Nephrotic Syndrome Pediatric Flashcards
Nephrotic Syndrome Clinical Features
Proteinuria (Nephrotic range proteinuria — Urinary protein excretion greater than 50 mg/kg per day) Hypoalbuminemia (aka hypoproteinemia) Edema Hyperlipidemia
Causes
Acute or Transient
Chronic and Progressive
Relapsing and Remitting
Not a disease itself, but the result of many diseases
Acute or Transient: postinfectious glomerulonephritis Chronic and Progressive: focal segmental glomerulsclerosis (FSGS)
Relapsing and Remitting: minimal change nephrotic syndrome (MCNS)
Primary Nephrotic Syndrome
refers to nephrotic syndrome in the absence of an identifiable systemic disease.
Within this category are patients with idiopathic nephrotic syndrome, who have no glomerular inflammation on renal biopsy, and patients with primary glomerulonephritis, who have an active sediment and glomerular inflammation on renal biopsy
Secondary Nephrotic Syndrome
Secondary nephrotic syndrome- nephrotic syndrome in the presence of an identifiable systemic disease.
Postinfectious glomerulonephritis and infective endocarditis
Systemic lupus erythematosus
Vasculitides, such as Henoch-Schönlein purpura, and, rarely, in Wegener’s granulomatosis
Other causes: sickle cell disease, Alport syndrome, hemolytic uremic syndrome
Congenital or Infantile Nephrotic Syndorme
occur in children less than one year of age and can be either secondary (mostly due to infection) or primary.
Epidemiology
Nephrotic syndrome occurs as a result of immune, systemic, nephrotoxic, allergic, infectious, malignant, vascular, or idiopathic processes.
Primary mechanism is believed to be immunologic rather than renal.
Incidence
2 to 3 per 100,000 with a 15 times greater incidence in children than in adults.
90% of children with nephrotic syndrome have a form of idiopathic nephrotic syndrome with a peak incidence at 2-6 years of age.
85% “steroid responders” = prednisone used to reduce remission. Treat w/steroids x4-6 weeks until proteinuria is resolved
In younger children, male:female ratio is 1:2 incidence is more equal by mid-adolescence
Clinical Findings
Edema: cardinal clinical feature esp periorbital edema may occur soon after infection or insect bite detectable when fluid retention exceeds 3-5% of body wt Low urine production
Gastrointestinal Symptoms: anorexia, paleness, listlessness, diarrhea, vomiting, abdominal pain (RUQ) Recent prodromal infection
Respiratory difficulties secondary to ascites, effusion, pneumonia, if advanced disease.
Dx Labs
U.A. and microscopic examination (protein of 2+ or greater, hyaline and fine granular casts, microhematuria (in 33%), elevated specific gravity, fat bodies, and casts in urine).
Quantitative urine protein excretion (24-hour collection or protein-creatinine ratio on a random first morning urine).
CBC, electrolytes, BUN, creatinine (normal), calcium, serum albumin (less than 2g/dL), total protein, liver enzymes, triglycerides, lipoproteins, cholesterol (elevated); C3 and C4 (normal); ANA.
Dx Studies
Kidney Bx recommended if:
If criteria for Minimal Change Nephrotic Syndrome (MCNS) aka primary nephrotic syndrome, unrelated to systemic disease are not met.
If systemic disease is present In the presence of hematuria and hypertension
With hypocomplementemia or nonselective proteinemia If the patient is older than 7 years or an adolescent
If the patient is nonresponsive to steroids If relapses are frequent
DD
hypoproteinemia from starvation, liver disease, and protein-losing enteropathy; none of these includes associated proteinuria
Also consider glomerulonephritis Infants (newborn-1yr): usually have congenital renal problem
Children 7yrs and older: likely to have focal glomerulosclerosis or mesangeal proliferative glomerulonephritis
Teens: membranous neuropathy
Management
Consultation with and/or referral to a nephrologist! Hospitalization may be necessary initially
Prednisone (2mg/kg/day; maximum 60 mg/day) to induce remission, which can occur as early as 14 days as evidenced by diuresis.
Steroids for at least 4-6 weeks
Pt Education
Importance of continued, regular care to monitor renal function.
Relapses are the rule.
An understanding of the disease process, side effects of steroids, recognition of infection, and the importance of monitoring proteinuria for relapses is crucial.
Prognosis is good in steroid responders, with relapses that decrease in frequency as the child grows older without any residual renal dysfunction.
Complications
Children with nephrotic syndrome as susceptible to pneumococcal, E.Coli, Pseudomonas, and H. influenzae infection because of stasis of fluid (seen as peritonitis, pneumonia, cellulitis, or septicemia).
Hypertension or hypotension is a possiblity. Thomboembolism is a possiblity (due to hypercoaguable state).
