Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic nephrotic syndrome (iNS) with resistance or dependence to steroids is a common disease in children but in spite of an increasing clinical impact its pathogenesis is unknown. We screened for the presence of circulating antibodies against glomerular (podocytes, mesangium) and tubular cells (tubular epithelia) a cohort of 60 children with iNS including 8 patients with a familial trait of iNS or with proven mutation of NPHS1-NPHS2 and 12 with good sensitivity to steroids. Positive sera were found in 8 cases, all belonging to the category without familial trait/molecular defects. The targets of antibodies were characterized with Western blot and MALDI-Mass utilizing beta-hexyl cell extracts separated with two-dimensional electrophoresis. In all cases antibodies of the IgM class were directed against ATP synthase beta chain alone (4 cases) or in combination with actin (3 cases); one child presented IgG against aldose reductase. The clinical picture was nephrotic syndrome with steroid resistance or dependence and variable cyclosporin sensitivity; 3 patients developed end stage renal failure. The basic pathology picture was focal segmental glomerulosclerosis (FSGS) in 4 cases and mesangial proliferative glomerulonephrites with deposition of IgM in 2. Overall, patients with circulating auto-antibodies could not be readely differentiated on clinical grounds with the exception of 3 children who developed positivity for antinuclear antibodies during the follow-up. Affinity-purified IgM from one patient who underwent plasmapheresis for therapeutical pourposes (but not from a normal pool) induced proteinuria in Sprague-Dawley rats and concomitant human IgM deposition within glomeruli. This is the first report of circulating anti-actin/ATP synthase beta chain antibodies in a subset of patients with iNS. Both pathological significance and clinical impact given by the presence of these antibodies and the relationship with other conditions such as lupus-erythematosus, characterized by their presence, must be defined.
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PMID:Circulating anti-actin and anti-ATP synthase antibodies identify a sub-set of patients with idiopathic nephrotic syndrome. 1604 39

In this retrospective study, we reviewed the medical records and histopathology findings of 135 patients who underwent renal biopsies at two special hospitals affiliated to Kermanshah medical university during a six-year period (2003-2007). All were performed using Tru-Cut needle under ultrasound guidance. Twenty four specimens were unsatisfactory. There were 38 males (34.2%) and 73 females (65.7%) in 111 patients with adequate specimens (each specimen has more than 5 glomeruli); the mean age was 16.5 years (range 2-64 years). Side effects of the renal biopsies included pain at the site of biopsy in 2 (2.7%), gross hematuria in 1 (0.9%). Nephrotic syndrome was the most common indication for biopsy followed by acute renal failure of unknown etiology and nephritic syndrome. Primary glomerular disease was reported in 78 patients (70.2%) and also secondary glomerular disease in 33 patients (29.7%). Among the primary glomerulonephritis disease, minimal change disease and membranous glomerulonephritis were the commonest findings in children below the age of 16 years. Minimal change disease ranked first in adults whole membranous glomerular disease and focal segmental glomerulosclerosis were more common in the elderly. In all patients lupus glomerular disease was the commonest secondary glomerular disease. We conclude that study on renal biopsy makes final diagnosis which is associated with an acceptably low rate of complications in our practice, and in all, the patterns of renal histology in our study vary slightly from those reported from other countries.
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PMID:Kidney biopsy in west of Iran: Complications and histopathological findings. 2036 27

Glomerular diseases continue to be the leading cause of end-stage renal disease globally. Renal biopsy plays a fundamental role in the evaluation of glomerular diseases not only to establish an accurate diagnosis but also help deciding on appropriate treatment and assessing prognosis. The prevalence of glomerular disease and the clinical indications for kidney biopsies are poorly delineated in Puerto Rico. We undertook a retrospective analysis of the indications, clinical presentation and pathologic reports in renal biopsies performed at the University District Hospital in San Juan, Puerto Rico from the year 1995 to 2008. A total of 208 kidney biopsies showed a predominance of membranous nephropathy representing 20% of the studied population. Women were more frequently biopsied than men (57.2% vs. 42.7%). Lupus nephritis, a condition affecting mostly women was identified in 16.9% of the patients. Minimal change disease was reported in 13.6% of the patients, a condition that affects mostly children and adolescents. In contrast to other geographical areas IgAN was reported only in 6.3% and FSG in 0.9% of patients. In our biopsied patient population, membranous nephropathy is the most common primary glomerular disease and lupus erythematosus the most frequent secondary glomerular disease.
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PMID:Registry of kidney biopsy in a single center in Puerto Rico: university district hospital. 2321 Mar 26

This prospective study was undertaken to evaluate the distribution and pattern of glomerular diseases from a clinico-pathological perspective in our hospital, which is a tertiary care center in Southern India. Seventy-five consecutive patients who underwent renal biopsy and were diagnosed to have glomerular disease over a two-year period were studied and followed-up for one year. Primary glomerular disorders were more common than secondary glomerular disorders. Minimal change disease was the most common primary glomerular disease and lupus was the most common secondary glomerular disorder. Other common primary glomerular disorders were IgA nephropathy (16%) and post-infectious glomerulonephritis (10.7%), while focal and segmental glomerulosclerosis accounted for only 5.3% of the cases. In one-third of the cases, the initial clinical diagnosis did not correlate with the final biopsy diagnosis, further emphasizing the importance of renal biopsy in glomerular disorders.
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PMID:Pattern of glomerular diseases in a tertiary care center in South India: a prospective study. 2335 19

Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Minimal change disease, the most common cause in childhood, generally responds to corticosteroids, although most patients experience disease relapses. Focal segmental glomerulosclerosis is usually resistant to corticosteroids and carries a significant risk of kidney failure, necessitating renal transplantation. Nephrotic syndrome may also be secondary to gene mutations and systemic diseases such as lupus. Clinical evaluation involves distinguishing primary and secondary causes and monitoring for disease complications, including blood clots and serious infections such as spontaneous bacterial peritonitis. Immunosuppressive medications are used to prevent relapses and treat corticosteroid-resistant disease.
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PMID:Nephrotic Syndrome. 3045 52