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Nephrotic syndrome is a collection of symptoms which occur because the tiny blood vessels (the glomeruli) in the kidney become leaky. This allows protein (normally never passed out in the urine) to leave the body in large amounts.
Causes & Development
A number of different kidney disorders are associated with nephrotic syndrome, including:- Minimal change disease or MCD (responsible for about 80% of nephrotic syndrome in children, and about 20% in adults) MCD is a disorder of the glomeruli
- Focal glomerulosclerosis
- Membranous glomerulopathy
- Membranoproliferative glomerulonephropathy.
Other types of diseases can also result in nephrotic syndrome. These include diabetes, sickle-cell anemia, amyloidosis, systemic lupus erythematosus, sarcoidosis, leukemia, lymphoma, cancer of the breast, colon, and stomach, reactions to drugs (including nonsteroidal anti-inflammatory drugs, lithium, and street heroine), allergic reactions (to insect stings, snake venom, and poison ivy), infections (malaria, various bacteria, hepatitis B, herpes zoster, and the virus which causes AIDS), and severe high blood pressure.
Diagnosis & Tests
Diagnosis is based first on the laboratory examination of the urine and the blood. While the urine will reveal significant quantities of protein, the blood will reveal abnormally low amounts of circulating proteins. Blood tests will also reveal a high level of cholesterol. In order to diagnose one of the kidney disorders which cause nephrotic syndrome, a small sample of the kidney (biopsy) will need to be removed for examination. This biopsy can be done with a long, very thin needle which is inserted through the skin under the ribs.
Prognosis
Prognosis depends on the underlying disorder. Minimal change disease has the best prognosis of all the kidney disorders, with 90% of all patients responding to treatment. Other types of kidney diseases have less favorable outcomes, with high rates of progression to kidney failure. When nephrotic syndrome is caused by another, treatable disorder (infection, allergic or drug reaction), the prognosis is very good.
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Signs, symptoms & indicators of Nephrotic Syndrome: | | | | | Lab Values - Common |  Low/high systolic blood pressure | Because the kidneys are involved in blood pressure regulation, abnormally low or abnormally high blood pressure may develop from nephrotic syndrome. |
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Symptoms - Abdominal Pain |
(Severe) abdominal discomfort |
Symptoms - Food - General |
Weak appetite| | As nephrotic syndrome progresses, appetite is greatly decreased. |
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Symptoms - General |
Constant fatigue| | As nephrotic syndrome progresses, the patient feels increasingly weak and fatigued. |
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Symptoms - Hair |
Brittle hair| | Over time, the protein loss occurring in nephrotic syndrome will result in a generally malnourished state. Hair and nails become brittle, and growth is stunted. Bone becomes weak, and the body begins to lose other important nutrients (sugar, potassium, calcium). |
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Symptoms - Immune System |
 Blood clotting problems| | Disorder of blood clotting is a serious and frequent complication. |
History of infections| | Infection is a serious and frequent complication of nephrotic syndrome. |
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Symptoms - Metabolic |
Edema of the eyelids| | As nephrotic syndrome progresses, edema is noticed in the eyelids, hands, feet, knees, abdomen and (in males) scrotum. |
Edema of the hands
Edema of the feet
Edema of the scrotum
Edema of the knees
Edema of the abdomen |
Symptoms - Muscular |
Poor muscle tone| | Over time, the loss of protein due to nephrotic syndrome causes muscles to become weak and small (muscle wasting). |
Individual weak muscles |
Symptoms - Nails |
Brittle fingernails| | Over time, the protein loss occurring in nephrotic syndrome will result in a generally malnourished state, causing hair and nails become brittle. |
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Symptoms - Urinary |
Having foamy urine| | The first symptom of nephrotic syndrome is often foamy urine. |
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Conditions that suggest Nephrotic Syndrome:
Risk factors for Nephrotic Syndrome: | | | | | Organ Health | Glomerulonephritis| | If proteinuria is heavy enough, patients will begin to show symptoms of nephrotic syndrome. Nephrotic syndrome is not a disease in itself, but a group of symptoms which are caused by the heavy loss of protein in the urine.
The majority of IgAN patients do not develop nephrotic syndrome, but it is fairly common in pediatric IgAN. |
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Nephrotic Syndrome can lead to:
Recommendations and treatments for Nephrotic Syndrome: | | | | | Diet |  Reduced Water Consumption| | Occasionally, the quantity of fluid a patient is allowed to drink is restricted. |
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Drug |
 Conventional Drug Use| | Treatment depends on the underlying disorder which has caused nephrotic syndrome. Medications which dampen down the immune system are a mainstay of treatment. The first choice is usually a steroid drug such as prednisone. Some conditions may require even more potent medications, such as cyclophosphamide or cyclosporine.
Treating the underlying conditions (for example lymphoma, cancers, heroine use, infections) which have led to nephrotic syndrome will often improve the symptoms of nephrotic syndrome as well. Some patients will require the use of specific medications to control high blood pressure. Some patients benefit from the use of diuretics (which allow the kidney to produce more urine) to decrease swelling.
Nephrotic syndrome may require aggressive treatment with corticosteroids until the level of proteinuria goes down. A person usually needs to lose more than 3gm per day of protein in the urine before this begins to happen. |
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KEY | | Weak or unproven link | | | Strong or generally accepted link | | | May do some good | | | Highly recommended |
GLOSSARY
Abdomen (Abdominal)
That part of the body between the chest and the hips that contains the stomach, intestines, liver, bladder, pancreas and other organs.
AIDS
Acquired Immune Deficiency Syndrome. An immune system deficiency disorder that suddenly alters the body's ability to defend itself. The AIDS virus invades the T4 helper/inducer lymphocytes and multiplies, causing a breakdown in the body's immune system, eventually leading to overwhelming infection and/or cancer, with ultimate death.
Anemia (Anaemia, Anemias)
A condition resulting from an unusually low number of red blood cells or too little hemoglobin in the red blood cells. The most common type is iron-deficiency anemia in which the red blood cells are reduced in size and number, and hemoglobin levels are low. Clinical symptoms include shortness of breath, lethargy and heart palpitations.
Anti-inflammatory (Antiinflammatory)
Reducing inflammation by acting on body mechanisms, without directly acting on the cause of inflammation, e.g., glucocorticoids, aspirin.
Bacteria (Bacterial, Bacterium)
Microscopic germs. Some bacteria are "harmful" and can cause disease, while other "friendly" bacteria protect the body from harmful invading organisms.
Biopsy
Removal of a sample of tissue from a living being for diagnosis. A pathologist later uses a microscope to look for certain features, such as cancer cells, in the sample. A fine-needle aspiration biopsy involves inserting a thin needle to remove a small amount of tissue, sometimes using CT or ultrasound to guide the needle. A core biopsy involves obtaining a sample of tissue with a thick needle or by inserting a thin, lighted tube (laparoscope) into a small incision in the abdomen. Another biopsy method is to remove tissue during an operation.
Calcium
The body's most abundant mineral. Its primary function is to help build and maintain bones and teeth. The body also needs calcium to carry nerve signals, keep the heart functioning, contract muscles, clot blood and maintain healthy skin. Calcium helps control blood acid-alkaline balance, plays a role in cell division, muscle growth and iron utilization, activates certain enzymes, and helps transport nutrients through cell membranes. Calcium also forms a cellular cement called ground substance that helps hold cells and tissues together.
Cancer
Refers to the various types of malignant neoplasms that contain cells growing out of control and invading adjacent tissues, which may metastasize to distant tissues.
Cholesterol
A waxy, fat-like substance manufactured in the liver and found in all tissues, it facilitates the transport and absorption of fatty acids. In foods, only animal products contain cholesterol. An excess of cholesterol in the bloodstream can contribute to the development of atherosclerosis.
Chronic Renal Failure (Chronic Renal Insufficiency, Kidney Failure, Renal Insufficiency)
(CRF) Irreversible, progressive impaired kidney function. The early stage, when the kidneys no longer function properly but do not yet require dialysis, is known as Chronic Renal Insufficiency (CRI). CRI can be difficult to diagnose, as symptoms are not usually apparent until kidney disease has progressed significantly. Common symptoms include a frequent need to urinate and swelling, as well as possible anemia, fatigue, weakness, headaches and loss of appetite. As the disease progresses, other symptoms such as nausea, vomiting, bad breath and itchy skin may develop as toxic metabolites, normally filtered out of the blood by the kidneys, build up to harmful levels. Over time (up to 10 or 20 years), CRF generally progresses from CRI to End-Stage Renal Disease (ESRD, also known as Kidney Failure). Patients with ESRD no longer have kidney function adequate to sustain life and require dialysis or kidney transplantation. Without proper treatment, ESRD is fatal.
Colon (Colonic)
The part of the large intestine that extends to the rectum. The colon takes the contents of the small intestine, moving them to the rectum by contracting.
Corticosteroid (Corticosteroids)
Steroid hormone produced by the adrenal cortex.
Diabetes Mellitus (Diabetes, Diabetic, Diabetics)
A disease with increased blood glucose levels due to lack or ineffectiveness of insulin. Diabetes is found in two forms; insulin-dependent diabetes (juvenile-onset) and non-insulin-dependent (adult-onset). Symptoms include increased thirst; increased urination; weight loss in spite of increased appetite; fatigue; nausea; vomiting; frequent infections including bladder, vaginal, and skin; blurred vision; impotence in men; bad breath; cessation of menses; diminished skin fullness. Other symptoms include bleeding gums; ear noise/buzzing; diarrhea; depression; confusion.
Diuretic (Diuretics)
An agent increasing urine flow, causing the kidneys to excrete more than the usual amount of sodium, potassium and water.
Edema
Abnormal accumulation of fluids within tissues resulting in swelling.
Glomeruli (Glomerulus)
Tiny tufts of capillaries which carry blood within the kidneys. The blood is filtered by the glomeruli. The blood then continues through the circulatory system, but a certain amount of fluid and specific waste products are filtered out of the blood, to be removed from the body in the form of urine.
Gram (gm, gms, Gramme, Grammes, Grams)
A metric unit of weight, there being approximately 28 grams in one ounce.
Hepatitis B
A serious viral infection with the potential for long term consequences. It is caused by a DNA virus that has been found in virtually all body secretions and excretions. However, only blood, saliva, semen and vaginal fluids have been shown to be infectious. Transmission occurs through sexual contact, blood-to-blood contact (blood products, needle sharing, etc.), and from infected mother to infant. Virtually all affected infants and children, and many adults, receive a lesser, even symptom-free, infection. Symptoms, when present, tend to be more severe and prolonged than those for Hepatitis A: initially flu-like, with malaise, fatigue, muscle pain and chest pain on the right side. This is followed by jaundice (slight skin yellowing), anorexia, nausea, fatigue, pale stools, dark urine and tender liver enlargement, but usually no fever.
Immune System (Immune Response, Immunity)
A complex that protects the body from disease organisms and other foreign bodies. The system includes the humoral immune response and the cell-mediated response. The immune system also protects the body from invasion by making local barriers and inflammation. The process may involve acquired immunity (the ability to learn and remember a specific infectious agent), or innate immunity (the genetically programmed system of responses that attack, digest, remove, and initiate inflammation and tissue healing).
Kidneys (Kidney, Renal)
Bean-shaped organs, each about the size of a fist. They are located near the middle of the back, just below the rib cage. The kidneys are sophisticated reprocessing machines, each day handling about 50 gallons of blood to sift out about half a gallon of waste products and extra water. The waste and extra water become urine, which flows to the bladder through tubes called ureters. The actual filtering occurs in tiny units inside the kidneys called nephrons. Every kidney has about a million nephrons. In a nephron, a glomerulus -- which is a tiny blood vessel, or capillary -- intertwines with a tiny urine-collecting tube called a tubule. A complicated chemical exchange takes place, as waste materials and water leave your blood and enter your urinary system. The kidneys recycle chemicals such as sodium, phosphorus, and potassium and thus regulate their levels. Renal: Pertaining to the kidneys.
Leukemia
Cancer of the lymph glands and bone marrow resulting in overproduction of white blood cells (related to Hodgkin's disease).
Lymphoma (Lymphomas)
Any tumor of the lymphatic tissues.
Potassium
A mineral that serves as an electrolyte and is involved in the balance of fluid within the body. Our bodies contain more than twice as much potassium as sodium (typically 9oz versus 4oz). About 98% of total body potassium is inside our cells. Potassium is the principal cation (positive ion) of the fluid within cells and is important in controlling the activity of the heart, muscles, nervous system and just about every cell in the body. Potassium regulates the water balance and acid-base balance in the blood and tissues. Evidence is showing that potassium is also involved in bone calcification. Potassium is a cofactor in many reactions, especially those involving energy production and muscle building.
Prognosis
A prediction (estimate) of the future course and outcome of a disease and an indication of the likelihood of recovery from that disease.
Protein (Proteins)
Compounds composed of hydrogen, oxygen, and nitrogen present in the body and in foods that form complex combinations of amino acids. Protein is essential for life and is used for growth and repair. Foods that supply the body with protein include animal products, grains, legumes, and vegetables. Proteins from animal sources contain the essential amino acids. Proteins are changed to amino acids in the body.
Proteinuria
The presence of protein in the urine, sometimes a symptom of kidney compromise.
Shingles (Herpes Zoster)
A severe infection caused by the Varicella-Zoster virus (VZV), affecting mainly adults. It causes painful skin blisters that follow the underlying route of brain or spinal nerves infected by the virus. Also know as herpes zoster.
Steroid (Steroids)
Any of a large number of hormonal substances with a similar basic chemical structure containing a 17-carbon 14-ring system and including the sterols and various hormones and glycosides.
Stomach
A hollow, muscular, J-shaped pouch located in the upper part of the abdomen to the left of the midline. The upper end (fundus) is large and dome-shaped; the area just below the fundus is called the body of the stomach. The fundus and the body are often referred to as the cardiac portion of the stomach. The lower (pyloric) portion curves downward and to the right and includes the antrum and the pylorus. The function of the stomach is to begin digestion by physically breaking down food received from the esophagus. The tissues of the stomach wall are composed of three types of muscle fibers: circular, longitudinal and oblique. These fibers create structural elasticity and contractibility, both of which are needed for digestion. The stomach mucosa contains cells which secrete hydrochloric acid and this in turn activates the other gastric enzymes pepsin and rennin. To protect itself from being destroyed by its own enzymes, the stomach’s mucous lining must constantly regenerate itself.
Syndrome
A medical condition characterized by a collection of related symptoms (what the patient feels) and signs (what a doctor can observe or measure).
Virus (Viri, Viruses)
Any of a vast group of minute structures composed of a protein coat and a core of DNA and/or RNA that reproduces in the cells of the infected host. Capable of infecting all animals and plants, causing devastating disease in immunocompromised individuals. Viruses are not affected by antibiotics, and are completely dependent upon the cells of the infected host for the ability to reproduce.
Last updated: Apr 13, 2008
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