Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec
BACKGROUND: Progressive cholestasis of northwestern Quebec (PCNQ) is a rare and severe form of cirrhosis affecting children from Quebec’s First Nations. First described by our group in 1981 and historically named North American Indian childhood cirrhosis, such a condition often requires liver transp...
Published in: | Canadian Liver Journal |
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University of Toronto Press Inc. (UTPress)
2022
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Online Access: | http://dx.doi.org/10.3138/canlivj-2021-0033 https://canlivj.utpjournals.press/doi/pdf/10.3138/canlivj-2021-0033 |
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crunivtoronpr:10.3138/canlivj-2021-0033 2023-12-31T10:06:57+01:00 Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec Khendek, Léticia Diaz, Candice Drouin, Eric Lallier, Michel Alvarez, Fernando Paganelli, Massimiliano 2022 http://dx.doi.org/10.3138/canlivj-2021-0033 https://canlivj.utpjournals.press/doi/pdf/10.3138/canlivj-2021-0033 en eng University of Toronto Press Inc. (UTPress) Canadian Liver Journal volume 5, issue 3, page 402-410 ISSN 2561-4444 2561-4444 Religious studies Cultural Studies journal-article 2022 crunivtoronpr https://doi.org/10.3138/canlivj-2021-0033 2023-12-01T08:17:57Z BACKGROUND: Progressive cholestasis of northwestern Quebec (PCNQ) is a rare and severe form of cirrhosis affecting children from Quebec’s First Nations. First described by our group in 1981 and historically named North American Indian childhood cirrhosis, such a condition often requires liver transplantation during the pediatric age. This study aimed at suggesting a more culturally sensitive name for the disease and identifying early prognostic factors for an unfavourable outcome. METHODS: We retrospectively collected data of all 14 consecutive patients diagnosed with PCNQ over the last 20 years and compared children listed for liver transplant before 18 years of age (LT, n = 7) to those with milder disease progression (no-LT, n = 7). RESULTS: Compared with the no-LT group, LT children developed serious complications with an unusually high incidence of gastrointestinal bleeding. Over the first 12 months from presentation, a greater increase of alanine aminotransferase plasma levels, decrease of total bilirubin, and increase of alanine aminotransferase-to-total bilirubin ratio was observed in the LT group. Bone mineral density was lower in LT children independently of vitamin D levels. Patients with PCNQ showed poorer bone health than age-matched children with other cholestatic disorders. CONCLUSIONS: In the name of cultural sensitivity, PCNQ should be the preferred name for this condition. Variation of alanine aminotransferase and total bilirubin plasma levels over the first 12 months from presentation might be used for the early identification of children with PCNQ who are at higher risk of unfavourable outcomes. This might help optimize clinical management to populations that are underserved by health care services. Article in Journal/Newspaper First Nations University of Toronto Press (U Toronto Press - via Crossref) Canadian Liver Journal |
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University of Toronto Press (U Toronto Press - via Crossref) |
op_collection_id |
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English |
topic |
Religious studies Cultural Studies |
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Religious studies Cultural Studies Khendek, Léticia Diaz, Candice Drouin, Eric Lallier, Michel Alvarez, Fernando Paganelli, Massimiliano Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec |
topic_facet |
Religious studies Cultural Studies |
description |
BACKGROUND: Progressive cholestasis of northwestern Quebec (PCNQ) is a rare and severe form of cirrhosis affecting children from Quebec’s First Nations. First described by our group in 1981 and historically named North American Indian childhood cirrhosis, such a condition often requires liver transplantation during the pediatric age. This study aimed at suggesting a more culturally sensitive name for the disease and identifying early prognostic factors for an unfavourable outcome. METHODS: We retrospectively collected data of all 14 consecutive patients diagnosed with PCNQ over the last 20 years and compared children listed for liver transplant before 18 years of age (LT, n = 7) to those with milder disease progression (no-LT, n = 7). RESULTS: Compared with the no-LT group, LT children developed serious complications with an unusually high incidence of gastrointestinal bleeding. Over the first 12 months from presentation, a greater increase of alanine aminotransferase plasma levels, decrease of total bilirubin, and increase of alanine aminotransferase-to-total bilirubin ratio was observed in the LT group. Bone mineral density was lower in LT children independently of vitamin D levels. Patients with PCNQ showed poorer bone health than age-matched children with other cholestatic disorders. CONCLUSIONS: In the name of cultural sensitivity, PCNQ should be the preferred name for this condition. Variation of alanine aminotransferase and total bilirubin plasma levels over the first 12 months from presentation might be used for the early identification of children with PCNQ who are at higher risk of unfavourable outcomes. This might help optimize clinical management to populations that are underserved by health care services. |
format |
Article in Journal/Newspaper |
author |
Khendek, Léticia Diaz, Candice Drouin, Eric Lallier, Michel Alvarez, Fernando Paganelli, Massimiliano |
author_facet |
Khendek, Léticia Diaz, Candice Drouin, Eric Lallier, Michel Alvarez, Fernando Paganelli, Massimiliano |
author_sort |
Khendek, Léticia |
title |
Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec |
title_short |
Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec |
title_full |
Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec |
title_fullStr |
Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec |
title_full_unstemmed |
Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec |
title_sort |
early predictors of unfavourable outcome in progressive cholestasis of northwestern quebec |
publisher |
University of Toronto Press Inc. (UTPress) |
publishDate |
2022 |
url |
http://dx.doi.org/10.3138/canlivj-2021-0033 https://canlivj.utpjournals.press/doi/pdf/10.3138/canlivj-2021-0033 |
genre |
First Nations |
genre_facet |
First Nations |
op_source |
Canadian Liver Journal volume 5, issue 3, page 402-410 ISSN 2561-4444 2561-4444 |
op_doi |
https://doi.org/10.3138/canlivj-2021-0033 |
container_title |
Canadian Liver Journal |
_version_ |
1786839161790005248 |