Variable phenotype in

CAKUT HNF1B MODY hypomagnesaemia pancreatic structural anomalies

Journal

Clinical kidney journal
ISSN: 2048-8505
Titre abrégé: Clin Kidney J
Pays: England
ID NLM: 101579321

Informations de publication

Date de publication:
Jun 2019
Historique:
received: 07 05 2018
entrez: 15 6 2019
pubmed: 15 6 2019
medline: 15 6 2019
Statut: epublish

Résumé

Mutations in This study reports the results of This study identified a pathogenic variant in 23 patients from 21 families. The most frequent finding was bilateral cystic dysplasia or hyperechogenic kidneys (87% of patients). Sixty percent of them also fulfilled the criteria for impaired glucose metabolism, and these were significantly older than those patients with an Renal anomalies found in patients with

Sections du résumé

BACKGROUND BACKGROUND
Mutations in
METHODS METHODS
This study reports the results of
RESULTS RESULTS
This study identified a pathogenic variant in 23 patients from 21 families. The most frequent finding was bilateral cystic dysplasia or hyperechogenic kidneys (87% of patients). Sixty percent of them also fulfilled the criteria for impaired glucose metabolism, and these were significantly older than those patients with an
CONCLUSIONS CONCLUSIONS
Renal anomalies found in patients with

Identifiants

pubmed: 31198537
doi: 10.1093/ckj/sfy102
pii: sfy102
pmc: PMC6543961
doi:

Types de publication

Journal Article

Langues

eng

Pagination

373-379

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Auteurs

Leire Madariaga (L)

Pediatric Nephrology Department, Cruces University Hospital, UPV/EHU, Barakaldo, Spain.
Biocruces Health Research Institute, Barakaldo, Spain.
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain.

Alejandro García-Castaño (A)

Biocruces Health Research Institute, Barakaldo, Spain.
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain.

Gema Ariceta (G)

Pediatric Nephrology Department, Vall d'Hebron University Hospital, Autonomous University of Barcelona UAB, Barcelona, Spain.

Rosa Martínez-Salazar (R)

Biocruces Health Research Institute, Barakaldo, Spain.
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain.
Centro de Investigación Biomédica en Red de Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), Madrid, Spain.

Aníbal Aguayo (A)

Biocruces Health Research Institute, Barakaldo, Spain.
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain.
Centro de Investigación Biomédica en Red de Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), Madrid, Spain.

Luis Castaño (L)

Pediatric Nephrology Department, Cruces University Hospital, UPV/EHU, Barakaldo, Spain.
Biocruces Health Research Institute, Barakaldo, Spain.
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain.
Centro de Investigación Biomédica en Red de Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), Madrid, Spain.

Classifications MeSH