Objectives: To assess correlations of patient demographics, including enzyme replacement therapy (ERT) with bone histology, to facilitate decisions of whether and when to perform hip replacement surgery in patients with Gaucher disease.
Methods: We examined the histology of surgically removed femoral heads and categorized findings by the presence or extent of osteonecrosis, Gaucher cell (GC) infiltration, and bone regeneration qualifiers using a tripartite histology-based scoring system.
Results: Twenty-two patients with 26 bone specimens were evaluated.
Background. Abdominal cocoon, or sclerosing encapsulating peritonitis, is a rare condition characterized by partial or total encasement of small bowel and mesentery by a thick fibrocollagenous sack that looks like a cocoon. Within the sack, bowel loops are drawn together causing intestinal obstruction.
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