Myhre syndrome is a rare multiple congenital anomalies syndrome recorded by Orphanet under ORPHAcode 2588. Orphanet describes affected people as short in stature, with distinctive facial features, short fingers and toes known as brachydactyly, stiff and thickened skin, an unusually muscular build caused by muscular pseudohypertrophy, restricted movement in the joints, hearing loss and a variable degree of intellectual disability. Involvement of the heart and circulation and of the airways is common. Orphanet lists several older and descriptive names, among them Myhre-LAPS syndrome and facial dysmorphism, intellectual disability, short stature and hearing loss syndrome. It is typed as a malformation syndrome and cross-referenced to the ICD-10 code Q87.8, in the chapter on congenital malformations, deformations and chromosomal abnormalities.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 1 Communicable or Non-communicable Transmission Route Causative Agent
Causative Agent (category) First Described Sources
1. Myhre syndrome (Orphadata)
ExternalReference, ICD-10 attributed code
Q87.8
Typology
Malformation syndrome
SummaryInformation, Definition
stiff and thick skin
SummaryInformation, Definition [body-system]
muscular pseudohypertrophy
SummaryInformation, Definition [body-system 2]
hearing loss
SummaryInformation, Definition [body-system 3]
Cardiovascular and respiratory involvement are common
View the Source2. MedlinePlus, National Library of Medicine: Myhre syndrome (MedlinePlus Genetics)
MedlinePlus, National Library of MedicineCausesQuote, Causes
Mutations in the SMAD4 gene cause Myhre syndrome.
View the Source 3. National Organization for Rare Disorders (NORD): Myhre syndrome (NORD Rare Disease Database)
National Organization for Rare Disorders (NORD)sentence that gives the year the condition was first describedQuote, sentence that gives the year the condition was first described
Myhre syndrome was first described in two unrelated males in 1981 by Drs. Myhre, Ruvalcaba and Graham.
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