Nephrotic Syndrome||Symptoms||Investigations||Treatment скачать в хорошем качестве

Nephrotic Syndrome||Symptoms||Investigations||Treatment

Nephrotic Syndrome in Pediatrics Introduction Nephrotic syndrome (NS) in pediatrics is a common and significant kidney disorder that presents with proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Nephrotic syndrome can be divided into primary (idiopathic) and secondary causes. Primary Causes Minimal Change Disease (MCD) Focal Segmental Glomerulosclerosis (FSGS) Causes of FSGS include genetic mutations, viruses (HIV, hepatitis B), and obesity. Membranous Nephropathy (MN) It is relatively rare in children but can be more common in adolescents. Congenital Nephrotic Syndrome This form of nephrotic syndrome presents at birth or in infancy and is due to genetic mutations, often involving the NPHS1 gene encoding nephrin. Secondary Causes Systemic Lupus Erythematosus (SLE) Hepatitis B and C Chronic viral infections can lead to nephrotic syndrome due to immune complex deposition. Infections Post-infectious glomerulonephritis, following infections such as streptococcal infections, can result in nephrotic syndrome. Amyloidosis Amyloidosis, although rare in children, can cause nephrotic syndrome. Abnormal protein deposits (amyloids) damage the kidneys. Diagnostic Approach Urinalysis The hallmark of nephrotic syndrome is proteinuria. A spot urine protein-to-creatinine ratio greater than 2 mg/mg is indicative of significant proteinuria. A 24-hour urine collection can also confirm protein loss (greater than 3.5 grams per day). Serum Tests Hypoalbuminemia (serum albumin less than 2.5 g/dL) and hyperlipidemia (elevated cholesterol and triglycerides) are typical. Kidney function: Serum creatinine and blood urea nitrogen (BUN) are often normal early in the disease but may increase in severe cases. Kidney Biopsy A kidney biopsy is essential in many cases to determine the underlying cause, especially in children older than 1 year, or when the child has atypical features. The biopsy can help differentiate between conditions like minimal change disease, focal segmental glomerulosclerosis, and membranous nephropathy. Immunological and Infectious Screen Tests for autoimmune diseases (ANA for lupus), hepatitis B and C, and HIV help identify secondary causes of nephrotic syndrome. Management of Nephrotic Syndrome in Pediatrics The management of nephrotic syndrome in children requires a combination of pharmacologic treatments, dietary interventions, and supportive care. The goals are to reduce proteinuria, manage edema, prevent infections, and treat the underlying cause of the disease. 1. Corticosteroids for Minimal Change Disease (MCD) Prednisone is the first-line treatment for minimal change disease, which is the most common cause of nephrotic syndrome in children. Induction phase: 2 mg/kg/day (up to 60 mg/day) of prednisone is given for 4 to 6 weeks. Tapering phase: Once remission is achieved, the dose is reduced gradually. Typically, after 4 to 6 weeks, the dose is decreased to 1.5 mg/kg/day (up to 40 mg/day) and then further tapered to 0.5 mg/kg/day (up to 20 mg/day). The total treatment duration may extend up to 6 months depending on the response and whether relapses occur. If a child relapses, the corticosteroid regimen is often restarted at the full dose. 2. Diuretics for Edema Management Children with significant edema may be treated with furosemide, a loop diuretic, to help reduce fluid retention. Furosemide is typically prescribed at a dose of 1-2 mg/kg/day, administered once or twice daily. The dose can be increased up to 6 mg/kg/day for severe edema or non-responsive cases, but careful monitoring is needed to prevent electrolyte imbalances. 3. Immunosuppressive Therapy for FSGS, MN, or Lupus Nephritis For children with focal segmental glomerulosclerosis or membranous nephropathy, or those with lupus nephritis, immunosuppressive medications are often required. Cyclophosphamide (for severe cases or steroid-resistant nephrotic syndrome): The typical dose is 2 mg/kg/day for 3-6 months, or in a pulse regimen of 500 mg/m2 every 3-4 weeks. Tacrolimus (Calcineurin Inhibitor): Initial dose: 0.1-0.2 mg/kg/day divided into two doses. Maintenance dose: Adjust according to therapeutic drug monitoring, aiming for trough levels of 5-15 ng/mL. Mycophenolate Mofetil (MMF): Typical starting dose: 600 mg/m2/day in two divided doses. Dose adjustment is based on the child’s tolerance and response. Rituximab (monoclonal antibody, often used for FSGS or steroid-resistant nephrotic syndrome): Dose: 375 mg/m2 as a single dose. 4. Supportive Care Angiotensin-converting enzyme inhibitors (ACE inhibitors) or Angiotensin II receptor blockers (ARBs): These are often used to reduce proteinuria and provide renal protection. Common drugs include enalapril or losartan, typically starting at 0.1 mg/kg/day and adjusting based on response. Antibiotic prophylaxis: In cases of high-risk infection (e.g., peritonitis, sepsis), prophylactic antibiotics may be used. #nephroticsyndrome #science