Milestones in the discovery of Budd‐Chiari syndrome
| Autor: | Monica Pellone, Alberto Zanetto, Marco Senzolo |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
History
Pediatrics medicine.medical_specialty Polycythaemia medicine.medical_treatment Budd-Chiari Syndrome Liver transplantation Therapeutic approach Liver disease Portacaval Shunt Surgical vascular liver disease Humans Medicine portal vein thrombosis anticoagulation 19th Century Hepatology Portacaval Shunt Surgical business.industry portal hypertension Anticoagulants History 19th Century History 20th Century medicine.disease Budd-Chiari syndrome splanchnic thrombosis Liver Transplantation Portal vein thrombosis 20th Century Budd–Chiari syndrome Portal hypertension Syphilis business |
| Zdroj: | Liver International. 39:1180-1185 |
| ISSN: | 1478-3231 1478-3223 |
| DOI: | 10.1111/liv.14088 |
| Popis: | In 1845, George Budd published a brief report regarding three patients who developed an obstruction of the hepatic veins. The condition has never been reported before, and was related to sepsis and alcoholism. Fifty-three years later, Hans Chiari postulated that syphilis was causing the obstruction of the hepatic veins, and enriched the debate with clinical and pathological correlations. Following the hypothesis on the 'phlebitis obliterans', several authors proposed other pathophysiological explanations including congenital causes, chronic trauma and exogenous toxins. RG Parker, in 1959, first recognized the relationship between obstruction of hepatic veins and thrombophilic conditions such as polycythaemia vera, pregnancy and hormonal therapy. Based on that, anticoagulant treatment was attempted, but with unsatisfactory outcome. We need to wait until the mid 1980s to see a widespread adoption of anticoagulants, with a consequent improvement of patients' survival. The fear of haemorrhagic events in patients with liver disease discouraged this therapeutic approach, and other surgical interventions (mainly port-systemic shunts) were conceived, but with high morbidity and mortality. The first liver transplantation in 1976 and the first trans-jugular intra-hepatic porto-systemic shunt in 1993 represented two major cornerstones in the management of Budd-Chiari syndrome (BCS). Such progresses allowed modifying the treatment of BCS until the modern concept of stepwise therapy. The present review thoroughly reviews the major landmarks in the discovery, treatment and clinical management of patients with BCS. |
| Databáze: | OpenAIRE |
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