Disease Information for Lymphedema, hereditary

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Clinical Manifestations
Signs & Symptoms
Edema Children
Bilateral ankle swelling
Bilateral leg edema/swelling
Hypertrophic lymphatics/subcutaneous/signs
Indurated lymphatics/extremity/regional
Edema of Lower Extremities
Edema, lower extremity (leg) , unilateral
Foot Swelling
Leg/lower extremity pains
Lymphedema of lower extremities
Lymphedema of one leg
Swelling one extremity/limb
Swollen Limb
Thigh/Leg Swelling Unilateral Severe
Unilateral leg swelling
Unilateral Leg swelling in Children
Neonatal edema/anasarca
Disease Progression
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Associated Diseases & Rule outs
Rule Outs
Elephantiasis
Associated Disease & Complications
Cellulitis, leg/ankles
Edema
Hemihypertrophy
Lymphedema, hereditary
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Dermatologic/Subcutaneous (category)
CLASS/Lymphatics/channels disorders (ex)
CLASS/Lymphatics/lymph (category)
CLASS/Extremities/Digits/Hand feet disorder (category)
CLASS/Muscle/tendon/extremities (category)
Pathophysiology
Pathophysiology/Edematous process
Pathophysiology/Gene locus 16q24.3
Pathophysiology/Gene locus 5q35.3
Pathophysiology/Gene locus Chromosome 16
Pathophysiology/Gene locus Chromosome 5
Pathophysiology/Gene Locus Identified/OMIM database
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Process
PROCESS/Congenital/developmental (category)
PROCESS/Hereditofamilial (category)
Synonyms
Synonym
Congenital lymphedema, Congenital lymphoedema, Disease Milroy, Disease Milroy's, edema Milroy, Hereditary lymphedema type I, Hereditary lymphedema type I (disorder), Hereditary lymphoedema type I, Infantile lymphedema (praecox), Infantile lymphedema(praecox), Infantile lymphoedema (praecox), Infantile lymphoedema(praecox), Lymphedema congenital, LYMPHEDEMA EARLY ONSET, LYMPHEDEMA HEREDITARY I, Lymphoedema congenital, Milroy, MILROY DIS, MILROY DISEASE, Milroy edema, Milroy lymphedema, Milroy lymphoedema, MILROYS DIS, Milroys Disease, Milroy's Disease, Milroy's disease (disorder), Nonne Milroy, NONNE MILROY LYMPHEDEMA, Nonne Milroy lymphoedema, NONNE MILROY MEIGE SYNDROME, PCL, Primary (congenit )lymphedema, Primary (congenit )lymphoedema, Primary (congenital) lymphedema, Primary (congenital) lymphoedema, PRIMARY CONGENITAL LYMPHEDEMA, Primary congenital lymphoedema, Synonym/Milroy's disease, Synonym/Nonne-Milroy-Meige syndrome
Definition

Meige"s syndrome I; here is some confusion among authors about what is what with Meige’s syndrome, Milroy’s syndrome, Nonne’s syndrome and Nonne-Milroy-Meige syndrome; According to one author, hereditary lymphoedema type II is the Meige syndrome; hereditary lymphoedema I is the Nonne-Milroy syndrome, also commonly referred to as Milroy disease; Some authors still combine these into a single syndrome, the Nonne-Milroy-Meige syndrome;Meige"s disease;

Milroy"s disease; Nonne’s syndrome; Nonne-Milroy syndrome

Meige"s syndrome I; Chronic hereditary trophoedema, chronic hereditary lymphoedema, congenital elephantiasis, congenital lymphedema, elephantiasis congenita hereditaria (Nonne); familial hereditary oedema, hereditary lymphedema I (Nonne-Milroy); hereditary lymphedema II (Meige), hereditary tropholymphoedema syndrome; idiopathic hereditary lymphedema, lymphoedema; lymphedema, early onset type; trophoedema hereditarium (chronicum extremitatum inferiorum); tropholymphoedema;

Chronic familial lymphoedema of the limbs, which manifests with pittings and brawny swellings (lymphoedema) of the ankles and shins; Often associated with inflammation and various defects, including distichiasis, extradural cysts, vertebral anomalies, cerebrovascular malformations, yellow nails, and sensorineural hearing loss; Congenital or onset in first or second decade of life; It is due to inadequate lymph flow through an anatomically abnormal lymphatic system; Prevalent in females (70-80%); Pain, sometimes severe, is a common feature; Autosomal dominant inheritance with variable expressivity; There is a lot of confusion about the correct term for this condition; According to some authors the term Nonne-Milroy-Meige syndrome is obsolete, as this is really two distinct entities, Hereditary lymphedema I (Nonne-Milroy syndrome) with onset at birth to 35 years of age; and hereditary lymphedema type II (Meige syndrome) with onset after 35 years; -------------

[whonamedit_com 2005]---------------

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