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Glomerulonephritis

  • Overview

    Glomerulonephritis is a type of kidney disease that involves the glomeruli — very small structures in the kidneys that supply blood to units in the kidneys that filter urine — called the nephrons. During glomerulonephritis, the glomeruli become inflamed and impair the kidney's ability to filter urine.

    Glomerulonephritis can be acute (a sudden attack of inflammation) or chronic (coming on gradually).

    Glomerulonephritis may develop after a bacterial infection, like strep throat, or it may be caused by a chronic condition.

    Kidney

      
    Nephrology
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  • In-Depth

    There are two main types of glomerulonephritis:

    • Acute post-streptococcal glomerulonephritis (APSGN)
      • APSGN is caused by a bacterial infection, like strep throat.
      • APSGN is a relatively uncommon disease affecting about one of every 10,000 people, but it is the most common form of glomerulonephritis in children.
      • It is most common among boys between age 3 and 7, but it can occur at any age.
      • Many people with APSGN are asymptomatic (showing no symptoms of the disease).
    • Glomerulonephritis (GN)

    GN may be caused by several different disease states or by a genetic disorder.    Some disease states associated with GN include:

    • systemic diseases, such as lupus
    • polyarteritis nodosa group - an inflammatory disease of the arteries
    • Wegener vasculitis - a progressive disease that leads to widespread inflammation of all of the organs in the body
    • Henoch-Schvnlein purpura - a disease usually seen in children and associated with purpura (small or large purple lesions on the skin and internally on the organs) that involves multiple organ systems

    GN can also result from a gene on the X chromosome passed from carrier mothers who have no features, or minimal features of GN, to their sons, who are affected with the disorder in 50 percent of the cases.

    What are the symptoms of glomerulonephritis?

    Symptoms of glomerulonephritis may depend on whether your child has the acute or chronic form, and symptoms also differ depending on what caused the glomerulonephritis.

    Symptoms may include:

    • cola-colored or iced tea-colored urine (from blood and protein)
    • sore throat
    • diminished urine output
    • fatigue
    • lethargy
    • nausea and vomiting
    • increased breathing effort
    • headache
    • high blood pressure
    • seizures (may occur as a result of high blood pressure)
    • rash, especially on the buttocks and legs
    • weight loss
    • joint pain
    • pale skin color
    • edema (fluid accumulation in the tissues)
    • hyperpigmentation (skin may appear yellow or brown)
  • Tests

    How is glomerulonephritis diagnosed?

    In addition to a thorough physical examination and complete medical history, your child's physician may order the following diagnostic tests:

    • throat culture
    • urine tests
    • blood tests
    • electrocardiogram (ECG or EKG) - a test that records the electrical activity of your child's heart, shows abnormal rhythms (arrhythmias or dysrhythmias) and detects heart muscle damage
    • renal ultrasound (also called sonography) - a non-invasive test in which a transducer is passed over your child's kidney producing sound waves that bounce off of the kidney and transmit a picture of the organ on a video screen.
    • chest x-ray - a diagnostic test which uses invisible X-ray energy beams to produce images of internal tissues, bones and organs onto film.
    • renal biopsy - a procedure in which a small sample of tissue is taken from you child's kidney through a needle. We conduct tests on the tissue to determine the specific disease.
  • It truly is heart-breaking to have to look a child in the eye and know there's currently little that can be done to cure them of [FSGS]. [Brown's] study shows that the answers are there to be found.

    Henry Brehm, executive director of the NephCure Foundation, on Elizabeth Brown, MD's FSGS research.

    Glomerulonephritis may be a temporary and reversible condition, or it may progress and lead to complications and/or chronic kidney failure.

    If your child has APSGN, treatment will focus on curing her infection and treating her symptoms associated with the infection. Unfortunately, a cure for GN has not been found; therefore treatments focus on slowing the progression of the disease and preventing complications.

    Treatment for glomerulonephritis may include:

    • fluid restriction
    • decreased protein diet
    • decreased salt and potassium diet
    • medication, such as blood pressure medicines, corticosteroids or immunosuppressives
    • diuretics
    • blood pressure medications
    • phosphate binders - medications to decrease the amount of the mineral phosphorus in the blood
    • immunosuppressive agents
    • dialysis - a medical treatment that removes waste and additional fluid from the blood after the kidneys have stopped functioning
    • dietary restrictions on salt, fluids, protein and other substances
  • Research & Innovation

    Elizabeth Brown

    Elizabeth Brown, Glomerulonephritis Gene for devastating kidney disease discovered

    Discovery suggests how toxin-filtering cells go awry

    Researchers from Boston Children's Hospital and Brigham and Women's Hospital have identified an important genetic cause of a devastating kidney disease that is the second leading cause of kidney failure in children, according to The NephCure Foundation.

    The study, published online December 20 by Nature Genetics, may provide clues to developing treatments for the disease, focal segmental glomerulosclerosis (FSGS), which currently forces children and young adults onto dialysis and often requires a kidney transplant. No effective treatments are known, and years of research have failed to uncover the underlying disease mechanism.

    FSGS attacks the kidney's filtering system, causing proteins to be lost into the urine and reducing the kidney's ability to filter wastes from the blood. According to NephCure, which helped fund the study, 26 million Americans suffer from chronic kidney disease, of which FSGS is one of the most common forms.

    Patients with FSGS are often treated with steroids, which are only partially effective and have very harsh side effects. In addition, they often face several trips a week to the hospital for dialysis, and many require a kidney transplant, along with lifelong treatment with powerful immunosuppressants to prevent organ rejection.

    The research team, led by Elizabeth Brown, MD, of Children's Division of Nephrology, working in the laboratory of Martin Pollak, MD, of the Renal Division at Brigham and Women's Hospital, identified the gene by performing a genetic linkage analysis in two large families with FSGS. Linkage analysis is a gene-finding technique that compares affected with unaffected family members, looking for a piece of DNA whose location is already known, and that is inherited only by affected members. Using that piece of DNA as a "signpost," researchers can then look nearby to find the disease gene.

    Using this technique, Brown and colleagues homed in on a region of chromosome 14q. By sequencing multiple genes in this region, they detected nine different mutations, all of them in a gene called INF2. They then sequenced INF2 in 91 additional families. In all, they found INF2 mutations in 11 of 93 families.

    There have been a few descriptions of other genes that result in FSGS, but Brown and colleagues think INF2 is an important find. Mutations on this gene seem to affect larger numbers of families than those on previously discovered genes, and may be more relevant in understanding how the disease originates physiologically.

    INF2 encodes a protein that regulates actin, a protein vital to creating and maintaining the architecture of the cell. Both actin and INF2 are abundant in podocytes, the kidney cells that are crucial to filtering toxins. These cells are structurally complex, with extensions that interlock with those of other cells. Based on their findings, the researchers believe that disruption of INF2 in podocytes compromises their structure and, hence, their function.

    In 2007 alone, 1,117 kidney transplants were performed on FSGS patients, according to NephCure. "To make matters worse, many patients have recurrence of the disease soon after transplant," says William Harmon, MD, chief of Children's Division of Nephrology. "First it ruins your native kidney, then it can return instantly in the transplant and ruin that also."

    says Henry Brehm, executive director of The NephCure Foundation, which has dedicated over $6 million towards research of FSGS and Nephrotic Syndrome in recent years.

    The study abstract can be accessed online. Co-authors were Johannes Schlandorff and Daniel Becker of HMS and Brigham and Women's Renal Division, Hiroyasu Tsukaguchi, Andrea Uscinski of Brigham and Women's Renal Division, Henry Higgs of Dartmouth Medical School, and Joel Henderson of Brigham and Women's Department of Pathology.

    For more information on FSGS, visit The NephCure Foundation's website.

    The study was supported by the National Institute of Diabetes and Digestive and Kidney Diseases, the Clinical Investigator Training Program: Beth Israel Deaconess Medical Center Harvard and Massachusetts Institute of Technology Health Sciences and Technology, Pfizer Inc., Merck and Co., The NephCure Foundation, and the Cole Pasqualucci Nephrotic Syndrome and FSGS Research fund.

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