Nephrotic syndrome management in children

BACKGROUND: Renal biopsy has a definite role in the management of pediatric kidney diseases. Most centers have given up the earlier practice of performing blind biopsy using Tru-Cut needle and started doing real time ultrasound-guided renal biopsy with an automated spring-loaded gun, which has become standard of care in the last decade or so. METHODS: We performed a cross-sectional analysis of the pediatric biopsies conducted at our center over the years by both methods to evaluate whether ultrasound-guided renal biopsy with a disposable automated spring-loaded gun was superior to blind biopsy with a disposable needle of the same size in terms of reduction of complications and improving the yield...

We report three sporadic infections by Elizabethkingia meningosepticum from Government Medical College Kozhikode in Kerala state, South India over a period of three years. Two cases were commenced in the community in immunocompromised children beyond the newborn period, but both recovered promptly. Another was a hospital-acquired meningitis in a newborn baby who developed neurologic sequelae. In contrast to widespread antimicrobial resistance exhibited by this pathogen, there was good susceptibility to commonly used antimicrobials such as ampicillin, cefotaxime, piperacillin, ciprofloxacin, and vancomycin...

Nephrotic syndrome (NS) is a common glomerular disease characterised by massive proteinuria, hypoalbuminaemia, oedema and hyperlipidaemia. Cerebral venous sinus thrombosis (CVST) is a rare complication in children with NS. Here, we report a case of relapsing NS on steroid therapy, in a male in early childhood who presented with symptoms of headache, vomiting and double vision. On prism cover test there was 25 PD esotropia with abduction restriction in the left eye. Fundus examination showed bilateral papilloedema...

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is rare, but potentially life-threatening. The clinical course definitely become more unpredictable and fatal in patients complicated by pulmonary embolism (PE). Nephrotic syndrome (NS) is an uncommon etiology of CVST. Concurrence of CVST and PE at the initial onset of NS is extremely unusual and rarely reported. Considering that edema might be absent in NS individuals, thromboembolic events probably become unrecognized, thereby causing a missed or delayed diagnosis and poor outcome...

BACKGROUND: Nephrotic syndrome (NS) is one of the most common kidney disorders seen by pediatric nephrologists and is defined by the presence of heavy proteinuria (>3.5 g/24 h), hypoalbuminemia (<3.5 g/dL), edema, and hyperlipidemia. Most children with NS are steroid-responsive and have a good prognosis following treatment with prednisolone. However, 10%-20% of them have steroid-resistant nephrotic syndrome (SRNS) and fail to respond to treatment. A significant proportion of these children progress to kidney failure...

INTRODUCTION: The nephrotic syndrome (NS) is a common childhood illness characterized by massive proteinuria, hyperlipidemia, and hypoalbuminemia. It is a disease of relapse, and therefore, it is a major problem to manage cases with frequent relapses. Prediction and prevention of risk factors is the key to successful management of childhood NS. An understanding of the risk factors that determine the course is useful in taking decisions regarding therapy and enables counseling. MATERIALS AND METHODS: Sample size of 100 children of age 1-12 years of age with steroid sensitive nephrotic syndrome over duration of 1 year from April 2020 to May 2021...

Myocardial infarction is rare in children, teenagers and young adults (aged<20 years). The most common aetiologies identified include Kawasaki disease, familial hypercholesterolaemia, collagen vascular disease-induced coronary arteritis, substance abuse (cocaine, glue sniffing), trauma, complications of congenital heart disease surgery, genetic disorders (such as progeria), coronary artery embolism, occult malignancy and several other rare conditions. Nephrotic syndrome is a very rare cause of myocardial infarction, but it is probably underestimated...

BACKGROUND: There is paucity of information on rituximab-associated hypogammaglobulinemia (HGG) and its potential infectious consequences in children treated for idiopathic nephrotic syndrome (INS). METHODS: A survey was distributed by the European Society Pediatric Nephrology to its members. It addressed the screening and management practices of pediatric nephrology units for recognizing and treating RTX-associated HGG and its morbidity and mortality. Eighty-four centers which had treated an overall 1328 INS children with RTX responded...

BACKGROUND: Urinary tract infection (UTI) is a common infection in children with nephrotic syndrome (NS). Clinical experience suggests that childhood nephrotic syndrome is frequently diagnosed incorrectly and managed inadequately on the top of this existing UTI in the episode becomes an additive obstacle for the primary care physicians or pediatricians towards optimum management, leading to poor outcome. So, we have conducted this clinico- microbiological study of UTI in NS in children to provide the exact picture of UTI with NS so that the primary care providers can be helped in having high index of suspicion of this infection and knowing prevalent organisms and their antimicrobial sensitivity pattern...

Nephrotic syndrome is a common childhood glomerular disease that is associated with massive proteinuria and edema. Children with nephrotic syndrome are at risk of chronic kidney disease, disease-related complications, and treatment-related complications. Patients with frequently relapsing disease or steroid toxicity may require newer immunosuppressive medications. However, access to these medications is limited in many African countries owing to prohibitive cost, the need for frequent therapeutic drug monitoring, and a lack of appropriate facilities...

BACKGROUND: Infections associated with nephrotic relapses (NR) are often managed according to physician preferences. A validated prediction tool will aid clinical decision-making and help in rationalizing antibiotic prescriptions. Our objective was to develop a biomarker-based prediction model and a regression nomogram for the prediction of the probability of infection in children with NR. We also aimed to perform a decision curve analysis (DCA). METHODS: This cross-sectional study included children (1-18 years) with NR...

BACKGROUND: Schimke immunoosseous dysplasia (SIOD) is an ultra-rare inherited disease affecting many organ systems. Spondyloepiphyseal dysplasia, T-cell immunodeficiency and steroid resistant nephrotic syndrome are the main symptoms of this disease. CASE PRESENTATION: We aimed to characterize the clinical, pathological and genetic features of SIOD patients received at tertiary Pediatric Nephrology Center, University Hospital Motol, Prague, Czech Republic during the period 2001-2021...

Children with congenital nephrotic syndrome (CNS) can present with oro-dental complications secondary to the disease or from the treatment of the condition. This case report describes the multidisciplinary management of a 12-year-old male patient with CNS. It highlights the importance for pediatricians and nephrologists to be aware of the dentists' role in the general health and well-being of children with chronic renal disease.

UNLABELLED: Diuretics are frequently prescribed drugs and help managing several pathological conditions, including acute and chronic kidney disease, nephrotic syndrome, congestive heart failure, ascites, systemic and pulmonary hypertension. Diuretic classes include among others osmotic diuretics and carboanhydrase inhibitors, loop diuretics, thiazides, and potassium-sparing diuretics. In this educational article, we aim at reviewing indications, mechanisms of action, and side effects, as well as basic pharmacokinetics considerations and data on diuretics in children, supporting practicing clinicians in choosing (and understanding the background of) the best-suited diuretic regimen for the individual patient...

BACKGROUND: Genetic nephrotic syndrome is caused by pathogenic variants in genes encoding proteins necessary for the stability and functionality of the glomerular filtration barrier. To date, more than 70 genes associated with steroid-resistant nephrotic syndrome have been identified. We review the clinical and molecular aspects of genetic nephrotic syndrome with a particular focus on genes associated with slit membrane and podocyte cytoskeleton defects. Sanger sequencing and next-generation sequencing are widely used in the identification of novel gene variants and help us gain a better understanding of the disease...

BACKGROUND: During the SARS-CoV-2 global pandemic, one of the longest lockdowns worldwide occurred in Ontario, Canada, during the first wave. For parents and children managing care at home and at risk for COVID-19, the impact on their psychosocial functioning is unknown. METHODS: A total of 122 families of children aged 2-18 years were enrolled as part of the prospective cohort of childhood nephrotic syndrome and completed a survey during the first wave of the pandemic (August 21-December 10), 2020...

BACKGROUND: An association between nephrotic syndrome and extrarenal neoplasia was described for the first time in 1922. The reported incidence of nephrotic syndrome in Hodgkin lymphoma is less than 1%. CLINICAL DESCRIPTION: We present a 13 year old boy who was admitted with complaints of abdominal pain, vomiting and loose stools for 2 months. He had a history of significant weight loss of 5kg in a couple of months.On examination, he had bilateral pedal oedema and right cervical lymphadenopathy...

BACKGROUND: Hypothyroidism in children with nephrotic syndrome (NS) is often attributed to prolonged loss of thyroxine binding globulin and thyroid hormones alongside protein in the urine. It has been historically associated with steroid-resistant NS alone. However, recent evidence supports the fact that subclinical hypothyroidism (SCH) does occur even in children with steroid responsive NS. Complications such as weight gain, hypercholesterolemia, delayed growth, delayed puberty, and depression could result from hypothyroidism and be erroneously attributed to NS, or the effect of steroid used in treatment...

Patients with nephrotic syndrome (NS) are at an increased risk for thromboembolic events, such as deep venous and arterial thrombosis and pulmonary embolism (PE). In general, PE in children differs from adults in incidence, predisposition, pathophysiology, presenting symptoms, and management strategies. There is a lack of treatment guidelines for PE in children, and the management strategies are mostly extrapolated from adult data. This case report highlights the presentation of acute cor pulmonale due to massive PE associated with NS and a successful pulmonary endarterectomy that reversed the child's pulmonary hypertension and normalized right ventricular function...

Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85-90% of patients attain complete remission of proteinuria within 4-6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70-80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission...