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[Porto sinusoidal vascular disease: an unusual cause of digestive bleeding].
Rev Gastroenterol Peru
July 2024
Servicio de Anatomía Patológica, Hospital Nacional Daniel Alcides Carrión, Lima, Perú.
Portal hypertension (PHT) is defined as an increase in pressure at the level of the portal vein above 5 mmHg, the most common cause being liver cirrhosis. Among the presinusoidal intrahepatic causes of PHT with portal venular involvement, what was traditionally known as idiopathic non-cirrhotic portal hypertension (NCIH) is described, with the requirements of excluding those patients who did not present PHT, as well as those with the presence of liver cirrhosis and thrombosis. portal venous vein (PVT).
View Article and Find Full Text PDFPortal Hypertension in Children: A Tertiary Center Experience in Turkey.
Pediatr Gastroenterol Hepatol Nutr
November 2023
Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University, Ankara, Turkey.
Purpose: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH.
Methods: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed.
Liver Transplantation in a Woman with Mahvash Disease.
N Engl J Med
November 2023
From the Department of Internal Medicine (J.R., M.D.), the Division of Gastroenterology and Hepatology (D.H.-D., T.S.), the Department of Medical Oncology, Sidney Kimmel Cancer Center (A.B.M., D.S.L.), the Department of Surgery (H.L., W.M., A.P.S.), the Division of Endocrinology, Diabetes, and Metabolic Diseases (J.M.), and the Department of Pathology and Genomics (M.A.), Thomas Jefferson University, the Department of Medical Oncology, Fox Chase Cancer Center (N.V.), and the Division of Human Genetics, Children's Hospital of Philadelphia (R.G.) - all in Philadelphia; the Division of Clinical Cancer Genetics, Section of Medical Oncology, Department of Medicine, Yale School of Medicine and Yale Cancer Center, New Haven, CT (V.N.G.); and the Division of Endocrinology, Diabetes, and Metabolism, David Geffen School of Medicine at UCLA, Los Angeles (R.Y.).
Mahvash disease is an exceedingly rare genetic disorder of glucagon signaling characterized by hyperglucagonemia, hyperaminoacidemia, and pancreatic α-cell hyperplasia. Although there is no known definitive treatment, octreotide has been used to decrease systemic glucagon levels. We describe a woman who presented to our medical center after three episodes of small-volume hematemesis.
View Article and Find Full Text PDFEvolving Understanding of Noncirrhotic Portal Hypertension.
Surg Pathol Clin
September 2023
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA. Electronic address:
Although cirrhosis is one of the most common causes of portal hypertension, noncirrhotic portal hypertension can result from hemodynamic perturbations occurring in the prehepatic, intrahepatic, and posthepatic circulation. Intrahepatic portal hypertension can be further subclassified relative to the hepatic sinusoids as presinusoidal, sinusoidal, and postsinusoidal. For many of these differential diagnoses, the etiology is known but the cause of idiopathic noncirrhotic portal hypertension, recently included in porto-sinusoidal vascular disease (PSVD), remains poorly understood.
View Article and Find Full Text PDFNoncirrhotic portal hypertension in primary biliary cholangitis with coexisting CREST syndrome.
Clin Res Hepatol Gastroenterol
May 2023
Division of Gastroenterology and Hepatology, Weill Cornell Medical College, New York, NY, USA.
Primary Biliary Cholangitis (PBC) is an autoimmune liver disease that is sometimes associated with CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. If left untreated, PBC eventually progresses to liver cirrhosis. We describe an adult patient with CREST-PBC who presented with recurrent variceal bleeding and ultimately required transjugular intrahepatic portosystemic shunt (TIPS) insertion.
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