Correspondence to ‘Neonatal hypoglycaemia due to ABCC8 gene mutation’

Sir,

I read with interest the article by Kumar et al.,[¹] on neonatal hypoglycemia which includes a case report of ABCC-associated congenital hyperinsulinism (CHI) and a comprehensive approach to neonatal hypoglycemia, despite being a letter to the editor! I noted a few concerns in the article. The patient had diazoxide-unresponsive but octreotide-responsive disease. ¹⁸F-DOPA PET/CT is not routinely indicated in all patients with CHI but is recommended only for diazoxide-unresponsive CHI with paternally inherited/de-novo monoallelic mutations in ABCC8/KCNJ11 or no mutations in ABCC8/KCNJ11.[²] The patient was subjected to functional imaging awaiting the genetic results, that too with one of the least sensitive modalities, ^99mTc-HYNIC-TOC scintigraphy. I believe that the scintigraphy should not have been performed on this patient. Secondly, it is surprising to note that the authors decided that the patient should undergo near-total pancreatectomy despite the disease being octreotide-responsive. Fortunately, the parents deferred the surgery. In such patients, monthly intramuscular administration of long-acting somatostatin analogs (SSA) is recommended.[²] The results of near-total pancreatectomy are unsatisfactory with low success rates, and high rates of complications including permanent diabetes mellitus, and exocrine insufficiency.[³] Hence, its use is not recommended for CHI that responds to medical therapy.[²] I note that the authors do not mention offering long-acting SSA to the patient which should have been preferred over near-total pancreatectomy.

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