| Autor: |
Fiordaliso M; Department of Medicine and Ageing Sciences, University 'G. D'Annunzio' of Chieti-Pescara, Via dei Vestini 29, 66100 Chieti, Italy., Marincola G; Bariatric and Metabolic Surgery Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo Agostino Gemelli 8, 00168 Rome, Italy., Pala B; Division of Cardiology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Sapienza University of Rome, Via di Grottarossa, 1035/1039, 00189 Rome, Italy., Muraro R; Department of Innovative Technologies in Medicine & Dentistry, University 'G. D'Annunzio' of Chieti-Pescara, Via dei Vestini 29, 66100 Chieti, Italy., Mazzone M; Department of Innovative Technologies in Medicine & Dentistry, University 'G. D'Annunzio' of Chieti-Pescara, Via dei Vestini 29, 66100 Chieti, Italy., Di Marcantonio MC; Department of Innovative Technologies in Medicine & Dentistry, University 'G. D'Annunzio' of Chieti-Pescara, Via dei Vestini 29, 66100 Chieti, Italy., Mincione G; Department of Innovative Technologies in Medicine & Dentistry, University 'G. D'Annunzio' of Chieti-Pescara, Via dei Vestini 29, 66100 Chieti, Italy. |
| Abstrakt: |
Non-cirrhotic portal hypertension (NCPH), also known as idiopathic non-cirrhotic portal hypertension (INCPH) and porto-sinusoidal vascular disorder (PSVD), is a rare disease characterized by intrahepatic portal hypertension (IPH) in the absence of cirrhosis. The precise etiopathogenesis of IPH is an area of ongoing research. NCPH diagnosis is challenging, as there are no specific tests available to confirm the disease, and a high-quality liver biopsy, detailed clinical information, and an expert pathologist are necessary for diagnosis. Currently, the treatment of NCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied that aimed to modify the natural history of the disease; however, transjugular intrahepatic porto-systemic shunt (TIPS) placement, shunt and liver transplantation are considerable symptomatic options. In this review, we discuss the heterogeneity of NCPH as well as its etiopathogenesis, clinical presentation and management issues. Starting from the assumption that portal hypertension does not always mean cirrhosis, cooperative studies are probably needed to clarify the issues of etiology and the possible genetic background of this rare disease. This knowledge might lead to better treatment and perhaps better prevention. |
