Question 1 of 10

    Nephrotic Syndrome (Nephrotic Syndromes)

    Diabetic glomerulosclerosis (1) HE[1]

    This is a nonspecific disease of the kidney characterized by proteinuria, edema and hypoalbuminemia. It results from increased permeability of the glomerulus leading to massive loss of protein in the urine.

    Nephrotic Syndrome is related to the following process: auto-immune.

    Presentation

    The commonest presenting features are those due to fluid retention. Facial edema is usually the first sign of nephrotic syndrome in children. This is followed be generalized swelling of the whole body known as anasarca. There could be ascites, pleural effusion, and pitting pedal edema. The patient could also present with frothy urine. There could be dyspnea and cough. In cases of an underlying disease, patients will present with symptoms of that disease e.g. rash in patients with lupus.

    On presentation, the cardiovascular and hepatic systems should be evaluated to rule out other causes of generalized edema [7].

    Entire body system
    Fatigue
    • Dr Vishwanth urged parents to look out for early symptoms of kidney ailments in their children, such as sudden weight gain, unexplained anaemia and weakness, fatigue and recurring urinary infection.[thenewsminute.com]
    • Symptoms may include: Fatigue and malaise Decreased appetite Weight gain and facial swelling Abdominal swelling or pain Foamy urine Fluid accumulation in the body spaces (edema) Pale fingernail beds Dull hair Ears cartilage may feel less firm Food intolerances[childrensnational.org]
    Leg Edema
    • Edema — Swelling in the lower legs (edema) and collection of fluid in the abdomen (ascites) can occur in people with nephrotic syndrome.[uptodate.com]
    Leg Swelling
    • Protein loss causes low protein count in the blood (hypoalbuminemia) and edemas (excess fluid in the interstitial cavity which is between the cells, causes leg swelling most often, and also in the adbomen and around the eyes).[medical-dictionary.thefreedictionary.com]
    Malaise
    • […] that may occur include: Blurred vision , usually from burst blood vessels in the retina of the eye Cough containing mucus or pink, frothy material from fluid buildup in the lungs Shortness of breath, from fluid buildup in the lungs General ill feeling (malaise[nlm.nih.gov]
    • Symptoms may include: Fatigue and malaise Decreased appetite Weight gain and facial swelling Abdominal swelling or pain Foamy urine Fluid accumulation in the body spaces (edema) Pale fingernail beds Dull hair Ears cartilage may feel less firm Food intolerances[childrensnational.org]
    • The following are the most common symptoms of nephrotic syndrome: Extreme tiredness (fatigue) and a general feeling of discomfort (malaise) Decreased appetite Weight gain and facial swelling Belly swelling or pain Foamy urine Fluid buildup in the body[urmc.rochester.edu]
    • Symptoms of nephrotic syndrome The symptoms of nephrotic syndrome include: foamy and frothy urine unexplained weight loss general malaise (feeling unwell) oedema (fluid retention or swelling), particularly around the abdomen (belly area), legs and eyes[betterhealth.vic.gov.au]
    Weight Gain
    • gain Specific adverse reactions reported in IS clinical trials in infants and children under 2 years of age included: infection, hypertension, irritability, Cushingoid symptoms, constipation, diarrhea, vomiting, pyrexia, weight gain, increased appetite[actharnephrology.com]
    • Prednisone can cause increased appetite mood changes; he may be moody or irritable while taking the medicine weight gain Weigh your child as directed and record his weight.[nationwidechildrens.org]
    • Weight gainWeight gain can occur in people who develop swelling.[uptodate.com]
    • Weight gain (from fluid buildup).[cigna.com]
    • Symptoms may include: Fatigue and malaise Decreased appetite Weight gain and facial swelling Abdominal swelling or pain Foamy urine Fluid accumulation in the body spaces (edema) Pale fingernail beds Dull hair Ears cartilage may feel less firm Food intolerances[childrensnational.org]
  • more...
  • Face, Head & Neck
    Facial Swelling
    • Symptoms may include: Fatigue and malaise Decreased appetite Weight gain and facial swelling Abdominal swelling or pain Foamy urine Fluid accumulation in the body spaces (edema) Pale fingernail beds Dull hair Ears cartilage may feel less firm Food intolerances[childrensnational.org]
    • The following are the most common symptoms of nephrotic syndrome: Extreme tiredness (fatigue) and a general feeling of discomfort (malaise) Decreased appetite Weight gain and facial swelling Belly swelling or pain Foamy urine Fluid buildup in the body[urmc.rochester.edu]
  • more...
  • neurologic
    Headache
    • […] including swelling in face, belly, or arms and legs, or skin sores You're being treated for nephrotic syndrome, but your symptoms don't improve New symptoms develop, including cough , decreased urine output , discomfort with urination , fever , severe headache[nlm.nih.gov]
    • […] burst blood vessels in the retina of the eye Cough containing mucus or pink, frothy material from fluid buildup in the lungs Shortness of breath, from fluid buildup in the lungs General ill feeling (malaise), drowsiness , confusion , aches and pains, headache[nlm.nih.gov]
  • more...
  • Skin
  • more...
  • gastrointestinal
    Abdominal Pain
    • The patient may note abdominal pain and difficulty breathing.[encyclopedia.com]
    • Think of spontaneous bacterial peritonitis in patient with fever and abdominal pain.[pedemmorsels.com]
    • Anorexia, abdominal pain and diarrhea may be seen, possibly secondary to the formation of ascites.[hawaii.edu]
    • Symptoms include vomiting, abdominal pain, and diarrhea.[emedicine.medscape.com]
    Loss of Appetite
    • Other common signs include loss of appetite, diarrhea , less frequent urination, and urine that looks dark and foamy.[kidshealth.org]
    • However, as kidney function continues to worsen, symptoms of kidney failure can develop, including shortness of breath, weakness and easy fatigability (from anemia) and loss of appetite.[uptodate.com]
  • more...
  • cardiovascular
  • more...
  • urogenital
    Oliguria
    • As mentioned previously, the nephritic syndrome ischaracterized by haematuria (which is absent in nephrotic syndrome) as well asuraemia, hypertension, proteinuria and oliguria.[newhealthadvisor.com]
    • The signs include cold hands and feet, delayed capillary filling, oliguria, and tachycardia.[emedicine.medscape.com]
    • Actual urinary complaints such as haematuria or oliguria are uncommon, though these are seen commonly in nephritic syndrome.[en.wikipedia.org]
  • more...
  • Eyes
  • more...
  • Workup

    • Laboratory investigations are the mainstay of diagnosis and they center on urine investigations. The first test should be urinalysis, 3 or 4 pluses of urine on the dipstick corresponds to at least 3g/L of protein and this is well within the nephrotic syndrome range. The presence of glucose should prompt further investigation of diabetes.
    • Urine sediment examination under a microscope with show waxy cast which indicates a proteinuric disease. It will also show red blood cells which indicates glomerular disease [8].
    • Measurement of 24-hour protein will reveal > 150mg of protein which is more than normal for a healthy kidney. Urine protein electrophoresis will show the exact protein and proteinuria without albumin may rule our nephrotic syndrome.
    • Serum electrolyte, urea and creatinine should also be checked to determine renal function.
    • A renal ultrasound can also be done and high echogenicity of the kidney is a pointer to chronic kidney disease. It will also show if a patient has one or two kidneys. One kidney is a contraindication for renal biopsy which may be done if the nephrotic syndrome is of unknown origin.

    Pathology

    Biopsy
  • more...
  • Laboratory

    Serum
    Beta Globulin Decreased
    • Lab Increased alpha2-globulin, increased beta-globulin, decreased albumin, increased cholesterol, increased TGs, increased phospholipids; these increases are confined to lipoproteins containing apoB (chylomicrons and LDL-C), due to increased production[medical-dictionary.thefreedictionary.com]
    Globulins Increased
    • Lab Increased alpha2-globulin, increased beta-globulin, decreased albumin, increased cholesterol, increased TGs, increased phospholipids; these increases are confined to lipoproteins containing apoB (chylomicrons and LDL-C), due to increased production[medical-dictionary.thefreedictionary.com]
  • more...
  • Urine
  • more...
  • Pleura
    Pleural Effusion
    • Fluid in the pleural cavity causing pleural effusion.[en.wikipedia.org]
    • Fluid retention may cause Dyspnea (pleural effusion or laryngeal edema) Arthralgia (hydrarthrosis) Abdominal pain (ascites or, in children, mesenteric edema) Corresponding signs may develop, including peripheral edema and ascites.[merckmanuals.com]
    • As the edema accumulates, pleural effusions, ascites and decreased urine output may develop.[hawaii.edu]
    • Pleural effusions may cause chest pain and breathlessness.[patient.info]
    • Additional consequences include the following: Hypertension related to fluid retention and reduced kidney function may occur Edema of the gut may cause defective absorption, leading to malnutrition Ascites and pleural effusions may develop Infection Infection[emedicine.medscape.com]
  • more...
  • Imaging

    X-ray
  • more...
  • Treatment

    • The treatment of nephrotic syndrome is directed at the specific cause of the disease.
    • In minimal change nephropathy, glucocorticoids are used. Children who do not respond to glucocorticoid treatment should be given rituximab. Rituximab also has application in the treatment of adult membranous nephropathy [9].
    • Anti-inflammatory treatment is used in amyloidosis and cyclophosphamide along with prednisone for lupus.
    • In patients with membranous nephropathy who have a low risk of progression, expectant management without immunosuppression can be employed.
    • Diuretics are used to control edema, antibiotics for infections, anticoagulation therapy to decrease risk of clots and the diet should be rich in protein and calories.

    Prognosis

    Treatment greatly reduces the morbidity and mortality associated with this condition. The prognosis is however dependent on the cause of the nephrotic syndrome. It is poor in infants with congenital nephrotic syndrome as they hardly survive beyond months. The best prognosis is in patients with minimal change nephropathy. Mesangiocapillary glomerulonephrits carries the worst prognosis and patients develop renal failure within 3 years. Without treatment, this condition carries a very bad prognosis especially in adults over 30 years [6].

    Complications

    Acute Glomerulonephritis
    • Glomerulonephritis - acute; Acute glomerulonephritis; Nephritis syndrome - acute Appel GB, Radhakrishnan.[nlm.nih.gov]
    Anasarca
    Ascites
    • Clinical presentation Patients diagnosed with nephrotic syndrome most commonly present for abdominal distention secondary to ascites.[veterinarycalendar.dvm360.com]
    • Fluid retention may cause Dyspnea (pleural effusion or laryngeal edema) Arthralgia (hydrarthrosis) Abdominal pain (ascites or, in children, mesenteric edema) Corresponding signs may develop, including peripheral edema and ascites.[merckmanuals.com]
    • Paracentesis is performed if there is respiratory compromise secondary to severe ascites.[hawaii.edu]
    • […] changes that occur to the small, functional structures in the kidneys, such as: Very high levels of protein in the urine Low levels of protein in the blood due to its loss in the urine Tissue swelling all over the body (edema) especially in the abdomen (ascites[childrensnational.org]
    • In later stages, swelling may occur in the abdomen (ascites), hands, and around the eyes in the morning (called periorbital edema).[healthcommunities.com]
    Deep Vein Thrombosis
    Eclampsia
    • […] protein-repellant membrane) Postinfectious (occurs after an infection) IgA nephropathy (Berger's disease) (deposit of specific immunoglobulin A causing an inflammatory reaction and leading to glomerulonephritis) Minimal change disease (Nil's disease) Pre-eclampsia[healthcommunities.com]
    Edema
    Glomerulonephritis
    • Of the glomerulonephritis cases approximately 60% to 80% are primary, while the remainder are secondary.[en.wikipedia.org]
    • Rapidly Progressive Glomerulonephritis (RPGN): also known as crescentic glomerulonephritis, characterized by rapid loss of kidney function.[newhealthadvisor.com]
    • Glomerulonephritis - acute; Acute glomerulonephritis; Nephritis syndrome - acute Appel GB, Radhakrishnan.[nlm.nih.gov]
    • Membranous glomerulonephritis is the most common cause in adults. [nlm.nih.gov]
    • The nephrotic syndrome may follow kidney damage from GLOMERULONEPHRITIS or DIABETES or it may be caused by severe high blood pressure ( HYPERTENSION ), poisoning or adverse reactions from drugs.[medical-dictionary.thefreedictionary.com]
    Hereditary Nephritis
    • nephritis* Sickle cell disease Physiologic Adaptation to reduced nephrons Morbid obesity Oligomeganephronia Miscellaneous Chronic allograft nephropathy Malignant hypertension Preeclampsia *More commonly manifests as nephritic syndrome. † Infectious and[merckmanuals.com]
    Hypercholesterolemia
    • Brett AS (1989) Treating hypercholesterolemia.[link.springer.com]
    • Nephrotic Syndrome Signs and Symptoms In addition to proteinuria , there are three main symptoms of nephrotic syndrome associated with protein leaking into the urine: Hypoalbuminemia (low level of albumin in the blood) Edema (swelling) Hypercholesterolemia[healthcommunities.com]
    • That is in the range of serum cholesterol seen in familial hypercholesterolemia , a disease that predisposes individuals to myocardial infarction.[emedicine.medscape.com]
    • […] sin'drōm ) Clinical state characterized by edema, albuminuria, decreased plasma albumin, doubly refractile bodies in the urine, and usually increased blood cholesterol. nephrotic syndrome a condition marked by edema, marked proteinuria, hypoalbuminemia and hypercholesterolemia[medical-dictionary.thefreedictionary.com]
    • The diagnostic criteria are marked proteinuria, generalized edema, hypoalbuminemia, and hyperlipidemia (with hypercholesterolemia).[hawaii.edu]
    Hyperlipidemia
    • The pathophysiology of nephrotic hyperlipidemia is complex.[link.springer.com]
    • The substantial loss of protein in the urine results in complications such as HYPOPROTEINEMIA; generalized EDEMA; HYPERTENSION; and HYPERLIPIDEMIAS.[fpnotebook.com]
    • A diet low in saturated fat and cholesterol may also be recommended to help control hyperlipidemia.[my.clevelandclinic.org]
    • Patients with hypoalbuminemia, proteinuria, and hyperlipidemia but no third-spacing of fluid are occasionally referred to as having 'incomplete' or 'incipient' nephrotic syndrome.[veterinarycalendar.dvm360.com]
    • Patients present with marked oedema, proteinuria, hypoalbuminemia, and often hyperlipidemia 1 .[radiopaedia.org]
    Hypertension
    • The hallmark features of nephritic syndrome are RBC casts and hypertension.[newhealthadvisor.com]
    • […] infections are suspected in infants Acthar is contraindicated in patients with scleroderma, osteoporosis, systemic fungal infections, ocular herpes simplex, recent surgery, history of or the presence of a peptic ulcer, congestive heart failure, uncontrolled hypertension[actharnephrology.com]
    • Treatment with Acthar was well tolerated; one patient had an early termination of treatment due to weight gain and hypertension.[prnewswire.com]
    • Rarely also accelerated phase hypertension can present this way.[bestpractice.bmj.com]
    • Hypercoagulability Hypertension in adults Renal sodium retention Bone disorder Corticosteroid use Chronic kidney disease Unknown Possibly hypovolemia, interstitial edema, and use of NSAIDs Proximal tubular dysfunction (acquired Fanconi syndrome), with[merckmanuals.com]
    Hypertriglyceridemia
    • Patients have increased LDL, increased lipoproteins, and hypertriglyceridemia .[pedemmorsels.com]
    • Elevation of plasma total cholesterol, or more specifically low-density lipoprotein cholesterol, is the major lipid abnormality in NS, although hypertriglyceridemia may develop as the disorder progresses.[link.springer.com]
    Hypoalbuminemia
    • […] glomerular damage: Proteinuria and hypoalbuminemia: Hypoalbuminemia in patients with nephrotic syndrome is a direct consequence of proteinuria.[veterinarycalendar.dvm360.com]
    • Hypoalbuminemia: is treated using the medical nutrition therapy described as a treatment for edema.[en.wikipedia.org]
    • Definition (CSP) diseases involving defective kidney glomeruli, characterized by massive proteinuria and lipiduria with varying degrees of edema, hypoalbuminemia, and hyperlipidemia.[fpnotebook.com]
    • ., New York, NY 10016. (800) 622-9010. . nephrotic syndrome [ nĕ-frot ik ] any of several conditions marked by massive edema, heavy proteinuria, hypoalbuminemia, and unusual susceptibility to intercurrent infections.[medical-dictionary.thefreedictionary.com]
    • Patients present with marked oedema, proteinuria, hypoalbuminemia, and often hyperlipidemia 1 .[radiopaedia.org]
    Hypocalcemia
    • Hypocalcemia Hypocalcemia is common in the nephrotic syndrome, but rather than being a true hypocalcemia, it is usually caused by a low serum albumin level.[emedicine.medscape.com]
    • Because of the hypoalbuminemia, hypocalcemia is often seen, with calcium levels less than 9.0 mg/dL.[hawaii.edu]
    Hypokalemia
    • Monitor patients for signs and symptoms Acthar can cause elevation of blood pressure, salt and water retention, and hypokalemia.[actharnephrology.com]
    • Acthar can cause elevation of blood pressure, salt and water retention, and hypokalemia.[prnewswire.com]
    • If hypokalemia develops, an oral potassium supplement or spironolactone may be added.[hawaii.edu]
    Hyponatremia
    • Hyponatremia and hyperkalemia can be seen, with hyperkalemia developing in patients who are oliguric.[hawaii.edu]
    • Hyponatremia also occurs with a low fractional sodium excretion.[en.wikipedia.org]
    Hypovolemia
    • Hypovolemia Hypovolemia occurs when hypoalbuminemia decreases the plasma oncotic pressure, resulting in a loss of plasma water into the interstitium and causing a decrease in circulating blood volume.[emedicine.medscape.com]
    • […] thromboembolism (especially renal vein thrombosis and pulmonary embolism, which occur in up to 5% of children and 40% of adults) Loss of antithrombin III Increased hepatic synthesis of clotting factors Platelet abnormalities Hyperviscosity caused by hypovolemia[merckmanuals.com]
    • Acute kidney failure due to hypovolemia: the loss of vascular fluid into the tissues (edema) produces a decreased blood supply to the kidneys that causes a loss of kidney function.[en.wikipedia.org]
    • Urinary losses of anticoagulants (antithrombin III, plasminogen, Protein S) Increased procoagulants (fibrinogen, Factor VIII, plasminogen activity factor-1) Procoagulant state can be further exacerbated by hypovolemia .[pedemmorsels.com]
    • This increased prevalence may be due to vascular stasis and/or reduced rate of blood flow due to hypovolemia, and reduced activity levels.[veterinarycalendar.dvm360.com]
    Lipoid Nephrosis
    • Causes of nephrotic syndrome Some of the causes of nephrotic syndrome include: changes to the immune system (minimal change or lipoid nephrosis) - this type is most common in children.[betterhealth.vic.gov.au]
    • Hopper J, Ryan P, Lee J, Rosenau W (1970) Lipoid nephrosis in 31 adult patients: renal biopsy study by light, electron and fluorescence microscopy with experience in treatment.[link.springer.com]
    Lupus Nephritis
    • These can include: membranous nephropathy, focal segmental glomerulosclerosis, minimal change disease, membranoproliferative glomerulonephritis, lupus nephritis, and IgA nephropathy.[prnewswire.com]
    • ., IgA nephropathy), fibrillary glomerulopathies (the most common being amyloidosis), lupus nephritis, and multiple myeloma (e.g., light-chain deposition diseases). [14] Membranoproliferative glomerulonephritis is a relatively rare cause of nephrotic[bestpractice.bmj.com]
    • The histopathologic findings in our patient are similar to those originally described in MPGN type III (but lacking proliferative changes) and also often seen in proliferative lupus nephritis, but they differ from the typical pattern of renal involvement[casesjournal.biomedcentral.com]
    • The disease can also cause lupus nephritis.[en.wikipedia.org]
    • Urine protein-to-creatinine ratio in an untimed urine collection is a reliable measure of proteinuria in lupus nephritis.[aafp.org]
    Malignant Hypertension
    • hypertension Preeclampsia *More commonly manifests as nephritic syndrome. † Infectious and postinfectious causes.[merckmanuals.com]
    Membranoproliferative Glomerulonephritis
    • Nephrotic syndrome can also develop in patients with IgA nephropathy, membranoproliferative glomerulonephritis, and post-infectious glomerulonephritis.[bestpractice.bmj.com]
    • Membranoproliferative glomerulonephritis . . . . . c.[hawaii.edu]
    • Membranoproliferative Glomerulonephritis (MPGN): also known as mesangiocapillary glomerulonephritis, the main Hepatitis C associated nephropathy.[newhealthadvisor.com]
    • Glomerulonephritis ( 6%) others Losing protein and protein-bound molecules lead to several complications.[pedemmorsels.com]
    • Membranoproliferative glomerulonephritis (MPGN): is the inflammation of the glomeruli along with the deposit of antibodies in their membranes, which makes filtration difficult.[en.wikipedia.org]
    Membranous Glomerulonephritis
    • Membranous glomerulonephritis is the most common cause in adults. [nlm.nih.gov]
    • ., focal segmental glomerulosclerosis), IgA nephropathy, light-chain disease, mesangiocapillary, membranous glomerulonephritis (may by linked to neoplasia), myeloma, amyloidosis, diabetes, infection, SLE, toxins (e.g., colloidal gold, “street” heroin,[medical-dictionary.thefreedictionary.com]
    • ) Glomerulonephritis (GN) Diffuse mesangial proliferative GN (affecting the messangium) Membranous (damages the protein-repellant membrane) Postinfectious (occurs after an infection) IgA nephropathy (Berger's disease) (deposit of specific immunoglobulin[healthcommunities.com]
    • The less commonly seen types of primary idiopathic nephrotic syndrome are focal segmental glomerular sclerosis, membranous glomerulonephritis and membranoproliferative glomerulonephritis.[hawaii.edu]
    • Membranous nephropathy This is sometimes called membranous nephritis or membranous glomerulonephritis.[patient.info]
    Polyarteritis Nodosa
    • For example, nephrotic syndrome is a possible complication of diabetes , systemic lupus erythematosus (SLE) , rheumatoid arthritis , polyarteritis nodosa, Henoch-Schönlein purpura , various infections, some cancers and amyloidosis.[patient.info]
    Preeclampsia
    • In adults, nephrotic syndrome is usually secondary, most often to diabetes or preeclampsia.[merckmanuals.com]
    • […] hypoalbuminemia, 30 g/L Etiology Diabetic nephropathy, 1º glomerular disease; membranous glomerulonephritis may by linked to neoplasia, myeloma, amyloidosis, DM, infection, SLE, toxins–eg, colloidal gold, 'street' heroin, penicillamine, rarely, HIV, preeclampsia[medical-dictionary.thefreedictionary.com]
    • […] hepatomegaly, peripheral neuropathy Multiple myeloma Abnormal urine protein electrophoresis, back pain, renal insufficiency HIV Pathologically similar to focal segmental glomerulosclerosis; risk factors for HIV transmission, possible reduced CD4 cell count Preeclampsia[aafp.org]
    Proteinuria
    • Another symptom is a pronounced proteinuria.[en.wikipedia.org]
    • Despite heavy proteinuria and lipiduria, the urine contains few cells or casts.[bestpractice.bmj.com]
    • […] and hypoalbuminemia: Hypoalbuminemia in patients with nephrotic syndrome is a direct consequence of proteinuria.[veterinarycalendar.dvm360.com]
    • In nine of the 13 patients who completed at least six months of treatment with Acthar, 66.7 percent (6/9) showed proteinuria reduction of 37 percent or greater, with a range of proteinuria reduction from 37.3 percent to 99.4 percent.[prnewswire.com]
    • For example, one of the major features of RPGN is proteinuria exceeding 3 g/day, which falls within the range that is usually associated with proteinuria in nephrotic syndrome.[newhealthadvisor.com]
    Rheumatoid Arthritis
    • Some disorders (for example, diabetes, rheumatoid arthritis, etc) that cause nephrotic syndrome can have various other symptoms and complications.[patient.info]
    Serum Sickness
    • sickness Sjögren syndrome SLE* Idiopathic Castleman disease Sarcoidosis Neoplastic Carcinoma (eg, bronchus, breast, colon, stomach, kidney) Leukemia Lymphomas Melanoma Multiple myeloma Drug-related Gold Heroin Interferon alfa Lithium NSAIDs Mercury Pamidronate[merckmanuals.com]
    Thromboembolism
    • Venous thromboembolism in pediatric nephrotic syndrome.[pedemmorsels.com]
    • Venous thromboembolism in patients hospitalized with nephrotic syndrome.[aafp.org]
    • While classically warfarin has been used as an anticoagulant in NS, newer oral anticoagulants, such as apixaban, are increasingly used to treat venous thromboembolism (VTE) in the general population.[clinicaltrials.gov]
    • A number of complications are noted more frequently in human patients with nephrotic syndrome as opposed to those with asymptomatic proteinuria, the most common of which is thromboembolism.[veterinarycalendar.dvm360.com]
    • Complications venous thromboembolism 1, 2 acute renal failure 1 superimposed bacterial infection 1 It may be classified as congenital or acquired 1 : congenital nephrotic syndrome of Finnish type focal and segmental hyalinosis (FSH) neonatal glomerulonephritis[radiopaedia.org]
  • more...
  • Etiology

    The causes of nephrotic syndrome are primary when it is due to a specific disease or secondary when it is an exhibition of a systemic illness.

    Primary causes of nephrotic syndrome include; Minimal change nephropathy, focal segmental glomerulosclerosis, rapidly progressing glomerulonephritis, membranous glomerulonephritis and mesangial proliferating glomerulonephritis.

    Secondary causes are; Diabetes mellitus causing diabetic nephropathy, systemic lupus erythematosus, sarcoidosis, preeclampsia, viral illnesses like hepatitis C, hepatitis B and HIV, amyloidosis, other infections like syphilis, multiple myeloma, vasculitis, genetic disorders and drugs [2].

    Epidemiology

    In adults, the main cause is diabetic nephropathy which presents mainly as mesangiocapillary glomerulonephritis. While in children, the most frequent glomerulopathy is minimal change disease. Overall, diabetic nephropathy is the most common cause of this disease with an estimated 50 cases per million population.African Americans, American Indians and Hispanics have a higher incidence which is attributable to the incidence of diabetes amongst these races. It generally affects more men than women although lupus nephritis is common in women. It affects more adult than children with an estimated adult:child ratio of 26:1. The peak period of incidence is between 40 – 50 years [3].

    Sex distribution
    Age distribution

    Pathophysiology

    The glomerulus is responsible for filtration in the kidney. In normal healthy individuals, the amount of protein filtered through the glomerulus is less than 0.1%. In nephrotic syndrome, there is destruction of the glomerular structure as it destructs the endothelial surface, glomerular basement membrane or the podocytes. This will lead to an increase in glomerular permeability that leads to loss of albumin in the urine. The loss of albumin, which is the major protein responsible for maintaining the plasma colloid osmotic pressure, will lead to a drop in the pressure. This drop in plasma oncotic pressure will lead to greater filtration of water at the capillaries throughout the body leading to edema. Eventually, as the damage worsens, bigger proteins like the immunoglobulins are also lost in urine [4].

    The metabolic effects of proteinuria are far reaching and include infections due to loss of immunoglobulins, hyperlipidemia secondary to the liver's attempt to compensate for protein loss by synthesizing lipoproteins. Other effects are hypocalcemia, hypercoagulability, and hypovolemia [5].

    Prevention

    In patients who are risk of developing this condition and have mild proteinuria, the use of Angiotensin converting enzyme inhibitors like elanapril has been shown to prevent worsening of proteinuria and kidney function. Also, treating infections that may cause it and preventing risk factors are known preventive measures [10].

    Summary

    Nephrotic syndrome is when there is proteinuria of over 3 grams daily or 2 grams of protein per gram of creatinine. It has a number of causes and water retention is a major component. Primary or secondary damage to the glomeruli alters their capacity to filter protein leading to the massive loss of protein [1].

    Patient Information

    Definition: Nephrotic syndrome is the presence of protein in the urine. It is characterized by an increase loss of protein due to damage to the filtering mechanisms in the kidney. In normal individuals, there is very little protein loss in urine

    Cause: It is caused by factors that either damage the filtration apparatus directly or as a result of another disease like diabetes causing the damage. It is also caused by infections and certain drug use.

    Symptoms: The early symptoms are facial puffiness which is usually worse in the morning. The may be leg and abdominal swelling and generalized swelling of the whole body. The urine could also be unusually foamy. Cough and difficulty in breathing as well as a general feeling of malaise.

    Diagnosis: This is usually done by urine investigations to check the amount and type of protein in urine. Blood tests could also be done to check for renal function. Ultrasound is also done to check the structure of the kidneys.

    Treatment: Treatment is directed at the cause of the disease. Drugs are also given to reduce the fluid level and to treat infections. Most children require steroid treatment.

    Self-assessment

    Ask Question


    5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.

    References

    1. Vaziri ND. Endocrinological consequences of the nephrotic syndrome. Am J Nephrol 1993; 13:360.
    2. MacDonald NE, Wolfish N, McLaine P, et al. Role of respiratory viruses in exacerbations of primary nephrotic syndrome. J Pediatr 1986; 108:378.
    3. Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol. Mar 2012;7(3):513-20
    4. [Guideline] International Study of Kidney Disease in Children (ISKDC). 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. Feb 1978;13(2):159-65.
    5. Tryggvason K, Patrakka J, Wartiovaara J. Hereditary proteinuria syndromes and mechanisms of proteinuria.N Engl J Med. Mar 30 2006;354(13):1387-401.
    6. YAMAUCHI H, HOPPER J Jr. HYPOVOLEMIC SHOCK AND HYPOTENSION AS A COMPLICATION IN THE NEPHROTIC SYNDROME. REPORT OF TEN CASES. Ann Intern Med 1964; 60:242.
    7. White RH, Glasgow EF, Mills RJ. Clinicopathological study of nephrotic syndrome in childhood. Lancet 1970; 1:1353.
    8. Howard AD, Moore J Jr, Gouge SF, et al. Routine serologic tests in the differential diagnosis of the adult nephrotic syndrome. Am J Kidney Dis 1990; 15:24.
    9. Kamei K, Ito S, Nozu K, et al. Single dose of rituximab for refractory steroid-dependent nephrotic syndrome in children. Pediatr Nephrol. Jul 2009;24(7):1321-8.
    10. Bonilla-Felix M, Parra C, Dajani T, et al. Changing patterns in the histopathology of idiopathic nephrotic syndrome in children. Kidney Int 1999; 55:1885.

    • 1 1 Nephrotic Syndrome - A Bagga, RN Srivastava - Pediatric Nephrology, 2011 - books.google.com
    • Acute lymphoblastic leukemia in a child with nephrotic syndrome - M Bhatia, K Kher, CP Minniti - Pediatric Nephrology, 2004 - Springer
    • Aldosterone and the edema of congestive heart failure - LL SANDERS, JC MELBY - Archives of internal medicine, 1964 - Am Med Assoc
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • Berger disease: Henoch-Schönlein syndrome without the rash - SR Meadow, DG Scott - The Journal of pediatrics, 1985 - Elsevier
    • Congestive heart failure and outpatient risk of venous thromboembolism: a retrospective, case-control study - MD Howell, JM Geraci, AA Knowlton - Journal of clinical epidemiology, 2001 - Elsevier
    • And oxidase activity in sera of normal human subjects, pregnant women, and patients with infection, hepatolenticular degeneration and the nephrotic syndrome - H Markowitz, CJ Gubler, JP Mahoney - Journal of Clinical , 1955 - ncbi.nlm.nih.gov
    • Chylous ascites in an adult patient with nephrotic syndrome due to membranous nephropathy - A Kato, S Kohno, T Ohtake, T Takita, A Hirshida - Nephron, 2001 - content.karger.com
    • A relationship between proteinuria and acute tubulointerstitial disease in rats with experimental nephrotic syndrome. - AA Eddy, L McCulloch, E Liu - The American journal of , 1991 - ncbi.nlm.nih.gov
    • Acute interstitial nephritis with the nephrotic syndrome following recombinant leukocyte A interferon therapy for mycosis fungoides - SD Averbuch, HA Austin III, SA Sherwin - England Journal of , 1984 - Mass Medical Soc
    • Study of anti‐tumour necrosis factor receptor superfamily 1A fusion protein, in tumour necrosis factor receptor associated periodic syndrome (TRAPS): clinical and - E Drewe, EM McDermott, PT Powell, JD Isaacs - , 2003 - Br Soc Rheumatology
    • OF THE INFERIOR VENA CAVA AND THE RENAL AND PORTAL VEINSWITH THE CLINICAL PICTURE OF THE NEPHROTIC SYNDROME: REPORT OF A CASE, - HA DEROW, MJ SCHLESINGER - Archives of Internal , 1939 - archinte.jamanetwork.com
    • 1 1 Nephrotic Syndrome - A Bagga, RN Srivastava - Pediatric Nephrology, 2011 - books.google.com
    • A study of the mechanisms of edema formation in patients with the nephrotic syndrome - HA Eder, HD Lauson, FP Chinard, RL Greif - Journal of Clinical , 1954 - ncbi.nlm.nih.gov
    • Acute glomerulonephritis with the nephrotic syndrome - SGF Wilson, W Heymann - Pediatrics, 1959 - Am Acad Pediatrics
    • 1 1 Nephrotic Syndrome - A Bagga, RN Srivastava - Pediatric Nephrology, 2011 - books.google.com
    • Change nephrotic syndrome complicated with recurrence of malignant thymoma: an interesting case with remission due to steroid therapy of both nephrotic syndrome - A Fukuda, Y Sato, S Iwatsubo, H Komatsu - Jinzo Gakkai shi, 2009 - ncbi.nlm.nih.gov
    • 1 1 Nephrotic Syndrome - A Bagga, RN Srivastava - Pediatric Nephrology, 2011 - books.google.com
    • Artificial intelligence in medicine - P Szolovits - 1982 - courses.csail.mit.edu
    • 1 1 Nephrotic Syndrome - A Bagga, RN Srivastava - Pediatric Nephrology, 2011 - books.google.com
    • Clinical features of benign hypertensive nephrosclerosis at time of renal biopsy - A Innes, PA Johnston, AG Morgan, AM Davison - QJM, 1993 - Oxford Univ Press
    • A pharmacoepidemiologic assessment of nonsteroidal anti-inflammatory drug exposure and the association withcolorectal cancer, gastrointestinal and renal adverse - TE Niecko - 2001 - athenaeum.libs.uga.edu
    • A case of “pure” preeclampsia with nephrotic syndrome before 15 weeks of gestation in a patient whose renal biopsy showed glomerular capillary endotheliosis - T Imasawa, T Nishiwaki, M Nishimura - American journal of , 2006 - Elsevier
    • Benign paroxysmal torticollis in infancy - T Deonna, D Martin - Archives of disease in childhood, 1981 - ncbi.nlm.nih.gov
    • Focal glomerular sclerosis - R Habib - Kidney int, 1973 - nature.com
    • Preparations for topical administration comprising 2-amino-2-(2-(4-octylphenyl) ethyl) propane-1, 3-diol for the treatment of diseases induced from immune disorder - T IMAYOSHI - EP Patent 0,812,588, 2004 - freepatentsonline.com
    • A relationship between proteinuria and acute tubulointerstitial disease in rats with experimental nephrotic syndrome. - AA Eddy, L McCulloch, E Liu - The American journal of , 1991 - ncbi.nlm.nih.gov
    • Bench-to-bedside review: Pulmonary–renal syndromes–an update for the intensivist - , GE Kapotsis, A Karakatsani, C Roussos - Crit Care, 2007 - biomedcentral.com
    • Bilateral renal vein thrombosis and the nephrotic syndrome: Associated with lesions of polyarteritis nodosa - G Miller, JC Hoyt, BE Pollock - The American journal of medicine, 1954 - Elsevier
    • CHRONIC ALLOGENEIC DISEASE I. DEVELOPMENT OF GLOMERULONEPHRITIS - RM Lewis, MYK Armstrong - The Journal of , 1968 - jem.rupress.org
    • Complications of chronic use of skin lightening cosmetics - YM Olumide, AO Akinkugbe, D Altraide - International journal , 2008 - Wiley Online Library
    • A Clinical Study Of Nail Changes In Geriatric Age Group - NS Haneef - 2006 - 119.82.96.198
    • Bilateral anterior tibial syndrome in association with the nephrotic syndrome: Report of a case - HE SWEENEY, GF O'BRIEN - Archives of internal medicine, 1965 - Am Med Assoc
    • Acute Syphilitic Nephrosis: Case Report - CJ McDONALD, AW BARILE - Archives of Internal Medicine, 1963 - Am Med Assoc
    • Amyloidosis (AL). Clinical and laboratory features in 229 cases. - RA Kyle, PR Greipp - Mayo Clinic proceedings. Mayo Clinic, 1983 - ukpmc.ac.uk
    • Abnormalities of lipoprotein metabolism in patients with the nephrotic syndrome - J Joven, C Villabona, E Vilella, L Masana - England Journal of , 1990 - Mass Medical Soc
    • A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome - C Ponticelli, G Rizzoni, A Edefonti, P Altieri - Kidney , 1993 - nature.com
    • A relationship between proteinuria and acute tubulointerstitial disease in rats with experimental nephrotic syndrome. - AA Eddy, L McCulloch, E Liu - The American journal of , 1991 - ncbi.nlm.nih.gov
    • Acute renal failure in children with idiopathic nephrotic syndrome - N Agarwal, KD Phadke, I Garg, P Alexander - Pediatric Nephrology, 2003 - Springer
    • Copper deficiency in long-term parenteral nutrition - JT Karpel, VH Peden - The Journal of pediatrics, 1972 - Elsevier
    • Current medical diagnosis & treatment 2010 - SJ McPhee, MA Papadakis, MW Rabow - 2010 - media.matthewsbooks.com.s3.
    • Benign intracranial hypertension following corticosteroid withdrawal in childhood - BGR Neville, J Wilson - British medical journal, 1970 - ncbi.nlm.nih.gov
    • A case of fever and unexplained acute renal failure - V Sakhuja, R Agarwal, N Kalra - Indian journal of nephrology, 2008 - ncbi.nlm.nih.gov
    • 1 1 Nephrotic Syndrome - A Bagga, RN Srivastava - Pediatric Nephrology, 2011 - books.google.com
    • Disseminated autoimmune disease during levamisole treatment of nephrotic syndrome - G Barbano, F Ginevri, GM Ghiggeri, R Gusmano - Pediatric Nephrology, 1999 - Springer
    • Abdominal Thrombotic and Ischemic Manifestations of the Antiphospholipid Antibody Syndrome: CT Findings in 42 Patients1 - S Kaushik, MP Federle, PH Schur, M Krishnan - , 2001 - radiology.rsna.org
    • Abnormalities of lipoprotein metabolism in patients with the nephrotic syndrome - J Joven, C Villabona, E Vilella, L Masana - England Journal of , 1990 - Mass Medical Soc
    • Nephrotic syndrome induced by puromycin aminonucleoside in rats. I. The role of proteinuria, hypoproteinemia, and renin-angiotensin-aldosterone system on sodium - J Pedraza-Chaverri, C Cruz, ME Ibarra-Rubio - clinica; organo del , 1990 - ukpmc.ac.uk
    • Guidelines for thoracentesis and needle biopsy of the pleura - JW Sokolowski, LW Burgher, FL Jones - American Journal of , 1989 - Am Thoracic Soc
    • Clinical and histologic spectrum of the nephrotic syndrome - LB Berman, GE Schreiner - The American Journal of Medicine, 1958 - Elsevier
    • Renal disease: alkylating and antimetabolic agents in the treatment of nephrotic syndrome, lupus nephritis, chronic glomerulonephritis, and purpura nephritis in - WE GRUPE, W HEYMANN - Archives of Pediatrics & Adolescent , 1966 - Am Med Assoc
    • Acute interstitial nephritis with the nephrotic syndrome following recombinant leukocyte A interferon therapy for mycosis fungoides - SD Averbuch, HA Austin III, SA Sherwin - England Journal of , 1984 - Mass Medical Soc
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • Abnormalities of lipoprotein metabolism in patients with the nephrotic syndrome - J Joven, C Villabona, E Vilella, L Masana - England Journal of , 1990 - Mass Medical Soc
    • Atrial natriuretic factor in the acute nephritic and nephrotic syndromes. - B Rodríguez-Iturbe, D Colic, G Parra - Kidney , 1990 - ukpmc.ac.uk
    • Abnormalities of lipoprotein metabolism in patients with the nephrotic syndrome - J Joven, C Villabona, E Vilella, L Masana - England Journal of , 1990 - Mass Medical Soc
    • Albuminuria and the nephrotic syndrome following exposure to mercury and its compounds - G Kazantzis, KFR Schiller, AW Asscher, RG Drew - QJM, 1962 - Oxford Univ Press
    • Effect of plasmapheresis on familial type III hyperlipoproteinemia associated with glomerular lipidosis, nephrotic syndrome and diabetes mellitus - K Suzaki, S Kobori, S Ueno, M Uehara, T Kayashima - Atherosclerosis, 1990 - Elsevier
    • Abnormalities of lipoprotein metabolism in patients with the nephrotic syndrome - J Joven, C Villabona, E Vilella, L Masana - England Journal of , 1990 - Mass Medical Soc
    • 1 1 Nephrotic Syndrome - A Bagga, RN Srivastava - Pediatric Nephrology, 2011 - books.google.com
    • 30 Kidney Involvement in Systemic Lupus Erythematosus - GG Illei, JE Balow - Systemic lupus erythematosus: A companion , 2007 - books.google.com
    • Of congestive heart failure: vasopressin, aldosterone, and angiotensin II: further evidence for renal-adrenal interaction from studies in hypertension and in cirrhosis - JH LARAGH - Circulation, 1962 - Am Heart Assoc
    • A Case of Edematous Striae Distensae by Corticosteroid and Generalized Edema in Nephrotic Syndrome - SW Choe, YH Yoon, SJ Seo, CK Hong - Korean Journal of , 2004 - KoreaMed
    • Current medical diagnosis & treatment 2010 - SJ McPhee, MA Papadakis, MW Rabow - 2010 - media.matthewsbooks.com.s3.
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • A comparison of subcutaneous low-molecular-weight heparin with warfarin sodium for prophylaxis against deep-vein thrombosis after hip or knee implantation - R Hull, G Raskob, G Pineo - England Journal of , 1993 - Mass Medical Soc
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • Assessment of the antibody response to pneumococcal vaccine in high-risk populations - SH Landesman, G Schiffman - Review of Infectious Diseases, 1981 - cid.oxfordjournals.org
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • Abnormalities of lipoprotein metabolism in patients with the nephrotic syndrome - J Joven, C Villabona, E Vilella, L Masana - England Journal of , 1990 - Mass Medical Soc
    • Acquired hyperlipidemia (secondary dyslipoproteinemias). - A Chait, JD Brunzell - Endocrinology and metabolism clinics of North , 1990 - ukpmc.ac.uk

    Media References

    1. Diabetic glomerulosclerosis (1) HE, CC BY-SA 3.0

    Languages

    Self-assessment