Congenital contractural arachnodactyly (Q46716): Difference between revisions
From determinar.ia.br - Determine suas informações
Created a new Item |
Changed an Item |
||||||||||||||
| (7 intermediate revisions by the same user not shown) | |||||||||||||||
| description / pt-br | description / pt-br | ||||||||||||||
Aracnodactilia contratural congênita (ACC, síndrome de Beals) é um transtorno do tecido conjuntivo caracterizado por múltiplas contraturas em flexão, aracnodactilia, cifoescoliose grave, pavilhão auricular anormal e hipoplasia muscular. Embora as características clínicas possam ser semelhantes à síndrome de Marfan (SMF), múltiplas contraturas articulares (especialmente de articulações de cotovelo, joelho e dedos) e orelhas dobradas na ausência de dilatação significativa da raiz da aorta são características da síndrome de Beals e raramente encontradas em SMF. | |||||||||||||||
| description / en | description / en | ||||||||||||||
Congenital contractural arachnodactyly (CCA, Beals syndrome) is a connective tissue disorder characterised by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia. Although the clinical features can be similar to Marfan syndrome (MFS), multiple joint contractures (especially of the elbow, knee, and finger joints), and crumpled ears in the absence of significant aortic root dilatation are characteristic of Beals syndrome and rarely found in MFS. | |||||||||||||||
| Property / Canonical URI | |||||||||||||||
| Property / Canonical URI: https://id.who.int/icd/entity/1376425921 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / CURIE | |||||||||||||||
CID11:LD28.00 | |||||||||||||||
| Property / CURIE: CID11:LD28.00 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Canary Token | |||||||||||||||
dki-india-LD28.00 | |||||||||||||||
| Property / Canary Token: dki-india-LD28.00 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Verification Status | |||||||||||||||
Concluído | |||||||||||||||
| Property / Verification Status: Concluído / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Knowledge Architect | |||||||||||||||
| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Collection date | |||||||||||||||
13 August 2026
| |||||||||||||||
| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Linked ICD 10 | |||||||||||||||
| Property / Linked ICD 10: MAURO SERGIO BRUNO / rank | |||||||||||||||
Normal rank | |||||||||||||||
Latest revision as of 15:30, 13 August 2026
Congenital contractural arachnodactyly (CCA, Beals syndrome) is a connective tissue disorder characterised by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia. Although the clinical features can be similar to Marfan syndrome (MFS), multiple joint contractures (especially of the elbow, knee, and finger joints), and crumpled ears in the absence of significant aortic root dilatation are characteristic of Beals syndrome and rarely found in MFS.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | LD28.00 |
||
| English | Congenital contractural arachnodactyly |
Congenital contractural arachnodactyly (CCA, Beals syndrome) is a connective tissue disorder characterised by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia. Although the clinical features can be similar to Marfan syndrome (MFS), multiple joint contractures (especially of the elbow, knee, and finger joints), and crumpled ears in the absence of significant aortic root dilatation are characteristic of Beals syndrome and rarely found in MFS. |
Statements
CID11:LD28.00
0 references
dki-india-LD28.00
0 references
Concluído
0 references
13 August 2026
0 references
