Turkiye Klinikleri Pediatri, cilt.29, sa.3, ss.187-194, 2020 (Scopus)
Copyright © 2020 by Türkiye Klinikleri. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).Congenital hyperinsulinemic hypoglycemia (CHH) is the most common cause of persistant hypoglycemia in the neonatal period. Histologically, CHH is classified in three subgroups as diffuse, focal, and atypical forms. 18F-fluoroDOPA positron emission tomography/computed tomography (PET/CT) is a method with 100% accuracy for detecting the focal lesion. We presented a case with severe hypoglycemia in the neonatal period. The case was heterozygous for ABCC8 mutation and diagnosed with CHH. It was unresponsive to diazoxide. Focal lesion in the pancreas was detected by 18F-DOPA PET/CT in our hospital, it was cured after focal pancreatectomy. In conclusion, here, we presented treatment and follow-up process of the case with focal CHH, which was identified by 18F-DOPA PET/CT in our hospital, for the first time in our country. Due to 18F-DOPA PET/CT imaging, patients diagnosed with focal CHH and unresponsive to the medical treatment are both cured and prevented from acute and long-term serious complications of total pancreatectomy.