K Ferdinande, T Lamiroy, A Hoorens, X Verhelst, H Degroote, S Raevens, H Van Vlierberghe, A Geerts
{"title":"门静脉高压的一个不寻常的原因。","authors":"K Ferdinande, T Lamiroy, A Hoorens, X Verhelst, H Degroote, S Raevens, H Van Vlierberghe, A Geerts","doi":"10.51821/86.3.11925","DOIUrl":null,"url":null,"abstract":"A 66-year-old male with a medical history of coronary artery disease presented with nonspecific symptoms of anorexia, lethargy and an increase in abdominal girth. He was diagnosed 3 months ago with chronic liver disease of undetermined aetiology. He did not smoke and was a social drinker till 4 months ago. He was taking tests showed progressive cholestatic liver enzymes (total bilirubin 1,09 mg/dL, AST 130 U/L, ALT 129 U/L, GGT 517 U/L and ALP 641 U/L) and a severe hypoalbuminemia. Laboratory workup made a metabolic, autoimmune, and viral aetiology of the suspected cirrhosis less probable. Urinalysis showed a rapidly progressive proteinuria in the nephrotic range accompanied by the presence of serum spike of monoclonal lambda light Furosemide, Aldactone, Pantoprazole, Acetylcysteine and Atorvastatin. He had no personal or familial history of liver diseases. Physical examination revealed a tender but distended abdomen and large ascites was present. The laboratory chain protein on electrophoresis with immunofixation. Doppler echocardiography revealed a hypertrophic cardiomyopathy based on diffuse left and right ventricular hypertrophy with relative apical sparing. A diagnostic paracentesis was undertaken and the results were consistent with portal hypertension (SAAG 1,6 g/ dL). Considering the concomitant presence of hypertrophic cardiomyopathy, nephrotic syndrome and a presentation of portal hypertension of undetermined aetiology in presence of a monoclonal IgG lambda light chain spike, a transjugular liver biopsy (figure 1 and 2) was performed with HVPG measurement consistent with significant portal hypertension (15 mmHg).","PeriodicalId":7322,"journal":{"name":"Acta gastro-enterologica Belgica","volume":null,"pages":null},"PeriodicalIF":1.3000,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"An unusual cause of portal hypertension.\",\"authors\":\"K Ferdinande, T Lamiroy, A Hoorens, X Verhelst, H Degroote, S Raevens, H Van Vlierberghe, A Geerts\",\"doi\":\"10.51821/86.3.11925\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"A 66-year-old male with a medical history of coronary artery disease presented with nonspecific symptoms of anorexia, lethargy and an increase in abdominal girth. He was diagnosed 3 months ago with chronic liver disease of undetermined aetiology. He did not smoke and was a social drinker till 4 months ago. He was taking tests showed progressive cholestatic liver enzymes (total bilirubin 1,09 mg/dL, AST 130 U/L, ALT 129 U/L, GGT 517 U/L and ALP 641 U/L) and a severe hypoalbuminemia. Laboratory workup made a metabolic, autoimmune, and viral aetiology of the suspected cirrhosis less probable. Urinalysis showed a rapidly progressive proteinuria in the nephrotic range accompanied by the presence of serum spike of monoclonal lambda light Furosemide, Aldactone, Pantoprazole, Acetylcysteine and Atorvastatin. He had no personal or familial history of liver diseases. Physical examination revealed a tender but distended abdomen and large ascites was present. The laboratory chain protein on electrophoresis with immunofixation. Doppler echocardiography revealed a hypertrophic cardiomyopathy based on diffuse left and right ventricular hypertrophy with relative apical sparing. A diagnostic paracentesis was undertaken and the results were consistent with portal hypertension (SAAG 1,6 g/ dL). Considering the concomitant presence of hypertrophic cardiomyopathy, nephrotic syndrome and a presentation of portal hypertension of undetermined aetiology in presence of a monoclonal IgG lambda light chain spike, a transjugular liver biopsy (figure 1 and 2) was performed with HVPG measurement consistent with significant portal hypertension (15 mmHg).\",\"PeriodicalId\":7322,\"journal\":{\"name\":\"Acta gastro-enterologica Belgica\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.3000,\"publicationDate\":\"2023-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta gastro-enterologica Belgica\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.51821/86.3.11925\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta gastro-enterologica Belgica","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.51821/86.3.11925","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
A 66-year-old male with a medical history of coronary artery disease presented with nonspecific symptoms of anorexia, lethargy and an increase in abdominal girth. He was diagnosed 3 months ago with chronic liver disease of undetermined aetiology. He did not smoke and was a social drinker till 4 months ago. He was taking tests showed progressive cholestatic liver enzymes (total bilirubin 1,09 mg/dL, AST 130 U/L, ALT 129 U/L, GGT 517 U/L and ALP 641 U/L) and a severe hypoalbuminemia. Laboratory workup made a metabolic, autoimmune, and viral aetiology of the suspected cirrhosis less probable. Urinalysis showed a rapidly progressive proteinuria in the nephrotic range accompanied by the presence of serum spike of monoclonal lambda light Furosemide, Aldactone, Pantoprazole, Acetylcysteine and Atorvastatin. He had no personal or familial history of liver diseases. Physical examination revealed a tender but distended abdomen and large ascites was present. The laboratory chain protein on electrophoresis with immunofixation. Doppler echocardiography revealed a hypertrophic cardiomyopathy based on diffuse left and right ventricular hypertrophy with relative apical sparing. A diagnostic paracentesis was undertaken and the results were consistent with portal hypertension (SAAG 1,6 g/ dL). Considering the concomitant presence of hypertrophic cardiomyopathy, nephrotic syndrome and a presentation of portal hypertension of undetermined aetiology in presence of a monoclonal IgG lambda light chain spike, a transjugular liver biopsy (figure 1 and 2) was performed with HVPG measurement consistent with significant portal hypertension (15 mmHg).
期刊介绍:
The Journal Acta Gastro-Enterologica Belgica principally publishes peer-reviewed original manuscripts, reviews, letters to editors, book reviews and guidelines in the field of clinical Gastroenterology and Hepatology, including digestive oncology, digestive pathology, as well as nutrition. Pure animal or in vitro work will not be considered for publication in the Journal. Translational research papers (including sections of animal or in vitro work) are considered by the Journal if they have a clear relationship to or relevance for clinical hepato-gastroenterology (screening, disease mechanisms and/or new therapies). Case reports and clinical images will be accepted if they represent an important contribution to the description, the pathogenesis or the treatment of a specific gastroenterology or liver problem. The language of the Journal is English. Papers from any country will be considered for publication. Manuscripts submitted to the Journal should not have been published previously (in English or any other language), nor should they be under consideration for publication elsewhere. Unsolicited papers are peer-reviewed before it is decided whether they should be accepted, rejected, or returned for revision. Manuscripts that do not meet the presentation criteria (as indicated below) will be returned to the authors. Papers that go too far beyond the scope of the journal will be also returned to the authors by the editorial board generally within 2 weeks. The Journal reserves the right to edit the language of papers accepted for publication for clarity and correctness, and to make formal changes to ensure compliance with AGEB’s style. Authors have the opportunity to review such changes in the proofs.