AUDIT OF THE CLINICAL MANAGEMENT OF CHILDREN WITH AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE

A RAMANAYAKE 1, H MCCARTHY 1,2,3,  A DURKAN A2,3

1School of Women’s and Children’s Health, University of NSW, Randwick, Australia , 2Faculty of Medicine and Health, University of Sydney, Sydney, Australia, 3The Children’s Hospital at Westmead, Westmead, Australia

Aim: An audit of the management of children with autosomal dominant polycystic kidney disease (ADPKD) at the Sydney Children’s Hospital Network against recently published international consensus guidelines.

Background: The management of ADPKD in children to date has been physician dependent.  The new guidelines provide a framework for surveillance however, it is unclear how closely they are adhered to within NSW.

Methods: A retrospective case notes review of all children with definite or probable ADPKD was performed to extract data on the recommendations from the international consensus guidelines, with information supplemented by an ongoing questionnaire of the parents of children with ADPKD.

Results: Of 49 patients, (mean age 10.5 years), identified across 2 major paediatric hospitals, 42.9% were female and 75.5% had a confirmed family history of ADPKD. Annual office blood pressure measurements were recorded in 69.4% (n=34), whilst only 6.5% (n=2 of 31 patients aged over 5 years) had had an ambulatory blood pressure measurement (ABPM). Annual monitoring for albuminuria occurred in 81.6% (n=40). Compared to the recommendation for ultrasound only every 3 years in asymptomatic children, the majority (93.5%; n=29) had serial scans between every 6 months and 2 years. Some form of genetic counselling had been offered to 51% (n=25) of families. Data regarding lifestyle interventions and psychosocial aspects was generally not available through record review.

Conclusion: This audit highlights practices that follow the international consensus recommendations, and others that could be improved. Serial ultrasounds can be less frequent in asymptomatic patients while ABPM should be more readily offered. Genetic counselling resources could be developed for this large cohort. This information has been disseminated to the clinical teams to enable change in practice.


Biography:

I am a fourth year medical student at the University of New South Wales. I am currently completing my Honours research year at the St Vincent’s Hospital in Darlinghurst.

 

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