Hepatic Encephalopathy Is Not Always due to Liver Cirrhosis
Hepatic encephalopathy (HE) is a frequent and debilitating complication of liver disease and is oftentimes associated with hyperammonaemia. However, hyperammonaemia may occur in underlying conditions other than acute or chronic liver failure and clinical awareness is needed in order to be recognized...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2022-05-01
|
| Series: | Case Reports in Gastroenterology |
| Subjects: | |
| Online Access: | https://www.karger.com/Article/FullText/524551 |
| _version_ | 1818161510873038848 |
|---|---|
| author | Miriam Eva Ecker Maria Paparoupa Bernd Sostmann Karin Weissenborn Frank Schuppert |
| author_facet | Miriam Eva Ecker Maria Paparoupa Bernd Sostmann Karin Weissenborn Frank Schuppert |
| author_sort | Miriam Eva Ecker |
| collection | DOAJ |
| description | Hepatic encephalopathy (HE) is a frequent and debilitating complication of liver disease and is oftentimes associated with hyperammonaemia. However, hyperammonaemia may occur in underlying conditions other than acute or chronic liver failure and clinical awareness is needed in order to be recognized and treated properly. A 24-year-old woman presented herself to our emergency department with acute confusion and altered mental state due to severe hyperammonaemia. The patient was diagnosed in the age of 14 with ornithine transcarbamylase (OTC) deficiency and was incompliant regarding her diet and suggested medication to treat this disorder. She was treated with sodium benzoate 250 mg/kg i.v., sodium phenylbutyrate/sodium phenylacetate 250 mg/kg i.v., L-arginine 250 mg/kg i.v., and received continuous hemofiltration. Under simultaneous medical treatment and haemodialysis, ammonia levels dropped to normal within 24 h and symptomatic encephalopathy ceased completely. OTC deficiency is rare in adults, and the majority of patients are diagnosed in childhood. It can lead to death if not diagnosed and treated properly. Our case underlines the importance of considering causes of HE other than liver cirrhosis. |
| first_indexed | 2024-12-11T16:18:55Z |
| format | Article |
| id | doaj.art-500a7b4519b94e3599587187816ab45e |
| institution | Directory Open Access Journal |
| issn | 1662-0631 |
| language | English |
| last_indexed | 2024-12-11T16:18:55Z |
| publishDate | 2022-05-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Gastroenterology |
| spelling | doaj.art-500a7b4519b94e3599587187816ab45e2022-12-22T00:58:53ZengKarger PublishersCase Reports in Gastroenterology1662-06312022-05-0116231331910.1159/000524551524551Hepatic Encephalopathy Is Not Always due to Liver CirrhosisMiriam Eva Ecker0https://orcid.org/0000-0001-7116-7957Maria Paparoupa1https://orcid.org/0000-0003-1713-6759Bernd Sostmann2Karin Weissenborn3https://orcid.org/0000-0002-1157-8938Frank Schuppert4Department of Gastroenterology, Endocrinology, Diabetology and General Medicine, Klinikum Kassel, Kassel, GermanyDepartment of Intensive Care Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, GermanyGeneral Practitioner´s Private Practice, Melsungen, GermanyDepartment of Neurology, Hannover Medical School, Hannover, GermanyDepartment of Gastroenterology, Endocrinology, Diabetology and General Medicine, Klinikum Kassel, Kassel, GermanyHepatic encephalopathy (HE) is a frequent and debilitating complication of liver disease and is oftentimes associated with hyperammonaemia. However, hyperammonaemia may occur in underlying conditions other than acute or chronic liver failure and clinical awareness is needed in order to be recognized and treated properly. A 24-year-old woman presented herself to our emergency department with acute confusion and altered mental state due to severe hyperammonaemia. The patient was diagnosed in the age of 14 with ornithine transcarbamylase (OTC) deficiency and was incompliant regarding her diet and suggested medication to treat this disorder. She was treated with sodium benzoate 250 mg/kg i.v., sodium phenylbutyrate/sodium phenylacetate 250 mg/kg i.v., L-arginine 250 mg/kg i.v., and received continuous hemofiltration. Under simultaneous medical treatment and haemodialysis, ammonia levels dropped to normal within 24 h and symptomatic encephalopathy ceased completely. OTC deficiency is rare in adults, and the majority of patients are diagnosed in childhood. It can lead to death if not diagnosed and treated properly. Our case underlines the importance of considering causes of HE other than liver cirrhosis.https://www.karger.com/Article/FullText/524551hepatic encephalopathyliver cirrhosishyperammonaemialiver failureornithine transcarbamylase deficiencyurea cycle enzymes |
| spellingShingle | Miriam Eva Ecker Maria Paparoupa Bernd Sostmann Karin Weissenborn Frank Schuppert Hepatic Encephalopathy Is Not Always due to Liver Cirrhosis Case Reports in Gastroenterology hepatic encephalopathy liver cirrhosis hyperammonaemia liver failure ornithine transcarbamylase deficiency urea cycle enzymes |
| title | Hepatic Encephalopathy Is Not Always due to Liver Cirrhosis |
| title_full | Hepatic Encephalopathy Is Not Always due to Liver Cirrhosis |
| title_fullStr | Hepatic Encephalopathy Is Not Always due to Liver Cirrhosis |
| title_full_unstemmed | Hepatic Encephalopathy Is Not Always due to Liver Cirrhosis |
| title_short | Hepatic Encephalopathy Is Not Always due to Liver Cirrhosis |
| title_sort | hepatic encephalopathy is not always due to liver cirrhosis |
| topic | hepatic encephalopathy liver cirrhosis hyperammonaemia liver failure ornithine transcarbamylase deficiency urea cycle enzymes |
| url | https://www.karger.com/Article/FullText/524551 |
| work_keys_str_mv | AT miriamevaecker hepaticencephalopathyisnotalwaysduetolivercirrhosis AT mariapaparoupa hepaticencephalopathyisnotalwaysduetolivercirrhosis AT berndsostmann hepaticencephalopathyisnotalwaysduetolivercirrhosis AT karinweissenborn hepaticencephalopathyisnotalwaysduetolivercirrhosis AT frankschuppert hepaticencephalopathyisnotalwaysduetolivercirrhosis |