| ID | 398 |
|---|---|
| Name | NEPHROTIC SYNDROME It is a clinical syndrome characterised by massive proteinurea, hypoproteinemia, generalised oedema & hyperlipidemia. |
| Cause | Diabetic kidney disease. Diabetes can lead to kidney damage (diabetic nephropathy) that affects the glomeruli. Minimal change disease. This is the most common cause of nephrotic syndrome in children. ... Focal segmental glomerulosclerosis. ... Membranous nephropathy. ... Systemic lupus erythematosus. ... Amyloidosis. |
| Signs Symptoms | Symptoms in Child Extreme tiredness (fatigue) A general feeling of discomfort (malaise) Decreased appetite. Weight gain and facial swelling. Belly swelling or pain. Foamy urine. Fluid buildup in the body (edema) Fluid buildup in the belly area (ascites) Symptoms in Adults Severe swelling (edema), particularly around your eyes and in your ankles and feet. Foamy urine, a result of excess protein in your urine. Weight gain due to fluid retention. Fatigue. Loss of appetite. |
| Diagnosis | Essentials of diagnosis:1 1. Proteinuria > lgm/m2/24 hr. 2. Hypoalbuminemia (serum albumin < 2.5gm/dl). 3. Hypercholesterolemia (fasting serum cholesterol level > 220mg/dl). 4. Peripheral oedema (or anasarca with ascites). Diagnosis: A. Clinical history & presentation. B. Physical examination! C. Laboratory investigations- 1. Urine for- R/E, proteinuria, microscopic hem-aturia, urinary total protein, C/S with colony count (UTI) etc. 2. Blood- complete picture- Hb% increased (due to hemo cone). TC- increased (due to infection). Platelet- increased (due to hemo cone.) Hematocrit- increased (due to hemo cone.) 3. Blood biochemistry-S. total protein- decreased S. A/G ratio- reverse S. Gamma-globulin (by electroporesis)- i S. cholesterol- increased Blood urea S. creatinine gives normal results until S. electrolytes the final stage of illness 4. Serology- ASO litre, to exclude the VDRL test respective HBs Ag test diseases DNA- antibody ANF- anti-nuclear factor Compliment C3 5. Protein selectivity index- to determine the selectivity of proteinuria. 6. X-ray chest 7. I.V.U 8. Renal biopsy-Indication- when N. sydrome found resistant to steroid therapy with 8 weeks treatment. |
| Investigations | Severe swelling (edema), particularly around your eyes and in your ankles and feet |
| Management | Management: A. General management- 1. Careful monitoring of daily fluid intake & output; water intake should be restricted only if there is progressive oedema. 2. Diet - should be a full nutritious diet adequate in calorie & rich in protein (if no uremia). Salt should be restricted as long as there is oedema & proteinuria. 3. Nursing care- a. Adequate nursing care is essential to prevent any secondary infection or skin infection, b. Regular body weight should be taken. 4. Antibiotic to prevent secondary infection. B. Specific treatment Treatment of proteinurea by- steroid. Before giving steroid be confirm about the followings- a. ne hematuria b. no hypertension. c. renal function is not impaired. Steroid therapy (in minimal changed nephropathy)- regimens for the prednisone treatment of the initial attack and relapses of MCNS, proposed by APN (Arbeitsgemeinschaft fuer Pediatrische Nephrologie, German): as practiced commonly in our country: Initial attack - Prednisolone 2mg/kg/day in 2-3 divided doses for 6 weeks followed by 1.5mg/kg/48 hr. single morning dose for 6 weeks.39 Relapse - Prednisolone 2mg/kg/day in 3 divided doses until urine protein-free for 3 days followed by alternate-day prednisolone 1.5mg/kg/48 hr. given as a single morning dose for 4 weeks. If the diagnosis of a primary NS is confirmed, the first step is initiation of prednisone therapy in a standard regimen for 8 weeks. Most children respond already within 4 weeks of continuous prednisone therapy. In case they do not achieve remission after 8 weeks of prednisone treatment, a renal biopsy is indi-cated in order to recognize the underlying his to pathology and to design further treatment. In frequent relapsing cases, pednisone 60mg /m2/day in 3-4 divided doses can be used until urine becomes protein-free for 10-14 days, followed by alternate day prednisone 60mg/m2/48 hr given at a single morning dose for 6-12 months. 2. Cytotoxic drugs in MCNS: Cyclophosphamide produce long lasting remissions in frequent relapsers with or without steroid dependency. Cyclophosphamide in a dose of 2mg/kg/day for 8 weeks is effective. Steroid dependent cases should receive a prolonged course of Cyclophosphamide i.e. 2mg/kg/day for 12 weeks. Indications- a. Steroid resistant. b. Frequent relapser. c. Steroid toxic children occured as a result of long term steroid treatment. 3. Relief of oedema & ascites by- a. Rest in bed & salt restriction. b. High protein diet (1 gm/kg/day)- (is now seldom advise) if blood urea is not raised. c. Diuretics: Mild to moderate oedema can be treated with chlorothiazide 10-40mg/kg/day until corticos-teroid induced diuresis begins. If hypoka-lemia develops, spironolactone 3-5mg/ kg/day in 4 divided doses can be added. 4. Treatment of complications-Massive edema, hypovolemia, thromboembolism, infections, hypertension, renal failure etc. should be treated, when present, i. Massive oedema can be treated with hydrochlo-rothiazide with or without spirono-lactone before prednisone therapy. Frusemide should be used with caution, it may cause intravascular volume to contract more, may induce shock, ii. Salt-poor albumin Igm/kg by i.v infusion over 2 hr, followed by i.v frusemide, may be useful in severe hypoalbuminemia & refractory oedema, iii. Daily oral penicillin is recommended by some nephrologists for prophylaxis against pneumococcal infections, iv. Hypertension, hematuria, UTI if present, should be treated first, then steroid should be given, v. Infections (peritonitis etc.) if any must be treated vigorously. 5. Advise on discharge from hospital-Urine test for every 15 days or 1 month interval to detect the recurrence. If recurre-nce then give steroid again in full dose. In steroid resistant cases cytotoxic drug- cyclophospha-mide 2mg/kg/day and continued for 2 weeks after remission. Note : In some patients the response to corticosteroids is partial with reduction in proteinuria only to levels of 1-2 gm/24 hour; such patients may loose the features of NS hut are at increased risk for relapse. Some gradually reduce the protein excretion to normal levels over a period of months. In these patients, the best approach is an alternate day prednisone regimen sustained over a period of months. The long-term outlook is fairly good, only a few progress to chronic renal failure. Table : Definitions & terminology to describe patients with MCNS (from ISKDC&APN)” Urinary remission : Protein free urine (< 4mg/M2/hr). Complete remission : Serum albumin > 35gm/l. Relapse : Protein-positive urine - 3gm or more on 3 consecutive examinations on 3 separate days. Fast relapse : Relapse which occurs during the prednisone treatment given for an earlier relapse or within 14 days after the end of prednisone treatment. Frequent relapser : A relapser who has 2 relapses within 6 months of the initial response, without being steroid dependent. Steroid depender : A frequent relapse in whom two consecutive relapses are fast relapses, or 2 of 4 relapses within 6 months were fast relapses. Late responder : Initial non-responder who responds at some time following the initial 8 weeks of treatment. |
| Introduction | A kidney disorder that causes your body to pass too much protein in your urine |
| History | |
| Etiology | Etiology:2 Glomerular lesions resulting in nephrotic syndrome- 1. Primary (or idiopathic) glomerulonephritis (GN)-Minimal change nephropathy- 85% Focal segmental glomerular sclerosis- 10% Membranous GN Membranoproliferative GN-mesangial prolifera-tive, focal proliferative- 5% Mesangiocaplillary GN 2. Associated with systemic disease-Systemic lupus erythematosus Polyarteritis nodosa; microscopic polyartertis; Wegener’s granulomatosis Amyloidosis Diabetes mellitus 3. Associated with infection-Bacterial endocarditis; malaria; hepatitis-B; syphilis 4. Associated with tumours-Carcinoma; Hodgkin’s disease; chronic lymphatic leukemia 5. Associated with drugs- Penicillamine, captopril, gold, mercury, trimethadione, phenindione, heroin (contaminated). |
| Clinical Features | Severe swelling (edema), particularly around your eyes and in your ankles and feet. Foamy urine, a result of excess protein in your urine. Weight gain due to fluid retention. Fatigue. Loss of appetite. |
| Preventions | Prevent some causes of nephrotic syndrome. Avoid damage to your glomeruli: Manage high blood pressure and diabetes, Be sure to get vaccines for common infections, especially if you work around people who have hepatitis or other diseases. |
| Treatment | Child At least a 4-week course of the steroid medicine prednisolone, followed by a smaller dose every other day for 4 more weeks. Adult Blood pressure medications. Drugs called angiotensin-converting enzyme (ACE) inhibitors reduce blood pressure and the amount of protein released in urine. ... Water pills (diuretics). ... Cholesterol-reducing medications. ... Blood thinners (anticoagulants). ... Immune system-suppressing medications. |
| Complications | Complications of N.S: 1. Intercurrent infection, such as- - peritonitis - cellulitis - pneumonia - septicemia (sepsis) -UTI 2. Massive oedema 3. Hypovolemia 4. Peritonitis 5. Diarrhoea or loose motion 6. Hypertension 7. Renal failure with uremia 8. Thromboembolism 9. Infection with chicken pox or measles. 10. Hemorrhagic tendency (due to lack of factor ix, xi & xii). 11. Vitamin-D deficiency (due to hypoalbuminemia) |
| Prognosis | Prognosis & course of N.S: Nephrotic sydrome in childhood is mostly due to MCNS (75-80%), which responds to corticosteroid therapy about 90-95%. Early diagnosis & treatment with steroid brings a good prognosis. Some resistant cases of MCNS may develop renal failure. Most of the steroid-response nephrotic syndrome cases have repeated relapses until disease resolves spontaneously towards the end of second decades of life (14-20 years) with no residual renal dysfunction. Prognosis is guarded in steroid resistant cases. Intercurrent infection in N.S can be prevented & treated with adequate antibiotic therapy. Indeed complete recovery may be expected with proper management in about 60-70% of cases. Death now occur usually from uremia after a long course of oedema & massive proteinuria with or without remission, spontaneous or induced by treatment, intercurrent infection still causes death to a good percentage usually of untreated (or even treated) cases. Other forms of N.S. (usually resistant to steroid) are almost certain to progress to renal failure & ultimately die. |
| Types | |
| Classification | Classification (histopathologic): Types Percent % Steroid response MCNS (Minimal change .NS) 76.4 90-95% GS (Glomerular sclerosis) 8.6 20% MPGN (Membrano proliferative) GN. 7.5 Proliferative GN. (mesangial) 4.6 50-60% Membranous GN 1.5 Misc. 1.4 Characteristics of MCNS : The MCNS is the most frequent type of nephrotic syndrome in childhood^ it has few characteristics: 1. Nephrotic syndrome (NS) of unknown etiology (disease is some-how related to abnormal changes in T-lymphocyte function). 2. Characteristic clinical course. 3. Responsiveness to glucocorticosteroids (90%) and cytotoxic agents. 4. High tendency to relapse. 5. Absence of progressive renal deterioration 6. Negative morphological findings by light and immunomicroscopy. (L.M- normal; E.M- fused podocytes) Clinical features (MCNS): 1. Age- most commonly appears between 2 & 6 yrs. of age (but may be as early as second half of the first year & not uncommon in the adult). 2. Onset- insidious. 3 Oedema- first noted around the eyes, face & in the lower limbs then becomes generalized & associated with weight gain. 4. Scanty micturition with high coloured urine (may be frothy due to proteinuria). 5. Ascites &/or pleural effusion. 6. May be associated with- fever, anemia, cough, anorexia, loose motion, boils or skin infection 7. Abdominal pain- may be due to hypovolemic ischemic pains. 8. Hematuria- rarely (if poor prognosis). 9. Hypertension- rarely or uncommon. 10. Sometimes miserable. |
| Observation | |
| Pathology |
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