Biomaterial Database

[Larsen syndrome--symptomatics and therapy (author's transl)]. 1978

A Haarmeyer

We report of three children suffering from Larsen-syndrome with the typical deformity of the face and multiple, mostly bilateral joint-luxations with severe affection of the hip-joints and knee-joints. Two patients had short terminal phalanxes with the typically spatulate terminal phalanxes of the thumb, radiographically visible. One child showed bilaterally dysplastic metacarpophalangeal bones with luxated metaphalanxes. The diagnosis could furtheron be verified by the characteristically twin-formed bone-nucleus of the calcaneus within all patients. Two children early showed a severe, progrediently developing scoliosis of the thoracal spine. The genetic aetiology of this syndrome, caused by a congenital weakness of the connective tissue, is not yet cleared completely. Especially the severe knee-joint luxations are therapeutically a critical problem. The multiple operative interventions which are necessary in the regions of the knee-joint, do not always lead to the expected success of treatment on account of the considerable weakness of the connective tissue. Therefore, additionally orthopaedic remedies are necessary. The already early progrediently developing scoliosis of the spine requires careful treatments with Milwaukee brace. A spondylodesis should be performed in the age from 6 to 8 years on account of the progredience. In any case, it is necessary to investigate carefully for accompanying malformations of the urinary passage.

UI	MeSH Term	Description	Entries
D007719	Knee Joint	A synovial hinge connection formed between the bones of the FEMUR; TIBIA; and PATELLA.	Superior Tibiofibular Joint,Joint, Knee,Joint, Superior Tibiofibular,Knee Joints,Superior Tibiofibular Joints,Tibiofibular Joint, Superior
D008297	Male		Males
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003025	Clubfoot	A deformed foot in which the foot is plantarflexed, inverted, and adducted.	Equinovarus,Talipes Equinovarus,Clubfeet,Clubfeet, Congenital,Clubfoot, Congenital,Congenital Talipes Equinovarus,Pie Torcido,Congenital Clubfeet,Congenital Clubfoot,Pie Torcidos,Talipes Equinovarus, Congenital
D004204	Joint Dislocations	Displacement of bones from their normal positions at a joint.	Inferior Dislocation,Joint Subluxations,Luxatio Erecta,Dislocation, Joint,Dislocations, Joint,Inferior Dislocations,Joint Dislocation,Joint Subluxation,Subluxation, Joint,Subluxations, Joint
D005145	Face	The anterior portion of the head that includes the skin, muscles, and structures of the forehead, eyes, nose, mouth, cheeks, and jaw.	Faces
D005260	Female		Females
D006618	Hip Dislocation, Congenital	Congenital dislocation of the hip generally includes subluxation of the femoral head, acetabular dysplasia, and complete dislocation of the femoral head from the true acetabulum. This condition occurs in approximately 1 in 1000 live births and is more common in females than in males.	Hip Dysplasia, Congenital,Congenital Dysplasia Of The Hip,Congenital Hip Dislocation,Congenital Hip Displacement,Congenital Hip Dysplasia,Dislocation Of Hip, Congenital,Dislocation, Congenital Hip,Displacement, Congenital Hip,Dysplasia, Congenital Hip,Hip Displacement, Congenital,Hip Dysplasia, Congenital, Nonsyndromic,Hip, Dislocation Of, Congenital,Congenital Hip Dislocations,Congenital Hip Displacements,Congenital Hip Dysplasias,Dislocations, Congenital Hip,Displacements, Congenital Hip,Dysplasias, Congenital Hip,Hip Dislocations, Congenital,Hip Displacements, Congenital,Hip Dysplasias, Congenital
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man