We present the case of a one-year-old male patient with infantile primary hyperoxaluria type 1 (PH1). The patient visited hospital because of growth delay and poor feeding when he was six months old, and was diagnosed as PH1 with chronic renal failure. He underwent peritoneal dialysis until receiving a living-related liver transplantation when he was seventeen months old, and after the operation, underwent hemodialysis or hemodiafiltration four times per week. Six months after the liver transplantation, his serum oxalate level decreased to around 20 micromol/l and a living-related kidney transplantation was successfully performed. Nine months have passed since the kidney transplantation, and the patient's liver and kidney functions have been good and his growth and development much better than before the sequential liver and kidney transplantation. However, his serum and urine oxalate levels remained high and he has required high dose hydration to prevent deposition of calcium oxalate crystals in his grafted kidney. The key-points for treating infantile PHI patients are summarized as follows; 1) make a precise diagnosis as soon as possible, 2) perform a combined liver-kidney transplantation successfully, 3) conduct careful monitoring of the serum and urine oxalate levels and continue adequate hydration after kidney transplantation until the serum and urine oxalate levels normalize. Furthermore, cooperation between the medical staff and the patient's family seems to be essential.

The aim of the present study is to clarify the improvement of peripheral muscle oxygen consumption after successful renal transplantation. We investigated change of forearm (brachioradial muscle) muscular oxygen consumption in chronic renal failure children before and after renal transplantation. by using near-infrared spectroscopy.Oxygen consumption of brachioradial muscle was increased significantly after succesful renal transplantation. And half recovery time of brachioradial muscle oxygenation in arterial occlusion and exercise were decreased after renal transplantation.These results suggest that increased muscular blood flow and increased oxidative generation of ATP might contribute to the increased oxygen turn over after renal transplantation.

We report the case of a 4.5-year-old girl with microscopic polyangiitis (MPA) manifesting antineutrophil cytoplasmic autoantibody (ANCA)-positive necrotizing crescentic glomerulonephritis and pulmonary hemorrhage. She was initially on induction therapy with corticosteroids and azathioprine. Plasma exchange (PE) combined with immunosuppressants was used to treat an episode of recurrent pulmonary hemorrhage, and achieved remission. At 9.8 years of age her kidney disease relapsed, associated with renal dysfunction and increased proteinuria. To minimize the toxic effects of immunossuppressants, she was treated with PE again, and her renal dysfunction resolved. Plasma exchange was effective in reducing the risk of death and preserving long-term renal function without the severe adverse effects of immunosuppressants. Our preliminary results indicate that PE is likely to be a treatment option for children in acute phase of ANCA-associated MPA, who should be protected from the toxic effects of immunosuppressants.

In the present study, we reviewed the effect of post-transplant double filtration plasmapheresis (DFPP) on recurrent focal segmental glomerulosclerosis (FSGS) in the transplanted kidney allograft. Sixteen patients with post-transplant recurrent FSGS were enrolled in this study. Out of 16 patients with recurrent FSGS after transplantation, five did not receive DFPP and lost their grafts, while 11 did receive DFPP and four of these patients lost their grafts. Seven patients were able to maintain normal renal function for an average observation period of 57.1 +/- 40.7 months (range 7-125 months). In five patients who had a significant reduction in urinary protein after DFPP, the urinary protein level decreased from 26.60 +/- 23.05 g/day (range 3.34-62.6 g/day) to 2.95 +/- 3.42 g/day (range 0.02-8.64 g/day) and renal function was maintained. The beneficial effects of DFPP on graft outcome were more likely to occur if the patients experienced a marked drop in urinary excretion. Thus, post-transplant DFPP appears to be effective for reducing urinary protein levels and improving long-term graft survival. With the small numbers in this trial, however, none of the findings were statistically significant. We recommend the use of post-transplant DFPP to prevent the progression of recurrent FSGS.

BACKGROUNDTreatment of steroid-resistant (SR) primary focal segmental glomerulosclerosis (FSGS) remains a major challenge in nephrology. A prospective study was conducted to clarify the therapeutic role of low-density lipoprotein apheresis (LDL-A) in 11 nephrotic children with SR and cyclosporine A (CsA)-resistant primary FSGS.METHODSBased on entry criteria, all 11 eligible patients had biopsy-proven primary FSGS presenting with nephrotic syndrome (NS) and were resistant to steroid and conventional-dose CsA therapy. LDL-A was performed twice a week for 3 weeks (first course), then weekly for 6 weeks (second course). Beginning from the second LDL-A course, a dosage of 1 mg/kg/d of prednisone was administered for 6 weeks, then tapered.RESULTSSeven patients experienced remission of NS, 5 of whom achieved complete remission within 4 weeks after initiating prednisone therapy with LDL-A. These 5 patients maintained normal renal function during follow-up (median, 4.4 years). Of 2 patients with partial remission, 1 patient maintained stable renal function during follow-up (4.5 years), whereas the other patient showed a gradual decline in renal function and progressed to end-stage renal failure (ESRF) 7.8 years after LDL-A therapy. Four patients who were considered to experience treatment failure had persistent NS and progressed to ESRF in 1.3 years (median) after LDL-A therapy. Complete remission (n = 5) was associated with significantly more highly selective proteinuria compared with treatment failure (n = 4).CONCLUSIONThis study suggests that combined LDL-A and prednisone therapy can be a valuable addition to therapeutic options for treating patients with SR-FSGS. The role of LDL-A in treating these patients deserves to be assessed further in larger randomized controlled trials.

NephrologyVolume 8, Issue s4 p. A115-A115 A clinicopathological study of recurrent IgA nephropathy following renal transplantation in children Hiroko CHIKAMOTO, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorMotoshi HATTORI, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorShigeru HORITA, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorToshihiro SAWAI, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorTae OHMORI, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorDaisuke OGINO, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorHyogo NAKAKURA, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorSanpei MIYAKAWA, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorYutaka YAMAGUTI, Department of Pathology, Tokyo Jikei Medical University, School of Medicine, Tokyo, JapanSearch for more papers by this authorKatsumi ITO, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this author Hiroko CHIKAMOTO, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorMotoshi HATTORI, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorShigeru HORITA, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorToshihiro SAWAI, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorTae OHMORI, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorDaisuke OGINO, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorHyogo NAKAKURA, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorSanpei MIYAKAWA, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this authorYutaka YAMAGUTI, Department of Pathology, Tokyo Jikei Medical University, School of Medicine, Tokyo, JapanSearch for more papers by this authorKatsumi ITO, Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine,Search for more papers by this author First published: 12 January 2004 https://doi.org/10.1111/j.1440-1797.2003.00199.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume8, Issues4January 2003Pages A115-A115 RelatedInformation

A 15-year-old boy with chronic renal failure secondary to Alport’s syndrome underwent living-related renal transplantation from his 48-year-old father. His primary immunosuppressive regimen was composed of tacrolimus, mizolibine, and methylprednisolone. The postoperative course was satisfactory with one episode of mild acute rejection, treated successfully with methylprednisolone pulse therapy. Two months later, hypercalcemia (11.8–13.2 mg/dl) and hypophosphatemia (2.5–3.0 mg/dl) were noted without any bone symptoms. The serum intact-parathyroid hormone (PTH) and serum alkaline phosphatase levels were 240 pg/ml and 2483 IU/l, respectively. Ultrasound studies revealed enlargement of the two parathyroid glands. Under the diagnosis of ter-tiary hyperparathyroidism, he underwent percutaneous ethanol injection (PEIT) into the left parathyroid gland. Although levels of serum calcium and phosphorus returned to normal ranges and the intact PTH level decreased to 95 pg/ml with the three injections, another injection was needed to normalize recurrent hypercalcemia 2 months later. The patient experienced only transient mild dysphonia and local pain after PEIT. Although PEIT is believed less effective than parathyroidectomy, it has some advantages such as applicability to high-risk patients, repeatability of treatment, low incidence and severity of side effects.

Aspergillus peritonitis is a rare, potentially fatal complication of continuous ambulatory peritoneal dialysis (CAPD). We report the successful treatment of refractory fungal peritonitis in an 8-year-old girl treated by peritoneal dialysis for 3.3 years. This is the second report of Aspergillus thermomutatus(telemorph: Neosartorya pseudofischeri) in humans. Comprehensive treatment included early removal of the CAPD catheter, the use of liposomal amphotericin B, and the use of itraconazole.

Premature infants often present metabolic acidosis without protein load in the early neonatal period, around days 4-6. In order to elucidate the cause of acidosis, we investigated urinary acidification of infants in the early neonatal period. Urine pH, fractional excretion of HCO(3)(-) (FEHCO(3)), excretion of HCO(3)(-) and NH(4)(+) of the appropriate-for-date infants were measured on days 0-2 and on days 4-6 of life. Extremely low birth weight (ELBW) infants showed higher urine pH than more than 1500 g birth weight infants. FEHCO(3) and HCO(3)(-) excretion were of high values in ELBW infants on days 0-2, but decreased on days 4-6. Urine NH(4)(+) excretion rate was lower in ELBW infants than in birth weight more than 1000 g on days 0-2 of life and still remained at a low rate on days 4-6. These data indicated that insufficiency of NH(4)(+) excretion is the main cause for metabolic acidosis of ELBW infants in the early neonatal period.

小児慢性腎不全患者における維持血液透析 (HD) ではブラッドアクセスが大きな問題である. 今回, 小児維持HD患者における長期留置用ダブルルーメンカテーテルの有用性を明らかにする目的で, Hickman Catheter® (Bard社) 使用群とPerm Cath® (Quinton社) またはHEMO-CATH® (Medcomp社) カテーテル使用群間で合併症の種類やその頻度について比較検討したので報告する. 16例の小児慢性腎不全患者 (平均年齢6.2歳, 平均体重14.1kg) で使用された合計19本のカテーテル (Hickman Catheter® 12本, Perm Cath® 2本, そしてHEMO-CATH® 5本) を対象とした. Hickman Catheter®では他の2種に比べて脱血不良や返血圧上昇が, 一方, Perm Cath®やHEMO-CATH®ではカテーテル内血栓, 出口部感染, そしてカテーテルの押出しが合併症として問題であった. 平均留置期間は5.2か月 (0.8-19か月) で, 4本は菌血症や脱血不良にて抜去されたが, 13本のカテーテルは腎移植や腹膜透析への移行のために選択的に抜去され, 残り2本は現在も使用中である. 以上の結果より, 小児のブラッドアクセスとして長期留置用ダブルルーメンカテーテルは有用であるが, その使用に際しては, カテーテルの特性を十分に考慮することが重要と思われた.

Hemolytic uremic syndrome(HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute nephropathy. Clinical features and outcome of children with HUS initiated by infections with Shiga toxin(Stx)-producing strains of Escherichia coli(E. coli) infection are different from those of patients with the other forms of HUS or thrombotic thrombocytopenic purpura(TTP). Childhood Stx-E. coli-associated HUS usually recovers spontaneously and dose not require specific treatments including plasma therapy. In contrast, a general consensus has been achieved that plasma exchange or infusion should always be tried in adult HUS/TTP to minimize the risk of death or long-term sequelae. In this paper, we briefly reviewed therapy for patients with Stx-E. coli-associated HUS.

原発性巣状糸球体硬化症 (FSGS) の組織学的バリアントのひとつであるcellular lesionを, 病初期高度に認めた男児例に対して, LDL吸着療法を行い完全寛解が得られたので報告する. 症例は全身性の浮腫で発症し, ネフローゼ症候群を呈した11歳男児で, 腎生検にて原発性FSGS cellular variantと診断した. 経過中ステロイド抵抗性を示したため, LDL吸着療法を開始したところ完全寛解が得られた.FSGS cellular variantは典型的FSGSと比較して, 腎機能予後は明らかに不良とされている. しかし一方では, 寛解が得られた場合, その予後は良好であったと報告されている. そのためFSGS cellular variantに対する治療上, 寛解を目指して適切な治療を行うことが重要であるが, 本症例の経験より, LDL吸着療法を早期から積極的に実施する意義は大きいと考えられた.

症例は8歳女児で, 原発性巣状糸球体硬化症を原疾患として95年7月に腹膜透析導入となった. 98年11月にアスペルギルス腹膜炎 (起炎菌は真菌のrRNA解析よりAspergillus (Neosartorya) fennelliaeと同定) を発症し, 途中空腸壁の穿孔を合併した. 直ちに, CAPDカテーテルを抜去するとともに, 従来のnon-lipid Amphotericin B (AMPH-B) よりも副作用が少なく投与量の増量が可能であるliposomal AMPH-Bや, 胃酸の作用を必要とせず腸瘻からの投与が可能なItraconazole (ITCZ) oral solutionを導入, さらに適切な栄養管理 (経静脈栄養に空腸人工肛門肛門側からの経腸栄養を併用) など集学的治療にて救命し得た.アスペルギルス腹膜炎は稀な疾患であるが致死的であり, 早期診断と適切な治療が必要であるため, 今回使用したIiposomal AMPH-Bが本邦でも臨床使用できることが望まれる.