and Minimal Change,
MPGN, IgA nephropathy (Bergers
- hypercoaguability (due to loss of Antithrombin III)
- Note: Many patients who have NS may display low serum sodium values,
primarily during relaspes. Hypernatremia is rarely observed in NS.
Etiologies: minimal change disease (most common), FSGS, MPGN, and associated
with systemic diseases (e.g., SLE, HSP, HUS). The first step in the evaluation is to obtain a thorough medical history and
family history. In general, diseases that cause NS are not familial in origin,
but the presence of disease in a family member may provide clues to the ultimate
Physical examination should focus on vital signs because severe NS may be
- increased respiratory rate (possible pulmonary edema or pneumonia)
- significant tachycardia (possible intravascular depletion)
- elevated blood pressure.
- Often present with asymptomatic edema
- Lungs: assess for pulmonary edema
- evaluation of heart sounds, especially for the S3 component (possible
- palpation of the abdomen (ascites, fluid wave, and tenderness may indicate
- assessment for the severity of peripheral edema
Look at the pee:
- If the diagnosis of NS is suspected, a urinalysis must be obtained.
evidence of proteinuria on urine dipstick: get
This may not aid in determining the etiology of NS, but it is a good reference
point for future comparison.
- Determination of the presence of red blood cells (RBCs)
in the urine is essential.
- Proteinuria in the absence
of hematuria suggests such glomerular
diseases, such as:
- minimal-change nephrotic syndrome (MCNS)
(note: 50% of children w/ MCNS may have RBCs intermittently)
- membranous nephropathy
- Children who have focal segmental glomerulosclerosis (FSGS)
also may exhibit RBCs in the urine sporadically.
- The persistence of RBCs
in the urine with proteinuria
must alert the physician to the presence of
in addition to nephrosis.
Nephrotic vs Nephritis Chart
- Diseases characterized by RBCs in the urine with or without proteinuria:
- infection, spontaneous bacterial peritonitis
- thrombosis because of hypercoagulability
Nephrotic Syndrome in Infants:
- prognosis is guarded; etiology is rarely minimal change before 6 months of
- often due to secondary causes (e.g., infections, drug reactions, toxins,
Congenital Nephrotic Syndrome:
- poor prognosis
- often donít respond to steroids or cytotoxic therapy
- many infectious complications
- Children who have NS almost uniformly are treated with oral steroids
- The response to such treatment aids in determining the etiology of the NS.
- 98% of
will experience complete resolution of edema and proteinuria after 4
weeks of oral steroid therapy.
persists after 4 weeks of therapy, as for the boy described in the
vignette, a diagnosis other than
MCNS must be considered.
Although a renal biopsy would be indicated at this point, the presence of RBCs
in the urine and findings on other laboratory tests may aid in the diagnosis.
suggests glomerulonephritis, including MPGN and IgAN.
hematuria, with or without
episodes of gross hematuria, always is present in children who have LN.
- The complete absence of
in the urine implies either FSGS or membranous nephropathy.
- Serum complement (C3,
C4) levels may be reduced in
PIAGN (usually only reduced C3),
Note: The incidence of FSGS
is on an exponential rise in children and must be suspected in any child who has
NS and does not undergo a remission within 4 weeks of beginning steroid therapy.
Kashgarian M, Hayslett JP, Seigel NJ. Lipoid nephrosis and focal
sclerosis: distinct entities or spectrum of disease. Nephron.
Levy M, Gonzalez-Burchard G, Broyer M, et al. Berger's disease in
children. Natural history and outcome. Medicine. 1985;64:157-180
Nephrotic syndrome in children: prediction of histopathology from
clinical and laboratory characteristics at time of diagnosis. A report
of the International Study of Kidney Disease in Children. Kidney Int.
Southwest Pediatric Nephrology Study Group. Comparison of idiopathic
and systemic lupus erythematosus-associated membranous
glomerulonephropathy in children. Am J Kidney Dis. 1986;7:115-124
Wyatt RJ, Forristal J, West CD, Sugimoto S, Curd JG. Complement
profiles in acute post-streptococcal glomerulonephritis. Pediatr