Pathology of idiopathic non-cirrhotic portal hypertension

Kage, Masayoshi

doi:10.1007/s12072-017-9823-0

Cited by 8 publications

(4 citation statements)

References 17 publications

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“…However, the essence of cirrhosis nodule is actually the regeneration nodules after diffuse destruction of the liver parenchyma. Liver biopsy is mandatory for differential diagnosis and treatment planning . Notably, 51.8% patients in our cohort for whom ultrasonic records were available showed widening of portal vein, which is liable to decrease flow velocity, contribute to the formation of blood clots, and further the PVT on the luminal surface of the portal vein.…”

Section: Resultsmentioning

confidence: 84%

“…Liver biopsy is mandatory for differential diagnosis and treatment planning. 31,43 Notably, 51.8% patients in our cohort for whom ultrasonic records were available showed widening of portal vein, which is liable to decrease flow velocity, contribute to the formation of blood clots, and further the PVT on the luminal surface of the portal vein. Subsequently, the thrombosis typically extends to the splenic and superior mesenteric veins or the intrahepatic portal branches.…”

Section: Discussionmentioning

confidence: 87%

“…12,[47][48][49] From a microcosmic perspective, the pathology of IPH is characterized by fibrotic expansion of intermediate-size portal tracts, round-shaped fibrous expansion of portal tracts, and narrowing, collapse, and loss of portal vein branches; in addition, aberrant vessels adjacent to portal tracts are a prominent feature in most peripheral portal tracts. 43 Thus, portal fibrosis and obliteration of the small portal vein may be the main pathological process that leads to portal hypertension in IPH. Therefore, any factor that can cause portal fibrosis and obliteration of the small portal vein can lead to IPH, although the etiology is probably multifactorial.…”

Section: Discussionmentioning

confidence: 99%

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Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Sun

Shao

et al. 2018

J of Gastro and Hepatol

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Background and Aim Idiopathic portal hypertension (IPH) refers to a relatively rare condition characterized by intrahepatic portal hypertension in the absence of underlying disease such as liver cirrhosis. Methods We retrospectively reviewed 338 patients with IPH that were diagnosed at the pathological consultation center of our hospital. Results The ratio of male to female patients was 1:1. Mean age at onset was 35.1 ± 16.5 years; male patients on average were 12 years younger than female patients at onset. The median duration from onset to IPH diagnosis was 12 months. In 50 patients, medication use may have been an etiological factor. The most common clinical manifestations were splenomegaly (91.3%) and hypersplenism (68.9%); 57.0% patients presented varicosis, while 25.1% patients had a history of variceal bleeding. Nodular regenerative hyperplasia was found in 22.2% liver biopsies. Among patients for whom laboratory data were available, 65.0%, 50.3%, and 71.4% patients presented leukopenia, anemia, and thrombocytopenia due to hypersplenism. Liver function was mostly in the compensated stage. Female patients showed worse leukopenia and anemia, while male patients were more likely to have abnormal serum transaminase and bilirubin levels. Sixty‐seven patients received surgical or interventional treatment. Conclusions High‐quality liver biopsy, detailed clinical information, and expert pathologist are necessary for diagnosis of IPH. IPH can occur concurrently with other liver disease such as hepatitis and drug‐induced liver injury. Medication appears to be an important etiological factor for IPH in China. Management approach was largely focused on treatment of portal hypertension and its complications.

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Section: Resultsmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

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Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Sun

Shao

et al. 2018

J of Gastro and Hepatol

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“…Other common findings on biopsy of NCPH are NRH, phlebosclerosis, periportal shunting, sinusoidal dilatation, and rudimentary portal tract (71,72). NCPH has been associated with a broad range of infectious, immunological, toxic, pro-thrombotic, genetic diseases discussed above (67,73), as well as exposure to certain medications, such as didanosine and azathioprine (74,75). Interestingly, in rare cases, loss-of-function mutations in DGUOK (76) and KCNN3 (77), in a recessive and dominant inheritance pattern, respectively, have been shown to cause NCPH, providing new mechanistic insights into this poorly understood liver disease.…”

Section: The Known Liver Diseases Of Unknown Causementioning

confidence: 99%

Undiagnosed liver diseases

Gao¹,

Hercun²,

Heller³

et al. 2021

Transl Gastroenterol Hepatol

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The landscape of chronic liver disease has drastically changed over the past 20 years, largely due to advances in antiviral therapy and the rise of metabolic syndrome and associated non-alcoholic fatty liver disease (NAFLD). Despite advances in the diagnosis and treatment of a variety of liver diseases, the burden of chronic liver disease is increasing worldwide. The first step to addressing any disease is accurate diagnosis.Here, we discuss liver diseases that remain undiagnosed, either because they are difficult to diagnose or due to hepatic manifestations of an unrecognized systemic disease. Additionally, their underlying etiology may remain unknown or they represent previously uncharacterized and therefore novel liver diseases. Our goal is to provide a framework for approaching undiagnosed liver diseases which elude standard hepatic diagnostic work-up and whose patterns of disease are often overlooked.

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Nichtzirrhotische portale Hypertension – Ursachen und praktisches Management

Queck

Trebicka

2021

Gastroenterologe

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ZusammenfassungUnter dem Krankheitsbild der nichtzirrhotischen portalen Hypertension (NCPH) wird eine heterogene Gruppe nichtzirrhotischer Pathogenesen portaler Hypertension zusammengefasst. Es wird anhand der korrespondierenden anatomischen Veränderungen zwischen einer prä-, intra- und posthepatischen Genese unterschieden. Gefäßveränderung proximal der Leber (z. B. Pfortaderthrombose) führen zu einer prähepatischen, distal der Leber gelegene (z. B. Budd-Chiari-Syndrom) zu einer posthepatischen NCPH. Intrahepatisch kann die NCPH durch angeborene Defekte (z. B. kongenitale hepatische Fibrose), granulomatöse Erkrankungen (Schistosomiasis, Sarkoidose), Malignome, Medikamente, das sinusoidale Obstruktionssyndrom, Speichererkrankungen (Amylodiose), idiopathisch und durch die portosinusoidale vaskuläre Erkrankung ausgelöst werden. Klinisch manifestiert sich die Erkrankung durch Komplikationen portaler Hypertension, jedoch häufig auch als Zufallsbefund (Varizen; leichtgradige Blutbildveränderungen; Transaminasen/γ-Glutamyltransferase-Erhöhung). Die Basisdiagnostik umfasst den laborchemischen und histologischen Ausschluss einer Lebergrunderkrankung und Zirrhose, die abdominelle Sonographie und die Gastroskopie. Aufgrund einer Assoziation zu hämato- und immunologischen Erkrankungen ist eine interdisziplinäre Abklärung indiziert. Neben der Therapie der Grunderkrankung ist beim Vorliegen einer Thrombose oder einer prothrombotischen Veranlagung eine Antikoagulation empfohlen. Komplikationen der portalen Hypertension werden ähnlich wie bei der zirrhotischen portalen Hypertension behandelt, jedoch findet die Shunttherapie als endgültige Therapie bei erhaltener Leberfunktion mehr Verwendung.

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Pathology of idiopathic non-cirrhotic portal hypertension

Cited by 8 publications

References 17 publications

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Undiagnosed liver diseases

Nichtzirrhotische portale Hypertension – Ursachen und praktisches Management

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