Hypergalactosaemia and portosystemic encephalopathy due to persistence of ductus venosus Arantii

التفاصيل البيبلوغرافية
العنوان: Hypergalactosaemia and portosystemic encephalopathy due to persistence of ductus venosus Arantii
المؤلفون: U. V. Arbenz, U. Willi, R. Gitzelmann
المصدر: European Journal of Pediatrics. 151:564-568
بيانات النشر: Springer Science and Business Media LLC, 1992.
سنة النشر: 1992
مصطلحات موضوعية: Galactosemias, Umbilical Veins, medicine.medical_specialty, Encephalopathy, Vena Cava, Inferior, Internal medicine, medicine, Humans, Abnormalities, Multiple, Hepatic encephalopathy, Ultrasonography, Newborn screening, Vascular disease, business.industry, medicine.disease, Surgery, Portal System, Child, Preschool, Hepatic Encephalopathy, Pediatrics, Perinatology and Child Health, cardiovascular system, Cardiology, Female, Complication, Splanchnic, business, Ductus venosus, Venous return curve
الوصف: Hypergalactosaemia was discovered in a newborn girl during routine metabolic screening. Hereditary enzyme deficiency was ruled out. Because hypergalactosaemia persisted, an open ductus venosus Arantii was suspected but remained undetected by conventional two-dimensional ultrasonography. It was demonstrated by combined colour and pulsed wave Doppler sonography. At age 3 years 6 months, the girl developed initial symptoms of portosystemic encephalopathy which progressed and was treated by protein restriction, oral lactulose and flumazenil, with some success. In the absence of enzyme deficiency, hypergalactosaemia in the newborn is an early sign of duct persistence. For the unambiguous diagnosis of an open duct, colour Doppler sonography is the method of choice. Pulsed wave Doppler sonography is recommended for pathophysiological characterisation of the splanchnic venous return.
تدمد: 1432-1076
0340-6199
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a5e57dae437d17feecea5c8d12438cf7
https://doi.org/10.1007/bf01957721
حقوق: CLOSED
رقم الأكسشن: edsair.doi.dedup.....a5e57dae437d17feecea5c8d12438cf7
قاعدة البيانات: OpenAIRE