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Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach

Indian childhood cirrhosis is an entity believed to be on the verge of extinction. We present the case of a 13-month-old girl presenting acutely with jaundice, fever, and persistently increasing bilirubin. Investigations revealed direct hyperbilirubinemia, elevated transaminases, anemia, a blood wit...

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Autores principales: Gaur, K, Sakhuja, P, Mandal, RN, Kapoor, S
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5954805/
https://www.ncbi.nlm.nih.gov/pubmed/29692402
http://dx.doi.org/10.4103/jpgm.JPGM_359_17
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author Gaur, K
Sakhuja, P
Mandal, RN
Kapoor, S
author_facet Gaur, K
Sakhuja, P
Mandal, RN
Kapoor, S
author_sort Gaur, K
collection PubMed
description Indian childhood cirrhosis is an entity believed to be on the verge of extinction. We present the case of a 13-month-old girl presenting acutely with jaundice, fever, and persistently increasing bilirubin. Investigations revealed direct hyperbilirubinemia, elevated transaminases, anemia, a blood with few schistocytes, positive direct coombs test, and deranged prothrombin time. Viral, autoimmune, and metabolic workup was unremarkable. Ultrasonography showed chronic liver disease, portal hypertension, and ascites. Due to numerous confounding factors and a low index of suspicion, the diagnosis of Indian childhood cirrhosis remained elusive and was clinched only on liver biopsy, albeit more than three weeks later, shortly after which the child expired. The timing and technique of the liver biopsy may have profound impact on the ultimate clinical outcome. Close coordination between the clinical and pathological teams is essential for deciphering acute presentations where the etiology is uncertain. We highlight the clinical considerations, varied morphological pointers, and offer a diagnostic algorithm facilitating the consideration of this disease.
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spelling pubmed-59548052018-06-01 Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach Gaur, K Sakhuja, P Mandal, RN Kapoor, S J Postgrad Med Clinicopathological Forum Indian childhood cirrhosis is an entity believed to be on the verge of extinction. We present the case of a 13-month-old girl presenting acutely with jaundice, fever, and persistently increasing bilirubin. Investigations revealed direct hyperbilirubinemia, elevated transaminases, anemia, a blood with few schistocytes, positive direct coombs test, and deranged prothrombin time. Viral, autoimmune, and metabolic workup was unremarkable. Ultrasonography showed chronic liver disease, portal hypertension, and ascites. Due to numerous confounding factors and a low index of suspicion, the diagnosis of Indian childhood cirrhosis remained elusive and was clinched only on liver biopsy, albeit more than three weeks later, shortly after which the child expired. The timing and technique of the liver biopsy may have profound impact on the ultimate clinical outcome. Close coordination between the clinical and pathological teams is essential for deciphering acute presentations where the etiology is uncertain. We highlight the clinical considerations, varied morphological pointers, and offer a diagnostic algorithm facilitating the consideration of this disease. Medknow Publications & Media Pvt Ltd 2018 /pmc/articles/PMC5954805/ /pubmed/29692402 http://dx.doi.org/10.4103/jpgm.JPGM_359_17 Text en Copyright: © 2018 Journal of Postgraduate Medicine http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Clinicopathological Forum
Gaur, K
Sakhuja, P
Mandal, RN
Kapoor, S
Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach
title Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach
title_full Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach
title_fullStr Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach
title_full_unstemmed Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach
title_short Indian childhood cirrhosis – down but not out: Report of a rare case with a practical clinicopathological diagnostic approach
title_sort indian childhood cirrhosis – down but not out: report of a rare case with a practical clinicopathological diagnostic approach
topic Clinicopathological Forum
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5954805/
https://www.ncbi.nlm.nih.gov/pubmed/29692402
http://dx.doi.org/10.4103/jpgm.JPGM_359_17
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