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Natural history, risk factors, and outcome of hepatopulmonary syndrome in pediatric liver diseases

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Abstract

Background

Limited pediatric literature is available regarding hepatopulmonary syndrome (HPS) especially in subjects with biliary atresia (BA) despite its proven prognostic significance. Thus, we aimed to study the natural history, risk factors, and outcome of HPS in BA and other chronic liver disease (CLD) subjects.

Methods

All children (BA and other non-BA CLDs) older than 6 months of age were included in the study. HPS was diagnosed on the basis of standard international criteria. Also, fractional exhaled nitric oxide (FeNO) was measured at baseline.

Results

During the study period from January 2017 to December 2018, there were 42 children in BA and 62 in the CLD group. The overall prevalence of HPS was 42.3%: 57.1% in the BA group and 32.2% in the CLD group. Median age at HPS diagnosis was 14.4 months and 90 months in the BA and non-BA CLD groups, respectively. By the end of study period, the prevalence of HPS in the BA group further increased to 73.8% at 0.7% per month. Lower serum albumin (p < 0.05) in BA and higher splenic Z scores (p 0.013) in other CLDs were found to be significant risk factors for HPS. FeNO measurement did not reach diagnostic significance.

Conclusion

Prevalence of HPS is higher and also develops at an earlier age in the BA group compared to other CLDs. Also, risk of HPS development increases with increasing disease duration in BA. Lower serum albumin in BA and higher splenic Z scores in other CLDs may predict risk for HPS development.

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Author information

Authors and Affiliations

  1. Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110 070, India

    Snehavardhan Pandey, Vikrant Sood, Rajeev Khanna, Bikrant B. Lal & Seema Alam

  2. Department of Cardiology, Institute of Liver and Biliary Sciences, New Delhi, India

    Arun Kumar Sood

  3. Division of Pediatric Pulmonology and Intensive Care, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110 029, India

    S. K. Kabra

Authors
  1. Snehavardhan Pandey
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  2. Vikrant Sood
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  3. Rajeev Khanna
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  4. Bikrant B. Lal
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  5. Arun Kumar Sood
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  6. S. K. Kabra
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  7. Seema Alam
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Contributions

SA, AKS, and SK conceptualized and designed the work; SP, VS, BBL, and RK acquired, analyzed, and reviewed the data; AKS performed the echocardiography and SK helped in FeNO measurement; SP, VS, and BBL prepared first draft. SA, AKS, RK, and SK critically reviewed, revised, and approved the final version.

Corresponding author

Correspondence to Seema Alam.

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Conflict of interest

SP, VS, RK, BBL, AKS, SKK, and SA declare that they have no conflict of interest.

Ethics statement

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Approval for the study was obtained from Institutional Review Board.

Statement of informed consent

Informed consent was obtained from parents of all children included in the study.

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The authors are solely responsible for the data and the contents of the paper. In no way, the Honorary Editor-in-Chief, Editorial Board Members, or the printer/publishers are responsible for the results/findings and content of this article.

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Pandey, S., Sood, V., Khanna, R. et al. Natural history, risk factors, and outcome of hepatopulmonary syndrome in pediatric liver diseases. Indian J Gastroenterol 39, 66–74 (2020). https://doi.org/10.1007/s12664-020-01015-0

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  • DOI: https://doi.org/10.1007/s12664-020-01015-0

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