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hereditary North American Indian childhood cirrhosis (NAIC) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

hereditary North American Indian childhood cirrhosis

MONDO:0011497

Also known as:NAICNORTH American Indian childhood cirrhosis

Overview

Hereditary North American Indian childhood cirrhosis is a severe autosomal recessive intrahepatic cholestasis that has only been described in aboriginal children from northwestern Quebec. Manifesting first as transient neonatal jaundice, the disease evolves into periportal fibrosis and cirrhosis during a period ranging from childhood to adolescence.

GeneticsAutosomal recessive

Patient Orgs1 organizations · North American Menopause Society

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include: Portal hypertension, Biliary cirrhosis, and Prolonged neonatal jaundice.

Organ System	Phenotype Count	Example Features
Digestive system	2	Biliary cirrhosis, Prolonged neonatal jaundice
Heart and blood vessels

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 9:35 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Patient Support

Patient Advocacy Groups

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North American Menopause SocietyPrimary Focus

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Patient Advocacy Groups (PAGs) provide support, resources, and community for patients and caregivers.

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Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:604901

Orphanet

European rare disease database

Orphanet:168583

GARD

Genetic and Rare Diseases Info Center

GARD:17037

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
→Are there specialists or centers of excellence for this condition in my area?
→How can I connect with North American Menopause Society and other patient organizations for support?

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hereditary North American Indian childhood cirrhosis

Overview

Clinical Presentation

Prognosis

Patient Support

Patient Advocacy Groups

Medical References

External Resources

OMIM

Orphanet

GARD

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Questions for Your Care Team

Questions for Your Care Team

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