Untitled-6

G U I D E L I N E S F O R P R A C T I C E Management of Steroid Sensitive
INTRODUCTION
Nephrotic Syndrome:
Nephrotic syndrome is an important chronic disease Revised Guidelines
in children. About 80% children with idiopathicnephrotic syndrome show remission of proteinuriafollowing treatment with corticosteroids, and are INDIAN PEDIATRIC NEPHROLOGY GROUP,
classified as ‘steroid sensitive’. Most patients have INDIAN ACADEMY OF PEDIATRICS
multiple relapses, placing them at risk for steroidtoxicity, systemic infections and other complica-tions. A small proportion of patients who are not ABSTRACT
steroid sensitive (steroid resistant) are also at risk forsimilar complications and renal insufficiency.
Justification: In 2001, the Indian Pediatric Nephrology
Group formulated guidelines for management of patients
Most pediatricians would encounter few patients with steroid sensitive nephrotic syndrome. In view of with nephrotic syndrome in their practice. They emerging scientific evidence, it was felt necessary to should be familiar with management of these review the existing recommendations. Process:
patients and be aware of situations in which referral Following a preliminary meeting in March 2007, a draft to a pediatric nephrologist is required. Long-term statement was prepared and circulated among pediatric management of these patients should thereafter be a nephrologists in the country to arrive at a consensus on joint effort between the pediatrician and the pediatric the evaluation and management of these patients.
Objectives: To revise and formulate recommendations
for management of steroid sensitive nephrotic syndrome. OBJECTIVES
Recommendations: The need for adequate cortico-
steroid therapy at the initial episode is emphasized.
Guidelines on the management of children with Guidelines regarding the initial evaluation, indications nephrotic syndrome were first formulated by the for renal biopsy and referral to a pediatric nephrologist Indian Pediatric Nephrology Group in 2001(1).
are updated. It is proposed that patients with frequently Since a number of studies on management of these relapsing nephrotic syndrome should, at the first patients have been published during the last 7 years, instance, be treated with long-term, alternate-dayprednisolone. The indications for use of alternative it was felt desirable to review the existing immunosuppressive agents, including levamisole, recommendations. Therefore, following a prelimi- cyclophosphamide, mycophenolate mofetil and nary meeting in New Delhi (7 March 2007), a draft cyclosporin are outlined. The principles of dietary statement was prepared, circulated and reviewed therapy, management of edema, and prevention and by pediatric nephrologists across the country management of complications related to nephrotic (Annexure I). The present document reflects the syndrome are described. These guidelines, formulated on current opinion on management of patients with basis of current best practice, are aimed to familiarize steroid sensitive nephrotic syndrome.
physicians regarding management of children withsteroid sensitive nephrotic syndrome. RECOMMENDATIONS
Key words: Nephrotic syndrome, Practice guidelines,
Important revisions in this document are listed in
Table I.
Correspondence to: Dr. Arvind Bagga, Department of Definitions
Pediatrics, All India Institute of Medical Sciences, AnsariNagar, New Delhi 110 029, India. Nephrotic syndrome is characterized by heavy proteinuria, hypoalbuminemia (serum albumin TABLE I IMPORTANT REVISIONS IN THIS DOCUMENT
Investigations necessary at initial and subsequent evaluation are described.
While updating the literature, the Group endorses the existing guidelines on therapy for the initial episode of nephroticsyndrome.
The role of alternative medications, including mycophenolate mofetil, cyclosporin and tacrolimus in patients withfrequent relapses and steroid dependence is discussed and therapeutic choices further clarified.
Details on dose and duration of therapy with corticosteroids, when co-administered with other agents are included.
Guidelines on immunization, isoniazid prophylaxis and hypertension updated in conformity with recommendations ofthe Indian Academy of Pediatrics.
Management of complications updated.
<2.5 g/dL), hyperlipidemia (serum cholesterol >200 creatinine. Estimation of blood levels of anti- mg/dL) and edema(1,2). Nephrotic range proteinuria streptolysin O and C3 is required in patients is present if early morning urine protein is 3+/4+ (on with gross or persistent microscopic hematuria.
dipstick or boiling test), spot protein/creatinine ratio Appropriate tests are performed, if necessary, for >2 mg/mg, or urine albumin excretion >40 mg/m2 associated conditions (e.g., chest X-ray and tuber- per hr (on a timed-sample). Precise quantitative culin test, hepatitis B surface antigen, antinuclear assessment of proteinuria, including 24-hr urine antibodies). Urine culture is not necessary unless the protein measurement is seldom necessary.
patient has clinical features suggestive of a urinary Definitions for clarifying the course of nephrotic syndrome are shown in Table II.
Treatment of the Initial Episode
Initial Evaluation
Adequate treatment of the initial episode, both in A detailed evaluation is necessary before starting terms of dose and duration of corticosteroids, is treatment with corticosteroids. The height, weight important. Evidence from multiple studies suggests and blood pressure should be recorded. Regular that appropriate therapy at the first episode of weight record helps monitor the decrease or increase nephrotic syndrome is an important determinant of of edema. Physical examination is done to detect the long-term course of the disease(3).
infections and underlying systemic disorder, e.g.,systemic lupus erythematosus, Henoch Schonlein Medication: The standard medication for treatment
purpura, etc. Infections should be treated before is prednisolone or prednisone. The medication is starting therapy with corticosteroids.
administered after meals to reduce its gastro-intestinal side effects. The use of methyl- Investigations recommended at the initial prednisolone, dexamethasone, betamethasone, tri- episode include: (i) urinalysis; (ii) complete blood amcinolone or hydrocortisone is not recommended.
count, blood levels of albumin, cholesterol, urea and There is also limited evidence on the efficacy or TABLE II DEFINITIONS RELATED TO NEPHROTIC SYNDROME
Urine albumin nil or trace (or proteinuria <4 mg/m2/h) for 3 consecutive early morning specimens.
Urine albumin 3+ or 4+ (or proteinuria >40 mg/m2/h) for 3 consecutive early morning specimens,having been in remission previously.
Two or more relapses in initial six months or more than three relapses in any twelve months.
Two consecutive relapses when on alternate day steroids or within 14 days of its discontinuation.
Absence of remission despite therapy with daily prednisolone at a dose of 2 mg/kg per day for 4 weeks.
benefits of therapy with deflazocort for nephrotic rarely result in spontaneous remission, thereby avoiding the need for treatment with corticosteroids.
Treatment regimen: Various treatment regimens
Prednisolone is administered at a dose of have been used for the treatment of the initial episode 2 mg/kg/day (single or divided doses) until urine of nephrotic syndrome. The International Study protein is trace or nil for three consecutive days.
for Kidney Diseases in Children had originally Subsequently, prednisolone is given in a single recommended a regimen comprising of four-weeks morning dose of 1.5 mg/kg on alternate days for each of daily and alternate day steroid therapy(4), 4 weeks, and then discontinued(1,5). The usual which was used for almost three decades. Controlled duration of treatment for a relapse is thus 5-6 weeks.
studies later suggested that prolongation of initial Prolongation of therapy is not necessary for patients steroid therapy for 12 weeks or longer is associated with infrequent relapses (see below).
with significantly reduced risk for subsequentrelapses. However, prolonged treatment with In case the patient is not in remission despite two steroids is associated with a higher frequency of weeks treatment with daily prednisolone, the treatment is extended for 2 more weeks. Patientsshowing no remission despite 4 weeks’ treatment The Cochrane Renal Group(3), on systematic with daily prednisolone should be referred for analysis of the literature, recommends that the duration of initial prednisolone therapy should be fora minimum of 12 weeks. It further suggests that the Infrequent Relapsers
benefits of sustained remission and reduction in Patients who have three or less relapses a year and relapse rates are superior if alternate-day treatment is respond promptly to prednisolone are managed not stopped abruptly at 12 weeks, but tapered over using the aforementioned regimen for each relapse.
the next 2-4 months. It is emphasized that none of the Such children are at a low risk for developing steroid studies included in this analysis was placebo- controlled, most lacked allocation concealment andwere not powered to evaluate side effects of Frequent Relapsers and Steroid Dependence
prolonged treatment(3). The debate regarding Patients with frequent relapses or steroid appropriate dose and duration of steroid treatment is dependence should be managed in consultation with not resolved. Other regimens are being examined that reduce the risk of relapse without increased sideeffects.
It is usually not necessary to perform a renal biopsy in these cases. Following treatment of a Based on current evidence and opinion, the relapse, prednisolone is gradually tapered to Group recommends that the initial episode of maintain the patient in remission on alternate day nephrotic syndrome be treated with prednisolone at a dose of 0.5-0.7 mg/kg, which is administered for 9- dose of 2 mg/kg per day (maximum 60 mg in single 18 months. A close monitoring of growth and blood or divided doses) for 6 weeks, followed by 1.5 mg/kg pressure, and evaluation for features of steroid (maximum 40 mg) as a single morning dose on toxicity is essential. If the prednisolone threshold, alternate days for the next 6 weeks; therapy is then to maintain remission, is higher or if features of discontinued. The benefits and safety of prolonged corticosteroid toxicity are seen, additional use of the initial steroid therapy, beyond 12 weeks, require following immuno-modulators is suggested.
Treatment of Relapse
Levamisole is administered at a dose of2-2.5 mg/kg on alternate days for 12-24 The patient should be examined for infections, months(7-9). Co-treatment with prednisolone which should be treated before initiating steroid at a dose of 1.5 mg/kg on alternate days is given therapy. Appropriate therapy of an infection might for 2-4 weeks; its dose is gradually reduced by 0.15-0.25 mg/kg every 4 weeks to a mainte- nephrotoxicity (increase in serum creatinine by nance dose of 0.25-0.5 mg/kg that is continued 30% or more from the baseline)(12). Trough for six or more months. Occasionally, it might (12-hr) CsA levels should be kept between 80- be possible to discontinue treatment with 120 ng/mL(12). Side effects of CsA therapy corticosteroids. The chief side effect of levamisole is leukopenia; flu-like symptoms, liver toxicity, convulsions and skin rash are toxicity; hypercholesterolemia and elevated rare. The leukocyte count should be monitored transaminases may occur. Estimation of blood levels of creatinine is required every 2-3months and a lipid profile annually. A repeat Cyclophosphamide is administered at a kidney biopsy, to examine for histological dose of 2-2.5 mg/kg/day for 12 weeks(10).
evidence of nephrotoxicity, should be done if Prednisolone is co-administered at a dose of therapy with calcineurin inhibitors is extended followed by 1 mg/kg for the next 8 weeks;steroid therapy is tapered and stopped over the Tacrolimus is an alternative agent, adminis- tered at a dose of 0.1-0.2 mg/kg daily for 12-24 months. Side effects include hyperglycemia, diarrhea and rarely neurotoxicity (headache,seizures). The use of tacrolimus is preferred Total leukocyte counts are monitored every especially in adolescents, because of lack of 2 weeks; treatment with cyclophosphamide is cosmetic side effects(13). Blood levels of temporarily discontinued if the count falls creatinine and glucose should be estimated Mycophenolate mofetil (MMF) is given at a complication of hemorrhagic cystitis; other dose of 800-1200 mg/m2 along with tapering side effects are alopecia, nausea and vomiting.
doses of prednisolone for 12-24 months(7,14).
The risk of gonadal toxicity is limited with The principal side effects include gastro- intestinal discomfort, diarrhea and leukopenia.
phosphamide(7,10,11). The use of more than Leukocyte counts should be monitored every one course of this agent should preferably be 1-2 months; treatment is withheld if count falls Choice of agent: The advantages of using these
chlorambucil, unless under close supervision, drugs should be balanced against their potential toxicity. There are few studies comparing one agent Calcineurin inhibitors: Cyclosporin (CsA) is with another, but evidence for efficacy is strongest given at a dose of 4-5 mg/kg daily for 12-24 for cyclophosphamide and CsA. Levamisole has a months. Prednisolone is co-administered at a modest steroid sparing effect and is a satisfactory dose of 1.5 mg/kg on alternate days for 2-4 initial choice for patients with frequent relapses or weeks; its dose is gradually reduced by 0.15- steroid dependence. Treatment with cyclophospha- 0.25 mg/kg every 4 weeks to a maintenance mide is preferred in patients showing: (i) significant dose of 0.25-0.5 mg/kg that is continued for six steroid toxicity, (ii) severe relapses with episodes or more months. Occasionally, treatment with of hypovolemia or thrombosis, and (iii) poor corticosteroids may be discontinued.
compliance or difficult follow up, where 12 weekstherapy might be possible to ensure than long-term Estimation of trough blood levels of CsA is required in patients with suspected non-compliance, unsatisfactory response or recommended for patients who continue to show corticosteroids stimulates appetite, parents should be steroid dependence or frequent relapses despite advised regarding ensuring physical activity and treatment with the above medications(12). Either of these agents is effective in maintaining remission in Edema: Control of edema is an integral part of
most patients with steroid sensitive nephrotic supportive care. Since treatment with corticosteroids syndrome. The chief concern with their use is usually leads to diuresis within 5-10 days, diuretics nephrotoxicity, but with careful assessment of renal are avoided unless edema is significant. Diuretics function, minimizing the maintenance dose and should also not be given to patients with diarrhea, utilizing renal biopsies in those receiving prolonged therapy, this risk can be minimized. Recent caseseries(14) and a controlled trial(15) support the use Patients with persistent edema and weight gain of of MMF as a steroid sparing agent. The lack of renal, 7-10% are treated with oral frusemide (1-3 mg/kg hemodynamic and metabolic toxicity with this agent daily). Additional treatment with potassium sparing makes it an attractive alternative to calcineurin diuretics is not required if frusemide is used at this dose for less than one week. Patients requiringhigher doses and prolonged duration of treatment with frusemide should receive potassium sparing levamisole, MMF and calcineurin inhibitors, diuretics, e.g., spironolactone (2-4 mg/kg daily).
treatment with prednisolone might be tapered and Blood pressure should be monitored frequently. A discontinued after 6-12 months. Some patients who gradual reduction of edema, over one week, is respond to therapy with levamisole, MMF and calcineurin inhibitors may relapse once thesemedications are discontinued. Relapses during or Edema not responding to the above therapy following therapy with these agents are treated with should be managed in a hospital. A combination of a loop and thiazide diuretic, and/or a potassiumsparing agent is occasionally necessary. For patients Failure of alternative medication: If a patient has
with refractory edema, a combination of diuretics two or more relapses over 6 months while on and albumin infusion is used. Albumin (20%) is treatment with any of the above agents, its given as an infusion at a dose of 0.5-1 g/kg over 2-4 replacement with an alternative medication should hr, followed by administration of frusemide (1-2 mg/ be considered. A protocol summarizing the kg intravenously). While infusion of albumin results management of patients with steroid sensitive in increased urine output, the effect is not sustained nephrotic syndrome is shown in Fig. 1.
and repeated administration might be necessary.
Supportive Care
Albumin should be administered very cautiously inpatients with renal failure, pneumonia or pulmonary This forms an important aspect of managing children edema due to its potential to increase the plasma volume. Patients receiving albumin should beobserved for respiratory distress, hypertension Diet: A balanced diet, adequate in protein (1.5-2 g/
and congestive heart failure. Refractory ascites kg) and calories is recommended. Patients with interfering with respiration or associated with breaks persistent proteinuria should receive 2-2.5 g/kg of in the skin may be removed by cautious paracentesis.
protein daily(16). Not more than 30% calories A protocol for treatment of edema is shown in Fig. 2.
should be derived from fat and saturated fatsavoided. While salt restriction is not necessary in Patient and parent education: Long-term outcome of
most patients with steroid sensitive nephrotic children with steroid sensitive nephrotic syndrome is syndrome, reduction of salt intake (1-2 g per day) is satisfactory, with the majority in sustained remission advised for those with persistent edema. Salt should and with normal renal functions by adolescence. A not be added to salads and fruits, and snacks proportion of patients, especially those (i) with early containing high salt avoided. Since treatment with onset of nephrotic syndrome, (ii) with a frequently Absence of hypertension, hematuria, azotemia Prednisolone 2 mg/kg daily for 6 weeks, followed by 1.5 mg/kg on alternate days for 6 weeks Threshold <0.5-0.7 mg/kg on alternate days FIG. 1. Management of patients with steroid sensitive nephrotic syndrome. relapsing course, and (iii) requiring treatment with expected course and risk of complications. The alkylating agents or CsA may continue to show relapses beyond adolescence(18). Parents should be Urine examination for protein at home using reassured that despite a relapsing course, progression dipstick, sulfosalicylic acid or boiling test. The to end stage renal failure necessitating dialysis or during a relapse, during intercurrent infections Parental motivation and involvement is essential or if there is even mild periorbital puffiness.
in the long-term management of these children. They should be provided information about the disease, its remission. The importance of detecting relapse before development of significant edema is Maintain a diary showing results of urine protein examination, medications received and Ensure normal activity and school attendance; the child should continue to participate in all Since infections are an important cause ofmorbidity, patients should receive appropriate Other medications: The use of antacids or histamine
receptor antagonists (e.g., ranitidine) is not
necessary, unless there are symptoms of upper gastrointestinal discomfort. Long-term calciumsupplementation (calcium carbonate, 250-500 mg) is necessary if the patient receives more than 3 months treatment with prednisolone(19). Patients withsteroid sensitive nephrotic syndrome do not usually require medications for hyperlipidemia, since lipids Immunization: Parents should be advised regarding
the need for completing the primary immunization.
Administration of some vaccines, e.g., hepatitis B,measles-mumps-rubella or meningococcal vaccines Patients receiving prednisolone at a dose of 2 mg/ kg/day or greater, or total 20 mg/day or greater (for patients weighing >10 kg) for more than 14 days areconsidered immunocompromised(20). Such patients FIG. 2. Management of edema in patients with nephrotic should not receive live attenuated vaccines; syndrome. Patients requiring high-dose frusemide or inactivated or killed vaccines are safe(20). Live addition of other diuretics should be under closesupervision, preferably in a hospital. Monitoring of vaccines are administered once the child is off serum electrolytes is necessary in all patients immunosuppressive medications for at least 4 receiving diuretics. Patients showing hypokalemia weeks. If there is a pressing need, these vaccines may require potassium supplements or co-administration be given to patients receiving alternate day of spironolactone. The medications are reduced prednisolone at a dose less than 0.5 mg/kg.
* Management of hypovolemia consists of rapid infusion of All children with nephrotic syndrome should normal saline at a dose of 15-20 mL/kg over 20-30 minutes; receive immunization against pneumococcal this may be repeated if clinical features of hypovolemia infections(21). It is important to note that not all persist. Infusion of 5% albumin (10-15 mL/kg) or 20% pneumococcal serotypes are included in the vaccines albumin (0.5-1 g/kg) may be used in subjects who do notrespond despite two boluses of saline. and that antibody levels may decline during a relapse. Previously vaccinated children may, there- TABLE III INDICATIONS FOR KIDNEY BIOPSY
fore, develop pneumococcal peritonitis and sepsis.
The Expert Group endorses the recommendations ofthe Immunization Committee of the Indian Academy of Pediatrics(22). The Committee recommends 2-4 • Gross hematuria, persistent microscopic hematuria or doses of the heptavalent conjugate pneumococcal vaccine for children below 2 yr of age. For previously unimmunized children between 2-5 yr • Renal failure not attributable to hypovolemia.
old, a priming dose of the conjugate vaccine should • Suspected secondary causes of nephrotic syndrome.
be followed 8 weeks later, by a dose of the 23-valent polysaccharide vaccine. Children older than 5 yr • Proteinuria persisting despite 4-weeks of daily require only a single dose of the polysaccharide vaccine. The vaccine should be given during • Before treatment with cyclosporin A or tacrolimus.
remission, preferably when the child is not receivingdaily prednisolone. Revaccination after 5 yr isconsidered for children (<10-yr-old) with active Complications
Patients with steroid sensitive nephrotic syndrome suppressive therapy should receive the varicella are at risk for certain complications, early detection vaccine. One dose is recommended for children between 12 months and 12 yr of age, and 2 doses Infections: Children with nephrotic syndrome are
separated by an interval of at least 4 weeks for susceptible to severe infections, which need prompt treatment. Common infections include peritonitis, Kidney Biopsy
cellulitis and pneumonia. Viral and bacterial infec-tions may occasionally precipitate relapses in Children with idiopathic nephrotic syndrome not patients previously in remission. The clinical having hematuria, hypertension or impaired renal features and management of common serious function are treated with corticosteroids without infections are summarized in Table V.
requiring a kidney biopsy. A biopsy is usually not Varicella may be a severe illness in patients with necessary in patients with frequent relapses or nephrotic syndrome receiving corticosteroids or steroid dependence before starting treatment with other immunosuppressive drugs. Susceptible levamisole, cyclophosphamide or MMF, but should patients (those unimmunized or with no history of be performed before therapy with calcineurin varicella) who are exposed to a case of chickenpox inhibitors. A biopsy is required to identify the
underlying renal disease in certain cases
(Table III).
TABLE IV INDICATIONS FOR REFERRAL TO A PEDIATRIC
Kidney biopsies must be performed by experts with experience in the procedure. Centers that Onset below 1-year of age; family history of nephrotic perform kidney biopsies should have facilities for evaluation of the specimens by light and immuno- Nephrotic syndrome with hypertension, gross/persistent microscopic hematuria, impaired renal function, orextrarenal features (e.g., arthritis, serositis, rash).
Referral to Pediatric Nephrologist
Complications: refractory edema, thrombosis, severeinfections, steroid toxicity.
Indications for referral of patients are given in
Table IV. The care of these patients should be a joint
collaboration between the pediatrician and pediatric Frequently relapsing or steroid dependent nephrotic should therefore receive a single dose of varicella immobilization, indwelling vascular catheters, zoster immunoglobulin, within 96 hr of exposure to aggressive diuretic use and puncture of deep vessels prevent or lessen the severity of the disease(17).
predispose to thrombus formation. Renal vein Since, this preparation is expensive and not easily thrombosis is suspected in a patient with oligoanuria, available, a single dose of intravenous immuno- hematuria or flank pain, especially following an globulin (400 mg/kg) may be used instead(23).
episode of dehydration. Femoral and mesenteric However, no clinical data showing the effectiveness arterial thrombosis may occasionally occur. Deep of the latter strategy are available.
vein thrombosis of calf veins is less common inchildren but may lead to pulmonary embolism.
Patients who develop varicella should receive Saggital sinus and cortical venous thrombosis may intravenous acyclovir (1500 mg/m2/day in 3 doses) follow episodes of diarrhea and present with or oral acyclovir (80 mg/kg/day in 4 doses) for convulsions, vomiting, altered sensorium and 7-10 days(23). The dose of prednisolone should be neurological deficits. Ultrasonography, Doppler tapered to 0.5 mg/kg/day or lower during the studies and cranial MRI are useful in confirming the Patients with thrombotic complications require urgent treatment. The treatment includes correction tuberculosis should receive prophylaxis with INH of dehydration and other complications, and for six months(24). Those showing evidence of use of heparin (IV) or low-molecular-weight active tuberculosis should receive standard therapy heparin (subcutaneously) initially, followed by oral anti-coagulants on the long-term(16,17). There Thrombosis: Children with nephrotic syndrome
is no role for prophylactic treatment with are at risk for venous and, rarely, arterial anticoagulants in patients with hypoalbuminemia thrombosis(16,17). Reduced intravascular volume, TABLE V CLINICAL FEATURES AND MANAGEMENT OF INFECTIONS*
Parenteral: ampicillin andaminoglycoside; orcefotaxime/ceftriaxonefor 7-10 days Systemic: Amphotericinfor 14-21 days *Supplemental stress doses of hydrocortisone or prednisolone are usually necessary Hypertension: This may be detected at the onset of
and revised based on systematic reviews, published nephrotic syndrome or later due to steroid toxicity.
studies and expert opinion of the members of the Therapy is initiated with ACE inhibitors, calcium channel blockers or  adrenergic antagonists, guidelines are intended to familiarize physicians keeping the blood pressure at less than the 90th with principles of management of children with steroid sensitive nephrotic syndrome. Therapy needsto be individualized for each patient and optimal care Hypovolemic shock: This complication can occur
will be achieved by combined inputs of the primary due to unsupervised use of diuretics especially if pediatrician and pediatric nephrologist. Further accompanied by septicemia, diarrhea or vomiting.
revisions of these guidelines, indicating best current The diagnosis is suggested by moderate to severe practice, shall be periodically necessary.
abdominal pain, hypotension, tachycardia, coldextremities and poor capillary refill; hematocrit and Annexure I
blood levels of urea and uric acid are elevated.
Management consists of rapid infusion of normal Members of the Review Committee
saline at a dose of 15-20 mL/kg over 20-30 minutes;this is repeated if clinical features of hypovolemia Kamran Afzal, Jawaharlal Nehru Medical College, persist. Infusion of 5% albumin (10-15 mL/kg) or Aligarh; Indira Agarwal, Christian Medical College 20% albumin (0.5-1 g/kg) may be used in subjects Hospital, Vellore; Vinay Agarwal, Max Hospital, who do not respond despite two boluses of saline.
New Delhi; Uma Ali, Bai Jerbai Wadia Hospital forChildren, Mumbai; Sanjeev Bagai, Rockland Corticosteroid side effects: Prolonged steroid
Hospital, New Delhi; Arvind Bagga, All India therapy may be associated with significant side ef- fects. Patients (if they can understand) and the par- (Convenor); Sushmita Banerjee, Calcutta Medical ents should be explained about the side effects of the Research Institute, Kolkata; Ashima Gulati, All India medications, including increased appetite, impaired Institute of Medical Sciences, Delhi; Sanjeev Gulati, growth, behavioral changes, risk of infections, salt Fortis Hospital, New Delhi; Pankaj Hari, All India and water retention, hypertension and bone deminer- alization. All patients should be monitored for (Secretary); Arpana Iyengar, St. John’s Medical cushingoid features and blood pressure; six-monthly College, Bangalore; OP Jaiswal, Sunder Lal Jain record of height and weight, and yearly evaluation Hospital, New Delhi; Rupesh Jain, Ekta Hospital for for cataract should be done. Patients on prolonged Children, Raipur; M Kanitkar, Armed Forces (>3 months) treatment with steroids should receive Medical College, Poona; Mukta Mantan, Maulana daily supplements of oral calcium (250-500 mg Azad Medical College, New Delhi; Kamini Mehta, daily) and vitamin D (125-250 IU)(19).
Lilavati Hospital & Research Center, Mumbai; Steroids during stress: Patients who have received
Kumud Mehta, Jaslok Hospital & Research Center & high-dose steroids for more than 2 weeks in the past Bai Jerbai Wadia Hospital for Children, Mumbai; BR year are at risk of suppression of the hypothalamo- Nammalwar, Kanchi Kamakoti CHILDS Trust pituitary-adrenal axis. These children require Hospital, Chennai; Amitava Pahari, Apollo supplementation of steroids during surgery, Hospital, Kolkata; Saroj K Patnaik, No.12 Air Force anesthesia or serious infections(26). Corticosteroids Hospital, Gorakhpur; KD Phadke, St. John’s are supplemented, as parenteral hydrocortisone Medical College, Bangalore; PK Pruthi, Sir at a dose of 2-4 mg/kg/day, followed by oral Gangaram Hospital, New Delhi; Abhijeet Saha, prednisolone at 0.3-1 mg/kg/day. This is given for the Government Medical College, Chandigarh; VK duration of stress and then tapered rapidly.
Sairam, Sri Ramchandra Medical College, Chennai;Jayati Sengupta, AMRI Hospital, Kolkata; Prabha CONCLUSIONS
Senguttuvan, Institute of Child Health, Chennai Recommendations on management of nephrotic (Chairperson); Sidharth K Sethi, All India Institute of syndrome, proposed in 2001, have been reexamined Medical Sciences, New Delhi; Mehul Shah, Apollo Hospital, Hyderabad; Jyoti Sharma, Bharti Davin JC, Merkus MP. Levamisole in steroid- Vidyapeeth Medical College, Poona; RN Srivastava, sensitive nephrotic syndrome of childhood: the lost Indraprastha Apollo Hospital, New Delhi; AS paradise? Pediatr Nephrol 2005; 20: 10-14.
Vasudev, Indraprastha Apollo Hospital, New Delhi; Latta K, von Schnakenburg C, Ehrich JHH. A meta- Anil Vasudevan, St. John’s Medical College, analysis of cytotoxic treatment for frequently Bangalore; and M Vijayakumar, Mehta Children’s relapsing nephrotic syndrome in children. Pediatr Kyrieleis HA, Levtchenko EN, Wetzels JF. Long- REFERENCES
term outcome after cyclophosphamide treatment in Consensus Statement on Management of Steroid children with steroid-dependent and frequently Sensitive Nephrotic Syndrome. Indian Pediatric relapsing minimal change nephrotic syndrome. Am Nephrology Group, Indian Academy of Pediatrics.
Cattran DC, Alexopoulos E, Heering P, Hoyer PF, Consensus Statement on Management and Audit Johnston A, Meyrier A, et al. Cyclosporin in Potential for Steroid Responsive Nephrotic idiopathic glomerular disease associated with the Syndrome. Report of a Workshop by the British nephrotic syndrome: Workshop recommendations.
Association for Paediatric Nephrology and Research Unit, Royal College of Physicians. Arch Westhoff TH, Schmidt S, Zidek W, Beige J, van der Giet M. Tacrolimus in steroid-resistant and steroid- Hodson EM, Willis NS, Craig JC. Corticosteroid dependent nephrotic syndrome. Clin Nephrol 2006; therapy for nephrotic syndrome in children.
Cochrane Database System Rev 2007; CD001533.
Afzal K, Bagga A, Menon S, Hari P, Jordan SC.
Primary nephrotic syndrome in children: clinical significance of histopathologic variants of minimal prednisolone for steroid-dependent nephrotic change and of diffuse mesangial hypercellularity. A syndrome. Pediatr Nephrol 2007; 22: 2059- Report of the International Study of Kidney Disease in Children. Kidney Int 1981; 20: 765-771.
Dorresteijn E, Kist-van Holthe J, Levtchenko E, Hogg R, Portman RJ, Milliner D, Lemley KV, Eddy Nauta J, Hop W, Van der Hejden A. Randomized A, Ingelfinger J. Evaluation and management of controlled trial of mycophenolate mofetil versus proteinuria and nephrotic syndrome in children: cyclosporine A in children with frequently Recommendations from a pediatric nephrology relapsing nephrotic syndrome. Pediatr Nephrol Srivastava RN, Bagga A. Nephrotic syndrome. In: albuminuria, risk, assessment, detection and Pediatric Nephrology. 4th edition. New Delhi: elimination (PARADE). Pediatrics 2000; 106: Vogt AB, Avner ED. Nephrotic syndrome. In: Bagga A, Hari P, Srivastava RN. Prolonged versus Nelson Textbook of Pediatrics. 18th edition.
standard initial prednisolone treatment for Kliegman RM, Behrman RE, Jenson HB, Stanton idiopathic nephrotic syndrome. Pediatr Nephrol BF, editors. Philadelphia: WB Saunders; 2007. p Durkan A, Hodson EM, Willis NS, Craig JC. Non- Ruth EM, Kemper MJ, Leumann EP, Laube GF, corticosteroid treatment for nephrotic syndrome in Neuhaus TJ. Children with steroid-sensitive children. Cochrane Database System Rev 2005; nephrotic syndrome come of age: long-term outcome. J Pediatr 2005; 147: 202-207.
Bagga A, Sharma A, Srivastava RN. Levamisole Gulati S, Sharma RK, Gulati K, Singh U, Srivastava therapy in corticosteroid dependent neph- A. Longitudinal followup of bone mineral density rotic syndrome. Pediatr Nephrol 1997; 11: in children with idiopathic nephrotic syndrome.
Nephrol Dial Transpl 2005; 20: 1598-1603.
American Academy of Pediatrics. Immunization in Committee on Infectious Diseases, 27th edn. Elk special clinical circumstances. In: Pickering LK, Baker CJ, Long SS, McMillan JA. Red Book: 2006 Report of the Committee on Infectious Chauhan LS, Arora VK. Management of pediatric IL: American Academy of Pediatrics; 2006. p. 67- Tuberculosis Control Program (RNTCP). Indian American Academy of Pediatrics, Committee on Indian Pediatric Nephrology Group. Evaluation Infectious Diseases. Recommendations for the and management of hypertension. Indian Pediatr prevention of pneumococcal infections, including the use of pneumococcal conjugate vaccine(Prevnar), pneumococcal polysaccharide vaccine Axelrod L. Glucocorticoid therapy. In: Endo- and antibiotic prophylaxis. Pediatrics 2000; 106: crinology. 5th edition. DeGroot LJ, Jameson JL.
Philadelphia: Elsevier Saunders; 2006. p. 2329-2342.
Shah RC, Shah NK, Kukreja S. Pneumococcalvaccines. In: Shah RC, Shah NK, Kukreja S, Writing Committee
editors. IAP Guide Book on Immunization.
Mumbai: 2007. p. 35-36.
Uma Ali, Arvind Bagga, Sushmita Banerjee, American Academy of Pediatrics. Varicella-zoster Madhuri Kanitkar, Kishore D Phadke, Prabha infections. In: Pickering LK, Baker CJ, Long SS, Senguttuvan, Sidharth Sethi and Mehul Shah, on McMillan JA. Red Book: 2006 Report of the behalf of the Indian Pediatric Nephrology Group.

Source: http://www.iapindia.org/files/IAP%20Guidelines/nephrotic%20syndrome%20I%20guidelines.pdf