A case of Henoch-Schoenlein purpura nephritis recurring after 3 years remission.

Henoch-Schoenlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries with IgA immune complexes deposition, and about 7% of patients with HSP experience recurrence. Most patients with recurring of HSP nephritis show a recurrence of clinical symptoms over a period ranging from 2 to 5 months, even after the disappearance of initial symptoms. Here we report a 9-year-old girl diagnosed with recurrent HSP and severe crescentic glomerulonephritis 3 years after complete resolution of the initial symptoms of HSP. Our case is unique in respect of the recurrence at more than 3 years after the complete resolution of initial symptoms, suggesting that careful followup is required in spite of improved renal symptoms in cases of HSP.

A 10-year-old girl with IgA nephropathy who 5 years later developed the characteristic features of Henoch-Schönlein purpura nephritis.

We report a patient who developed Henoch-Schönlein purpura (HSP) 5 years after she presented with immunoglobulin A nephropathy (IgAN). A 10-year-old Japanese female was identified with proteinuria and hematuria by a school urinary screening. The first renal biopsy showed mesangial proliferative glomerulonephritis with immunofluorescent findings consistent with IgAN. She was treated with prednisolone, warfarin, and dilazep dihydrochloride, and the proteinuria and hematuria disappeared 4 months after the onset of treatment. Five years later she developed abdominal pain, gross hematuria and a classic purpuric rash of HSP after acute pharyngitis. The second renal biopsy showed diffuse mesangial proliferation with cellular crescent formation, and the patient was treated with methylprednisolone pulse therapy, prednisolone and mizoribine, resulting in a gradual decrease in urinary protein excretion. Our patient is unusual in that she developed Henoch-Schönlein purpura nephritis 5 years after clinical and biopsy evidence of IgAN, which suggests that IgAN and HSP are different clinical manifestations of the same disease, probably sharing a common pathogenesis.

Sponsor-based evaluation of the system for the execution of clinical trial at hospitals and related issues.

With the revision of the Good Clinical Practice (GCP) in 1997, the Clinical Trial Center was established at Saga University Hospital in 1999, where clinical research coordinators (CRC) of nurses and pharmacists have been carrying out support for clinical trials since June 2000. At present, two pharmacists, two nurses, and three clerical work assistants support the execution of clinical trials; however, in recent years the number of clinical trial commissions has been gradually decreasing. On this occasion, in order to carry out even higher quality and smoother clinical trials, we conducted a questionnaire targeting the sponsors of clinical trials (head monitors) to evaluate this hospital's system for the execution of clinical trials from the sponsor's standpoint. Moreover, for the purpose of comparison with the systems of other institutions, the same questionnaire was conducted on two other hospitals-the University of Occupational and Environmental Health, Japan and the Social Insurance Shimonoseki Kousei Hospital. The problems of the clinical trial execution in our team turned out lack of knowledge concerning GCP and our complex system from the result of the questionnaire.

Tonsillectomy with methylprednisolone pulse therapy as rescue treatment for steroid-resistant IgA nephropathy in children.

Primary immunoglobulin A (IgA) nephropathy is characterized by microhematuria and proteinuria and by the deposition of IgA in the glomerular mesangium. Steroid was a main drug for treatment of IgA nephropathy. However, some of children with IgA nephropathy are resistance to steroid treatment, but the therapy for steroid-resistant IgA nephropathy was not established. There have been reports on the efficacy of tonsillectomy as an initial treatment for IgA nephropathy in adults and children. We examined whether tonsillectomy with methylprednisolone pulse therapy (tonsillectomy pulse therapy) was effective as rescue treatment for steroid-resistant pediatric IgA nephropathy. We studied 11 patients (age at onset and duration of follow-up, 11.7 +/- 2.0 and 6.2 +/- 1.1 years) who had been diagnosed with steroid-resistant IgA nephropathy. Clinical features, laboratory data, and pathological findings were retrospectively compared between before and after tonsillectomy pulse therapy. Urinary protein excretion was significantly decreased at 24.7 +/- 7.3 months after tonsillectomy pulse therapy. On renal pathologic examination of 6 patients who underwent renal biopsy at 17.1 +/- 6.9 months after tonsillectomy pulse therapy, the activity index, an index of inflammation, was lower compared to the index evaluated before the therapy, but the chronic index, an index of renal sclerosis, remained unchanged. At 24.7 +/- 7.3 months after tonsillectomy pulse therapy, seven patients had normal urine and four had minor urinary abnormalities; namely, none had active renal disease or renal insufficiency. Our findings suggest that tonsillectomy pulse therapy may be effective as rescue treatment for steroid-resistant IgA nephropathy in childhood.

Accumulation of macrophages expressing myeloid-related protein 8 associated with the progression of sclerotic changes in children with IgA nephropathy.

Myeloid-related protein (MRP) 8 is a calcium-binding protein of the S100 family. The renal accumulation of macrophages expressing MRP8 is associated with the inflammatory activity of glomerulonephritis. We evaluated the renal accumulation of macrophages expressing MRP8 in children with IgA nephropathy (IgAN). We collected data on 25 IgAN children who had been treated with prednisolone and divided these patients into two groups: Favorable group, consisting of 11 patients with normal urine and 6 with minor urinary abnormalities at 4.3 +/- 1.3 years after initial treatment; and Unfavorable group, consisting of 8 patients with persistent nephropathy. The pathological renal findings were compared between both groups. The second biopsy was performed at two years after first biopsy at 5.5 +/- 4.9 months from onset. In Favorable group, the glomerular accumulation of macrophages expressing MRP8, and mesangial cells expressing alpha-smooth muscle actin (alpha-SMA) were lower in the second biopsy specimens than those of the first biopsy specimens. In Unfavorable group, the glomerular accumulation of macrophages expressing MRP8 detected in the second biopsy specimens was similar to that of the first biopsy, while the number of mesangial cells expressing alpha-SMA and the index of renal sclerosis were higher in the second biopsy than in the first biopsy. The indexes of renal sclerosis were higher in children with more macrophages expressing MRP8 than in children with less macrophages expressing MRP8. Our results suggest that renal macrophages expressing MRP8 may be involved in the progression of sclerotic changes in children with IgAN.