A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 microg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.

Hiperplasia nodular regenerativa del hígado secundaria a uso de azatioprina por trasplante renal previo, tratado con trasplante combinado hepático y renal / Combined hepatic and renal transplant after nodular regenerative hyperplasia of the liver secondary to azathioprine in renal transplant patient

Nodular regenerative hyperplasia is an uncommon condition, characterized by the presence of regenerative nodules with minimal or absence of fibrosis, which can lead to non-cirrhotic portal hypertension. There are numerous diseases, conditions and drugs that can cause it. Thiopurines, a group of immunosuppressors used in transplanted patients, has been linked to this entity. We report a case of a renal transplant woman, who has been on chronic therapy with azathioprine and that develops portal hypertension and end-stage renal disease, undergoing combined hepatic and renal transplant. Histological examination of the explanted liver was compatible with nodular regenerative hyperplasia. How azathioprine causes this entity is unknown, but endothelial vascular damage in a dose-dependent manner is postulated as the main mechanism. To our knowledge, this is the first case report of a renal transplant patient who develops nodular regenerative hyperplasia of the liver in association with azathioprine, and undergoes combined hepatic and renal transplant, with a favorable outcome 5 years post procedure.

La hiperplasia nodular regenerativa es una entidad infrecuente, que se caracteriza por la presencia de nódulos hepáticos con ausencia o mínima fibrosis y que puede llevar a hipertensión portal de origen no cirrótico. Existen diversas enfermedades, condiciones y medicamentos que la causan, destacando entre estos últimos las tiopurinas, inmunosupresores utilizados habitualmente en trasplantados. Se presenta el caso de una paciente trasplantada renal usuaria crónica de azatioprina, que desarrolla hipertensión portal además de deterioro de la función renal, requiriendo de un doble trasplante hepático y renal, destacando en la biopsia del explante hallazgos histológicos compatibles con hiperplasia nodular regenerativa. Los mecanismos de daño por azatioprina en esta entidad son desconocidos, pero se postula al daño endotelial dosis-dependiente como principal causa. La revisión de la literatura demuestra que este caso corresponde al primero de hiperplasia nodular regenerativa secundaria a azatioprina en trasplantado renal, que requiere de doble trasplante hepático y renal con evolución favorable hasta 5 años post trasplante.

Nodular regenerative hyperplasia of the liver:a clinical study of 12 cases / 中华消化杂志

Objective To review the clinical,histological and diagnostic aspects of 12 documented cases of nodular regenerative hyperplasia of the liver(NRHL),to make this condition be understood and dealt with better. Method Twelve NRHL cases were diagnosed based on liver biopsy from 300 portal hypertension patients who had been underwent splenectomy.Imaging studies were performed as part of the diagnostic evaluation.Clinical manifestation and biochemical tests were recorded at the time of diagnosis.Management and prognosis were also reviewed.Results Most patients were complicated with autoimmune disease,6 cases were diagnosed systemic lupus erythematosus and 1 was Crohn's disease and 1 suspected ulcerative colitis.Six cases were treated by prednisone and 3 cases by immunosuppressant.Eleven cases had suffered from portal hypertension.All cases had no history of viral hepatitis.Biochemical tests showed mild increase of liver enzyme and relative normal synthetic liver function.The histological finding was nodular in the hepatic parenehyma,with mild periportal fibrosis,intraportal lymphocytic infiltration,narrow and obstruction of branch of portal vein,and lack of hepatocyte necrosis.All cases were diagnosed liver cirrhosis and portal hypertension before operation.Management was directed to portal hypertension and varices bleeding with satisfactory results.Most of them keep a stable condition during the follow-up. Conclusion The NRHL was uncommon and its cause and pathogenesis was unclear,may be related with immune and hepatic blood circulation disorder.It should be considered in patients with unexplained portal hypertension and distinguished it from liver cirrhosis.Liver biopsy confirms the diagnosis.Management directed to portal hypertension may improve clinical condition.