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Kongenitte portosystemiske shunter, diagnose og behandling af en multisystemisk sygdom hos børn

Forfatter(e)

Fie Brantbjerg Tinning1, Olav Bennike Bjørn Petersen2, Jesper Normann Steensberg3, Frederik Fouirnaies Buchvald1, Eva Fallentin4, Allan Rasmussen5, Susanne Frevert4 & Marianne Hørby Jørgensen1

1) BørneUngeAfdelingen, Juliane Marie Centret, Københavns Universitetshospital – Rigshospitalet, 2) Center for Føtalmedicin og Graviditet, Obstetrisk Klinik, Københavns Universitetshospital – Rigshospitalet, 3) Børnekardiologisk Klinik, Københavns Universitetshospital – Rigshospitalet, 4) Afdeling for Røntgen og Skanning, Københavns Universitetshospital – Rigshospitalet, 5) Abdominal Surgery, Københavns Universitetshospital – Rigshospitalet

Ugeskr Læger 2021;183:V01210041

Reference: 
UgeskrLæger 2021;183:V01210041
Diagnosis and treatment of a multisystemic disorder in children called congenital portosystemic shunts

Fie Brantbjerg Tinning, Olav Bennike Bjørn Petersen, Jesper Normann Steensberg, Frederik Fouirnaies Buchvald, Eva Fallentin, Allan Rasmussen, Susanne Frevert & Marianne Hørby Jørgensen

Ugeskr Læger 2021;183:V01210041

Congenital portosystemic shunts (CPSS) are rare malformations, which connect the portal venous system and the systemic circulation. The disorder is discovered at prenatal screening, neonatal cholestasis as an incidental finding or by systemic complications such as pulmonary hypertension, encephalopathies or liver nodules. CPSS are associated to cardiac malformations and several syndromes. Intervention radiology plays a key role in treatment by closure of the shunt, which is indicated, if the patient has complications, the shunt is extrahepatic, or it is still persistent at two years of age, using a two-step approach, as summarised and discussed in this review.

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