As nephrotic syndrome excretion of> 3 g protein / day in urine due to glomerular disease is plus. Edema and hypoalbuminemia referred. It comes among children more frequently and has both primary and secondary causes. The diagnosis is made by determining the protein / creatinine ratio in a urine sample or measurement of protein in 24h urine. Underlying causes are diagnosed by history, physical examination, serological tests and kidney biopsy. Prognosis and treatment vary depending on the cause.
As nephrotic syndrome excretion of> 3 g protein / day in urine due to glomerular disease is plus. Edema and hypoalbuminemia referred. It comes among children more frequently and has both primary and secondary causes. The diagnosis is made by determining the protein / creatinine ratio in a urine sample or measurement of protein in 24h urine. Underlying causes are diagnosed by history, physical examination, serological tests and kidney biopsy. Prognosis and treatment vary depending on the cause. The etiology of nephrotic syndrome occurs in any age but is more common in children (minimal change disease) that usually between 1½ and 4 years. Congenital nephrotic syndromes occur during the first year of life. In the younger age (<8 years) are boys affected more often than girls. In higher childhood both are equally affected. The causes vary by age (see table: Glomerular disorders by age and manifestations) and can be primary or secondary (see table: causes of nephrotic syndrome). The most common primary causes are: minimal-change glomerulonephritis focal segmental sclerosing glomerulonephritis membranous nephropathy Secondary causes make <10% of pediatric cases, but> 50% of adult cases. The most common are: Diabetic nephropathy) pre-eclampsia, the amyloidosis, an underestimated cause is responsible for 4% of cases. HIV-associated nephropathy is a type of focal segmental glomerulosclerosis that occurs in AIDS patients. Causes of nephrotic syndrome causes Examples Primary causes idiopathic fibrillary and immunotaktoide GN focal segmental sclerosing glomerulonephritis IgA nephropathy * Membranoproliferative GN membranous nephropathy minimal-change glomerulonephritis Rapidly progressive GN * Secondary causes Metabolically amyloidosis diabetes mellitus Immunologically cryoglobulinemia erythema multiforme immunoglobulin-A-associated vasculitis * Microscopic polyarteritis serum sickness Sjögren’s syndrome SLE * Idiopathic Castleman’s disease Sarcoidosis Neoplastic carcinomas (eg. B. bronchi, breast, colon, stomach, kidney) leukemia lymphoma Melanoma Multiple Myeloma In connection with drugs Gold heroin interferon alpha lithium NSAIDs mercury pamidronate penicillamine Probenecid Bacterially Infectious endocarditis leprosy syphilis protozoa filariasis helminth infections Loiasis Malaria Schistosomiasis (by Schistosoma haematobium) toxoplasmosis viral Epstein-Barr virus infection Hey patitis B and C Herpes Zoster HIV infection Allergic antitoxins insect bites, poison ivy or poison oak snake genetic syndromes Congenital nephrotic syndrome Diffuse mesangial sclerosis Finnish type d. H. Nephrin defect Kortikosteroidresistentes nephrotic syndrome (Podocin effect) Denys-Drash syndrome Familial FSGS Nage-patella syndrome Fabry disease Familial FSGS hereditary nephritis sickle cell anemia physiologically adapt to reduced nephrons Morbid obesity Oligomeganephronie Other Chronic allograft urate nephropathy Malignant hypertension preeclampsia * Manifested more often than nephritic syndrome. infectious and post-infectious causes. FSGS = focal segmental glomerulonephritis; GN = glomerulonephritis. Pathophysiology proteinuria arises due to changes in the capillary endothelial cells of the glomerular basement membrane (GBM) or the podocytes, which filter the serum protein by size and charge normally selectively. The pathogenic mechanism of damage to these structures is unknown at the primary and secondary glomerular disease, but it seems as though even T cells upregulate response to unidentified immunogens and a circulating cytokines permeability factor or down-regulate an inhibitor of the permeability factor. Other possible factors are hereditary defects in proteins that are essential for the “slit diaphragms” the glomeruli; activation of complement, which leads to damage of the glomerular epithelial cells; and the loss of the negatively charged groups which are bound to the proteins of the GBM and glomerular epithelial cells. Complications of nephrotic syndrome, the disorder results in the loss of macromolecular proteins in the urine, mainly albumin, as well as opsonin, immunoglobulins, erythropoietin, transferrin, hormone-binding proteins (including thyroxine-binding globulin and vitamin D binding protein) and antithrombin III. A lack of these and other proteins contributes to a number of complications (see Table: Complications of nephrotic syndrome). Other physiological factors also play a role. Complications of nephrotic syndrome complication Causal factors edema (including ascites and pleural effusions) Generalized capillary leak due to hypoalbuminemia renal reduced oncotic pressure may sodium retention infection (especially cellulitis and spontaneous bacterial peritonitis at 2-6%) Unknown Possibly loss of opsonin and immunoglobulins anemia Loss of erythropoietin and transferrin Changes in the test results of thyroid function ((under previously hypothyroid patients at a higher dose to the thyroid hormone replacement therapy) The loss of the thyroxine-binding globulin hypercoagulability and thromboembolism, in particular renal vein thrombosis and pulmonary embolism, which occur in up to 5% in children and at 40 % of adults) loss of antithrombin III Increased hepatic synthesis of coagulation factors platelet changes hyperviscosity hypovolemia caused by a deficiency of protein in children (sometimes with brittle hair and brittle nails, Alopecia and dwarfism) loss of proteins Decreased hepatic production Sometimes decreased oral intake due to mesenteric edema dyslipidemia Elevated lipoprotein in the liver coronary heart disease in adults dyslipidemia with atherosclerosis hypertension hypercoagulable hypertension in adults Renal sodium retention bone disease corticosteroid use Chronic kidney disease Unknown Possibly hypovolemia, interstitial edema and use of NSAIDs Proximal tubular dysfunction (acquired Fanconi’s syndrome), with glucosuria, aminoaciduria, “Kaliumdepletion” phosphaturia, renal tubular acidosis, Bicarbonaturie, Hypercitraturie and Urikosurie Toxic effects on proximal tubular cells secondary to large amounts of protein to reabsorb symptoms and complaints the primary findings are anorexia, malaise and foamy urine (due to the high protein concentration). Fluid retention can cause dyspnea (pleural effusion or laryngeal edema) arthralgia (hydrarthrosis) abdominal pain (abdominal dropsy or, in children, possible mesenteric edema) Associated symptoms may develop, including peripheral edema, and ascites. Edema can superimpose the signs of muscle wasting and white bands in the nail bed causing (Muehrcke lines). Other symptoms and findings are due to the many complications of nephrotic syndrome (see table: complications of nephrotic syndrome) caused. Diagnostic urine sample ( “Spot”) protein / creatinine ratio ? 3 or proteinuria ? 3 g / 24 h Serological tests and kidney biopsy, unless the cause is obvious clinically suspected diagnosis is in patients with edema and protein in the urine close and is a sample of urine protein and creatinine or secured by the 24-hour urine protein measurement. Clinical findings (eg. As SLE, preeclampsia, cancer) may indicate the cause. In additional unknown cause (z. B. serological) tests and kidney biopsy are displayed. Urinalysis The finding of significant proteinuria (3 g protein in a 24-hour urine collection) is diagnostic (normal excretion <150 mg / day). Alternatively, the protein / creatinine ratio can be in a single urine sample in a 24-h urine the amount of protein in grams per 1.73 m2 body surface (eg. B. correspond to 40 mg / dL protein and 10 mg / dl creatinine in a single sample 4 g / appreciate 1.73 m2 body surface area in a 24-hour sample) reliably. Calculations on the basis of random samples may be less reliable when the creatinine is high (eg. As during athletic training) or low (z. B. cachexia). However, the calculations on the basis of a single sample are compared with the 24-h collection preferable because a single sample more convenient and less prone to errors (e.g., as due to lack of compliance). A more comfortable examination facilitates the monitoring of changes that occur during treatment. Clinical Calculator: body surface (Du Bois Method) Clinical Calculator: Estimate of urinary protein excretion addition to proteinuria, the urine findings show cylinders (hyaline, granulocyte, grease, wax or Epithelzellzylinder). Lipidurien, d. H. the presence of free lipids or lipids in tubule cells (oval fat bodies) in cylinders (fat cylinder) or as free fat droplets, suggest a glomerular disorder that causes nephrotic syndrome. Urine cholesterol can be detected with simple microscopy and shows under crossed polarized light, a Maltese cross shape. Evidence of triglycerides by means Sudanfärbung.Andere diagnostic studies in nephrotic syndrome Additional studies may help to assess the severity and complications. Urea and creatinine concentrations vary according to renal damage. Serum albumin often is <2.5 g / dl. Total cholesterol and triglyceride levels are usually increased. The routine determination of levels of alpha- and gamma-globulins, immunoglobulins, hormone binding hormones, ceruloplasmin, transferrin and complement is not required, but these levels may also lowers sein.Untersuchung on secondary causes of nephrotic syndrome, the importance of testing for secondary causes of nephrotic syndrome (see table: causes of nephrotic syndrome) is seen controversial because the yield may be low. Tests are best performed, as indicated by the clinical context. The tests may include the following: serum glucose or glycosylated hemoglobin (HbA1c) Antinuclear antibodies Serological tests for hepatitis B and C serum or Urinproteinelektrophorese Cryoglobulins rheumatoid factor Serological tests for syphilis (eg Rapid plasma reagin.) HIV antibody test complement levels (CH50, C3, C4) by the test results, it can lead to a change of the treatment and a biopsy may be necessary. For example, the detection of cryoglobulins, a mixed cryoglobulinemia suggest (z. B. in chronic inflammatory diseases such as SLE, Sjögren's syndrome or hepatitis C virus infection), and the detection of a monoclonal protein by serum or urine protein electrophoresis can, especially in patients who are> 50 years old and suffer from anemia, a monoclonal gammopathy include (z. B. multiple myeloma). A kidney biopsy is indicated in adults to help diagnose the disorder that is causing the idiopathic nephrotic syndrome. The idiopathic nephrotic syndrome in children impresses mostly as minimal change glomerulopathy and can usually be assumed without biopsy, unless the condition does not improve after treatment with corticosteroids. Special biopsy findings are discussed with reference to the respective disorders. Prognosis The prognosis is variable, depending on the cause. Complete remission can occur spontaneously or for therapeutic reasons. The prognosis is favorable in kortikosteroidempfindlichen disorders in general. In all cases, the prognosis may be deteriorated if accompanied by: infection hypertension Significant azotemia hematuria thrombosis in cerebral, pulmonary, peripheral or renal veins renal transplant patients with focal segmental glomerulosclerosis, IgA nephropathy and membranoproliferative glomerulonephritis (particularly type 2) have a high recurrence rate on. Treatment Treatment of the causative disease Angiotensinhemmung sodium restriction statins diuretics nephrectomy with excessive fluid overload Rarely treating the causes that cause nephrotic syndrome, the treatment of disorders underlying, the immediate treatment of infections (eg. As staphylococci, endocarditis, malaria, syphilis, schistosomiasis ), which include allergic desensitization (z. B. opposite poison oak, poison ivy [Toxicodendrum radicans] or insect antigen) and discontinuation of medication (eg. as gold, penicillamine, NSAIDs). These measures may in specific cases for the healing of nephrotic syndrome beitragen.Proteinurie treatment Angiotensinhemmung (with ACE inhibitors or angiotensin II receptor blockers) is indicated to reduce systemic and intraglomerular pressure and proteinuria. These drugs may occur in patients with moderate to severe renal insufficiency cause hyperkalemia or worse. A protein restriction is no longer recommended, as their effect can not be detected on the progression konnte.Ödembehandlung sodium restriction (<2 g of sodium, or about 100 mmol / day) is recommended in patients with symptomatic edema. Loop diuretics are usually needed to control edema, but may worsen preexisting renal insufficiency and hypovolemia, hyperviscosity and hypercoagulability and should be used only when a sodium restriction is ineffective, or if there is evidence of intravascular fluid overload. In severe cases of nephrotic syndrome can also i.v. Infusions of albumin, followed by a loop diuretic for control of edema given werden.Dyslipidämie treatment statins are indicated for dyslipidemia. A limitation of saturated fat and cholesterol intake is recommended for control of dyslipidemia are favorable to beeinflussen.Behandlung hypercoagulable anticoagulants indicated for the treatment of thromboembolism, but there are few data to support its use in primary prevention. Treatment of an infection risk All patients should receive pneumococcal vaccine if it is not otherwise contraindicated. Nephrectomy in nephrotic syndrome rarely bilateral nephrectomy in severe nephrotic syndrome due to the persistent hypoalbuminemia is necessary. The same result can be achieved by embolization of renal artery with coils sometimes, making surgery in patients can be avoided with high risk. Dialysis is performed according to necessity. usually in idiopathic nephrotic syndrome Conclusion The most commonly occurs in young children, and mostly as minimal change glomerulopathy. In adults, nephrotic syndrome usually occurs secondary, often with diabetes or pre-eclampsia. The nephrotic syndrome should in patients, particularly children, should be considered with unexplained edema or ascites. The diagnosis of nephrotic syndrome is confirmed by a protein / creatinine ratio ? 3 or urine protein ? 3 g / 24 h. Tests for secondary causes and a renal biopsy should be performed selectively based on clinical findings. A minimal change glomerulopathy must be assumed if the status of a child improves with idiopathic nephrotic syndrome following treatment with corticosteroids. The causative disorder is treated with Angiotensinhemmung, sodium restriction and often diuretics and / or statins.