Autosomal Dominant Polycystic Kidney Disease Complicated by Glomerulonephritis

Abstract

Two patients with autosomal dominant polycystic kidney disease (ADPKD) and concurrent glomerulonephritis are described. Both developed nephrotic-range proteinuria and one showed a concomitant acceleration in the rate of decline of renal function. Subsequent open renal biopsy revealed membrano-proliferative type-1 and mesangio-proliferative glomerulonephritis, respectively. Nephrotic-range proteinuria in the presence of ADPKD, with or without an accompanying decline in renal function, should prompt further investigation to exclude coexisting glomerular disease.

Autosomal dominant polycystic kidney disease (ADPKD) associated with steroid-sensitive nephrotic syndrome in childhood

“…A presumed diagnosis of mesangiocapillary glomerulonephritis was made because of the other sister's history. A similar case of mesangiocapillary glomerulonephritis in a 25-year-old woman, which progressed to ESRD within 10 months, has been reported [2].…”

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Sirs, Nephrotic syndrome (NS) associated with autosomal dominant polycystic kidney disease (ADPKD) is observed only occasionally in adults [1,2], and it is extremely uncommon in childhood; to the best of our knowledge only three cases have been reported in the literature [3][4][5].We report a case of ADPKD associated with steroidsensitive NS in a 9-year-old boy, who was referred to the Nephrology Department with features of NS of 1 week's duration. He was born prematurely (28 weeks' gestation), suffered chronic lung disease, cerebral palsy with spastic quadriplegia and epilepsy (on sodium valproate), secondary to the neonatal problems.

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“…Sirs, Nephrotic syndrome (NS) associated with autosomal dominant polycystic kidney disease (ADPKD) is observed only occasionally in adults [1,2], and it is extremely uncommon in childhood; to the best of our knowledge only three cases have been reported in the literature [3][4][5].…”

Idiopathic membranous nephropathy associated with polycystic kidney disease

“…These include focal segmental glomerular sclerosis, membranoproliferative type-1 glomerulonephritis, mesangio-proliferative glomerulonephritis, amyloidosis, and IgA nephropathy [5][6][7][8].…”

Nephrotic syndrome and IgA nephropathy in polycystic kidney disease

“…Contreras et al 3 reported an ADPKD patient with FSGS and reviewed 14 cases of ADPKD in which the renal lesion had been evaluated by histopathologic studies. [9][10][11][12][13][14][15][16][17][18][19][20] Unfortunately, their 3 summary (in a Table) did not include previous reports of Japanese cases; 21-26 therefore, we modified their list, adding seven more Japanese cases, with an oral corticosteroid (prednisolone 30 mg), dipyridamole 300 mg, temocapril hydrochloride 2 mg, and losartan potassium 50 mg daily, resulting in a decrease of urinary protein excretion to 0.2 g/24 h and a decrease in the blood pressure to 140/70 mmHg. In February 2005, after a 5-year follow-up, his urinary protein excretion was 0.08 g/24 h and CCr was 40.9 ml/min, with 5 mg prednisolone daily and the same antihypertensive agents.…”