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The spectrum of autosomal dominant polycystic kidney disease in children.

G M Fick, I T Duley, A M Johnson, J D Strain, M L Manco-Johnson and P A Gabow
JASN March 1994, 4 (9) 1654-1660;
G M Fick
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I T Duley
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A M Johnson
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J D Strain
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M L Manco-Johnson
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P A Gabow
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Abstract

The natural history of autosomal dominant polycystic kidney disease (ADPKD) has not been well described in children, and it is not known whether a relationship exists between renal structural abnormalities and function in children as has been seen in adults. Therefore, 140 children from 67 ADPKD families were studied in a prospective study. Only 22 children came with a previous diagnosis of ADPKD. In 44% of all children, at least one cyst was found on ultrasound at a mean age of 8.7 yr. Of these, 60% were classified as having moderate disease on the basis of a total cyst number of 1 to 10 cysts, whereas 40% were considered to have severe disease with a total of more than 10 cysts. There was a significant relationship between the severity of the renal structural involvement and the frequency of flank and back pain, hypertension, and impaired renal concentrating capacity. However, GFR were not reduced in children with ADPKD and did not relate to structural severity. Thirty-nine children were seen for a follow-up visit 2 to 5 yr after the initial visit. No child had progressed from nonaffected to affected with ADPKD, but three of four children with only one cyst at the time of the initial study had progressed to bilateral cysts. Among the 22 ADPKD children who had a follow-up study, there was progression of the disease, reflected by an increase in cyst number and an increase in the frequency of pain and hypertension. However, GFR remained stable in all children.(ABSTRACT TRUNCATED AT 250 WORDS)

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Journal of the American Society of Nephrology
Vol. 4, Issue 9
1 Mar 1994
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The spectrum of autosomal dominant polycystic kidney disease in children.
G M Fick, I T Duley, A M Johnson, J D Strain, M L Manco-Johnson, P A Gabow
JASN Mar 1994, 4 (9) 1654-1660;

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The spectrum of autosomal dominant polycystic kidney disease in children.
G M Fick, I T Duley, A M Johnson, J D Strain, M L Manco-Johnson, P A Gabow
JASN Mar 1994, 4 (9) 1654-1660;
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Cited By...

  • Prevalence of Hypertension in Children with Early-Stage ADPKD
  • Predictors of Autosomal Dominant Polycystic Kidney Disease Progression
  • Kidney Function and Plasma Copeptin Levels in Healthy Kidney Donors and Autosomal Dominant Polycystic Kidney Disease Patients
  • Vasopressin, Copeptin, and Renal Concentrating Capacity in Patients with Autosomal Dominant Polycystic Kidney Disease without Renal Impairment
  • Incompletely Penetrant PKD1 Alleles Mimic the Renal Manifestations of ARPKD
  • A Case for Water in the Treatment of Polycystic Kidney Disease
  • Volume Progression in Autosomal Dominant Polycystic Kidney Disease: The Major Factor Determining Clinical Outcomes
  • Hypertension in Autosomal-Dominant Polycystic Kidney Disease: Early Occurrence and Unique Aspects
  • Quantification and Longitudinal Trends of Kidney, Renal Cyst, and Renal Parenchyma Volumes in Autosomal Dominant Polycystic Kidney Disease
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