To assess the detection rate of nutcracker syndrome in children with isolated hematuria, renal Doppler ultrasound examinations were routinely performed on 216 consecutive children (176 microscopic hematuria and 40 gross hematuria). Renal Doppler ultrasound was also performed on 32 healthy normal children. The peak velocity (PV) was measured at the hilar portion of the left renal vein (LRV) and at the LRV between the aorta and the superior mesenteric artery. The PV at the aortomesenteric portion (P=0.003) and the PV ratios of the LRV (P=0.003) were significantly higher in children with hematuria than in normal children, while the PV at the hilar portion was not different. If a PV ratio of the LRV of at least 4.1 (the cut-off level set at the mean ±2 SD of the value for the normal children) was defined as abnormal, 72 cases (33.3%) in children with hematuria and no cases in normal children were diagnosed as having nutcracker syndrome. The prevalence of nutcracker syndrome is relatively high in children with isolated hematuria, and the inclusion of renal Doppler ultrasound as a screening examination has a substantial effect on the detection of nutcracker syndrome.

To identify factors affecting spontaneous resolution of hematuria in children with nutcracker syndrome, 20 patients diagnosed as having nutcracker syndrome using renal Doppler ultrasound (US) were analyzed retrospectively. Sixteen patients had microscopic hematuria, and four had gross hematuria at presentation. The mean age was 10.6 years (range 2.5-14 years). All underwent a follow-up Doppler US examination after a mean period of 1.4 years (range 0.5-3.5 years) after the first US was performed, and height and weight were measured at the time of US. At the time of follow-up US, hematuria disappeared in 15 patients and improved in 3. The peak velocity (PV) ratios of the left renal vein (LRV) at the follow-up US decreased significantly when compared to the first US examination (7.74+/-2.64 vs 3.50+/-1.09, p<0.0001), and height (147.4+/-20.1 vs 152.3+/-18.8 cm) and weight (36.1+/-10.9 vs 42.3+/-12.7 kg) increased (p<0.0001). Changes in the PV ratios of the LRV correlated positively with changes in the PV at the aortomesenteric portion (r=0.569, p=0.009). Changes in the PV at the aortomesenteric portion correlated negatively with changes in body mass index (BMI) (r=-0.543, p=0.013). Although spontaneous resolution of hematuria in children with nutcracker syndrome is obscure, our findings suggest the increase in BMI may be a possible hemodynamic factor.

To evaluate the efficacy of cyclosporin A (CyA) for treating severe Henoch-Schönlein nephritis (HSN), seven patients with nephrotic syndrome, aged 3.9-13.8 years (mean 6.5 years), were analyzed retrospectively. Mean follow-up times were 5.5 years (range 2-9 years). All underwent renal biopsy before treatment, and follow-up renal biopsy was performed in six of the seven patients. All patients improved, with 24-h protein declining from a mean of 9.2 g/m(2)/day (range 1.5-16 g/m(2)/day) to 0.3 g/m(2)/day (range 0.03-1.2 g/m(2)/day) (p=0.016) and serum albumin increasing from a mean of 2.1 g/dl (range 1.5-2.4 g/dl) to 4.6 g/dl (range 3.5-5.3 g/dl) (p=0.016) after CyA therapy. The activity index decreased significantly at the second renal biopsies obtained at a mean interval of 11.7 months after the first (6.4+/-3.3 vs 3.5+/-1.2, p=0.042, respectively), while the chronicity index and the tubulointerstitial scores did not change. On the immunofluorescent findings at the second biopsies, the degree of deposits of immunoglobulins such as IgA, IgM, C3, and fibrinogen decreased in five of the six patients. Although this case series is without controls, our study suggests that CyA may be beneficial to a subset of HSN patients with nephrotic syndrome.

To evaluate the effect of azathioprine with steroids on the clinical course and histologic parameters of severe Henoch-Schönlein nephritis (HSN), 20 patients with a median age of 9.3 years (range 4.4-17 years) and a follow-up period of 4.8 years (range 1-14 years) were included in this study. The patients were divided into two groups. Ten patients received azathioprine with steroids for 8 months (group A), and the other ten received steroids alone (group B). All patients underwent renal biopsy at presentation, and ten of them who were treated with azathioprine underwent follow-up biopsy after therapy. Six patients in group A achieved clinical remission, and the remaining four showed minor urinary abnormalities at the latest observation. The histological grades of the International Study of Kidney Disease in Children (ISKDC) improved in four of the ten patients, and the activity index decreased significantly from 6.8+/-2.0 to 4.3+/-2.3 (p=0.016). In group B, however, four patients had normal urine and renal function; two had minor urinary abnormalities; one had active renal disease, and three had chronic renal insufficiency, at the latest observation. The combination treatment of azathioprine and steroids may be beneficial in ameliorating histopathological features and improving the clinical course of severe HSN.