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[Multicystic kidney dysplasia]
F Krull1, P F Hoyer, R Habenicht
1Abteilung für Pädiatrische Nephrologie, Medizinische Hochschule Hannover.
Insights
Multicystic kidney dysplasia in children is often diagnosed prenatally or in early childhood. Prognosis is linked to co-occurring renal and other malformations.
Area of Science:
- Pediatric Nephrology
- Congenital Anomalies
- Medical Imaging
Context:
- Retrospective analysis of 48 children with multicystic kidney dysplasia (MKD) treated between 1976-1989.
- MKD diagnosis occurred prenatally via ultrasound or postnatally up to 12 years of age.
- No familial occurrence noted, but other renal diseases observed in 8 families.
Purpose:
- To analyze the clinical course and outcomes of pediatric multicystic kidney dysplasia.
- To identify presenting symptoms and associated malformations in children with MKD.
- To evaluate the impact of associated conditions on patient prognosis.
Summary:
- 48 children (27 boys, 21 girls) with MKD analyzed.
- Presenting symptoms included abdominal mass, UTIs, and vomiting.
- Associated malformations found in 18 patients (cardiac, other kidney dysplasia, ureter obstruction).
- Normal renal function (serum creatinine) and no hypertension in patients without associated kidney malformations.
- 42 patients underwent surgical removal of the affected kidney.
Impact:
- Highlights the importance of prenatal diagnosis and early detection of MKD.
- Emphasizes the significant role of associated malformations in determining the prognosis of MKD.
- Provides insights into the long-term clinical course and management of pediatric MKD.
Abstract:
The clinical course of 48 children (27 boys and 21 girls) with multicystic kidney dysplasia was analysed retrospectively. The patients were seen and treated at the Children's Hospital of Medical School Hannover between 1976 to 1989. There was no familial occurrence of the disease, yet in eight families various other renal diseases could be observed. In 20 patients the diagnosis of multicystic renal dysplasia was presumed by prenatal sonographic examination, in the other patients the diagnosis was established at the age between 1 day to 12 years. The first manifestations were palpable abdominal mass (n = 16), urinary tract infection (n = 4), casually because of a sonographic evaluation for other reasons (n = 4) and vomiting (n = 2). Associated malformations were found in 18 patients: cardiac malformations (n = 6), dysplasia of the other kidney (n = 5), ureter obstruction of the other kidney (n = 3), horseshoe kidney (n = 1) and others (n = 3). There was no hypertension and serum creatinine levels were normal in those children, who did not suffer from associated malformations of the other kidney. The multicystic kidney was removed operatively in 42 patients at the age of 3 days to 9.5 years (median 4 weeks). The prognosis of multicystic kidney dysplasia depends on associated renal and other malformations.