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Pediatric emergency medicine trisk 2905 2905

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MCD causing approximately 77% of cases and focal segmental
glomerulonephritis (FSGS) and MPGN the majority of the remaining
occurrences. Children typically present between the ages of 2 and 6 years,
and the reported ratio of boys to girls who are diagnosed at a younger age is
as high has 2:1. The gender ratio is closer to 1:1 in those who present later
in childhood or as adolescents.

Goals of Treatment
Many children with nephrotic syndrome present to the ED with signs of
fluid overload. Initial management should focus on improving fluid balance
while monitoring for signs of intravascular volume depletion. Children
should also be assessed for underlying complications of nephrotic syndrome
such as infection and thrombosis. If a diagnosis of nephrotic syndrome has
not been established in the past, an initial workup for potential underlying
causes may be initiated.

Clinical Considerations
Clinical recognition. Nephrotic syndrome results when there is increased
permeability across the glomerular filtration barrier. It is characterized by
hypoproteinemia, edema, hyperlipidemia, and massive proteinuria
exceeding 50 mg/kg/day. Hypertension may be present, especially in the
setting of FSGS, but is often absent in MCD. Edema, often the most
noticeable clinical manifestation of nephrotic syndrome, is the result of
excessive salt and water retention. Periorbital edema is often the initial
finding and may be misdiagnosed as signs of allergy. The associated edema
is gravity dependent and therefore will vary in location based on patient
position and activity. Upon awakening, edema may be more marked in the
face and then shift to the lower extremities with ambulation. It may also be
notable in the scrotal or vulvar regions. Other complications of third
spacing, such as ascites, pulmonary edema, and pleural effusions, may also
occur.


Although children with nephrotic syndrome and edema have total body
sodium and water excess, some will present with evidence of intravascular
depletion. This is more likely to occur in those with severe
hypoalbuminemia and will be exacerbated by diuretic use, gastrointestinal



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