Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Phenotypic variation and long-term outcome in children with congenital hepatic fibrosis.

Journal of pediatric gastroenterology and nutrition - 1 Aug 2013

Rawat Dinesh, Kelly Deirdre A, Milford David V, Sharif Khalid, Lloyd Carla, McKiernan Patrick J

Abstract excerpt

BACKGROUND AND OBJECTIVE: Congenital hepatic fibrosis (CHF) and Caroli syndrome are frequently associated with renal cystic diseases. They have a variable clinical course, and the natural history is not well defined despite molecular advances. Our study describes the clinical manifestations and long-term outcome in children with this disorder. METHODS: A retrospective case review of children with CHF at a single...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Genetic Diseases, Inborn
Share to Hypertension, Portal

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Kidney Failure, Chronic

Share to Kidney Transplantation

Share to Liver Cirrhosis

Share to Liver Transplantation

Share to Polycystic Kidney, Autosomal Recessive

Share to Renal Insufficiency, Chronic

Source

Identifiers

PMID: 23518487
DOI: 10.1097/MPG.0b013e318291e72b

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Phenotypic variation and long-term outcome in children with congenital hepatic fibrosis.PMID 23518487Read ↗

Hepatorenal fibrocystic diseases in children.Semantic proximity Read ↗

Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes.Semantic proximity Read ↗

Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease.Semantic proximity Read ↗

Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD).Semantic proximity Read ↗

Adult Congenital Hepatic Fibrosis and Autosomal Dominant Polycystic Kidney Disease with PAX2 and PKD1 Variants: A Case ReportSemantic proximity Read ↗

Clinical manifestations of autosomal recessive polycystic kidney diseaseSemantic proximity Read ↗

Rare variants in PKHD1 associated with Caroli syndrome: Two case reports.Semantic proximity Read ↗

Caroli disease, bilateral diffuse cystic renal dysplasia, situs inversus, postaxial polydactyly, and preauricular fistulas: a ciliopathy caused by a homozygous NPHP3 mutation.Semantic proximity Read ↗

Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis.Semantic proximity Read ↗

Biliary atresia.Semantic proximity Read ↗

Genetic contributors and modifiers of biliary atresia.Semantic proximity Read ↗

Identification of Polycystic Kidney Disease 1 Like 1 Gene Variants in Children With Biliary Atresia Splenic Malformation SyndromeSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.