FSGS

Extracted Attributes

• Full Name

  Focal segmental glomerulosclerosis

• Medical Field

  Nephrology

• Classifications

  Primary, secondary, and genetic

• Diagnostic Method

  Renal biopsy and urinalysis

• Standard Therapies

  Glucocorticoids, immunosuppressive agents, ACE inhibitors, and diuretics

• Estimated Incidence

  Approximately 7 per million persons (in the United States)

• Primary Complications

  Nephrotic syndrome and end-stage renal disease (ESRD) / kidney failure

• High-Risk Demographics

  Male African-Americans

• Clinical Manifestations

  Proteinuria, foamy urine, edema, hypoalbuminemia, hypercholesterolemia, and weight gain

• Affected Organs & Structures

  Kidneys, specifically glomeruli and renal podocytes

Timeline

• The National Organization for Rare Disorders (NORD) released comprehensive educational material on navigating the complexities, diagnosis, and management of Focal Segmental Glomerulosclerosis. (Source: National Organization for Rare Disorders (NORD))

  2025-04-04

Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) is a histopathologic finding of scarring (sclerosis) of glomeruli and damage to renal podocytes. This process damages the filtration function of the kidney, resulting in protein presence in the urine due to protein loss. FSGS is a leading cause of excess protein loss—nephrotic syndrome—in children and adults in the US. Signs and symptoms include proteinuria and edema. Kidney failure is a common long-term complication of the disease. FSGS can be classified as primary, secondary, or genetic, depending on whether a particular toxic or pathologic stressor or genetic predisposition can be identified as the cause. Diagnosis is established by renal biopsy, and treatment consists of glucocorticoids and other immune-modulatory drugs. Response to therapy is variable, with a significant portion of patients progressing to end-stage kidney failure. An American epidemiological study 20 years ago demonstrated that FSGS is estimated to occur in 7 persons per million, with male African-Americans at higher risk.

Web Search Results

• Familial Focal Segmental GlomeruloSclerosis (FSGS) | Duke Molecular Physiology Institute | Duke Molecular Physiology Institute

  Focal Segmental GlomeruloSclerosis (FSGS) is a disorder in the blood-filtering parts of the kidney called the glomeruli. Focaland segmentalrefer to the pattern of damage to the glomerulus and glomerulosclerosisrefers to the damage or scarring (sclerosis) of the glomerulus. Specifically, focalrefers to patches within the kidney that are affected and segmentalindicates that only a portion of the glomerulus is affected. FSGS damages the glomerulus and allows protein to leak into the urine (proteinuria) resulting in swelling and a group of clinical changes called nephrotic syndrome. Over time, the damage to the kidney may cause kidney failure. FSGS is only one of many causes of kidney failure or end-stage renal disease (ESRD). In the last 25 years, we have identified over 150 families from [...] The research team at the DMPI has made progress in the search for genes that causes familial FSGS by using linkage analysis on families that have two or more individuals affected by FSGS. This is done by testing many different markers on each chromosome. These markers have different forms called alleles. An individual has two alleles for each chromosomes, having inherited one allele from each parent. Linkage analysis is used to find those markers in which the same alleles are shared by all the FSGS affected individuals in a family. Since the chromosomal location of the shared marker is known, we are able to determine the chromosomal location of the disorder that is being studied, in this case, FSGS. Our linkage analysis has indicated that one form of FSGS is located on chromosome 11. [...] Once blood samples are received in the laboratory, the genetic material (DNA) is removed from the white blood cells. The DNA is used to compare the DNA from individuals affected by FSGS with individuals who are not affected by FSGS. The urine sample will be tested for protein. Finding protein in the urine (proteinuria) helps to identify individuals who may be in the early stages of FSGS but who have not yet had any kidney problems. No individual or family-specific genetic results will be given to study participants. We will share our overall findings with families through periodic newsletters and scientific publications. If a research breakthrough or a genetic test becomes available, we will notify participants.

• FSGS in Focus: What is FSGS?

  Focal segmental glomerulosclerosis (FSGS) is a type of rare kidney disease that causes scarring in parts of the kidney filters. FSGS can

• FSGS - RKD - About the Disease & Find Resources

  Contact Us Careers Facebook Instagram LinkedIn Twitter YouTube # Focal Segmental Glomerulosclerosis (FSGS) Home ▶ Therapeutic Areas ▶ Focal Segmental Glomerulosclerosis (FSGS) ▶ IgA Nephropathy (IgAN) Focal Segmental Glomerulosclerosis (FSGS) Classical Homocystinuria (HCU) ## ABOUT FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS) Focal segmental glomerulosclerosis (FSGS) refers to scarring (sclerosis) of the glomeruli – blood vessels in the kidneys that filter wastes and excess fluids from the blood. The condition occurs in children and adults and is due to diverse causes. FSGS can lead to decline in kidney function and progression to end-stage kidney disease (ESKD), also known as kidney failure. The name of the condition can be broken down like this:

• Focal Segmental Glomerulosclerosis (FSGS): Navigating the Complexities of a Rare Kidney Disorder

  # Focal Segmental Glomerulosclerosis (FSGS): Navigating the Complexities of a Rare Kidney Disorder ## Channel: National Organization for Rare Disorders (NORD) 8.37K subscribers 9 likes ### Description 1,017 views Posted: 2025-04-04 Focal Segmental Glomerulosclerosis (FSGS) is a kidney disease marked by scarring in parts of the glomeruli, leading to protein leakage into the urine and potential kidney dysfunction. Common symptoms include swelling in the legs and around the eyes, foamy urine, and reduced kidney function. Treatment strategies aim to manage symptoms and slow disease progression, incorporating medications such as ACE inhibitors, corticosteroids, and, in severe cases, dialysis or kidney transplantation. [...] ### Transcript [0:04] Focal segmental glomemeilosclerosis or FSGS is a disease of the kidneys. The kidneys are two bean-shaped [0:12] organs in our body that filter out toxic waste products and excess water from the blood and excrete them in urine. [0:21] Now inside each kidney there are clusters of tiny blood vessels called glomemerieli which act like filters of the kidney that let extra water and the [0:30] harmful wastes through into the urine while keeping important things like red blood cells and proteins in the [0:38] body. In FSGS segments of the glomemerieli the filters get damaged and scarred. As a result harmful wastes [0:47] build up in the blood. Furthermore, the damaged glomemerieli start leaking out important proteins like albammen into [...] [4:18] Lifestyle changes like healthier eating habits, vitamins, exercise, and smoking sessation are also encouraged. In [4:26] secondary FSGS, treatment is mainly focused on treating the underlying disease or stopping the use of the medication or elicit drug that's causing [4:35] the damage. Lastly, for relieving symptoms, diuretics are also used which flushes out excess water from the [4:43] circulation and this helps in reducing the puffiness of the face and legs. [4:49] All right, as a quick recap, FSGS is a disease which affects glomemeriuli causing leakage of excess protein into [4:57] the urine. This causes foamy urine, puffiness of the face and the legs, and in some cases low kidney function, and

• Focal Segmental Glomerulosclerosis (FSGS) | National Kidney Foundation

  ## About FSGS FSGS (focal segmental glomerulosclerosis) is a type of glomerular disease that involves scarring (sclerosis) in your kidney. The scarring of FSGS only takes place in small sections of the glomeruli. Glomeruli are tiny filtering units in the kidney that filter, or clean, your blood to get rid of toxins and make urine. Damaged glomeruli can lead to kidney disease. A kidney nephron and a glomerulus. Glomeruli filter out waste products. ## Signs and Symptoms Early stages may not cause any symptoms. You may only see some signs on your own, while others may be found by your healthcare professional. Signs and symptoms of FSGS can include: [...] Swelling in body parts like your legs, ankles and around your eyes (edema) Weight gain due to extra fluid building in your body Foamy urine caused by high protein levels in the urine (proteinuria) High fat levels in the blood (high cholesterol) Low levels of protein in the blood (hypoalbuminemia) ## Causes FSGS is not caused by a single disease. It can have many different causes. The scarring may happen because of an infection, drug, or disease that affects the entire body, like diabetes, HIV infection, sickle cell disease or lupus. Certain genes have also been linked to a higher risk for FSGS. FSGS can also be caused by another glomerular disease that you had before you got FSGS. ## Types FSGS has different types based on the cause. Types of FSGS include: [...] ### Future Health You should talk with a healthcare professional about your condition because the progression of the disease depends on many factors. FSGS is a chronic disease because the scarred glomeruli cannot be repaired. Treatment can slow the process of kidney disease. Everyone is different in how they respond to treatment. Over time, some patients with FSGS gradually get worse until they reach kidney failure. If this occurs, they will need a kidney transplant or dialysis to stay alive. Some people respond well to treatment and may live with the disease for many years while being monitored for any signs of change. ## Preparing for your appointment ### Questions to ask