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


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Article Info

DATE

2020-08-24

AUTHORS

Yuko Hamasaki, Riku Hamada, Masaki Muramatsu, Shinsuke Matsumoto, Kunihiko Aya, Kenji Ishikura, Tetsuji Kaneko, Kazumoto Iijima

ABSTRACT

BackgroundCongenital 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.MethodsThis 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.ResultsA 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.ConclusionsThe 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 registrationNot applicable. More... »

PAGES

363

References to SciGraph publications

Journal

TITLE

BMC Nephrology

ISSUE

1

VOLUME

21

Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/s12882-020-02010-5

DOI

http://dx.doi.org/10.1186/s12882-020-02010-5

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1130306759

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/32838745


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40 schema:description BackgroundCongenital 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.MethodsThis 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.ResultsA 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.ConclusionsThe 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 registrationNot applicable.
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46 schema:keywords BackgroundCongenital nephrotic syndrome
47 CNS
48 Denys-Drash syndrome
49 Finnish type
50 Japan
51 ResultsA total
52 accord
53 bilateral nephrectomy
54 characteristics
55 characteristics of children
56 children
57 common renal replacement therapy
58 complete remission
59 cross-sectional nationwide survey
60 current treatment recommendations
61 data
62 diagnosis
63 diagnosis of CNS
64 dialysis
65 disease
66 disease type
67 early progression
68 end-stage kidney disease
69 epidemiological survey
70 examination
71 extra-renal symptoms
72 frequency
73 frequent disease type
74 genetic examination
75 genetic testing
76 group
77 high frequency
78 higher proportion
79 immunosuppressants
80 infantile nephrotic syndrome
81 infection
82 institutions
83 kidney disease
84 knowledge
85 lack
86 lack of knowledge
87 large placenta
88 light
89 mail
90 management
91 means
92 mental retardation
93 morbidity
94 mortality
95 nationwide survey
96 nephrectomy
97 nephrotic syndrome
98 outcomes
99 patient management
100 patients
101 peritoneal dialysis
102 placenta
103 postal mail
104 practice
105 present epidemiological survey
106 primary outcome
107 progression
108 proportion
109 questionnaire
110 recommendations
111 remission
112 renal replacement therapy
113 replacement therapy
114 retardation
115 steroids
116 study
117 substantial morbidity
118 survey
119 survey questionnaire
120 symptoms
121 syndrome
122 testing
123 therapy
124 thrombosis
125 total
126 treatment
127 treatment recommendations
128 type disease
129 types
130 unilateral nephrectomy
131 urogenital symptoms
132 years
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