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Patients with intestinal lymphangiectasia present with a range of symptoms, significantly influenced by the extent of protein loss. [4] Chronic diarrhea and malabsorption are common symptoms. [ 4 ] The loss of protein can lead to edema, particularly in the legs and abdomen, due to decreased oncotic pressure. [ 4 ]
Waldmann's disease, Primary intestinal lymphangiectasia The lower legs and feet of a 23-year-old woman with Waldmann’s since infancy Waldmann disease, also known as Primary Intestinal Lymphangiectasia (PIL), is a rare disease [ 1 ] characterized by enlargement of the lymph vessels supplying the lamina propria of the small intestine . [ 2 ]
The signs and symptoms of protein losing enteropathy include diarrhea, fever, and general abdominal discomfort. [4] Swelling of the legs due to peripheral edema can also occur; however, if the PLE is related to a systemic disease such as congestive heart failure or constrictive pericarditis, then these symptoms could be due directly to the underlying illness. [2]
Although symptom presentation may vary, patients generally present with early-onset gastrointestinal symptoms, edema, malnutrition, hypoalbuminemia, and hypogammaglobulinemia. [1] Histopathological assessment of intestinal biopsy samples or resections revealed extensive lymphangiectasia , and suggest a diagnosis of primary intestinal ...
The treatment of intestinal ischemia depends on the cause and can be medical or surgical. However, if bowel has become necrotic, the only treatment is surgical removal of the dead segments of bowel. [34] In non-occlusive disease, where there is no blockage of the arteries supplying the bowel, the treatment is medical rather than surgical ...
Hennekam syndrome, also known as intestinal lymphagiectasia–lymphedema–mental retardation syndrome, [1] is an autosomal recessive disorder consisting of intestinal lymphangiectasia, facial anomalies, peripheral lymphedema, and mild to moderate levels of growth and intellectual disability.
Aplasia cutis congenita-intestinal lymphangiectasia syndrome is a very rare genetic disorder which is characterized by aplasia cutis congenita, intestinal lymphangiectasia-induced generalized edema, hypoproteinemia, and lymphopenia. [1] [2] It has been described in two Ashkenazi Jewish brothers. [3] [4]
Vitamin D treatment may be associated with less inflammatory bowel disease reoccurrence of symptoms (relapse). It is not clear if this treatment improves the person's quality of life or what the clinical response to vitamin D treatment. The ideal treatment regime and dose of vitamin D therapy has not been well enough studied. [90]