Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble”
Wilson's disease is one of the infrequent causes of hepatic failure with a wide clinical spectrum. Hepatic and neuropsychiatric manifestations are the most common, whereas atypical presentations were also reported as the complications of hepatic failure such as hepatopulmonary syndrome (HPS) an...
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| Format: | Article |
| Language: | English |
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Jaypee Brothers Medical Publisher
2022-01-01
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| Series: | Indian Journal of Respiratory Care |
| Subjects: | |
| Online Access: | http://www.ijrc.in/article.asp?issn=2277-9019;year=2022;volume=11;issue=2;spage=176;epage=180;aulast=Sagar |
| _version_ | 1827827455851757568 |
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| author | V V. S. S. Sagar Sourya Acharya Gajendra Agrawal Samarth Shukla Sunil Kumar Chitturi Venkata Sai Akhil |
| author_facet | V V. S. S. Sagar Sourya Acharya Gajendra Agrawal Samarth Shukla Sunil Kumar Chitturi Venkata Sai Akhil |
| author_sort | V V. S. S. Sagar |
| collection | DOAJ |
| description | Wilson's disease is one of the infrequent causes of hepatic failure with a wide clinical spectrum. Hepatic and neuropsychiatric manifestations are the most common, whereas atypical presentations were also reported as the complications of hepatic failure such as hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). These are considered pulmonary complications of hepatic disease with underlying complex pathology and various vasoactive substances such as endothelin 1 and nitric oxide on the pulmonary blood vessels. HPS usually presents with breathlessness and central cyanosis. The triad of HPS comprises dilatation of pulmonary vasculature, intrapulmonary shunting, and hypoxia, whereas POPH causes vasoconstriction and vascular remodeling leading to increased resistance in pulmonary blood vessels and subsequent right ventricular dysfunction. In this case report, we highlight two antagonistic pathological processes coexisting in the same patient. |
| first_indexed | 2024-03-12T03:30:43Z |
| format | Article |
| id | doaj.art-44fc6352831a49498f2d01fb4bfc0bdc |
| institution | Directory Open Access Journal |
| issn | 2277-9019 2321-4899 |
| language | English |
| last_indexed | 2024-03-12T03:30:43Z |
| publishDate | 2022-01-01 |
| publisher | Jaypee Brothers Medical Publisher |
| record_format | Article |
| series | Indian Journal of Respiratory Care |
| spelling | doaj.art-44fc6352831a49498f2d01fb4bfc0bdc2023-09-03T13:26:27ZengJaypee Brothers Medical PublisherIndian Journal of Respiratory Care2277-90192321-48992022-01-0111217618010.4103/ijrc.ijrc_150_21Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble”V V. S. S. SagarSourya AcharyaGajendra AgrawalSamarth ShuklaSunil KumarChitturi Venkata Sai AkhilWilson's disease is one of the infrequent causes of hepatic failure with a wide clinical spectrum. Hepatic and neuropsychiatric manifestations are the most common, whereas atypical presentations were also reported as the complications of hepatic failure such as hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). These are considered pulmonary complications of hepatic disease with underlying complex pathology and various vasoactive substances such as endothelin 1 and nitric oxide on the pulmonary blood vessels. HPS usually presents with breathlessness and central cyanosis. The triad of HPS comprises dilatation of pulmonary vasculature, intrapulmonary shunting, and hypoxia, whereas POPH causes vasoconstriction and vascular remodeling leading to increased resistance in pulmonary blood vessels and subsequent right ventricular dysfunction. In this case report, we highlight two antagonistic pathological processes coexisting in the same patient.http://www.ijrc.in/article.asp?issn=2277-9019;year=2022;volume=11;issue=2;spage=176;epage=180;aulast=Sagarendothelinhepatichypertensionhypoxianeuropsychiatric |
| spellingShingle | V V. S. S. Sagar Sourya Acharya Gajendra Agrawal Samarth Shukla Sunil Kumar Chitturi Venkata Sai Akhil Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble” Indian Journal of Respiratory Care endothelin hepatic hypertension hypoxia neuropsychiatric |
| title | Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble” |
| title_full | Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble” |
| title_fullStr | Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble” |
| title_full_unstemmed | Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble” |
| title_short | Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble” |
| title_sort | hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson s disease cirrhosis double trouble |
| topic | endothelin hepatic hypertension hypoxia neuropsychiatric |
| url | http://www.ijrc.in/article.asp?issn=2277-9019;year=2022;volume=11;issue=2;spage=176;epage=180;aulast=Sagar |
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