Nephrotic syndrome in 1st year of life and the role of unilateral nephrectomy

Mattoo, Tej K.; Al-Sowailem, Abdul Mohsin; Al-Harbi, Mansoor S.; Mahmood, Mustapha A.; Katawee, Yousef; Hassab, M. Hany

doi:10.1007/bf00856821

Cited by 33 publications

(22 citation statements)

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“…Diffuse mesangial sclerosis, characterized by mesangial expansion as a result of over-deposition of extracellular matrix (ECM) in more than 80% of the total glomeruli, is commonly observed in patients with hereditary NS [2,3]. The infants manifest massive proteinuria and then develop renal failure complicated by infections within a few years after birth [2].…”

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confidence: 99%

Nephrotic Mice (ICGN Strain): A Model of Diffuse Mesangial Sclerosis in Infantile Nephrotic Syndrome

et al. 1999

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The ICGN mouse strain is a unique model for naturally occurring nephrotic syndrome. In the present study, we examined the onset of the clinical manifestation of nephrotic syndrome and determined the sequence of intraglomerular events associated with progression of nephrotic conditions. Laboratory analysis revealed that homozygous (nep/nep) mice showed urinary albumin excretion during the suckling stage, rapidly leading to hypoalbuminemia accompanied by body growth failure. Renal pathology demonstrated that an initial intraglomerular event in the nephrotic mice was observed 3 weeks after birth in the form of mesangiolytic lesions, characterized by microaneurysm, platelet accumulation and capillary ballooning. In 6-week-old homozygous mice, mesangial sclerosis, characterized by mesangial expansion and glomerular hypertrophy, was observed in a diffuse fashion. Immunohistochemistry revealed that the glomerular cells in the 3-week-old homozygous suckling mice were positive for α-smooth muscle actin, suggesting a phenotypic change in the mesangial cells. Mesangial expansion, confirmed by the over-deposition of type I collagen, was evident until 6 weeks after weaning, while it was of interest that fibrogenic cytokines such as platelet-derived growth factor and transforming growth factor-β were not detected in the sclerotic glomeruli throughout the observations. Furthermore, the nephrotic features were shown to be resistant to steroid therapy with a high dose of prednisolone. Our results suggest that diffuse mesangial sclerosis, a hereditary glomerular disease, may be genetically generated through early myofibroblast formation, which occurs and develops probably independently of up-regulation of these fibrogenic cytokines. In conclusion, the homozygous nephrotic mouse (ICGN strain) is believed to be a good model for investigating not only nephrotic conditions but also cellular and molecular pathogenesis of diffuse mesangial sclerosis in steroid-resistant infantile nephrotic syndrome.

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Nephrotic Mice (ICGN Strain): A Model of Diffuse Mesangial Sclerosis in Infantile Nephrotic Syndrome

et al. 1999

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“…This may be due to genetic predisposition related to a high incidence of consanguineous marriages. Further details on their renal histology has been reported separately [12]. …”

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Spectrum of Renal Pathology in 100 Selected Children with Nephrotic Syndrome

Mattoo

Al-Sowailem

1993

Ann Saudi Med

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The study includes 100 renal biopsies from an equal number of patients referred to us with nephrotic syndrome (NS). Their ages ranged from one month to 13 years (medial five years) and included 59 males and 41 females. Indications for kidney biopsy were steroid resistance in 41 patients, steroid dependence with frequent relapses on maintenance steroid therapy in 38, NS associated with gross hematuria, hypertension, azotemia and/or systemic manifestations in eight and age of less than one year at the onset of the disease in 13 patients. In 90 patients, NS was idiopathic in origin and in eight, it was secondary to IgA nephropathy, hemolytic-uremic syndrome, post streptococcal glomerulonephritis, antiglomerular basement membrane antibodies, Henoch-Schonlein nephritis and systemic lupus erythematosus. Two patients had hepatitis B infection and its relationship with NS could not be elucidated. Primary NS is the predominant type of NS in the local pediatric population. The disease is more common in the first year of life. A relatively high incidence of diffuse mesangioproliferative glomerulonephritis (MesPGN) and rarity of infection-related NS are noted. Ann Saudi Med 1993;13 (5) The term nephrotic syndrome (NS) refers to the presence of heavy proteinuria, hypoproteinemia, edema and hyperlipidemia. Occasionally it is associated with hypertension, hematuria and impaired renal function. In about 90% of the cases, it is idiopathic in origin, which is also known as primary NS. The remaining few cases occur secondary to certain infections, autoimmune disease, systemic illness, malignancy, drug or heavy metal ingestion, etc.[1].The pattern of primary NS and the spectrum of renal disease in local children are well documented [2][3][4]. The purpose of this study was to determine the spectrum of renal pathology in children who have a renal biopsy for NS and to compare the results with similar studies from other countries. Patients and MethodsOne hundred patients who had a kidney biopsy were selected from 210 patients referred to us with nephrotic syndrome over a period of four years, from 1986 to 1990. The indications for kidney biopsy were steroid resistance, steroid dependent frequent relapsing course, NS associated with gross hematuria, hypertension and azotemia, and age of less than one year at the onset of the disease. Kidney biopsies were done percutaneously, or by an open surgical approach in those with single kidneys and in severely edematous babies with congenital NS. The tissue obtained was processed for light microscopy, immunofluorescence and electron microscopy. Definitions used in the study were as follows: Nephrotic syndrome (NS): presence of proteinuria of more than 40 mg/hr per m 2 , serum albumin level of less than 25 g/L and edema. Response: urine remaining free of proteins on semiquantitative testing (Meditest Combi 6) for at least three consecutive days. Relapse: Three consecutive days of 3+ proteinuria on semiquantitative testing (Meditest Combi 6). Frequent relapses: Two or more relapses in six months fo...

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“…Nefrektomi bilateral dapat mengurangi kebutuhan akan terapi nutrisi dan subsitusi albumin. 4,5 Coulthard melaporkan pasien SNK yang tumbuh dan berkembang secara normal setelah nefrektomi unilateral.…”

Section: Tata Laksanaunclassified

“…1,3,4,5,12,21 Insidens SMD sangat jarang ditemukan dan dapat terjadi setiap saat pada tahun pertama, 1,3,4,12,21 SMD sering terdapat dalam satu keluarga sehingga diduga penyakit ini diturunkan secara autosomal resesif. Ras atau etnik bukan merupakan faktor predisposisi.…”

Section: Sklerosis Mesangial Difusunclassified

“…Adanya SNK akan menyebabkan kerentanan terhadap infeksi dan dapat menyebabkan malnutrisi, hipovolemia, dan tromboemboli. [1][2][3][4][5] Prognosis SNK sangat buruk dan kematian biasanya terjadi dalam 6 bulan pertama, namun dengan tata laksana yang adekuat termasuk asupan nutrisi, b. Toksik: merkuri yang menyebabkan immunecomplex-mediated epimembranous nephritis c. Lupus eritematosus sistemik (LES) d. Sindrom hemolitik uremik e. Reaksi obat f. Nefroblastoma atau tumor Wilms g. Korioangiomatosis plasenta.…”

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Sindrom Nefrotik Kongenital

Pardede

2016

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Nephrotic syndrome in 1st year of life and the role of unilateral nephrectomy

Cited by 33 publications

References 10 publications

Nephrotic Mice (ICGN Strain): A Model of Diffuse Mesangial Sclerosis in Infantile Nephrotic Syndrome

Nephrotic Mice (ICGN Strain): A Model of Diffuse Mesangial Sclerosis in Infantile Nephrotic Syndrome

Spectrum of Renal Pathology in 100 Selected Children with Nephrotic Syndrome

Sindrom Nefrotik Kongenital

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