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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.
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 ]
For babies with atypical ACD, response to medical therapy is more sustained, lasting for several months. For those that can be stabilized, definitive treatment is bilateral lung transplantation. [1] ACD is a rare disease. About 100 cases have been reported. [1] The first case was reported in 1981. [5]
Macrocystic have cysts greater than 2 cubic centimetres (0.12 cu in), and microcystic lymphatic malformation have cysts that are smaller than 2 cubic centimetres (0.12 cu in). [2] These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck.
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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]
Children often have generalized lymphadenopathy of the head and neck, or even PGL, without the finding of a sinister cause. At puberty this usually disappears. The immune system of some people may be sensitized by exposure to a living exogenous irritant such as a bacterial or viral infection , which then results in PGL after the organism has ...