Preferred Name

先天性多发性关节挛缩 / Arthrogryposis multiplex congenita

Definitions

Arthrogryposis multiplex congenita, comprises nonprogressive congenital conditions characterized by multiple joint contractures. The term is currently used in connection with a very heterogeneous group of disorders that all include multiple congenital joint contractures. The major cause of arthrogryposis is fetal akinesia due to fetal abnormalities (e.g. neurogenic, muscle, or connective tissue abnormalities; mechanical limitations to movement) or maternal disorders (e.g. infection, drugs, trauma, other maternal illnesses). Generalized fetal akinesia can also lead to polyhydramnios, pulmonary hypoplasia, micrognathia, ocular hypertelorism, and short umbilical cord. Lack of fetal movement causes extra connective tissue to develop around the joint, limiting movement and further aggravating the joint contracture.

ID

http://purl.bmicc.cn/ontology/ICD11CN/LD26.41

Coded_Elsewhere

Arthrogryposis - renal dysfunction - cholestasis (5C58.0Y)

definition

Arthrogryposis multiplex congenita, comprises nonprogressive congenital conditions characterized by multiple joint contractures. The term is currently used in connection with a very heterogeneous group of disorders that all include multiple congenital joint contractures. The major cause of arthrogryposis is fetal akinesia due to fetal abnormalities (e.g. neurogenic, muscle, or connective tissue abnormalities; mechanical limitations to movement) or maternal disorders (e.g. infection, drugs, trauma, other maternal illnesses). Generalized fetal akinesia can also lead to polyhydramnios, pulmonary hypoplasia, micrognathia, ocular hypertelorism, and short umbilical cord. Lack of fetal movement causes extra connective tissue to develop around the joint, limiting movement and further aggravating the joint contracture.

exclusions

Arthrogryposis multiplex congenita - lissencephaly (LD2F.1)

COFS syndrome (LD2B)

hasDbXref

http://id.who.int/icd/release/11/2019-04/mms/1930990330

label

先天性多发性关节挛缩 / Arthrogryposis multiplex congenita

mappingRelation

http://purl.bmicc.cn/ontology/ICD10CN/Q74.3

notation

LD26.41

prefixIRI

ICD11CN:LD26.41

prefLabel

先天性多发性关节挛缩 / Arthrogryposis multiplex congenita

subClassOf

http://purl.bmicc.cn/ontology/ICD11CN/LD26.4

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