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Clinical and pathological characteristics of primary focal segmental glomerulosclerosis in adult
الخصائص السريرية والمرضية للتصلب الكبيبي القطاعي الأساسي في البالغين

Authors: Salam Fareed Wadee --- Safa Ezzidin Al-Mukhtar
Journal: Zanco Journal of Medical Sciences مجلة زانكو للعلوم الطبية ISSN: 19955588/19955596 Year: 2017 Volume: 21 Issue: 1 Pages: 1645-1652
Publisher: Hawler Medical Univeristy جامعة هولير الطبية

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Abstract

Background and objective: Focal segmental glomerulosclerosis shares overlapping patterns of injury with segmental consolidation and obliteration of glomerular architecture by the accumulation of collagenous extracellular matrix or by increased cellularity or both. This study aimed to investigate the patterns of primary focal segmental glomerulosclerosis in adults in Erbil. The specific objectives of this study included determining the frequency of histological variant of Focal segmental glomerulosclerosis, defining the clinical characteristics of the primary type in general and recognizing the clinical and pathological characteristics for each variant separately.Methods: A cross-sectional, clinico-pathologic study was conducted for 50 patients attending the Nephrology Department in Erbil Teaching Hospital between March and December 2013. This study included patients of >16 years old with biopsy-proven idiopathic focal segmental glomerulosclerosis. Results: A total of 50 patients were enrolled into this study. The median age of patients was 33 years, ranged from 18 to 54, 31 (62%) males and 19 (38%) females. The frequency of histopathological variants was 80% not otherwise specific focal segmental glomerulosclerosis, 14% glomerular tip lesion, and 6% cellular type. Nephrotic syndrome and hypertension were the main presenting features (92% and 68%, respectively). The mean percentage of sclerosed glomeruli was 40% and the mean interstitial fibrosis and tubular atrophy was 24%.Conclusion: A not otherwise specific variant is a common morphological lesion in many glomerular and non-glomerular diseases, and it is just like a junk drawer of multiple glomerular alterations with this common pattern of the lesion.


Article
Clinical Course of Children and Adolescents with Primary Focal Segmental Glomerulosclerosis and the Predictors of Their Outcome

Author: Faleeha Obaid Hasan
Journal: Iraqi Academic Scientific Journal المجلة العراقية للاختصاصات الطبية ISSN: 16088360 Year: 2008 Volume: 7 Issue: 4 Pages: 351-357
Publisher: The Iraqi Borad for Medical Specialization المجلس العراقي للاختصاصات الطبية

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Abstract

ABSTRACT:BACKGROUND:The aim of this retrospective study is to report the clinical course of children and adolescents with primary focal segmental glomerulosclerosis (FSGS), and to study their outcome and to identify the predictors of progression to end stage renal disease (ESRD).PATIENTS AND METHODS:This is a retrospective study of 50 patients with biopsy-proven primary FSGS who were admitted from April 1995 - January 2007, during the study period from May 2005 - June 2007. Clinical, Laboratory and histopathological data were recorded. The median follow-up time of 4.5 year.RESULTS:The commonest age and sex group is male between 1 - 5 year. At presentation all patients had nephrotic-range proteinuria, hypertension was noted in 22 (44%) of patients, microscopic haematuria was detected in 20 (40%) of patients, five patients had evidence of abnormal renal function. The distribution of patients according to steroid responsiveness show that the steroid sensitive patients were 21 (42%), 8 (38.09%) of them were frequent relapsers and 11 (52.38%) of them were steroid dependant and 2 (9.52%) of them developed secondary steroid resistance. But those who had steroid resistance from the start of treatment were 29 (58%) patients. During follow-up 30 (62%) patients had complete remission, 15 patients (30%) developed chronic kidney disease (9 of them stage 5).At the end of follow-up, 24 (80%) of 30 patients with normal renal function had short stature. The univariate analysis identified the presence of hypertension (P=0.0027), heamaturia (P=0.0107) and presence of abnormal renal function (P=0.0001) at presentation, also presence of initial steroid resistance (P=0.0383), resistance to cytotoxic therapy (P=0.0032), capsular adhesions in renal biopsy (P=0.0066), tubular atrophy (P=0.0027), interstitial fibrosis (P=0.0010), all expect to be significant predictors of progression to ESRD.CONCLUSION:Considering the clinical and histological characteristics of studied patients, apparently our results are comparable to other published series. The progression to chronic kidney disease (CKD) occurs in 30% of patients after 5 years follow-up, must of them with ESRD, this is relatively good out come compared to other studies.

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