Materiały źródłowe

• #1 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

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

  Nephrotic syndrome (NS) is a clinical syndrome defined by massive proteinuria (greater than 40 mg/m^2 per hour) responsible for hypoalbuminemia (less than 30 g/L), with resulting hyperlipidemia, edema, and various complications. […] Nephrotic syndrome (NS) is a clinical syndrome defined by massive proteinuria responsible for hypoalbuminemia, with resulting hyperlipidemia, edema, and various complications. […] The first indication of nephrotic syndrome in children is the swelling of the face which then progresses to the entire body. […] The most common cause in children is minimal change glomerulonephritis. […] Nephrotic-range proteinuria will be apparent by 3+ or 4+ readings on the dipstick or by semiquantitative testing by sulfosalicylic acid. […] Urine samples over 24 hours (for an accurate measure) and proteinuria (3 g protein) is diagnostic.

• #1 Nephrotic Syndrome: In Children, Treatment, and Causes

  https://www.healthline.com/health/nephrotic-syndrome

  Both primary and secondary nephrotic syndrome can occur in children. Primary nephrotic syndrome is the most common type in children. […] In children, nephrotic syndrome causes these symptoms: fever, fatigue, irritability, and other signs of infection, loss of appetite, blood in the urine, diarrhea, high blood pressure. […] To diagnose nephrotic syndrome, your doctor will first take your medical history. You’ll be asked about your symptoms, any medications you’re taking, and whether you have any underlying health conditions. […] Several tests are used to help diagnose nephrotic syndrome. They include: Urine tests. You’ll be asked to provide a sample of urine. This can be sent to a laboratory to determine whether you have high amounts of protein in your urine. […] Blood tests. In these tests, a sample of blood will be taken from a vein in your arm. This sample can be analyzed to check blood markers of overall kidney function, blood levels of albumin, and cholesterol and triglyceride levels.

• #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 diagnosis of nephrotic syndrome includes: Heavy proteinuria (dipstick 34+ or urine protein/creatinine ratio 0.2 g/mmol = 200 mg/mmol) […] Hypoalbuminaemia (25 g/L) […] The family should be taught to test urine protein each morning […] After remission, the urine protein should still be checked and documented every day (for at least 12 years), in order to identify a relapse (defined as 3+ or 4+ protein for 3 consecutive days), at which point the family should contact their treating clinician […] This allows for re-institution of prednisolone prior to the onset of oedema, thus avoiding the associated consequences (admission, risk of sepsis, thrombosis).

• #1 Nephrotic syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/nephrotic-syndrome/diagnosis-treatment/drc-20375613

  Tests and procedures used to diagnose nephrotic syndrome include: […] A urinalysis can reveal abnormalities in your urine, such as large amounts of protein. You might be asked to collect urine samples over 24 hours. […] A blood test can show low levels of the protein albumin and often decreased levels of blood protein overall. Loss of albumin is often associated with an increase in blood cholesterol and blood triglycerides. The creatinine and urea nitrogen levels in your blood also might be measured to assess your overall kidney function. […] Your doctor might recommend removing a small sample of kidney tissue for testing. During a kidney biopsy, a needle is inserted through your skin and into your kidney. Kidney tissue is collected and sent to a lab for testing. […] If your doctor suspects you or your child has a kidney problem, such as nephrotic syndrome, you might be referred to a doctor who specializes in the kidneys (nephrologist). […] What tests do I or my child need?

• #1 Proteinuria in Children: Evaluation and Differential Diagnosis | AAFP

  https://www.aafp.org/pubs/afp/issues/2017/0215/p248.html

  The Chronic Kidney Disease in Children study demonstrated that persistent proteinuria with a high urine protein-to-creatinine (UPr/Cr) ratio (more than 2 in patients with nonglomerular disease and more than 0.5 in patients with glomerular disease) predicts significant chronic kidney disease progression. […] When a child’s UPr/Cr ratio is more than 0.2 (more than 0.5 for children six to 24 months of age) or urinalysis results are abnormal, further evaluation with history, physical examination, and additional blood work is recommended to rule out significant renal disease. […] A 24-hour urine protein excretion test is often difficult in children. […] Spot, first-morning urine testing for determining UPr-to-osmolality ratio is more useful and correlates well with the 24-hour urine collection; it should be used in children if the urine dipstick test result is 1 or more.

• #1 Pediatric Nephrotic Syndrome Workup: Approach Considerations, Urine Studies, Blood Studies

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

  The first step in evaluating the child with edema is to establish whether nephrotic syndrome is present, because hypoalbuminemia can occur in the absence of proteinuria (such as protein-losing enteropathy), and edema can occur in the absence of hypoalbuminemia (for example, in angioedema, capillary leak, venous insufficiency, or congestive heart failure). […] In order to establish the presence of nephrotic syndrome, laboratory tests should confirm (1) nephrotic-range proteinuria, (2) hypoalbuminemia, and (3) hyperlipidemia. Therefore, initial laboratory testing should include the following: Urinalysis, Urine protein quantification (by first-morning urine protein/creatinine ratio or 24-hour urine protein measurement), Serum albumin, Lipid panel. […] Once the diagnosis of nephrotic syndrome has been established, the next step is to determine whether the nephrotic syndrome is primary (idiopathic) or secondary to a systemic disorder and, if idiopathic nephrotic syndrome (INS) has been confirmed, whether signs of chronic kidney disease, kidney insufficiency, or other renal disorders exclude the possibility of minimal change nephrotic syndrome (MCNS).

• #1 Nephrotic Syndrome in Children | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/nephrotic-syndrome-children

  What is nephrotic syndrome? Nephrotic syndrome is characterized by the following symptoms that result from changes that occur to the small, functional structures in the kidneys, such as: Very high levels of protein in the urine. Low levels of protein in the blood (albumin) due to its loss in the urine. Tissue swelling all over the body (edema) especially in the abdomen (ascites). High cholesterol levels in the blood. Decrease in frequency of urination. Weight gain from excess fluid. […] How is nephrotic syndrome diagnosed? In addition to a thorough physical examination and complete medical history, your child’s doctor may recommend the following diagnostic tests: Urine tests. Tests to check for protein in the urine. Blood tests. Tests to check the levels of cholesterol and a special blood protein called albumin. Renal ultrasound. A noninvasive test in which a transducer is passed over the kidney producing sound waves which bounce off of the kidney, transmitting a picture of the organ on a video screen. The test is used to determine the size and shape of the kidney and to detect a mass, kidney stone, cyst, or other obstruction or abnormalities. Renal biopsy. A procedure where a small sample of tissue is taken from the kidney through a needle. The tissue is sent for special testing to determine the specific disease.

• #1 Nephrotic Syndrome in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=nephrotic-syndrome-in-children-90-P03098

  Nephrotic syndrome is a problem where too much protein called albumin is released from the body into the urine. It means that one or both kidneys are damaged. […] The healthcare provider will ask about your child’s symptoms and health history. He or she may also ask about your family’s health history. He or she will give your child a physical exam. Your child may also have tests, such as: Urine test. This is done to check the level of protein. Blood tests. These check your child’s levels of cholesterol, a blood protein called albumin, and other blood chemicals. Renal ultrasound (sonography). This is a painless test that uses sound waves and a computer to create images of body tissues. During the test, a healthcare provider moves a device called a transducer over the belly in the kidney area. This sends a picture of the kidney to a video screen. The healthcare provider can see the size and shape of the kidney. He or she can also see a growth, kidney stone, cyst, or other problems. Renal biopsy. The healthcare provider takes a small sample of kidney tissue. This is done through the skin with a needle or during surgery. The sample is looked at under a microscope.

• #1 Pediatric Nephrotic Syndrome: Practice Essentials, Background, Pathophysiology

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

  The following tests should be performed in selected patients to determine whether the nephrotic syndrome is primary and idiopathic (INS) or secondary and, if INS has been confirmed, whether signs of chronic kidney disease or extrarenal disease exclude minimal change nephrotic syndrome (MCNS): Complete blood cell (CBC) count, Complete metabolic panel that includes serum electrolytes, calcium, phosphorus, albumin, blood urea nitrogen (BUN), creatinine, aspartate aminotransferase (AST), and alanine aminotransferase (ALT). […] To exclude a secondary form of nephrotic syndrome in selected patients, the following tests may be considered: Testing for human immunodeficiency virus (HIV), Testing for hepatitis B and C viruses, Complement components (C3, C4), Antinuclear antibody (ANA), antidouble-stranded DNA antibody (in selected patients). […] Other tests and procedures in selected patients may include the following: Genetic studies, Kidney ultrasonography, Chest radiography, Mantoux test or Quantiferon Gold TB test, Kidney biopsy.

• #1 Pediatric Nephrotic Syndrome Workup: Approach Considerations, Urine Studies, Blood Studies

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

  A kidney biopsy is not indicated for the first presentation of INS in children aged 1-12 years unless the history, physical findings, or laboratory results indicate the possibility of secondary nephrotic syndrome or primary nephrotic syndrome other than MCNS. […] Kidney biopsy is indicated in patients younger than 1 year, when genetic forms of congenital nephrotic syndrome are more common, and in patients older than 12 years, when chronic glomerular diseases such as FSGS have a higher incidence. […] Finally, in patients who are initially or subsequently unresponsive to steroid treatment, kidney biopsy should be performed, because steroid unresponsiveness has a high correlation with prognostically unfavorable histopathological findings, such as those associated with FSGS or membranous glomerulonephritis (MGN).

• #1 Nephrotic Syndrome in Children | National Kidney Foundation

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

  Your child’s doctor may also decide if a kidney biopsy is needed if: Test results suggest a diagnosis other than MCD. […] There is no cure for most cases of nephrotic syndrome. However, in most children it can be managed with medications. […] Corticosteroids (medicines called prednisone or prednisolone) are the main treatment for nephrotic syndrome due to minimal change disease. […] About 80% of children with minimal change disease between 2 and 9 years of age will respond to treatment within 3 to 4 weeks. […] It is important to monitor the urine protein at home using a urine test strip or dipstick. This will help your doctor know when the nephrotic syndrome is in remission and when it may have relapsed.

• #1 Nephrotic Syndrome in Children | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/nephrotic-syndrome-in-children

  Idiopathic nephrotic syndrome accounts for about 90% of children with nephrotic syndrome. […] The type of nephrotic syndrome that is most common in children is called idiopathic nephrotic syndrome. It accounts for about 90 percent of children with nephrotic syndrome. […] The symptoms of nephrotic syndrome may resemble other conditions and medical problems. Always consult your child’s doctor for a diagnosis. […] In addition to a thorough physical examination and complete medical history, your child’s doctor may recommend the following diagnostic tests: […] Urine tests. Tests to check for protein […] Blood tests. Tests to check the levels of cholesterol and a special blood protein called albumin […] Renal ultrasound. A noninvasive test in which a transducer is passed over the kidney producing sound waves which bounce off of the kidney, transmitting a picture of the organ on a video screen. The test is used to determine the size and shape of the kidney and to detect a mass, kidney stone, cyst, or other obstruction or abnormalities. […] Renal biopsy. A procedure where a small sample of tissue is taken from the kidney through a needle. The tissue is sent for special testing to determine the specific disease.

• #1 Evaluation of Children with Nephrotic Syndrome: A Single Center Experience – The Journal of Pediatric Research

  https://jpedres.org/articles/evaluation-of-children-with-nephrotic-syndrome-a-single-center-experience/doi/jpr.galenos.2023.37928

  The patients were treated according to the Kidney Disease: Improving Global Outcomes clinical practice guidelines for glomerulonephritis and oral prednisolone was initiated as steroid therapy at the first step. […] The patients were classified according to their steroid responses; 24 (63%) patients were steroid sensitive, and 14 (37%) were steroid resistant NS. […] At their last recorded visits, 33 (86.8%) of the patients were in remission, 2 (5.3%) were in partial remission, and 3 (7.9%) were diagnosed as chronic kidney disease (CKD). […] Histopathological features are of great importance in determining steroid sensitivity and the long-term prognosis in NS. […] In our patients, while the rate of steroid sensitive NS was 63%, 42% of these patients were steroid dependent. […] The pathological diagnosis of 54% (8/14 patients) of the patients with steroid-resistant NS in our study was FSGS. The steroid response rate was 88% in those patients with MCD, 41% in those with FSGS; and 33% in those with MPGN. All of the patients who progressed to CKD were steroid-resistant NS and their histopathological diagnosis was FSGS. […] NS is also classified according to steroid sensitivity in addition to its histopathological classification, and this classification and the treatment responses of the patients also guide the prognosis.

• #1 Pediatric Idiopathic Nephrotic Syndrome

  https://www.uspharmacist.com/article/pediatric-idiopathic-nephrotic-syndrome

  Corticosteroid responsiveness is the most significant prognostic factor in INS. […] Children with steroid-sensitive nephrotic syndrome (SSNS) are more likely to have favorable long-term outcomes, whereas those with steroid-resistant nephrotic syndrome (SRNS) have comparatively lower renal survival rates. […] Approximately 90% of pediatric patients with INS achieve complete remission (CR) with corticosteroids and are classified as having SSNS. […] Therefore, renal biopsy is not required for all children, owing to the high likelihood of response to initial and subsequent courses of corticosteroids.

• #1 Evaluation of Children with Nephrotic Syndrome: A Single Center Experience – The Journal of Pediatric Research

  https://jpedres.org/articles/evaluation-of-children-with-nephrotic-syndrome-a-single-center-experience/doi/jpr.galenos.2023.37928

  NS is the most common glomerular disease of childhood. Early diagnosis and the histopathological features of this disease have an important place in its prognosis. Knowing the demographic, clinical and pathological features of the disease is helpful in monitoring its progress and its prognosis. […] The mean age at diagnosis was 6.4 years. The histopathological diagnoses were focal segmental glomerulosclerosis (FSGS) in 17 patients, minimal change disease (MCD) in 8, membranoproliferative glomerulonephritis (MPGN) in 3, and membranous glomerulonephritis in 1 patient. […] Nephrotic syndrome (NS) is the most common childhood glomerular disease manifested by proteinuria, edema and hypoalbuminemia. The aim of this study was to examine children with primary NS in terms of their clinical laboratory and histopathological features, and to evaluate their treatment responses.

• #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

  Infants with CNS present with nephrotic-range proteinuria and oedema with or without kidney failure. The diagnosis and management require expertise and a combined approach that can only be guaranteed in a centre with experience in treating this condition. […] We recommend that all patients with CNS are referred to specialized teams in tertiary paediatric nephrology centres and managed by multidisciplinary teams, including neonatologists, paediatric nephrologists, paediatric nephrology nurses, paediatric renal dieticians, paediatric surgeons, child and/or youth psychologists and social workers. […] We recommend performing an initial diagnostic work-up, including medical history, clinical and biological evaluation of CNS complications and associated non-kidney features. […] We recommend comprehensive genetic screening comprising all podocytopathy-related genes as a first-line diagnostic measure in every patient with CNS.

• #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 recommend providing genetic counselling promptly in families with a history of CNS or prenatal signs of CNS. […] We suggest that kidney biopsy be considered only in patients with sporadic, non-syndromic disease in whom comprehensive genetic testing has not yielded a molecular diagnosis. […] Identification of a genetic cause of CNS establishes the aetiology, informs management, particularly with regard to the potential development of Wilms tumour or neurological involvement, and enables genetic counselling of the family. We therefore recommend genetic screening as a first-line diagnostic measure in every patient with CNS. […] We do not recommend routine kidney biopsy in patients with CNS. Kidney biopsy may be indicated in patients for whom a genetic diagnosis cannot be established or in those with a substantial reduction in eGFR for whom a biopsy sample could be informative in establishing a rare diagnosis. […] We recommend prompt genetic screening in all children with CNS and genetic counselling of their families. Routine kidney biopsy is not recommended but may be considered in patients with sporadic, non-syndromic disease if comprehensive genetic testing has not yielded a molecular diagnosis.

• #1 Pediatric Nephrotic Syndrome Workup: Approach Considerations, Urine Studies, Blood Studies

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

  Therefore, in addition to the above tests, the following should be selectively included in the workup: Complete blood cell (CBC) count, Metabolic panel (serum electrolytes, blood urea nitrogen [BUN] and creatinine, calcium, phosphorus, and ionized calcium levels), Testing for human immunodeficiency virus (HIV), hepatitis B and C viruses, Complement studies (C3, C4), Antinuclear antibody (ANA), antidouble-stranded DNA antibody, anti-neutrophil cytoplasmic antibodies (in selected patients). […] Patients with INS lose vitamin D binding protein, which can result in low vitamin D levels, and thyroid-binding globulin, which can result in low thyroid hormone levels. Consideration should be given, especially in the child with frequently relapsing or steroid-resistant nephrotic syndrome, to testing for 25-OH-vitamin D; free T4; and thyroid-stimulating hormone (TSH).

• #1 Nephrotic Syndrome in Children – NIDDK

  https://www.niddk.nih.gov/health-information/kidney-disease/children/nephrotic-syndrome-children

  How do health care professionals diagnose nephrotic syndrome in children? […] Nephrotic syndrome in children is diagnosed with a medical and family history, a physical exam, urine tests, to look for excess urine proteins, blood tests, to test kidney function and to look for underlying diseases. […] Additional tests to identify the cause of nephrotic syndrome may include ultrasound of the kidney, kidney biopsy, genetic testing. […] Many children with nephrotic syndrome will not need a kidney biopsy. The test is usually reserved for children who have complex disease, who have low kidney function, or who are 12 years old or older.

• #1

  https://journalhosting.ucalgary.ca/index.php/jura/article/view/30231

  Nephrotic syndrome is a common childhood acquired kidney disease, characterized by proteinuria and edema. […] Despite knowledge of symptoms, treatment and complications, the clinical experience of nephrologists at the Alberta Childrens Hospital suggests that there are delays in diagnosis and mistakes in treatment, due to the relative non-specificity of the symptoms. […] The study showed a diagnosis delay range of 1 day to approximately 110 days. […] A total of 12 participants were included, with an age range 2.8-11.4 years. […] Nephrotic syndrome is commonly misdiagnosed by family doctors, resulting in delayed treatment. […] Given the results that diagnostic success was high in the ER, where a urinalysis was almost always used, the low rate of successful diagnosis by family doctors can be avoided. […] There appears to be a need to further educate physicians on the importance of using a urine analysis.

• #2 Pediatric Nephrotic Syndrome Workup: Approach Considerations, Urine Studies, Blood Studies

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

  The first step in evaluating the child with edema is to establish whether nephrotic syndrome is present, because hypoalbuminemia can occur in the absence of proteinuria (such as protein-losing enteropathy), and edema can occur in the absence of hypoalbuminemia (for example, in angioedema, capillary leak, venous insufficiency, or congestive heart failure). […] In order to establish the presence of nephrotic syndrome, laboratory tests should confirm (1) nephrotic-range proteinuria, (2) hypoalbuminemia, and (3) hyperlipidemia. Therefore, initial laboratory testing should include the following: Urinalysis, Urine protein quantification (by first-morning urine protein/creatinine ratio or 24-hour urine protein measurement), Serum albumin, Lipid panel. […] Once the diagnosis of nephrotic syndrome has been established, the next step is to determine whether the nephrotic syndrome is primary (idiopathic) or secondary to a systemic disorder and, if idiopathic nephrotic syndrome (INS) has been confirmed, whether signs of chronic kidney disease, kidney insufficiency, or other renal disorders exclude the possibility of minimal change nephrotic syndrome (MCNS).

• #2 Nephrotic Syndrome In Children: Symptoms, Diagnosis & Treatment | National Kidney Foundation

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

  Children with too much protein in their urine, sudden weight gain, and swelling in various body parts could have a condition called nephrotic syndrome. […] How is nephrotic syndrome diagnosed? Your child’s doctor will check for symptoms, such as swelling and pale skin. In addition, a urine test can check for the amount of protein, blood and other things to look for kidney damage. A blood test can show how well your child’s kidneys are working. The doctor will also check for other diseases that may be causing the nephrotic syndrome. In some cases, diagnosis may also require a kidney biopsy. […] Nephrotic syndrome is almost always treatable, but the treatment depends on the cause. The treatment’s goal is to stop the loss of protein in the urine and increase the amount of urine passed from the body. Your doctor probably will prescribe a drug called prednisone for your child. Most children get better on this drug.

• #2 Nephrotic syndrome in children | MSF Medical Guidelines

  https://medicalguidelines.msf.org/en/viewport/CG/english/nephrotic-syndrome-in-children-18482330.html

  Nephrotic syndrome (NS) is characterized by the presence of oedema, heavy proteinuria, hypoalbuminemia, and hyperlipidaemia. […] Once SAM is excluded, the following two criteria must be met to make a clinical diagnosis of primary NS: Presence of heavy proteinuria, and Absence of associated infections: see Hepatitis B and C and HIV infection, Malaria and Schistosomiases. […] In NS, proteinuria is equal or greater than +++ or equal or greater than 300 mg/dl or 30 g/litre. Nephrotic range proteinuria in children is defined as urinary protein excretion greater than 50 mg/kg daily. […] NS is excluded if heavy proteinuria is not consistently present.

• #2 Nephrotic Syndrome in Children | National Kidney Foundation

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

  Your child’s doctor may also decide if a kidney biopsy is needed if: Test results suggest a diagnosis other than MCD. […] There is no cure for most cases of nephrotic syndrome. However, in most children it can be managed with medications. […] Corticosteroids (medicines called prednisone or prednisolone) are the main treatment for nephrotic syndrome due to minimal change disease. […] About 80% of children with minimal change disease between 2 and 9 years of age will respond to treatment within 3 to 4 weeks. […] It is important to monitor the urine protein at home using a urine test strip or dipstick. This will help your doctor know when the nephrotic syndrome is in remission and when it may have relapsed.

• #2 Pediatric Nephrotic Syndrome Workup: Approach Considerations, Urine Studies, Blood Studies

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

  Therefore, in addition to the above tests, the following should be selectively included in the workup: Complete blood cell (CBC) count, Metabolic panel (serum electrolytes, blood urea nitrogen [BUN] and creatinine, calcium, phosphorus, and ionized calcium levels), Testing for human immunodeficiency virus (HIV), hepatitis B and C viruses, Complement studies (C3, C4), Antinuclear antibody (ANA), antidouble-stranded DNA antibody, anti-neutrophil cytoplasmic antibodies (in selected patients). […] Patients with INS lose vitamin D binding protein, which can result in low vitamin D levels, and thyroid-binding globulin, which can result in low thyroid hormone levels. Consideration should be given, especially in the child with frequently relapsing or steroid-resistant nephrotic syndrome, to testing for 25-OH-vitamin D; free T4; and thyroid-stimulating hormone (TSH).

• #2 Nephrotic Syndrome | Riley Children’s Health

  https://www.rileychildrens.org/health-info/nephrotic-syndrome

  The doctor may perform an ultrasound of the kidney to look for structural abnormalities or blockages. […] The doctor may perform a renal biopsy to determine the cause of your child’s symptoms. […] Symptoms related to childhood nephrotic syndrome that can indicate kidney damage include edema, proteinuria and hypoalbuminemia.

• #2 Nephrotic Syndrome in Children | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/n/nephrotic-syndrome-in-children.html

  Nephrotic syndrome is a problem where too much protein called albumin is released from the body into the urine. It means that one or both kidneys are damaged. […] The healthcare provider will ask about your child’s symptoms and health history. They may also ask about your family’s health history. They will give your child a physical exam. Your child may also have tests, such as: […] Urine test. This is done to see if the urine contains any protein. Sometimes the healthcare provider may ask for one sample. Other times, they may need you to collect all urine over a period of time. […] Blood tests. These check your child’s levels of cholesterol, a blood protein called albumin, and other blood chemicals. […] Renal ultrasound (sonography). This is a painless test that uses sound waves and a computer to create images of body tissues. During the test, a healthcare provider moves a device called a transducer over the belly in the kidney area. This sends a picture of the kidney to a video screen. The healthcare provider can see the size and shape of the kidney. They can also see a growth, kidney stone, cyst, or other problems.

• #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. […] Nephrotic syndrome is a kidney condition that leads to swelling and other symptoms. […] In children with nephrotic syndrome, tiny filters in the kidneys, called glomeruli, are injured. They let too much protein leak out of the blood and into the urine. […] Swelling in the legs, abdomen and around the eyes is usually the first sign of nephrotic syndrome. […] Sometimes a diagnosis is made from lab results and physical exam. Other times a kidney biopsy is needed to confirm the type of nephrotic syndrome. […] If your physician thinks your child has MCD, a kidney biopsy typically is not needed. However, if treatment does not stop protein from leaking into the urine, your kidney doctor may do a kidney biopsy.

• #2 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

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

  The serum albumin level is classically low in nephrotic syndrome. […] Renal biopsy: This is indicated for the following: congenital nephrotic syndrome, children older than eight years at the onset, steroid resistance, frequent relapses or steroid dependency, significant nephritic manifestations. […] Corticosteroids are mainly used for children with idiopathic nephrotic syndrome. […] Rituximab, an anti-B cell antibody, has proved to be an effective steroid-sparing agent in the pediatric population. […] Hospitalization is not usually necessary with close outpatient follow-up care and good parental and patient education. […] Diuretics are usually needed. […] The diet in patients with nephrotic syndrome is aimed to provide sufficient caloric and protein (1 g/kg/d) intake. […] The patient’s edema and proteinuria define the adjustment of diuretics and angiotensin antagonists.

• #2 Evaluation of Children with Nephrotic Syndrome: A Single Center Experience – The Journal of Pediatric Research

  https://jpedres.org/articles/evaluation-of-children-with-nephrotic-syndrome-a-single-center-experience/doi/jpr.galenos.2023.37928

  The patients were treated according to the Kidney Disease: Improving Global Outcomes clinical practice guidelines for glomerulonephritis and oral prednisolone was initiated as steroid therapy at the first step. […] The patients were classified according to their steroid responses; 24 (63%) patients were steroid sensitive, and 14 (37%) were steroid resistant NS. […] At their last recorded visits, 33 (86.8%) of the patients were in remission, 2 (5.3%) were in partial remission, and 3 (7.9%) were diagnosed as chronic kidney disease (CKD). […] Histopathological features are of great importance in determining steroid sensitivity and the long-term prognosis in NS. […] In our patients, while the rate of steroid sensitive NS was 63%, 42% of these patients were steroid dependent. […] The pathological diagnosis of 54% (8/14 patients) of the patients with steroid-resistant NS in our study was FSGS. The steroid response rate was 88% in those patients with MCD, 41% in those with FSGS; and 33% in those with MPGN. All of the patients who progressed to CKD were steroid-resistant NS and their histopathological diagnosis was FSGS. […] NS is also classified according to steroid sensitivity in addition to its histopathological classification, and this classification and the treatment responses of the patients also guide the prognosis.

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

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

  Nephrotic syndrome (NS) is the name given to a collection of kidney-related findings in your child’s body. These include: […] While nephrotic syndrome is not a disease, it can be the first sign of disease that can damage the kidney’s tiny blood-filtering unit (glomeruli) where urine is made. […] In the vast majority of children, NS is idiopathic, meaning that doctors don’t yet know what causes it. […] Nephrotic syndrome always affects both kidneys. […] It usually appears between the toddler and elementary school years, although it may appear later. […] There are thought to be two forms of nephrotic syndrome, minimal change disease (MCD) and focal sclerosis (FSGS). […] Most children with NS outgrow it by young adulthood. […] The first step in treating your child is forming an accurate and complete diagnosis.

• #2 Pediatric Nephrotic Syndrome: Practice Essentials, Background, Pathophysiology

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

  The following tests should be performed in selected patients to determine whether the nephrotic syndrome is primary and idiopathic (INS) or secondary and, if INS has been confirmed, whether signs of chronic kidney disease or extrarenal disease exclude minimal change nephrotic syndrome (MCNS): Complete blood cell (CBC) count, Complete metabolic panel that includes serum electrolytes, calcium, phosphorus, albumin, blood urea nitrogen (BUN), creatinine, aspartate aminotransferase (AST), and alanine aminotransferase (ALT). […] To exclude a secondary form of nephrotic syndrome in selected patients, the following tests may be considered: Testing for human immunodeficiency virus (HIV), Testing for hepatitis B and C viruses, Complement components (C3, C4), Antinuclear antibody (ANA), antidouble-stranded DNA antibody (in selected patients). […] Other tests and procedures in selected patients may include the following: Genetic studies, Kidney ultrasonography, Chest radiography, Mantoux test or Quantiferon Gold TB test, Kidney biopsy.

• #2 Pediatric Idiopathic Nephrotic Syndrome

  https://www.uspharmacist.com/article/pediatric-idiopathic-nephrotic-syndrome

  Corticosteroid responsiveness is the most significant prognostic factor in INS. […] Children with steroid-sensitive nephrotic syndrome (SSNS) are more likely to have favorable long-term outcomes, whereas those with steroid-resistant nephrotic syndrome (SRNS) have comparatively lower renal survival rates. […] Approximately 90% of pediatric patients with INS achieve complete remission (CR) with corticosteroids and are classified as having SSNS. […] Therefore, renal biopsy is not required for all children, owing to the high likelihood of response to initial and subsequent courses of corticosteroids.

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

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

  The process usually starts when a child goes to his pediatrician’s office or even to an emergency room because his body is suddenly very swollen. […] Your child’s doctors will perform diagnostic tests to check for high levels of protein in his urine and low levels of protein in his blood. […] If they don’t see a lot of kidney inflammation, there’s a good chance that he has minimal change disease. […] If your child hasn’t responded to the steroids, his doctors may order a biopsy to check to see if the nephrotic syndrome may be caused by focal sclerosis (FSGS). […] After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child’s condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.

• #2 Diagnostic and Management Challenges in Congenital Nephrotic Syndrome | PHMT

  https://www.dovepress.com/diagnostic-and-management-challenges-in-congenital-nephrotic-syndrome-peer-reviewed-fulltext-article-PHMT

  The advent of genetic mutational analysis has led to more specific diagnoses of CNS. […] The majority of cases of CNS are caused by mutations in four notable genes; NPHS1, NPHS2, WT1 and LAMB2. […] The development of nephrotic range proteinuria in infancy is rare, with most cases in the developed world resulting from genetic mutation leading to CNS. […] The increasing availability of mutational analysis and technology that permits interrogation of multiple relevant genes on a single sample has altered the diagnostic pathway for infants with significant proteinuria. […] Overall, two main management strategies persist. Bilateral nephrectomy, especially in those with severe disease, or those with significant malignant risk, i.e. WT1 mutations, with early consideration of transplantation. […] Management of CNS should be individualised. Both unilateral and bilateral nephrectomy offer benefits.

• #2 RKD Symptoms, Treatments & Causes in Children | NephCure

  https://nephcure.org/intro-to-rkd/pediatric-care/

  If your child is less than 12 months or older than 12 years or does not respond to prednisone, a kidney biopsy may be recommended to obtain a small sample of kidney tissue for detailed examination. […] If a genetic or hereditary component is suspected, genetic testing may be performed to identify specific gene mutations associated with certain kidney diseases. […] A pediatric nephrologist, a doctor specialized in kidney disorders in children, should be consulted for a comprehensive evaluation and management plan.

• #2

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

  Childhood nephrotic syndrome is a kidney condition that results from abnormal leakage of protein in the urine. […] How do we confirm that your child has Nephrotic Syndrome? Blood tests will be done to measure the level of protein (albumin) in the blood and assess kidney function. Urine will be collected to check for the presence of protein. […] More than 90% of children will have resolution of protein in the urine and body swelling with the treatment (Steroid Responsive Nephrotic Syndrome) and this carries an excellent outcome in the vast majority who will continue to have normal kidney function and normal growth. […] 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.

• #2

  https://link.springer.com/article/10.1007/s00467-022-05739-3

  We recommend using spot urine samples, preferably a first morning void, or alternatively a 24-h urine sample to assess proteinuria (grade B, moderate recommendation). […] We recommend confirming nephrotic range proteinuria at least once by quantification of proteinuria before initiating treatment for the first episode (grade B, moderate recommendation). […] We do not recommend routine kidney biopsy and genetic testing in the initial diagnostic work-up of children with NS who present with typical features and age <1 year (grade B, moderate recommendation). [...] We recommend considering genetic testing and/or kidney biopsy in infantile onset NS (age 3-12 months) (grade B, weak recommendation).

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

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

  Nephrotic syndrome (NS) is the name given to a collection of kidney-related findings in your child’s body. These include: […] While nephrotic syndrome is not a disease, it can be the first sign of disease that can damage the kidney’s tiny blood-filtering unit (glomeruli) where urine is made. […] In the vast majority of children, NS is idiopathic, meaning that doctors don’t yet know what causes it. […] Nephrotic syndrome always affects both kidneys. […] It usually appears between the toddler and elementary school years, although it may appear later. […] There are thought to be two forms of nephrotic syndrome, minimal change disease (MCD) and focal sclerosis (FSGS). […] Most children with NS outgrow it by young adulthood. […] The first step in treating your child is forming an accurate and complete diagnosis.

• #3 Pediatric Nephrotic Syndrome: Practice Essentials, Background, Pathophysiology

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

  The following tests should be performed in selected patients to determine whether the nephrotic syndrome is primary and idiopathic (INS) or secondary and, if INS has been confirmed, whether signs of chronic kidney disease or extrarenal disease exclude minimal change nephrotic syndrome (MCNS): Complete blood cell (CBC) count, Complete metabolic panel that includes serum electrolytes, calcium, phosphorus, albumin, blood urea nitrogen (BUN), creatinine, aspartate aminotransferase (AST), and alanine aminotransferase (ALT). […] To exclude a secondary form of nephrotic syndrome in selected patients, the following tests may be considered: Testing for human immunodeficiency virus (HIV), Testing for hepatitis B and C viruses, Complement components (C3, C4), Antinuclear antibody (ANA), antidouble-stranded DNA antibody (in selected patients). […] Other tests and procedures in selected patients may include the following: Genetic studies, Kidney ultrasonography, Chest radiography, Mantoux test or Quantiferon Gold TB test, Kidney biopsy.

• #3 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 that PDN be given at 60 mg/m2/day, with a maximum dose of 60 mg/day. […] We suggest that kidney biopsy be performed after the first four weeks of therapy; the continuation of steroid treatment depends on the histological findings. […] Indications for renal biopsy in children with NS are listed in Table 10. […] A renal biopsy should be recommended in patients 12 months or 12 years of age at the onset of NS or when secondary NS is suspected.

Zobacz więcej