increased serum uric acid has been considered with different metabolic , cardiovascular , and renal disorders . glomerular , tubulointerstitial , and vascular involvement with eventual chronic renal disease has been reported in hyperuricemia . recognition of the different manifestations and complications of hyperuricemia seems beneficial to prevent renal damage in the early phase . a 2 year and 9 months girl was admitted for seizure , severe hypertension ( 200/110 ) , and renal failure . she had a previous admission in another hospital for isolated proteinuria , with no clinical follow - up . laboratory exams [ table 1 ] revealed hyperuricemia ( uric acid : 18 mg / dl ) , increased serum creatinine , microscopic hematuria , and nephrotic syndrome . liver function tests , serum cathecolamines , ldh , vitamin b12 , and complement level , were normal . ana , anti - dsdna , anca , anti - gbm , rubella , cmv and hcv antibody , bk virus , hbs ag , and urine mps were all negative . decreased urinary excretion with increased serum level of uric acid ( hypouricosuric hyperuricemia ) confirmed by excluding all other causes of hyperuricemia . normal to mild renal enlargement with bilateral increased echogenicity and increased resistive index in favor of parenchymal renal disease with no renal stenosis detected in ultrasound exam . severe diastolic dysfunction with concentric left ventricular hypertrophy and decreased ejection fraction in favor of chronic hypertension were documented in echocardiography . laboratory variables at presentation and post treatment peritoneal dialysis was performed for acute renal failure in addition to antihypertensive treatment . renal biopsy showed diffuse global sclerosis with glomerular hypertrophy , increased mesengial cellularity , segmental solidification , tubular atrophy , focal inflammation , and arterial narrowing with the impression of esrd and end arteritis fibrosa , suggested the possible changes of chronic malignant hypertension superimposed on focal segmental glomerulosclerosis [ figure 1 ] . diffuse global glomeruli sclerosis ( 400 , pas stain ) despite of improved renal function ( serum creatinine : 0.8 mg / dl ) , serum uric acid was persistently elevated in repeated samples with low urine uric acid . a second renal biopsy performed for disproportional improved renal function compared to distorted renal biopsy revealed segmental and global glomerular sclerosis , mild to moderate hyperplasia in small arterioles and chronic inflammation with interstitial fibrosis [ figure 2 ] . therefore , the first renal biopsy assumed to be taken from a nidus of glomerular sclerosis . as a primary focal segmental glomerular sclerosis , she was treated with corticosteroids followed by conventional immunosuppressive treatments ( cyclosporine , mycophenolate mofetile ) , with no clinical response . genetic analysis revealed no documented mutation for common nphs2 gene mutation . during the follow - up period on allopurinole and anti - proteinuirc treatment , the patient had normal renal function with mild to moderate proteinuria and no recurrence of nephrotic syndrome . hyperuricemia occurs in conditions with uric acid over production such as ketogenic diet , increased intake of purine rich diets , hprt or arpt deficiency , or prpp overactivity , cellular proliferation , malignancies , and rhabdomyolysis , or decreased uric acid excretion in chronic kidney disease , diabetic ketoacidosis , starvation , and volume contraction . miscellaneous causes of hyperuricemia include metabolic syndrome , hypothyroidism , hyperparathyroidism , siadh syndrome , gsd type 1 , sarcoidosis , and drug administration . familial juvenile hyperuricemic nephropathy is an inherited disorder characterized by hypouricosuric hyperuricemia , progressive tubulointerstitial nephritis , and chronic renal failure and is considered as the possible underlying disease in this patient . nephrolithiasis is the most common renal involvement in hyperuricemia , complicated by acute obstructive renal failure . a pathogenic link has been reported between hyperuricemia and essential hypertension through activation of renin  angiotensin system , downregulation of nitric oxide , vascular muscle proliferation , afferent arteriolosclerosis , altered pressure natriuresis , endothelial dysfunction , and abnormal cellular sodium transport . lowering uric acid level controls hypertension in some patients initially , becomes irreversible , salt sensitive , and resistant to uric acid lowering treatment later . glomerular hypertrophy / hypertension , afferent arteriolar sclerosis , and macrophage infiltration , vasoconstriction , chronic ischemia , morphologic changes similar to focal mesangio - capillary or mesangial proliferative glomerulonephritis with increased mesangial cells and matrix , focal segmental glomerular sclerosis , capillary basement membrane thickening , and chronic tubulointerstitial damage progressive to end - stage kidney disease have been reported in chronic hyperuricemia . this patient had persistent proteinuira originated from glomerular sclerotic lesions secondary to chronic hypouricosuric hyperuricemia . therefore , measurement of serum uric acid and early treatment of hyperuricemia are recommended in patients with idiopathic proteinuria to prevent further renal damage .
nephrolithiasis , obstructive renal failure , essential hypertension , and chronic tubulointerstitial nephritis have been considered as the renal complications of hyperuricemia . 
 massive proteinuria has been rarely reported as the primary manifestation of increased serum uric acid . 
 this is the report of a child presented with proteinuira , hypertension , and glomerular scelrosis secondary to hypouricosuric hyperuricemia , who was treated by uric acid lowering management .