scholarly journals Hereditary North American Indian childhood cirrhosis

2020 ◽  
Author(s):  
Keyword(s):  
American Indian ◽  
North American ◽  
Indian Childhood Cirrhosis ◽  
Indian Childhood ◽  
North American Indian

scholarly journals NOL11, implicated in the pathogenesis of North American Indian Childhood Cirrhosis, is required for pre‐rRNA transcription and processing

2013 ◽  
Vol 27 (S1) ◽  
Author(s):  
Susan Baserga ◽  
Emily Freed ◽  
Jose‐Luis Prieto ◽  
Kathleen McCann ◽  
Brian McStay
Keyword(s):  
American Indian ◽  
North American ◽  
Indian Childhood Cirrhosis ◽  
Rrna Transcription ◽  
Indian Childhood ◽  
North American Indian

scholarly journals p53-Mediated Biliary Defects Caused by Knockdown of cirh1a, the Zebrafish Homolog of the Gene Responsible for North American Indian Childhood Cirrhosis

PLoS ONE ◽  
2013 ◽  
Vol 8 (10) ◽  
pp. e77670 ◽  
Author(s):  
Benjamin J. Wilkins ◽  
Kristin Lorent ◽  
Randolph P. Matthews ◽  
Michael Pack
Keyword(s):  
American Indian ◽  
North American ◽  
Indian Childhood Cirrhosis ◽  
Indian Childhood ◽  
North American Indian

scholarly journals A219 EARLY PREDICTORS OF UNFAVORABLE OUTCOME IN NORTH AMERICAN INDIAN CHILDHOOD CIRRHOSIS

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 252-253
Author(s):  
L Khendek ◽  
F Alvarez ◽  
M Beaunoyer ◽  
E Drouin ◽  
M Lallier ◽  
...  
Keyword(s):  
Liver Disease ◽  
American Indian ◽  
North American ◽  
Bone Health ◽  
Unfavorable Outcome ◽  
Indian Childhood Cirrhosis ◽  
End Stage Liver Disease ◽  
Indian Childhood ◽  
North American Indian ◽  
End Stage

Abstract Background North American Indian Childhood Cirrhosis (NAIC) has only been described in the Cree-Ojibway First Nations of Northern Quebec. NAIC presents with transient neonatal jaundice and progresses to biliary cirrhosis often requiring liver transplantation (LT) in childhood. Only 30 patients have been described to date and risk factors associated with an earlier progression to LT have not yet been identified. Moreover, NAIC patients seem to experience more fractures than other cholestatic patients, but this has not been confirmed. Aims With this study, we aimed to identify predictors at 6 months from presentation that might suggest progression to end-stage liver disease as well as evaluate bone health in affected patients. Methods The records of all NAIC patients diagnosed between 2000–2020 were reviewed. Subjects were split into 2 groups based on whether they had undergone LT or not (No_LT) before age 18. Recorded complications included: hepatic encephalopathy (HE), variceal bleeding (VB), ascites, spontaneous bacterial peritonitis (SBP), bacteremia, and pulmonary shunts. Laboratory data (alanine aminotransferase, ALT; total bilirubin, TB) were collected at presentation and follow-up. Bone mineral density (BMD) of the lumbar spine (Z-scores) and number of fractures were compared between groups. NAIC patients were then compared to cohorts with other chronic cholestatic diseases such as biliary atresia (BA, n=24) and Alagille syndrome (AS, n=11). Results A total of 14 patients (M=9, F=5) were diagnosed with NAIC. Average age at presentation was 2.1 months (IQR 1–16.9 months), with 3 patients older than 18 months. Overall, 6 patients were transplanted (avg 8.6±1.7 years), one was listed for LT but died waiting, and 7 remained in a state of compensated cirrhosis. All complications were only observed in the LT group except for VB which also occurred in 2 patients of the No_LT group. Between presentation and 6 months, ALT and TB levels increased more in the LT vs No_LT group (p=ns). There was a greater variation of ALT/TB levels in the LT group (p=0.0047) even once the 3 patients with late referral were excluded (p=0.0381). No patient in the No_LT group had fractures, while 3 did in the LT group. BMD was lower in the LT group vs No_LT group (-2.2±1.2 vs. -1.1±1.3, p=ns). NAIC patients had lower BMD (-1.7±1.3) than those with AS (0.7±0.9, p=0.003) or BA (-0.9±1.4, p=ns) and had a higher prevalence of fractures (21.4% vs. 12.5% for BA and 18.2% for AS patients). Conclusions In patients with NAIC, variation of ALT/TB levels at 6 months from presentation may be used as an early predictor of unfavorable outcome and progression towards end-stage liver disease. Patients who evolved to LT had more complications, higher prevalence of fractures and lower BMD values. Compared to children with BA or AS, NAIC patients had poorer bone health. Funding Agencies None

Nucleolar localization of cirhin, the protein mutated in North American Indian childhood cirrhosis

2005 ◽  
Vol 311 (2) ◽  
pp. 218-228 ◽  
Author(s):  
Bin Yu ◽  
Grant A. Mitchell ◽  
Andrea Richter
Keyword(s):  
American Indian ◽  
North American ◽  
Indian Childhood Cirrhosis ◽  
Nucleolar Localization ◽  
Indian Childhood ◽  
North American Indian

scholarly journals Localization of a Recessive Gene for North American Indian Childhood Cirrhosis to Chromosome Region 16q22—and Identification of a Shared Haplotype

2000 ◽  
Vol 67 (1) ◽  
pp. 222-228 ◽  
Author(s):  
Christine Bétard ◽  
Andrée Rasquin-Weber ◽  
Carl Brewer ◽  
Eric Drouin ◽  
Suzanne Clark ◽  
...  
Keyword(s):  
American Indian ◽  
North American ◽  
Chromosome Region ◽  
Recessive Gene ◽  
Indian Childhood Cirrhosis ◽  
Shared Haplotype ◽  
Indian Childhood ◽  
North American Indian

scholarly journals NOL11, Implicated in the Pathogenesis of North American Indian Childhood Cirrhosis, Is Required for Pre-rRNA Transcription and Processing

PLoS Genetics ◽  
2012 ◽  
Vol 8 (8) ◽  
pp. e1002892 ◽  
Author(s):  
Emily F. Freed ◽  
José-Luis Prieto ◽  
Kathleen L. McCann ◽  
Brian McStay ◽  
Susan J. Baserga
Keyword(s):  
American Indian ◽  
North American ◽  
Indian Childhood Cirrhosis ◽  
Rrna Transcription ◽  
Indian Childhood ◽  
North American Indian

scholarly journals A Missense Mutation (R565W) in Cirhin (FLJ14728) in North American Indian Childhood Cirrhosis

2002 ◽  
Vol 71 (6) ◽  
pp. 1443-1449 ◽  
Author(s):  
Pierre Chagnon ◽  
Jacques Michaud ◽  
Grant Mitchell ◽  
Jocelyne Mercier ◽  
Jean-François Marion ◽  
...  
Keyword(s):  
American Indian ◽  
Missense Mutation ◽  
North American ◽  
Indian Childhood Cirrhosis ◽  
Indian Childhood ◽  
North American Indian

Stylistic Variety in North American Indian Music

1953 ◽  
Vol 6 (2) ◽  
pp. 160-168
Author(s):  
Bruno Nettl
Keyword(s):  
American Indian ◽  
North American ◽  
North American Indian ◽  
Indian Music

North American Indian Linguistic Families

1978 ◽  
Vol 1 (1) ◽  
pp. 28a-36
Keyword(s):  
American Indian ◽  
North American ◽  
North American Indian ◽  
Indian Linguistic
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