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A Japanese boy aged 14 years presented with gross hematuria associated with mild proteinuria and was diagnosed as having nutcracker syndrome. Magnetic resonance angiography (MRA) revealed significant compression of the left renal vein between the aorta and the superior mesenteric artery with collaterals. A percutaneous renal biopsy on the right kidney revealed no evidence of glomerular or interstitial changes with immune deposition. He was observed closely without any intervention thereafter. Although repeat MRA performed 4 years after our first observation disclosed the development of collateral veins, severe hematuria with an intermittent exacerbation remained unchanged. During the next 2 years, the hematuria completely subsided spontaneously. Although the etiology of spontaneous remission of the disease remains speculative, his good physical development (i.e., approximately 10 cm taller than his height at the onset) may change presumptive hemodynamic factors. These clinical observations suggest that a proportion of pubertal patients with nutcracker syndrome should be treated conservatively for a relatively long time.

A Japanese girl aged 8 years who presented with a 2-month history of uveitis subsequently developed tubulointerstitial nephritis. A percutaneous renal biopsy revealed massive interstitial mononuclear cell infiltrates consisting of CD4-positive T cells. Despite administration of topical corticosteroids, the ocular symptoms persisted. Systemic corticosteroid therapy dramatically reduced the ocular symptoms and urinary beta2-microglobulin (beta 2MG) concentration. However, reducing the prednisolone dosage induced recurrence of uveitis associated with increased levels of urinary beta 2MG. The CD4-positive T cell infiltration persisted in the second renal biopsy performed 6 months after the first renal biopsy. These observations suggest that the interstitial cell infiltration persists for a relatively long time in a proportion of patients with tubulointerstitial nephritis and uveitis syndrome (TINU). Although the renal outcome of TINU has been reported to be favorable, prolonged interstitial cell infiltration may affect long-term renal outcome. Selected patients with TINU should be followed with close observation.

Although only a small number of patients were examined, these clinical findings suggest that a combined therapy of enalapril and losartan may attenuate histologic progression in at least a proportion of patients with severe proteinuric IgA nephropathy.

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