Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26537820
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Child+Orthop
2015 ; 9
(6
): 427-32
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
AMC: amyoplasia and distal arthrogryposis
#MMPMID26537820
Kimber E
J Child Orthop
2015[Dec]; 9
(6
): 427-32
PMID26537820
show ga
Arthrogryposis multiplex congenita (AMC) is a heterogeneous condition defined as
multiple congenital joint contractures in two or more body areas. The common
pathogenesis is impaired fetal movements. Amyoplasia, the most frequent form, is
a sporadically occurring condition with hypoplastic muscles and joint
contractures. Distal arthrogryposis (DA) syndromes are often hereditary, and
joint involvement is predominantly in the hands and feet. In a Swedish study, 131
patients with arthrogryposis were investigated. The most frequent diagnoses were
amyoplasia and DA. In amyoplasia, muscle strength was found to be more important
than joint range of motion (ROM) for motor function. In DA, muscle weakness was
present in 44 % of investigated patients. The clinical findings were found to be
highly variable between families and also within families with DA. Fetal myopathy
due to sarcomeric protein dysfunction can cause DA. An early multidisciplinary
team evaluation of the child with arthrogryposis for specific diagnosis and
planning of treatment is recommended. Attention should be directed at the
development of muscle strength with early stimulation of active movements.
Immobilization should be minimized.