Shwachman syndrome
Natural Standard Bottom Line Monograph, Copyright © 2009 (www.naturalstandard.com). Commercial distribution prohibited. This monograph is intended for informational purposes only, and should not be interpreted as specific medical advice. You should consult with a qualified healthcare provider before making decisions about therapies and/or health conditions.
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Related Terms:
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Acute myeloid leukemia, AML, anemia, aplastic anemia, autosomal recessive, bone marrow, congenital lipomatosis of pancreas, malabsorption, MDS, metaphyseal chondrodysplasia, metaphyseal dysostosis, myelodysplastic syndrome, neutropenia, osteopenia, pancreatic insufficiency, SBDS gene, SDS, Shwachman-Bodian syndrome, Shwachman-Bodian-Diamond syndrome, Shwachman-Diamond syndrome, Shwachman-Diamond-Oski syndrome, Shwachman-Diamond-Oski-Knaw syndrome, steatorrhea, steatosis, thrombocytopenia. |
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Shwachman syndrome, also known as Shwachman-Diamond syndrome (SDS), is a rare inherited disorder that usually affects the bone marrow, pancreas, and skeletal system. |
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The main function of bone marrow is to produce blood cells for the body. These include red blood cells (which bring oxygen to tissues), white blood cells (which help fight infection), and platelets (which help in blood clotting). In patients with SDS, there is a dysfunction in the bone marrow, which may lead to bone marrow failure. |
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The pancreas is the organ responsible for making digestive enzymes that help to break down food into nutrients that can be absorbed. In patients with SDS, the pancreas loses the ability to make these enzymes. |
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More than 50% of patients with SDS are of short stature that is unrelated to nutritional problems. Bone abnormalities, also called metaphyseal chondrodysplasia, are seen mainly in the femur and tibia (leg bones) and the ribs. |
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Content available for subscribers only.
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Content available for subscribers only.
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Content available for subscribers only.
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Content available for subscribers only.
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Content available for subscribers only.
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Content available for subscribers only.
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Content available for subscribers only.
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Content available for subscribers only.
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Natural Standard Monograph
(www.naturalstandard.com)
Copyright © 2009 Natural Standard Inc. Commercial distribution or
reproduction prohibited.
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The information in this monograph is intended for informational purposes
only, and is meant to help users better understand health concerns.
Information is based on review of scientific research data, historical
practice patterns, and clinical experience. This information should not be
interpreted as specific medical advice. Users should consult with a
qualified healthcare provider for specific questions regarding therapies,
diagnosis and/or health conditions, prior to making therapeutic decisions.
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