ASPECTOS CLINICOS Y MOLECULARES DE LA ACIDOSIS TUBULAR RENAL DISTAL EN NIÑOS

Clinical and molecular aspects of distal renal tubular acidosis in children

Martine T. P. Besouw1 & Marc Bienias2 & Patrick Walsh3 & Robert Kleta1,5 & William G. van’t Hoff1 & Emma Ashton4 & Lucy Jenkins4 & Detlef Bockenhauer1,5

1 Department of Pediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK 2 Department of Pediatrics, Medical Faculty Carl Gustav Carus, Technical University Dresden, Dresden, Germany 3 Great North Children’s Hospital, Newcastle upon Tyne NHS Foundation Trust, Newcastle, UK 4 North East Thames Regional Genetics Service Laboratories, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK 5 Centre for Nephrology, University College London Institute of Child Health, London, UK

Pediatr Nephrol (2017) 32:987–996 DOI 10.1007/s00467-016-3573-4

RESUMEN: EL TRABAJO CONCLUYE QUE EL PRONOSTICO DE LOS NIÑOS CON ACIDOSIS TUBULAR RENAL DISTAL TRATADOS CON ÁLCALI ES BUENO, ALGO SORPRENDENTE ES QUE LA MAYORÍA DE LOS PACIENTES ESTUDIADOS PRESENTARON DISFUNCIÓN TUBULAR PROXIMAL EXPRESADA COMO FANCONI INCOMPLETO