The Manifestations Of Nonne-Milroy Disease
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The Manifestations Of Nonne-Milroy Disease

Possibly caused by breaks in the VEGFR3 gene Nonne-Milroy Disease, a congenital lymphangiectasia, is the rarest form of inherited Primary Lymphedema and may manifest at birth as unilateral or bilateral edema of the legs.

Many congenital diseases can develop from abnormalities of autosomal dominant or recessive genes, that may be inherited from only one parent, and may affect more than one part of the body.   Nonne-Milroy Disease, the rarest form of Primary Lymph edema, possibly caused by breaks in the VEGFR3 gene, is a congenital lymphangiectasia, or pathological dilation of lymph vessels, that may present itself at birth as unilateral or bilateral edema of the legs.  Due to inadequate lymph fluid flow through an abnormal lymphatic system, especially in females, Nonne-Milroy Disease may manifest in the enlargement of various body parts. Family history may also contribute to this ailment's development.

The various symptoms of Nonne-Milroy Disease may include such things as fluid accumulation in the scrotum, urethra abnormalities, up-slanting toenails, prominent leg veins, Steatorrhea caused by the malfunctioning transport of chylomicron lipoprotein particles by the intestinal lymphatic system, Lymphopenia, the low level of lymphocytes in the blood that have important immune system functions, cell-mediated immunity malformations that do not release cytokines in response to antigens, Chylous ascetic fluid accumulations in the peritoneal abdominal cavity, the hardening and thickening resulting from limb fibrosis, Lymphangitis, Lymphangiosarcoma, limb tissue texture changes and Cellulitis.

Other deficiencies that may be related to Nonne-Milroy Disease include extradural cysts, vertebral deformities, pleural effusions, cleft palate, hearing loss, yellow nails, cerebrovascular defects, and Distichiasis, (as covered in Lymphedea Straight Talk, Volume 1 / Number 9, Lymph edema Distichiasis Syndrome, associated, by Brett Matthew West). 

Mutations of the FLT4 gene, which provides information for producing the endothelial growth factor receptor regulating the development and maintenance of the lymphatic system, may be another possible contributor to manifestations of Nonne-Milroy Disease..  These mutations can create growth, movement, and lymphatic cell survival abnormalities, leading to small or absent lymph vessels, in Nonne-Milroy Disease patients, however, many patients with the disease do not have mutations of this gene and the cause of their Lymph edema remains unknown, and about fifteen percent of patients with FLT4 gene mutations do not develop Nonne-Milroy Disease.

Examinations used to help determine Nonne-Milroy Disease may include CT scans, MRIs, physical examinations, Lymphangiographic x-rays of lymph nodes and lymph vessels, and Lymphoscintigraphic nuclear medicine images of the lymphatic system.  Nonne-Milroy Disease can not be cured, and De congestive Therapy remains its main treatment option, however, Compression Pump Therapy may also be prescribed for these patients.

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