The Budd‐chiari Syndrome in Paroxysmal Nocturnal Haemoglobinuria—revisited

Leibowitz, Alan I.; Hartmann, Robert C.

doi:10.1111/j.1365-2141.1981.tb07278.x

Cited by 12 publications

(12 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In BCS caused by hematologic disorders such as polycythemia rubra vera and paroxysmal nocturnal hemoglobinuria, dramatic responses to intravenous heparin therapy have been reported, although relapses have been common. 18,20,22 The prognosis of BCS with nonsurgical therapy is poor. In his large collective review in 1959, Parker 7 wrote, "The majority of cases have proved fatal and there is little evidence to suggest that recovery from occlusion of the major hepatic veins occurs once symptoms have been produced."…”

Section: Discussionmentioning

confidence: 99%

A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome

Orloff¹,

Daily²,

Orloff³

et al. 2000

Annals of Surgery

150

104

View full text Add to dashboard Cite

ObjectiveTo determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. Summary Background DataExperiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. MethodsFrom 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation.

show abstract

Section: Discussionmentioning

confidence: 99%

A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome

Orloff¹,

Daily²,

Orloff³

et al. 2000

Annals of Surgery

150

104

View full text Add to dashboard Cite

show abstract

“…Abnormalities associated with PNH include abnormal interaction of platelets with complement [40], hepatic venous thrombosis [82], thrombocytopenia, and leukopenia. It has been reported that normal human platelets and lymphocytes possess membrane-associated decay accelerating activity which is inhibitable by antibodies to DAF [129].…”

Section: Other Ceilsmentioning

confidence: 99%

The alternative pathway of complement

Pangburn

Müller‐Eberhard

1984

Springer Semin Immunopathol

254

120

View full text Add to dashboard Cite

“…2 –5 In various series, 15% to 40% of patients with PNH had hepatic venous obstruction. 1,3,4,6 –8 However, literature on PNH being diagnosed in primary thrombotic presentation is scarce. Most current data stem from retrospective series and case reports.…”

Section: Introductionmentioning

confidence: 99%

Large Clones With PNH-Type Phenotype are Not Common in Patients Presenting With Intra-Abdominal Thrombosis—A Prospective Study

Baloda

Ahluwalia

Varma

et al. 2012

Clin Appl Thromb Hemost

View full text Add to dashboard Cite

Intra-abdominal thrombosis is a complication of paroxysmal nocturnal hemoglobinuria (PNH). There is scarcity of data on cases presenting with thrombosis in whom PNH is the predisposing factor. We assessed the role of PNH defect in 81 patients with intra-abdominal thrombosis, 44 patients of Budd Chiari syndrome and 37 patients of extra hepatic venous obstruction. Flowcytometry with glycosylphosphatidyl inositol-anchored proteins (GPI-AP)-CD55, -CD59, and -CD16 was performed on all patients and controls to assess the prevalence of deficiencies and PNH-type phenotype clone size. Deficiencies of individual GPI-AP were seen in 17.3% cases versus 3.4% controls. This was due to CD55 deficiency on red blood cells and CD16 deficiency on the granulocytes. Deficiency of multiple GPI-APs was less frequent (3.7% cases). Data of this study indicate that the PNH defect as detected with CD55, CD59, and CD16 is not an important cause of intra-abdominal thrombosis in northwestern India.

show abstract

The Budd‐chiari Syndrome in Paroxysmal Nocturnal Haemoglobinuria—revisited

Cited by 12 publications

References 3 publications

A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome

A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome

The alternative pathway of complement

Large Clones With PNH-Type Phenotype are Not Common in Patients Presenting With Intra-Abdominal Thrombosis—A Prospective Study

Contact Info

Product

Resources

About