S2660 A Case of Hepatopulmonary Syndrome Requiring Living Donor Liver Transplantation in a Patient With Nodular Regenerative Hyperplasia and Autoimmune Lymphoproliferative Syndrome-Associated Granulomatous Hepatitis Without Cirrhosis

Johnson, Guylda; Huber, Aaron R; Levstik, Mark; Laryea, Marie

doi:10.14309/01.ajg.0000784172.66947.2f

Cited by 2 publications

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“…Of the 87 full-text studies reviewed, 29 were included for data extraction. These included 17 studies pertaining to NRH in the native liver resulting in liver transplant or listing for liver transplant (6,7,(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29), and 11 studies pertaining to the development of NRH after liver transplantation (30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40).…”

Section: Search Resultsmentioning

confidence: 99%

Nodular regenerative hyperplasia and liver transplantation: a systematic review

Biesterveld,

Schroder,

Hitchcock

et al. 2023

Front. Transplant.

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Nodular regenerative hyperplasia (NRH) is a primary disease of the liver that may cause noncirrhotic portal hypertension. Common causes include autoimmune, hematologic, immune deficiency, and myeloproliferative disorders. Given the limited data regarding the development of NRH in contemporary immunosuppressive protocols and the occurrence of NRH post-liver transplantation, we systematically reviewed NRH as it pertains to liver transplantation. We performed a comprehensive search for NRH and transplantation. Nineteen studies were identified with relevant data for NRH as an indication for a liver transplant. Thirteen studies were identified with relevant data pertaining to NRH development after liver transplant. Pooled analysis revealed 0.9% of liver transplant recipients had NRH. A total of 113 patients identified with NRH underwent liver transplantation. Most series report transplants done after the failure of endoscopic banding and TIPS management of portal hypertension. Reported 5-year graft and patient survival ranged from 73%–78% and 73%–90%. The pooled incidence of NRH after liver transplant for all indications was 2.9% and caused complications of portal hypertension. Complications related to portal hypertension secondary to NRH are a rare indication for a liver transplant. NRH can develop at any time after liver transplantation often without an identifiable cause, which may lead to portal hypertension requiring treatment or even re-transplantation.

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Section: Search Resultsmentioning

confidence: 99%

Nodular regenerative hyperplasia and liver transplantation: a systematic review

Biesterveld,

Schroder,

Hitchcock

et al. 2023

Front. Transplant.

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Hepatopulmonary Syndrome in a Patient With Autoimmune Hepatitis Without Liver Cirrhosis: A Case Report

Tarantinos,

Georgakopoulou,

Nella

et al. 2024

Cureus

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Intrapulmonary vasodilation leads to impaired arterial oxygenation, a hallmark of hepatopulmonary syndrome (HPS), a common pulmonary complication in end-stage liver disease. We present a case of HPS primarily diagnosed due to orthodeoxia in a 62-year-old ex-smoker with autoimmune hepatitis, under immunosuppressive treatment, but without liver cirrhosis. The patient reported dyspnea at rest that improved when supine. A recent chest CT scan showed no pulmonary embolism but indicated small nodules, bronchiectasis, and emphysema lesions. Functional breath monitoring revealed a low diffusing capacity for carbon monoxide (48% predicted). Blood gas analysis showed an increased alveolar-arterial difference, and contrast-enhanced echocardiography confirmed HPS with bubbles in the left heart chambers after the fourth cardiac cycle. Lung perfusion scintigraphy was negative for thromboembolic disease, but kidney imaging reinforced the HPS diagnosis. Our case is, to the best of our knowledge, the first presentation of HPS in a patient with autoimmune hepatitis without evidence of liver cirrhosis. This case highlights a rare instance of HPS in a patient with autoimmune hepatitis without liver cirrhosis, where orthodeoxia was the first clinical manifestation.

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S2660 A Case of Hepatopulmonary Syndrome Requiring Living Donor Liver Transplantation in a Patient With Nodular Regenerative Hyperplasia and Autoimmune Lymphoproliferative Syndrome-Associated Granulomatous Hepatitis Without Cirrhosis

Cited by 2 publications

References 0 publications

Nodular regenerative hyperplasia and liver transplantation: a systematic review

Nodular regenerative hyperplasia and liver transplantation: a systematic review

Hepatopulmonary Syndrome in a Patient With Autoimmune Hepatitis Without Liver Cirrhosis: A Case Report

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