Upper-tract genitourinary malformations in girls with congenital adrenal hyperplasia

Research output: Contribution to journalArticle

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Abstract

OBJECTIVE. The objective of this study was to determine the incidence, specific abnormalities, and clinical significance of upper-tract genitourinary malformations in girls with congenital adrenal hyperplasia. METHODS. Medical charts of children who received a diagnosis of congenital adrenal hyperplasia between 1985 and 2005 were reviewed. RESULTS. Of 107 patients with congenital adrenal hyperplasia, 66 girls were identified. Of these, 14 were found to have upper-tract genitourinary abnormalities. The anomalies consisted of vesicoureteral reflux grades 1 to 3 (9), hydronephrosis (4), and duplicated collecting system (1). Two boys with congenital adrenal hyperplasia and known upper-tract genitourinary anomalies were also identified. One had ureteropelvic junction obstruction, and the other had a left malrotated kidney with hydronephrosis. Four patients (1 boy, 3 girls) underwent surgery that consisted of ureteral reimplantation (2) and ureteral stent placement (2). The reflux eventually resolved in 3 patients and worsened in 1. Hydronephrosis improved in 1 patient. None of the patients developed any impairment in renal function. CONCLUSION. The incidence of upper-tract genitourinary anomalies in girls with congenital adrenal hyperplasia is much higher in our cohort than in the general population (21.2% vs 2%-4%). This association represents a potentially significant medical issue because some patients may require surgical intervention and antibiotic prophylaxis to prevent urinary tract infections and impairment in renal function.

Original languageEnglish
JournalPediatrics
Volume120
Issue number2
DOIs
StatePublished - Aug 2007

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Congenital Adrenal Hyperplasia
Hydronephrosis
Kidney
Urogenital Abnormalities
Vesico-Ureteral Reflux
Antibiotic Prophylaxis
Replantation
Incidence
Urinary Tract Infections
Stents
Population

Keywords

  • Congenital adrenal hyperplasia
  • Genitourinary malformations
  • Ureteral reimplantation
  • Vesicoureteral reflux

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Upper-tract genitourinary malformations in girls with congenital adrenal hyperplasia. / Nabhan, Zeina; Eugster, Erica.

In: Pediatrics, Vol. 120, No. 2, 08.2007.

Research output: Contribution to journalArticle

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abstract = "OBJECTIVE. The objective of this study was to determine the incidence, specific abnormalities, and clinical significance of upper-tract genitourinary malformations in girls with congenital adrenal hyperplasia. METHODS. Medical charts of children who received a diagnosis of congenital adrenal hyperplasia between 1985 and 2005 were reviewed. RESULTS. Of 107 patients with congenital adrenal hyperplasia, 66 girls were identified. Of these, 14 were found to have upper-tract genitourinary abnormalities. The anomalies consisted of vesicoureteral reflux grades 1 to 3 (9), hydronephrosis (4), and duplicated collecting system (1). Two boys with congenital adrenal hyperplasia and known upper-tract genitourinary anomalies were also identified. One had ureteropelvic junction obstruction, and the other had a left malrotated kidney with hydronephrosis. Four patients (1 boy, 3 girls) underwent surgery that consisted of ureteral reimplantation (2) and ureteral stent placement (2). The reflux eventually resolved in 3 patients and worsened in 1. Hydronephrosis improved in 1 patient. None of the patients developed any impairment in renal function. CONCLUSION. The incidence of upper-tract genitourinary anomalies in girls with congenital adrenal hyperplasia is much higher in our cohort than in the general population (21.2{\%} vs 2{\%}-4{\%}). This association represents a potentially significant medical issue because some patients may require surgical intervention and antibiotic prophylaxis to prevent urinary tract infections and impairment in renal function.",
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