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      Trends in the histopathology of childhood nephrotic syndrome in Ibadan Nigeria: preponderance of idiopathic focal segmental glomerulosclerosis

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          Abstract

          Background

          Reports on the histopathology of childhood nephrotic syndrome (NS) had emanated from our Centre since the 1960s and by the late 1980s and early 1990s, a change was observed and reported. Taking into consideration the worldwide changing trend in the histopathology of the NS and our Unit policy change in the indications for renal biopsy, a change was envisaged. We therefore evaluated the current histologic pattern of childhood NS in Ibadan with the view to highlighting any variations from the past and comparing the findings with regional and global trends.

          Methodology

          We reviewed our database and analyzed the renal biopsy findings in patients who were biopsied before treatment was administered between 1997 and 2001 and those with mostly idiopathic steroid resistant NS (SRNS) and secondary NS, managed between 2006 and 2013. A comparative analysis of the findings from the present study was carried out with two previous reports from our Unit in the 1970s and early 1990s and also with reports from other Centres.

          Results

          A total of 78 patients had successful biopsies done during the study period in children aged between 2 ½ and 16 years. In both pre-treatment biopsy era (1997–2001) and post-treatment biopsy era (2006–2013), focal segmental glomerulosclerosis (FSGS) predominated. 75 % of the patients had idiopathic NS and among the patients that had idiopathic steroid resistant NS, FSGS was the most common followed by MPGN. For secondary NS, MCD was the most common but could be the early stages of either membranous nephropathy (MN) or FSGS. Chronic pyelonephritis and chronic interstitial nephritis occurred in 25 % of the study population but they were more prevalent in secondary nephrotic syndrome.

          Conclusion

          FSGS is the most common histopathology in children requiring renal biopsy in Ibadan presently. FSGS is also the most common histopathology in idiopathic SRNS, which is in keeping with reports from most parts of the world.

          There has been a transition from the preponderance of Quartan Malarial Nephropathy (QMN) in the 1960s to MPGN in the 1980s to FSGS presently. This has great implications with regards to searching for new aetiologic factors, providing more efficacious treatment modalities and ensuring facilities for immunofluorescence, electron microscopic and genetic studies.

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          Most cited references38

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          Obesity-related glomerulopathy: an emerging epidemic.

          We report the first large renal biopsy-based clinicopathologic study on obesity-related glomerulopathy. Obesity was defined as body mass index (BMI)> 30 kg/m2. Obesity-related glomerulopathy (ORG) was defined morphologically as focal segmental glomerulosclerosis and glomerulomegaly (O-FSGS; N = 57) or glomerulomegaly alone (O-GM; N = 14). Review of 6818 native renal biopsies received from 1986 to 2000 revealed a progressive increase in biopsy incidence of ORG from 0.2% in 1986-1990 to 2.0% in 1996-2000 (P = 0.0001). Mean BMI in ORG was 41.7 (range 30.9 to 62.7). Indications for renal biopsy included proteinuria (N = 40) or proteinuria and renal insufficiency (N = 31). Seventy-one patients with ORG were compared to 50 patients with idiopathic FSGS (I-FSGS). Patients with ORG were older (mean 42.9 vs. 32.6 years, P < 0.001) and more often Caucasian (75% vs. 52%; P = 0.003). ORG patients had a lower incidence of nephrotic range proteinuria (48% vs. 66%; P = 0.007) and nephrotic syndrome (5.6% vs. 54%; P < 0.001), with higher serum albumin (3.9 vs. 2.9 g/dL; P < 0.001), lower serum cholesterol (229 vs. 335 mg/dL; P < 0.001), and less edema (35% vs. 68%; P = 0.003). On renal biopsy, patients with ORG had fewer lesions of segmental sclerosis (10 vs. 39%; P < 0.001), more glomerulomegaly (100% vs. 10%; P < 0.001), and less extensive foot process effacement (40 vs. 75%; P < 0.001). Glomerular diameter in ORG (mean 226 mu) was significantly larger than age- and sex-matched normal controls (mean 168 mu; P < 0.001). Follow-up was available in 56 ORG patients (mean 27 months) and 50 idiopathic FSGS controls (mean 38 months). A total of 75% of ORG patients received angiotensin-converting enzyme (ACE) inhibition or A2 blockade while 78% of the I-FSGS patients received immunosuppressive therapy. ORG patients had less frequent doubling of serum creatinine (14.3% vs. 50%; P < 0.001) and progression to ESRD (3.6% vs. 42%; P < 0.001). On multivariate analysis, presenting serum creatinine and severity of proteinuria were the only predictors of poor outcome in ORG. ORG is distinct from idiopathic FSGS, with a lower incidence of nephrotic syndrome, more indolent course, consistent presence of glomerulomegaly, and milder foot process fusion. The ten-fold increase in incidence over 15 years suggests a newly emerging epidemic. Heightened physician awareness of this entity is needed to ensure accurate diagnosis and appropriate therapy.
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            Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK.

            Against the background of the increasing incidence of many immune mediated childhood conditions, this study aimed to identify recent time trends and ethnic patterns of childhood nephrotic syndrome. A population-based cohort of children (0-15 years) diagnosed according to strict criteria with nephrotic syndrome (NS) was ascertained within the northern UK region of Yorkshire between 1987 and 1998. South Asian ethnicity was assigned based on the child's full name using a dedicated computer algorithm and expert individual checks. NS was diagnosed in 194 children, 170 (88%) of whom were steroid sensitive. The incidence of steroid sensitive NS was 2.0/100,000 pyrs (95% CI 1.7-2.3), peaking in 1-4 year olds (4.1/100,000 pyrs). Over the 12-year study period incidence rates of steroid sensitive NS were fairly stable although south Asian children displayed significantly higher rates than non-south Asians (P<0.01). The size of our population-based series reflects the relative rarity of paediatric nephrotic syndrome but is nonetheless recent and includes larger numbers than previous reports. The absence of any increase in incidence over the last decade contrasts with other paediatric immune mediated conditions such as asthma and diabetes.
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              The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children.

              The accuracy of using the initial response to prednisone to identify children with minimal glomerular changes has been assessed in a prospective study of children between 12 weeks and 16 years of age with the primary nephrotic syndrome. The results indicate that, as generally held, the likelihood is quite high, although not 100%, that a child with the primary nephrotic syndrome who responds during eight weeks of initial intensive steroid treatment has MCNS. Prognosis in these patients can be considered to be very favorable and renal biopsy need not be done unless indicated by the subsequent clinical course. However, the prediction that a patient who fails to respond has a glomerular lesion other than MCNS would be incorrect in about one-fourth of all patients with the primary nephrotic syndrome, and in as many as one-half of patients less than or equal to 6 years of age. For these patients, predictions concerning prognosis should be withheld until a renal biopsy provides a histopathologic diagnosis.
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                Author and article information

                Contributors
                adasinobi@yahoo.com
                dr_deboademola@yahoo.co.uk
                clemabuokolo@yahoo.ca
                jyaria@yahoo.com
                Journal
                BMC Nephrol
                BMC Nephrol
                BMC Nephrology
                BioMed Central (London )
                1471-2369
                15 December 2015
                15 December 2015
                2015
                : 16
                : 213
                Affiliations
                [ ]Department of Paediatrics, College of Medicine, University of Ibadan, Ibadan, Oyo State Nigeria
                [ ]Department of Paediatrics, University College Hospital Ibadan, Ibadan, Oyo State Nigeria
                [ ]Department of Pathology, College of Medicine, University of Ibadan, Ibadan, Oyo State Nigeria
                [ ]Department of Pathology, University College Hospital Ibadan, Ibadan, Oyo State Nigeria
                [ ]Department of Medicine, University College Hospital Ibadan, Ibadan, Oyo State Nigeria
                Article
                208
                10.1186/s12882-015-0208-0
                4681037
                26670137
                6085ca75-713d-4ab7-8160-96a82df46631
                © Asinobi et al. 2015

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 8 June 2015
                : 7 December 2015
                Categories
                Research Article
                Custom metadata
                © The Author(s) 2015

                Nephrology
                renal histopathology,childhood nephrotic syndrome,fsgs,mpgn,mcd,ibadan nigeria
                Nephrology
                renal histopathology, childhood nephrotic syndrome, fsgs, mpgn, mcd, ibadan nigeria

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