Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 22 (13 females, 9 males) of 224 patients presenting with nephrotic syndrome, onset occurred during the second decade of life. Lipoid nephrosis and its variants (nil, focal segmental, or focal global glomerulosclerosis and mesangial proliferative) accounted for the disease in the majority of patients (13 patients). In all with nil change (7 patients) and one of the 4 with mesangial proliferation the disease completely resolved with steroids. Forty-one percent of patients had nephrotic syndrome as the result of other forms of glomerulonephritis: 1 patient had nephrotic syndrome secondary to poststreptococcal glomerulonephritis which resolved spontaneously. Three patients had membranoproliferative glomerulonephritis and developed renal insufficiency. Proteinuria decreased or disappeared in 4 patients with systemic lupus erythematosus. One patient had membranous nephropathy that was steroid resistant. Presenting symptoms and laboratory data failed to separate nil change from the more severe glomerular lesions. Early biopsy for diagnosis is suggested for patients presenting with de novo nephrotic syndrome in the second decade of life.
 ...
PMID:Nephrotic syndrome in the second decade of life. 730 97

Minimal change nephrotic syndrome (MCNS) developed in a 17-year-old female and spontaneously remitted. One month later the nephrotic syndrome relapsed. Prednisolone therapy, 60 mg/day, was started and resulted in a full remission within a week and the prednisolone dose was subsequently tapered. Seven months later, when 10 mg/day of prednisolone was being administered, she developed erythematous rash with photosensitivity and polyarthralgia without exacerbation of the nephrotic syndrome, and fulfilled four of the American College of Rheumatology criteria for classification of systemic lupus erythematosus (SLE). Avoidance of direct sunlight ameliorated the erythematous rash and the polyarthralgia disappeared even though the prednisolone dose was decreased further. This is the first reported case of SLE developed in a patient with remitting MCNS.
 ...
PMID:Systemic lupus erythematosus in a patient with remitting minimal change nephrotic syndrome. 940 19

We report the clinical and pathologic findings in 7 patients with systemic lupus erythematosus and minimal change disease. All 7 patients presented with full nephrotic syndrome including peripheral edema, nephrotic range proteinuria (mean 9.6 g/day), and hypoalbuminemia (mean 1.8 g/dl). In all cases, renal biopsy revealed diffuse foot process effacement in the absence of significant peripheral capillary wall immune deposits, findings consistent with minimal-change disease. In addition, 5 cases displayed mesangial electron-dense deposits, with or without associated mesangial proliferation, consistent with underlying lupus nephritis class II. In all cases, steroid therapy induced a rapid remission of nephrotic syndrome. Minimal change disease is an underrecognized and readily reversible form of nephrotic syndrome in systemic lupus erythematosus. Because it may occur superimposed on mild mesangial proliferative lupus nephritis, this entity may be misinterpreted as an atypical presentation of lupus nephritis class II. Proper recognition of this entity requires careful integration of the renal biopsy immunofluorescence and electron microscopic findings.
 ...
PMID:Minimal change disease in systemic lupus erythematosus. 1186 21

There are few studies that examine, prospectively, the epidemiological profile of glomerulopathy (GP) and its clinicopathological correlation. All patients referred to Al-Amiri renal center in Kuwait from January 1st, 1995 to December 31st, 2001 were screened for GP. Detailed clinical data were collected and serological markers were done. Renal biopsy was performed whenever indicated. During those 7 years, a total of 584 patients were diagnosed, on histological basis, to have GP, 315 of whom were Kuwaiti nationals. During the same period of the study, 26 patients presented with bilateral small kidneys, history of proteinuria > 2 g/day and lacked systemic manifestations of autoimmune disease. Furthermore, 164 patients with clinical manifestations of diabetic glomerulosclerosis were not subjected to kidney biopsy. Hence, the calculated annual incidence rate of GP in Kuwaiti nationals was 34.5 per 100,000 population (PTP). The calculated rate of diabetic glomerulosclerosis was 13.4 PTP and that of nondiabetic 21.1 PTP. The calculated incidence rates of GP increased with age and were twice as high in males compared to females. Vasculitis was more common in elderly males while SLE nephritis was a disease of adults, 88.7% of whom were females. In the subgroup of primary GP, focal segmental glomerulosclerosis was the most common histological lesion accounting for 18.0% of the total biopsies in Kuwaiti patients, yet only 36.8% of those who fulfilled the criteria of primary type. Minimal change disease was the second primary GP (13.0%), followed by immunoglobulin A deposition disease (7.9%) and membranous glomerulonephritis (5%). Autoimmune diseases such as systemic lupus erythematosus (SLE) and vasculitis were common. Interestingly, only 44 of 72 (61.1%) of patients with SLE and 11 of the 62 (17.7%) of patients with vasculitis presented with rapidly progressive glomerulonephritis. On the other hand, 10 of 58 (17.2%) patients with nephroangiosclerosis presented with renal failure and protein excretion > 2 g/day simulating primary GP. Furthermore, only 21 of 40 (52.5%) patients with IgA nephropathy presented with "benign disease". Prospective studies are essential to ascertain the actual incidence and etiology of GP. The loose clinicopathological correlation in GP dictates an aggressive diagnostic approach in its study and management.
 ...
PMID:Glomerulopathy in Kuwait: the spectrum over the past 7 years. 1291 Nov 67

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.
 ...
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.
 ...
PMID:Kidney biopsy in west of Iran: Complications and histopathological findings. 2036 27

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.
 ...
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.
 ...
PMID:Nephrotic Syndrome. 3045 52