Obliterative portal venopathy: a study of 48 children.
Childhood obliterative portal venopathy presents at any age and may be genetic in origin. We report 48 children with obliterative portal venopathy, based on strict histologic criteria, investigated between 1972 and 2011. Diagnosis requires histology and is suggested by ultrasonography findings. Portal hypertension is the main complication but is absent in some cases. Prognosis is relatively good, but the detection of cardiopulmonary complications is essential.
Department of Pediatric Radiology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Stéphanie
S
Franchi-Abella
Department of Histopathology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Monique
M
Fabre
Department of Hematology and Biological Immunology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Emmanuel
E
Mselati
Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Marise Elia
ME
De Marsillac
Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Mohamed
M
Bayari
Department of Pediatric Radiology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Danièle
D
Pariente
Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; Inserm U 757, Université Paris-Sud 11, Orsay, France.
Emmanuel
E
Jacquemin
Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. Electronic address: olivier.bernard@bct.aphp.fr.
Olivier
O
Bernard
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000175:diagnosis; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology*; D008297:Male; D011169:Portal Vein / Q000473:pathology*; D011379:Prognosis; D014652:Vascular Diseases / Q000150:complications* / Q000175:diagnosis