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PubMed Central
Other literature type . 2024
License: CC BY
Data sources: PubMed Central
Cureus
Article . 2024 . Peer-reviewed
Data sources: Crossref
Cureus
Article
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Nephrotic Syndrome Without Nephrotic Range Proteinuria

Authors: Sheikh, Jibran A; Syed, Usheem; Osama, Sayed M;

Nephrotic Syndrome Without Nephrotic Range Proteinuria

Abstract

Nephrotic syndrome in adults is defined as nephrotic-range (≥3.5g/24h) proteinuria with low serum albumin, usually associated with edema, hyperlipidemia, and lipiduria. The 3.5g/24h threshold was selected arbitrarily and might not be reached in certain cases despite severe defects in glomerular permeability. We describe the case of a 57-year-old male who presented with progressively worsening swelling involving his limbs and abdomen. He also reported decreased urine output and fatigue. Physical examination was notable for severe pitting edema over legs, arms, and abdomen, in addition to peri-orbital puffiness. Labs revealed low serum albumin (1.3 g/dL), moderate proteinuria (2.3g/24h), and elevated total cholesterol (334 mg/dL). Renal biopsy showed amyloid light chain (AL) amyloidosis and bone marrow biopsy confirmed the presence of lambda-restricted plasma cells. Computed tomography, ultrasound, elastography, and laboratory findings were congruent with those seen in hepatic amyloidosis. A diagnosis of nephrotic syndrome caused by systemic AL amyloidosis was made despite the absence of nephrotic range proteinuria. The primary abnormality in nephrotic syndrome is increased glomerular permeability, leading to severe proteinuria causing low serum albumin, decreased oncotic pressure, and increased water retention by kidneys due to activation of the epithelial sodium channel (ENaC). The amount of albuminuria is influenced by both the extent of glomerular permeability and the rates of glomerular filtration and albumin synthesis. In cases where albumin synthesis is decreased secondary to concurrent liver disease, as in our case, a steady state of renal protein excretion may be reached at a lower threshold than 3.5g/24h despite severe defects in glomerular permeability.

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Internal Medicine

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
0
Average
Average
Average
Green