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Publications (26)
Background:
Proteinuria is broadly classified into glomerular and tubular proteinuria. Urinary beta-2-microgobulin (β2-MG) is known as a marker for detecting tubulointerstitial diseases. However, tubulointerstitial damage can also lead to an increase in urinary β2-MG level in some patients with glomerular diseases. This study aimed to determine th...
Background:
The Kidney Disease: Improving Global Outcomes (KDIGO) guidelines suggest initially using angiotensin-converting-enzyme inhibitors (ACE-Is) and/or angiotensin receptor blockers (ARBs) to treat Henoch-Schönlein purpura nephritis (HSPN). However, these guidelines might overlook the potential benefits of aggressive therapy. Therefore, we e...
Background
Eculizumab was approved for atypical haemolytic-uremic syndrome (aHUS) in Japan in 2013. Post-marketing surveillance (PMS) was mandated by regulatory authorities to assess the safety and effectiveness of eculizumab in patients with aHUS in the real-world setting.
Methods
Paediatric patients in the PMS cohort who were < 18 years old at f...
Among children with spina bifida, more than 90% of cases with spina bifida aperta and approximately 40% with spina bifida occulta present with lower urinary tract dysfunction, which occasionally causes renal impairment. In this retrospective study, we aimed to investigate the clinical characteristics associated with renal impairment in children wit...
Mycophenolate mofetil is effective for the treatment of pediatric idiopathic nephrotic syndrome (INS). The dosage of mycophenolate mofetil is adjusted according to the serum concentration of mycophenolic acid (MPA). Kidney function or cyclosporine (CsA) concentrations affect serum MPA levels. However, few studies have focused on the association bet...
Background
Ravulizumab, a humanized anti-complement C5 monoclonal antibody designed by targeted modification of eculizumab to achieve an extended half-life, is approved to treat aHUS in the USA (2019), EU and Japan (2020). Data at 26 weeks (wk) and 1 year (yr) from the phase 3 studies of ravulizumab in adults and children with aHUS have been publis...
Background
Some pediatric patients on maintenance dialysis may need end-of-life care in the future because of being excluded from the indication of kidney transplantation and experiencing difficulty in continuation of their dialysis. This study aimed to thoroughly elucidate mortality outcomes of children on maintenance dialysis including the cause...
Introduction
WT1 missense mutation in exon 8 or 9 causes infantile nephrotic syndrome with early progression to end-stage kidney disease (ESKD), Wilms’ tumor, and 46,XY female. However, some patients with missense mutations in exon 8 or 9 progress to ESKD in their teens or later. Therefore, we conducted a systematic review and functional analysis o...
A Correction to this paper has been published: https://doi.org/10.1007/s00467-020-04874-z
Background
Atypical hemolytic uremic syndrome (aHUS) is a rare, complement-mediated disease associated with poor outcomes if untreated. Ravulizumab, a long-acting C5 inhibitor developed through minimal, targeted modifications to eculizumab was recently approved for the treatment of aHUS. Here, we report outcomes from a pediatric patient cohort from...
Renal tubular dysgenesis (RTD) is the absence or poor development of the renal proximal tubules caused by gene mutations in the renin-angiotensin system. Although RTD has been considered fatal, improving neonatal intensive care management has enhanced survival outcomes. However, little has been reported on the survival of extremely preterm infants....
Mutations in the TTC21B gene have been identified in patients with nephronophthisis and were recently found in some patients with focal segmental glomerulosclerosis. We herein report a Japanese boy with end-stage renal disease due to medullary polycystic kidney disease and primary focal segmental glomerulosclerosis. Next-generation sequencing detec...
Eculizumab, a humanized monoclonal antibody to complement C5, is a therapeutic drug for atypical hemolytic–uremic syndrome (aHUS) that inhibits the terminal pathway of complement. Patients on eculizumab therapy may become more susceptible to infection with capsule-forming bacteria, including meningococci. Therefore, meningococcal vaccination is req...
Large‐dose cyclic tidal peritoneal dialysis (TPD) is an original prescription of TPD involving frequent infusion and drainage of the dialysate to increase weekly urea clearance normalized to total body water (Kt/Vurea) and fluid removal. This study aimed to evaluate the efficiency of solute clearance and fluid removal achieved with large‐dose cycli...
Alport syndrome (AS) is a progressive hereditary renal disease that is characterized by sensorineural hearing loss and ocular abnormalities. It is divided into three modes of inheritance, namely, X-linked Alport syndrome (XLAS), autosomal recessive AS (ARAS), and autosomal dominant AS (ADAS). XLAS is caused by pathogenic variants in COL4A5, while A...
To determine the optimal method of evaluating kidney function in patients with thyroid dysfunction, this study compared the estimated glomerular filtration rate derived from serum creatinine, cystatin C, or β2-microglobulin with inulin or creatinine clearance in two pediatric patients, one with hypothyroidism and the other with hyperthyroidism. It...
The short D4Z4 repeat on chromosome 4q35 is a confirmatory genetic cause of facioscapulohumeral muscular dystrophy (FSHD), which presents with no renal complications. We herein report a five-year-old girl previously diagnosed with Coat's-like retinopathy, deafness, and mental retardation, who was found to have early-onset, severe FSHD. Despite the...
Background:
The suitable dosage regime of mycophenolate mofetil (MMF) based on the pharmacokinetics of mycophenoric acid (MPA) for pediatric patients with idiopathic nephrotic syndrome (INS) is controversial. The pharmacokinetics of MPA is influenced by renal function, serum albumin concentration, and concomitant medications, especially calcineuri...
Thromboembolism is one of the serious complications of pediatric idiopathic nephrotic syndrome (NS). We report the case of a 4-year-old boy suffering from steroid-resistant NS complicated with posterior reversible encephalopathy syndrome (PRES), acute kidney injury and multiple vein thrombosis. Although his symptoms with PRES subsequently improved...
Background
This retrospective study was performed to assess the three-year outcome of a unified protocol for childhood idiopathic nephrotic syndrome.Methods
Cyclosporine A (CsA) or CsA plus mycophenolate mofetil (MMF) were administered in cases without remission on high-dose steroid therapy.CsA was maintained at an area under the whole blood concen...
抄録
症例は6歳の女児。4歳8か月時，肉眼的血尿，蛋白尿，腎機能障害のため入院となった。C3，C4は正常，ANCAや抗GBM抗体は陰性であり，光顕上尿細管間質障害を伴う半月体形成性糸球体腎炎像を呈し，蛍光抗体法では糸球体係蹄壁へC3のみ沈着していたこと，などから特発性半月体形成性糸球体腎炎と診断した。一時的に血液透析が必要となったが，ステロイドパルス療法により腎機能や尿所見は徐々に改善し，カクテル療法に移行した。5歳11か月時の腎生検上では，活動病変は認めず，プレドニソロン(PSL)を中止した。しかし4か月後よりC3優位の低補体血症を伴う尿異常が再出現し，保存的治療でも改善がないため，3回目の腎生検を施行した。初回と同様，重度な分節性壊死性腎炎像であったが，Humpも認め，初回腎生検の電顕上...
腎不全医療を行っているわれわれの最終目標は，患児を一般の社会人として自立させることにある。腎代替療法の一つである腎移植医療の進歩はその目標を容易にした。われわれは1987年より腎移植を行っており，19歳以上で当院外来でフォローを行っている患者は24例いる。そのうち，仕事を持たずに自宅で生活している患者は，うつ病で精神科医に受診している1例だけであった。学生を除いた就職年齢の患者は17例であり，失業率は5.8%となり，同年齢の完全失業率が6.4～9.4%であることから比べ，遜色のない就業率であった。何らかの資格を持っている症例5例は，全例その資格に関する職業に就いていた。4名は結婚しており，男性の2名は父親となっている。腎移植は確立した医療であるとともに，社会生活についても有用である。
