Although prognosis of post-streptococcal acute glomerulonephritis (PSAGN) in childhood is generally thought to be benign, it may show fatal outcome due to acute renal failure, congestive heart failure and hypertensive encephalopathy (HE). We here present a 9-year-old girl with PSAGN associated with HE manifested by status epilepticus. She was transferred to our hospital and had been successfully treated by anti-epileptic drugs and continuous drip infusion of intravenous anti-hypertensive agent, nicardipine hydrocholoride. As prompt diagnosis and treatment are essential to manage HE, this condition should be listed in the differential diagnosis in the childhood convulsions. Nicardipine hydrochloride has been recently reported to be safe and effective to manage the condition caused by severe hypertension such as HE even in children: this was also effective in our case and may be one of the drugs for hypertensive emergency.

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  We report a case of Clq nephropathy associated with frequent relapsing nephrotic syndrome. The patient was 7-year-old boy. He was referred to our hospital because of nephrosis in 4-year-old. He gradually became frequent relapsing nephrotic syndrome. The renal biopsy was performed in 7-year old. The light microscopy showed the slight cell increase in glomeruli. The most remarkable findings were granular depositions of Clq most strongly in glomeruli along with the depositions of IgG and fibrinogen on the immunofluorescence study. We diagnosed our case as Clq nephropathy. Clq nephropathy was comparatively rare disease, and there were very few reports about the case of Clq nephropathy presented with the frequent relapsing nephrotic syndrome, so our case must be the precious one.

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  The aim of our study is to elucidate the relationship between IgA subclasses and complement activation in IgA glomerulonephritis (IgAGN). Immunohistological staining was performed on biopsied renal specimens obtained from 26 patients with IgAGN who received repeated biopsy, using antibodies against IgG, IgA, IgA1, IgA2, IgM, C1q, C3c, C4, factor B, mannose-binding lectin (MBL) and MBL-associated serine protease-1 (MASP-1). Mesangial deposits of both IgA1 and IgA2 were found in 11 of 26 patients at the first biopsy, accompanied by mesangial deposits of C3c, C4, factor B and MBL/MASP-1. Mesangial deposits of IgA2 and MBL/MASP-1 were colocalized. The remaining 15 patients showed mesangial deposits of IgA1 alone with neither C4 nor MBL/MASP-1, accompanied by mesangial deposits of C3c in 13 and factor B in 15. Mesangial deposits of Iga1, Iga2 and MBL/MSAP-1 were found in 15 patients at the second biopsy, including 4 with mesangial deposits of IgA1 alone at the first biopsy. The remaining 11 patients showed mesangial deposits of IgA1 alone with neither C4 nor MBL/MASP-1 at the second biopsy. Proteinuria was unchanged in patients with mesangial deposits of both IgA1 and IgA2 in spite of treatment, whereas proteinuria remarkably decreased in those with mesangial deposits of IgA1 alone at the second biopsy. There was no difference in the clinical outcome and the histological severity between those with and without mesangial deposits of IgA2. We reconfirmed immunohistologically that mesangial deposits of IgA1 might activate the alternative pathway, and mesangial deposits of IgA2 might activate the lectin pathway. Our current study indicates a novel evidence that IgA2 subsequently deposits in the mesangial area where IgA1 already deposited, and proteinuria prolongs in those with mesangial deposits of both IgA1 and IgA2.

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  We reported the clinical course of a 21-day old female who presented postrenal acute renal failure from bilateral congenital hydronephrosis. She was detected as having bilateral hydronephrosis at about 23 weeks of gestation. She was delivered through cesarean section at 36 weeks due to the decrease of amniotic fluid volume. Through both antenatal and postnatal ultrasonography, renal pelvic dilatation was more prominent on the right side than on the left. Although we performed emergency placement of percutaneous nephrostomy first on the right kidney, electrolytes imbalance and elevation of SCr and BUN continued. We then placed the nephrostomy on the left kidney because the RI-renography done on 33th day revealed better differential renal function on the left, less dilated kidney. Renal function improved just after this procedure, and she went home 2 weeks after bilateral, simultaneous pyeloplasty. We discussed the importance of RI-renography to evaluate split renal function in small children with acute renal failure from bilateral hydronephrosis.

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  We previously reported that p38 mitogen-activated kinase (p38) is upregulated and activated in cysts and tubules of human renal dysplasia. Increased proliferation and upregulation of Pax2 and TGF-β1 in dysplastic epithelia have previously been reported. Dysplastic kidneys are often associated with urinary tract obstruction. In the present study, we investigated whether cyclic mechanical stretch, which mimics the hydrodynamic derangement after urinary tract obstruction, activates p38 and stimulates proliferation and the expression of Pax2 and TGF-β1 in ureteric bud (UB) cells. The UB cell line was generated from mice transgenic for SV40 T-antigen. Cells were subjected to cyclic mechanical stretching (20% elongation, 20 cycles per minute). Protein levels of p38, phospho-p38, and Pax2 was assessed by immunoblot analysis. Proliferation of UB cells was measured by BrdU incorporation. TGF-β1 secretion was evaluated in conditioned media by enzyme-linked immuno-sorbent assay. Cyclic stretch induced expression of phospho-p38 activation at 10 minutes, with a maximum 5-fold increase at 24 hours. Protein expression of p38 remained unchanged. Pax2 expression also increased by cyclic stretch in a time-dependent manner with a maximum 1.7-fold increase at 24 hours. Stretch-induced Pax2 expression was completely abrogated by a specific p38 inhibitor SB203580 5μM. BrdU incorporation in stretched cells was significantly increased compared with non-stretched control cells at 24 hours (0.659±0.078 vs 0.349±0.052, n=7, P<0.05). SB203580 5μM again abolished stretch-induced proliferation of UB cells (0.364±0.060, NS vs control). TGF-β1 secretion was not affected by either stretch or SB203580. These results demonstrate that cyclic stretch stimulates proliferation and Pax2 expression via activation of p38 in UB cells. p38-mediated UB cell proliferation may lead to cyst formation in fetal urinary tract obstruction.

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  In order to investigate the number of monocytes/macrophages (Mo/MΦ) in urine as an activity index of glomerulonephritis of children, urinary Mo/MΦ were measured in two cases of post streptococcal acute glomerulonephritis (PSAGN), one case of Henoch-Schonlein purpura nephritis (HSPN), two cases of IgA nephropathy (IgAN), and one case of membranoproliferative glomerulonephritis (MPGN). The urine samples of all cases during each stage of both pre-therapy (active stage) and post-therapy (inactive stage) were collected, respectively. The Mo/MΦ contained in urine were measured as the number of CD68 positive Mo/MΦ (CD68⁺ Mo/MΦ), using anti-CD68 antibody. The number of urinary CD68⁺ Mo/MΦ in a clinically active stage was significantly higher than that in an inactive stage.
  From these results, it was suggested that the number of CD68⁺ Mo/MΦ in urine indicated clinical activity in proliferative glomerulonephritis groups of children.

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