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Cystic renal disease and cardiovascular anomalies
1Department of Human Genetics, University of Manitoba, Winnipeg, Canada.
Insights
Renal cysts in children are linked to cardiovascular defects, especially unilateral multicystic dysplasia. Early diagnosis and monitoring are crucial for managing these complex congenital conditions.
Area of Science:
- Pediatric Nephrology
- Congenital Malformations
- Medical Genetics
Background:
- Renal cysts are a common, heterogeneous group of disorders often diagnosed prenatally.
- A previously suggested association between cystic kidneys and cardiovascular malformations warrants further investigation.
Purpose of the Study:
- To investigate the association between renal cysts and associated anomalies in children.
- To determine the incidence of cardiovascular defects in children with renal cysts.
Main Methods:
- A population-based study of children born in Manitoba between 1979 and 1983.
- Identification of 46 children with renal cysts from multiple sources.
- Analysis of associated anomalies, focusing on cardiovascular defects.
Main Results:
- Renal cysts occurred in 1 of 1,824 births; common types included multicystic dysplasia, cystic dysplasia due to lower urinary tract obstruction, and infantile polycystic kidney disease.
- 72% of cases had other anomalies, with 33% having cardiovascular anomalies.
- A strong association (P < 0.001) was confirmed between renal cysts and cardiovascular defects, particularly in unilateral multicystic dysplasia (28% of cases).
Conclusions:
- Renal cysts are significantly associated with cardiovascular defects in children.
- Unilateral multicystic dysplasia with a normal contralateral kidney generally has a good prognosis, but associated cardiac defects can be severe.
- Prenatal diagnosis and comprehensive evaluation for associated anomalies are essential for managing pediatric renal cystic diseases.
Abstract:
Renal cysts are a relatively common but heterogeneous group of disorders that are increasingly being diagnosed prenatally. Prognosis depends on the type of cystic disease, extent of involvement, and the presence or absence of other anomalies. Czeizel (American Journal of Medical Genetics [Supplement] 2:17-21, 1986) indicated that a cystic kidney-cardiovascular malformation association exists. To investigate this further a population based study of renal cysts and associated anomalies in Manitoba children born between 1979 and 1983 was carried out. A total of 46 children with renal cysts were identified from multiple sources (1 per 1,824 total births). The most common types were multicystic dysplasia (1/3,226), cystic dysplasia due to lower urinary tract obstruction (1/13,932), and infantile polycystic kidney disease (1/20,973). Other anomalies were seen in 72% of cases and 33% had cardiovascular anomalies. A further 11% had patent ductus arteriosus or single umbilical artery. The incidence of cardiovascular defects excluding patent ductus arteriosus or single umbilical artery was 1/174 in the total birth cohort and our data confirmed a strong association of renal cysts with cardiovascular defects in children with malformations (P less than 0.001). The association of cardiac defects with unilateral multicystic dysplasia is especially important. Of the 14 children ascertained with unilateral dysplasia, 4 (28%) had major cardiac defects leading to early death in all instances. Only one of the 4 had additional defects. Unilateral multicystic dysplasia with a normal contralateral kidney usually has a good prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)