Japanese journal of pediatric nephrology
Online ISSN : 1881-3933
Print ISSN : 0915-2245
ISSN-L : 0915-2245
Volume 20, Issue 1
Displaying 1-19 of 19 articles from this issue
Original Article
  • Shuichiro Fujinaga, Yoshiyuki Ohtomo, Daisuke Umino, Satoshi Hara, Mas ...
    2007 Volume 20 Issue 1 Pages 1-8
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     Although a long-term prognosis of children with malignancies has improved by the advances in medicine, these patients occasionally develop acute renal failure (ARF) requiring continuous renal replacement therapy (CRRT) during the course of a disease. We retrospectively assessed the clinical course of 15 patients (mean age 8.7 years) treated with CRRT. Of the 15 patients, 10 survived an initial ARF episode. However, remaining 5 patients died during CRRT. In order to clarify the factors associated with increased mortality, the clinical characteristics of both survivors and non-survivors were analysed. Age at the start of CRRT, body weight, duration of CRRT, urinary volume before CRRT, glomerular filtration rate, hemoglobin and platelets counts did not correlate with survival. We found lager number of organ failure (p<0.01), presence of sepsis (p<0.05) and lower white blood cell counts (p<0.05) were significantly associated with greater mortality. The development of pump-driven volumetric-control CRRT machines with small extra corporeal volumes has lead to the safety use of CRRT even in children with hemodynamic instability. Our data suggest that patients with ARF in multiple organ failure due to sepsis under leukopenia have a high probability of death.
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  • —bio-availability varies with age—
    Osamu Uemura, Katsumi Ushijima, Takuji Yamada, Yuri Kimpara
    2007 Volume 20 Issue 1 Pages 9-13
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     There is not a strong correlation between dosage and blood concentration of mizoribine, an immunosuppressive agent used for pediatric kidney diseases such as nephrotic syndrome. Therefore, we considered that there are differences in the bioavailability of mizoribine between individuals. In addition, the quantity of urinary excretion for mizoribine is thought to be equal to that of intestinal absorption, because it is excreted by the kidney without sustained metabolism of all of the mizoribine absorbed in the bowel. We gave pediatric kidney disease patients mizoribine and examined the blood concentration and quantity of urinary excretion. The urinary excretion rate was 32.2±19.3% (n=15, 8.7%∼86.5%) for 24 hours. Ibara reported a urinary excretion rate of 55.4±17.7% (30.0%∼79.8%) in adults with rheumatoid arthritis. There was a marked difference between their findings and our results, which we attributed to lower intestinal absorption rates in children than in adults. There was a correlation (R2=0.60, p=0.0008) between AUCinf (dosage was converted to 1mg/kg) and urinary excretion rate (%). There was also a correlation (R2=0.31, p=0.03) between age and urinary excretion rate. It is thought that one of the causes of the differences between individuals in bioavailability of mizoribine is age-related, and it seems that high dose mizoribine administration is required to obtain an adequate blood concentration in younger children.
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  • Kaori Yoneda, Akio Furuse, Seishi Seguti, Satoru Komaki, Tomoyasu Kawa ...
    2007 Volume 20 Issue 1 Pages 15-19
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     We have reported 9 cases with multicystic dysplastic kidney (MCDK), diagnosed 5 case in prenatal period, 2 cases in neonatal period and 3 case in 3 months' infantile examination. The mean observation period after diagnosed was 40 months. Multi-cystic lesions followed by ultra-sonography (US) disappeared in 2 cases in this period. Abnormality of the contralateral kidney was complicated in 2 out of 9 cases, which was hydronephrosis with pyelo-ureteral junction (PUJ). 7 cases without abnormalities of the contralateral kidney and 1 case with PUJ showed normal∼over estimated renal function by DTPA renal scintigraphy and compensatory hypertrophy by DMSA renal scintigraphy. One case with PUJ and frequent UTI showed renal impairment. MCDK is diagnosed in prenatal period by US and imaging techniques. The majority of MCDK undergo spontaneous resolution during the follow-up period. There is a possibility that as before MCDK has been handled as solitary kidney. Early, especially in prenatal period and accurate diagnosis of MCDK by imaging techniques, and long term follow-up studies are necessary.
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Review
  • Kandai Nozu, Masafumi Matsuo, Kazumoto Iijima
    2007 Volume 20 Issue 1 Pages 21-25
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
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  • Masafumi Hasui, Anna Iharada, Shoji Tsuji, Katue Funaki, Yuichiro Imai ...
    2007 Volume 20 Issue 1 Pages 26-29
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     Nitric oxide (NO) is a major form of the endothelium-derived relaxing factor. Recently, its role in the pathogenesis of renal diseases including idiopathic nehphrotic syndrome (INS) has been intensively investigated. However, its rapid turnover has made us to investigate the quantity and the source difficult. With a novel fluorescent indicator for NO, we have developed the methodology to measure the NO production by diverse cells and studied the NO production by lymphocytes in INS: the patients with INS showed significantly elevated NO production by both T and B lymphocyte in comparison with children with other renal diseases and healthy adult volunteers. In contrast, there were no significant differences among children with INS after achieving remission, children with other renal diseases and normal healthy volunteer.
     These findings indicate that NO plays an important role in the pathogenesis of INS and abnormal immune system in INS may exist not only in T lymphocytes but also in B lymphocytes and other immunological cells.
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Case Report
  • Yasushi Utsunomiya, Hitoshi Sano, Hideyuki Ohtani, Masami Togawa, Akik ...
    2007 Volume 20 Issue 1 Pages 31-34
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     We reported a 14 years old girl with TINU syndrome. At the onset of uveitis, she presented renal glycosuria, pyuria, and insufficiency of urinal concentration. Elevated serum creatinine level and urinal β2 microglobulin (β2MG) excretion were also found in the laboratory test. The renal biopsy showed significant inflammatory cell infiltration in the interstitium but no morphological change of glomeruli, which findings were considered those of acute interstitial nephritis. As the result of informed consent to the patient, we decided to observe the natural course without giving systemic steroid therapy. Then the serum creatinine level and urinary β2MG excretion decreased gradually and recovered within the normal range after 8 months.
     The renal symptom of this case was improved without systemic steroid therapy. This case suggests that we should observe the renal symptom of TINU syndrome without aggressive systemic steroid therapy, as long as any of rapid deterioration of renal function or systemic symptom is not present.
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  • Kyoko Kanda, Kandai Nozu, Hiroshi Kaito, Masafumi Matsuo
    2007 Volume 20 Issue 1 Pages 35-37
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
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  • Takuhito Nagai, Yoshimitsu Gotoh
    2007 Volume 20 Issue 1 Pages 38-41
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     Pathological findings of nephronophthisis-medullary cystic kidney disease complex at 14 years old girl with normal renal function were obtained. She had only mild proteinuria, no renal concentrating disability, no extrarenal disease and congenital history. Renal pathology showed significant thickening and breaking of tubular basement membrane, interstitial fibrosis. In the patient many mitochondria was abnormally aggregated in tubular epithelial cell. That meaning was not clear, but these mitochondria may have an intimate involved in formation of pathogenic condition in tubular basement membrane alteration. We will track to renal sufficiency and analyzing gene mutation.
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  • Akio Yamada, Kiyotaka Usui, Yosiko Nagano, Motohiro Shibata, Kazuo Tsu ...
    2007 Volume 20 Issue 1 Pages 42-47
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     Juvenile nephronophthisis combined with retinitis pigmentosa is known as renal-retinal dysplasia (Senior-Loken Syndrome). Recently several genes responsible for juvenile nephronophthisis have been identified. Juvenile nephronophthisis can be distinguished according to the onset-age of renal failure (infantile, juvenile and adolescent type) and causative genes.
     We report here of a 3 year old girl who presented with anemia, poor physical activity, and visual impairment. Blood examination revealed that she had renal failure. She was diagnosed as Senior-Loken Syndrome by renal biopsy. It is difficult to screen this disease by the routine urinary examinations taken at the 3-Year-Old health check and also in the Elementary School Urinary Screening Program. An infant with visual impairment of unknown etiology should be undergo a urinary specific gravity and a β2-microglobulin test for early diagnosis of this disease.
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  • Kazumi Okamura, Masanori Takamatsu, Maki Urushihara, Shoji Kagami
    2007 Volume 20 Issue 1 Pages 48-51
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     A 14-year-old girl was admitted because of proteinuria and hematuria that had persisted one year before. The serum BUN and creatinine level were increased and a high titer of ANCA was detected. Renal biopsy specimens revealed fibro-cellular crescentic glomerulonephritis with sclerosis. After steroid therapy combined with cyclophosphamide, mizoribin, dipyridamole, heparin and warfarin, renal function and pathological findings were improved. This present case demonstrates that early diagnosis and treatment are very important in the ANCA associated nephritis.
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  • Toshiaki Suzuki, Yohei Ikezumi, Tamaki Karasawa, Keisuke Nagasaki, Yuk ...
    2007 Volume 20 Issue 1 Pages 52-54
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     We report on a 12-year-old girl with posttransplant diabetes mellitus (PTDM) who had a family history of type 2DM.
     She received TAC-based immunosuppression as the pretransplant OGTT showed normal glucose tolerance. She was managed with insulin therapy for 2 months after the onset PTDM and able to discontinue insulin therapy after conversion to CyA. We believe that conversion to CyA is effective to early presentation of PTDM and one should consider avoiding the use of tacrolimus in patients with a family history of Type 2DM.
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  • Tatsuyuki Sokoda, Toshihiro Sawai, Masaru Iwai, Yasuyuki Nomura, Yoshi ...
    2007 Volume 20 Issue 1 Pages 55-59
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     We reported a case of acute tubulointerstitial nephritis (ATIN) with acute renal failure.
     The patient was an 8-year-old boy, who was pointed out autism at age of 3 years old and had no previous history of influenza infection. In November 2005, at age of 8 years and 10 months, he was given influenza HA vaccine for the first time. 3 days later, he presented with high grade fever. He was admitted to a hospital with persistent fever of unknown cause. 14 days after vaccination, He progressed to acute renal failure, and then was transferred to our institution. The laboratory tests revealed the following values: white blood cells count 10,900/cmm, C-reactive protein 26.7mg/dl, serum creatinine 5.61mg/dl, urinary beta 2 micro globlin 21,061μg/g creatinine. Continuous hemodiafiltration was performed for seven days, and the renal function recovered. Renal biopsy results were compatible with ATIN. A drug lymphocyte stimulation test (DLST) was positive for influenza HA vaccine. Therefore, influenza HA vaccine was suspected the cause of ATIN. To our knowledge, no previous case of ATIN associated with influenza HA vaccine has been reported.
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  • Tetsuo Mori, Takahumi Nishimura, Ei Shimazaki, Kan Tooyama, Toshiyuki ...
    2007 Volume 20 Issue 1 Pages 60-66
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     We report a girl of idiopathic chyluria who was found in school urinary screening programme at the age of 10 years. It took one year from the finding of hematuria and proteinuria in school urinary screening programme to confirm the prescence of chyluria. One of the reasons of the delay of confirmation of chyluria, is that hematuria, proteinuria and pyuria were exacerbated in the morning urine, but improved in the day urine. The confirmation of chyluria was done by ether adding test, Sternheimer stein and Prescott-Brodie stein. Lymphoscintigraph with Tc-99m-HAS-DTPA showed very well the site of the fistula, right renal pelvis and dilated lymphatics on the right side. She had not any symptoms, any treatments and the chyluria disappeared spontaneously after 4 yeares of the finding of chyluria.
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  • Kyoko Takahagi, Toshihiro Sawai, Hiroshi Fujito, Noriko Bamba, Tatsuya ...
    2007 Volume 20 Issue 1 Pages 67-70
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     We report a 14-year-old-boy with recurrent poststreptococcal acute glomerulonephritis (PSAGN).
     On 17 November 2003 (at the age of 11y10mo), he was brought to hospital because of facial edema, macrohematuria and dyspnea. His blood pressure was 170/130mmHg. Laboratory data showed depression of complement C3 44mg/dl (norma l86-160mg/dl) and mild renal insufficiency. He was diagnosed as PSAGN, and improved in a short time by intravenous administration of furosemide. Four weeks later, urinary abnormalities had subsided completely. In June 2006 (at the age of 14y5m), he had a sore throat, which subsided without any medication. On 10 June 2006, he was brought to hospital and diagnosed as PSAGN again. Therefore, he was referred to our hospital and renal biopsy was performed. Light microscopic examination showed diffuse and global endocapillary proliferation. Immunofluorescense showed diffuse and granular deposition of C3. Because of histological findings compatible with PSAGN, no specific therapy was perfomed. Four months later, his laboratory data were normal except for mild proteinuria (less than 0.5g/day).Recurrence of PSAGN is a rare phenomenon. While the mechanism leading to the recurrence of PSAGN has not yet been identified, Watanabe et al suggested that an absence of a natural immune response against a streptococcal cytoplasmic antigen (nephritis-associated plasmin receptor protein, NAPlr) caused recurrent PSAGN in some patients. We consider that present case is similar to their report.
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  • Hirotaka Kihara, Toshiyuki Ohta, Rie Fukuhara, Shin Fujiwara, Kougoro ...
    2007 Volume 20 Issue 1 Pages 71-76
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     We report a case of neonatal septic shock due to gastric rupture treated with PMX-direct hemoperfusion (PMX-DHP). A healthy G2P1 mother delivered a 3,215g male neonate at 38 weeks gestation. He was admitted to our hospital because of frequent vomiting of bloody gastric juice, abdominal distension, and free air of abdominal cavity. He was in bad condition and revealed significant hyperammonemia and high endotoxin concentration. He underwent continuous hemodiafiltration (CHDF) leading to mild decrease of plasma ammonia level, but the low blood pressure was not improved. There was a significant increase in blood pressure immediately after initial of PMX-DHP and his clinical condition improved remarkably. No complication was observed with this therapy. At laparotomy, spontaneous gastric rupture was confirmed and repaired. The effectiveness of PMX-DHP in clinical experience was reported in adults, but few in neonatal cases. We consider PMX-DHP to be an effective therapeutic option for infants with severe sepsis.
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  • Nihoko Ito, Kayoko Umemura, Yasuto Yamamoto, Masashi Morooka, Yoshizo ...
    2007 Volume 20 Issue 1 Pages 77-81
    Published: April 15, 2007
    Released on J-STAGE: November 15, 2007
    JOURNAL FREE ACCESS
     The patient is a 13 year-old girl who had glucosuria in the course of enuresis. Though she had continuous glucosuria, her blood sugar and HbA1c levels were normal. However, as her serum creatinine and urine β2MG were high, tubulointerstitial nephritis was suspected. Although she had no eye symptoms, she was diagnosed with bilateral uveitis. A renal biopsy specimen showed typical tubulointerstitial nephritis, and she was also diagnosed with TINU syndrome. Her urinalysis and serum creatinine level improved without specific agents like prednisolone. It is reported that most cases of TINU syndrome recover spontaneously unlike other types of tubulointerstitial nephritis. Therefore, when we encounter renal glucosuria, it is important to consider TINU syndrome even if no eye symptoms exist.
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