A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia

A 3-year-old boy developed hypoglycemia with convulsions, rectal bleeding and hyperammonemia. Three-dimensional computed tomography scanning revealed the missing main branch of the portal vein, which confirmed the diagnosis of congenital absence of the portal vein (CAPV). Oral glucose tolerance test...

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Autores principales:	Hisakazu Nakajima, M.D., Ph.D., Kazuki Kodo, M.D., Ph.D.
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	congenital absence of the portal vein hypoglycemia hyperglycemia with hyperinsulinemia Pediatrics RJ1-570 Surgery RD1-811
Acceso en línea:	https://doaj.org/article/53ea3903351d47689e8096aba4728d69
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spelling	oai:doaj.org-article:53ea3903351d47689e8096aba4728d692021-11-24T04:29:18ZA 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia2213-576610.1016/j.epsc.2021.102052https://doaj.org/article/53ea3903351d47689e8096aba4728d692021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2213576621002736https://doaj.org/toc/2213-5766A 3-year-old boy developed hypoglycemia with convulsions, rectal bleeding and hyperammonemia. Three-dimensional computed tomography scanning revealed the missing main branch of the portal vein, which confirmed the diagnosis of congenital absence of the portal vein (CAPV). Oral glucose tolerance test showed hyperglycemia with hyperinsulinemia at 30 min, whereas hypoglycemia was observed during fasting and at 120 min. Postprandial hyperglycemia was observed repeatedly. Repetitive hypoglycemia and rectal bleeding were uncontrollable. Living-donor liver transplantation, which was successfully performed, was apparently effective in preventing fasting hypoglycemia and improving hyperammonemia; rectal bleeding ceased after the surgery. Considerably, CAPV could result in hyperinsulinemia with partial failure of hepatic insulin degradation and hypoglycemia due to the lack of glycogen accumulation. Postprandial hyperglycemia could result from the fact that glucose-abundant splanchnic blood directly flows into the systemic circulation through the portosystemic shunt. Therefore, it is important to be aware of CAPV as a cause of glucose metabolism abnormalities including fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia.Hisakazu Nakajima, M.D., Ph.D.Kazuki Kodo, M.D., Ph.D.Elsevierarticlecongenital absence of the portal veinhypoglycemiahyperglycemia with hyperinsulinemiaPediatricsRJ1-570SurgeryRD1-811ENJournal of Pediatric Surgery Case Reports, Vol 75, Iss , Pp 102052- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	congenital absence of the portal vein hypoglycemia hyperglycemia with hyperinsulinemia Pediatrics RJ1-570 Surgery RD1-811
spellingShingle	congenital absence of the portal vein hypoglycemia hyperglycemia with hyperinsulinemia Pediatrics RJ1-570 Surgery RD1-811 Hisakazu Nakajima, M.D., Ph.D. Kazuki Kodo, M.D., Ph.D. A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia
description	A 3-year-old boy developed hypoglycemia with convulsions, rectal bleeding and hyperammonemia. Three-dimensional computed tomography scanning revealed the missing main branch of the portal vein, which confirmed the diagnosis of congenital absence of the portal vein (CAPV). Oral glucose tolerance test showed hyperglycemia with hyperinsulinemia at 30 min, whereas hypoglycemia was observed during fasting and at 120 min. Postprandial hyperglycemia was observed repeatedly. Repetitive hypoglycemia and rectal bleeding were uncontrollable. Living-donor liver transplantation, which was successfully performed, was apparently effective in preventing fasting hypoglycemia and improving hyperammonemia; rectal bleeding ceased after the surgery. Considerably, CAPV could result in hyperinsulinemia with partial failure of hepatic insulin degradation and hypoglycemia due to the lack of glycogen accumulation. Postprandial hyperglycemia could result from the fact that glucose-abundant splanchnic blood directly flows into the systemic circulation through the portosystemic shunt. Therefore, it is important to be aware of CAPV as a cause of glucose metabolism abnormalities including fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia.
format	article
author	Hisakazu Nakajima, M.D., Ph.D. Kazuki Kodo, M.D., Ph.D.
author_facet	Hisakazu Nakajima, M.D., Ph.D. Kazuki Kodo, M.D., Ph.D.
author_sort	Hisakazu Nakajima, M.D., Ph.D.
title	A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia
title_short	A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia
title_full	A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia
title_fullStr	A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia
title_full_unstemmed	A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia
title_sort	3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/53ea3903351d47689e8096aba4728d69
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A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia

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