Today we talked about a patient presenting with bilateral leg edema and proteinuria. This brought up the topic of Nephrotic syndrome:
1) Nephrotic Syndrome:
- Characterized by nephrotic range proteinuria (usually more than 3 g/day), edema and hypoalbuminemia (<3g also="also" and="and" associated="associated" g--="g--" hypercoagulable="hypercoagulable" hyperlipidemia="hyperlipidemia" state.="state." with="with">
- Hyperlipidemia: Triglyceride rich lipoproteins increased in nephrotic syndrome due to decreased catabolism. This is likely due to decreased binding of lipoprotein lipase (LPL) to endothelial cells secondary to the reduced oncotic pressure.
- Hypercoagulable state: Likely secondary to an imbalance between naturally occuring pro-coagulant/pro-thrombotic factors and anti-coagulant and anti-thrombotic factors. Likely multifactorial, related to increased urinary losses of anti-thrombin, Protein C, Protein S and increased levels of fibrinogen. See this paper for a review of hypercoagulability and nephrotic syndrome
- Increased susceptibility to infections: unclear etiology, but may be related to urinary losses of IgG.
2) Etiology
- Focal segmental glomerulosclerosis: common cause of idiopathic nephrotic syndrome in adults
- Reflux nephropathy
- Nephron loss: surgical or congenital
- Intraglomerular hypertension from primary renal vasodilation: Diabetes, sickle cell disease
- Obesity
- Interferon
- Minimal change disease: Most common cause in children, also occurs in adults.
- Drugs: NSAIDs, antimicrobials (ampicllin, rifampin), penicillamine, lithium, sulfasalazine
- Paraneoplastic phenomena: Hodgkin's lymphoma, NHL, leukemia, rarely solid tumors
- Infectons: TB, syphilis, Hep C, HIV, erlichiosis, mycoplasma, echinococcus
- Systemic diseases: Diabetes, SLE, PCKD, HIV
- Allergy
- Membranous nephropathy
- Malignancy, typically solid tumor (GI, prostate, lung) and less frequently heme malignancy
- Infections: Hepatitis B/Hepatitis C, schistosomiasis, malaria, syphilis
- Autoimmune disease: SLE (lupus nephritis type V)
- Drugs: NSAIDs, penicillamine,
- Amyloidosis
- AL/primary amyloid: Light chain dyscrasia where fragments of monoclonal light chains form amyloid fibrils.
- AA/ Secondary amyloid: Secondary to chronic inflammation where the acute phase reactant serum amyloid A forms amyloid fibrils. Occurs for example in Rheumatoid Arthritis or osteomyelitis.
- In this case, urine dipstick will be negative and serum albumin is normal.
2 comments :
Unbelievable, I remembered I was once here in need of help on how to go about the problem of Nephrosis I was having, I must say those years was so frustrating, the experience was something outside the world of being normal,my wife had to join me in search of various kind of help,and the search lasted for 1 year and some months and like a dove sent from heaven, a friend of mine referred me to Dr Johnson a specialist with a difference full of knowledge, with his vaccines/medications and his advice on what to stay clear off and how to approach the problem, after one month of using his product, I was made well, I never believed there is a cure to Nephrosis, but now am a testimony.
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