Congenital anomalies of kidney and urinary tract in siblings: An uncommon condition
A 5-year-old boy presented to the outpatient department with recurrent abdominal pain and fever off and on for the last 6 months. General physical and systemic examination of the child was normal. Urine examination revealed pyuria and urine culture grew Escherichia coli. Blood urea and serum creatinine levels were 35 mg/dL and 0.6 mg/dL, respectively. The urinary tract infection was treated with appropriate antibiotics and further evaluation was carried out. Ultrasonography of the abdomen revealed right-sided hydronephrosis with suspicion of PUJO; the left kidney appeared normal. The micturating cystourethrogram (MCUG) did not reveal any abnormality. A radionuclide scan showed bilateral obstructive hydronephrosis with moderate impairment of the left kidney. The child underwent a pyeloplasty of the left kidney followed by pyeloplasty of the right kidney 3 months later. A Diethylene triamine penatacetic acid (DTPA) scan after 8 months of surgery showed mild hydronephrosis of both kidneys with preserved parenchymal function and slow but non-obstructed drainage pattern (differential function: Right kidney 49%, left kidney 51%). Subsequently, the child has been under regular follow-up of our pediatric nephrology clinic. At present, the child is 13 years old and has CKD stage 3 (estimated glomerular function rates (eGFR) 50 mL/min/1.73 m² by Schwartz formula).\nThe younger sibling of this patient presented with bilateral antenatal hydronephrosis detected in the second trimester of pregnancy. The child was born at term of a non-consanguineous marriage. Postnatal investigations confirmed bilateral PUJO. The first postnatal ultrasound showed bilateral hydronephrosis with right kidney measuring 5.2 × 3 cm in size and left kidney 6.6 × 3.8 cm in size without ureteric dilatation. The MCUG was normal. The DTPA scan at 45 days of age revealed right kidney with well-preserved function with obstructive hydronephrosis and grossly enlarged left kidney with moderately impaired function and obstructive hydronephrosis (differential function: right kidney 64% left kidney 36%). The child underwent pyeloplasty of the left kidney at 2 months of age and pyeloplasty of the right kidney at 8 months. A DTPA scan 1 year after surgery showed both kidneys with normal function and partial mechanical obstruction; differential function of right kidney was 45% and left kidney 55%. Subsequent scans have also shown similar findings. This child is now 8 years old and is on regular follow-up at our clinic (CKD stage 1). The family has another male child aged 17 years who has no renal abnormality. The ultrasounds of both parents revealed no abnormality.