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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 ]
Lymphangiectasia, also known as "lymphangiectasis", [1] is a pathologic dilation of lymph vessels. [2] When it occurs in the intestines it is known as intestinal lymphangiectasia, colloquially recognized as Waldmann's disease in cases where there is no secondary cause. [3]
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.
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]
Symptoms of intestinal ischemia vary and can be acute (especially if embolic), [11] subacute, or chronic. [12] Case series report prevalence of clinical findings and provide the best available, yet biased, estimate of the sensitivity of clinical findings. [13] [14] In a series of 58 patients with intestinal ischemia due to mixed causes: [14]
In infants, feeding difficulties and poor weight gain are also frequent symptoms. [6] In some cases of SMA syndrome, severe malnutrition accompanying spontaneous wasting may occur. [7] This, in turn, increases the duodenal compression, which worsens the underlying cause, creating a cycle of worsening symptoms. [8]
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]
Bowel obstruction, also known as intestinal obstruction, is a mechanical or functional obstruction of the intestines which prevents the normal movement of the products of digestion. [2] [5] Either the small bowel or large bowel may be affected. [1] Signs and symptoms include abdominal pain, vomiting, bloating and not passing gas. [1]