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'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
__NOTOC__
 
{{Infobox_Disease |
  Name          = Nephrotic syndrome |
  Image          = |
  Caption        = |
  DiseasesDB    = 8905 |
  ICD10          = {{ICD10|N|04||n|00}} |
  ICD9          = {{ICD9|581.9}} |
  ICDO          = |
  OMIM          = |
  MedlinePlus    = |
  MeshID        = D009404 |
}}
 
{{Nephrotic syndrome}}
{{Nephrotic syndrome}}


{{CMG}} '''Associate Editor-In-Chief:''' {{CZ}}
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
 
==Overview==
 
:''Not to be confused with [[nephritic syndrome]]''
'''Nephrotic syndrome''' is a nonspecific disorder in which the [[kidney]]s are damaged, causing them to leak large amounts of [[protein]] (at least 3 grams per day) from the [[blood]] into the [[urine]].
 
==Presentation==
It is characterised by proteinuria (>3.5g/day), hypoalbuminemia, hyperlipidemia and edema. A few other characteristics are:


* The most common sign is excess fluid in the body. This may take several forms:
{{CMG}}; {{APM}} {{AE}}{{OO}} {{CZ}}, [[User:YazanDaaboul|Yazan Daaboul]], [[User:Sergekorjian|Serge Korjian]]
** Puffiness around the [[eyes]], characteristically in the morning.
** [[Edema]] over the [[leg]]s which is ''pitting'' (i.e. leaves a little pit when the fluid is pressed out, which resolves over a few seconds).
** Fluid in the [[pleural cavity]] causing [[pleural effusion]].
** Fluid in the [[peritoneal cavity]] causing [[ascites]].
* [[Hypertension]] (rarely)
* Some patients may notice foamy [[urine]], due to a lowering of the [[surface tension]] by the severe [[proteinuria]]. Actual urinary complaints such as [[hematuria]] or [[oliguria]] are uncommon, and are seen commonly in [[nephritic syndrome]].
* May have features of underlying cause, such rash associated with SLE, or neuropathy with diabetes.
* Examination should also exclude other causes of gross edema- especially the cardiovascular and hepatic system.


===Maltese cross===
==[[Nephrotic syndrome overview|Overview]]==
The classic Maltese cross pattern is evident in fatty casts with polarized microscopy because of the birefringence of the lipid. <ref> http://www.gamewood.net/rnet/renalpath/ch3.htm  The Nephrotic Syndrome access date=2007-11-20</ref>  Maltese crosses are due to cholesterol, which is increased in nephrotic syndrome.


==Investigations==
== [[Nephrotic syndrome historical perspective|Historical Perspective]] ==
The following are baseline, essential investigations
* Urine sample shows [[proteinuria]]. It is also examined for active casts; which is more a feature of active nephritis.
* [[Hypoalbuminemia]]: albumin levels in blood < 30g/L
* High levels of [[cholesterol]] ([[hypercholesterolemia]]), specifically elevated [[Low density lipoprotein|LDL]], usually with concomitantly elevated [[Very low density lipoprotein|VLDL]]
* Electrolytes, urea and creatinine (EUCs): to evaluate renal function


Further investigations are indicated if the cause is not clear
==[[Nephrotic syndrome classification|Classification]]==
* Biopsy of kidney (not usually done in children)
* Auto-immune markers ([[Anti-nuclear antibody|ANA]], [[Antistreptolysin O titre|ASOT]], [[C3 (complement)|C3]], [[cryoglobulin]]s, [[Serum protein electrophoresis|serum electrophoresis]])


==Pathogenesis==
==[[Nephrotic syndrome pathophysiology|Pathophysiology]]==
The [[glomerulus (kidney)|glomeruli]] of the kidneys are the parts that normally filter the blood. They consist of [[capillaries]] that are fenestrated (leaky, due to little holes called ''fenestrae'' or windows) and that allow fluid, salts, and other small solutes to flow through, but normally not proteins.


In nephrotic syndrome, the glomeruli become damaged due to inflammation and hyalinisation so that small proteins, such as [[albumins]] immunoglobulins and anti-thrombin can pass through the kidneys into urine.
==[[Nephrotic syndrome causes|Causes]]==


Albumin is the major protein in the blood which maintains colloid osmotic pressure- this prevents leakage of blood from vessels into tissue. However, experiments show that the edema formation in nephrotic syndrome is more so due to microvascular damage and intense salt and water retention by the damaged kidneys (due to increased angiotensin secretion). The mechanism is very complex and still not fully understood.
==[[Nephrotic syndrome differential diagnosis|Differentiating Nephrotic Syndrome from other Diseases]]==


In response to leakage of albumin, the liver begins to make more of all its proteins, and levels of large proteins (such as [[alpha 2-macroglobulin]] and lipoproteins) increase. The excess lipoproteins end up in the urine filtrate, which is then rebsorbed by the tubular cells, which end up shedding and forming oval fat bodies or [[urinary casts#fatty casts|fatty casts]].
==[[Nephrotic syndrome epidemiology and demographics|Epidemiology and Demographics]]==


==Classification and causes==
==[[Nephrotic syndrome risk factors|Risk Factors]]==
Nephrotic syndrome has many causes and be the result of a disease limited to the kidney, called ''primary'' nephrotic syndrome, or a condition that affects the kidney and other parts of the body, called ''secondary'' nephrotic syndrome.


===Etiologic classification===
==[[Nephrotic syndrome screening|Screening]]==
A broad classification of nephrotic syndrome based on etiology:
<center>
{{familytree/start}}
{{familytree | | | |A11| | | | |A11 =Nephrotic<br>syndrome}}
{{familytree | |,|-|-|^|-|-|.|}}
{{familytree | B11 | | | | B12 |B11=Primary|B12=Secondary}}
{{familytree/end}}
</center>


===Histologic classification===
==[[Nephrotic syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
Nephrotic syndrome is often classified histologically:
<center>
{{familytree/start}}
{{familytree | | | | | | | | | A01 | | | | | |A01=Nephrotic<br>syndrome}}
{{familytree | | | | |,|-|-|-|-|+|-|-|-|-|.| |}}
{{familytree | | | | B01 | | | B02 | | | B03|B01=[[Minimal change disease|MCD]]|B02=[[focal segmental glomerulosclerosis|FSGS]]|B03=[[membranous nephropathy|MN]] }}
{{familytree/end}}
</center>
===Primary causes===
Primary causes of nephrotic syndrome are usually described by the [[histology|histology]], i.e. [[minimal change disease]] (MCD), [[focal segmental glomerulosclerosis]] (FSGS) and [[membranous nephropathy]] (MN). 


They are considered to be "[[Diagnosis of exclusion|diagnoses of exclusion]]", i.e. they are diagnosed only after secondary causes have been excluded. 
==Diagnosis==
 
[[Nephrotic syndrome diagnostic study of choice|Diagnostic Study of Choice]] | [[Nephrotic syndrome history and symptoms| History and Symptoms]] | [[Nephrotic syndrome physical examination | Physical Examination]] | [[Nephrotic syndrome laboratory findings|Laboratory Findings]] | [[Nephrotic syndrome electrocardiogram|Electrocardiogram]] | [[Nephrotic syndrome chest X ray|Chest X ray]] | [[Nephrotic syndrome echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Nephrotic syndrome CT|CT Scan]] | [[Nephrotic syndrome MRI|MRI]] | [[Nephrotic syndrome other imaging findings|Other Imaging Findings]] | [[Nephrotic syndrome other diagnostic studies|Other Diagnostic Studies]]
===Secondary causes===
Secondary causes of nephrotic syndrome have the same histologic patterns as the primary causes, though may exhibit some differences suggesting a secondary cause.
 
They are usually described by the underlying cause.
 
====Secondary causes by histologic pattern====
Membranous nephropathy (MN)<ref name=fogo>Fogo AB, Bruijn JA. Cohen AH, Colvin RB, Jennette JC. Fundamentals of Renal Pathology. Springer. ISBN 978-0387-31126-5.</ref>
*[[Hepatitis B]]
*[[Sjogren's syndrome]]
*[[Systemic lupus erythematosus]] (SLE)
*[[Diabetes mellitus]]
*[[Sarcoidosis]]
*[[Syphilis]]
*[[Drugs]]
*[[Malignancy]] (cancer)
 
Focal segmental glomerulosclerosis (FSGS)<ref name=fogo/>
*[[Human immunodeficiency virus]] (HIV)
*[[Diabetes mellitus]]
*[[Obesity]]
*Kidney loss
 
Minimal change disease (MCD)<ref name=fogo/>
*[[Drugs]]
*[[Malignancy]], especially [[Hodgkin's lymphoma]]
 
== Complete Differential Diagnosis of Nephrotic Syndrome==
===GN with Nephrotic Syndrome===
 
*[[Captopril]]
*[[Cancer]]
*[[Diabetes Mellitus]]
*[[Drugs]]
*[[Hepatitis B]]
*[[Hodgkin's Lymphoma]]
*[[Mixed Connective Tissue Disease]]
*[[Penicillamine]]
*[[Streptococcal]] throat infection
*[[Systemic Lupus Erythematosus]]
*[[Thyroiditis]]
 
===Acute Glomerulonenephritis===
*[[Berger's Disease]]
*[[Goodpasture's Syndrome]]
*[[Henoch-Schonlein Syndrome]]
*[[Streptococcal Infection]]
*Shunt infections
*Subacute [[Endocarditis]]
*[[Systemic Lupus Erythematosus]]
*[[Wegener's Granulomatosis]]
 
===Secondary===
*[[Alport's Syndrome]]
*[[Amyloidosis]]
*Congenital [[Nephrotic Syndrome]]
*[[Dermatomyositis]]
*[[Diabetes Mellitus]]
*[[Drugs]]
*[[Endocarditis]]
*[[Fabry's Disease]]
*[[Goodpasture's Syndrome]]
*[[Henoch-Schonlein purpura]]
*[[Hepatitis]] A,B,C
*[[HIV]]
*[[Infectious Mononeucliosis]]
*[[Lipoatrophy]]
*[[Lymphomas]]
*[[Malaria]]
*[[Multiple Myeloma]]
*Orthostatic [[Proteinuria]]
*[[Polyarteritis Nodosa]]
*[[Rheumatoid arthritis]]
*[[Sarcoidosis]]
*[[Sickle Cell Anemia]]
*[[Sjogren's Syndrome]]
*[[Streptococcal Infection]]
*[[Syphillis]]
*[[Systemic Lupus Erythematosus]]
*[[Toxoplasmosis]]
*[[Vasculitis]]
 
===Miscellaneous===
*Chronic interstitial nephritis
*[[Graft vs. Host Disease]]
*[[Hypothyroidism]]
*[[Pre-eclampsia]]
*[[Renal vein thrombosis]]
*Severe [[Obesity]]
*[[Thyroiditis]]
 
==Differential diagnosis of gross edema==
When someone presents with generalised edema, the following causes should be excluded
 
1) Heart failure:
The patient is older, with a history of heart disease.
 
Jugular venous pressure is elevated on examination, might hear heart murmurs
 
An echocardiogram is the gold standard investigation
 
2) Liver failure:
History suggestive of hepatitis/ cirrhosis: alcoholic, IV drug user, some hereditary causes
 
Stigmata of liver disease are seen: dilated veins over umbilicus (caput medusae), scratch marks, enlarged spleen, spider angiomata, encephalopathy, bruising, nodular liver
 
3) Acute fluid overload in someone with kidney failure:
These people are known to have kidney failure, and have either drunk too much or missed their dialysis.
 
4) Metastatic cancer:
When cancer seeds the lungs or abdomen it causes effusions and fluid accumulation due to obstruction of lymphatics and veins as well as serous exudation.


==Treatment==
==Treatment==
Treatment includes:
[[Nephrotic syndrome medical therapy|Medical Therapy]] | [[Nephrotic syndrome surgery|Surgery]] | [[Nephrotic syndrome primary prevention|Primary Prevention]] | [[Nephrotic syndrome secondary prevention|Secondary Prevention]] | [[Nephrotic syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Nephrotic syndrome future or investigational therapies|Future or Investigational Therapies]]
===A) General measures (supportive)===


Monitoring and maintaining euvolemia (the correct amount of fluid in the body)
==Case Studies==
[[Nephrotic syndrome case study one|Case #1]]


- monitoring urine output, BP regularly
[[Category:Medicine]]
 
- fluid restrict to 1L
 
- diuretics (IV furosemide)
 
Monitoring kidney function
 
-do EUCs daily and calculating GFR
 
Prevent and treat any complications [see below]
 
Albumin infusions are generally not used because their effect lasts only transiently.
 
===B) Specific treatment of underlying cause===
 
Immunosupression for the glomerulonephritides ([[steroid]]s,<ref name="pmid17943754">{{cite journal |author=Hodson E, Willis N, Craig J |title=Corticosteroid therapy for nephrotic syndrome in children |journal=Cochrane database of systematic reviews (Online) |volume= |issue=4 |pages=CD001533 |year=2007 |pmid=17943754 |doi=10.1002/14651858.CD001533.pub4}}</ref> [[cyclosporin]])
 
Standard ISKDC Regime for first episode:Prednisolone -60mg/m<sup>2 </sup>/day in 3 divided doses for 4weeks followed by 40mg/m<sup>2</sup>/day in a single dose on every alternate day for 4 weeks.
 
Relapses by prednisolone 2mg/kg/day till urine becomes negetive for protein.Then,1.5mg/kg/day for 4 weeks.
 
Frequent Relapses treated by:cyclophosphamide or nitrogen mustard or cyclosporin or levamisole.
 
Achieving stricter blood glucose control if diabetic
 
BP control. ACE inhibitors are the drug of choice. Independent of their blood pressure lowering effect, they have been shown to decrease protein loss.
 
===C) Dietary recommendations===
 
Limit high protein animal foods to 1 oz per meal (prefferably lean cuts of meat, fish, and poultry)
 
Limit high phosphorous foods such as cheese, cooked dried beans and peas, nut butters, soy, tofu, and yogurt, including cokes and colas.
 
Limit high potassium vegetables and fruits such as artichokes, avocado, bamboo shoots, beets, brussels sprouts, chard, greens (such as beet and collards), kohlrabi, okra, parsnips, potatoes, pumpkin, rutabagas, spinach, sweet potatoes, tomatoes, tomato juice, tomato sauce, wax beens, winter squash, yams. Fruits include, apricots, bananas, dates, honey dew, nectarines, orange juice, oranges, prune juice.
 
Avoid saturated fats and eat unsaturated fats in moderation.
 
Eat low-fat desserts only.
 
Monitor fluid intake which includes all fluids and foods that are liquid at room temperature.
 
==Complications==
[[Renal vein thrombosis]]: In [[nephrotic syndrome]] there is an excessive urinary protein loss which is in turn associated with decreased [[antithrombin III]]. This leads to a relative rise in the activity of [[Factor II]] and [[Factor X]] and in increased tendency to [[thrombosis]]. [[Renal vein thrombosis]] is a manifestation of this hypercoagulable state. It is unclear why the renal vein is susceptible to thrombosis in patients with nephrotic syndrome.
 
[[Infection]]: due to leakage of [[immunoglobulin]]s, encapsulated bacteria such as ''[[Haemophilus influenzae]]'' and ''[[Streptococcus pneumonia]]'' can cause infection.
 
[[Acute renal failure]] is due to hypovolemia. Despite the excess of fluid in the tissues, there is ''less'' fluid in the vasculature (the patient is intravascularly deplete]]. Decreased blood flow to the kidneys causes pre renal azotemia. Thus it is therapeutic challenge to reduce the total body fluid overload while maintaining ciculalatory euvolemia.
 
[[Pulmonary edema]]: again due to fluid leak associated with the low protein content of the serum, there can be non cardiogenic pulmonary edema causing [[hypoxia]] and [[dyspnea]].
 
[[Growth retardation]]: does not occur in MCNS. It occurs in cases of relapses or resistance to therapy.Causes of growth retardation are protein deficiency(loss of protein in urine), anorexia(reduced protein intake), steroid therapy(catabolism). <ref>Brenner, Barry M. (editor) Brenner & Rector's The Kidney, seventh edition W.B. Saunders Company 2004 ISBN 0-7216-0164-2 </ref>
 
==Prognosis==
The prognosis depends on the cause of nephrotic syndrome. It is usually good in children, because [[minimal change disease]] responds very well to [[steroids]] and does not cause [[chronic renal failure]]. However other causes such as [[focal segmental glomerulosclerosis]] frequently lead to end stage renal disease. Factors associated with a poorer prognosis in these cases include level of [[proteinuria]], [[blood pressure]] control and kidney function (GFR).
 
==References==
{{reflist|2}}
 
==External links==
*[http://www.nephcure.org NephCure Foundation] Only organization solely committed to support research seeking the cause of Nephrotic Syndrome and FSGS, improve treatment and find the cure.
*[http://kidney.niddk.nih.gov/kudiseases/pubs/childhoodnephrotic/ Childhood Nephrotic Syndrome] - National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), [[NIH]]
*[http://kidney.niddk.nih.gov/kudiseases/pubs/nephrotic/ Adult Nephrotic Syndrome] - National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), [[NIH]]
*[http://www.kidcomm.org KidComm] - An online resource for parents dealing with childhood kidney diseases (FSGS, Nephrotic Syndrome and others). Features an email support group for parents plus an email support group for adults with kidney disease (YakTalk).
*[http://www.asnit.it Associazione sindrome nefrosica italia onlus] - Italian organization  dedicated to support patients and parents dealing with nephrotic syndrome
 
{{Nephrology}}
 
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Urology]]
[[Category:Syndromes]]
[[Category:Up-To-Date]]
 
[[de:Nephrotisches Syndrom]]
[[es:Síndrome nefrótico]]
[[fr:Syndrome néphrotique]]
[[hr:Nefrotski sindrom]]
[[nl:Nefrotisch syndroom]]
[[ja:ネフローゼ症候群]]
[[pl:Zespół nerczycowy]]
[[pt:Síndrome nefrótica]]
[[sv:Nefrotiskt syndrom]]
[[zh:肾病综合症]]
 


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Latest revision as of 06:35, 28 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3] Cafer Zorkun, M.D., Ph.D. [4], Yazan Daaboul, Serge Korjian

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Nephrotic Syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X ray | Echocardiography or Ultrasound | CT Scan | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1