Congenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: A case report and review of the literature

被引:8
作者
Weigert A. [1 ,6 ]
Bierwolf J. [1 ]
Reutter H. [2 ,3 ]
Gembruch U. [4 ]
Woelfle J. [1 ,5 ]
Ganschow R. [1 ]
Mueller A. [2 ]
机构
[1] Clinic of General Pediatrics, University Hospital Bonn, Bonn
[2] Department of Neonatology and Pediatric Intensive Care, University Hospital Bonn, Bonn
[3] Institute of Human Genetics, University of Bonn, Bonn
[4] Department of Obstetrics and Prenatal Medicine, University of Bonn, Bonn
[5] Pediatric Endocrinology Division, Children's Hospital, University of Bonn, Bonn
[6] Universitätskliniken Bonn - Zentrum für Kinderheilkunde, Adenauerallee 119, Bonn
关键词
Case report; Congenital portosystemic shunts (CPSS); Hypoglycemia; Intrahepatic portocaval shunt; Secondary hyperinsulinism;
D O I
10.1186/s13256-018-1881-y
中图分类号
学科分类号
摘要
Background: Congenital portosystemic shunts present with various associated complications, such as other congenital malformations, hyperammonemia, or hepatopulmonary syndrome. Few cases of associated hypoglycemia have been reported so far and our case, to the best of our knowledge, describes the most severe extent of hypoglycemia. Case presentation: We describe the case of a newborn Arab boy with two intrahepatic portosystemic shunts, resulting in severe and persistent hypoglycemia, due to which one of the shunts was closed by interventional radiology whereas the other shunt had already closed spontaneously. Conclusions: Because he showed elevated levels for insulin and prolonged high insulin levels in an oral glucose tolerance test, our case supports the theory that portocaval shunts cause a reduced hepatic insulin reduction due to the high blood volume bypassing the liver. This case provides further insights into glucose regulation mechanisms of the liver and we suggest a consistent screening for hypoglycemia in patients with congenital portosystemic shunts. © 2018 The Author(s).
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