RESUMEN
BACKGROUND/AIM: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schönlein purpura (HSP) nephritis in children. METHODS: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). RESULTS: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. CONCLUSION: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely.
Asunto(s)
Vasculitis por IgA/epidemiología , Vasculitis por IgA/patología , Nefritis/epidemiología , Nefritis/patología , Adolescente , Niño , Preescolar , Comorbilidad , Femenino , Humanos , Incidencia , Masculino , Reproducibilidad de los Resultados , Medición de Riesgo , Factores de Riesgo , Sensibilidad y Especificidad , Turquía/epidemiologíaRESUMEN
OBJECTIVE: Associations between human leukocyte antigens (HLA) and sarcoidosis have been reported in several studies. We aimed to investigate these associations in Turkish patients. PATIENTS AND METHOD: We performed HLA-A, HLA-B, HLA-C, and HLA-D typing in 83 patients with sarcoidosis and in 250 healthy controls using a microlymphocytotoxicity method to investigate genetic susceptibility to the disease. RESULTS: Because of significant violation of Hardy-Weinberg equilibrium at HLA-C and HLA-DQB1 loci, only results obtained at other HLA loci were used. Although HLA-A9, HLA-B5, and HLA-B8 allele frequencies were significantly higher in the patient group compared to the controls (odds ratio [OR]= 21.8, P= .015; OR= 9.34, P= .049; OR= 2.26, P= .031, respectively), none of the differences remained significant after applying the Bonferroni correction. HLA-A24, HLA-A26, and HLA-B62 alleles were significantly less frequent in the patient group compared to the controls (OR= 0.48, P= .018; OR= 0.19, P= .003; OR= 0.11, P= .044, respectively). However, the differences also failed to remain significant after Bonferroni correction. CONCLUSIONS: These results suggest that both HLA may play significant roles (either increasing or reducing risk) in the pathogenesis of sarcoidosis and in its distinct clinical forms and laboratory findings.
Asunto(s)
Antígenos HLA-A/sangre , Antígenos HLA-B/sangre , Sarcoidosis/sangre , Sarcoidosis/inmunología , Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , TurquíaRESUMEN
OBJETIVO: En varios estudios se ha demostrado la existencia de asociaciones entre los antígenos leucocitarios humanos (HLA) y la sarcoidosis. El objetivo de nuestro estudio ha sido la investigación de estas asociaciones en pacientes turcos. PACIENTES Y MÉTODO: Se ha realizado la tipificación HLAA, HLA-B, HLA-C y HLA-C en 83 pacientes con sarcoidosis y en 250 controles sanos mediante un método de microlinfocitotoxicidad, con objeto de determinar la susceptibilidad frente a la enfermedad. RESULTADOS: Debido a la importante violación del equilibrio de Hardy-Weinberg en los loci HLA-C y HLA-DQB1, sólo se utilizaron los resultados obtenidos en los demás loci HLA. Aunque las frecuencias de los alelos HLA-A9, HLAB5 y HLA-B8 fueron significativamente mayores en el grupo de pacientes que en el grupo control (cociente de posibilidades [CP] = 21,8, p = 0,015; CP = 9,34, p = 0,049; CP = 2,26, p = 0,031, respectivamente), ninguna de estas diferencias mantuvo la significación estadística tras la aplicación de la corrección de Bonferroni. Los alelos HLA-A24, HLA-A26 y HLA-B62 fueron significativamente menos frecuentes en el grupo de pacientes que en el grupo de controles (CP = 0,48, p = 0,018; CP = 0,19, p = 0,003; CP = 0,11, p = 0,044, respectivamente). Sin embargo, las diferencias tampoco fueron estadísticamente significativas después de la corrección de Bonferroni. CONCLUSIONES: Estos resultados indican que los HLA pueden desempeñar una función significativa (con aumento o reducción del riesgo) en la patogenia de la sarcoidosis, así como en sus diferentes formas clínicas y en sus alteraciones analíticas (AU)