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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]
Idiopathic multicentric Castleman disease (iMCD) is a subtype of Castleman disease (also known as giant lymph node hyperplasia, lymphoid hamartoma, or angiofollicular lymph node hyperplasia), a group of lymphoproliferative disorders characterized by lymph node enlargement, characteristic features on microscopic analysis of enlarged lymph node tissue, and a range of symptoms and clinical findings.
Thomas A. Waldmann was born in New York City as the only child of Elisabeth Sipos and Charles Waldmann. He received his M.D. from Harvard Medical School in 1955. He joined the National Cancer Institute in 1956 and became chief of the Lymphoid Malignancies Branch (formerly Metabolism Branch) in 1973.
The investigators then obtain a standard set of clinical observations on each patient and a test or clinical follow-up to define the true state of the patient. They then use statistical methods to identify the best clinical predictors of the patient's true state. The probability of disease will depend on the patient's key clinical predictors.
Human infectious diseases may be characterized by their case fatality rate (CFR), the proportion of people diagnosed with a disease who die from it (cf. mortality rate).It should not be confused with the infection fatality rate (IFR), the estimated proportion of people infected by a disease-causing agent, including asymptomatic and undiagnosed infections, who die from the disease.
Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease and azathioprine, methotrexate, or rituximab to keep the disease under control. [1] [7] [8] Plasma exchange is also used in severe cases with damage to ...
Behavioral symptoms affect relations with other people and may include short attention span, intractability, and delays in achieving age-appropriate behaviors. Sociability with peers, however, is a strength in GLUT1 deficiency patients. [5] Movement symptoms relate to the quality of motor functions. Walking may be delayed or difficult because ...