IgM nephropathy in children : clinicopathologic analysis [documento electrónico] / Catalina Vélez Echeverri, ; Lina Maria Serna Higuita, ; Carolina Lucía Ochoa García, ; Juan José Vanegas Ruiz,  . - 2013.
Obra : Nefrología

Idioma : Inglés (eng)

Palabras clave :	Minimal change disease  glomerular diseases  focal segmental glomeruloesclerosis  nephrotic syndrome  IgM nephropathy
Resumen :	Introduction: IgM nephropathy (IgMN) is a glomerulonephritis characterised by diffuse mesangial immunoglobulin M (IgM) deposits. It usually presents with nephrotic range proteinuria and, according to some previous work, it occurs most often in patients who are resistant to or dependent on steroid treatment. Objective: To perform a clinical, histological and immunopathological description and assess the response to steroid treatment of paediatric patients diagnosed with nephrotic syndrome and diffuse mesangial IgM deposits. Method: This is a descriptive, retrospective study carried out in two hospitals, where the clinical records of paediatric patients with IgMN were analysed and the histological sections were re-assessed. Results: thirteen children were included in this study. IgMN corresponded to 5.17% of all paediatric renal biopsies. The age of patients ranged from 1 year to 12 years (median: 2 years), 46.7% were women. The most common morphological finding was diffuse mesangial hypercellularity (46.1%), followed by focal segmental glomerulosclerosis (30.8%) and minimal glomerular changes (23.1%). All patients received steroids; in 4 cases (30.7%) as the only immunosuppressant medication, 3 (23.1%) also received cyclophosphamide, 5 (38.4%) mycophenolate, and 1 (7.7%) cyclosporine. Seven patients (53.8%) had frequent relapses, 5 (38.5%) were cortico-resistant and 1 (7.7%) cortico-dependent. Two patients (15.38%) had chronic impairment of renal function. Conclusion: The presence of diffuse mesangial IgM in paediatric patients with nephrotic syndrome is not a very uncommon finding; its clinical presentation has been associated with lower response to steroids. However, the long-term prognosis of these patients is still unknown.
Mención de responsabilidad :	Luis F Arias, M Claudia Prada, Catalina Vélez-Echeverri, Lina M Serna-Higuita, Ana K Serrano-Gayubo, Carolina L Ochoa, Juan J Vanegas-Ruiz
Referencia :	Nefrologia. 2013;33(4):532-8.
DOI (Digital Object Identifier) :	10.3265/Nefrologia.pre2013.Mar.11962
PMID :	23897185
Derechos de uso :	CC BY-NC-ND
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