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Dialysis, children, nephrotic syndrome, outcome

Tuula Hölttä, Marjolein Bonthuis, Karlijn J Van Stralen, Anna Bjerre, Rezan Topaloglu, Fatih Ozaltin, Christer Holmberg, Jerome Harambat, Kitty J Jager, Franz Schaefer, Jaap W Groothoff
BACKGROUND: Congenital nephrotic syndrome (CNS) of the Finnish type, NPHS1, is the most severe form of CNS. Outcomes of renal replacement therapy (RRT) in NPHS1 patients in Europe were analysed using data from the ESPN/ERA-EDTA Registry. As NPHS1 is most prevalent in Finland and the therapeutic approach differs from that in many other countries, we compared outcomes in Finnish and other European patients. METHODS: NPHS1 mutations were confirmed in 170 children with CNS who initiated RRT (dialysis or renal transplantation) between 1991 and 2012...
December 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Sudheer Kumar Reddy, Afsana Jahan, Swasti Chaturvedi, Indira Agarwal
BACKGROUND: Outcome data in paediatrics regarding the use of plasmapheresis for immunological kidney disease are scarce. OBJECTIVES: We aimed to evaluate the role of plasmapheresis in children presenting with severe renal impairment secondary to immunological kidney diseases. METHODS: A retrospective chart review of children admitted between January 2009 and August 2013 to the Paediatric Nephrology Unit, Christian Medical College, Vellore, India, and requiring plasma exchange was undertaken...
December 2015: Clinical Kidney Journal
Michiel J S Oosterveld, Mark R Garrelfs, Bernd Hoppe, Sandrine Florquin, Joris J T H Roelofs, L P van den Heuvel, Kerstin Amann, Jean-Claude Davin, Antonia H M Bouts, Pietrik J Schriemer, Jaap W Groothoff
BACKGROUND AND OBJECTIVES: Dense deposit disease (DDD), a subtype of C3 glomerulopathy, is a rare disease affecting mostly children. Treatment options are limited. Debate exists whether eculizumab, a monoclonal antibody against complement factor C5, is effective in DDD. Reported data are scarce, especially in children. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The authors analyzed clinical and histologic data of five pediatric patients with a native kidney biopsy diagnosis of DDD...
October 7, 2015: Clinical Journal of the American Society of Nephrology: CJASN
Manel Jellouli, Sondos Maghraoui, Kamel Abidi, Yosra Hammi, Rim Goucha, Ouns Naija, Chokri Zarrouk, Tahar Gargah
BACKGROUND: Rapidly progressive glomerulonephritis is a rare form of postinfectious glomerulonephritis. The aim of this study was to describe the outcome of our patients with severe post-streptococcal glomerulonephritis. METHODS: This retrospective study was conducted in the department of pediatrics in Charles-Nicolle Hospital during a period of 13 years (1997-2009). RESULTS: Twenty-seven children were identified. The mean age was 8.7 years...
November 2015: Néphrologie & Thérapeutique
Stefaan J Vandecasteele, Sara Ombelet, Sophie Blumental, Willy E Peetermans
BACKGROUND: In the general population, pneumococcal polysaccharide vaccines (PPV) decrease the incidence of invasive pneumococcal disease (IPD) whereas the impact on the prevention of noninvasive pneumococcal disease is less clear. As compared with PPV, pneumococcal conjugate vaccines (PCV) provoke a higher, longer-lasting immune response resulting in a 45% decreased incidence in vaccine-type pneumonia, and a 75% decrease in vaccine-type IPD. METHODS: Literature review on pneumococcal vaccination in end-stage renal disease...
June 2015: Clinical Kidney Journal
Wen Y Ding, Ania Koziell, Hugh J McCarthy, Agnieszka Bierzynska, Murali K Bhagavatula, Jan A Dudley, Carol D Inward, Richard J Coward, Jane Tizard, Christopher Reid, Corinne Antignac, Olivia Boyer, Moin A Saleem
Of children with idiopathic nephrotic syndrome, 10%-20% fail to respond to steroids or develop secondary steroid resistance (termed initial steroid sensitivity) and the majority progress to transplantation. Although 30%-50% of these patients suffer disease recurrence after transplantation, with poor long-term outcome, no reliable indicator of recurrence has yet been identified. Notably, the incidence of recurrence after transplantation appears reduced in patients with steroid-resistant nephrotic syndrome (SRNS) due to monogenic disorders...
June 2014: Journal of the American Society of Nephrology: JASN
Adanze O Asinobi, Adebowale D Ademola, Oluwatoyin O Ogunkunle, Susan A Mott
BACKGROUND: Children and adolescents with end-stage renal disease (ESRD) in sub-Saharan Africa may have the worst outcomes globally. Barriers to management include late presentation, poor socioeconomic conditions, absence of medical insurance, limited diagnostic facilities and non-availability of chronic renal replacement therapy (RRT). Our study was to determine the incidence, aetiology, management and outcomes of paediatric ESRD in a tertiary hospital in Nigeria. METHODS: A retrospective case review of paediatric ESRD at the University College Hospital Ibadan, Nigeria, over 8 years, from January 2005 to December 2012...
2014: BMC Nephrology
Camille Nicolas, Vincent Vuiblet, Veronique Baudouin, Marie-Alice Macher, Isabele Vrillon, Nathalie Biebuyck-Gouge, Maud Dehennault, Sophie Gié, Denis Morin, Hubert Nivet, François Nobili, Tim Ulinski, Bruno Ranchin, Maria Chiarra Marinozzi, Stéphanie Ngo, Véronique Frémeaux-Bacchi, Christine Pietrement
BACKGROUND: C3 glomerulopathy (C3G) is characterized by predominant C3 deposits in glomeruli and dysregulation of the alternative pathway of complement. Half of C3G patients have a C3 nephritic factor (C3NeF). C3G incorporated entities with a range of features on microscopy including dense deposit diseases (DDD) and C3 glomerulonephritis (C3GN). The aim of this work was to study children cases of C3G associated with C3NeF. METHODS: We reviewed 18 cases of C3G with a childhood onset associated with C3NeF without identified mutations in CFH, CFI, and MCP genes...
January 2014: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Alok Sharma, Ruchika Gupta, Charanjit Lal, Sanjay K Agarwal, Amit K Dinda
Membranoproliferative glomerulonephritis (MPGN) is a rare cause of the nephrotic syndrome in adults and children. Though small focal crescents may be seen in up to 10% of cases of MPGN, the presence of more than 50% crescents (crescentic MPGN) is rare. Very few cases of crescentic transformation of MPGN, documented by subsequent renal biopsies, have been described in the literature. A young female patient underwent kidney biopsy for the nephrotic-nephritic syndrome and was diagnosed as idiopathic MPGN. She was administered immunosuppressive therapy (steroids and cyclophosphamide), with which her renal functions stabilized...
March 2013: Saudi Journal of Kidney Diseases and Transplantation
John Dotis, Nikoleta Printza, Chrisa Ghogha, Fotios Papachristou
BACKGROUND: Secondary hyperparathyroidism (HPT) constitute a high-turnover bone disease, manifested by elevated parathyroid hormone. Cinacalcet, belonging to calcimimetics, has been shown to be promising in the control of secondary HPT with limited data in children. OBJECTIVE: To evaluate the safety and efficacy of cinacalcet in children on peritoneal dialysis (PD) with secondary HPT. METHODS: Four patients on PD with severe secondary HPT, uncontrolled with phosphorus dietary restrictions combined with phosphate binders and analog of 1,25 vitamin D3 received cinacalcet...
2013: Journal of Pediatric Endocrinology & Metabolism: JPEM
Imed Helal, Hayet Kaaroud, Rym Goucha, Fatma Ben Moussa, Hedi Ben Maiz, Adel Kheder
INTRODUCTION: Acute post-infectious glomerulonephritis (APIGN) is uncommon in adults. It is widely recognized that the prognosis of APIGN is good in children. There is however little information about its long-term prognosis in adults. METHODS: Between December 1976 and October 2004, 148 adult cases of APIGN were managed in our center. We retrospectively reviewed these patients' records and evaluated their clinical course and outcome. RESULTS: The mean age of studied patients was 36 ± 15 years, and the male to female ratio was 2...
May 2012: Arab Journal of Nephrology and Transplantation
Marta Azocar, Lily Quiroz, Angela Delucchi, Hector Dinamarca, Marcos Emilfork, Francisco Cano
BACKGROUND: Nephrotic syndrome (NS) in children has been associated with a systemic circulating permeability factor. Therefore, once peritoneal dialysis (PD) has been started, peritoneal protein losses should be higher in the nephrotic than in the non-nephrotic population. OBJECTIVE: We compared peritoneal protein losses in children with and without NS on PD. METHODS: Our retrospective 4-year study analyzed Hispanic patients with NS under PD...
July 2012: Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis
Karlijn J Van Stralen, Francesco Emma, Kitty J Jager, Enrico Verrina, Franz Schaefer, Guido F Laube, Malcolm A Lewis, Elena N Levtchenko
BACKGROUND AND OBJECTIVES: Nephropathic cystinosis (NC) is an autosomal recessive disorder occurring in one to two per 100,000 newborns. Because of the rarity of NC, long-term outcome data are scarce. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: 245 NC patients from 18 countries provided data to the ESPN/ERA-EDTA registry. We matched NC patients on renal replacement therapy (RRT) to non-NC children on RRT. RESULTS: Between 1979 and 2008, mean age at the start of RRT among NC children increased by 0...
October 2011: Clinical Journal of the American Society of Nephrology: CJASN
Jameela A Kari, Manal Halawani
Achieving remission in children with steroid-resistant nephrotic syndrome (SRNS) could be difficult. Many immunosuppressive drugs are used with variable success rates. We have studied the response of children with SRNS who presented to our pediatric's renal unit between 2002 and 2007 to various modalities of therapy. We included patients with no response to prednisolone (60 mg/M2/day) after four weeks of therapy; all the patients had renal biopsy and followup duration for at least one year. We excluded patients with congenital nephrotic syndrome, lupus, or sickle cell disease...
May 2010: Saudi Journal of Kidney Diseases and Transplantation
Ryszard Grenda, Wioletta Jarmużek, Barbara Piątosa, Jacek Rubik
Early recurrence of nephrotic syndrome after renal transplantation is a common serious adverse event in children with severe primary FSGS, affecting long-term outcome. There is no consensus in terms of uniform management in these cases. We describe the long-term effect of four unadjusted doses of 375 mg/m(2) i.v. rituximab, given to a five and a half-yr-old, nephrectomized child with immediate recurrence of nephrotic syndrome post-transplantation and dependency from repeated PF. Rituximab was introduced at three months post-transplantation after performing 18 sessions of PF and development of established dependency of the disease from plasma exchange...
September 2011: Pediatric Transplantation
Olivia Boyer, Eve Balzamo, Marina Charbit, Nathalie Biebuyck-Gougé, Rémi Salomon, Marie-Agnès Dragon-Durey, Véronique Frémeaux-Bacchi, Patrick Niaudet
We report 3 children with atypical hemolytic uremic syndrome associated with anti-complement factor H (CFH) autoantibodies who presented with sustained remission with low antibody titers and normal kidney function after plasma exchanges (PEs) and cyclophosphamide pulses. The 3 children initially presented with acute vomiting, fatigue, gross hematuria, hypertension, hemolytic anemia, thrombocytopenia, nephrotic syndrome, and acute kidney injury. C3 levels were normal in patients 1 and 3 and low in patient 2 (0...
May 2010: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Nikoleta Printza, Fotini Koukourgianni, Thaer Saleh, Chrissa Goga, Fotis Papachristou
Acute renal failure (ARF) is a rare but severe complication of active idiopathic nephrotic syndrome (INS) in children. It may be due to several causes with different outcomes. Both the clinical picture of the patient as well as laboratory, imaging and histopathological findings may help in the diagnosis. We present a case of drug-induced acute interstitial nephritis (AIN), complicated with ARF, in a 2(1/2) -year-old girl with active INS. The child was referred to the Hippokration General Hospital, Thessaloniki, Greece hospital with steroid-resistant NS; renal biopsy was performed, which did not show any remarkable findings and cyclosporine was administered in addition to steroid therapy...
November 2009: Saudi Journal of Kidney Diseases and Transplantation
Gianluca Caridi, Maddalena Gigante, Pietro Ravani, Antonella Trivelli, Giancarlo Barbano, Francesco Scolari, Monica Dagnino, Luisa Murer, Corrado Murtas, Alberto Edefonti, Landino Allegri, Alessandro Amore, Rosanna Coppo, Francesco Emma, Tommaso De Palo, Rosa Penza, Loreto Gesualdo, Gian Marco Ghiggeri
BACKGROUND AND OBJECTIVES: Mutations in nephrin (NPHS1) and podocin (NPHS2) genes represent a major cause of idiopathic nephrotic syndrome (NS) in children. It is not yet clear whether the presence of a single mutation acts as a modifier of the clinical course of NS. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We reviewed the clinical features of 40 patients with NS associated with heterozygous mutations or variants in NPHS1 (n = 7) or NPHS2 (n = 33). Long-term renal survival probabilities were compared with those of a concurrent cohort with idiopathic NS...
June 2009: Clinical Journal of the American Society of Nephrology: CJASN
Rajendra Bhimma, Miriam Adhikari, Kareshma Asharam, Catherine Connolly
The burden of chronic kidney disease (CKD) in children in developing countries remains unknown, due to the lack of a national data-reporting system. We undertook a retrospective study of all children < 16 years old in our hospital, which is the tertiary referral centre for children with complex kidney disorders, to analyse the spectrum of CKD (stages 2-5) from 1994-2006. Six hundred and fifty-three children with kidney disorders were screened for CKD; 286 (44.0%) were < 5 years old. Of these, 177 (62%) were male, 202 (70...
October 2008: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
A G Assounga, C Assambo-Kieli, A Mafoua, G Moyen, S Nzingoula
Acute renal failure (ARF) is a common problem in the Congo. This is a six-year retrospective study aiming at analyzing the etiology and the outcome of ARF at the Brazzaville's University Hospital from 1989 through 1994. One hundred and five cases of ARF (0.99%), including 54 boys (51.4%) and 51 girls (48.6%), out of 10,512 children admitted in the department of Pediatrics have been recorded. ARF represented 13.09% of the causes in 802 patients with renal disorder. The main etiologies of ARF included acute gastroenteritis with dehydration (25...
January 2000: Saudi Journal of Kidney Diseases and Transplantation
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