 months follow up, the baby was reported to be developmentally normal.

Diagnosis of multiple pituitary hormone deficiency can be challenging because the clinical manifestations are often non-specific and can mimic other disease processes in the neonatal period. Neonates with growth hormone deficiency often have a normal size at birth and have refractory hypoglycemia and are at risk for clinical decompensation if there is a delay in diagnosis and treatment.

 

dr-kumar

Dr. Kumar Ankur
Consultant
BLK Centre For
Child Health
BLK Super Speciality
Hospital, New Delhi

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