Primer caso registrado en Cuba de nefropatía C1q / First case of C1q nephropathy in Cuba
Rev. cuba. pediatr
; 85(2)abr.-jun. 2013.
Article
in Spanish
| CUMED
| ID: cum-61098
Responsible library:
CU1.1
Localization: CU1.1
RESUMEN
La nefropatía C1q es una glomerulopatía no comprendida completamente y con algunas controversias conceptuales, pero con característica inmunológica distintiva (depósito dominante o co-dominante de C1q) y no evidencia clínica ni serológica de lupus eritematoso sistémico. Se presenta paciente masculino que comienza a los 10 meses de edad con un síndrome nefrótico con hematuria, hipertensión arterial e insuficiencia renal. Se realiza una primera biopsia renal y se plantea una esclerosis mesangial difusa, pero su evolución posterior con respuesta parcial a la prednisona y el mantenimiento de proteinuria en rango nefrótico con normalización de los parámetros humorales, nos lleva a realizar una segunda biopsia renal que arroja, por la inmunofluorescencia, una nefropatía C1q(AU)
ABSTRACT
C1q nephropathy is a poorly understood glomerulopathy with some conceptual controversies, but with a distinctive immunologic characteristic (dominant or co-dominant deposit of C1q) and neither clinical nor serological evidence of systemic erythematous lupus. This is the case of a male patient who began suffering nephritic syndrome with hematuria, blood hypertension and renal failure at 10 months of age. A first renal biopsy was performed to detect diffuse mesangeal sclerosis; however after partial response of the patient to prednisone therapy and to maintenance treatment of proteinuria in nephritic range, with normalization of humoral parameters, then a second renal biopsy was performed with immunofluorescence. The final result was C1q nephropathy(AU)
Full text:
Available
Collection:
National databases
/
Cuba
Database:
CUMED
Main subject:
Nephrosis, Lipoid
/
Nephrotic Syndrome
Limits:
Child
/
Humans
Country/Region as subject:
Cuba
Language:
Spanish
Journal:
Rev. cuba. pediatr
Year:
2013
Document type:
Article
Institution/Affiliation country:
Hospital William Soler/Cuba