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    Fanconi syndrome

    De Toni-Fanconi syndrome

    Fanconi syndrome is a disorder of the kidney tubes in which certain substances normally absorbed into the bloodstream by the kidneys are released into the urine instead.

    Causes

    Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown.

    Common causes of Fanconi syndrome in children are genetic defects that affect the body's ability to break down certain compounds such as:

    • Cystine (cystinosis)
    • Fructose (fructose intolerance)
    • Galactose (galactosemia)
    • Glycogen (glycogen storage disease)

    Cystinosis is the most common cause of Fanconi syndrome in children.

    Other causes in children include:

    • Exposure to heavy metals such as lead, mercury, or cadmium
    • Lowe's disease, a rare genetic disorder of the eyes, brain, and kidneys
    • Wilson's disease

    In adults, Fanconi syndrome can be caused by various things that damage the kidneys, including:

    • Certain medications, including azathioprine, cidofovir, gentamicin, and tetracycline
    • Kidney transplant
    • Light chain deposition disease
    • Multiple myeloma
    • Primary amyloidosis

    Symptoms

    • Passing large amounts of urine, which can lead to dehydration
    • Bone pain
    • Weakness

    Exams and Tests

    Laboratory tests may show that too much of the following substances may be lost in the urine:

    • Amino acids
    • Bicarbonate
    • Glucose
    • Magnesium
    • Phosphate
    • Potassium
    • Sodium
    • Uric acid

    Loss of these substances can lead to a variety of problems. Further tests and a physical exam may show signs of:

    • Dehydration due to excess urination
    • Growth failure
    • Osteomalacia
    • Rickets
    • Type 2 renal tubular acidosis

    Treatment

    Many different diseases can cause Fanconi syndrome. The underlying cause and its symptoms should be treated as appropriate.

    Outlook (Prognosis)

    The prognosis depends on the underlying disease.

    When to Contact a Medical Professional

    Call your health care provider if you have dehydration or muscle weakness.

    References

    Seifter JL. Potassium disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 118.

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    • Kidney anatomy

      illustration

      • Kidney anatomy

        illustration

      A Closer Look

        Self Care

          Tests for Fanconi syndrome

            Review Date: 2/5/2012

            Reviewed By: Melissa B Bleicher, MD, Division of Renal, Electrolyte, and Hypertension, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

            The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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