Nephrotic Syndrome Children Research Articles

Incidence and Relapse Triggers of Childhood Idiopathic Nephrotic Syndrome between 2006 and 2016: A Population-Based Study in Fukushima, Japan.

Childhood idiopathic nephrotic syndrome (NS) is defined by proteinuria and hypoproteinemia. The incidence of childhood idiopathic NS varies with age, race, residential areas, and social conditions. In Japan, its incidence was estimated to be 6.49 cases/100,000 children. Our study aimed to investigate the incidence, characteristics, and rate of relapse of idiopathic NS in Fukushima between 2006 and 2016. Overall, 158 children aged from 6 months to 15 years old (65.8% male) developed idiopathic NS (median age at onset, 5.3 years). The peak age at onset was three years. The average annual incidence of childhood idiopathic NS was 5.16 (range, 3.47-9.26) cases/100,000 children. The highest incidence was in 2011, which was the year of the Great East Japan Earthquake and nuclear power plant accident, and reportedly caused psychological distress in the children at the time. Conversely, the five-year birth cohort showed minor difference from 2008 to 2012. The rate of incidence in males aged < 5 years was thrice greater than in females of the same age and almost the same for males and females aged 11-15 years. Of 507 total relapses in 115 NS children, common triggers of relapses were steroid discontinuation or reduction and infection. The average annual incidence of childhood NS based on the Fukushima population was lower than previously reported in Japan, and the annual incidence has changed over an 11-year period. These changes may be affected by social or environmental factors, including mental stress associated with lifestyle changes after the disaster.

Cognitive and Behavioral Aspects in Children with Nephrotic Syndrome; Does it Need Special Attention

Purpose: This study was done to see and assess the cognitive and behavioral impairment in children with nephrotic syndrome and to find out any relationship with disease-specific or treatment specific characteristics. Methods: This one-year case-control study was conducted from October 2018 to November 2019 among the diagnosed case of nephrotic syndrome, age range 3-16 year without an active stage of nephrosis or infection. Control groups were selected from age and sex-matched patients admitted in the same hospital after controlled active disease and without any chronic illness. The Wechsler Intelligence Scale for children (WISC) for assessment of Cognitive function was performed in both groups. Moreover, to assess behavioral impairment, the Strengths and Difficulties Questionnaire (SDQ) were performed in both groups. Results: Among the case group steroid dependency was noted with 58.1% and about 27% received for prolong period (&gt; 3months). A total cumulative dose before hospital admission was 384.16±206.94 mg/kg. All the domains of cognitive parameter and behavioral parameters affected significantly in nephrotic syndrome children than the control group(p&lt;0.05). Mostly affected cognition domains were verbal 72% and processing speed 74%. Considering behavioral parameters, conduct 44.2% and hyperactive 14.2% disorders were affected severely among these children. Both the cognitive and behavioral impairment were significantly affected by prolonged corticosteroid therapy(p&lt;0.0001). Conclusion: Severe psychosocial and emotional impairment are commonly seen among those children who are getting steroid for a prolonged period, which argues in favor of a low threshold for switching to sparing drugs.

Prevalence of NPHS2 gene R229Q polymorphism in Bangladeshi children with nephrotic syndrome

BackgroundLimited and contradictory pharmacogenetic studies of NPHS2 gene R229Q polymorphism in nephrotic syndrome (NS) children of different ethnicities steered us to investigate the genotype frequency and associated risk of this polymorphism in Bangladeshi NS children. MethodsA prospective case-control study was conducted which comprised a total of 142 children having nephrotic syndrome (NS), divided into 2 groups: case group consisted of 40 children with steroid-resistant nephrotic syndrome (SRNS), and control group involved 102 children with steroid-sensitive nephrotic syndrome (SSNS). Both were genotyped by using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method for R229Q polymorphism. ResultsThe results indicate the presence of R229Q polymorphism in 27.50% of SRNS and 12.75% of SSNS children. SRNS children possess 2.94-fold greater risk (p = 0.025) of carrying Arg/Gln genotype compared to SSNS children. Moreover, R229Q variant in SRNS children was observed as in a compound heterozygous form with p.Ala297Val located in exon 8. Age of onset (4–6 years) presents as a significant contributing factor (adjusted OR = 1.06; 95% CI = 1.023–1.094; p = 0.001) for SRNS susceptibility in Bangladeshi children. Contrarily, though the incidence of SRNS was higher in male children than female (80% vs 20%), gender remains to be a neutral factor (p = 0.257) in relation to SRNS susceptibility. ConclusionCompound heterozygosity of NPHS2 p.R229Q gene variant with p.Ala297Val may cause pathogenic SRNS in Bangladeshi children. Large scale studies are warranted to establish the genotype-phenotype correlation. It is recommended to screen for p.R229Q first and, if positive, for p.Ala297Val in Bangladeshi SRNS children.

Diagnostic value of urinary exosomal miR-23b-3p, miR-30a-5p, and miR-151-3p in children with primary nephrotic syndrome.

BackgroundPrimary nephrotic syndrome (NS) is a common disease of the urinary system with an unclear pathogenesis. We aimed to detect the levels of urinary exosomal miR-23b-3p, miR-30a-5p, and miR-151-3p in children with primary NS, and to explore their diagnostic value for NS.MethodsA total of 115 patients with NS who were admitted to the hospital from June 2017 to June 2019 were selected as the observation group. According to the disease progression, they were divided into an active group (acute active phase, n=68) and remission group (remission phase, n=47). In all, 50 healthy children were selected as the control group. Levels of urinary exosomal miR-23b-3p, miR-30a-5p, and miR-151-3p of each group in different periods were detected.ResultsThe 24-h urine protein, serum albumin (ALB), and serum total cholesterol (TC) levels were significantly higher in the observation group than in the control group (P<0.05), while those in the active group were significantly higher than those in the remission group (P<0.05). The levels of miR-23b-3p and miR-30a-5p were significantly higher in the observation group than in the control group, and significantly higher in the active group than in the remission group (P<0.05). No miR-151-3p was detected in the urinary exosomes of the two groups. After treatment, levels of exosomal miR-23b-3p and miR-30a-5p in the two groups both decreased significantly (P<0.05). Results of receiver operating curve (ROC) curve analysis showed that urinary exosomal miR-23b-3p and miR-30a-5p can be used to identify children with NS and healthy children. The area under the ROC curve (AUC) was 0.711 for miR-23b-3p and 0.844 for miR-30a-5p.ConclusionsThe levels of miR-23b-3p and miR-30a-5p in urinary exosomes of children with NS were significantly higher than those in healthy children, and decreased significantly after treatment, indicating that miR-23b-3p and miR-30a-5p in urinary exosomes are potential indicators for diagnosing the progression of NS and monitoring the treatment effect.

Salivary Cytokines in Children with Nephrotic Syndrome versus Healthy Children: A Comparative Study.

Background: The aims of this study were to compare salivary cytokines and total protein between children with nephrotic syndrome (NS) and healthy children, and to examine whether saliva parameters can differentiate between steroid sensitivity and resistance and between disease remission and relapse. Methods: Twenty-seven children with nephrotic syndrome were classified according to steroid sensitivity and resistance, and disease remission and relapse. Twenty healthy children served as controls. Whole saliva samples were collected from all the participants. Urine and blood tests done on the same day as the saliva collection were recorded. Salivary total protein was quantified using bicinchoninic acid and IFNγ, IL-4, IL-8, IL-6, and IL1β levels using ELISA. Results: The mean ages of the nephrotic syndrome and control groups were 11.3 ± 2.4 and 9 ± 4.2, respectively. Compared to the control group, for the nephrotic syndrome group, total salivary protein was significantly lower, as were the levels of all the cytokines examined except IFNγ. Statistically significant differences were not found in any of the salivary markers examined between the children with nephrotic syndrome who were treatment sensitive (n = 19) and resistant (n = 8). Protein and IL-8 salivary levels were lower in the active (n = 7) than in the remission (n = 20) group. Conclusions: Salivary parameters distinguished children with nephrotic syndrome in relapse from healthy children. This may be due to decreased salivary protein excretion, which reflects decreased plasma levels, consequent to proteinuria. Accordingly, salivary markers may be developed as a diagnostic or screening tool for NS activity.

STUDY OF NR1I2 GENE POLYMORPHISM (A7635G) AND STEROID RESISTANCE IN EGYPTIAN CHILDREN WITH IDIOPATHIC NEPHROTIC SYNDROME

Nephrotic Syndrome (NS) is a common chronic disorder, characterized by alterations of the permeability of the glomerular capillary wall, resulting in its inability to restrict the urinary loss of protein. INS ( also called nephrosis ) is defined by the combination of a nephrotic syndrome (proteinuria, hypoalbuminemia, hyperlipidemia, and edema) and non-specific histological abnormalities of the kidney including minimal changes (MC), focal and segmental glomerular sclerosis (FSGS), and diffuse mesangial proliferation. As INS response to treatment with steroids varies. Several studies was done to analyze the correlation between steroid -related genes and the response to steroid treatment.The influence of polymorphisms on treatment response of children with INS was investigated. The results may be useful for predicting the treatment response of children with INS.. Nowadays it is clear that NR1I2 gene is also involved in regulation of intermediate metabolism through trans-activation and trans-repression of genes controlling glucose, lipid, cholesterol, bile acid, and bilirubin homeostasis. so it affects steroid drug metabolism and as a consequence the mutation of this gene may affect the response of NS children to treatment. valuable information in prognosis and pathology of the disease types.

Prevalence of Urinary Tract Infection in Nephrotic Syndrome at Zagazig University Children Hospital

Background: Nephrotic syndrome (NS) is the most common chronic glomerular diseases in children. Nephrotic syndrome increases a child's susceptibility to infection. Urinary tract infection (UTI) is the second most common bacterial disease in children after upper respiratory tract infection. Aim of this work: To find out the prevalence of UTI (either community acquired or hospital acquired) in NS children and to uncover the bacterial spectrum and their sensitivity pattern for selecting the empirical antibiotic therapy till the results of culture are awaited. Subjects and methods: This study was descriptive cross sectional. 33 nephrotic syndrome cases were included in study. All patients were subjected to full history taking, complete clinical examination and investigations as urine analysis and urine culture. Results: The percentage of community acquired urinary tract infection (CA-UTI) was 57.6 % and hospital acquired urinary tract infection (HA-UTI) was 42.4 %. E. coli is the commonest organism causing UTI followed by klebsiella and proteus mirabilis. The isolated organisms showed resistance to many oral and parenteral antibiotics. Amikacin gentamycin may be the first option of empiric therapy while waiting for culture reports. Conclusion: Children with nephrotic syndrome are frequently predisposed to UTI and in most cases it is asymptomatic often undiagnosed. Identifying bacterial causes and their susceptibility pattern of UTI will help in selecting the empirical antibiotic therapy till the results of culture are awaited. Although dipstick and microscopic urinalysis can support the diagnosis of UTI, growth of a single type of bacteria on urine culture is the gold standard.

Urinary calcium and bone mineral density in children with nephrotic syndrome treated with glucocorticoids

Background: Mainstay of therapy in the idiopathic nephrotic syndrome is glucocorticoids. Glucocorticoid induced osteoporosis is considered as most prevalent type of secondary osteoporosis. Only limited studies are conducted in tropical nations. Therefore our study is undertaken with objectives to evaluate Glucocorticoid therapy impact on bone health in Nephrotic Syndrome (NS) children by 2 different tools, namely urinary calcium and bone mineral density (BMD) by Quantitative ultrasound (QUS) and compare both the tools.Methods: Total 42 children with NS who completed minimum 12 weeks of Glucocorticoid therapy (6 weeks of daily regimen and minimum 6 weeks of alternate day regimen) were subjected to 24 hour Urinary calcium and Bone Mineral density by QUS at Tertiary health centre, Kalaburagi.Results: Out of 42 cases, 45.2 % had Osteopenia and 2.4% had osteoporosis, so 47.6% of them had BMD measured by QUS. Hypercalciurea was seen in 10 out of 42 cases (23.8%). In normal BMD group only 0.5% had hypercalciurea, Osteopenia group had 47.4% of cases and all osteoporosis group had hypercalciurea.Conclusions: Present study data concludes that children with NS treated with Glucocorticoids are at risk of Negative impact on bone health. Though both the tools detect impact of Glucocorticoids on bone health, BMD by QUS has better rate then urinary calcium in detecting negative effect of Glucocorticoid on bone health. As BMD by QUS decreases, Urinary calcium increases reflecting inverse relation between them.

A Determination of the Serum Ascitic Fluid Albumin Concentration Gradient in Children with Chronic Liver Disease and Nephrotic Syndrome

This prospective cross sectional study was carried out in the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka Medical College Hospital and Dhaka Shishu Hospital, Dhaka for a period of one year and six months starting from July 2012 to December 2013. The main objective of the study was to determine the serum ascitic fluid albumin concentration gradient in children with chronic liver disease and nephrotic syndrome. A total of 55 children were studied. Among them, 31 children with chronic liver disease with ascites were designated as Group I and rest 24 nephrotic syndrome children with ascites were designated as group II. The mean age of the children was 8.3±3.6 years ranging from 1.0-15.0 years. The mean age of the male patient was 8.0±3.6 years and that of female patients was 9.3±3.5 years. No statistically significant mean age difference was found between male and female patients (p>0.05), although female patients had a bit higher mean age than male patients. The mean age of the group I patients was 9.2±3.7 years ranging from 1.0-14.0 years and group II patients was 7.2±3.2 ranging from 2.0 to 15.0 years and the mean age difference between two groups patients was statistically significant (p 1.2 mg/dl, serum ALT >40 IU/L was 25 (80.6%), prothrombin time more than 3 second than control 23 (74.2%) and serum albumin less than 3.5 mg/dl was 24 (77.4%). The mean serum albumin was 2.8±0.5 mg/dl in group I patients and 1.7±0.4 mg/dl in group II patients. The albumin in ascitic fluid was 1.2±0.4 mg/dl in group I patients and 0.9±0.3 mg/dl in group II patients. Similarly, serum-ascites albumin gradient was 1.6±0.5 in group I and 0.8±0.2 in group II. It was evident that mean serum albumin, ascetic albumin as well as serum-ascites albumin gradient was significantly higher among the group I patients compared to group II patients (p<0.001).

Quantitative analysis of retinal and choroidal microvascular parameters using optical coherence tomography angiography in children with nephrotic syndrome: a pilot study.

To study the retinal and choroidal microvascular parameters in children with nephrotic syndrome (NS). This is a cross-sectional study. Optical coherence tomography angiography was used to evaluate the changes of retinal and choroidal microvessels in patients with NS. Thirty NS children and 20 normal controls were included in this study. The macular vessel density (VD) of the superficial capillary plexus (SCP), deep capillary plexus (DCP), choroid capillary plexus (CCP), and foveal avascular zone (FAZ) area of the SCP and DCP was quantitatively calculated. Clinical data including serum protein, blood lipid, uric acid, urea, serum creatinine, urinary protein concentration, urinary creatinine, 24-h urine volume, 24-h urinary total protein, 24-h creatinine clearance rate, and urinary albumin to creatinine ratio were collected. The VDs of the DCP and CCP in children with NS were significantly lower than those in controls (59.35 ± 2.45 vs. 61.15 ± 1.53, p = 0.002, 66.34 ± 1.43 vs. 67.16 ± 1.23, p = 0.042, respectively). The VD of the SCP in children with NS had a tendency to decrease compared with that in controls, but there were no significant differences. There were also no significant differences in FAZ area between the two groups. The VD of the SCP was positively correlated with serum total protein (ρ = 0.446, p = 0.014), serum albumin (ρ = 0.431, p = 0.017), and 24-h urine volume (ρ = 0.389, p = 0.034) but negatively correlated with triglyceride (ρ = - 0.450, p = 0.013), low-density lipoprotein cholesterol (ρ = -0.432, p = 0.017), urinary protein concentration (ρ = - 0.606, p < 0.001), and 24-h urinary total protein (ρ = - 0.517, p = 0.004). The VDs of the SCP, DCP, and CCP were negatively correlated with the urinary albumin to creatinine ratio (ρ = - 0.473, p = 0.008, ρ = - 0.438, p = 0.015, ρ = -0.467, p = 0.009, respectively). Retinal and choroidal VDs were decreased in children with NS and paralleled the severity of kidney disease. Optical coherence tomography angiography can be used as a noninvasive method for evaluating renal injury in patients with NS.

Correlation between resilience and family management mode in nephrotic syndrome children

Objective To understand the family management and resilience in nephrotic syndrome children to explore the influence of family management on resilience so as to provide a reference for family management and environmental support for chronic disease children. Methods From April 2017 to April 2018, we selected 130 nephrotic syndrome children and main caregivers at a Class Ⅲ Grade A comprehensive children's hospital as subjects by convenience sampling. The investigation was carried out with the general information questionnaire, Chronic Illness Children's Resilience Scale (CICRS) and Family Management measure (FaMM) . A total of 130 questionnaires were sent out and 127 valid questionnaires were collected with 97.69% for the valid recovery rate. Results Among those 127 nephrotic syndrome children, the total score of resilience was (99.84±12.53) . There were 15 (11.81%) children with the high level of resilience, 102 (80.31%) children with medium level and 10 (7.87%) children with the low level. Results showed that the score of child daily life had a positive correlation with the total score of children's resilience (P<0.05) ; scores of family members' fears of disease and difficulty of disease management had negative correlations with the score of children's coping style (P<0.05) ; score of family members' management ability had a positive correlation with the score of children's coping style (P<0.05) ; score of disease burn out was negatively correlated with the score of interpersonal relation (P<0.05) ; score of supporting each other of parents were positively correlated with the scores of children's individual characteristics and children's perceived family influence (P<0.05) . Conclusions Resilience and family management of nephrotic syndrome children need to be improved. Nurses should pay attention to the influence of family support and environmental support on psychological factors in children, and guide children to improve their coping capacity and family members to positively perceive children's school function, interpersonal relation and family environment to improve children's psychological health. Key words: Nephrotic syndrome; Resilience, psychological; Family management; Correlation

A study to assess the prevalence of hypertension in children with nephrotic syndrome

Background: Hypertension is been one of the most common co morbidity of this disease. It was mostly attributed to sodium retention, which is a major clinical feature of nephrotic syndrome. These mechanisms likely have a role in the development of hypertension in nephrotic syndrome, where hypertension may be difficult to control, and provide new therapeutic options for the management of blood pressure in the setting of nephrotic syndrome. Objective of study the prevalence of hypertension in children with NS and also the number of antihypertensive required to control it.Method: A Retrospective study of the hospital records of 100 children diagnosed with nephrotic syndrome admitted to Pediatric and Nephrology Ward at YMCH was accessed.Results: In our study 35 (35%) of them were Infrequent relapse nephrotic syndrome (IFNS) and 35(35%) were Frequent relapse nephrotic syndrome (FRNS) ,while 30 cases (30%) were First episode nephrotic syndrome (FENS). 65 cases were steroid sensitive, while 28 and 7 of them were steroid dependent and resistant respectively. Of the 100 study population 54 of them had hypertension while 46 of them did not develop it .Of the 54 hypertensive nephrotic syndrome children, 15 of them (28.%) required three anti hypertensives to control the pressure, while 19 (35%) and 20 (37%) required single and dual anti hypertensives respectively.Conclusion: Prevalence of hypertension is increasing among the children with nephrotic syndrome. Its more prevalent among the male then female FRNS, SRNS and SDNS are more prone to develop hypertension and also they needed two or more antihypertensives to control the hypertension, whereas hypertension in SSNS could be managed with single drug.

-254C>G SNP in the TRPC6 Gene Promoter Influences Its Expression via Interaction with the NF-κB Subunit RELA in Steroid-Resistant Nephrotic Syndrome Children.

This study is aimed at exploring the mechanism by which the −254C>G single nucleotide polymorphism (SNP) on the transient receptor potential cation channel 6 (TRPC6) gene promoter could increase its activation in steroid-resistant nephrotic syndrome children of China. Plasmids containing the TRPC6 promoter region (with the −254C or G allele) were constructed and then transfected into human embryonic kidney (HEK) 293T cells and human podocytes. Luciferase assays were used to test the promoter activity in both cell lines with or without tumor necrosis factor-α (TNF-α) treatment, and chromatin immunoprecipitation-polymerase chain reaction (ChIP-PCR) analysis was used to verify the transcription factor that could bind to this mutant sequence. Luciferase results indicate that the activity of the mutant promoter was greater than that of the normal promoter of the TRPC6 gene in both cell lines. We further predicted and verified that this variation was mediated by the nuclear factor kappa B (NF-κB) subunit RELA, and TNF-α significantly enhanced the transcription activity of TRPC6 with the −254G allele. In conclusion, the −254C>G SNP is a gain-of-function variation of the TRPC6 gene, and it is also an early and effective factor for predicting steroid-resistant nephrotic syndrome (SRNS) in Chinese children.

Thyroid Function in Children with Nephrotic Syndrome and Normal Renal Function in a Tertiary Level Hospital, Rajshahi

Background: Nephrotic syndrome (NS) is a one of most common renal disease in children causing massive proteinuria. In patients with NS, other proteins like thyroid hormones and hormone binding protein are also lost in urine in addition to albuminuria. So, this study was done to find out the thyroid function status of nephrotic syndrome children and to compare the thyroid function of healthy control children.&#x0D; Method: This cross sectional comparative study was carried out in the Department of Paediatrics, Rajshahi Medical College and Nuclear Medicine Centre, Rajshahi. Total 30 patients of both sex and age between 2-12 years diagnosed as nephrotic syndrome were included in this study and another 30 non-nephrotic syndrome apparently healthy children were taken as control. History, clinical examinations and laboratory investigations were recorded in preformed questionnaire. Thyroid function statuses also were evaluated in both patient and healthy control. After data collection, statistical analysis was done by computerized software.&#x0D; Results: In this study, 63.3% (n=19) nephrotic syndrome children had secondary hypothyroidism, overt hypothyroidism was 27.3% (n=7) and subclinical hypothyroidism was 6.7 % (n=2).&#x0D; Conclusion: In patients of childhood nephrotic syndrome different forms of thyroid dysfunctions are commonly associated due to loss of thyroid hormones and hormone binding’s protein.&#x0D; TAJ 2018; 31(1): 62-69

Risk Factor of Tuberculosis in Children with Nephrotic Syndrome who Received Immunosuppressant Therapy in Hasan Sadikin Hospital

Patients with nephrotic syndrome (NS) are in immunocompromised condition due to illness and therapy given. Treatment of NS with long-term steroids, high doses and combination with cyclophosphamide decreases body resistance. Immunocompromised conditions facilitate the occurrence of infection, one of the infections that might occur is tuberculosis (TB) with a high incidence in Indonesia. The aim of the study was to investigate the risk factors of TB in children with NS, specifically related to the duration of steroids administration, and combinations of steroids with cyclophosphamide. This was cross sectional study data retrieval from out-patients and in-patients NS children in RS Hasan Sadikin Bandung (RSHS) during 1st January 2012 until 31st December 2016. Statistical analysis using chi-square test and multiple logistic regression. There were 34 children with TB from 199 NS patients, the prevalence of TB in children with NS in RSHS (17%). The results showed that duration of steroids administration, combination of steroids and cyclophosphamide, female gender, and under nutrition were significant to tuberculosis incidence (p <0.001). Multiple logistic regression analysis revealed duration of steroid 175,5 days OR 20,7 (CI95% 7,5-68,1); p<0.001, combination of steroids with cyclophosphamide OR 3,2 (CI95% 1,1-11,7); p=0,027, female gender OR 2,5 (CI95% 0,9-7,2); p=0,077, and under nutrition OR 7,2 (CI95% 2,4-3,7); p=0.001. Duration of steroid administration, combination of steroids with cyclophosphamide, female gender, and under nutrition were risk factors for tuberculosis. The duration of steroid administration was the most dominant factor in the incidence of tuberculosis in children with NS.

Carotid intima media thickness in children with nephrotic syndrome: an observational case control study.

Carotid intima media thickness (cIMT) is considered as a surrogate marker for the various cardiovascular events that complicate nephrotic syndrome (NS). The present work was conducted to study cIMT in children with NS and to find out its correlation with dyslipidemia and other risk factors. This was a case control study conducted at a tertiary care hospital in children with NS who were more than 2 years with normal serum complement, being on therapy for NS for at least 1 year, glomerular filtration rate more than 90 ml/minute/1.73 m2 and absence of acute infection in previous 3 months. Sixty six children with NS constituted the case material and 128 age and sex matched children were taken as control. The mean age in case and control cohort was 6.71 ± 3.3 and 7.89 ± 3.95 years, respectively. The mean age of onset of illness was 4.32 ± 2.25 years. The mean duration of illness was 2.39 ± 1.44 years. Thickness of cIMT was higher in NS children as compared to control group in all the ages, but this difference was statistically significant only after 4 years of age. There was statistically significant, but weak positive correlation between cIMT and age of NS children, duration of disease and number of relapses. There was no correlation of cIMT with hypertension, body mass index, serum creatinine, and dyslipidemia. A negative, but statistically insignificant correlation of cIMT was found with serum albumin and serum cholesterol.

NPHS1基因突变致儿童激素耐药型肾病综合征1例报道

NPHS1基因突变致儿童激素耐药型肾病综合征1例报道

Increased urinary exosomal microRNAs in children with idiopathic nephrotic syndrome.

BackgroundUrinary exosomal miRNAs are gaining increasing attention for their potential as ideal non-invasive biomarkers for kidney diseases; however, little is known about their diagnostic ability for paediatric nephrotic syndrome (NS). This study explored the clinical value of urinary exosomal miRNAs for paediatric idiopathic NS.MethodsUrine samples were collected from 129 NS children and 126 age−/sex-matched healthy controls. The miRNA profile of urinary exosomes was analysed by high-throughput Illumina sequencing via synthesis (SBS) technology followed by verification with a quantitative reverse-transcription polymerase chain reaction (RT-qPCR) assay arranged in two independent cohorts. Additionally, paired urine samples from 65 of these patients were collected before and after treatment.FindingsThe Illumina SBS identified 30 markedly increased urinary exosomal miRNAs in NS children compared with controls (≥ 5-fold, P < .05). Fifteen miRNAs were selected for further investigation, of which 5 (miR-194-5p, miR-146b-5p, miR-378a-3p, miR-23b-3p and miR-30a-5p) were verified by RT-qPCR to be significantly and steadily increased in NS (> 3-fold, P < .01) and markedly reduced during the clinical remission period (P < .001). Moreover, the concentrations of miR-194-5p and miR-23b-3p were significantly positively correlated with the urine protein content and were markedly higher in the high urine protein group than in the low urine protein group (P < .001 and P < .01, respectively).InterpretationsWe identified 5 altered urinary exosomal miRNAs in NS children with disease progression and treatment. These urinary exosomal miRNAs could be promising and non-invasive potential biomarker candidates for diagnosing, monitoring and stratifying paediatric NS.FundNational Natural Science Foundation of China; Fund of State Key Laboratory of Analytical Chemistry for Life Science; National Basic Research Programme of China; Foundation of Jiangsu Provincial Medical Youth Talent.

Thyroid function test in nephrotic syndrome children who are admitted in emergency ward of Government Tirunelveli Medical College and Hospital, India

Background: Nephrotic syndrome in childhood is largely primary or idiopathic, although a small proportion of cases are secondary to infectious agents and other glomerular and systemic diseases. The etiology of nephrotic syndrome is age-dependent. Most cases appearing in the first 3 months of life are referred as congenital nephrotic syndrome (CNS) and are due to genetic diseases. The objective of this study is to study the correlation between thyroid profile and different types of nephrotic syndrome.Methods: This cross-sectional study was done between March 2017 to October 2017 in the Department of Pediatrics Tirunelveli Government Medical College. 40 cases of nephrotic syndrome between 1 to12 years, which include all types of nephrotic syndrome. After history taking and clinical examination, blood samples were collected from the patients for thyroid function test and analyzed with standard techniques.Results: Serum T3, T4, TSH were found to be within normal limits. But TSH values in remission were significantly elevated compared to the first episode.Conclusions: Thyroid profiles in control were within normal range. The T4 and T3 levels in nephrotic syndrome were low and TSH was high. Hypothyroidism was more common in children less than 6 years.

Effect of an Educational Program on Knowledge and Practice of Nurses who are Caring for Nephrotic Children

Background: Pediatric nurses had unsatisfactory knowledge and practice regarding the care of nephrotic syndrome children as reported by many studies. Aim: This study aims to evaluate the effect of an-educational program on knowledge and practice of nurses who are caring for nephrotic children. Methods: A quasi- experimental design was utilized in current study. Sample: Convenience sample of 57 pediatric nurses who are caring children with nephrotic syndrome. Tools of data collection: Two tools were utilized for data collection; self- administered questionnaire sheet included personal characteristics of the pediatric nurses, their knowledge, and observational checklist for evaluation of nursing practices of children with nephrotic syndrome. The results Shows that majority of the pediatric nurses had unsatisfactory knowledge and inadequate practice about nephrotic syndrome before program implementation, that improved significantly immediate and 3 months after program implementation, there are a positive correlation between nurses' knowledge, age and years of experience, also between nurses practice, age, years of pediatric and general experience. Conclusion: The pediatric nurses' knowledge and practice improved significantly after program implementation and this improvement positively correlated with age and years of experience. The study recommended that in service training program should be conducted for the pediatric nurses in their work place to update their knowledge and improved their practice.