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

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuko Hamasaki ◽  
Riku Hamada ◽  
Masaki Muramatsu ◽  
Shinsuke Matsumoto ◽  
Kunihiko Aya ◽  
...  
2020 ◽  
Author(s):  
Yuko Hamasaki ◽  
Riku Hamada ◽  
Masaki Muramatsu ◽  
Shinsuke Matsumoto ◽  
Kunihiko Aya ◽  
...  

Abstract 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.


2020 ◽  
Author(s):  
Yuko Hamasaki ◽  
Riku Hamada ◽  
Masaki Muramatsu ◽  
Shinsuke Matsumoto ◽  
Kunihiko Aya ◽  
...  

Abstract 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.


2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Deng Yan ◽  
Wang Yan-Fang ◽  
Zhu Shi-Yang ◽  
Ma Rui-Lin ◽  
Ding Xue-Song ◽  
...  

Abstract Background To describe the diagnostic criteria used and their application accuracy in the practice of polycystic ovary syndrome (PCOS) caring among obstetricians and gynaecologists across China. Methods This was an Online cross-sectional survey of Obstetricians and gynecologists involved in PCOS caring conducted via the largest continuing education platform of obstetrics and gynecology across China from September 2019 to November 2019. Results A total of 2,328 respondents were eligible for the final analysis. Of these, 94.5 % were general obstetricians and gynaecologists (Ge-ObGyn), and 5.5 % were reproductive endocrinologists (Re-ObGyn). Overall, the most frequently used criteria were the Androgen Excess and Polycystic Ovary Syndrome Society (AE-PCOS) criteria (48.2 %), followed by the Rotterdam criteria (35.7 %) and NIH criteria (12.1 %). Of the respondents, 31.3 % used their diagnostic criteria in their clinical practice. More respondents who chose the Rotterdam criteria could accurately apply the diagnostic criteria than those who chose the AE-PCOS criteria (41.2 % vs. 32.1 %, P < 0.001). Compared with Ge-ObGyn, Re-ObGyn were less likely to use the AE-PCOS criteria (adjusted odds ratio, 0.513; 95 % CI, 0.328–0.802; P < 0.05) and 1.492 times more likely to accurately use their criteria (95 % CI, 1.014–2.196; P < 0.05). Conclusions Less than one-third of obstetricians and gynaecologists across China could accurately use the diagnostic criteria they choose to diagnose PCOS. There is an urgent need to train obstetricians and gynaecologists on PCOS diagnosis in an effort to improve the medical care quality of patients with PCOS.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Przemyslaw Kardas ◽  
Marek Dabrowa ◽  
Konrad Witkowski

Abstract Background Due to high prevalence, non-adherence to prescribed treatment seriously undermines the effectiveness of evidence-based therapies in paediatric patients. In order to change this negative scenario, physicians need to be aware of adherence problem, as well as of possible solutions. Unfortunately, full potential of adherence-targeting interventions is still underused in Poland. Therefore, the aim of this study was to assess the knowledge, attitudes and behaviours toward non-adherence in Polish paediatricians. Methods An anonymous cross-sectional nationwide survey was conducted in the convenience sample of Polish doctors providing care to paediatric patients. The survey focused on the prevalence of non-adherence, its causes, and interventions employed. Primary studied parameter was perceived prevalence of non-adherence in paediatric patients. Reporting of this study adheres to STROBE guidelines. Results One thousand and thirty-three responses were eligible for analysis. Vast majority of respondents were female (85.9%), most of them worked in primary care (90.6%). The respondents represented all 16 Polish Voivodeships, with the biggest number coming from the Mazowieckie Voivodeship (n = 144, 13.9%). Survey participants believed that on average 28.9% of paediatric patients were non-adherent to medication. More than half of the respondents (n = 548, 53.0%) were convinced that their own patients were more adherent than average. Duration of the professional practice strongly correlated with a lower perceived prevalence of non-adherence. Professionals with more than 40 years of practice believed that the percentage of non-adherent patients was <=20% particularly often (OR = 3.82 (95% CI 2.11–6.93) versus those up to 10 years in practice). Out of all respondents, they were also most often convinced that their own patients were more adherent than the general population (P < 0.01). Consequently, they underestimated the need for training in this area. Conclusions Physicians taking care of Polish paediatric patients underestimated the prevalence of medication non-adherence and believed that this was a problem of other doctors. This optimistic bias was particularly pronounced in older doctors. These results identify important barriers toward improving patient adherence that are worth addressing in the pre- and post-graduate education of Polish physicians. They also put some light over the challenges that educational activities in this area may face.


BMJ Open ◽  
2019 ◽  
Vol 9 (3) ◽  
pp. e023401 ◽  
Author(s):  
Fernanda G Duarte ◽  
Sandra da Silva Moreira ◽  
Maria da Conceição C Almeida ◽  
Carlos A de Souza Teles ◽  
Carine S Andrade ◽  
...  

ObjectiveExamine whether glycaemic control varies according to sex and whether the latter plays a role in modifying factors associated with inadequate glycaemic control in patients with type 2 diabetes (T2D) in Brazil and Venezuela.Design, setting and participantsThis was a cross-sectional, nationwide survey conducted in Brazil and Venezuela from February 2006 to June 2007 to obtain information about glycaemic control and its determinants in patients with diabetes mellitus attending outpatient clinics.Main outcome measuresHaemoglobin A1c (HbA1c) level was measured by liquid chromatography, and patients with HbA1c ≥7.0% (53 mmol/mol) were considered to have inadequate glycaemic control. The association of selected variables with glycaemic control was analysed by multivariate linear regression, using HbA1c as the dependent variable.ResultsA total of 9418 patients with T2D were enrolled in Brazil (n=5692) and in Venezuela (n=3726). They included 6214 (66%) women and 3204 (34%) men. On average, HbA1c levels in women were 0.13 (95% CI 0.03 to 0.24; p=0.015) higher than in men, after adjusting for age, marital status, education, race, country, body mass index, duration of disease, complications, type of healthcare, adherence to diet, adherence to treatment and previous measurement of HbA1c. Sex modified the effect of some factors associated with glycaemic control in patients with T2D in our study, but had no noteworthy effect in others.ConclusionsWomen with T2D had worse glycaemic control than men. Possible causes for poorer glycaemic control in women compared with men include differences in glucose homeostasis, treatment response and psychological factors. In addition, sex modified factors associated with glycaemic control, suggesting the need to develop specific treatment guidelines for men and women.


Author(s):  
C.-Y. Lee ◽  
Y. Sun ◽  
H.-J. Lee ◽  
T.-F. Chen ◽  
P.-N. Wang ◽  
...  

Background: Evidence of the associations of dietary habits and body mass index with dementia is inconsistent and limited in East Asian countries. Objective: We aim to explore the associations of dietary habits and body mass index with the odds of dementia. Design: Cross-sectional observational study. Setting: A nationwide, population-based, door-to-door, in-person survey. Participants: Selected by computerized random sampling from all 19 counties in Taiwan. Measurement: Diagnosis of dementia using the criteria recommended by the National Institute on Aging-Alzheimer’s Association. Lifestyle factors, dietary habits and demographic data were compared between normal subjects and participants with dementia. Results: A total of 10432 residents were assessed, among whom 2049 were classified as having a mild cognitive impairment (MCI), 929 were diagnosed with dementia, and 7035 were without dementia or MCI. After adjustment for age, gender, education, body mass index (BMI), dietary habits, habitual exercises and co-morbidities, including hypertension, diabetes and cerebrovascular diseases, we found inverse associations of dementia with the consumption of fish (OR 0.62, 95% CI 0.41-0.94), vegetables (OR 0.35, 95% CI 0.13-0.95), coffee (OR 0.59, 95% CI 0.35-0.97), green tea (OR 0.51, 95% CI 0.34-0.75) and other types of tea (OR 0.41, 95% CI 0.28-0.60). There was no association between dementia and fruit consumption. Compared with people who had a normal BMI (18 < BMI <= 24), older overweight people (24 < BMI <=30) had a reduced risk of dementia with an adjusted OR of 0.77 (95% CI 0.61-0.96). Conclusions: Our study provides preliminary evidence that suggests that the consumption of fish, vegetables, tea, and coffee has potential benefits against dementia in East Asian population. Being modestly overweight (nadir risk at BMI = 25) in late life was associated with decreased odds of dementia. The benefit of fruits may be offset by their high sugar content.


2018 ◽  
Vol 5 (4) ◽  
pp. 84
Author(s):  
Katie Waine ◽  
Rachel S. Dean ◽  
Chris Hudson ◽  
Jonathan Huxley ◽  
Marnie L. Brennan

Clinical audit is a quality improvement tool used to assess and improve the clinical services provided to patients. This is the first study to investigate the extent to which clinical audit is understood and utilised in farm animal veterinary practice. A cross-sectional study to collect experiences and attitudes of farm animal veterinary surgeons in the UK towards clinical audit was conducted using an online nationwide survey. The survey revealed that whilst just under three-quarters (n = 237/325; 73%) of responding veterinary surgeons had heard of clinical audit, nearly 50% (n = 148/301) had never been involved in a clinical audit of any species. The participants’ knowledge of what a clinical audit was varied substantially, with many respondents reporting not receiving training on clinical audit at the undergraduate or postgraduate level. Respondents that had participated in a clinical audit suggested that protected time away from clinical work was required for the process to be completed successfully. This novel study suggests that clinical audit is undertaken to some extent in farm animal practice and that practitioner perception is that it can bring benefits, but was felt that more resources and support were needed for it to be implemented successfully on a wider scale.


2018 ◽  
Vol 29 (5) ◽  
pp. 816-820
Author(s):  
Hitomi Suga

Abstract Background A household’s food unavailability due to financial constraints may act as a barrier to a healthy diet for children. This cross-sectional study aimed to examine the association between food unavailability due to financial constraints and the nutrient intake of children aged 1–15 years using data of a nationwide survey among the Japanese population. Methods Datasets from the National Health and Nutrition Survey and the Comprehensive Survey of Living Conditions in Japan, 2014 were used for this study. The dietary intakes of energy and nutrients were estimated using a one-day dietary record. Participants were categorized into four subgroups, according to their households’ frequency of food unavailability due to financial constraints. The adjusted means and 95% confidential intervals of the energy and nutrient intakes in each subgroup were calculated using analysis of covariance. The statistical trends across the subgroups were examined using analysis of covariance. Results Of the 895 children included in the analysis, 202 (22.6%) were classified into subgroups with frequent food unavailability due to financial constraints. Significant decreases in the mean intakes of carbohydrate, calcium, retinol activity equivalents, riboflavin, folate and vitamin C were observed with increases in the frequency of households’ food unavailability. In contrast, frequent food unavailability was associated with higher total fat and vitamin B12 intake. Conclusion A substantial proportion of children in Japan experienced food unavailability due to financial constraints, and this affected their nutrient intake. Adopting a population approach may help overcome this problem.


2019 ◽  
Vol 39 (1) ◽  
Author(s):  
Roberta da Silva Filha ◽  
Sérgio Veloso Brant Pinheiro ◽  
Thiago Macedo e Cordeiro ◽  
Victor Feracin ◽  
Érica Leandro Marciano Vieira ◽  
...  

AbstractIntroduction: Renin angiotensin system (RAS) plays a role in idiopathic nephrotic syndrome (INS). Most studies investigated only the classical RAS axis. Therefore, the aims of the present study were to evaluate urinary levels of RAS molecules related to classical and to counter-regulatory axes in pediatric patients with INS, to compare the measurements with levels in healthy controls and to search for associations with inflammatory molecules, proteinuria and disease treatment. Subjects and methods: This cross-sectional study included 31 patients with INS and 19 healthy controls, matched for age and sex. Patients and controls were submitted to urine collection for measurement of RAS molecules [Ang II, Ang-(1-7), ACE and ACE2] by enzyme immunoassay and cytokines by Cytometric Bead Array. Findings in INS patients were compared according to proteinuria: absent (<150 mg/dl, n = 15) and present (≥150 mg/dl, n = 16). Results: In comparison to controls, INS patients had increased Ang II, Ang-(1-7) and ACE, levels while ACE2 was reduced. INS patients with proteinuria had lower levels of ACE2 than those without proteinuria. ACE2 levels were negatively correlated with 24-h-proteinuria. Urinary concentrations of MCP-1/CCL2 were significantly higher in INS patients, positively correlated with Ang II and negatively with Ang-(1-7). ACE2 concentrations were negatively correlated with IP-10/CXCL-10 levels, which, in turn, were positively correlated with 24-h-proteinuria. Conclusion: INS patients exhibited changes in RAS molecules and in chemokines. Proteinuria was associated with low levels of ACE2 and high levels of inflammatory molecules.


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