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Nephrotic syndrome management in children

Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
Aravind Selvin Kumar Ramanathan, Murali Vijayan, Srilakshmi Rajagopal, Padmaraj Rajendiran, Prabha Senguttuvan
Nephrotic syndrome (NS) is a kidney disease predominantly present in children with idiopathic condition; final stage of the disease progresses into end-stage renal disease. Generally, NS is treated using standard steroid therapy, however; most of the children are steroid sensitive and about 15-20% are non-responders (SRNS). Non-responsiveness of these children would be a risk with the possibility of mutational changes in podocyte genes (NPHS1, NPHS2, WT1, PLCE1). The mutation in podocyte genes is associated with SRNS...
November 25, 2016: Molecular and Cellular Biochemistry
Yong Hee Lee, Yu Bin Kim, Ja Wook Koo, Ju-Young Chung
PURPOSE: To investigate the epidemiology, clinical manifestations, investigations and management, and prognosis of patients with Henoch-Schonlein purpura (HSP). METHODS: We performed a retrospective review of 212 HSP patients under the age of 18 years who were admitted to Inje University Sanggye Paik Hospital between 2004 and 2015. RESULTS: The mean age of the HSP patients was 6.93 years, and the ratio of boys to girls was 1.23:1. HSP occurred most frequently in the winter (33...
September 2016: Pediatric Gastroenterology, Hepatology & Nutrition
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
Fahad Aljebab, Imti Choonara, Sharon Conroy
BACKGROUND: Long courses of oral corticosteroids are commonly used in children in the management of conditions such as nephrotic syndrome, leukaemia, asthma and others. Various adverse drug reactions (ADRs) are known to occur with their use. This systematic review aimed to identify the most common and serious ADRs and to determine their relative risk levels. METHODS: A literature search of several databases; Embase, Medline, International Pharmaceutical Abstracts, CINAHL, the Cochrane Library and PubMed was performed to identify all studies where corticosteroids had been administered to paediatric patients ranging from 28 days to 18 years of age for at least 15 days of treatment...
September 2016: Archives of Disease in Childhood
Syed Sajid Hussain Shah, Farkhanda Hafeez
OBJECTIVE: To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS). STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015. METHODOLOGY: Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included...
July 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
T E Borovik, E K Kutafina, A N Tsygin, T V Sergeeva, A A Baranov, L S Namazova-Baranova, T S Voznesenskaya, I N Zakharova, N N Semenova, N G Zvonkova, S P Yatsyk
The prevalence of various kidney diseases in children remains high in recent decades. Adequate nutrition management can enhance the effectiveness of drug treatment, slow the frequency of relapses andprevent the progression of the disease. The article is devoted to modern approaches to diet therapy in various kidney diseases in children with the defeat of tubular and glomerular appa ratus. For the first time the therapeutic diets for children with various kidney diseases are presented. Particular attention is paid to diet therapy in nephrotic syndrome (steroid-responsive and steroid-refractory)...
2016: Voprosy Pitaniia
Kevin V Lemley, Christian Faul, Karla Schramm, Kevin Meyers, Frederick Kaskel, Katherine M Dell, Debbie S Gipson, Keisha Gibson, Howard Trachtman
Case reports have linked childhood nephrotic syndrome to food sensitivity, including gluten. We report our experience with 8 children (6 boys, 2 girls; age at implementation of special diet 2-14 years) with difficult-to-manage nephrotic syndrome who were placed on a gluten-free diet for 3.4 ± 4.3 years (range, 0.6-14 years) and who had clinical improvement enabling reduction or discontinuation in steroid dosage.
July 2016: Pediatrics
V Kumar, A K Varma, R Nada, R K Ghosh, D Suri, A Gupta, V Kumar, M Rathi, H S Kohli, V Jha, K L Gupta, R Ramachandran
BACKGROUND: Primary membranous nephropathy (PMN) accounts for only 1-2% of nephrotic syndrome in children. Antibodies to m-type phospholipase A2 receptor (aPLA2 R) is seen in 70% of adult PMN cases. The present study was undertaken to study m-type phospholipase A2 receptor (PLA2 R) status and clinical behavior in adolescent PMN cases. METHODS: The present prospective observational study included adolescent (10-19 years) onset biopsy proved PMN. Patients were followed on a monthly basis with urine protein, serum albumin and creatinine...
June 23, 2016: Nephrology
Yo Han Ahn, Eu Jin Park, Hee Gyung Kang, Seong Heon Kim, Hee Yeon Cho, Jae Il Shin, Joo Hoon Lee, Young Seo Park, Kyo Sun Kim, Il-Soo Ha, Hae Il Cheong
BACKGROUND: WT1 is one of the genes commonly reported as mutated in children with steroid-resistant nephrotic syndrome (SRNS). We analyzed genotype-phenotype correlations in pediatric SRNS patients with WT1 mutations. METHODS: From 2001 to 2015, WT1 mutations were detected in 21 out of 354 children with SRNS by genetic screening (5.9 %). The patients were grouped into missense (n = 11) and KTS splicing (n = 10) mutation groups. RESULTS: Nine (82 %) patients with missense mutations presented with congenital/infantile nephrotic syndrome, while 8 (80 %) with KTS splicing mutations presented with childhood-onset SRNS...
June 14, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Michelle R Denburg
PURPOSE OF REVIEW: This review summarizes recent findings on musculoskeletal health in three chronic renal conditions of childhood: chronic kidney disease stages 2-5D, nephrotic syndrome, and urolithiasis. Findings with important clinical implications warranting further investigation are highlighted. RECENT FINDINGS: Recent cohort studies have demonstrated a high burden of fracture and progressive deficits of cortical bone in children with chronic kidney disease...
July 2016: Current Opinion in Nephrology and Hypertension
Hee Gyung Kang, Il-Soo Ha, Hae Il Cheong
Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease and a common pathologic diagnosis of idiopathic nephrotic syndrome (NS), especially in steroid-resistant cases. FSGS is known to recur after kidney transplantation, frequently followed by graft loss. However, not all patients with FSGS suffer from recurrence after kidney transplantation, and genetic and secondary FSGS have a negligible risk of recurrence. Furthermore, many cases of recurrence achieve remission with the current management of recurrence (intensive plasmapheresis/immunosuppression, including rituximab), and other promising agents are being evaluated...
2016: BioMed Research International
S Imtiaz, K Nasir, M F Drohlia, B Salman, A Ahmad
Kidney biopsy occupies a fundamental position in the management of kidney diseases. There are very few renal pathology studies available in the literature from developing world. This study scrutinized the frequency and clinicopathological relationship of kidney biopsies done at the kidney center from 1997 to 2013 amongst pediatric patients. Kidney allograft biopsy were excluded. The specimen was examined under light microscopy and immunofluorescence while electron microscopy was not done. The study includes 423 patients, mean age was 10...
May 2016: Indian Journal of Nephrology
Song-Yi Kim, Ahram Han, Chanjoong Choi, Sang-Il Min, Hyo-Cheol Kim, Jongwon Ha, Seung-Kee Min
Acute arterial thromboembolism (ATE) is rare in childhood, but this medical emergency requires immediate treatment. Described herein are separate instances of lower extremity ATE in 2 children, both of whom were successfully managed through image-guided thromboembolectomy (IGT). One patient, a 34-month-old female child with nephrotic syndrome, developed bilateral iliac and popliteal thromboembolic arterial occlusions after high-dose steroid therapy. Another 9-year-old girl suffered an embolism of left popliteal artery due to infectious endocarditis...
July 2016: Annals of Vascular Surgery
Beth Vogt
Although kidney disease is much less common in children than in adults, clinicians must remain alert for the renal conditions that occur in this population because prompt diagnosis and management are essential. Acute postinfectious glomerulonephritis occurs as an uncommon sequela of streptococcal and other infections. Management is focused on providing supportive measures, including management of fluid-electrolyte imbalance and hypertension, until the patient recovers. Immunoglobulin (Ig) A nephropathy is a primary glomerulonephritis related to abnormal IgA glycosylation...
May 2016: FP Essentials
Biswanath Basu, Binu George Babu, T K S Mahapatra
BACKGROUND: Calcineurin inhibitors (CNI), mycophenolate mofetil (MMF), and levamisole are common treatment choices for patients with frequently relapsing (FRNS) and steroid-dependent nephrotic syndrome (SDNS). METHODS: In this retrospective cohort study, we analyzed the relative efficacy and safety of tacrolimus, MMF, and levamisole over a period of 30 months, in treating 340 children with idiopathic FRNS/SDNS. The children received either MMF 1200 mg/m(2) daily, or levamisole 2...
April 23, 2016: Clinical and Experimental Nephrology
Kenneth V Lieberman, Anna Pavlova-Wolf
Adrenocorticotropic hormone (ACTH) as a treatment for proteinuria due to nephrotic syndrome (NS) has re-emerged over the last decade. Current clinical data are primarily limited to adults with treatment-resistant NS. Largely unknown to today's clinicians is the existence of early clinical studies, following ACTH's introduction in the late 1940s, showing sustained proteinuria response in idiopathic NS in predominantly pediatric, treatment-naïve patients. Before ACTH, patients suffered severe edema and high mortality rates with no reliable or safe treatment...
April 16, 2016: Journal of Nephrology
Diana Rodà, Malka Huici, Sílvia Ricart, Jordi Vila, Clàudia Fortuny, Laia Alsina
Epstein-Barr virus (EBV) infection results in a spectrum of clinical manifestations. The host immune response to EBV plays a key role in the extent and degree of clinical features, which in children under 4 years of age are usually mild, non-specific and self-limiting. A 2-year-old boy in whom no known immune disorder could be found presented with acute acalculous cholecystitis, renal dysfunction with massive proteinuria, ascites, pleural effusion, minimal peripheral oedema and a severe systemic inflammatory response...
January 29, 2016: Paediatrics and International Child Health
Bahia Hassan Moustafa, Omar Atef Tolba
INTRODUCTION: Immunosuppressive agents are recommended for the management of children with steroid-resistant, frequently-relapsing, and steroid-dependent idiopathic nephrotic syndrome, i.e., SRNS, FRNS, and SDNS. This study evaluated the efficacy of immunosuppressive agents in these cases. METHODS: This is a retrospective analysis of the records of 130 pediatric cases recruited from a tertiary-care center over a period of two years. They were divided into two groups, i...
February 2016: Electronic Physician
Syed Sajid Hussain Shah, Farkhanda Hafeez, Naureen Akhtar
BACKGROUND: The management of Steroid Resistant Nephrotic Syndrome (SRNS) is an uphill task for paediatric nephrologists as immunosuppressive agents are the mainstay of treatment in these patients. Tacrolimus is used along with steroids. This study is conducted to see the relationship between the tacrolimus dose, drug level and response in the management of SRNS. METHODS: This quasi experimental study was conducted at The Children's Hospital Lahore over a period of one year...
October 2015: Journal of Ayub Medical College, Abbottabad: JAMC
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