Japanese journal of pediatric nephrology
Online ISSN : 1881-3933
Print ISSN : 0915-2245
ISSN-L : 0915-2245
Volume 21, Issue 2
Displaying 1-26 of 26 articles from this issue
Original Article
  • Hiroyoshi Matsukura
    2008 Volume 21 Issue 2 Pages 91-94
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Membranous nephropathy (MN) often shows covert onset, accompanied with edema, microscopic hematuria or asymptomatic proteinuria, but rarely with gross hematuria. A Japanese 4-year-old girl was referred for evaluation of proteinuria and gross hematuria. No family members had renal diseases. Urinalysis showed massive proteinuria and dark-colored urine containing many red blood cells. Laboratory investigations included serum creatinine of 0.4mg/dl, low serum total protein of 5.9g/dl, and elevated total cholesterol of 243mg/dl. Serum complement levels were normal. Anti-DNA antibodies and hepatitis B antigen were negative. Urine culture showed no growth. The SAS Adeno Test (SA Scientific, San Antonio, TX, USA) confirmed human adenovirus infection. A biopsied kidney specimen contained 50 glomeruli. The glomerular basement membrane appeared normal with the absence of mesangial proliferation. The tubules and the interstitium were normal. Immunofluorescence showed diffuse coarse granular staining of IgG and C3 along all the capillary walls. Electron microscopy showed epithelial deposits located beneath the foot process, and normal lamina densa without spiky projections. She received oral prednisolone and imidaprilhydrochloride, and then proteinuria promptly disappeared but isolated microscopic hematuria still persisted. We describe a female child with gross hematuria and proteinuria following adenoviral infection, whose renal biopsy specimen showed typical MN, and we discuss the possible association between adenovirus and MN.
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  • Kenichi Sakamoto, Hidekazu Kawakatsu, Fumi Nomura, Yoshiko Hibi, Hirok ...
    2008 Volume 21 Issue 2 Pages 95-99
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Purpose: The purpose of this study was to provide an introduction to evaluating the significance of limited exercise in IgA nephropathy.
     Patients and methods: The study population consisted of thirty-eight children participating in kidney disease consultation with Kyoto City Medical Association, and sixty-six additional children admitted to Kyoto City Hospital for renal biopsy. We evaluated urine protein before and after an exercise load test, and their creatinine clearance (Ccr) changes waking and sleeping.
     Results: An exercise load test showed an increase of urine protein after anexercise only in children who presented urine protein over 0.10g/gCre. Whereas, Ccr while awake was significantly higher than that of sleeping children who presented urine protein under 0.30g/gCre, there were no significant changes in sleeping children who presented urine protein over 0.30g/gCre.
     Conclusion: It is possible that exercise affects kidneys with IgA nephropathy, especially where urine proteins are at high levels. Therefore, limited exercise needs to be performed as prescribed in order to prevent kidney function problems.
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  • Chieko Matsumura, Hideaki Kurayama, Hiroshi Kitamura, Katsuyoshi Kanem ...
    2008 Volume 21 Issue 2 Pages 100-105
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     We investigated a long-term prognosis of 60 children with idiopathic membranoproliferative glomerulonephritis (MPGN) from 1973 to 2006; 50 patients had type I MPGN, 7 dense deposit disease and 3 type III MPGN. Forty-eight children had asymptomatic onset; 46 were identified by school urinary screening program; 40 (83%) had normal urinalysis, normal renal function and histopathological improvement at the last observation. Early detection and treatment was effective for a long-term prognosis. Twelve patients had symptomatic onset; at the last observation, 5 had urinary abnormalities, among which, 4 had nephrotic syndrome (NS) at the onset, and progressed to the end-stage renal failure. NS and frequent crescents formation at the onset associated with a poor prognosis in patients with symptomatic onset.
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  • Takuji Yamada, Osamu Uemura, Katsumi Ushijima
    2008 Volume 21 Issue 2 Pages 106-109
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     In the Schwartz calculation of creatinine clearance using height, serum creatinine concentration and coefficient k to estimate glomerular filtration rate. Creatinine concentration is assessed by Jaffe rate assay rather than the enzymatic method preferred in Japan. Objective: To determine k values for use with an enzymatic method. Methods: Urine of patients (N=172, age 2-21 yrs, males 99), was tested over exactly 24 h for creatinine clearance when examined for various kidney diseases between January, 2002 and July, 2007 (dialysis patients excluded). An enzymatic method, ACCURAS AUTO -CRE diagnosis kit (SHINO-TEST, Japan), was employed to test serum and urine. Measurements were analyzed using a Hitachi-7170S (Hitachi, Japan). Subjects were divided into three groups: 107 boys and girls, 2-12 yrs; 31 boys, 13-21 yrs; 34 girls, 13-21 yrs. Results: Mean total creatinine clearance=107.0±44.4ml/min/1.73m2. The relations with L/Cre (X) and Ccr (Y) were: Y=0.3977X (r2=0.5802); Y=0.3857X (r2=0.4471) and Y=0.5271X (r2=0.681), respectively. They revealed a close correlation. Conclusion: The Schwartz formula can be used when creatinine concentration is measured by an enzymatic method. The k coefficients were 0.53 for boys aged 13-21 yrs, 0.40 for girls aged 13-21 yrs and 0.40 for boys and girls aged 2-12 yrs.
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  • Yohei Ikezumi, Toshiaki Suzuki, Tamaki Karasawa, Makoto Uchiyama
    2008 Volume 21 Issue 2 Pages 110-115
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     The annual urinalysis screening program for all school children in Japan has contributed to the early diagnosis and improvement of a therapeutic regimen for childhood chronic kidney diseases. However, there are few nationwide epidemiological surveys based on the screening program. Though idiopathic nephrotic syndrome (NS) is one of the most common kidney diseases in children, there is little nationwide epidemiological data. We explored the incidence of IgA nephropathy (IgAN) in the city of Niigata based on a screening program, and the incidence of childhood NS in Niigata prefecture through a surveillance network.
     All school children in the city of Niigata who had a urinalysis screening test between 1993 and 2006 were examined. The results revealed that the screening program annually found about 0.68 cases of new onset IgAN per 10,000 school children. Children discovered in the screening program (screening group), underwent a renal biopsy at about 10.8 months after urinary abnormalities were first detected. Children who were hospitalized directly because of symptomatic onset (symptomatic onset group), underwent renal biopsy at about 5.3 months (p<0.05). By contrast with the symptomatic onset group, a significantly high number of children in the screening group archived remission of proteinuria within 2 years (p<0.05). The incidence of NS in children below age 15 in Niigata prefecture was about 4.2 per 100,000. The incidence was almost the same as the incidence about 40 years previously. However, since the number of children has decreased during this time, the cumulative number of children with NS appeared to have decreased by two thirds.
     In summary, a urinalysis screening system is a useful for collecting epidemiological data and for clinical management. The expansion of a network surveillance system is essential for a nationwide epidemiological survey.
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Review
  • Kandai Nozu, Kazumoto Iijima, Masafumi Matsuo
    2008 Volume 21 Issue 2 Pages 117-121
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Alport syndrome (AS) is a hereditary disorder that generally runs a progressive course. It usually presents in children as haematuria and proteinuria associated with neurosensory deafness and progresses to end-stage renal failure (ESRF). The median renal survival rate for male X-linked AS (XLAS) cases is<25 years. Characteristic alterations of the glomerular basement membrane (GBM) are observable with electron microscopy (EM). AS is characterized by irregular thinning and thickening of the GBM as well as replication of the lamina densa, which produces a laminated appearance or basket-weave pattern. When these changes are diffuse, a diagnosis of AS can be made. AS is genetically heterogeneous and X-linked, with both autosomal dominant and autosomal recessive modes of inheritance. However, <90% of AS cases show X-linked inheritance, caused by mutations in the COL4A5 gene, which encodes the α5 chain, a type IV collagen. The α5 chain is expressed in the GBM, Bowman's capsule (BC) and epidermal basement membrane (EBM). In cases with typical male XLAS, immunohistochemical analysis shows a complete loss of the α5 chain in the GBM, BC and EBM, whereas typical female cases show segmental loss with a pattern known as mosaicism.
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  • Mitsuru Okada, Hidehiko Yanagida, Keisuke Sugimoto, Shinsuke Fujita, M ...
    2008 Volume 21 Issue 2 Pages 122-125
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Little is known about the mechanisms of mizoribine (MZR) absorption. We evaluated the role of the human Na+/nucleoside cotransporter (hCNT2) transportation system in MZR absorption.
     It was proven to the absorption of MZR that the carrier mechanism in the enteric canal by hCNT2 was taking part. Our conclusive finding indicated that the consideration of food style could be necessary for MZR absorption rate, because MZR absorption can be compete from inosinic acid. Significant correlation between hCNT2 polymorphism and the rate of MZR absorption was not demonstrated in this study, however more large scale study should be required in future study.
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  • Keisuke Sugimoto, Shinsuke Fujita, Hidehiko Yanagida, Mitsuru Okada, T ...
    2008 Volume 21 Issue 2 Pages 126-133
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Heparin-binding EGF-like growth factor (HB-EGF) is a member of the EGF family of growth factors and is first synthesized as membrane associated precursor molecule (proHB-EGF). ProHB-EGF is cleaved enzymatically, resulting in the shedding of the mature secreted form (soluble HB-EGF). While soluble HB-EGF as a mitogenic signals bind to and activate the EGF receptor (EGFR) of neighboring cells as autocrine/paracrine manner, a considerable amount of proHB-EGF remains on the cell surface under normal physiological conditions, and it is biologically active as juxtacrine growth factor that signals to neighboring cells (juxtacrine activation). Juxtacrine activation plays a role to cell survival and epithelial integrity through EGFR. As epithelial cells attach and spread, they may make contact with neighboring cell, and focal complex processes including lamellipodia are considered premature cell-cell contact sites. In MDCK cells, stable expression of a non-cleavable and membrane-anchored human HB-EGF construct (5AA cell) results in enhanced cell spreading and formation of significantly extended lamellipodia and filopodia compared to wild type (WT) cells. Furthermore, there was increased tyrosine phosphorylation of Paxillin and FAK in the 5AA cells compared to the control cells, which Src tyrosine phosphorylation was not different between the two cell lines. In addition, there was a marked increase in phosphorylation of Akt in 5AA cells compared to WT cells. Cells expressing a non-cleavable HB-EGF construct mutated to prevent EGFR activation (m5AA cell) showed late attachment and had no change in cell spreading compared to WT. Western blot analysis also showed decreased expression levels of phosphorylation of FAK and Paxillin. These findings suggest that proHB-EGF can not only precursor for soluble forms but also important roles for biological functions of EGFR activation as juxtacrine manner.
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  • Shinsuke Fujita, Mitsuru Okada, Hidehiko Yanagida, Keisuke Sugimoto, T ...
    2008 Volume 21 Issue 2 Pages 134-137
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     It is generally understood that cyclosporin A (CyA) can be used safety without adverse effects including renal toxicity when keeping adequate plasma drug concentration for about two years. We encountered four boys with frequent relapsing nephrotic syndrome exhibited glomerular sclerosis despite optimal use of CyA therapy within two years. CyA arteriopathy representing hyalinosis of vessel walls was concomitantly observed with relatively high incidence. We noticed that this phenomenon is closely related to the frequency of premature glomerulus that exists after birth. Vasocontruction of glomerular arteries by CyA could be affect the blood supply into glomerulus. Lack of several glomerular maturation factors in blood could be lead to maturation failure of glomerulus. CyA therapy may inhibit glomerular maturation after birth. Caution is required when use CyA for younger children presumably having premature glomerulus exist even after birth.
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  • —the mechanism of exacerbation of renal pathological findings in HIGA mice by viral infection—
    Yukihiko Kawasaki
    2008 Volume 21 Issue 2 Pages 138-145
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     In chronic nephritis, renal symptoms are frequently preceded by episodes of upper respiratory tract infections and/or gastrointestinal infections, suggesting viruses as the etiology of this disease. There have been some reports on human nephritis and experimental models associated with acute viral infection, including coxsackie viruses. In order to clarify the pathogenetic role of virus infection on chronic glomerulonephritis, we investigate the efficacy of Coxsackie B4 virus (CoXB4), a most common human enteric pathogen, infection on hyper IgA (HIGA) mice and were analyzed for the presence of enteroviral RNA using polymerase chain reaction (PCR) in IgAN. CoXB4 provoke the exacerbation of HIGA mice renal findings and positive PCR results were obtained for 3 patients (30%) of IgA. Our results suggest that the CoXB4 infection may be one of the factors involved in the mechanism for onset or evolution of IgAN.
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  • —A Project for Standardization of Urinary Screening system—
    Makoto Ninomiya, Yuhei Ito, Shinzaburo Hattori, Zyunichi Miyata, Shige ...
    2008 Volume 21 Issue 2 Pages 146-156
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Approximately 60% of school urinary screening programs in Kyushu are carried out at local clinics by doctors without specialized training.Especially in rural areas, almost 70% of the examinations are performed individually at private clinics. In some parts of those areas, urinary screening system has not well established because there are no urinary screening committees, a system for managing the emergency visits to a hospital in case of the critical urine abnormalities, or a standardization of judgment for examination results.
     In order to resolve these problems, the Kyushu Council for the In-School Examination created a standard manual and a summation table in 2004.
     In Miyazaki prefecture, use of the standard manual and summation table resulted in a significant increase in the third examination rates, and a significant decrease in diagnostic reservation rates.
     The standardization of urinary screening system has not been achieved yet in whole Kyusyu area although the unified summation table was introduced 3 years ago. In fact, the prevalence of proteinuria and hematuria varies among prefectures. In addition, the questionnaire survey to school nurses in Kyushu and Okinawa showed that the rates of recognition were low (8.4-13.9%) and that the rates of utilization were also low (29.4-34.9%) in 2006.
     We hope the standard manual and summation table will be further recognized and well utilized not only in rural area but also in urban area in Kyusyu, and we expect in the future to establish a large-scale database for epidemiological research on the basis of the unified standard manual.
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  • Takeo Nakayama
    2008 Volume 21 Issue 2 Pages 157-165
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
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Case Report
  • Takuya Hanada, Atsushi Hayashi, Yasuo Kawaba, Shin-ichi Okada, Motoaki ...
    2008 Volume 21 Issue 2 Pages 167-171
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     We reported a case of juvenile nephronophthisis. The patient was 7-year-old boy who was detected at a mild proteinuria on urinary screening. He presented with hypertension, anemia, and renal dysfunction. He had mild proteinuria and renal concentrating disability, but no microhematuria. Renal histology revealed global sclerosis, renal tubular cysts, and tubulointerstitial cell infiltrates with interstitial fibrosis. He was diagnosed chronic renal failure caused by juvenile nephronophthisis by renal pathology, and is receiving peritoneal dialysis. Medical examination by interview revealed that he had polyuria at day and night time from three years old. It is difficult to screen nephronophthisis on 3-year old and school urinary screening. When we discovered that he had renal concentrating disability, we should discriminate this disease from others.
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  • Toshihiro Sawai, Aya Sakaue, Masahiro Nozawa, Masaru Iwai, Yasuyuki No ...
    2008 Volume 21 Issue 2 Pages 172-175
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Ellis-van Creveld syndrome is a rare disorder characterized by short ribs, polydactyly and growth retardation. We report here a case of Ellis-van Creveld syndrome with nephronophthisis. The patient was an 11-year-old girl, who was pointed out Ellis-van Creveld syndrome at birth. She developed proteinuria at 10-year-old and gradually progressive renal failure, requiring PD at 12-year-old. Histopathological findings in the renal biopsy specimen were compatible with nephronophthisis. To our knowledge, there is a single report in the literature regarding an association between nephronophthisis and Ellis-van Creveld syndrome.
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  • Yukiko Hashimoto, Hidekazu Kawakatsu
    2008 Volume 21 Issue 2 Pages 176-181
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Asymptomatic microhematuria and proteinuria were detected by school urinalysis screening in a 12-year-old Japanese female patient, whose case we followed for the subsequent two years, and diagnosed as antineutrophil cytoplasmic antibodies (ANCA) associated glomerulonephritis.
     She developed rapid progressive renal dysfunction and positive results for myeloperoxidase (MPO)-ANCA. As the initial treatment, we employed methylprednisolone pulse (MP) therapy, and her renal function improved significantly. Two months later, she experienced an exacerbation concomitant with infectious enterocolitis. She again received MP, and second percutaneous renal biopsy. The light microscope findings of renal biopsy specimen demonstrated progressive screlotic change, and global sclerosis, accordingly we discontinued MP, and started oral prednisolone (PSL), mizoribine (MZB), and other drugs. Although she retained renal function, clinical manifestations-microscopic hematuria and proteinuria-have persisted. MPO-ANCA titers were within normal ranges for about six months after the exacerbation, however then rose gradually. The patient received intravenous pulse cyclophosphamide (IVCY) for four months to decrease MPO-ANCA titers, but the response was not satisfactory. With persistent microscopichematuria and proteinuria, careful follow-up is required.
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  • [in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...
    2008 Volume 21 Issue 2 Pages 182-187
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     A 22-year-old severely disabled adult patient with valproate (VPA)-induced Fanconi syndrome was described. He developed to hypouricemic acute renal failure (ARF) when he had respiratory failure.
     He had been treated with VPA over a period of 20 years: VPA was initiated since 8 months of age when he was diagnosed as having West syndrome. At the age of 22 years and 3 months, the diagnosis of VPA-induced Fanconi syndrome was made based on typical laboratory findings, such as hypophosphatemia, severe hypouricemia (0.5mg/dl), renal glucosuria, tubular proteinuria and generalized aminoaciduria. Three months later, he was hospitalized because of respiratory failure due to pneumonia complicated by asthmatic attack. Although both anti-microbial and anti-asthmatic treatment improved his respiratory failure, he developed to ARF 4 days after admission:his renal function recovered gradually by diuretic therapy. Quantitative measurement of blood reactive oxygen metabolites revealed severe oxidative stress at the time of ARF.
     From these findings, we believe that the oxidative stress by respiratory failure caused ARF in our patient because it is speculated that renal reperfusion injury due to vasoconstriction results from an exercise-induced increase in oxygen free radicals and a lack of urate, free radical scavengers in patients with severe hypouricemia due to URAT 1 mutation.
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  • Daishi Hirano, Satoshi Hara, Naoto Nishizaki, Hitohiko Murakami, Shuic ...
    2008 Volume 21 Issue 2 Pages 188-194
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     We report two cases of acute poststreptococcal glomerulonephritis (APSGN) with persistent renal function impairment developed in brothers. Both patients were admitted to a foster home as they were from a single-father family. No other child in the foster home developed APSGN. Although their symptoms were typical, early renal biopsies were carried out because it was not possible to prove the streptococcal infection in the elder brother, and renal function impairment rapidly progressed in the younger brother. Both of them were diagnosed as having APSGN with the findings of endocapillary proliferative glomerulonephritis under light microscopy and positive immunofluorescent staining for the nephritis-associated plasmin receptor. Their glomerular lesions show no findings necessitating aggressive treatments such as crescent formation or the `garland type', and favorable outcomes were accomplished with conservative treatments. Because proper management in the early phase is important in APSGN patients, early renal biopsy is thought to be useful for determining the treatment plan as well as long-term follow-up in cases of rapidly progressive nephritis or diagnostic difficulty in acute aggravation of chronic nephritis.
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  • Yuichiro Imai, Yoshiaki Harada, Junji Takaya, Kazunari Kaneko, Takashi ...
    2008 Volume 21 Issue 2 Pages 195-198
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Oculo- cerebro- renal syndrome of Lowe (Lowe syndrome), caused by mutation of OCRL1, is a rare, multisystem disorder characterized by bilateral cataracts, mental retardation, hypotonia and renal tubular abnormalities, whereas Dent disease, mainly caused by mutations in the chloride channel gene (CLCN5), is distinguished by exclusive renal tubular dysfunction resulting in low- molecular- weight proteinuria, hypercalciuria, nephrocalcinosis and urolithiasis.
     We encountered a 3 year-old-boy with asymptomatic tubular proteinuria and hypercalciuria. He was initially diagnosed as having Dent disease because there were no clinical and laboratory findings indicating Lowe syndrome, mitochondrial encephalopathy, interstitial nephritis or Fanconi syndrome. However, short stature, mental retardation and cataract, all of which were not observed at first visit, have developed since 6 years of age. Genomic DNA analysis for OCRL1 disclosed nonsense mutation while that for CLCN5 did not.
     In summary, mutation of OCRL1 should be considered and sought in children having asymptomatic tubular proteinuria who do not demonstrate extra- renal manifestations featuring Lowe syndrome.
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  • Atsumi Uemura, Misako Hiramatsu, Yasuyuki Akita
    2008 Volume 21 Issue 2 Pages 199-202
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Both urinary tract infection (UTI) and enuresis (nocturnal enuresis and urinary incontinence) are common issue on children and often caused by urogenital anomalies, so that we should carefully make a differential diagnosis and evaluation. We here present a case of a girl suffered from multiple UTI and enuresis with a left ectopic ureterocele, an urethrostenosis and a small left kidney.
     A 9-year-old girl was admitted to the emergency department for fever of 40.2°C with frequent urination, sense of residual urine and abdominal pain. Urinalysis exhibited an elevated count of white blood cells. She was administered on antibiotics for pyelonephritis and her complaints and the pyuria were resolved. She also troubled with enuresis totalis. We found that she had a left ureterocele and that her left kidney was small and hypoplastic. No vesicoureteral reflux was observed, but there was residual urine and she had a weak sense of urination compared to the intrabladder pressure.
     With a diagnosis of unstable bladder, distigmine bromide and prazosin hydrochloride were started, and the diurnal urinary incontinence disappeared. Because a breakthrough infection occurred, we started to give her prophylactic dose of cefaclor. Since then she had no UTI. A year later the nocturnal enuresis continued. An urethrostenosis was found with cystoscopy and an urethrotomy was performed. The left ureterocele was found to open ectopically at the neck of bladder. She does not receive medication any more for more than a year after the urethrotomy and she is free from nocturnal enuresis and UTI.
     If there is a therapy-resistant enuresis with a urogenital anomaly (anomalies), it should be recommended to administer cystography or other procedure available to reevaluate the status of the present anomaly (anomalies) and to make clear whether there would be other anomalies.
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  • Daisuke Ogino, Yuko Akioka, Hiroko Chikamoto, Masaki Hisano, Tae Ohmor ...
    2008 Volume 21 Issue 2 Pages 203-207
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     We report a 12-year-old girl with recurrent focal segmental glomerulosclerosis with cellular lesions developing soon after living-related renal transplantation. Her graft function progressively deteriorated until she finally developed end-stage renal disease. We studied the association of the plasma circulating factors with clinicopathologic features.
     We treated cultured podocytes with her plasma and examined the time-course of integrin-linked kinase (ILK) activity, alterations of focal contacts, and detachments of cultured podocytes from culture dishes coated with laminin-1. We observed an increase in ILK activity, focal contacts were disassembled, and some cultured podocytes were detached from the laminin matrix.
     We speculated that circulating toxic factors in her plasma induced an increase in podocyte ILK activity that promoted the detachment of podocytes from the GBM. Podocyte detachment might be associated with the formation of cellular lesions, massive proteinuria, and glomerulosclerosis, which all led to the progressive decline of her graft function.
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  • Toshiaki Suzuki, Yohei Ikezumi, Tamaki Karasawa, Kazuhide Saito, Kota ...
    2008 Volume 21 Issue 2 Pages 208-211
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
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     Splenectomy has been considered to be necessary for successful ABO-incompatible kidney transplantation. However splenectomy can lead to severe complications for children.
     We report here a case of 12-year old boy who received ABO-incompatible kidney transplantation without splenectomy using rituximab. We started MMF/MP desensitization a month before the transplantation. Two doses of rituximab were administered at 12 and 2 days before the transplantaition. It has already been 3 years since the successful transplantation without any severe side effects.
     The recovery of the B-cell population was much earlier than adults treated with same protocol, however, it may be still over dose for children since only a few weeks of immunosuppression is required for the acquisition of immunological accommodation. Further investigations are necessary to determine a suitable dosage of rituximab for children.
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  • Machiko Nakano, Hidehiko Yanagida, Keisuke Sugimoto, Shinsuke Fujita, ...
    2008 Volume 21 Issue 2 Pages 212-216
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
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     We described an adolescent with renal-coloboma syndrome (RCS) showing developmental delay. Birth and perinatal histories were unremarkable. At the age of 7 years, proteinuria was detected in an annual mass screening program at the patient's elementary school, and his urine had been checked periodically at local hospital. Because of the increase in proteinuria, he was referred to our hospital for further clinical evaluation. Proteinuria was moderate, ranging from 1.0 to 1.5 g/day, and was coupled with mild renal dysfunction (creatinine clearance, 77.4 ml/min/1.73m2). At this time he was found out to have myopia associated with astigmatism. He exhibited mild developmental delay assessed by WISC-III test. Renal biopsy specimen showed marked glomerular enlargement, collapse of glomerular capillaries, mesangial matrix expansion, and tubulointerstitial change, demonstrating typical histologic features of RCS. Optic nerve coloboma was also evident. Genetic analysis revealed novel mutation of exon 3 in Pax2 (P130H) gene in our patient.
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  • Kenichi Suka, Shuji Kondo, Sato Matsuura, Masanori Takamatsu, Maki Uru ...
    2008 Volume 21 Issue 2 Pages 217-222
    Published: November 15, 2008
    Released on J-STAGE: July 10, 2009
    JOURNAL FREE ACCESS
     Bilateral multicystic dysplastic kidney (MCDK) can cause Potter's sequence and most of them are fatal in utero. Here, we report a rare case of bilateral MCDK who remains alive and well 14 months later.
     Her mother had been diagnosed as diabetes mellitus and took insulin treatment, however her level of fasting plasma glucose was not controlled. Antenatal sonography at 18 week gestation confirmed that both kidneys contained several cysts of varying sizes. Liquor volume was normal. However, the liquor volume decreased at 33 week gestation, and the baby was born by caesarean delivery. Urine and blood examinations, including serum creatinine levels, remained within the normal range.
     Postnatal sonography and enhanced-MRI revealed that the right kidney had the typical appearances of MCDK with multiple non-communicating cysts. Similarly, the left kidney comprised several cysts of varying sizes, however renal parenchyma was observed in the left. A static scintigraphy with 99 mtechnetium-dimercaptosuccinic acid (DMSA) scan showed the preserved function within the lower moiety of the left kidney as well as a non-functioning right kidney. Therefore, the left kidney was diagnosed as segmental MCDK.
     Her renal function became deteriolated, so peritoneal dialysis was commenced at 4 months of age. She continues to thrive and is doing well at 14 months old. Her long-term survival appears to be caused by the preserved left renal function. Because her mother had diabetes mellitus, it is possible that the occurrence of MCDK might be associated with the sustained exposure to hyperglycemia in uterine. In addition, it might be caused by genetic disorder, for example, the TCF-2 gene mutation which is a cause of MODY type 5.
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