[RARE ASPECTS OF THE INFANTILE NEPHROTIC SYNDROME. NEPHROTIC SYNDROME DUE TO RHEUMATIC PURPURA].
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Child↗
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Results for “Nephrotic Syndrome”
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[FAMILIAL NEPHROTIC SYNDROMES. NEPHROTIC SYNDROME ASSOCIATED WITH CONGENITAL CARDIOPATHY IN 4 SISTERS].
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Child↗
[New paraneoplastic syndrome: nephrotic syndrome].
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Antigen-Antibody Reactions↗
Pathobiochemistry of nephrotic syndrome.
Nephrotic syndrome is a clinical and laboratory syndrome caused by the increased permeability of the glomerular capillary wall for macromolecules. Nephrotic syndrome is a potentially life-threatening state and persistent nephrotic syndrome has a poor prognosis with a high risk of progression to end-stage renal failure and a high risk of cardiovascular complications due to severe hyperlipidemia. Pathogenesis of increased glomerular permeability in different glomerular diseases has not been fully elucidated. Recently, identification of the mutated genes for some podocyte proteins (nephrin, podocin, alpha-actinin-4) in rare familial forms of nephrotic syndrome shed has new light on the molecular mechanisms of glomerular permselectivity. Gradually it becomes apparent that sporadic mutations of podocyte proteins (e.g., podocin) may be present even in some patients with acquired nephrotic syndrome. Expression of other podocyte proteins may change during the course of experimental nephrotic syndrome, possibly as a response to podocyte damage resulting either in apoptosis or stimulation of proliferation and some form of repair, including glomerular sclerosis. Better understanding of these mechanisms could clearly also have therapeutic implications. Glomerular permeability factors are believed to play a role in some noninflammatory glomerular diseases, mainly minimal change disease and focal segmental glomerulosclerosis, but their molecular identification remains elusive, possibly due to the nonhomogeneous nature of the underlying diseases. As an example, focal segmental glomerulosclerosis possibly can be caused by the sporadic mutation of some genes for podocyte proteins, increased production of glomerular permeability factor (possibly by T lymphocytes), or the loss of inhibitors of glomerular permeability factors in nephrotic urine. Clearly the factors causing increased glomerular permeability and factors perpetuating glomerular sclerosis are not necessarily the same. Proteinuria does not seem to be only the consequence of glomerular damage, but it may possibly cause tubular damage and initiate interstitial fibrosis and thus contribute to the progression of chronic renal failure in proteinuric renal diseases. Recent insights into the mechanisms of tubular protein reabsorption may give new tools for preventing the progression of chronic renal disease. Cubilin inhibitors could potentially ameliorate tubular and interstitial damage in patients with heavy proteinuria refractory to treatment. Nephrotic hyperlipidemia is accompanied with increased risk of cardiovascular complications and should be treated in all patients with persistent nephrotic syndrome. The putative positive effect of hypolipidemic drugs (namely statins) on the cardiovascular risk and potentially also on the rate of progression of chronic renal failure remains to be demonstrated in prospective controlled studies. Recent progress in understanding podocyte biology in rare inherited glomerular diseases gives the chance to understand in the near future the molecular pathogenesis of increased glomerular permeability in the much more common acquired forms of nephrotic syndrome.
Animals↗
Arginine vasopressin gene expression in rats with puromycin-induced nephrotic syndrome.
Nephrotic syndrome is characterized by water and sodium retention, which leads to edema formation. The nonosmotic stimulation of arginine vasopressin (AVP) release from the pituitary gland has been implicated to be one of the important factors of abnormal water retention in patients with nephrotic syndrome. It is not known, however, whether nephrotic syndrome is associated with stimulation of hypothalamic vasopressin gene expression. Puromycin aminonucleoside is known to cause altered glomerular permeability, which results in experimental nephrotic syndrome in rats. In the present study, therefore, AVP gene expression has been studied in the hypothalamus of rats with puromycin aminonucleoside-induced nephrotic syndrome (PNS). Nephrotic syndrome was induced by a single intravenous injection of puromycin aminonucleoside (50 mg/kg body weight). Nephrotic syndrome was confirmed by urinary protein excretion (control 20.8 +/- 3.5 mg/24 hr v PNS 273.9 +/- 41.4 mg/24 hr; P < 0.0001, n = 6) and serum albumin concentrations (control 4.52 +/- 0.07 g/dL v PNS 2.96 +/- 0.22 g/dL; P < 0.001, n = 6). In PNS rats, plasma AVP was significantly higher than in control rats (control 0.77 +/- 0.10 pg/mL v PNS 2.13 +/- 0.42 pg/mL; P < 0.005, n = 12), even though there were no differences in plasma osmolality (control 292.0 +/- 2.0 mOsm/kg H2O v PNS 290.3 +/- 2.5 mOsm/kg H2O; P = NS, n = 12) or serum sodium concentration (control 142.7 +/- 0.7 v PNS 142.1 +/- 1.1; PNS, n = 12).(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Prothrombotic factors in nephrotic syndrome.
Nephrotic syndrome is a hypercoagulable state with variable prevalence of clinical thrombosis. The role of platelet aggregation, fibrinogen and antithrombin III and protein S levels in the pathogenesis of hypercoagulable state in these patients is controversial. Since no study on Indians is available, the clinical and laboratory profile of 22 patients of nephrotic syndrome (age 18-35 years with an MF ratio of 4:3), have been studied. The coagulation profile revealed a prolonged APTT in 12 patients (54.5%), and a prolonged TT in four (18.1%). In the rest APTT and TT were normal. PT was raised in two patients. Fibrinogen, an acute phase reactant was raised in five patients (22.7%). Antithrombin III levels were reduced in 19 patients (86.4%), normal in one and raised in two patients. Free Protein S levels were high in 12(54.5%), normal in seven and decreased in three patients. Platelet aggregation with adrenaline and adenosine diphosphate was raised in 6 patients. Ultrasonographically detected deep vein thrombosis was seen in one patient only (4.5%) who had ATIII levels of 48%. This low incidence can be explained by elevated protein S levels which was found to be raised in 12(54.5%) cases, protein S being an anticoagulant factor. This low level of clinical thrombosis in Indian patients of nephrotic syndrome may be an ethnic variable factor. It is thus concluded that although patients with nephrotic syndrome have a hypercoagulable state, clinical thrombosis is rarely seen in Indian patients with nephrotic syndrome.
Adolescent↗
Erythropoietin and transferrin metabolism in nephrotic syndrome.
Nephrotic syndrome is characterized by marked urinary excretion of albumin and other intermediate-size plasma proteins. This results in a profound alteration of the metabolism of many plasma proteins and protein-bound substances, as well as certain cellular and tissue proteins. This review summarizes available data on the effect of nephrotic syndrome on the metabolism and regulation of erythropoietin (EPO) and transferrin, which are essential for erythropoiesis. Studies of humans and animals have documented significant urinary losses of both EPO and transferrin in nephrotic syndrome. Urinary losses of EPO have been shown to cause EPO-deficiency anemia and prevent the normal increase in plasma EPO level in response to anemia and hypoxia in nephrotic syndrome. Similarly, transferrinuria and increased transferrin catabolism have been shown to cause hypotransferrinemia and, in some cases, iron-deficiency anemia. In addition, dissociation of iron from filtered transferrin, occasioned by a reduction in tubular fluid pH, can promote tubulointerstitial injury through the iron-catalyzed generation of oxygen free radicals. This can account in part for the role of proteinuria as a risk factor for the progression of renal disease. Subcutaneous administration of recombinant EPO has been successfully used in the management of EPO-deficiency anemia in nephrotic syndrome. Similarly, iron supplementation and nutritional support are indicated in nephrotic patients with severe transferrinuria and iron-deficiency anemia. However, correction or amelioration of the underlying proteinuria, when possible, is the ideal approach to reversal of these complications.
Animals↗
[Comparative study of coagulation in the primary nephrotic syndrome and nephrotic syndrome secondary to renal amyloidosis].
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Adult↗
[Treatment of nephrotic syndrome].
Nephrotic syndrome is characterized by increased permeability of glomerular filter which leads to proteinuria and consequently to the numerous metabolic, endocrine and immune disorders. Treatment of nephrotic syndrome should be directed to proteinuria and its causes as well as to its complications. As increased permeability of glomerular filter in primary nephrotic syndromes develops due to influence of immune reactions mediators, immunomodulating agents are used in their therapy. Therapeutic index of these agents is low and therefore the agents with antiproteinuric effects and symptomatic therapy are also used in the management of nephrotic syndromes.
Humans↗
Impaired aquaporin and urea transporter expression in rats with adriamycin-induced nephrotic syndrome.
Nephrotic syndrome is associated with abnormal regulation of renal water excretion. To investigate the role of collecting duct water channels and solute transporters in this process, we have carried out semiquantitative immunoblotting of kidney tissues from rats with adriamycin-induced nephrotic syndrome. These experiments demonstrated that adriamycin-induced nephrotic syndrome is associated with marked decreases in expression of aquaporin-2, aquaporin-3, aquaporin-4, and the vasopressin-regulated urea transporter in renal inner medulla, indicative of a suppression of the capacity for water and urea absorption by the inner medullary collecting duct. In contrast, expression of the alpha(1)-subunit of the Na,K-ATPase in the inner medulla was unaltered. Light and electron microscopy of perfusion-fixed kidneys demonstrated that the collecting ducts are morphologically normal and unobstructed. Inner medullary expression of the descending limb water channel, aquaporin-1, was not significantly altered, pointing to a selective effect on the collecting duct. Aquaporin-2 and aquaporin-3 expression was also markedly diminished in the renal cortex, indicating that the effect is not limited to the inner medullary collecting duct. Differential centrifugation studies and immunocytochemistry in inner medullary thin sections demonstrated increased targeting of aquaporin-2 to the plasma membrane, consistent with the expected short-term action of vasopressin on aquaporin-2 trafficking. The extensive down-regulation of aquaporin and urea transporter expression may represent an appropriate renal response to the extracellular volume expansion observed in nephrotic syndrome, but may occur at the expense of decreased urinary concentrating and diluting capacity.
Adenylyl Cyclases↗
Plasma levels and urinary excretion of fibrinolytic and protease inhibitory proteins in nephrotic syndrome.
Nephrotic syndrome is associated with numerous blood coagulation abnormalities and a marked propensity to thromboembolism. The present study was undertaken to examine the status of the fibrinolytic system in this hypercoagulable state. We measured the antigen concentrations or activities of plasminogen, tissue-type plasminogen activator (t-PA), plasminogen activator inhibitor (PAI), alpha 2-antiplasmin, alpha 1-antitrypsin, and alpha 2-macroglobulin as well as total antiplasmin activity and D-dimer concentration in the plasma of 39 patients with nephrotic syndrome and 32 normal controls subjects. In addition, antigen concentrations of plasminogen, alpha 2-antiplasmin, alpha 1-antitrypsin, and alpha 2-macroglobulin were measured in the urine of the study populations. The nephrotic group showed marked elevations of plasma t-PA, plasminogen, alpha 2-macroglobulin, and D-dimer and a significant reduction of plasma alpha 2-antiplasmin and alpha 1-antitrypsin as compared with the normal control group. Plasma alpha 2-macroglobulin was directly related to 24-hour urinary protein excretion and inversely related to serum albumin concentration. None of the proteins measured were detectable in the urine of normal controls. However, substantial amounts of plasminogen, alpha 2-antiplasmin, and alpha 1-antitrypsin and small amounts of alpha 2-macroglobulin were recovered in the urine of patients with nephrotic syndrome. Despite the lack of clinically demonstrable thrombosis, plasma D-dimer was markedly elevated in the nephrotic group, suggesting concurrent activation of coagulation and fibrinolytic pathways. In addition, the study revealed multiple abnormalities of the plasma fibrinolytic proteins and documented their urinary excretion in patients with nephrotic syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Regulation of podocyte structure during the development of nephrotic syndrome.
Nephrotic syndrome is a common kidney disease seen in both children and adults. The clinical syndrome includes massive proteinuria, hypoalbuminemia, edema, and usually hypercholesterolemia. Development of these clinical changes is closely correlated with profound structural changes in glomerular epithelial cells, or podocytes, which together with the glomerular basement membrane and endothelium comprise the kidney's blood filtration barrier. Although relatively little is known about the cellular or molecular changes which occur within podocytes during the development of nephrotic syndrome, cytoskeletal proteins very likely play a central role in these changes since they are primarily responsible for the maintenance of cell structure in almost all cells. This review focuses on: (a) the structure and function of podocytes in both the normal state and during nephrotic syndrome and (b) the potential roles of several cytoskeleton-associated proteins identified in podocytes in the development of and/or recovery from the pathophysiological cytoskeletal changes which occur in podocytes during nephrotic syndrome.
Actinin↗
Mesenteric thrombosis causing short bowel syndrome in nephrotic syndrome.
Nephrotic patients are at risk of developing venous and arterial thrombotic complications. Pulmonary embolism due to affected deep leg veins is by far the most common event. Renal or cerebral vein thromboses have been described. Thrombosis of arterial vessels is less frequent. Mesenteric infarction is a rare but severe complication in patients with nephrotic syndrome (NS). We report a 7-year-old boy with a steroid-dependent (SD) NS and a homozygous mutation of methylenetetrahydrofolate reductase, increasing the risk of thromboembolic events. He developed a thrombosis of his superior mesenteric artery during his ninth relapse, which was responsible for a necrosis of 240 cm of his small bowel, necessitating resection of necrotic parts and double external ostomy diversion. Remission was achieved with pulse prednisolone therapy. Corticoids were reduced over 4 months progressively. Oral cyclosporin A (CyA) was initiated for long-term treatment. Due to a short bowel syndrome with severe malabsorption, even oral administration of 22.5 mg/kg per day CyA did not lead to sufficient plasma levels. Intravenous cyclophosphamide pulse therapy over 6 months led to a complete remission. No relapse occurred over a period of more than 5 months after the last cyclophosphamide pulse. Anticoagulation and screening for increased susceptibility for thrombotic events are necessary in every nephrotic patient. Intravenous cyclophosphamide pulse therapy is a useful alternative in SDNS with impaired intestinal absorption of applied immunosuppressive drugs.
Child, Preschool↗
[Thrombosis of the abdominal aorta in a patient with nephrotic syndrome].
Nephrotic syndrome frequently causes thromboembolic complications in veins. Arterial thrombosis, however, is relatively rare. We report the case of a 47-year-old male with nephrotic syndrome complicated with abdominal aortic thrombosis. The patient complained of pain in the bilateral lower extremities 2 weeks after the development of nephrotic syndrome. The aortogram revealed complete occlusion of the abdominal aorta just below the origin of the inferior mesenteric artery. Necrosis of the legs extended rapidly, and he eventually lost his legs. Among various predisposing factors, hypercoagulability associated with nephrotic syndrome seemed to be responsible for the development of thrombosis. Thrombotic complications are sometimes serious in the nephrotic patient. Assessment for the risk factors is required to warrant prophylactic anticoagulation.
Amputation, Surgical↗
[Pathogenetic aspectics of nephrotic syndrome].
Nephrotic syndrome is characterized by protein loss in the urine, hypoalbuminemia, hyperlipidemia and edema. Several diseases cause a nephrotic syndrome, as they damage the glomerular podocytes. These specialized epithelial cells, together with endothelial cells of the glomerular capillaries and the basal membrane, form a filter that retains plasma proteins in the circulation. A disturbance of this filter causes proteinuria. The three most common primary glomerular diseases are minimal change, membranous glomerulonephritis, and the primary focal segmental glomerulosclerosis. The familiar forms are rare; however, the identification of the relevant gene defects has greatly advanced our understanding of podocyte function as well as the pathogenesis of nephrotic syndrome.
Gene Expression Regulation↗
[Perioperative management of pediatric patient with vena cava superior thrombosis complicated with nephrotic syndrome].
Nephrotic syndrome is a disease which accompanies hypoproteinemia, edema, hyperlipidemia and coagulopathy. This syndrome has also been recognized in pediatric patient. We experienced recently a case of 11-year-old girl, who had insertion of a plasma exchange catheter because of secondary hyperlipidemia due to nephrotic syndrome. She suffered soon from a severe SVC obstruction by thrombosis grown up around the catheter and an emergency thrombectomy was planned under the cardiopulmonary bypass. Renal function was maintained preoperatively, in comparison with nephrotic syndrome in adult where some problems in perioperative management, such as difficulties in tracheal intubation, choice of anesthetic drugs, blood and fluid management, monitoring without CVP and weaning from ventilator are observed. Induction was carried out carefully under spontaneous breathing and the anesthetic method we used consisted of balanced technique using N2O, O2, fentanyl and pancuronium bromide with moderate hyperventilation. Intraoperative course was uneventful and the patient was extubated on the second day after the operation without any neurological defects.
Anesthesia, Inhalation↗
Steroid sensitive nephrotic syndrome.
Nephrotic syndrome in children is a common recurrent disease. Most of the cases are due to minimal change disease with a favourable outcome. More than 90% of children with minimal change disease respond to corticosteroid therapy (steroid sensitive nephrotic syndrome). 40-60% experience frequent relapses or have steroid dependence. These children require frequent corticosteroid therapy and/or immunomodulators or treatment with immunosuppressants, and are at high risk of cumulative steroid toxicity and side effects of cytotoxic therapy. Children with frequent relapses or steroid dependence should be managed in consultation with a pediatric nephrologist. Despite relapsing course, progression of minimal change nephrotic syndrome to end stage renal disease is extremely rare.
Adrenal Cortex Hormones↗
Sagittal sinus thrombosis in adult minimal change nephrotic syndrome.
Nephrotic syndrome causes a hypercoagulable state, leading to both venous and arterial thrombosis. The mechanisms are as yet unclear, but numerous alterations in coagulant and anti-coagulant factors have been reported [Llach et al. 1985, Harris and Ismail 1994]. The most common clinical features in adults are renal vein thrombosis, femoral vein thrombosis and pulmonary embolism, although thrombosis in numerous other arterial and venous sites has been described [Cameron 1984, Llach et al. 1985]. Intracranial thrombosis is rare, although in adult nephrotic syndrome arterial thrombosis is well recognized [Fuh et al. 1991]. We report a patient with minimal change nephrotic syndrome who developed venous sinus thrombosis detected by magnetic resonance (MR) scanning.
Adult↗