Renal manifestations in children with Alagille syndrome.

INTRODUCTION

Alagille syndrome (AS) is a cholestatic disease secondary to scarcity of interlobular bile ducts. It is associated with extrahepatic manifestations, and renal involvement is frequent.

OBJECTIVES

To describe the prevalence, type and outcome of renal pathology in children with AS.

PATIENTS AND METHODS

The presence and outcome of renal pathology was retrospectively studied in 21 children who met AS criteria.

RESULTS

Renal pathology was observed in 18 patients (85.7%): (1) ultrasound variations in 7 patients (6 cases of bilateral renal dysplasia and 1 case of renal agenesis); (2) distal renal tubular acidosis in 2 patients; (3) a drop in glomerular filtration and/or proteinuria in 16 patients. The frequency of a drop in glomerular filtration was similar between patients with and without pathological kidney ultrasound findings.

CONCLUSIONS

Our study confirms a high prevalence of renal involvement, which enhances the importance of diagnosis and renal function follow-up in children with AS.