Long-term outcome of congenital nephrotic syndrome after kidney transplantation in Japan

Hamasaki, Yuko; Muramatsu, Masaki; Hamada, Riku; Ishikura, Kenji; Hataya, Hiroshi; Satou, Hiroyuki; Honda, Masashi; Nakanishi, Koichi; Shishido, Seiichiro

doi:10.1007/s10157-017-1508-4

Cited by 11 publications

(4 citation statements)

References 25 publications

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“…The main reason for this preference is that the management of patients after unilateral nephrectomy is easier than that after bilateral nephrectomy because dialysis for patients with anuria is more di cult. In a recent retrospective study conducted in Japan, the long-term outcome data from 14 Finnish-type CNS Japanese patients who underwent kidney transplantation showed satisfactory graft survival [26].…”

Section: Discussionmentioning

confidence: 99%

A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

Hamasaki

Hamada

Muramatsu

et al. 2020

Preprint

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Background: Congenital nephrotic syndrome (CNS) and infantile nephrotic syndrome (INS) cause substantial morbidity and mortality. In Japan, there is a lack of knowledge regarding the characteristics of CNS and INS. This study aimed to clarify the characteristics of CNS and INS in Japan.Methods: This cross-sectional nationwide survey obtained data from 44 institutions in Japan managing 92 patients with CNS or INS, by means of two survey questionnaires sent by postal mail. Patients aged <16 years by 1 April 2015, with a diagnosis of CNS or INS, were included in this study. The primary outcome was end-stage kidney disease.Results: A total of 83 patients with CNS or INS were analyzed. The most frequent disease type was non-Finnish (60.2%); 33 patients (39.8%) had Finnish type. Among those with non-Finnish-type disease, 26 had no syndrome and 24 had a syndrome, of which the most frequent was Denys–Drash syndrome (70.8%). Patients with non-Finnish-type disease with syndrome showed the earliest progression to end-stage kidney disease compared with the other two groups, whereas patients with non-Finnish-type disease without syndrome progressed more slowly compared with the other two groups. In the Finnish-type group, the disease was diagnosed the earliest; a large placenta was reported more frequently; genetic testing was more frequently performed (93.8%); mental retardation was the most frequent extra-renal symptom (21.2%); and thrombosis and infection were more frequent compared with the other groups. Patients with non-Finnish-type disease with syndrome had a higher frequency of positive extra-renal symptoms (79.2%), the most common being urogenital symptoms (54.2%). Treatment with steroids and immunosuppressants was more frequent among patients with non-Finnish-type disease without syndrome. Two patients with non-Finnish-type disease without syndrome achieved complete remission. In all groups, unilateral nephrectomy was performed more often than bilateral nephrectomy and peritoneal dialysis was the most common renal replacement therapy.Conclusions: The present epidemiological survey sheds light on the characteristics of children with CNS and INS in Japan. A high proportion of patients underwent genetic examination, and patient management was in accord with current treatment recommendations and practices.Trial registration: Not applicable.

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Section: Discussionmentioning

confidence: 99%

A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

Hamasaki

Hamada

Muramatsu

et al. 2020

Preprint

Self Cite

View full text Add to dashboard Cite

show abstract

“…The main reason for this preference is that the management of patients after unilateral nephrectomy is easier than that after bilateral nephrectomy because dialysis for patients with anuria is more difficult. In a recent retrospective study conducted in Japan, the long-term outcome data from 14 Finnish-type CNS Japanese patients who underwent kidney transplantation showed satisfactory graft survival [22].…”

Section: Discussionmentioning

confidence: 99%

A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

Hamasaki

Hamada

Muramatsu

et al. 2020

Preprint

Self Cite

View full text Add to dashboard Cite

Background: Congenital nephrotic syndrome (CNS) and infantile nephrotic syndrome (INS) cause substantial morbidity and mortality. In Japan, there is a lack of knowledge regarding the characteristics of CNS and INS. This study aimed to clarify the characteristics of CNS and INS in Japan.Methods: This cross-sectional nationwide survey obtained data from 44 institutions in Japan managing 92 patients with CNS or INS by means of two survey questionnaires sent by postal mail. Patients aged <16 years by 1 April 2015, with a diagnosis of CNS or INS, were included in this study. The primary outcome was end-stage kidney disease.Results: A total of 83 patients with CNS or INS were analyzed. The most frequent disease type was non-Finnish (60.2%); 33 patients (39.8%) had Finnish type. Among those with non-Finnish-type disease, 26 had no syndrome and 24 had a syndrome, of which the most frequent was Denys–Drash syndrome (70.8%). Patients with non-Finnish-type disease with syndrome showed the earliest progression to end-stage kidney disease compared with the other two groups, whereas patients with non-Finnish-type disease without syndrome progressed more slowly compared with the other two groups. In the Finnish-type group, the disease was diagnosed the earliest; a large placenta was reported more frequently; genetic testing was more frequently performed (93.8%); mental retardation was the most frequent extra-renal symptom (21.2%); and thrombosis and infection were more frequent compared with the other groups. Patients with non-Finnish-type disease with syndrome had a higher frequency of positive extra-renal symptoms (79.2%), the most common being urogenital symptoms (54.2%). Treatment with steroids and immunosuppressants was more frequent among patients with non-Finnish-type disease without syndrome. Two patients with non-Finnish-type disease without syndrome achieved complete remission. In all groups, unilateral nephrectomy was performed more often than bilateral nephrectomy and peritoneal dialysis was the most common renal replacement therapy.Conclusions: The present epidemiological survey sheds light on the characteristics of children with CNS and INS in Japan. A high proportion of patients underwent genetic examination, and patient management was in accord with current treatment recommendations and practices.Trial registration: Not applicable.

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“…Bilateral nephrectomy may also be done in preparation for a renal transplant and in patients with Denys-Drash syndrome because of a high risk for Wilms' tumor. Unilateral nephrectomy (6,13,26) along with anti-proteinuric agents is a viable alternative to bilateral nephrectomy (27)(28)(29), the major advantage being that it may decrease or stop the need for albumin infusions and facilitate outpatient management without an immediate need for RRT. However, in some patients, unilateral nephrectomy may not be enough, and second nephrectomy followed by dialysis may be needed (Supplementary Table 3).…”

Section: Discussionmentioning

confidence: 99%

Clinical presentation and management of nephrotic syndrome in the first year of life: A report from the Pediatric Nephrology Research Consortium

Constantinescu

Mattoo²,

Smoyer³

et al. 2022

Front. Pediatr.

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Background and objectivesNephrotic syndrome (NS) in the first year of life is called congenital (CNS) if diagnosed between 0–3 months, or infantile (INS) if diagnosed between 3–12 months of age. The aim of this study was to determine if there were clinically meaningful differences between CNS and INS patients, regarding clinical presentation, management and outcomes.Design, setting, participants, and measurementsEleven Pediatric Nephrology Research Consortium sites participated in the study, using IRB-approved retrospective chart reviews of CNS and INS patients born between 1998 and 2019. Data were collected on patient characteristics, pertinent laboratory tests, provided therapy, timing of unilateral/bilateral nephrectomy and initiation of renal replacement therapy (RRT).ResultsThe study included 69 patients, 49 with CNS and 20 with INS, with a median age at diagnosis of 1 and 6 months, respectively. Management for the two groups was similar regarding nutrition, thyroxin supplementation, immunoglobulin administration, and thrombosis prophylaxis. Within the first 2 months after diagnosis, daily albumin infusions were used more often in CNS vs. INS patients (79 vs. 30%; p = 0.006), while weekly infusions were more common in INS patients (INS vs. CNS: 50 vs. 3%; p = 0.001). During the 6 months preceding RRT, albumin infusions were more frequently prescribed in CNS vs. INS (51 vs. 15%; p = 0.007). Nephrectomy was performed more often in CNS (78%) than in INS (50%; p = 0.02). End-stage kidney disease tended to be more common in children with CNS (80%) vs. INS (60%; p = 0.09).ConclusionCompared to INS, patients with CNS had a more severe disease course, requiring more frequent albumin infusions, and earlier nephrectomy and RRT. Despite center-specific variations in patient care, 20–40% of these patients did not require nephrectomy or RRT.

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Long-term outcome of congenital nephrotic syndrome after kidney transplantation in Japan

Abstract: Long-term outcome in these 14 CNF patients showed satisfactory graft survival, improved height SD score, and favorable development. Although recurrent proteinuria after transplant was not predictive, it was associated with graft survival rate.

Cited by 11 publications

References 25 publications

A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

Clinical presentation and management of nephrotic syndrome in the first year of life: A report from the Pediatric Nephrology Research Consortium

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