Materiały źródłowe

• #1 Nephrotic syndrome in children

  https://www.nhs.uk/conditions/nephrotic-syndrome/

  The symptoms of nephrotic syndrome can usually be controlled with steroid medication. […] Most children with nephrotic syndrome respond well to steroids and are not at risk of kidney failure. […] The main treatment for nephrotic syndrome is steroids, but additional treatments may also be used if a child develops significant side effects. […] Children diagnosed with nephrotic syndrome for the first time are normally prescribed at least a 4-week course of the steroid medicine prednisolone, followed by a smaller dose every other day for 4 more weeks. This stops protein leaking from your child’s kidneys into their urine. […] Most children respond well to treatment with prednisolone, with the protein often disappearing from their urine and the swelling going down within a few weeks. This period is known as remission.

• #1 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  2. Kidney biopsy […] A kidney biopsy is usually not indicated in the instance of a prompt response to steroid treatment and in the absence of 2 or more relapses in the first year of treatment. However, a biopsy may be indicated if the patient is steroid-resistant, has an atypical clinical course, or is over the age of 12 years at initial presentation. […] 3. Treatment of first episode […] Initial treatment with prednisone or prednisolone should not extend beyond 12 weeks; evidence is insufficient to choose between 4 weeks at a full dose followed by 4 weeks of alternate-day dosing (total 8 weeks) or 6 weeks at full dose followed by 6 weeks of alternate-day dosing (total 12 weeks). […] 4. Treatment of relapse […] Prednisone as a single daily dose of 60 mg/m2 or 2 mg/kg (maximum 60 mg/d) until complete remission for at least 3 days. After achieving complete remission, reduce prednisone to 40 mg/m2 or 1.5 mg/kg (maximum 50 mg) on alternate days for at least 4 weeks.

• #1 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  A kidney biopsy and genetic testing during the initial workup are not advised for children presenting with typical features and age 1 year. However either or both is recommended for infants between 3 and 12 months of age. This is a „grade B weak recommendation.” […] The indications for referral to a pediatric nephrologist include patients with challenging management dilemmas, such as those with initial steroid resistance, steroid sensitive but a late responder, a frequent-relapsing or steroid-dependent nephrotic syndrome. […] Treatment of the initial episode with either an 8 week or 12 week course of steroid (prednisone or methylprednisolone). […] The two alternative regimens are either daily steroid for 4 weeks followed by alternate-day administration for 4 weeks or for 6 weeks of daily steroid followed by alternate-day steroid for 6 weeks. Daily prednisone (or methylprednisolone) dose is 60 mg/m2 or 2 mg/kg/day (maximum, 60 mg/day). The alternate-day dose is 40 mg/m2 or 1.5 mg/kg (maximum, 40 mg). Steroid is given as a single dose in the morning. Treatment at the conclusion of 6 or 8 weeks is stopped without tapering regardless of when cessation of proteinuria has occurred. This dosing schedule is rated as a „grade A, strong recommendation.”

• #1 Nephrotic syndrome

  https://www.rch.org.au/clinicalguide/guideline_index/nephrotic_syndrome/

  Nephrotic syndrome is a clinical disorder characterised by heavy proteinuria, hypoalbuminaemia and oedema […] The key acute complications are hypovolemia, infection and thrombosis […] 80-90% of cases of Idiopathic Nephrotic Syndrome (INS) are steroid sensitive and respond to initial therapy […] 1. Admit to hospital on first presentation […] 2. If the child is profoundly ill or appears to have sepsis treat accordingly […] 3. Manage oedema […] Albumin and Furosemide Indications include: intravascular volume depletion, severe or symptomatic oedema […] 4. Steroid therapy […] Prednisolone: to induce remission, followed by a slow wean to reduce risk of relapse […] 5. Prophylaxis against complications […] A relapse is defined as proteinuria 3+ or 4+ for 3 consecutive days, and should prompt re-introduction of full dose prednisolone […] The total time of weaning regimen can be shortened if the child relapses infrequently […] It is important to convey that, though their child will likely respond to therapy, they will likely have relapses (80% chance) […] The most common relapse trigger is intercurrent infection.

• #1 Pediatric Nephrotic Syndrome Treatment & Management: Approach Considerations, Corticosteroid Therapy, Diuretic Therapy

  https://emedicine.medscape.com/article/982920-treatment

  Diuretic therapy may be beneficial, particularly in children with symptomatic edema. […] Antihypertensive therapy should be given when hypertension is present and particularly if it persists, but caution should be exercised. […] Home monitoring of urine protein and, if in remission, the possible emergence of proteinuria, is an important aspect of management. […] Frequently relapsing nephrotic syndrome (FRNS) is defined as steroid-sensitive nephrotic syndrome (SSNS) with 2 or more relapses within 6 months, or 4 or more relapses within a 12-month period. […] The current KDIGO guidelines recommend that in FRNS and SDNS, prednisone be prescribed at 2 mg/kg/day (60 mg/m2/day) as a single morning dose until the patient has been free of proteinuria for at least 3 days. […] Cyclophosphamide (CYP) is the predominant alkylating agent used in the treatment of FRNS and SDNS.

• #1 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  5. Introduction of glucocorticoid sparing agent […] For children with frequent relapses who develop serious glucocorticoid related adverse events, and for all children with steroid-dependent nephrotic syndrome, a glucocorticoid sparing agent should be prescribed. […] 6. Choice of glucocorticoid sparing agent: the patient […] Choosing the most appropriate steroid-sparing agent between oral cyclophosphamide, levamisole, mycophenolate mofetil, rituximab, and a calcineurin inhibitor depends on specific patient related issues such as resources, compliance, potential for adverse effects, and patient preferences. […] 7. Choice of glucocorticoid sparing agent: the disease […] Among glucocorticoid sparing agents for steroid-sensitive nephrotic syndrome, oral cyclophosphamide or levamisole (although the latter is not available in the United States) may be preferable in frequent relapsing forms. Mycophenlolate mofetil, rituximab, calcineurin inhibitors may be preferable in steroid-dependent forms of disease.

• #1 Pediatric Nephrotic Syndrome Treatment & Management: Approach Considerations, Corticosteroid Therapy, Diuretic Therapy

  https://emedicine.medscape.com/article/982920-treatment

  Calcineurin inhibitors (eg, cyclosporin A [CSA] and tacrolimus [TAC]) are useful steroid-sparing agents. […] Mycophenolate mofetil (MMF) has been increasingly used in FRNS and SDNS because it has fewer adverse effects than CYP, CSA, and TAC. […] Rituximab may be considered in children with SDNS or FRNS in whom other treatments have failed or those with cumulative toxicity of other steroid-sparing agents. […] Genetic considerations play an important role in the treatment of SRNS and FSGS; approximately 30% of children with SRNS may have a single-gene cause of their disease. […] Plasmapheresis has been attempted in patients with treatment-resistant FSGS and SRNS. […] Routine use of plasmapheresis or immunoadsorption in SRNS and FSGS cannot be recommended at this time.

• #1 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  A steroid-sparing agent or low-dose steroid either as alternate-day or daily is recommended as second-line treatment as maintenance for frequently relapsing or steroid-dependent nephrotic syndrome. […] Discontinuation may be considered after at least 12 months. Steroid-sparing medications include the following: Calcineurin inhibitors (cyclosporine A or tacrolimus), Cyclophosphamide, Levamisole (not available in the United States), Mycophenolate mofetil, Rituximab. […] The strength of recommendation for the use of cyclosporin A is „grade B, moderate.” […] The starting dose for tacrolimus is 0.1-0.2 mg/kg/d (maximum, 10 mg) in 2 doses every 12 hours to achieve trough blood levels of 3-7 ng/mL. The strength of recommendation for the use of tacrolimus is „grade C, moderate.” […] Recommend avoiding prolonged use of calcineurin inhibitors for more than a total of 2-3 years but before further continuation beyond 2-3 years, a kidney biopsy be considered. […] The recommended starting dose for mycophenolate mofetil is 1200 mg/m2 BSA (maximum, 300 mg) divided into two doses every 12 hours. Therapy with this medication should begin while the child is still receiving alternate-day steroids. The latter can be tapered and discontinued within 6-12 weeks. If mycophenolate mofetil has been effective in preventing relapses for at least 12 months, tapering and discontinuing over 3-6 months may be considered.

• #1 Pediatric Nephrotic Syndrome Treatment & Management: Approach Considerations, Corticosteroid Therapy, Diuretic Therapy

  https://emedicine.medscape.com/article/982920-treatment

  A trial of corticosteroids is the first step in the treatment of idiopathic nephrotic syndrome (INS) in patients for whom a kidney biopsy is not initially indicated. […] Kidney biopsy should be performed prior to any immunosuppressive treatment, including steroids, in patients who meet one or more of the following criteria: […] The treatment of steroid-sensitive INS, steroid-dependent and frequently relapsing INS, steroid-resistant nephrotic syndrome (SRNS), and FSGS are discussed in detail below. […] A delay in initiating steroid treatment increases the risk of steroid resistance and chronic kidney disease. […] Not all edema needs to be treated, but when it becomes clinically significant and symptomatic, then treatment of the positive sodium balance using a loop diuretic is appropriate.

• #1 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  8. Genetic testing […] For steroid-resistant nephrotic syndrome, consider the possibility of a genetic cause where immunosuppression may not be useful. Genetic testing performed by experts should be rapidly implemented, particularly in infantile forms, if there is a positive family history of kidney disease and/or the patient has syndromic features. […] 9. RAAS blockade for SRNS […] In children with steroid-resistant nephrotic syndrome, a renin-angiotensin-aldosterone system inhibitor should be started with careful evaluation of volume depletion to minimize the risk of AKI. […] 10. Calcineurin inhibitor for SRNS […] Recommend using cyclosporine or tacrolimus as initial second line therapy. […] The International Pediatric Nephrology Association issued clinical practice guidelines for the diagnosis and management of children with steroid-sensitive nephrotic syndrome. […] Recommendations were derived from a comprehensive review of the literature and rating the strength of evidence supporting a recommendation using four levels, ranging from strong (grade A) to weak (grade D). Certain clinical characteristics defined by the response to steroid treatment, including steroid-sensitive, steroid-resistant, steroid late responder, frequently relapsing, steroid-dependent, and other responses to intervention largely recapitulate that of the KDIGO practice guidelines mentioned above. Some will be highlighted as follows:

• #1 Symptomatic management of nephrotic syndrome in children – UpToDate

  https://www.uptodate.com/contents/symptomatic-management-of-nephrotic-syndrome-in-children

  Symptomatic management of nephrotic syndrome in children […] The majority of children with NS will respond to steroid therapy. However, symptomatic treatment is important in the early course of therapy as response to steroid therapy may take several weeks. […] Symptomatic treatment also becomes the mainstay of therapy in children who fail to respond to steroids, especially in those with genetic mutations that cause their NS. […] The symptomatic management of NS in children will be reviewed here. Specific treatment of NS in children is discussed separately. […] Edema is treated by salt restriction because renal retention of sodium is one of two principal mechanisms that lead to edema in the NS. Dietary salt intake should be restricted to less than 2 to 3 mEq/kg per day.

• #1 Nephrotic syndrome in children

  https://www.nhs.uk/conditions/nephrotic-syndrome/

  Tablets that help you pee more (diuretics) may also be given to help reduce the fluid build-up. […] Penicillin is an antibiotic, and may be prescribed during relapses to reduce the chances of an infection. […] You may be advised to reduce the amount of salt in your child’s diet to prevent further water retention and oedema. […] Children with nephrotic syndrome are advised to have the pneumococcal vaccine. […] Other medications may be used alongside or in place of steroids if your child’s remission cannot be maintained with steroids or they experience significant side effects. […] If your child’s symptoms are severe, they may be admitted to hospital to receive albumin infusions. […] If your child has congenital nephrotic syndrome, they’ll need frequent albumin infusions to help them grow and develop normally. This often requires a stay in hospital. […] In some cases, your doctor may recommend surgery to remove 1 or both of your child’s kidneys. […] If both kidneys do need to be removed, your child will need dialysis.

• #1 The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I – Diagnosis and treatment of the first episode and the first relapse | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-017-0356-x

  The treatment of nephrotic edema in children, regardless of its severity, involves sodium restriction, diuretics and albumin infusions. […] We suggest that mild edema be managed with salt and fluid restriction only. […] We suggest that moderate edema be treated with a loop diuretic, with the addition of a potassium-sparing diuretic in the case of prolonged therapy. […] We suggest the co-administration of a thiazide diuretic in cases of severe edema unresponsive to oral or i.v. loop diuretics. […] We suggest albumin infusions in patients with severe edema unresponsive to oral or i.v. loop diuretics. […] We do not recommend administering either i.v. immunoglobulin or prophylactic antibiotics to children. […] We suggest that rapid diagnosis and antibiotic treatment of infections are the most effective interventions.

• #1 Nephrotic syndrome treatment | infoKID

  https://infokid.org.uk/conditions/nephrotic-syndrome/nephrotic-syndrome-treatment/

  When your child first develops nephrotic syndrome, he or she probably needs to stay in hospital for a few days to be carefully monitored. […] The first treatment for nephrotic syndrome is a steroid medicine called prednisolone. […] Your doctor will let you know how often and for how long to give prednisolone. […] It is important that you follow your doctors instructions about when and how much to give. […] It is important that your child has the vaccines (immunisations) that he or she needs to reduce the risk of some diseases. […] Children taking steroids are more likely to get infections. […] If your child does get an infection, he or she may need to take an antibiotic medicine, which kills the germs that cause infection. […] Eating too much salt can make the oedema worse and increase the risk of developing hypertension (high blood pressure), especially while on steroids. […] Children being treated for nephrotic syndrome are more likely to get infections. […] Very occasionally, children need to take diuretics, medicines that help reduce swelling in the body (oedema).

• #1 The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I – Diagnosis and treatment of the first episode and the first relapse | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-017-0356-x

  We suggest the use of oral acyclovir following exposure to chickenpox in non-immune patients. […] We do not recommend the routine use of prophylactic PPIs in combination with steroid therapy in NS. […] We do not suggest calcium and vitamin D supplementation in children at first episode or in SSNS unless vitamin D deficiency has been predicted or demonstrated. […] We do not recommend the use of lipid-lowering treatments at the onset of NS. […] We suggest that kidney biopsy be performed after the first four weeks of therapy; the continuation of steroid treatment depends on the histological findings.

• #1 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  Anticoagulation has been suggested to prevent thromboembolic complications, but its role in primary prevention is not proven. Hypolipidemic agents could be used. In patients with secondary nephrotic syndrome, such as that secondary to diabetic nephropathy, some medications are widely used to reduce proteinuria, such as angiotensin-converting enzyme (ACE) inhibitors and/or angiotensin 2 receptor blockers. By reducing proteinuria, these drugs will lead to reduced intraglomerular pressure causing a reduction in systemic blood pressure. […] The diet in patients with nephrotic syndrome is aimed to provide sufficient caloric and protein (1 g/kg/d) intake. Supplemental dietary proteins are of no proven value. A low-salt diet helps limit fluid retention and edema. […] The patient’s edema and proteinuria define the adjustment of diuretics and angiotensin antagonists. Follow-up in the nephrotic syndrome also involves immunizations and monitoring for steroid toxicity. Routine immunizations should be deferred until there are no relapses and the patient has been off immunosuppressants for at least three months.

• #1 Nephrotic Syndrome: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5989-nephrotic-syndrome

  In some cases, your healthcare provider may recommend medications that help prevent blood clots (anticoagulants or blood thinners). […] Diet and nutrition don’t cause or prevent nephrotic syndrome in adults. However, if you have nephrotic syndrome, changes to your diet may help relieve some of your symptoms. […] The best way to manage your symptoms is to take your medications as prescribed by your healthcare provider.

• #1 Diagnosis and Management of Nephrotic Syndrome in Adults | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0315/p479.html

  The decision to treat with anticoagulants should be made individually. […] Immunosuppressive therapy for NS secondary to systemic lupus erythematosus is highly effective and supported by multiple studies, and may lead to partial or complete remission in patients with minimal change disease or primary focal segmental glomerulosclerosis.

• #1 Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-020-00384-1

  Congenital nephrotic syndrome (CNS) is a heterogeneous group of disorders characterized by nephrotic-range proteinuria, hypoalbuminaemia and oedema, which manifest in utero or during the first 3 months of life. […] Management of CNS is very challenging because patients are prone to severe complications, such as haemodynamic compromise, infections, thromboses, impaired growth and kidney failure. In this consensus statement, experts from the European Reference Network for Kidney Diseases (ERKNet) and the European Society for Paediatric Nephrology (ESPN) summarize the current evidence and present recommendations for the management of CNS, including the use of renin-angiotensin system inhibitors, diuretics, anticoagulation and infection prophylaxis. […] Therapeutic management should be adapted to the clinical severity of the condition with the aim of maintaining intravascular euvolaemia and adequate nutrition, while preventing complications and preserving central and peripheral vessels.

• #1 Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-020-00384-1

  We do not recommend performing routine early nephrectomies but suggest that they are considered in patients with severe complications despite optimal conservative treatment, and before transplantation in patients with persisting nephrotic syndrome and/or a WT1-dominant pathogenic variant. […] Management should therefore be adapted to the clinical severity of the condition with the aim of maintaining intravascular euvolaemia and adequate nutrition, as well as preventing complications. […] We recommend rapid referral of children with CNS to a specialized paediatric nephrology unit due to the complexity of the disease and fluid management. […] We recommend avoiding intravenous fluids and saline. Oral fluid intake should be concentrated if necessary to avoid marked oedema. […] We recommend using albumin infusions based on clinical indicators of hypovolaemia (including oliguria, acute kidney injury, prolonged capillary refill time, tachycardia, hypotension and abdominal discomfort) or upon failure to thrive.

• #1 Nutritional Management of Idiopathic Nephrotic Syndrome in Pediatric Age

  https://www.mdpi.com/2076-3271/11/3/47

  The effective management of relapses represents a great challenge. […] Therefore, diet can play an important role in narrowing and preventing complications of NS: making dietary changes is crucial in replacing nutrient losses and correcting metabolic abnormalities, but also in avoiding renal disease progression. […] Standardized diet recommendations are needed to manage the condition and the side effects of steroid treatment. […] The aim of this article is to deepen the state of the art knowledge of nutritional management of these patients, who present unique nutrition support needs and require individualized nutritional choices, not only during the acute phase of the disease but also during the periods of remission, to prevent the progression of kidney damage.

• #1 Nephrotic Syndrome in Children | National Kidney Foundation

  https://www.kidney.org/kidney-topics/nephrotic-syndrome-children

  About 80% of children with minimal change disease between 2 and 9 years of age will respond to treatment within 3 to 4 weeks. This means protein in the urine and swelling will go away while taking the medication. When protein in the urine goes away completely, this is called remission. […] A low salt (sodium) diet to help limit the swelling in the body is often recommended for children with nephrotic syndrome. This is most important when there is protein leaking into the urine. A dietitian who works with the kidney doctor can provide recommendations.

• #1 Treatment of idiopathic nephrotic syndrome in children | Boletín Médico del Hospital Infantil de México (English Edition)

  https://www.elsevier.es/pt-revista-boletin-medico-del-hospital-infantil-201-articulo-treatment-idiopathic-nephrotic-syndrome-in-X2444340914741917

  Rituximab is a murine-human monoclonal antibody directed against the CD20 antigen present in pre-B and B lymphocytes and has been mainly developed for the treatment of blood-related cancers. […] During the period of nephrosis, it is advisable to restrict the addition of salt to foods. […] In children with steroid-resistant INS, the finding of FSGS on histological study of the renal biopsy is common. […] Treatment of patients with steroid-resistant INS incorporates the aspects of conservative management and diverse therapeutic schemes directed at controlling proteinuria and preservation of renal function. […] In patients with INS with FSGS and who respond to initial steroid treatment, it has been observed that 50% could remain in complete remission, 25% have subsequent partial remission and another 25% progress to late steroid resistance.

• #1 Nephrotic Syndrome (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/nephrotic-syndrome.html

  Most of the time, nephrotic syndrome goes away with medicine, and kids outgrow it by the time they’re teens. […] Nephrotic syndrome is almost always treatable, but the treatment depends on the cause. Kids with nephrotic syndrome usually are treated by a nephrologist (a doctor who specializes in kidney problems). […] To treat minimal change disease, the doctor will prescribe: Prednisone. This medicine works well to treat nephrotic syndrome, but can sometimes have side effects. The doctor may prescribe other medicines to ease or prevent them. […] A diuretic. This makes a child pee more often, which can help control the swelling until protein levels return to normal. […] Kids should eat a low-salt diet to help ease swelling. A low-protein diet isn’t necessary. For other dietary advice, talk to a doctor or dietitian. […] The sooner treatment for nephrotic condition starts, the better. If your child shows any signs of the condition, call your doctor so it can get checked out right away.

• #1 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  A detailed assessment is necessary before starting corticosteroids. The patient’s height, weight, and blood pressure should be monitored. Regular weight record helps in monitoring the decrease or increase of edema. Physical examination is carried out to detect infections and underlying systemic disorders. […] Specific treatment of nephrotic syndrome is dependent on its cause. Therefore, management varies between adult and pediatric populations. Kidney Disease Improving Global Outcomes (KDIGO) issued guidance in 2012 that included recommendations for treating nephrotic syndrome. […] Corticosteroids are mainly used for children with idiopathic nephrotic syndrome. Alternative immunosuppressive agents are often necessary for children with frequently relapsing or steroid-dependent nephrotic syndrome. Examples of these drugs include cyclophosphamide, mycophenolate mofetil (MMF), calcineurin inhibitors, and levamisole. In cases of steroid-resistant nephrotic syndrome, the first-line choice is calcineurin inhibitors, and if there is no response, then agents such as MMF or prolonged and/or intravenous pulse corticosteroids could be used.

• #1 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  Recommend using rituximab as a steroid-sparing agent if there has not been effective relapse-free maintenance with at least one other steroid-sparing agent at adequate dose. […] The recommended dose is 375 mg/m2 for each infusion (maximum, 1000 mg). The number of infusions may be between 1 and 4. B cell depletion may be monitored by CD19(+) cell counts at baseline and at 7 days post-infusion. The target for B cell depletion is an absolute CD19 cell count 5 cells/mm3 or 1% of total lymphocytes. Also recommend monitoring IgG levels at baseline and periodically thereafter for hypogammaglobulinemia. Pretreatment with acetaminophen, antihistamines and/or steroids is recommended to prevent an infusion reaction. After completion of a course of one or more infusions, steroid and other steroid-sparing agents are tapered within 2-3 months. Reconstitution of B cells is defined as a total B cell count 5/mm3.

• #1 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  Rituximab, an anti-B cell antibody, has proved to be an effective steroid-sparing agent in the pediatric population. However, rituximab may fail to achieve drug-free remission in children dependent on both calcineurin inhibitors and steroids. Rituximab may also have a role in children with steroid-resistant disease. […] Treatment varies by etiology, as follows: Minimal change nephropathy in adults usually responds to prednisone. In lupus nephritis, prednisone combined with cyclophosphamide or mycophenolate mofetil induces remission. Secondary amyloidosis with nephrotic syndrome will improve with the anti-inflammatory management of the primary disease. […] Hospitalization is not usually necessary with close outpatient follow-up care and good parental and patient education. Hospitalization becomes helpful if any of the following are present: Generalized edema severe enough to result in respiratory distress, tense scrotal or labial edema, complications such as bacterial peritonitis, pneumonia, sepsis, or thromboembolism, failure to thrive, uncertainty regarding the compliance of patient or family with treatment.

• #1 Nephrotic Syndrome (Kidney Disease) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/nephrotic-syndrome-kidney-disease

  Nephrotic syndrome always affects both kidneys. […] MCD is much more common in children, and likely to respond to therapy. […] Most children with NS outgrow it by young adulthood. […] Within a few days of diagnosis, your child will most likely start on a course of steroid treatment. […] If your child has FSGS and responds to new medication(s), its likely that your child will never be taken off of the medication, since he is considered to be much more likely to relapse. […] If your child’s FSGS progresses to end-stage renal disease, your child may need dialysis and/or a kidney transplantation. […] The vast majority of kids in the United States with nephrotic syndrome outgrow it by the time they reach early adulthood. […] For between 85 and 90 percent of kids with nephrotic syndromethose with minimal change diseasethe outlook is quite good. […] If your child has focal sclerosis (FSGS), nephrotic syndrome is harder to treat. […] If your child’s NS responds to the new medication(s), and his doctors suspect that he has minimal change disease (MCD) his doctors will use the medication to treat the NS until it goes into remission.

• #1 Pediatric Nephrotic Syndrome – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/nephrotic-syndrome

  During the first episode of nephrotic syndrome, your child may need to stay in the hospital. […] Other treatment options may include: […] Medicines may be used to treat initial symptoms and relapses. […] Changing the diet for a child with nephrotic syndrome may include limiting salt and fluids. […] In rare cases, a child may develop kidney failure and need dialysis. Dialysis is a procedure that filters waste and extra fluid from the blood. […] Treatment will depend on your child’s symptoms, age and general health. It will also depend on how severe the condition is. […] Medicines may be used to treat initial symptoms and relapses. These may include: […] Corticosteroids […] Immunosuppressives […] Diuretics to reduce the swelling […] Medicine to treat high blood pressure. […] The diet for a child with nephrotic syndrome may include limiting salt and fluids. This may help to regulate your child’s fluid balance. […] Your child’s healthcare provider will talk with you about how much salt and fluids your child should have each day. […] In rare cases, a child may develop kidney failure and need dialysis. Dialysis is a procedure that filters waste and extra fluid from the blood.

• #1 Nephrotic syndrome in children: Exploring new treatments | Northwell Health

  https://feinstein.northwell.edu/news/the-latest/nephrotic-syndrome-in-children-studying-new-treatments

  Researchers seek to better understand how best to treat nephrotic syndrome in children. […] Led by Christine B. Sethna, MD, division director of pediatric nephrology at Cohen Children’s Medical Center and associate professor at the Donald and Barbara Zucker School of Medicine at Hofstra/Northwell and the Feinstein Institute, the $1.03 million grant was awarded last year and will be used to fund a new clinical trial for children with nephrotic syndrome using vagus nerve stimulation. […] This research plans to study the mechanism of action by stimulating the vagus nerve which can be activated non-invasively on the ear to have immunomodulatory effects mediated by the inflammatory reflex and spleen. […] This funding will allow us to study, and ultimately help, children living with nephrotic syndrome and better understand how the condition can best be treated without negative side effects that steroids and medications could potentially leave, said Dr. Sethna, principal investigator on the studies. […] Dr. Sethna’s research into vagus nerve stimulation to reverse the inflammation is an important step towards finding alternate therapies.

• #1 Can children with nephrotic syndrome receive less aggressive steroid treatment? – International Society of Nephrology

  https://www.theisn.org/blog/2021/02/10/can-children-with-nephrotic-syndrome-receive-less-aggressive-steroid-treatment/

  Children who develop nephrotic syndrome are usually treated with corticosteroids (normally prednisone or prednisolone) over prolonged periods and at doses recommended by the International Study of Kidney Diseases in Children almost 50 years ago. […] Dr. Aditi Sinha, Associate Professor of Pediatrics at the All India Institute of Medical Sciences, Delhi, will argue that emerging evidence suggests that it may be possible to treat children with reduced or shortened courses of corticosteroids, which could help to reduce the short- and long-term adverse effects of the drug. However, further well-conducted, large randomized controlled trials are needed. […] If nephrotic syndrome is treated with corticosteroid therapy when it first appears, it can postpone relapse and lessen the severity of its subsequent course.

• #1 Tackling the Challenges of Nephrotic Syndrome Treatment

  https://www.childrens.com/research-innovation/research-library/research-details/tackling-the-challenges-of-nephrotic-syndrome-treatment

  Although corticosteroids, such as prednisone, are effective for most patients, the adverse effects of long-term corticosteroid use are challenging and potentially long-lasting: weight gain, emotional and behavioral changes, and an increased risk of diabetes, bone abnormalities and osteoporosis. Long-term immunosuppression can also increase the risk of infections. […] And then theres the small percentage of children who have steroid-resistant NS and dont respond to any treatment. Many will progress to end-stage renal disease that requires dialysis and transplant. […] Having multicenter consortia like NEPTUNE and CureGN conducting observational trials has really changed the field, Dr. Brown says. It brings people together to talk about NS and brainstorm solutions. Were making progress toward a day when we can offer personalized and more effective therapies to NS patients.

• #1 Nephrotic Syndrome: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5989-nephrotic-syndrome

  Nephrotic syndrome treatment includes addressing the underlying cause and taking steps to reduce high blood pressure, high cholesterol, swelling and infection risks. Treatment usually includes medications and changes to your diet. […] No, nephrotic syndrome isn’t curable. However, nephrotic syndrome often goes away in children once they reach their late teenage years or early 20s. […] Some blood pressure medications can slow down a kidney disease that causes nephrotic syndrome, including: Angiotensin-converting enzyme (ACE) inhibitors and Angiotensin receptor blockers (ARBs). […] To lower your cholesterol, your healthcare provider may recommend statin medications. […] If you have nephrotic syndrome, it’s also a good idea to get a yearly influenza (flu) vaccine and a pneumococcal (new-ma-cah-cole) vaccine.

• #1 Strategies to Improve Quality of Life in Children with Nephrotic Syndrome – Indian Journal of Nephrology

  https://indianjnephrol.org/strategies-to-improve-quality-of-life-in-children-with-nephrotic-syndrome/

  Nephrotic syndrome (NS) is one of the most common childhood kidney problems. Although most children do go into remission and end-stage kidney disease is rare, the relapsing-remitting nature of the disease increases the complexities of management. […] The treatment spectrum of children with nephrotic syndrome and factors affecting quality of life. […] With robust evidence supporting decreased QoL among these children, it is imperative to focus on ways to improve the situation. Understanding and addressing the root causes not only improves QoL but also impacts clinical care and leads to better overall outcomes. […] The first step to combating these challenges is raising awareness of the issue and implementing a comprehensive care plan encompassing medical and nonmedical strategies. […] Medical strategies include home monitoring of urine protein and maintaining a nephrotic diary, knowledge of potential complications and drug adverse effects, early attendance in case of relapse or complication, appropriate diet, steroid minimization, family education, early use of steroid-sparing agents, and up-to-date immunization.

• #2 Pediatric Nephrotic Syndrome Treatment & Management: Approach Considerations, Corticosteroid Therapy, Diuretic Therapy

  https://emedicine.medscape.com/article/982920-treatment

  A trial of corticosteroids is the first step in the treatment of idiopathic nephrotic syndrome (INS) in patients for whom a kidney biopsy is not initially indicated. […] Kidney biopsy should be performed prior to any immunosuppressive treatment, including steroids, in patients who meet one or more of the following criteria: […] The treatment of steroid-sensitive INS, steroid-dependent and frequently relapsing INS, steroid-resistant nephrotic syndrome (SRNS), and FSGS are discussed in detail below. […] A delay in initiating steroid treatment increases the risk of steroid resistance and chronic kidney disease. […] Not all edema needs to be treated, but when it becomes clinically significant and symptomatic, then treatment of the positive sodium balance using a loop diuretic is appropriate.

• #2 Nephrotic syndrome

  https://www.rch.org.au/clinicalguide/guideline_index/nephrotic_syndrome/

  Nephrotic syndrome is a clinical disorder characterised by heavy proteinuria, hypoalbuminaemia and oedema […] The key acute complications are hypovolemia, infection and thrombosis […] 80-90% of cases of Idiopathic Nephrotic Syndrome (INS) are steroid sensitive and respond to initial therapy […] 1. Admit to hospital on first presentation […] 2. If the child is profoundly ill or appears to have sepsis treat accordingly […] 3. Manage oedema […] Albumin and Furosemide Indications include: intravascular volume depletion, severe or symptomatic oedema […] 4. Steroid therapy […] Prednisolone: to induce remission, followed by a slow wean to reduce risk of relapse […] 5. Prophylaxis against complications […] A relapse is defined as proteinuria 3+ or 4+ for 3 consecutive days, and should prompt re-introduction of full dose prednisolone […] The total time of weaning regimen can be shortened if the child relapses infrequently […] It is important to convey that, though their child will likely respond to therapy, they will likely have relapses (80% chance) […] The most common relapse trigger is intercurrent infection.

• #2 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  A kidney biopsy and genetic testing during the initial workup are not advised for children presenting with typical features and age 1 year. However either or both is recommended for infants between 3 and 12 months of age. This is a „grade B weak recommendation.” […] The indications for referral to a pediatric nephrologist include patients with challenging management dilemmas, such as those with initial steroid resistance, steroid sensitive but a late responder, a frequent-relapsing or steroid-dependent nephrotic syndrome. […] Treatment of the initial episode with either an 8 week or 12 week course of steroid (prednisone or methylprednisolone). […] The two alternative regimens are either daily steroid for 4 weeks followed by alternate-day administration for 4 weeks or for 6 weeks of daily steroid followed by alternate-day steroid for 6 weeks. Daily prednisone (or methylprednisolone) dose is 60 mg/m2 or 2 mg/kg/day (maximum, 60 mg/day). The alternate-day dose is 40 mg/m2 or 1.5 mg/kg (maximum, 40 mg). Steroid is given as a single dose in the morning. Treatment at the conclusion of 6 or 8 weeks is stopped without tapering regardless of when cessation of proteinuria has occurred. This dosing schedule is rated as a „grade A, strong recommendation.”

• #2 Revised guidelines for management of steroid-sensitive nephrotic syndrome – Indian Journal of Nephrology

  https://indianjnephrol.org/revised-guidelines-for-management-of-steroid-sensitive-nephrotic-syndrome/

  In 2001, the Indian Pediatric Nephrology Group formulated guidelines for management of patients with steroid-sensitive nephrotic syndrome. […] The need for adequate corticosteroid therapy at the initial episode is emphasized. […] It is proposed that patients with frequently relapsing nephrotic syndrome should, at the first instance, be treated with longterm, alternate-day prednisolone. […] The indications for use of alternative immunosuppressive agents, including levamisole, cyclophosphamide, mycophenolate mofetil, and cyclosporin are outlined. […] On the basis of current evidence and opinion, the Group recommends that the initial episode of nephrotic syndrome be treated with prednisolone at a dose of 2 mg/kg per day (maximum 60 mg in single or divided doses) for 6 weeks, followed by 1.5 mg/kg (maximum 40 mg) as a single morning dose on alternate days for the next 6 weeks; therapy is then discontinued.

• #2

  https://www.healthychildren.org/English/health-issues/conditions/genitourinary-tract/Pages/nephrotic-syndrome-in-children.aspx

  Corticosteroids (medicines called prednisone or prednisolone) are the main treatment for nephrotic syndrome due to minimal change disease. […] If protein in the urine is severe and blood albumin levels are very low, your doctor may give albumin by an intravenous (IV) injection directly into the bloodstream. In some cases, medicines called diuretics (water pills) may be needed to help the kidneys remove excess fluid. […] About 80% of children with minimal change disease between 2 and 9 years of age will respond to treatment within 3 to 4 weeks. This means protein in the urine and swelling will go away while taking the medication. […] However, many children will have relapse (return) of protein in their urine. Some children will rarely have relapses while others have frequent relapses. Relapses require another course of prednisone, possibly in addition to other medications.

• #2 Tackling the Challenges of Nephrotic Syndrome Treatment

  https://www.childrens.com/research-innovation/research-library/research-details/tackling-the-challenges-of-nephrotic-syndrome-treatment

  Nephrotic syndrome (NS) is one of the most challenging conditions in pediatric nephrology. We recognize how difficult NS treatment can be for children and their parents. […] After a diagnosis through blood and urine tests, most children start a three-month course of corticosteroids, usually prednisone. Up to 80 percent of children achieve remission after four weeks of therapy. However, 60 to 70 percent of those kids will eventually relapse. […] If children are frequently relapsing, immunosuppressive therapies like tacrolimus or mycophenolate mofetil are the next option. These treatments can prolong the time between relapses, rather than actively putting patients into remission, but need to be taken every day for several years. If these medications are not effective, the next step for some patients is to use a stronger IV immunosuppressive medication called rituximab.

• #2 Idiopathic Nephrotic Syndrome Management in Children (201) | Right Decisions

  https://rightdecisions.scot.nhs.uk/shared-content/ggc-clinical-guidelines/paediatrics/kidney-diseases/idiopathic-nephrotic-syndrome-management-in-children-201/

  Prednisolone treatment should be restarted once a relapse has been diagnosed. Urine should be tested daily until negative/trace for 3 days in a row. […] If successful, treatment can continue for up to 3 years. […] Levamisole may be beneficial for children with frequent relapses. It is less useful in those who are steroid dependent. […] Cyclophosphamide can induce longer lasting remissions in children with frequently relapsing steroid sensitive NS. […] Ciclosporin (Neoral) is a steroid sparing agent and has been shown to be effective in NS. […] Tacrolimus is a steroid sparing agent and has been shown to be effective in NS, it is a calcineurin inhibitor and may be preferred to Ciclosporin due to the lack of cosmetic adverse effects. […] Mycophenolate mofetil has been used successfully as a second-line steroid sparing agent in frequently relapsing and steroid dependent NS. […] Rituximab is a chimeric monoclonal antibody directly linked to the depletion of CD20 B cells. It has been used successfully to resolve cases of difficult nephrotic syndrome when other treatment modalities have failed.

• #2 Treatment Guidelines for Steroid-Sensitive Idiopathic Nephrotic Syndrome in Childrenlogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/pa201304240000004/2013/04/24/treatment-guidelines-steroid-sensitive

  Steroid-sensitive nephrotic syndrome during childhood is frequently a chronic condition. […] Although 80% of patients respond to treatment with corticosteroids, numerous treatment regimens are practiced worldwide. […] As many as 90% of children with steroid-sensitive nephrotic syndrome experience relapse, but proper initial therapy can reduce relapse risk. […] These guidelines address management of steroid-sensitive nephrotic syndrome in children aged 1–18 years. […] Initial treatment: Once daily prednisone or prednisolone for at least 12 weeks, beginning with 60 mg/m²/day or 2 mg/kg/day (maximum, 60 mg/day) for 4–6 weeks. After 4–6 weeks, switch to 40 mg/m² or 1.5 mg/kg (maximum, 40 mg) on alternate days for 2–5 months with tapering (moderate-quality evidence). […] Treatment of infrequent relapse (1 relapse in 6 months or 1–3 relapses in 12 months): Administer initial treatment dose (60 mg/m²/day or 2 mg/kg/day) until urinary protein is negative for 3 days (very low-quality evidence). Switch to alternate-day doses per initial treatment for 4 weeks with tapering (low-quality evidence).

• #2 Treatment Guidelines for Steroid-Sensitive Idiopathic Nephrotic Syndrome in Childrenlogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/pa201304240000004/2013/04/24/treatment-guidelines-steroid-sensitive

  Treatment of frequent relapse (≥2 relapses in 6 months or ≥4 relapses in 12 months): Continue infrequent relapse treatment for 3 months at lowest dose to maintain remission or use corticosteroid-sparing agents, including alkylating agents, levamisole, calcineurin inhibitors, mycophenolate mofetil (low-quality evidence).

• #2 Strategies to Improve Quality of Life in Children with Nephrotic Syndrome – Indian Journal of Nephrology

  https://indianjnephrol.org/strategies-to-improve-quality-of-life-in-children-with-nephrotic-syndrome/

  Steroid minimization by use of restricted courses of the alternate day rather than daily regimes for remission maintenance, utilization of brief periods of low-dose daily steroids to prevent relapse during viral infections, and early use of steroid-sparing agents are all important strategies. […] Psychological support through counseling or support groups can improve psychosocial well-being. […] Early judicious implementation of steroid-sparing strategy is important, but it is equally important to ensure peer support and increase awareness of the disease and its challenges among families and peers. […] Comprehensive care strategies that address these are the need of the hour.

• #2 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  Recommended treatment of a relapse for a child with steroid-sensitive disease using the above dosing schedule until complete remission (urine protein-creatine ratio, 0.2 mg/mg or negative or trace dipstick for at least 3 consecutive days). […] Thereafter, steroid is switched to an alternate-day schedule for 4 weeks and then stopped without tapering. This is considered first-line treatment for a relapse. […] A brief course of daily steroid for a child in remission with an upper respiratory tract infection is not recommended. […] However, a short course of low dose daily steroid may be considered for a child with an upper respiratory tract infection who is already on an alternate-day course of steroid and who has a history of repeated infection-associated relapses. This is a „grade D, weak recommendation.” The duration of a „short course” is not defined and is presumably at the discretion of the nephrologist.

• #2

  https://link.springer.com/article/10.1007/s40746-016-0044-x

  Children with frequently relapsing or steroid-dependent NS should be treated with alternate-day steroids for at least 3 months after entering remission at the lowest dose possible to maintain remission. […] Alkylating agents such as cyclophosphamide and chlorambucil are alternate agents for children with frequently relapsing NS and steroid-dependent NS and can induce a sustained remission in some children. […] Cyclosporine and tacrolimus are calcineurin inhibitors that are commonly used as immunosuppressive agents in solid organ transplantation. […] Mycophenolate mofetil (MMF) is a medication that suppresses the immune system through inhibition of B- and T-lymphocyte proliferation. […] Rituximab has been found to be a useful drug to decrease relapse rates in children with frequently relapsing or steroid-dependent NS. […] In summary, a number of therapeutic options are available for treatment of children with NS. Careful consideration of potential complications should be discussed with families prior to prescribing treatments for NS.

• #2 Treatment of idiopathic nephrotic syndrome in children | Boletín Médico del Hospital Infantil de México (English Edition)

  https://www.elsevier.es/pt-revista-boletin-medico-del-hospital-infantil-201-articulo-treatment-idiopathic-nephrotic-syndrome-in-X2444340914741917

  In patients with steroid-dependent INS or with frequent relapses, a scheme of corticosteroids has been suggested that consists of administration of prednisone at doses of 40-60 mg/m2/day until the proteinuria is corrected for 4 to 5 days. […] Cyclophosphamide and chlorambucil have been used with good effect in children with INS who have frequent relapses or are steroid dependent and who developed serious side effects with prolonged steroid treatment. […] Different studies have shown that cyclosporin could reduce the incidence of relapses from 75-90% in patients with NS who are frequent relapsers or are steroid dependent. […] In patients in whom the cosmetic effects of cyclosporin are undesirable, tacrolimus can be used. […] The use of mycophenolate mofetil has been suggested in children with INS who are frequent relapsers and steroid dependent and require long-term treatment with cyclosporin and/or prednisone to maintain remission.

• #2 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  A steroid-sparing agent or low-dose steroid either as alternate-day or daily is recommended as second-line treatment as maintenance for frequently relapsing or steroid-dependent nephrotic syndrome. […] Discontinuation may be considered after at least 12 months. Steroid-sparing medications include the following: Calcineurin inhibitors (cyclosporine A or tacrolimus), Cyclophosphamide, Levamisole (not available in the United States), Mycophenolate mofetil, Rituximab. […] The strength of recommendation for the use of cyclosporin A is „grade B, moderate.” […] The starting dose for tacrolimus is 0.1-0.2 mg/kg/d (maximum, 10 mg) in 2 doses every 12 hours to achieve trough blood levels of 3-7 ng/mL. The strength of recommendation for the use of tacrolimus is „grade C, moderate.” […] Recommend avoiding prolonged use of calcineurin inhibitors for more than a total of 2-3 years but before further continuation beyond 2-3 years, a kidney biopsy be considered. […] The recommended starting dose for mycophenolate mofetil is 1200 mg/m2 BSA (maximum, 300 mg) divided into two doses every 12 hours. Therapy with this medication should begin while the child is still receiving alternate-day steroids. The latter can be tapered and discontinued within 6-12 weeks. If mycophenolate mofetil has been effective in preventing relapses for at least 12 months, tapering and discontinuing over 3-6 months may be considered.

• #2 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  Recommend using rituximab as a steroid-sparing agent if there has not been effective relapse-free maintenance with at least one other steroid-sparing agent at adequate dose. […] The recommended dose is 375 mg/m2 for each infusion (maximum, 1000 mg). The number of infusions may be between 1 and 4. B cell depletion may be monitored by CD19(+) cell counts at baseline and at 7 days post-infusion. The target for B cell depletion is an absolute CD19 cell count 5 cells/mm3 or 1% of total lymphocytes. Also recommend monitoring IgG levels at baseline and periodically thereafter for hypogammaglobulinemia. Pretreatment with acetaminophen, antihistamines and/or steroids is recommended to prevent an infusion reaction. After completion of a course of one or more infusions, steroid and other steroid-sparing agents are tapered within 2-3 months. Reconstitution of B cells is defined as a total B cell count 5/mm3.

• #2 Pediatric Nephrotic Syndrome Treatment & Management: Approach Considerations, Corticosteroid Therapy, Diuretic Therapy

  https://emedicine.medscape.com/article/982920-treatment

  Calcineurin inhibitors (eg, cyclosporin A [CSA] and tacrolimus [TAC]) are useful steroid-sparing agents. […] Mycophenolate mofetil (MMF) has been increasingly used in FRNS and SDNS because it has fewer adverse effects than CYP, CSA, and TAC. […] Rituximab may be considered in children with SDNS or FRNS in whom other treatments have failed or those with cumulative toxicity of other steroid-sparing agents. […] Genetic considerations play an important role in the treatment of SRNS and FSGS; approximately 30% of children with SRNS may have a single-gene cause of their disease. […] Plasmapheresis has been attempted in patients with treatment-resistant FSGS and SRNS. […] Routine use of plasmapheresis or immunoadsorption in SRNS and FSGS cannot be recommended at this time.

• #2 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

• #2 Nephrotic Syndrome in Children | National Kidney Foundation

  https://www.kidney.org/kidney-topics/nephrotic-syndrome-children

  About 80% of children with minimal change disease between 2 and 9 years of age will respond to treatment within 3 to 4 weeks. This means protein in the urine and swelling will go away while taking the medication. When protein in the urine goes away completely, this is called remission. […] A low salt (sodium) diet to help limit the swelling in the body is often recommended for children with nephrotic syndrome. This is most important when there is protein leaking into the urine. A dietitian who works with the kidney doctor can provide recommendations.

• #2 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  Diuretics are usually needed. Furosemide (1 mg/kg/day) and spironolactone (2 mg/kg/day) help when fluid retention is severe enough, provided there are no signs of kidney failure or volume contraction. Achieving a satisfactory diuresis is hard when serum albumin level is less than 1.5 g/dL, so sometimes albumin has to be given. […] To prevent infections, penicillin can be started in children with overt edema. Abdominal paracentesis is recommended in patients showing signs of peritonitis, and bacterial infections should be treated sooner. Non-immune patients with varicella should receive immunoglobulin therapy if exposure to chickenpox occurs, and acyclovir should be started if the patient develops chickenpox. […] The principles of treatment in adults with acute nephrotic syndrome are not different from those for children. Diuretics, such as furosemide, spironolactone, and even metolazone, may be needed. Diuretic use may lead to volume depletion, which should be assessed by monitoring symptoms, weight, pulse, and blood pressure.

• #2 Nephrotic Syndrome: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5989-nephrotic-syndrome

  Nephrotic syndrome treatment includes addressing the underlying cause and taking steps to reduce high blood pressure, high cholesterol, swelling and infection risks. Treatment usually includes medications and changes to your diet. […] No, nephrotic syndrome isn’t curable. However, nephrotic syndrome often goes away in children once they reach their late teenage years or early 20s. […] Some blood pressure medications can slow down a kidney disease that causes nephrotic syndrome, including: Angiotensin-converting enzyme (ACE) inhibitors and Angiotensin receptor blockers (ARBs). […] To lower your cholesterol, your healthcare provider may recommend statin medications. […] If you have nephrotic syndrome, it’s also a good idea to get a yearly influenza (flu) vaccine and a pneumococcal (new-ma-cah-cole) vaccine.

• #2 The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I – Diagnosis and treatment of the first episode and the first relapse | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-017-0356-x

  We suggest the use of oral acyclovir following exposure to chickenpox in non-immune patients. […] We do not recommend the routine use of prophylactic PPIs in combination with steroid therapy in NS. […] We do not suggest calcium and vitamin D supplementation in children at first episode or in SSNS unless vitamin D deficiency has been predicted or demonstrated. […] We do not recommend the use of lipid-lowering treatments at the onset of NS. […] We suggest that kidney biopsy be performed after the first four weeks of therapy; the continuation of steroid treatment depends on the histological findings.

• #2 Nephrotic Syndrome: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5989-nephrotic-syndrome

  In some cases, your healthcare provider may recommend medications that help prevent blood clots (anticoagulants or blood thinners). […] Diet and nutrition don’t cause or prevent nephrotic syndrome in adults. However, if you have nephrotic syndrome, changes to your diet may help relieve some of your symptoms. […] The best way to manage your symptoms is to take your medications as prescribed by your healthcare provider.

• #2 Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group | Nature Reviews Nephrology

  https://www.nature.com/articles/s41581-020-00384-1

  We do not recommend using immunosuppressive drugs to treat children with CNS. […] We suggest considering congenital membranous nephropathy owing to anti-NEP antibodies in patients who have kidney failure at presentation, transient proteinuria that resolves spontaneously or siblings with transient proteinuria at birth. […] We recommend treating patients with infection-related CNS with specific antimicrobial agents and performing genetic screening in these patients.

• #2 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  Anticoagulation has been suggested to prevent thromboembolic complications, but its role in primary prevention is not proven. Hypolipidemic agents could be used. In patients with secondary nephrotic syndrome, such as that secondary to diabetic nephropathy, some medications are widely used to reduce proteinuria, such as angiotensin-converting enzyme (ACE) inhibitors and/or angiotensin 2 receptor blockers. By reducing proteinuria, these drugs will lead to reduced intraglomerular pressure causing a reduction in systemic blood pressure. […] The diet in patients with nephrotic syndrome is aimed to provide sufficient caloric and protein (1 g/kg/d) intake. Supplemental dietary proteins are of no proven value. A low-salt diet helps limit fluid retention and edema. […] The patient’s edema and proteinuria define the adjustment of diuretics and angiotensin antagonists. Follow-up in the nephrotic syndrome also involves immunizations and monitoring for steroid toxicity. Routine immunizations should be deferred until there are no relapses and the patient has been off immunosuppressants for at least three months.

• #2

  https://www.singhealth.com.sg/patient-care/conditions-treatments/nephrotic-syndrome-children

  You will be asked to check your child’s urine for protein daily and to record the results in the Nephrotic Diary. These will help you and your doctor to monitor your child’s progress and to detect a relapse early. When your child starts to respond to treatment, he/she will pass large amounts of urine with gradual weight loss and resolution of the body swelling. His/her urine protein level will gradually decrease to negative.

• #2 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  A detailed assessment is necessary before starting corticosteroids. The patient’s height, weight, and blood pressure should be monitored. Regular weight record helps in monitoring the decrease or increase of edema. Physical examination is carried out to detect infections and underlying systemic disorders. […] Specific treatment of nephrotic syndrome is dependent on its cause. Therefore, management varies between adult and pediatric populations. Kidney Disease Improving Global Outcomes (KDIGO) issued guidance in 2012 that included recommendations for treating nephrotic syndrome. […] Corticosteroids are mainly used for children with idiopathic nephrotic syndrome. Alternative immunosuppressive agents are often necessary for children with frequently relapsing or steroid-dependent nephrotic syndrome. Examples of these drugs include cyclophosphamide, mycophenolate mofetil (MMF), calcineurin inhibitors, and levamisole. In cases of steroid-resistant nephrotic syndrome, the first-line choice is calcineurin inhibitors, and if there is no response, then agents such as MMF or prolonged and/or intravenous pulse corticosteroids could be used.

• #2 Nephrotic Syndrome in Children | National Kidney Foundation

  https://www.kidney.org/kidney-topics/nephrotic-syndrome-children

  Nephrotic syndrome in kids causes swelling and protein loss in urine. Managed with meds and a low-sodium diet. Monitoring is crucial for treatment. […] There is no cure for most cases of nephrotic syndrome. However, in most children it can be managed with medications. Many children with minimal change disease will stop having relapses of nephrotic syndrome by late childhood or early teenage years. […] Corticosteroids (medicines called prednisone or prednisolone) are the main treatment for nephrotic syndrome due to minimal change disease. If protein in the urine is severe and blood albumin levels are very low, your doctor may give albumin by an intravenous (IV) injection directly into the bloodstream. In some cases, medicines called diuretics (water pills) may be needed to help the kidneys remove excess fluid.

• #2 Can children with nephrotic syndrome receive less aggressive steroid treatment? – International Society of Nephrology

  https://www.theisn.org/blog/2021/02/10/can-children-with-nephrotic-syndrome-receive-less-aggressive-steroid-treatment/

  Despite decades of use, the dose and duration of corticosteroid therapy for various indications in nephrotic syndrome is usually empiric and not guided by robust evidence. […] Several recent, large, multicenter randomized controlled trials have shown that extending initial therapy beyond 8-12 weeks does not influence the time to first relapse or the risk of frequent relapses after one or two years follow-up. […] Results of these studies provide a rationale for minimizing the exposure to corticosteroids for children with nephrotic syndrome and are likely to become part of upcoming international and regional evidence-based guidelines. However, well-powered studies are required to evaluate the optimal dose and duration of prednisone therapy for relapses, concludes Dr. Sinha.

• #3

  https://link.springer.com/article/10.1007/s40746-016-0044-x

  Nephrotic syndrome (NS) is among the most common pediatric kidney diseases with a high risk of morbidity and mortality due to infection and thrombosis. Goals of treatment are to reduce proteinuria to normal levels thereby reducing symptoms and risk of complications. Children with NS should initially be treated with prednisone or prednisolone at a dose of 60 mg/m2/day daily for 6 weeks followed by 40 mg/m2/day given every other day for an additional 6 weeks. […] While most children are steroid responsive, approximately 20 % of children with NS do not go into remission with steroids and should be treated with a calcineurin inhibitor such as cyclosporine or tacrolimus. […] Treatment with mycophenolate mofetil is recommended as first-line therapy for treatment of frequently relapsing or steroid-dependent NS with steroid toxicity due to its favorable side effect profile compared to alternatives.

• #3 Treatment of idiopathic nephrotic syndrome in children | Boletín Médico del Hospital Infantil de México (English Edition)

  https://www.elsevier.es/pt-revista-boletin-medico-del-hospital-infantil-201-articulo-treatment-idiopathic-nephrotic-syndrome-in-X2444340914741917

  Treatment of idiopathic nephrotic syndrome in children […] In all patients with INS, corticosteroid treatment is indicated, as a renal biopsy is usually not required before starting treatment. The majority of patients (80-90%) respond to this treatment. Children with INS who do not achieve complete remission with corticosteroid therapy commonly present focal and segmental glomerular sclerosis and require treatment with calcineurin inhibitors (cyclosporin or tacrolimus), mycophenolate mofetil or rituximab, plus renin-angiotensin system blockade. […] In this article, we review the recent accepted recommendations for the treatment of children with INS. […] Treatment with corticosteroids is begun after the presence of infection has been ruled out or if the infections have been successfully treated. Currently, the following steroid treatment scheme is recommended: 1) First, prednisone 60 mg/m2/day or 2 mg/kg/day once daily for 6 weeks (maximum 60 mg) 2) Next, prednisone 40 mg/m2/day or 1.5 mg/kg/day as a single dose every 48 h for 6 weeks (maximum 40 mg).

• #3 Strategies to Improve Quality of Life in Children with Nephrotic Syndrome – Indian Journal of Nephrology

  https://indianjnephrol.org/strategies-to-improve-quality-of-life-in-children-with-nephrotic-syndrome/

  Steroid minimization by use of restricted courses of the alternate day rather than daily regimes for remission maintenance, utilization of brief periods of low-dose daily steroids to prevent relapse during viral infections, and early use of steroid-sparing agents are all important strategies. […] Psychological support through counseling or support groups can improve psychosocial well-being. […] Early judicious implementation of steroid-sparing strategy is important, but it is equally important to ensure peer support and increase awareness of the disease and its challenges among families and peers. […] Comprehensive care strategies that address these are the need of the hour.

• #3 Treatment of idiopathic nephrotic syndrome in children | Boletín Médico del Hospital Infantil de México (English Edition)

  https://www.elsevier.es/pt-revista-boletin-medico-del-hospital-infantil-201-articulo-treatment-idiopathic-nephrotic-syndrome-in-X2444340914741917

  Rituximab is a murine-human monoclonal antibody directed against the CD20 antigen present in pre-B and B lymphocytes and has been mainly developed for the treatment of blood-related cancers. […] During the period of nephrosis, it is advisable to restrict the addition of salt to foods. […] In children with steroid-resistant INS, the finding of FSGS on histological study of the renal biopsy is common. […] Treatment of patients with steroid-resistant INS incorporates the aspects of conservative management and diverse therapeutic schemes directed at controlling proteinuria and preservation of renal function. […] In patients with INS with FSGS and who respond to initial steroid treatment, it has been observed that 50% could remain in complete remission, 25% have subsequent partial remission and another 25% progress to late steroid resistance.

• #3

  https://www.healthychildren.org/English/health-issues/conditions/genitourinary-tract/Pages/nephrotic-syndrome-in-children.aspx

  Some children with nephrotic syndrome have steroid-resistant nephrotic syndrome. This means that protein in the urine does not go away with prednisone treatment. In this case, your child’s doctor will consider a kidney biopsy, prescribe other medications and sometimes recommend genetic testing. […] A low salt (sodium) diet to help limit the swelling in the body is often recommended for children with nephrotic syndrome. This is most important when there is protein leaking into the urine. A dietitian who works with the kidney doctor can provide recommendations.

• #3 Nephrotic Syndrome (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/nephrotic-syndrome.html

  Most of the time, nephrotic syndrome goes away with medicine, and kids outgrow it by the time they’re teens. […] Nephrotic syndrome is almost always treatable, but the treatment depends on the cause. Kids with nephrotic syndrome usually are treated by a nephrologist (a doctor who specializes in kidney problems). […] To treat minimal change disease, the doctor will prescribe: Prednisone. This medicine works well to treat nephrotic syndrome, but can sometimes have side effects. The doctor may prescribe other medicines to ease or prevent them. […] A diuretic. This makes a child pee more often, which can help control the swelling until protein levels return to normal. […] Kids should eat a low-salt diet to help ease swelling. A low-protein diet isn’t necessary. For other dietary advice, talk to a doctor or dietitian. […] The sooner treatment for nephrotic condition starts, the better. If your child shows any signs of the condition, call your doctor so it can get checked out right away.

• #3 Pediatric Nephrotic Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/982920-guidelines

  Recommend using rituximab as a steroid-sparing agent if there has not been effective relapse-free maintenance with at least one other steroid-sparing agent at adequate dose. […] The recommended dose is 375 mg/m2 for each infusion (maximum, 1000 mg). The number of infusions may be between 1 and 4. B cell depletion may be monitored by CD19(+) cell counts at baseline and at 7 days post-infusion. The target for B cell depletion is an absolute CD19 cell count 5 cells/mm3 or 1% of total lymphocytes. Also recommend monitoring IgG levels at baseline and periodically thereafter for hypogammaglobulinemia. Pretreatment with acetaminophen, antihistamines and/or steroids is recommended to prevent an infusion reaction. After completion of a course of one or more infusions, steroid and other steroid-sparing agents are tapered within 2-3 months. Reconstitution of B cells is defined as a total B cell count 5/mm3.

• #3 Pediatric Nephrotic Syndrome – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/nephrotic-syndrome

  During the first episode of nephrotic syndrome, your child may need to stay in the hospital. […] Other treatment options may include: […] Medicines may be used to treat initial symptoms and relapses. […] Changing the diet for a child with nephrotic syndrome may include limiting salt and fluids. […] In rare cases, a child may develop kidney failure and need dialysis. Dialysis is a procedure that filters waste and extra fluid from the blood. […] Treatment will depend on your child’s symptoms, age and general health. It will also depend on how severe the condition is. […] Medicines may be used to treat initial symptoms and relapses. These may include: […] Corticosteroids […] Immunosuppressives […] Diuretics to reduce the swelling […] Medicine to treat high blood pressure. […] The diet for a child with nephrotic syndrome may include limiting salt and fluids. This may help to regulate your child’s fluid balance. […] Your child’s healthcare provider will talk with you about how much salt and fluids your child should have each day. […] In rare cases, a child may develop kidney failure and need dialysis. Dialysis is a procedure that filters waste and extra fluid from the blood.

• #3 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  A detailed assessment is necessary before starting corticosteroids. The patient’s height, weight, and blood pressure should be monitored. Regular weight record helps in monitoring the decrease or increase of edema. Physical examination is carried out to detect infections and underlying systemic disorders. […] Specific treatment of nephrotic syndrome is dependent on its cause. Therefore, management varies between adult and pediatric populations. Kidney Disease Improving Global Outcomes (KDIGO) issued guidance in 2012 that included recommendations for treating nephrotic syndrome. […] Corticosteroids are mainly used for children with idiopathic nephrotic syndrome. Alternative immunosuppressive agents are often necessary for children with frequently relapsing or steroid-dependent nephrotic syndrome. Examples of these drugs include cyclophosphamide, mycophenolate mofetil (MMF), calcineurin inhibitors, and levamisole. In cases of steroid-resistant nephrotic syndrome, the first-line choice is calcineurin inhibitors, and if there is no response, then agents such as MMF or prolonged and/or intravenous pulse corticosteroids could be used.

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