Patterns of idiopathic nephrotic syndrome in Upper Egyptian children
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Background and aim of work: Idiopathic nephrotic syndrome (INS) is a common and important chronic kidney disease in childhood. Steroid response pattern and the underlying histopathological spectrum of INS show variabilities in the different geographical regions. This work aimed to highlight the clinical profile, steroid response pattern and histopathological spectrum of INS in our locality in Sohag, Upper Egypt. Patients and Methods: A prospective study included 77 children with INS aged from 1-15 years followed for at least one year, diagnosed and treated at our pediatric nephrology unit in the period from January 2010 to December 2014 was done.The patients' demographic features, clinical profile, steroid response pattern and the underlying histopathological spectrum were studied. Results: Total included patients were 77 children. The mean age at disease onset was 4.87±2.94 (range 1.5-15) years. Male/female ratio was 1.6/1. Steroid was given to all patients, 79.2% of patients had steroid sensitive nephrotic syndrome (SSNS), while 20.8% had steroid resistant nephrotic syndrome (SRNS). Out of 61 steroid sensitive patients, about 54% developed either steroid dependent nephrotic syndrome (SDNS) or frequently relapsing nephrotic syndrome (FRNS). Biopsy was done in 33.8% of patients and revealed focal segmental glomerulosclerosis (FSGS) in 42%, minimal change disease (MCD) in 39% and mesangio-proliferative glomerulonephritis (MesPGN) in 19% of patients. End stage renal disease (ESRD) developed in 1 patient with SRNS and FSGS. Conclusion: About eighty percent of our patients were steroid sensitive but more than half of them had SDNS or FRNS. The main histopathological pattern seen in renal biopsies was FSGSKeywords:
Minimal change disease
Nephrology
Minimal change disease
Membranous Nephropathy
Nephrosis
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Background: Glomerular diseases are an important cause of chronic renal failure in developing countries. The spectrum of diseases causing nephrotic syndrome is changing globally in the last few decades. Methods: Patients in the age group 18-60 years with nephrotic syndrome were consecutively included in the study. Renal biopsies were performed in all patients and were subjected to light microscopy, immunofluorescence (IF) and electron microscopy (EM). Results: 189 patients (67% males) were included in the study. The mean age was 43 years. Primary glomerular diseases accounted for 92.5% of cases while lupus nephritis was the most common secondary glomerular disease. Focal segmental glomerulosclerosis (FSGS) accounted for 28.6% of primary glomerular diseases making it the most common cause of nephrotic syndrome. It was followed by membranous glomerulonephritis (MGN) in 13.2%, membranoproliferative glomerulonephritis in 11.2%, diffuse proliferative glomerulonephritis in 10.6% and minimal change disease in 9.5%. Conclusions: The biopsy diagnosis of FSGS has increased considerably in last few decades and it is now the most common cause of nephrotic syndrome in adults in North India. MGN is the most common lesion in patients over 40 years of age.
Minimal change disease
Membranoproliferative glomerulonephritis
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Focal segmental glomerulosclerosis (FSGS) is an aggressive disease often leading to renal failure. It might be difficult to distinguish FSGS from minimal change disease (MCD) when renal biopsies from patients with nephrotic syndrome do not indicate sclerotic lesions. Based on the rat model study, reducing podocyte density (average podocyte number per glomerular volume), which suggests relative podocyte depletion, could be seen in FSGS, but not MCD. Here, we compared the following histological parameters of FSGS and MCD in renal biopsy specimens of nephrotic patients.
Minimal change disease
Glomerulosclerosis
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Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are both common causes of the nephrotic syndrome. Minimal change disease accounts for greater than 90% of cases of nephrotic syndrome in children, vs. 10% to 15% of adults with nephrotic syndrome (1). Focal segmental glomerulosclerosis has been increasing in incidence in the United States in both African Americans and in Hispanics, in both adult and pediatric populations (2–4). It is now the most common cause of nephrotic syndrome in adults in the U.S. Patients with FSGS may have hypertension and hematuria. Serologic studies, including complement levels, are typically within normal limits in both MCD and FSGS.
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Nephrotic syndrome is a common glomerular disease in children with significant variability in both incidence and steroid responsiveness among various ethnic groups. The average incidence of nephrotic syndrome is 2-16.9 per 100,000 children worldwide. Understanding the variability by ethnicity may point to potential factors leading to nephrotic syndrome, which remains elusive, and may highlight factors accounting for differences in medication response. The emerging role of genetic factors associated with steroid responsive and steroid resistant forms of nephrotic syndrome within an ethnic group can provide insight into potential biological mechanisms leading to disease. For example, among African Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a 2-fold greater risk of progression to end stage renal disease. Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes. In the future, the availability of whole genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to better understanding of the disease.
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Introduction: Nephrotic syndrome is the most common chronic renal disease in children. Mostly, it is controlled by steroids. Many underlying pathologies exist in patients with steroid-resistant nephrotic syndrome (SRNS). Among them are ‘focal segmental glomerulosclerosis (FSGS) and ‘minimal change disease’ (MCD). Examining patients’ clinicopathologic characteristics can be helpful by giving an insight into the etiology of steroid resistance and determining patient prognosis. Material and Methods : This cross-sectional study was performed in ‘Children’s Medical Center’ between 2001 and 2011. From 150 patients biopsied, seventy-one children with SRNS, aged 1-14 years, were included. Results: Among 150 patients biopsied, 71 children (47.3%) had steroid-resistant nephrotic syndrome. Forty-four (62%) of these were boys. Upon pathologic investigation of SRNS cases, FSGS came in first, with the highest prevalence at a rate of 32.4%, and MGN came in last, at a rate of 5.6%. The mean age of disease onset was 4.7 years and the mean age of undergoing biopsy was six years. Conclusions: In this study, the predominant pathologic pattern of steroid-resistant nephrotic syndrome was FSGS, a finding similar to that of most studies conducted in this field. MCD was observed in 21.1% of patients, which indicates the variety in reporting renal lesions, particularly, regarding the diagnoses of MCD, mesangio-proliferative glomerulonephritis and early stages of FSGS. Keywords : Steroid-resistant; Nephrotic syndrome; Child.
Minimal change disease
Etiology
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Prednisolone
Minimal change disease
Mesangial proliferative glomerulonephritis
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Minimal change disease
Membranous Nephropathy
Nephrology
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Focal segmental glomerulosclerosis (FSGS) is a glomerular disease defined by a characteristic histologic pattern that occurs either as a primary kidney disease (primary FSGS) or as a result of a systemic illness (secondary FSGS). Proteinuria, often in the nephrotic range, is the hallmark of FSGS. The occurrence of nephrotic syndrome after an insect sting is rarely reported in the literature. We present a case of nephrotic syndrome with focal segmental glomerulosclerosis with a glomerular tip lesion developing after an insect bite.A 51-year-old Caucasian female was bitten by an insect on her left leg, which immediately became swollen. Generalized edema developed and she was admitted for further investigations. Urinary 24-h protein excretion was 7 g. Percutaneous renal biopsy was performed and showed focal segmental glomerulosclerosis of the tip variant. Nephrotic syndrome was steroid-resistant, and when we added cyclophosphamide for 8 weeks complete remission was achieved. There was no relapse of the disease during the 2-month follow-up.This report demonstrates the useful role of cyclophosphamide in the treatment of steroid-resistant nephrotic syndrome due to FSGS with glomerular tip lesion. A causal relationship between the insect bite and the nephrotic syndrome is suggested and an immune response could be responsible for the nephrotic syndrome.
Minimal change disease
Glomerulosclerosis
Nephrosis
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