Associated anomalies in children with congenital solitary functioning kidney

Abstract

Congenital solitary functioning kidney (CSFK) is a relatively common renal malformation and in children is frequently complicated by anomalies of the ipsilateral genital organs and occasionally by anomalies of other organs. The aim of this study was to determine the incidence of associated urological, cardiac, gastrointestinal, hematological, neurological, skeletal, and other congenital malformations in children with CSFK. We retrospectively reviewed 87 consecutive cases of CSFK diagnosed at our hospital between 1995 and 2003. There were 45 boys and 42 girls, whose ages at diagnosis ranged from newborn to 16 years (mean 4.67+/-4.48 years). In all patients, CSFK was diagnosed by abdominal ultrasound and confirmed by radionuclide studies. In 46 patients (53%) the left kidney was absent, and in 41 patients (47%) the right kidney was absent. Overall associated anomalies were detected in 52 of the 87 children (60%) with CSFK. Urological anomalies were most common, with an incidence of 37% (32/87). Nonurological anomalies were detected in 38 children (44%) with CSFK; these included cardiac anomalies in 13, gastrointestinal anomalies in eight, hematological anomalies in five, neurological anomalies in three, and other organ anomalies in nine. In our study, more than half of the patients with CSFK had associated anomalies. For this reason we recommend abdominal ultrasound and voiding cystourethrogram for early recognition of urological anomalies and a careful physical examination for other organ anomalies in patients with CSFK.

Developmental programming of a reduced nephron endowment: more than just a baby's birth weight

“…Thus assessment of the relative importance of a reduced nephron endowment per se in the onset of adult disease is extremely difficult. In human patients with unilateral renal agenesis, in whom nephron endowment is reduced from birth, the incidence of renal insufficiency and proteinuria is common (2,61), and, in many cases, infants with one kidney also have contralateral renal abnormalities (18,35,62), the most common of which may further contribute to renal problems. Children with unilateral renal agenesis often develop elevated arterial pressure (73,95), although it is difficult to know whether this is directly due to a reduced nephron endowment.…”

Clinical characteristics and follow-up results of unilateral renal agenesis and hypoplasia of children

“…Konjenital böbrek anomalisi olan çocuklarda %10-50 oranında ürogenital sistem ve diğer sistemlere ait anormalikler eşlik edebilir (6,19,22) . Tek taraflı renal agenezi ve hipoplazi hastalarından oluşan bu çalışmada, 15 (%30) hastada ürogenital sistem anomalisi saptanırken, 7 (%28) hastada diğer sistemlere ait anomali mevcuttu.…”

Kidney, Urinary Bladder, and Ureter

“…Offspring and other relatives of individuals with congenital solitary kidney have a significantly increased incidence of renal disease (agenesis, dysplasia, cystic dysplasia) (McPherson 2007). A retrospective study of renal injury markers in children with SFK demonstrated that nearly one-third of the patients had hypertension or albuminuria or used renoprotective medication at a young age (Kaneyama et al 2004;Dursun et al 2005). We therefore emphasize that clinical follow-up of all children with an SFK is needed.…”