[Vena portae obstruction as a cause of portal hypertension in a child with Turner syndrome].
Cardiovascular and renal malformations are well-known in Turner syndrome. However, gastrointestinal bleeding is less frequent. The possible etiologies of gastrointestinal bleeding in Turner syndrome are intestinal teleangiectasia, inflammatory bowel disease and portal hypertension. The authors report a 3-year-old girl with Turner syndrome who presented with severe gastrointestinal bleeding requiring transfusion. The radiological examination indicated prehepatic portal hypertension as a reason for haematochezia. The liver biopsy demonstrated the anomaly of intrahepatic arteries and veins. In this report we describe a case of congenital portal vein obstruction and we reviewed liver abnormalities associated with Turner syndrome and causes of obstruction of vena portae in childhood.
Semmelweis Egyetem, Altalános Orvostudományi Kar I. Gyermekgyógyászati Klinika Budapest Bókay u. 53. 1083.
Katalin Eszter
KE
Müller
Katalin
K
Müller
Antal
A
Dezsofi
Eva
E
Kis
István
I
Máttyus
Gábor
G
Veres
András
A
Arató
László
L
Szonyi
D001706:Biopsy; D001803:Blood Transfusion; D002675:Child, Preschool; D016145:Endoscopy, Digestive System; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000209:etiology* / Q000628:therapy; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology; D008099:Liver / Q000098:blood supply / Q000473:pathology; D011169:Portal Vein / Q000473:pathology*; D014424:Turner Syndrome / Q000150:complications* / Q000473:pathology