Focal segmental glomerulosclerosis (FSGS) is a cause of nephrotic syndrome in children and adolescents, as well as an important cause of kidney failure in adults. Minimal change disease (MCD) is by far the most common cause of nephrotic syndrome in children. MCD and primary FSGS may have a similar cause. The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Renal failure is when the kidneys fail to function properly. ...

The individual components of the name refer to the appearance of the kidney tissue on biopsy: focal - only some of the glomeruli are involved (as opposed to diffuse), segmental - only part of an entire glomerulus is involved (as opposed to global), glomerulosclerosis - refers to scarring of the glomerulus (a part of the nephron (the functional unit of the kidney)) A biopsy (in Greek: bios = life and opsy = look/appearance) is a medical test involving the removal of cells or tissues for examination. ... Glomerulus refers to two unrelated structures in the body, both named for their globular form. ... Glomerulus refers to two unrelated structures in the body, both named for their globular form. ... A nephron is the basic structural and functional unit of the kidney. ... The kidneys are bean-shaped excretory organs in vertebrates. ...

Symptoms & Signs

In children and some adults, FSGS presents as a nephrotic syndrome (which is characterized by edema (associated with weight gain), hypoalbuminemia (low albumin (a protein) in the blood), hyperlipidemia and hypertension (high blood pressure)). In adults it may also present as kidney failure and proteinuria, without a full-blown nephrotic syndrome. Edema (American English) or oedema (British English), formerly known as dropsy or hydropsy, is swelling of any organ or tissue due to accumulation of excess lymph fluid, without an increase of the number of cells in the affected tissue. ... This article is in need of attention. ... Albumin can refer to ovalbumin, the principal protein in egg white albumins, a group of proteins including serum albumin and together constituting roughly 60% of the protein in blood plasma. ... A representation of the 3D structure of myoglobin, showing coloured alpha helices. ... Hypercholesterolemia (literally: high blood cholesterol) is the presence of high levels of cholesterol in the blood. ... For other forms of hypertension see hypertension (disambiguation). ... Renal failure is when the kidneys fail to function properly. ... Proteinuria (from protein and urine) means the presence of an excess of serum proteins in the urine. ...

Classification

Depending on the cause it is broadly classified as

• Primary, when no underlying cause is found; usually presents as nephrotic syndrome
• Secondary, when an underlying cause is identified; usually presents with kidney failure and proteinuria. This is actually a heterogeneous group including a myriad causes such as
  • Infections such as HIV
  • Toxins and drugs such as heroin and pamidronate
  • Familial forms
  • Secondary to nephron loss and hyperfiltration, such as with chronic pyelonephritis and reflux, morbid obesity, diabetes mellitus

There are many other classification schemes also. Renal failure is when the kidneys fail to function properly. ... Proteinuria (from protein and urine) means the presence of an excess of serum proteins in the urine. ... Human immunodeficiency virus or HIV is a retrovirus that causes Acquired Immunodeficiency Syndrome (AIDS), a condition in which the immune system begins to fail, leading to life-threatening opportunistic infections. ... Heroin, also known as diamorphine (BAN) or diacetylmorphine (INN), is a semi-synthetic opioid. ... Pamidronate disodium (brand name Aredia®) is a bisphosphonate used to prevent bone loss, and treat osteoporosis. ... A nephron is the basic structural and functional unit of the kidney. ... Pyelonephritis is an ascending urinary tract infection that has reached the pyelum (pelvis) of the kidney (nephros in Greek). ... Diagram of typical reflux apparatus. ... For the disease characterized by excretion of large amounts of severely diluted urine, see diabetes insipidus. ...

Pathologic variants

Five mutually exclusive variants of focal segmental glomerulosclerosis may be distinguished by the pathologic findings seen on renal biopsy:^[1]

1. Collapsing variant
2. Glomerular tip lesion variant
3. Cellular variant
4. Perihilar variant
5. Not otherwise specified (NOS) variant.

Recognition of these variants may have prognostic value in individuals with primary focal segmental glomerulosclerosis (i.e. where no underlying cause is identified). The collapsing variant is associated with higher rate of progression to end-stage renal disease, whereas glomerular tip lesion variant has low rate of progression to end-stage renal disease in most patients.^[1] Cellular variant shows similar clinical presentation to collapsing and glomerular tip variant but has intermediate outcomes between these two variants. However, because collapsing and glomerular tip variant show overlapping pathologic features with cellular variant, this intermediate difference in clinical outcomes may reflect sampling bias in cases of cellular focal segmental glomerulosclerosis (i.e. unsampled collapsing variant or glomerular tip variant). The prognostic significance of perihilar and NOS variants has not yet been determined. The NOS variant is the most common subtype.^[1] Chronic renal failure (CRF) is a slowly progressive loss of renal function over a period of months or years and defined as an abnormally low glomerular filtration rate, which usually determined indirectly by the creatinine level in blood serum. ...

Diagnosis

• urinalysis
• blood tests - cholesterol
• kidney biopsy

A urinalysis (or UA) is an array of tests performed on urine and one of the most common methods of medical diagnosis. ... Blood tests are laboratory tests done on blood to gain an appreciation of disease states and the function of organs. ... Needle aspiration biopsy is a procedure performed to diagnose and treat certain kind of illnesses. ...

Differential Diagnosis

• Minimal change disease, especially in children
• several others

Minimal change disease or nil disease (lipoid nephrosis) is a disease of the kidney which causes nephrotic syndrome and usually affects children (peak incidence at 2-3 years of age). ...

Treatment

• Salt restriction and diuretics (water pills), such as furosemide, for edema
• Antihypertensives (especially ACEIs) - if the blood pressure is too high
• treat present hyperlipidemia (e.g. statins, fibrates)
• Corticosteroids, such as prednisone - based on the clinical judgment of physician (no broad consensus/guideline)
• Cytotoxics, such as cyclophosphamide may be used to induce remission in patients presenting with FSGS refractory to corticosteroids, or in patients who do not tolerate steroids.
• Plasmapheresis - blood cleansing using a machine to remove the patient's blood plasma and replacing it with donor plasma.
• None - sometimes none of the above works and the patient will required dialysis with possibly later transplantation of a new kidney.

A diuretic is any drug that tends to increase the flow of urine from the body (diuresis). ... Furosemide (INN) or frusemide (former BAN) is a loop diuretic used in the treatment of congestive heart failure and edema. ... In medicine and pharmacology, antihypertensives are a class of drugs that are used in the treatment of arterial hypertension. ... ACE inhibitors, or inhibitors of Angiotensin_Converting Enzyme, are a group of pharmaceuticals that are used primarily in treatment of hypertension and congestive heart failure, in most cases as the drugs of first choice. ... Hypercholesterolemia (literally: high blood cholesterol) is the presence of high levels of cholesterol in the blood. ... Lovastatin, the first statin to be marketed The statins (or HMG-CoA reductase inhibitors) form a class of hypolipidemic agents, used as pharmaceuticals to lower cholesterol levels in people at risk for cardiovascular disease because of hypercholesterolemia. ... In pharmacology, the fibrates are a class of amphipathic carboxylic acids. ... In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ... Prednisone is a synthetic corticosteroid drug which is usually taken orally and can be used for a large number of different conditions. ... The Doctor by Samuel Luke Fildes This article is about the term physician, one type of doctor; for other uses of the word doctor see Doctor. ... Cytotoxicity is the quality of being poisonous to cells. ... Cyclophosphamide (the generic name for Cytoxan, Neosar) is a nitrogen mustard alkylating agent, used to treat various types of cancer and some autoimmune disorders. ... Plasmapheresis is the removal of (components of) blood plasma from the circulation. ... Blood plasma is the liquid component of blood, in which the blood cells are suspended. ... In medicine, dialysis is a type of renal replacement therapy which is used to provide an artificial replacement for lost kidney function due to renal failure. ... An organ transplant is the transplantation of an organ (or part of one) from one body to another, for the purpose of replacing the recipients damaged or failing organ with a working one from the donor. ...

References

1. ^ ^{a b c} Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ, Jennette JC. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int. 2006 Mar;69(5):920-6. PMID 16518352

External links

• Kidcomm An online resource for parents dealing with childhood kidney diseases (FSGS, Nephrotic Syndrome and others)

• FSGS Clinical Trial An NIH sponsored nation-wide Clinical Trial for patients suffering from FSGS