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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| Format: | Article |
| Language: | English |
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Elsevier
2021-12-01
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| Series: | Journal of Pediatric Surgery Case Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213576621002736 |
| _version_ | 1818826058949984256 |
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| 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. |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-19T00:21:38Z |
| format | Article |
| id | doaj.art-53ea3903351d47689e8096aba4728d69 |
| institution | Directory Open Access Journal |
| issn | 2213-5766 |
| language | English |
| last_indexed | 2024-12-19T00:21:38Z |
| publishDate | 2021-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of Pediatric Surgery Case Reports |
| spelling | doaj.art-53ea3903351d47689e8096aba4728d692022-12-21T20:45:32ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662021-12-0175102052A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemiaHisakazu Nakajima, M.D., Ph.D.0Kazuki Kodo, M.D., Ph.D.1Corresponding author. Department of Pediatrics, Yuseikai Midorigaoka Hospital, 1-13-3, Makami-cho, Takatsuki, Osaka 5691121, Japan.; Department of Pediatrics, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto City, Kyoto 6028566, JapanDepartment of Pediatrics, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto City, Kyoto 6028566, JapanA 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.http://www.sciencedirect.com/science/article/pii/S2213576621002736congenital absence of the portal veinhypoglycemiahyperglycemia with hyperinsulinemia |
| spellingShingle | 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 Journal of Pediatric Surgery Case Reports congenital absence of the portal vein hypoglycemia hyperglycemia with hyperinsulinemia |
| title | 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_short | 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 |
| topic | congenital absence of the portal vein hypoglycemia hyperglycemia with hyperinsulinemia |
| url | http://www.sciencedirect.com/science/article/pii/S2213576621002736 |
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