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https://www.readbyqxmd.com/read/29786149/national-survey-found-that-managing-childhood-nephrotic-syndrome-in-nigeria-varied-widely-and-did-not-comply-with-the-best-evidence
#1
Christopher I Esezobor, Adanze O Asinobi, Henrietta U Okafor, Rosamund Akuse, Rasheed Gbadegesin
AIM: This study explored any variations in managing childhood nephrotic syndrome between specialist centres in Nigeria and how closely the care reflected the best available evidence. METHODS: In 2016 the heads of Nigerian paediatric nephrology units were asked to complete a study questionnaire that focused on managing nephrotic syndrome. RESULTS: Of the 31 clinicians we approached, 81% returned the completed questionnaire. The majority (64%) had received paediatric nephrology training and 40% had practised for at least 10 years...
May 22, 2018: Acta Paediatrica
https://www.readbyqxmd.com/read/29769504/dipstick-method-versus-spot-urinary-protein-creatinine-ratio-for-evaluation-of-massive-proteinuria-in-childhood-nephrotic-syndrome
#2
M N Sultana, B Majumder, M J Rahman, A M Moniruzzaman, A M Suja, M E Ali, Z H Sarker, S N Nabi, M A Mostakim
Measurement of massive proteinuria is vital for diagnosis of childhood Nephrotic syndrome. Quantification of 24 hours urinary protein is the gold standard test. Dipstick method of urinary protein measurement gives instant result for massive proteinuria. Spot urinary protein creatinine ratio measurement is variable. This was a hospital based prospective cross sectional study done at Department of Paediatric Nephrology, Rangpur Medical College Hospital from January 2014 to December 2015 to evaluate accuracy of dipstick method versus spot urinary protein-creatinine ratio in estimation of massive proteinuria in childhood nephrotic syndrome...
April 2018: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29385997/proteinuric-kidney-disease-in-children-at-queen-elizabeth-central-hospital-malawi
#3
Zondiwe Victor Mwanza, Mignon McCulloch, Mark Drayson, Timothy Plant, David V Milford, Gavin Dreyer
BACKGROUND: There is a paucity of data on paediatric kidney disease in developing countries such as Malawi. Descriptive research on kidney disease is essential to improving patient outcomes. METHODS: We conducted a cross-sectional study at a tertiary hospital in Malawi from 2012 to 2013. Children under 14 years with proteinuric kidney disease were enrolled from paediatric wards and outpatient clinics at Queen Elizabeth Central Hospital (QECH). Demographic, clinical and laboratory data were collected from patients at enrolment and at 3 months review at which point clinical status and disease outcome were ascertained...
January 31, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29207799/change-in-bone-mineral-density-and-role-of-vitamin-d-and-calcium-supplementation-during-treatment-of-first-episode-nephrotic-syndrome
#4
Virendra Kumar Yadav, Shobha Sharma, Pradeep Kumar Debata, Seema Patel, Bhaskar C Kabi, Kailash Chandra Aggrawal
Introduction: Nephrotic Syndrome (NS) is one of the common illnesses in childhood. Oral glucocorticoids are mainstay of treatment, which are known to cause various short term and long term adverse effects including osteoporosis. Children with first episode and infrequent relapsing NS are not prescribed Calcium (Ca) and Vitamin D (VitD) supplements routinely. Aim: To observe change in Bone Mineral Density (BMD) in children during first episode of NS and role of VitD and Ca supplementation during the treatment of first episode of NS...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29155175/fcgr2a-single-nucleotide-polymorphism-confers-susceptibility-to-childhood-onset-idiopathic-nephrotic-syndrome
#5
Giovanni M Rossi, Francesco Bonatti, Alessia Adorni, Federico Alberici, Monica Bodria, Alice Bonanni, Gian M Ghiggeri, Davide Martorana, Augusto Vaglio
Childhood-onset idiopathic nephrotic syndrome affects 1.15-3.4 children/100,000 children/year in Western Countries. Immune-mediated mechanisms, particularly T cell-mediated, are thought to play a key pathogenic role. The genetic basis of the disease is still poorly understood. We tested the association between single nucleotide polymorphisms (SNPs) of four genes encoding Fc gamma receptors (FCGR2A, FCGR2B, FCGR3A, FCGR3B) and idiopathic nephrotic syndrome in a case-control study of paediatric patients. Children with idiopathic nephrotic syndrome (aged 1-16 years) were included...
January 2018: Immunology Letters
https://www.readbyqxmd.com/read/29141789/a-case-of-seropositive-neuromyelitis-optica-in-a-paediatric-patient-with-co-existing-acute-nephrotic-syndrome
#6
Thomas Volkman, Cheryl Hemingway
Neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD) is a rare relapsing autoimmune disease of the central nervous system constituting less than 1% of demyelinating diseases (Jeffery and Buncic, 1996). It preferentially affects the optic nerves and spinal cord, with the brain parenchyma generally spared. Demyelinating lesions are characterised by longitudinally extensive transverse myelitis (LETM) and often longitudinally extensive optic neuritis. Following the discovery of a novel pathogenic antibody, Aquaporin 4 in 2004 (Lennon et al...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28992235/treatment-by-immunoadsorption-for-recurrent-focal-segmental-glomerulosclerosis-after-paediatric-kidney-transplantation-a-multicentre-french-cohort
#7
Lise Allard, Theresa Kwon, Saoussen Krid, Justine Bacchetta, Arnaud Garnier, Robert Novo, Georges Deschenes, Rémi Salomon, Gwenaëlle Roussey, Emma Allain-Launay
Background: Primary focal segmental glomerulosclerosis (FSGS) frequently recurs after kidney transplantation (KTx) in children. This can lead to delayed graft loss. As the management of children with recurrent FSGS is not well established, apheresis strategies could be a cornerstone to control the disease. Immunoadsorption (IA) is a recent apheresis therapy. There have been few studies examining IA in this setting. We report the results of IA for management of recurrent FSGS after KTx in children in France...
July 28, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#8
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28846194/spectrum-of-biopsy-proven-renal-disease-in-south-asian-children-two-decades-at-a-tropical-tertiary-care-centre
#9
Anjali Mohapatra, Shailesh Kakde, Vellaichamy M Annapandian, Anna T Valson, Neelaveni Duhli, Anila Korula, Smita Mary Matthai, Anna B Pulimood, Vinoi G David, Suceena Alexander, Shibu Jacob, Santosh Varughese, Basu Gopal, Tamilarasi Veerasamy, George T John
AIM: We report findings from a large single centre paediatric renal biopsy cohort in South Asia. METHODS: We analyzed all renal biopsies performed on children aged ≤18 years between 1996 and 2015 at our centre. The clinical characteristics and histological diagnosis pertaining to each case, distribution of renal diseases in children with various clinical presentations, and changes in the pattern of kidney disease during the study period were analyzed. RESULTS: A total of 1740 paediatric kidney biopsies were performed during the study period...
August 28, 2017: Nephrology
https://www.readbyqxmd.com/read/28780565/clinical-genetic-testing-using-a-custom-designed-steroid-resistant-nephrotic-syndrome-gene-panel-analysis-and-recommendations
#10
Ethan S Sen, Philip Dean, Laura Yarram-Smith, Agnieszka Bierzynska, Geoff Woodward, Chris Buxton, Gemma Dennis, Gavin I Welsh, Maggie Williams, Moin A Saleem
BACKGROUND: There are many single-gene causes of steroid-resistant nephrotic syndrome (SRNS) and the list continues to grow rapidly. Prompt comprehensive diagnostic testing is key to realising the clinical benefits of a genetic diagnosis. This report describes a bespoke-designed, targeted next-generation sequencing (NGS) diagnostic gene panel assay to detect variants in 37 genes including the ability to identify copy number variants (CNVs). METHODS: This study reports results of 302 patients referred for SRNS diagnostic gene panel analysis...
December 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28639503/markers-of-disease-and-steroid-responsiveness-in-paediatric-idiopathic-nephrotic-syndrome-whole-transcriptome-sequencing-of-peripheral-blood-mononuclear-cells
#11
Hee Gyung Kang, Heewon Seo, Jae Hyun Lim, Jong Il Kim, Kyoung Hee Han, Hye Won Park, Ja Wook Koo, Kee Hyuck Kim, Ju Han Kim, Hae Il Cheong, Il-Soo Ha
Objective To identify markers of disease and steroid responsiveness in paediatric idiopathic nephrotic syndrome. Methods Whole-transcriptome sequencing was performed of peripheral blood mononuclear cells (PBMCs) from patients with NS. Differentially expressed genes (DEGs) were identified in patients with active NS vs those in remission, and those with steroid-sensitive NS (SSNS) vs steroid-resistant NS (SRNS). Results A total of 1065 DEGs were identified in patients with NS ( n = 10) vs those in remission ( n = 9)...
June 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28630858/pulsed-vincristine-therapy-in-steroid-resistant-nephrotic-syndrome
#12
Shenal Thalgahagoda, Shamali Abeyagunawardena, Heshan Jayaweera, Umeshi Ishanthika Karunadasa, Asiri Samantha Abeyagunawardena
Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. Methods . Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28547746/the-support-needs-of-parents-having-a-child-with-a-chronic-kidney-disease-a-focus-group-study
#13
W W Geense, B G I van Gaal, J L Knoll, E A M Cornelissen, T van Achterberg
BACKGROUND: Parents of children with a chronic kidney disease (CKD) have a crucial role in the management of their child's disease. The burden on parents is high: they are often exhausted, depressed and experience high levels of stress and a low quality of life, which could have a negative impact on their child's health outcomes. Support aiming at preventing and reducing parental stress is essential. Therefore, it is necessary to have insight in the problems and support needs among these parents...
November 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28541783/cyclosporine-causes-no-hearing-defect-in-paediatric-patients-with-nephrotic-syndrome
#14
Belde Kasap-Demir, Derya Özmen, Günay Kırkım, Ersoy Doğan, Alper Soylu, Bülent Şerbetçioğlu, Salih Kavukçu
OBJECTIVE: We aimed to evaluate the ototoxicity of cyclosporine A (CsA) in children with nephrotic syndrome (NS). DESIGN: Data of paediatric patients with NS followed in paediatric nephrology department were evaluated retrospectively, and hearing functions were evaluated by pure tone audiometry (PTA) and transient evoked otoacoustic emissions (TEOAEs). Age, gender, type of NS, duration and cumulative doses of immunosuppressives were noted. STUDY SAMPLE: The patients who had received CsA (n: 16) and immunosuppressives other than CsA (n: 13) for at least 6 months formed two patient groups and healthy cases formed a control group (n: 20)...
May 25, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28515029/tolvaptan-treatment-in-children-with-chronic-hyponatremia-due-to-inappropriate-antidiuretic-hormone-secretion-a-report-of-three-cases
#15
Gerdi Tuli, Daniele Tessaris, Silvia Einaudi, Luisa De Sanctis, Patrizia Matarazzo
Hyponatremia is the most common electrolyte disorder among hospitalized patients and it is sometimes considered as a poor outcome predictor. Its correction is thus indicated, even in asymptomatic patients. The conventional treatment consists of fluid restriction in presence of euvolemia or hypervolemia; loop diuretics are used in some hypervolemic conditions such as cardiac heart failure, liver cirrhosis and nephrotic syndrome, while intravenous isotonic or hypertonic solutions are administered in hypovolemic conditions...
September 1, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28463080/lupus-glomerulonephritis-in-788-chinese-children-a-multi-centre-clinical-and-histopathological-analysis-based-on-549-renal-biopsies
#16
Si-Yan Jin, Dan-Lin Huang, Xi-Qiang Dang, Zhu-Wen Yi
BACKGROUND: System lupus erythematosus (SLE) is a severe multisystem autoimmune disease. OBJECTIVE: To describe the clinical and pathological features, treatment, and renal outcome in children under 18 years with lupus nephritis (LN). METHODS: The study was undertaken by a questionnaire completed in 26 Grade 3A hospitals' paediatric renal units in China. The study comprised 788 children (619 girls, 169 boys) diagnosed with SLE by the American College of Rheumatology criteria (1997) during 2005-2010...
November 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-the-adult-population-of-poland
#17
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
April 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28112049/kidney-disease-in-hepatitis-b-surface-antigen-positive-children-experience-from-a-centre-in-south-west-nigeria-and-a-review-of-the-nigerian-literature
#18
Adanze O Asinobi, Adebowale D Ademola, Clement A Okolo, Adedayo A Adepoju, Susan M Samuel, Wendy E Hoy
BACKGROUND: Kidney disease is an important extra-hepatic manifestation of hepatitis B virus (HBV) infection. However, there is paucity of recent literature on kidney disease in children and adolescents with HBV infection from several parts of sub-Saharan Africa including Nigeria. OBJECTIVE: To review the pattern of kidney disease in hepatitis B surface antigen (HBsAg)-positive children and adolescents seen at a tertiary hospital in south-west Nigeria. METHODS: A retrospective study was undertaken of HBsAg-seropositive children with kidney disease managed at University College Hospital, Ibadan, from January 2004 to December 2015...
February 2018: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28058746/multidimensional-impact-on-families-of-children-with-steroid-sensitive-nephrotic-syndrome
#19
COMPARATIVE STUDY
Gurdeep S Dhooria, Harmeet P Singh, Deepak Bhat, Harmesh S Bains, Ravinder K Soni, Mohit Kumar
AIM: Children with nephrotic syndrome (NS) have prolonged disease course with relapses requiring frequent visits and prolonged steroid therapy with their long-term concerns. All these factors affect the child and their families in many domains of functioning. The objective of this study was to assess multidimensional impact on families of children with nephrotic syndrome using (PedsQL) Family Impact Module (FIM). METHODS: This cross-sectional study was conducted in a paediatric nephrology clinic of a tertiary care hospital...
April 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/27795625/a-prospective-audit-of-complications-in-100-consecutive-pediatric-percutaneous-renal-biopsies-done-under-real-time-ultrasound-guidance
#20
R Sinha, B Maji, B Sarkar, S Meur
Despite being a common procedure, percutaneous renal biopsy (PRB) carries the potential for complications. The British Association of Paediatric Nephrologist (BAPN) has published standards for pediatric PRB. As Indian data are scarce, we conducted a prospective audit of 100 consecutive pediatric renal biopsies (60% males) under real-time ultrasound guidance. Nephrotic syndrome was the most common indication for PRB (68%) with minimal change disease (30%) and focal segmental glomerulosclerosis (25%) being the most common histopathological lesions...
September 2016: Indian Journal of Nephrology
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