Assessment of Carotid Intima Media Thickness and Left Ventricular Mass Index in Children with Idiopathic Nephrotic Syndrome.

BACKGROUND: Children with nephrotic syndrome (NS) are at a greater risk of atherosclerosis due to recurrent exposures to hyperlipidemia, hypertension, and immunosuppressive medications. CIMT (carotid intima media thickness) is a reliable marker for assessment of atherosclerosis of large and medium-sized blood vessels; endothelial dysfunction and increased CIMT usually precede the development of cardiovascular diseases. Some manifestations of NS, like proteinuria and hyperlipidemia, are associated with an increased risk of cardiac morbidity and mortality. The aim of the current study was to evaluate the carotid intima media thickness and LVM (left ventricular mass) thickness in children with nephrotic syndrome. SUBJECTS AND METHODS: Eighty-one children with nephrotic syndrome and 100 healthy children as controls were enrolled in the study. The inclusion criteria were: disease duration of minimum of 12 months, glomerular filtration rate >60mL/min/1.73m 2 and children aged two years or more at the time of study. CIMT and left ventricular mass index, lipid profile, protein/creatinine ratio in urine and kidney function tests were done for cases and controls after approval of internal ethical committee. RESULTS: The mean CIMT (mm) was significantly higher in NS (0.51± 0.12) compared to controls (0.42± 0.09) (P <0.001). LVM and LVM Index were significantly higher in NS than controls (p< 0.001, for both). Subsequently, CIMT was significantly correlated to duration of the disease (p< 0.001), LVM index was significantly correlated with duration of the disease, body mass index (BMI), blood pressures and triglycerides level (p< 0.05). CONCLUSION: Children with NS are at increasing risk to develop atherosclerosis as measured by CIMT. LVM was significantly higher in NS and positively correlated to BP, disease duration, triglyceride levels and BMI.

Influenza vaccination among children with idiopathic nephrotic syndrome: an investigation of practices.

BACKGROUND: Annual influenza vaccination is recommended for all children with idiopathic nephrotic syndrome (INS) in France. Consequently, the Social Security automatically sends prescriptions to all patients suffering from a chronic disease. The aim of this study was to evaluate the follow-up to these recommendations. METHODS: We conducted a monocentric retrospective investigation of practices. We included all children with steroid-sensitive INS in remission who attended our clinics from January 1st 2015 to January 1st 2017, resided in France and had a valid phone number. Data were collected from May 2017 to June 2017 through a phone interview and review of clinical charts. RESULTS: 75 patients met the inclusion criteria. The parents of 57 children could be reached by phone and agreed to participate to the survey. 35/57 (61.4%) declared having received a prescription during the 2016-2017 campaign. Only 14 children (24.6%) were vaccinated. 17/43 (39.5%) parents of unvaccinated children had concerns about the safety of the vaccine, 16/43 (37.2%) were not aware of the recommendations, 5/43 (11.6%) had been recommended by their physician not to vaccinate their child, 3/43 (7%) forgot to have them vaccinated and 2/43 (4.6%) reported no reason. 13/43 (30%) unvaccinated children presented a relapse during the flu season - 2/13 during an influenza-like illness - whereas 1/14 (7%) immunized children presented a relapse during the six months of post-vaccination follow-up. Relapse rates were not increased in vaccinated children compared to unvaccinated children (p = 0.15), nor in the 6 months following vaccination compared to the 6 months prior (1/14 vs 5/14, p = 0.20). CONCLUSIONS: 1) < 2/3 patients were properly prescribed the recommended yearly influenza vaccination at our center 2) only 1/4 were vaccinated and most of their parents were misinformed. Physicians must be aware of this and should make every effort to better inform their patients on the risks of flu illness and the benefits and safety of the vaccination.

Clinical course & management of childhood nephrotic syndrome in Germany: a large epidemiological ESPED study.

BACKGROUND: Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in childhood, despite its rarely occurrence in the general population. Detailed information about clinical data of NS (e.g. average length of stay, complications) as well as of secondary nephrotic syndrome (SNS) is not well known. METHODS: A nationwide ESPED follow-up study presenting the clinical course and management of children with NS in Germany. RESULTS: In course of 2 years, 347 children developed the first onset of NS, hereof 326 patients (93.9%) had a primary NS, and 19 patients had a SNS (missing data in 2 cases), the majority due to a Henoch-Schönlein Purpura. Patients with steroid-resistant NS (SRNS) stayed significantly longer in hospital than children with steroid-sensitive NS (25.2 vs. 13.3 d, p <  0.001). Patients with bacterial/viral infections stayed longer in hospital (24.9 d/19.5d) than children without an infection (14.2 d/14.9 d; p <  0.001; p = 0.016). Additionally, children with urinary tract infections (UTI) (p < 0,001), arterial hypertension (AH) (p < 0.001) and acute renal failure (ARF) (p < 0,001) stayed significantly longer in hospital. Patients with SRNS had frequent complications (p = 0.004), such as bacterial infections (p = 0.013), AH (p < 0.001), UTI (p < 0.001) and ARF (p = 0.007). Children with a focal segmental glomerulosclerosis (FSGS) had significantly more complications (p = 0.04); specifically bacterial infections (p = 0.01), UTI (p = 0.003) and AH (p < 0,001). Steroid-resistance was more common in patients with UTI (p < 0.001) and in patients with ARF (p = 0.007). Furthermore, steroid-resistance (p < 0.001) and FSGS (p < 0.001) were more common in patients with AH. CONCLUSIONS: This nationwide, largest German study presents results on the clinical course of children with NS considering a diverse range of complications that can occur with NS. The establishment of a region-wide and international pediatric NS register would be useful to conduct further diagnostic and therapy studies with the aim to reduce the complication rate and to improve the prognosis of NS, and to compare the data with international cohorts.

Influence of the New Cooperative Medical Scheme on hospitalization cost of patients with nephrotic syndrome in rural China.

INTRODUCTION: Rural residents are benefitting from the current New Cooperative Medical Scheme (NCMS) in China. Treatment of diseases has improved and the total cost of hospitalization has decreased significantly because of the application of NCMS. Most articles in this area have mainly focused on the policy of NCMS, but few studies have been relevant to the influence of NCMS on a specific disease and the cost. In the present study, the impact of NCMS on hospitalization costs of patient with nephrotic syndrome from the countryside was investigated and discussed. METHODS: Three hundred and ninety patients from China and with nephrotic syndrome were enrolled into the present study and were divided into two groups according to whether they had joined the NCMS. The total hospitalization cost, check cost (such as laboratory testing and ultrasound), drugs cost, length of stay in hospital and ratio of renal biopsy in all patients were analyzed. RESULTS: The expenses for individuals decreased significantly in patients who were part of the NCMS, in contrast with the patients without the NCMS (p<0.001). The ratio of renal biopsy increased significantly in patients who were part of the NCMS (p<0.01). There was no significant difference in cost and length of stay between the two groups. CONCLUSIONS: The NCMS contributes to reducing personal expenses and therapy of disease.

The impact of pediatric nephrotic syndrome on parents' health-related quality of life and family functioning: an assessment made by the PedsQL 4.0 family impact module.

The multi-dimensional impact on the quality of life (QOL) of families of children with the nephrotic syndrome (NS) has not been studied sufficiently in the literature. We aimed to study this aspect and the predictors of poor QOL among Indian families having children with NS. A cross-sectional study was conducted to compare the parents of children with chronic NS on treatment for at least one year with parents of a matched healthy control group. The parents of both groups were asked to complete the standard self-administered multi-dimensional questionnaire of Pediatric Quality of Life Inventory 4 (PedsQL TM ) Family Impact Module (FIM). Descriptive and analytical statistics were performed to compare scores between the two groups. Possible predictors of poor outcome in each of the summary scales among the cases were assessed by both univariate and multivariate analysis. The parents of 61 cases and 72 controls completed the PedsQL TM FIM questionnaire. The scores in each of the categories, namely FIM Total Scale Score, Parent HRQOL Summary Score, Family Functioning Summary Score and eight individual domains, were found to be significantly higher among controls. Female gender of the affected child was an independent risk factor for poor Family Functioning Summary Score. Also, presence of serious complications during the course of the disease independently predicted poor Total FIM and Parent HRQOL Summary Scores. Even a relatively benign and potentially curable chronic disorder in children, like the NS, can disturb the QOL of parents in multiple domains of functioning.

Genetic testing in nephrotic syndrome--challenges and opportunities.

Monogenic nephrotic syndrome (nephrotic syndrome caused by a single gene defect) is responsible for only a small percentage of cases of nephrotic syndrome, but information from studies of the unique cohort of patients with this form of the disease has dramatically improved our understanding of the disease pathogenesis. The use of genetic testing in the management of children and adults with nephrotic syndrome poses unique challenges for clinicians in terms of who to test and how to use the information obtained from testing in the clinical setting. In our view, not enough data exist at present to justify the routine genetic testing of all patients with nephrotic syndrome. Testing is warranted, however, in patients with congenital nephrotic syndrome (onset at 0-3 months), infantile nephrotic syndrome (onset at 3-12 months), a family history of nephrotic syndrome, and those in whom nephrotic syndrome is associated with other congenital malformations. The family and/or the patient should be given complete and unbiased information on the potential benefits and risks associated with therapy, including the reported outcomes of treatment in patients with similar mutations. Based on the data available in the literature so far, intensive immunosuppressive treatment is probably not indicated in monogenic nephrotic syndrome if complete or partial remission has not been achieved within 6 weeks of starting treatment. We advocate that family members of individuals with genetic forms of nephrotic syndrome undergo routine genetic testing prior to living-related kidney transplantation. Prospective, multicentre studies are needed to more completely determine the burden of disease caused by monogenic nephrotic syndrome, and randomized controlled trials are needed to clarify the presence or absence of clinical responses of monogenic nephrotic syndrome to available therapies.

Occurrence of infection among children with nephrotic syndrome during hospitalizations.

AIM: The present study was conducted to investigate the trends of childhood nephrotic syndrome (NS) admissions and factors associated with childhood NS admissions with major infections in Taiwan. METHODS: A retrospective analysis was performed using Taiwan National Health Research Insurance Database (NHIRD) to explore the associated factors and health care burden for childhood NS admissions with major infections in 1997 to 2007. RESULTS: Of 133,927 children, a total of 176 children had NS, which incurred 508 hospital admissions. Nineteen percent of admissions were associated with major infections. Pneumonia was the most common infection (49%), followed by urinary tract infection (UTI), bacteraemia/sepsis, peritonitis and cellulitis. Pneumonia was the most common infection among children age younger than 10 years, whereas UTI was more common among children aged greater than 10 years. NS admission with infections had longer periods of hospital length of stay and higher hospital total costs compared to those without infections. Regression analysis reveals that younger age, regional hospitals, admission hospital located in middle and south areas and admission made in spring were associated with increased risk for developing major infections. CONCLUSIONS: While 19% of childhood NS admissions were associated with major infections, young age, admissions made in spring, located in middle and south Taiwan and in regional hospitals were the major associated factors for infection. Age plays an important role in risk and types of infection.

Obstacles to health care: a nurse's experience in Sudan.

Sudan is a country known for its long history of wars, poverty, and disease. These factors combine to cause a high incidence of morbidity and mortality and the inability of the population to seek and receive medical care.

Evaluation and management of proteinuria and nephrotic syndrome in children: recommendations from a pediatric nephrology panel established at the National Kidney Foundation conference on proteinuria, albuminuria, risk, assessment, detection, and elimination (PARADE).

OBJECTIVE: The development of this review article evolved from a National Kidney Foundation consensus conference on recent advances in the importance of evaluating and treating proteinuria. From this conference, a series of recommendations for the evaluation of adults with proteinuria was published. Because specific pediatric aspects of the problem were outside the scope of the original National Kidney Foundation publication, an ad hoc committee of 6 pediatric nephrologists who were active participants in the National Kidney Foundation conference was established to provide primary care physicians with a concise, up-to-date reference on this subject. METHODS: The recommendations that are given represent the consensus opinions of the authors. These are based on data from controlled studies in children when available, but many of the opinions are, by necessity, based on uncontrolled series in children or controlled trials performed in adults, because controlled trials in children have not been performed to evaluate many of the treatments described. RESULTS AND CONCLUSIONS: These recommendations are intended to provide primary care physicians with a useful reference when they are faced with a young child or teenager who presents with proteinuria, whether this is mild and asymptomatic or more severe, leading to nephrotic syndrome.

Valoración de un nuevo esteroide (deflazacort) en el tratamiento inicial en niños con síndrome nefrótico idiopático / Evaluation of deflazacort, a new steroid, in the initial theraphy of children with idiopathic nephrotic syndrome

Evaluamos un nuevo derivado oxazolínico de la prednisona comparando su respuesta y efectos secundarios con los observados en el tratamiento clásico con prednisona, en un grupo de 24 niños durante el primer episodio de Síndrome Nefrótico Idiopático. Grupo A: 12 niños de edad media: 43 meses (r: 10 á 72 m) recibieron deflazacort en dosis de 1.8 á 2.2 mg/kg/día durante 30 días contínuos y 30 días alternos (serie completa). Grupo B: 12 niños, edad media: 37 meses (r: 29 á 70) recibieron metilprednisona a la dosis de 1.5 á 1.7 mg/kg/día con igual esquema que los del grupo A. En 6 pacientes del Grupo A la proteinuria desapareció entre los 7 y 26 días de tratamiento (x 13.8 d). Los otros 6 no negativizaron la proteinuria. En 7 niños del Grupo B la proteinuria remitió entre 5 y 15 días (x 10.5 d), los 5 restantes persistieron con proteinuria masiva hasta la finalización de la primera serie de tratamiento. Los parámetros bioquímicos pre y post tratamiento en los pacientes corticosensibles no mostraron diferencias significativas entre ambos grupos. Los valores de colesterol fueron menores en los que recibieron deflazacort aunque sin significancia estadística. Los efectos esteroides colaterales estuvieron presentes en la mayoría de los niños tratados con prednisona, y fueron significativamente menores en los que recibieron deflazacort

Can decision analysis adequately represent clinical problems?

A major weakness of medical decision analysis has been the inability of the commonly used single attribute utility models to adequately represent clinical decision making situations. To illustrate this problem, I reanalyzed a well known decision analysis that is widely interpreted as proof that two decision alternatives are equivalent in all clinically meaningful respects. The reanalysis was based on a more representative decision model made possible by the use of the analytic hierarchy process (AHP), a multiobjective decision making technique. The use of this model resulted in the identification of a clearly preferred alternative, indicating that the results of the original analysis have been widely misinterpreted. The degree to which a decision model represents clinical reality influences the correct interpretation of a decision analysis. Limited decision models can yield only limited conclusions. The use of more representative multiobjective decision models would improve the clinical usefulness of medical decision analyses.