What is Gorham’s Disease?

What is Gorham’s Disease?

br br br Gorham’s (GOR-amz) disease is a very rare skeletal condition of uncertain etiology, characterized by the uncontrolled proliferation of distended, thin-walled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone with angiomas andor fibrosis.1, 2 Because of the loss of the affected bone, the condition has been referred to as disappearing bone disease, vanishing bone disease, and massive osteolysis. In medical terminology osteolysis means: bone (osteo) breaking down or destruction (lysis). br br The first known report of the condition came in 1838 in an article titled “A Boneless Arm” in what was then The Boston Medical and Surgical Journal, now The New England Journal of Medicine.3 It is a brief but fascinating report chronicling the case of Mr. Brown who had, in 1819 at age 18 years, broken his right upper arm in an accident. The patient suffered two subsequent accidents, which fractured the arm twice more “before the curative process had been completed.” At the time of the report in 1838 the patient was reported as having remarkable use of the arm, in spite of the humerus bone having apparently disappeared—x-rays did not yet exist. Thirty-four years later, in 1872, a follow-up report was published in the same journal, following Mr. Brown’s death from pneumonia at the age of 70 years.4 The patient had requested the arm “be dissected and preserved for the benefit of medical science” and this report contains a detailed pathological description of the arm and shoulder. Abnormalities of the remaining b br ones of the arm and shoulder are noted and the authors report that the arteries, veins, and nerves appeared normal. There is no mention of lymphatic vessels. Though several reports of similar cases were published in the interim, more than 80 years would pass before another significant report of the condition appeared in the medical literature. br br Both born in 1885, Lemuel Whittington Gorham, MD, and Arthur Purdy Stout, MD, had long, distinguished careers in medicine and shared a lifelong interest in pathology.5, 6, 7 Dr. Gorham practiced and taught medicine and oncology and from the mid-1950’s through the early 1960’s conducted and reported the classical clinicopathological investigations of pulmonary embolism. During this time period he also authored several case series on osteolysis of bone. Dr. Stout began his career as a surgeon and became a pioneer in tumor pathology, publishing Human Cancer in 1932. This work became the model for the Atlas of Tumor Pathology project, which Stout oversaw as Chairman of the National Research Council in the 1950s.


User: Balanced Health Today

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Uploaded: 2017-03-29

Duration: 15:28

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