肾病综合征英文

Prepared by:-Mohammad Ali Al-shehri.Supervised by : Dr.,Nephrotic Syndrome.(NS),Introduction,Definition of NSEtiology of NSPathology of NSPathophysiology of NS Clinical Manifestation of NSComplication NSLaboratory DataDiagnosisTreatment,Nephrotic syndrome,Nephrotic syndrome (NS) results from increased permeability of Glomeulrar basement membrane (GBM) to plasma protein. It is clinical and laboratory syndrome characterized by massive proteinuria, which lead to hypoproteinemia ( hypo-albuminemia), hyperlipidemia and pitting edema. (4-increase, 1-decrease).,Nephrotic Criteria:-,*Massive proteinuria: qualitative proteinuria: 3+ or 4+, quantitative proteinuria : more than 40 mg/m2/hr in children (selective). *Hypo-proteinemia : total plasma proteins 5.7mmol/L *Edema: pitting edema in different degree,Nephritic Criteria,-Hematuria: RBC in urine (gross hematuria) -Hypertension:130/90 mmHg in school-age children120/80 mmHg in preschool-age children110/70 mmHg in infant and toddlers children-Azotemia（renal insufficiency）: Increased level of serum BUN 、Cr-Hypo-complementemia: Decreased level of serum c3,Classification:,A-Primary Idiopathic NS (INS): majority The cause is still unclear up to now. Recent 10 years ,increasing evidence has suggested that INS may result from a primary disorder of T cell function. Accounting for 90% of NS in child. mainly discussed.B-Secondary NS: NS resulted from systemic diseases, such as anaphylactoid purpura , systemic lupus erythematosus, HBV infection. C-Congenital NS: rare*1st 3monthe of life ,only treatment renal transplantation,Secondary NS,Drug,Toxic,Allegy: mercury, snake venom, vaccine, pellicillamine, Heroin, gold, NSAID, captopril, probenecid, volatile hydrocarbonsInfection: APSGN, HBV, HIV, shunt nephropathy, reflux nephropathy, leprosy, syphilis, Schistosomiasis, hydatid diseaseAutoimmune or collagen-vascular diseases: SLE, Hashimotos thyroiditis, HSP, VasculitisMetabolic disease: Diabetes mellitus Neoplasma: Hodgkins disease, carcinoma ( renal cell, lung, neuroblastoma, breast, and etc)Genetic Disease: Alport syn, Sickle cell disease, Amyloidosis, Congenital nephropathy Others: Chronic transplant rejection, congenital nephrosclerosis,Idiopathic NS (INS): Pathology:-,Minimal Change Nephropathy (MCN): 40mg/m2/hr for childrenc- volume: oliguria (during stage of edema formation)d-Microscopically:-microscopic hematuria 20%, large number of hyaline cast,Investigations:-,2-Blood:A-serum protein: decrease 5.5gm/dL , Albumin levels are low (2.5gm/dL). B-Serum cholesterol and triglycerides: Cholesterol 5.7mmol/L (220mg/dl).C- ESR100mm/hr during activity phase .3.Serum complemen: Vary with clinical type. 4.Renal function.,Kidney Biopsy:-,Considered in: 1-Secondary N.S 2-Frequent relapsing N.S 3-Steroid resistant N.S4- Hematuria5-Hypertension6- Low GFR,Differential Diagnosis of NS:,D.D of generalized edema:-1-Protein losing enteropathy2-Hepatic Failure.3-HF4-Protein energy malnutrition5-Acute and chronic GN6-urticaria? Angio edema,Complications of NS:-,1-Infections:Infections is a major complication in children with NS. It frequently trigger relapses.Nephrotic pt are liable to infection because :A-loss of immunoglobins in urine.B-the edema fluid act as a culture medium.C-use immunosuppressive agents.D- malnutritionThe common infection : URI, peritonitis, cellulitis and UTI may be seen.Organisms: encapsulated (Pneumococci, H.influenzae), Gram negative (e.g E.coli,Complication ,Vaccines in NS;-polyvalent pneumococcal vaccine (if not previously immunized) when the child is in remission and off daily prednisone therapy.Children with a negative varicella titer should be given varicella vaccine.,Complication.,2-Hypercoagulability (Thrombosis).Hypercoagulability of the blood leading to venous or arterial thrombosis:Hypercoagulability in Nephrotic syndrome caused by:1-Higher concentration of I,II, V,VII,VIII,X and fibrinogen 2- Lower level of anticoagulant substance: antithrombin III 3-decrease fibrinolysis. 4-Higher blood viscosity 5- Increased platelet aggregation6- Overaggressive diuresis,3-ARF: pre-renal and renal 4- cardiovascular disease :-Hyperlipidemia, may be a risk factor for cardiovascular disease.5-Hypovolemic shock6-Others: growth retardation, malnutrition, adrenal cortical insufficiency,Management of NS:,General (non-specific ) *Corticosteroid therapy,General therapy:-,Hospitalization:- for initial work-up and evaluation of treatment.Activity: usually no restriction , except massive edema,heavy hypertension and infection.Diet Hypertension and edema: Low salt diet ( 2 + protein for 7 consecutive days. *Frequent relapsing: 2 relapses within 6 months ( 4/year). *Steroid resistant: failure to achieve remission with prednisolone given daily for 28 days.,Side Effects With Long Term Use of Steroids “Steroid toxicity,hyperglycemiamyopathypeptic ulcerpoor healing of wound.HirsutismThromboembolism,-Stunted growthCataracts- Pseudotumor cerebri-Psycosis -Osteo