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At least 19 recordsLinked to original sources
PubMed2026

COMMD9-regulated endothelial cell abnormality-induced hypercoagulability is associated with Budd-Chiari syndrome.

BACKGROUND: Budd-Chiari syndrome (BCS) presents diagnostic and treatment challenges owing to its insidious onset. Genetic variants associated with BCS vary geographically; in Asian populations, the condition is primarily caused by membranous obstruction composed of endothelial cells (ECs). A better understanding of the genetic pathogenesis of membranous BCS may offer new insights into disease mechanisms. METHODS: This study employed whole-exome sequencing to identify candidate genes responsible for EC abnormalities in 485 patients with membranous BCS and 329 patients with vascular malformations (VaMs). Functional investigations were conducted to validate the selected genes in vitro and in vivo. RESULTS: Whole-exome data revealed that the frequency of variants in the vascular function-related KLHDC2 exceeded that of JAK2 in BCS. Knockdown of KLHDC2 promoted adhesion and suppressed proliferation of ECs. In addition, 92 genes enriched for rare variants overlapped between BCS and VaMs. Systems biology analysis revealed two gene clusters, including COMMD9, enriched in proteins intolerant to loss-of-function mutations. Furthermore, suppression of COMMD9 impaired EC migration and tube formation, inhibited subintestinal angiogenic sprouting in zebrafish, and elevated EC adhesion. Transcriptomic analysis linked COMMD9 to EC abnormalities via the PI3K-Akt pathway. Commd9 knockdown promoted venous hypercoagulability in vivo following drug or ligation-induced stenosis. CONCLUSIONS: These findings indicate that multiple rare genetic variants, particularly in COMMD9, are involved in the development of membranous BCS by regulating hypercoagulability induced by EC abnormalities. These findings may help guide future clinical research towards improved understanding and treatment of BCS.

Budd–Chiari syndrome↗
PubMed1975

The Budd-Chiari syndrome: angiography and its complications.

Four patients with the Budd-Chiari syndrome are presented. Hepatic venography and inferior vena cavography confirmed the diagnosis in these patients. Angiography was used to evaluate the liver and the superior mesenteric and portal venous drainage. Contrast medium complications, ranging from nausea to renal shut-down and death, were encountered in three of these patients, an unusually high number of angiographic complications. The role of angiography in the Budd-Chiari syndrome is assessed.

Adult↗
PubMed1978

Mesoatrial shunt: a new treatment for the Budd-Chiari syndrome.

A patient is presented with the Budd-Chiari Syndrome. Because of a thrombosed inferior vena cava, none of the standard portal-systemic shunts could be utilized for decompression of the engorged liver. A new shunt constructed by interposing a Dacron graft between the superior mesenteric vein and the right atrium was performed. Portal pressure was reduced by the shunt from 30 cm of H2O to 10 cm of H2O. Patency has been confirmed post-operatively by catheterization and with angiography. The patient is asymptomatic with normal liver function tests nine months following the procedure. A surgical approach is outlined for symptomatic patients with the Budd-Chairi Syndrom.

Blood Pressure↗
PubMed1977

[Hepatogenic polyglobulinemias and polycythemias].

The authors distinguish three kinds of hepatogenous polyglobulia: Polycythaemia caused by Budd-Chiari syndrome, polycythaemia caused by a Mosse syndrome (cirrhosis without liver venous thrombosis) and polyglobulia caused by liver tumours. In all three cases the same mechanism is likely to induce polycythaemia or polyglobulia respectively. In addition to the three cases of the Mosse syndrome published in 1966, the present paper deals with three cases of Budd-Chiari syndrome. Twice the Budd-Chiari syndrome was followed by a polycythaemia, once a Budd-Chiari syndrome was developed in the course of a polycythaemia vera.

Adult↗
PubMed1977

[Sequential hepato-splenic scintigraphy with 99mTc-pertechnetate in experimental post-hepatic block (author's transl)].

Using baloon catheters in the inferior vena cava, post-hepatic blocks were produced in experimental animals. Subsequent liver perfusion scintigraphy showed marked reduction in portal flow. The significance of these findings in the diagnosis of the Budd-Chiari syndrome and its differentiation by scintigraphy from an intrahepatic block in cirrhosis of the liver is discussed.

Animals↗
PubMed1976

Leiomyosarcoma of the inferior vena cava.

The thirty-second case of leiomyosarcoma of the inferior vena cava and the twelfth resectable patient in the series is presented. A review of the literature demonstrates a marked female preponderance (5 to 1). The most common presenting symptom is right abdominal pain and a palpable mass. The different surgical problems generated by the involved segment of the cava are discussed. Noteworthy is the high incidence of Budd-Chiari syndrome owing to hepatic vein obstruction with involvement of the upper third of the cava in the postmortem cases. An argument is developed for debulking the tumor for palliation when it is not completely removable.

Humans↗
PubMed1978

The normal and abnormal left superior intercostal vein.

A study of 500 normal erect posteroanterior chest radiographs was undertaken to determine the incidence of visualization and size of the left superior intercostal vein in normal individuals. The vein produces a small "nipple" lateral to the aortic knob on 1.4% of normal erect posteroanterior chest films; its diameter can be up to 4.5 mm in normal patients. Dilatation of the vein beyond 4.5 mm is a useful sign of a circulatory abnormality warranting further study. Dilatation may be due to absence of the inferior vena cava, hypoplasia of the left innominate vein, congestive failure, portal hypertension, Budd-Chiari syndrome, or superior or inferior vena caval obstruction. The differential diagnosis of an enlarged left superior intercostal vein includes mediastinal mass, especially lymphadenopathy, and aneurysm of the arch of the aorta.

Adult↗
PubMed1975

[Segmental angiography of the liver with laparoscopy and electro-coagulation. Selective hepatography (author's transl)].

Segmental angiography of the liver with laparoscopy and electro-coagulation has high diagnostic value. The circulatory systems of the liver may be studied selectively or in various combinations. Morphologic lesions as well as functional disturbances of liver arteries and veins, the portal vein and to a certain degree lymph vessels can be visualized precisely. An improved indication for shunt operations and a more reliable prognosis may be achieved by direct transhepatic pressure measurements, especially within the hepatic artery and portal vein. An early diagnosis of the Budd-Chiari-Syndrome is enabled by the combined segmental veno- and portography which could not be achieved by the known techniques so far. Complications are rarely seen. In 1070 examinations there was one suspicion for bilihemia, one case of hemobilia, and one case of a subcapsular hematoma. Twice a liver lobe was perforated. There was never a permanent damage to the patient. Biochemical and nuclear medicine studies are suggested.

Adult↗
PubMed1975

Intracavitary cardiac extension of hepatoma.

Acute right atrial obstruction in a 62-year-old man was demonstrated angiographically to be due to a tumor, which was later proved to be metastatic hepatoma. Resection of the tumor resulted in total symptomatic relief.

Bile↗
PubMed1975

[Indirect (arterial) splenoportography and portography in intraphepatic block].

Morphology and flow dynamics in the lieno-mesentericoportal territory were investigated in 15 normal persons and 199 patients with pre/intra and intra-hepatic blocks. Indirect (arterial) splenoportography with selective catherization of the splenic artery provided angiograms varying between good and diagnostically useful in 98% of cases. With injection into the truncus coeliacus, this figure falls to 77%. The various types of collateral circulation are stated and discussed. The scope and limits of the methods are shown by comparative surveys between direct splenoportography and indirect splenoportography and portography, using 16 patients. As far as the chronological sequence of angiographical diagnostics in the intrahepatic block is concerned, the indirect method shall precede direct splenoportography, which shall only be employed if the indirect method of examination does not provide sufficient information, the patient has shunt capacity and an operation is to be performed immediately.

Adult↗
PubMed1975

Arteriographic demonstration of intravenous tumor extension.

The arteriographic demonstration of a linear, striated arterial pattern in the anatomic course of the draining vein is reliable evidence of venous extension by a tumor and represents the supply to the intravenous component of the neoplasm. In addition to its previously described occurrence with hypernephromas, this report documents the characteristic angiographic appearance with hepatocellular tumors and a retroperitoneal osteosarcoma as reliable evidence of tumor extension into the inferior vena cava. It is possible that in some cases, the parallel arteries represent markedly enlarged vasa vasorum of the involved venous structure. The frequent invasion of the inferior vena cava by hepatomas and the importance of inferior vena cavography in their angiographic evaluation is also emphasized.

Adult↗
PubMed1979

Veno-occlusive disease of the liver after allogeneic bone marrow transplantation: possible association with graft-versus-host disease.

Acute veno-occlusive disease of the liver developed in seven of 29 patients undergoing bone marrow transplantation for treatment of leukemia, aplastic anemia, or disseminated carcinomatosis. All seven died despite successful marrow engraftment. Hepatic failure was the principal cause of death in four and contributory in three. The veno-occlusive disease did not relate to the nature of the pretransplant immunosuppressive regimen, since it occurred in patients receiving irradiation alone, chemotherapy alone, or both. Twenty-two of the patients were autopsied. Among these, the lesion was found in seven of 11 in whom a graft-versus-host reaction developed but in none of the 11 without such a reaction who had received similar pretransplant immunosuppression (P less than 0.025). Hence, acute veno-occlusive disease of the liver appears to be a complication of allogeneic bone marrow transplantation related to the development of a graft-versus-host reaction.

Acute Disease↗