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Abstract Introduction: The aim of the study is to compare clinical data of primary focal segmental glomerulosclerosis patients with other data in the literature. In addition, initial immunosuppressive therapy (steroid, calcineurin inhibitors) responses are aimed to be compared with the results in the literature. Methods: Forty seven patients, who were followed up for at least 12 months with primary focal segmental glomerulosclerosis as a result of kidney biopsy. Results of biochemical tests and treatment modalities were evaluated. Results: The mean age of the 47 patients with primary focal segmental glomerulosclerosis was 45.68 (± 13.92). Twenty-one (44.6 %) of 47 patients had anangiotensin converting enzyme inhibitor / angiotensin receptor blocker, 7 (14.8 %) had only corticosteroids, and 6 (12. 7%) had calcineurin inhibitor + low-dose corticosteroids treatment. Thirteen patients (27.6 %) used calcineurin inhibitor + low-dose corticosteroids after receiving corticosteroids treatment. The patients who received corticosteroids or calcineurin inhibitor + low-dose corticosteroids treatment as the initial treatment were compared at the 0, 6 and 12 months of treatment in terms of laboratory and clinical features. At the end of the first year, 4 out of 6 (66 %) patients with low-dose corticosteroids and calcineurin inhibitor and 6 out of (86 %) of 7 patients with corticosteroids had remission (p>0.05). Conclusion: We found the initial steroid treatment and calcineurin inhibitor treatment to be equally effective. We thought that patients with steroid intolerance could be given calcineurin inhibitor in the first step, but if the cost is considered, the first option, such as The Kidney Disease Improving Global Outcomes recommendation, was again steroid.
Resumen Introducción:El objetivo del estudio es comparar los datos clínicos de pacientes con glomeruloesclerosis segmentaria focal primaria con otros datos de la bibliografía. Asimismo, se busca comparar las respuestas de la terapia inmunosupresora inicial (esteroides, inhibidores de la calcineurina) con los resultados en las publicaciones citadas. Material y métodos: Se incluyeron 47 pacientes con glomeruloesclerosis segmentaria focal primaria como resultado de una biopsia renal, y cuyo seguimiento duró, al menos, 12 meses. Se evaluaron los resultados de las pruebas bioquímicas y las modalidades de tratamiento. Resultados: La edad media de los 47 pacientes con glomeruloesclerosis segmentaria focal primaria fue de 45,68 (± 13,92). 21 (44,6 %) del total de los pacientes tenían un inhibidor de la enzima convertidora de la angiotensina / bloqueante del receptor de la angiotensina. Siete pacientes (14,8 %) solo tenían corticosteroides y 6 (12,7 %) tenían un inhibidor de la calcineurina + un tratamiento con esteroides en dosis bajas. Se aplicóinhibidores de la calcineurina + dosis bajas de corticoesteroides en13 pacientes (27,6 %) después de recibir tratamiento con ciclosporina. Los pacientes que recibieron ciclosporina o inhibidores de la calcineurina+ dosis bajas de esteroides como tratamiento inicial se compararon al inicio del tratamiento, a los 6 y 12 meses en sus parámetros de laboratorio y características clínicas. Al final del primer año, 4 de 6 pacientes (66,7 %) con dosis bajas de ciclosporina e inhibidores de la calcineurina y 6 de 7 pacientes (85,7 %) con ciclosporina presentaron remisión (p>0,05). Conclusión: Se encontró que el tratamiento inicial con esteroides y el tratamiento con inhibidores de la calcineurinason igualmente efectivos. Pensamos que, a los pacientes con intolerancia a los esteroides, se les podría administrarinhibidores de la calcineurinaen el primer paso, pero si se considera el costo, la primera opción son nuevamente los esteroides, como lo recomienda el consorcio Kidney Disease Improving Global Outcomes.
ABSTRACT
BACKGROUND: Primary focal segmental glomerulosclerosis (FSGS) is a cause of nephrotic syndrome in adult. Although primary FSGS has been known to be refractory to treatment, recent studies reveal higher remission rate and better prognosis. And it has been reported that some clinical and histopathologic parameters are significant to prognosis. But, confirmative prognostic indices remain to be defined. In order to further clarify the prognostic factors for therapeutic response and risk factors for progression to chronic renal failure (CRF), we reviewed the medical records of primary adult FSGS patients. METHODS: Forty-adult patients diagnosed as primary FSGS between 1991 to 2002 were enrolled. We retrospectively analyzed the clinical and histopathological parameters of all patinents at the time of renal biopsy. In addition, the therapeutic responses to immunosuppressants and the renal survival were analyzed. RESULTS: At the time of renal biopsy, 26 patients (65%) had proteinuria of the nephrotic range and 14 patients (35%) had proteinuria of the non-nephrotic range. The serum creatinine level was higher in nephrotic-ranged patients than that in non nephrotic-ranged patients (p<0.05). The other parameters were not significantly different between two groups. Twenty-seven patients were treated with immunosuppressants and 15 patients (55.6%) responded to the treatment. There was no significant difference in clinical or histopathological variables between the responders and the non-responders. High serum creatinine level at diagnosis and responsiveness to treatment appeared to be significant as risk factors for progression to CRF (p<0.05). The patients treated with immunosuppressants had longer survival period, compared with those without treatment. And the responders had significantly longer survival period compared with the non-responders (p<0.05). CONCLUSION: The patients with initial impairment of renal function or poor response to therapy may have worse prognosis, and the intense treatment with regular follow-up of renal function should be recommended for these patients.
Subject(s)
Adult , Humans , Biopsy , Creatinine , Diagnosis , Follow-Up Studies , Glomerulosclerosis, Focal Segmental , Immunosuppressive Agents , Kidney Failure, Chronic , Medical Records , Nephrotic Syndrome , Prognosis , Proteinuria , Retrospective Studies , Risk FactorsABSTRACT
0.05).Two cases were lost to follow up,19 out of 21 cases were followed up.The follow-up duration was from 4 to 51 months[mean follow-up duration was(22.84?12.13) months].At last follow-up,10 cases(53%) had complete remission,4 cases(21%) had significant remission,4 cases(21%) had partial remission and 1 case(5%) had no remission.Tubulointerstitial lesions were related with prognosis(P1).Conclusions 1.The predominant clinical manifestation of primary FSGS is nephrotic syndrome in children.The not otherwise specified variant is the commonest pathological variant in primary FSGS.There is no relationship between patholo-gical types and clinical manifestations of FSGS.2.Most of the children with primary FSGS have a good short-term prognosis,but their long-term prognosis must be followed up.3.Tubulointerstitial lesions are risk factors for prognosis.