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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 ]
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 ]
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 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]
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.
Types of mesenteric ischemia are generally separated into acute and chronic processes, because this helps determine treatment and prognosis. [4] Bowel obstruction is most often caused by intestinal adhesions, which frequently form after abdominal surgeries, or by chronic infections such as diverticulitis, hepatitis, and inflammatory bowel disease.
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Treatment targets nutritional support, improving intestinal motility, and minimizing surgical intervention. [4] Bacterial overgrowth of the small intestine can occur in chronic cases – presenting as malabsorption , diarrhea, and nutrient deficiencies [ 12 ] – which may require the use of antibiotics.