Glomerular injury is a documented complication of congenital cyanotic heart disease (CCHD). Some of the abnormalities reported in patients with CCHD include proteinuria and nephrotic syndrome (NS). The pathology recorded in such patients include glomerulomegaly, capillary dilatation, thickening of the capillary walls, focal or diffuse proliferation of mesangial cells and segmental or global glomerulosclerosis. Most of the reports have been among adults. Major presentation has been NS, and documented reports have been mainly among Caucasians living in the developed world. We describe a nine-year-old African child (living in Nigeria, a developing country) with truncus arteriosus. He was stable for some time but developed NS and, subsequently, renal failure, which required renal replacement therapy at the most recent admission for phlebotomy.
A nine-year-old black African boy with congenital cyanotic heart disease was admitted to the hospital on account of two weeks’ history of facial swelling, exertional dyspnea and orthopnea. He was diagnosed to have congenital cyanotic heart disease (CCHD, truncus arteriosus) since birth. He was symptom free until the age of three years, when he experienced recurrent facial swelling. The swelling worsened in the last two weeks, becoming generalized with exertional dyspnea, cough, orthopnea and oliguria. Physical examination revealed an acutely ill-looking boy with anasarca, plethoric conjunctiva and in respiratory distress (respiratory rate was 60/min). He was cyanosed and febrile (39°C) with a grade-4 finger clubbing. Jugular venous pressure was raised, pulse was 120 beats/min, full volume and regular, while blood pressure was 110/70 mmHg. Apex beat was at the fifth intercostal space anterior axillary line. Both heart sounds were present with grade-4 systolic murmur, loudest at the third left parasternal area. Pulmonic component of the second heart sound was accentuated. Abdominal examination revealed a tender hepatomegaly 6 cm below the costal margin and a liver span of 14 cm. Urinary output remained at <0.1 ml/kg/hr since admission; serum protein, albumin and cholesterol were 51 g/1, 20 g/1 and 5.7 mmmol/1, respectively. Dipstick urinalysis was 3+, while the 24-hour urinary protein was 2.7 g/24 hours. Serum urea and creatinine rose progressively to an upper limit of 16.7 mmol/1 and 481 mmol/1, respectively. Blood culture and urine microscopy and culture yielded no growth. Abdominal ultrasound showed marked increase in renal parenchyma echoes with total loss of corticomedullary differentiation. Both kidneys were normal in position. They measured 9 cm each in their bipolar length. There was polycythemia (PCV 70%), which required phlebotomy on three occasions before a PCV of 55% was achieved. Electrocardiogram revealed biventricular hypertrophy, while the echocardiography still showed truncus arteriosus. An assessment of CCHD with acute and chronic renal failure + nephrotic syndrome, congestive cardiac failure was made. The child received diuretics (frusemide 1 mg/kg/dose, six hourly and hydrochlorothiazide 2 mg/kg/dose, eight hourly), and ceftriaxone 50 mg/kg every 24 hours and was digitalized. The patient’s renal status deteriorated with declining urinary output and worsening serum biochemical profiles. The child was therefore referred to a center where renal replacement therapy could be offered. He died in that center. Because of the deteriorating renal condition and cardiac status, we could not carry out a renal biopsy. Don’t blow your budget on pharmacy items levitra professional canada now