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Article

PubMed

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Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

The Journal of pediatrics. 2018 Aug ; 199 () : 22-28.e6. [epub] 20180509

J Pediatr
ISSN 1097-6833 | 0022-3476
Source

Language English Country United States Media print-electronic

Document type Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't

Persistent link https://www.medvik.cz/link/pmid29753540

PubMed 29753540
DOI 10.1016/j.jpeds.2018.03.052
PII: S0022-3476(18)30424-4
Knihovny.cz E-resources

Keywords
PKHD1, ciliopathy, oligohydramnios, renal replacement therapy,
MeSH
Time Factors MeSH
Renal Dialysis * MeSH
Risk Assessment * MeSH
Infant MeSH
Humans MeSH
Follow-Up Studies MeSH
Infant, Newborn MeSH
Polycystic Kidney, Autosomal Recessive diagnosis therapy MeSH
Prospective Studies MeSH
Retrospective Studies MeSH
Risk Factors MeSH
Pregnancy MeSH
Ultrasonography, Prenatal MeSH
Check Tag
Infant MeSH
Humans MeSH
Male MeSH
Infant, Newborn MeSH
Pregnancy MeSH
Female MeSH
Publication type
Journal Article MeSH
Multicenter Study MeSH
Research Support, Non-U.S. Gov't MeSH

OBJECTIVE: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. STUDY DESIGN: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. RESULTS: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. CONCLUSIONS: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.

1st Department of Pediatrics Hippokration Hospital Aristotle University of Thessaloniki Thessaloniki Greece

Bioscientia Center for Human Genetics Ingelheim Germany; Renal Division Department of Medicine University Freiburg Medical Center Freiburg Germany

Clinic for Children and Adolescents Hospital St Georg Leipzig Germany

Clinic of Children Diseases Institute of Clinical Medicine Vilnius University Vilnius Lithuania

Department of General Pediatrics and Hematology Oncology Children's University Hospital Tuebingen Germany

Department of General Pediatrics University Hospital Muenster Muenster Germany

Department of Inherited and Acquired Kidney Diseases Research Clinical Institute for Pediatrics Pirogov Russian National Research Medical University Moscow Russia

Department of Internal Medicine 1 University Hospital of the RWTH Aachen Aachen Germany

Department of Nephrology and Hypertension of Children and Adolescents Medical University of Gdansk Gdansk Poland

Department of Paediatrics and Nephrology Medical University of Bialystok Bialystok Poland

Department of Pediatric Kidney Liver and Metabolic Diseases Hannover Medical School Hannover Germany

Department of Pediatric Nephrology Ali Asghar Children Hospital Iran University of Medical Sciences Tehran Iran