دورية أكاديمية
Fatty Liver and Autoimmune Hepatitis: Two Forms of Liver Involvement in Lipodystrophies ; Fígado gordo e hepatite autoimune: duas formas de atingimento hepático nas lipodistrofias
| العنوان: | Fatty Liver and Autoimmune Hepatitis: Two Forms of Liver Involvement in Lipodystrophies ; Fígado gordo e hepatite autoimune: duas formas de atingimento hepático nas lipodistrofias |
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| المؤلفون: | Ribeiro, A., Brandão, J., Cleto, Esmeralda, Santos, M., Borges, T., Santos Silva, E. |
| بيانات النشر: | Karger Publishers Open Access |
| سنة النشر: | 2019 |
| المجموعة: | Repositório Científico do Centro Hospitalar do Porto |
| مصطلحات موضوعية: | Acquired generalized lipodystrophy, Acquired partial lipodystrophy, Autoimmune hepatitis, Fatty liver, Lipodystrophy, Lipodistrofia, Lipodistrofia generalizada adquirida, ipodistrofia parcial adquirida, Fígado gordo, Hepatite autoimune |
| الوصف: | Introduction: Lipodystrophies are a heterogeneous group of rare diseases (genetic or acquired) characterized by a partial or generalized deficit of adipose tissue, resulting in less energy storage capacity. They are associated with severe endocrine-metabolic complications with significant morbidity and mortality. In the pathogenesis of the acquired forms, immunological disorders may be involved. Case 1: A 13-year-old female was diagnosed with acquired generalized lipodystrophy and observed for suspicion of portal hypertension. She presented with generalized absence of adipose tissue, cervical and axillary acanthosis nigricans, and massive hepatosplenomegaly. Laboratory tests revealed AST 116 IU/L, ALT 238 IU/L, GGT 114 IU/L, HOMA-IR 28.2, triglycerides 491 mg/L, and leptin < 0.05 ng/mL. Upper gastrointestinal endoscopy saw no signs of portal hypertension. Hepatic histology showed macrovesicular fatty infiltration (60% of hepatocytes) and advanced fibrosis/cirrhosis. Her clinical condition worsened progressively to diabetes requiring treatment with subcutaneous insulin and hepatopulmonary syndrome. Case 2: A 15-year-old female, diagnosed with acquired partial lipodystrophy, Parkinson syndrome, autoimmune thyroiditis, and autoimmune thrombocytopenia was observed for hypertransaminasemia since the age of 8 years. She had absence of subcutaneous adipose tissue in the upper and lower limbs and ataxia. Laboratory tests showed AST 461 IU/L, ALT 921 IU/L, GGT 145 IU/L, HOMA-IR 32.6, triglycerides 298 mg/dL, normal leptin levels, platelets 84,000/μL, IgG 1,894 mg/dL, positive anti-LKM and anti-LC-1. Hepatic histology was suggestive of autoimmune hepatitis, without steatosis. She progressed favorably under metformin and immunosuppressive treatment. Conclusion: Early recognition and adequate characterization of liver disease in lipodystrophies is essential for a correct treatment approach. In acquired generalized lipodystrophy, the severe endocrine-metabolic disorder, which leads to steatohepatitis with cirrhotic ... |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 2341-4545 |
| العلاقة: | https://www.karger.com/Article/FullText/495767Test; Ribeiro A, Brandão JR, Cleto E, Santos M, Borges T, Santos Silva E. Fatty Liver and Autoimmune Hepatitis: Two Forms of Liver Involvement in Lipodystrophies. GE Port J Gastroenterol. 2019;26(5):362‐369. doi:10.1159/000495767; http://hdl.handle.net/10400.16/2387Test |
| DOI: | 10.1159/000495767 |
| الإتاحة: | https://doi.org/10.1159/000495767Test http://hdl.handle.net/10400.16/2387Test |
| حقوق: | openAccess ; http://creativecommons.org/licenses/by-nc-nd/4.0Test/ |
| رقم الانضمام: | edsbas.3944A2E5 |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 23414545 |
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| DOI: | 10.1159/000495767 |