Japanese journal of pediatric nephrology
Online ISSN : 1881-3933
Print ISSN : 0915-2245
ISSN-L : 0915-2245
Volume 28, Issue 1
Displaying 1-14 of 14 articles from this issue
Reviews
  • Shori Takahashi
    2015 Volume 28 Issue 1 Pages 1-5
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    There are two possible pathways of reabsorbing filtered proteins in the renal tubular cells. One is receptor mediated system and the other is cationic charge preferential interaction between cationic protein molecules and the polyanionic tubular brush border. Recently, the receptor mediated reabsorption systems have been clarified such as multi-ligand receptor “Megalin-Cubilin complex” and neonatal Fcγ receptor. However, cationic charge preferential interaction needs to be proven. The brush border of the renal proximal tubules has a polyanionic charge. Since IgG molecules have a wide range of charge diversity, reabsorption of urinary IgG molecules are supposed to be influenced by the electrostatic interaction. We investigated the cationic charge preferential interaction system by analyzing the charge diversities of serum and urinary IgG. Here, I will show our results to prove the cationic charge preferential reabsorption in renal proximal tubules and illustrate the mechanisms of tubular protein reabsorption.
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  • Masaki Shimizu
    2015 Volume 28 Issue 1 Pages 6-11
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    Enterohemorrhagic Escherichia coli (EHEC) produce Shiga toxins (STx). These toxins not only can induce cell death by inhibition of protein synthesis but also implicate a role for activation of proinflammatory cytokines and chemokines. These cytokines and chemokines can enhance STx induced cytotoxicity by enhancement of the expression of globotriaosylceramide (Gb3), the receptor of STx. Furthermore, these cytokines induce inflammatory cells infiltration. In this way, proinflammatory cytokines play an important role in the pathogenesis of EHEC infection and development of severe complications, including hemolytic uremic syndrome (HUS) and encephalopathy. It is desired to establish a monitoring system with useful clinical markers for predicting severe clinical outcomes in patients with HUS. In this review, I introduce our studies for clinical significance of serum cytokine profiles in HUS and clinical usefulness of apheresis therapy for HUS.
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Original Articles
  • Norio Omori, Ryugo Hiramoto, Tomohiko Yamamura, Shinsuke Matsumoto, Hi ...
    2015 Volume 28 Issue 1 Pages 12-17
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    We report a retrospective study of the safety and efficacy of combination therapy of Cyclosporine A (CsA) and Mizoribine (MZR) for children with frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS). The subjects were 20 patients with FR-SDNS treated with combination therapy of CsA and MZR in this retrospective analysis. Only one patient experienced mild hyperuricemia, known to be one of the most common side effects of mizoribine. No other adverse effects were noted in the rest of the patients. Nine out of 20 patients treated with CsA only for at least 6months followed by the combination therapy of CsA and MZR for at shortest 6months were analyzed for total prednisolone (PSL) dose and incidence of relapse to compare before and after the combination therapy. The treatment with combination therapy over a period of 10.1 months (median) resulted in significant reduction of the mean prednisolone dose from 0.37±0.28 to 0.15±0.15 mg/kg/day (p=0.016) and the median 6-month relapse rate from 1.7±0.9 to 0.8±0.7 episodes/6months (p<0.01). The combination therapy of CsA and MZR could be one of treatment choices for patients with FR-SDNS
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  • Masayuki Sato, Kenta Takahashi, Moyu Horii, Kaori Nakanishi, Eiki Naka ...
    2015 Volume 28 Issue 1 Pages 18-23
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    We routinely perform renal ultrasound screening for congenital anomalies of the kidney and urinary tract (CAKUT) in all infants who undergo a health check-up in at 1 month of age. To evaluate the efficacy of our screening program, we analyzed the results of renal ultrasound examinations that had been performed over the past 8 years. Screening of 3521 infants yielded 134 positive cases (3.8%). Of these, 120 cases had renal pelvis dilatation with an anteroposterior diameter > 7.0 mm. Most of these cases resolved spontaneously before the age of 1 year. The remaining 14 infants had other anomalies, including renal hypoplasia and crossed renal ectopia. Among all infants with abnormal findings, a diagnosis was confirmed in 20 cases (0.57%). In 12 of these 20 cases, no anomaly had been detected during prenatal screening. Given that these results are similar to those of previous reports, our findings indicate that renal ultrasound screening of 1-month-old infants is effective for early detection of congenital anomalies of the kidney and urinary tract.
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  • Hitoshi Wakaki, Kazuetsu Mori, Hiroshi Hataya, Kenji Ishikura, Masatak ...
    2015 Volume 28 Issue 1 Pages 24-31
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    The health-related quality of life (QOL) in patients with chronic renal disease carry-over case was assessed using SF-36 and other questionnaire. The evaluation of mental QOL was intentionally low in the group which had received restriction of physical activity as a result. This study suggests that restriction of physical activity influences a patient’s future QOL, and medical staff should be aware that the restriction should be minimized.
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  • Shinsuke Matsumoto, Ryugo Hiramoto, Norio Omori, Hironobu Eguchi, Buns ...
    2015 Volume 28 Issue 1 Pages 32-36
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    Although there are some reports that cyclosporine A (CsA) is effective for Henoch-Schönlein purpura nephritis (HSPN), there are a few reports in Japan and the effectiveness of CsA for HSPN remains to be elucidated. The objects of our study were 6 patients with refractory HSPN, who satisfied the diagnostic criteria of nephrotic syndrome and suffered from lasting heavy proteinuria even after starting the treatment including prednisolone (PSL). We added CsA for these patients and analyzed the effectiveness. The average age was 6.9 years old, and the average administration period of CsA was 2.6 years. Proteinuria disappeared and pathological findings of kidney were improved in all the 6 cases. CsA could be effective enough under the following conditions of the early initiation of CsA treatment and the usage of both PSL and ACE inhibitor together. The safe and appropriate period of using CsA was considered to be 2 years with the target trough level of 50–90 ng/ml. Although it is necessary to appropriately choose which HSPN case should be treated with CsA, CsA can surely be one of the immunosuppressants to use for refractory HSPN accompanied with lasting heavy proteinuria after the treatment including PSL.
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  • Hiroko Nagata, Akio Furuse, Shinji Irie, Kyoko Kanou, Tatsuya Kawasaki ...
    2015 Volume 28 Issue 1 Pages 37-42
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    This study retrospectively investigated the annual incidence, first-line antibiotic therapy, and risk factors for extended-spectrum β-lactamase (ESBL)-producing upper urinary tract infection (UUTI). The 166 cases of UUTI at our hospital from April 2009 to March 2013 were divided into 2 groups: UUTI due to ESBL-producing bacteria (group A, 28 cases), and UUTI due to non-ESBL-producing bacteria (group B, 138 cases). The bacteriuria in group A consisted of Escherichia coli (25 cases), Klebsiella oxytoca (2 cases), and K. pneumoniae (1 case). In both group A and B, the annual incidence of UUTI increased during the study period. Treatment antibiograms in group A showed that cefmetazole (CMZ), meropenem (MEPM), flomoxef (FMOX), imipenem/cilastatin sodium (IPM/CS), and amikacin (AMK) had 100% sensitivity. Twelve cases of ESBL-UUTI were resistant to the selected antibiotics. However, those patients were treated without changing antibiotics. The mean duration of intravenous antibiotic therapy in group A was significantly longer than in group B (8.0 vs. 6.0 days; P=0.001). There were no significant differences in age, sex, congenital anomalies of the kidney urinary tract or the number of cases treated with prophylactic antibiotics between groups. Our data suggest that the annual incidence of ESBL-UUTI is increasing; and in cases of UUTI, antibiotics against ESBL-producing bacteria should be considered.
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Case Reports
  • Maki Shimizu, Yukiko Kinoshita, Tetsuro Furumoto, Yuko Ichihara, Keisu ...
    2015 Volume 28 Issue 1 Pages 43-49
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    Familial juvenile hyperuricemic nephropathy (FJHN) is an autosomal dominant disease characterized by hyperuricemia of the underexcretion type, impairment of urinary concentration and tubulointerstitial nephritis leading to chronic renal failure. In 2002, it was revealed that FJHN is caused by mutation of the uromodulin (UMOD) gene located on chromosome 16p11–13. Here, the proband was a 14-year-old boy referred to our hospital because of renal insufficiency (creatinine 1.33 mg/dℓ). His father had been affected by gout and chronic renal failure since the age of 26 years, and treated with peritoneal dialysis since he was 35 years old. Renal biopsy of the proband showed severe tubulointerstitial injury with sclerotic glomeruli. Additionally, he presented with the complications of hyperuricemia (uric acid 9.4 mg/dℓ) of the underexcretion type and impairment of urinary concentration. To confirm the diagnosis, the UMOD gene was analyzed and a heterozygous missense mutation (c.281 G>T [C94F]) in exon 3 of UMOD of both the proband and his father was identified. This mutation has not been reported previously. In conclusion, we report a 14-year-old boy and his father with FJHN due to a novel mutation, highlighting the importance of FJHN as a differential diagnosis in autosomal dominant renal insufficiency.
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  • Hitomi Nishi, Megumi Nagai, Takuji Enya, Tomoki Miyazawa, Shinsuke Fuj ...
    2015 Volume 28 Issue 1 Pages 50-54
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    Henoch-Schönlein purpura nephritis (HSPN) is the major complication of Henoch-Schönlein purpura. In the case of the intractable condition of the resistance for corticosteroid therapy, no second-line therapeutic strategy has not been established at present. We encountered a 2-year-old infant who is resistance for the cocktail therapy using corticosteroid including methylpredonisolone pulse therapy, immunosuppressants including cyslophosphamide, cyclosporine A, and mizoribine combined with plasmapheresis. Regarding such intractable condition, mycophenolate mofetil was given, resulting in the incomplete remission of nephrotic condition. The infant is the youngest reported patient showing severe HSPN of ISKDC IV. The infant also showed heavy nephrotic range of proteinuria and intractable hypertension involving posterior reversible encephalopathy syndrome. Since histologic findings are unusual involving the survival of extraordinary number of the premature glomeluruli, the subsequent failure of glomerular maturation leading to the glomerular sclerosis is deeply concerned. We are afraid of the renal function would be worsen in near future in this infant.
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  • Shinichiro Ohara, Yukihiko Kawasaki, Kazuhide Suyama, Atsushi Ono, Syu ...
    2015 Volume 28 Issue 1 Pages 55-59
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    Wunderlich syndrome is a rare anomaly affecting the Mullerian and Wolff ducts, and consisting of vaginal duplication, uterus didelphy and ipsilateral renal agenesis. The clinical manifestation is progressive menstrual pain with dysmenorrhea. We report the case of an 11-year-old female. After birth, ultrasound examinations revealed right renal agenesis and she was thereafter followed from infancy. Abdominal magnetic resonance (MR) imaging at 11 years of age showed uterus didelphy, an obstructed hemivagina, and right renal agenesis. Although she had experienced no abdominal symptoms after the onset of menstruation, the MR images clearly demonstrated uterus didelphys with hematocolpos due to an obstrucution on one side of the vagina. She underwent surgery in which the vaginal septum was incised and hematocolopos drained. She has experienced no postoperative complications to date, has regular menstruation and remains asymptomatic. Due to the difficulty in diagnosis during infancy, the existence of uterine didelphy and obstructed hemivagina should be taken into consideration in cases of ipsilateral renal agenesis. We suggest MR imaging is necessary to clarify complications in adolescent girls examined for ipsilateral renal agenesis.
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  • Yukiko Mori, Soichi Tamamura, Kenta Yamada, Sosuke Ooto, Yasuhiro Wata ...
    2015 Volume 28 Issue 1 Pages 60-67
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    A 3-month-old female infant was admitted to our hospital because of convulsions and anuria. Routine laboratory investigation showed renal dysfunction, electrolyte abnormalities, and advanced metabolic acidosis, consistent with end-stage renal disease (ESRD). An abdominal computed tomography scan revealed bilateral renal cortex calcification suggestive of primary hyperoxaluria type 1 (PH1). Since she was anuric, blood oxalic acid and glycolic acid levels were measured using gas chromatography/mass spectrometry. Both oxalic acid and glycolic acid were elevated. We detected a homozygous nonsense mutation in the AGXT gene, and diagnosed PH1. When a patient with PH1 develops ESRD, dialysis cannot excrete the oxalic acid produced in the body, resulting in oxalosis. Infantile PH1 has a particularly poor prognosis, and as such, we considered liver transplantation immediately. The facilities capable of performing liver transplantation in Japan are limited, necessitating early referral. At 7 months of age, the patient received a segmental liver transplant from the mother. Although it may be considered difficult to continue peritoneal dialysis (PD) after abdominal surgery, PD has been continued postoperatively in this patient, without complication. This patient will be followed up and will receive renal transplantation therapy.
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  • Etsuko Tanaka, Takao Konomoto, Hideaki Imamura, Mayuko Orita, Hiromi S ...
    2015 Volume 28 Issue 1 Pages 68-74
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    Hemolytic uremic syndrome (HUS) results from thrombotic microangiopathy following injury to vascular endothelial cells. Notably, the involvement of apoptosis has been considered to be one of the etiologies of HUS. Cytochrome c is a marker of apoptosis and has a positive correlation with the severity of acute encephalopathy. However, there have been no reports regarding HUS cases with or without encephalopathy. Here we report a case of HUS caused by enterohemorrhagic Escherichia coli O157 in a 4-year-old girl, in which serum cytochrome c levels were evaluated during different phases of acute encephalopathy. Her serum cytochrome c levels were high and fluctuated followed by neurological status. Additionally, we evaluated the association between serum cytochrome c levels and clinical features in 9 patients with HUS. Serum cytochrome c levels were higher in HUS cases complicated with encephalopathy and/or acute kidney injury with an estimated glomerular filtration rate under 20 ml/min/1.73 m2. Our study suggests that apoptosis contributes to the pathophysiology of HUS, and that cytochrome c might be a useful marker for assessing its severity, especially in HUS cases with encephalopathy or acute kidney failure requiring dialysis. Further studies with a larger number of patients are required to clarify these associations.
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  • Dai Suzuki, Naonori Kumagai, Makiko Nakayama, Hiroki Kudo, Takaaki Kig ...
    2015 Volume 28 Issue 1 Pages 75-80
    Published: 2015
    Released on J-STAGE: October 15, 2015
    JOURNAL FREE ACCESS
    We experienced a patient with Lowe Syndrome in whom acute kidney injury failed to improve and advanced to end stage renal failure. The patient now requires maintenance hemodialysis with a long-term indwelling catheter. In this case, hemodialysis was initiated with the expectation that the patient would recover from the acute kidney injury. Unfortunately, the nephropathy advanced to end stage renal disease, forcing maintenance hemodialysis to be continued. It is rare that maintenance hemodialysis is used in cases of Lowe syndrome complicated by end stage renal disease, and this is the first case of a patient with Lowe syndrome receiving this treatment in Japan. The failure in recovery from acute kidney injury and early arrival at the end stage of renal disease in this case may be attributable to the presence of kidney fragility associated with Lowe syndrome.
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