Actualizaciones en el tratamiento del Síndrome Nefrótico Idiopático Recomendaciones de la Rama de Nefrología de la Sociedad Chilena de Pediatría

S, Andrea Vogel; P, Marta Azócar; Nazal, Vilma; C, Paulina Salas del

doi:10.4067/s0370-41062006000300011

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“…El Síndrome Nefrótico (SN) es una enfermedad caracterizada por edema, hipoalbuminemia, proteinuria masiva e hiperlipidemia. Afecta niños principalmente de 2 a 8 años con una incidencia de 2 a 7 casos por 100.000 niños y una prevalencia de 16 casos por 100.000 habitantes, siendo mayor en poblaciones asiáticas y afro-americanas [1][2][3] . La glomerulopatía asociada más frecuente corresponde a los cambios mínimos glomerulares (76%), de buen pronóstico, en contraposición con la glomeruloesclerosis focal y segmentaria (GEFS) (8%), en que un importante porcentaje de ellos no responde al tratamiento inmunosupresor y llegan a insuficiencia renal crónica (IRC) terminal 4 .…”

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Pérdidas Proteicas Peritoneales en Niños Portadores de Síndrome Nefrótico en Diálisis Peritoneal

et al. 2009

Rev. chil. pediatr.

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Primary Nephrotic Syndrome (NS) responds favorably to steroids in 80-90% of cases. Most corticoresistant (CR) patients evolve into Chronic Renal Failure (CRF), Of unknown origin, a permeability factor in these patient's serum has been reported, with some known effects in membranes including the peritoneum. Objective: To evaluate peritoneal protein loss in CR children on Chronic Peritoneal Dialysis (CPD). Patients and Methods: Four year retrospective analysis. Group 1 included 9 CR children, Group 2 was a control group of 10 children with CRF of other causes on CPD. Children in both groups were comparable in age, gender, weight, body surface, duration of CPD, concentration of solution, modality and dose of dialysis. Both groups were evaluated at 1, 6 and 12 months after admission. Results: No differences were observes in biochemical parameters: creatinine, urea nitrogen, calcium, phosphorus. PTH (Parathyroid hormone) was significantly higher in the control group (164 ± 144 vs 564 ± 454 pg/dl p < 0,05), albumin was lower in NS patients at the beginning (2.27 ± 0.63 gr/dl vs 3.62 ± 1.45 gr/dl p < 0,05) and end (2.8 ± 0.5 gr/ dl vs 3.9 ± 0.86 gr/dl, p < 0,05) of the evaluation. Peritoneal protein loss was significantly larger in the index group at the beginning (3,41 ± 2,01 vs 1,76 ± 1,45 gr/m 2 /día), and end (4,27 ± 3,47 vs 1,66 ± 1,31 gr/m 2 /día, (p < 0.05) of the evaluation. The same happened with urinary loss: while there was no difference in protein intake, peritoneal KtV or total KtV between groups, residual KtV was significantly lower among NS patients at the end of the study, suggesting an earlier drop in residual renal function. No differences were observed in rates of peritonitis between groups in the study period. Conclusion: Peritoneal protein loss in CPD children with NS are significantly larger than other patients in CPD, suggesting a possible systemic permeability factor in these patients.

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Section: Introductionunclassified

Pérdidas Proteicas Peritoneales en Niños Portadores de Síndrome Nefrótico en Diálisis Peritoneal

et al. 2009

Rev. chil. pediatr.

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“…En el debut del SN no es posible distinguir entre ambas entidades, sin embargo, la respuesta al tratamiento con esteroides es distinta, con un 95% de los niños con ECM obteniendo remisión completa con prednisona, versus sólo el 30-40% de los pacientes con GEFS, porcentaje que en el tiempo va disminuyendo en relación al número de recaídas 10,11 . El esquema de tratamiento ha sido publicado previamente por la Rama de Nefrología de la Sociedad Chilena de Pediatría 12 .…”

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Síndrome nefrótico cortico-resistente secundario a mutación genética, a propósito de 2 casos clínicos

Sch

2008

Rev. chil. pediatr.

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Corticoresistant Nephrotic Syndrome secondary to genetic mutation, 2 Case-report Background: Around 10-30% patients with Corticoresistant Nephrotic Syndrome (CRNS) have hereditary glomerulopathy. Objective: Describe 2 children with CRNS on chronic peritoneal dyalisis (CPD), with positive Podocin mutation genetic study. Case-report 1: A 4 years-old male with CRNS and diagnosis of focal segmental glomerulosclerosis (FSGS) at renal biopsy, with positive R 229Q and A 284V Podocin mutation genetic study. The treatment included Enalapril, steroids and Cyclophosphamide without remission, requiring CPD at 12 years-old. Case-report 2: A 6 years-old female with CRNS and diagnosis of focal segmental glomerulosclerosis (FSGS) at renal biopsy, with positive R 229Q and A 284V Podocin mutation genetic study. The treatment included Enalapril, steroids and Cyclophosphamide without remission, requiring CPD at 7 years-old. Conclusion: The genetic mutation study should be included in all CRNS cases, in order to guide the therapy and prognosis of the disease.

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Actualizaciones en el tratamiento del Síndrome Nefrótico Idiopático Recomendaciones de la Rama de Nefrología de la Sociedad Chilena de Pediatría

Cited by 2 publications

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Pérdidas Proteicas Peritoneales en Niños Portadores de Síndrome Nefrótico en Diálisis Peritoneal

Pérdidas Proteicas Peritoneales en Niños Portadores de Síndrome Nefrótico en Diálisis Peritoneal

Síndrome nefrótico cortico-resistente secundario a mutación genética, a propósito de 2 casos clínicos

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