{"orpha":168,"name":"Loose anagen syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Loose anagen syndrome is a rare benign hair disorder affecting predominantly blond females in childhood and characterized by the presence of hair that can be easily and painlessly pulled out. Most of the hair is in the anagen phase and lacks an external epithelial sheath. Hair grows back quickly and the condition improves spontaneously with aging. Loose anagen hair can be associated with other anomalies, such as coloboma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-168/","orphanet_url":"https://www.orpha.net/en/disease/detail/168","names":{"fr":{"name":"Maladie des cheveux anagènes caducs","definition":"La maladie des cheveux anagènes caducs est une maladie bénigne et rare des cheveux affectant surtout les femmes blondes au cours de l'enfance. Elle se caractérise par la présence de cheveux pouvant s'arracher facilement et sans douleur. La plupart des cheveux sont en phase anagène et sont dépourvus de gaine épithéliale externe. Les cheveux repoussent rapidement et la maladie s'améliore spontanément avec l'âge. Elle peut être associée à d'autres anomalies, telles qu'un colobome."},"de":{"name":"Lose Anagenhaar-Syndrom","definition":"Das Lose Anagenhaar-Syndrom ist eine benigne Haaranomalie, von der vor allem Mädchen betroffen sind. Die Haare können leicht und schmerzlos ausgezogen werden. Die meisten dieser Haare sind in der Anagen-Phase und haben keine äußere Epithelschicht. Die Haare wachsen schnell nach, mit zunehmendem Alter kommt es zur Besserung. Lockeres Anagenhaar kann mit anderen Anomalien, z.B. Kolobom, assoziiert sein."},"es":{"name":"Síndrome del cabello anágeno suelto","definition":"El síndrome del cabello anágeno suelto es una enfermedad rara benigna del pelo que afecta predominantemente a niñas rubias y que se caracteriza por la presencia de pelo que puede arrancarse fácilmente y sin dolor. La mayoría del pelo está en la fase anágena y carece de una vaina epitelial externa. El pelo crece de nuevo rápidamente y la enfermedad mejora espontáneamente con la edad. El cabello anágeno suelto puede estar asociado con otras anomalías, como un coloboma."},"it":{"name":"Sindrome dei capelli caduchi in anagen","definition":"La sindrome dei capelli caduchi in anagen è una malattia benigna rara dei capelli che interessa, prevalentemente, le femmine bionde durante l'infanzia; è caratterizzata dalla presenza di capelli che possono essere asportati facilmente e in modo indolore. La maggior parte dei capelli è in fase anagen ed è priva di una guaina epiteliale esterna. I capelli ricrescono in fretta e la malattia migliora spontaneamente con il passare degli anni. I capelli caduchi in anagen possono associarsi ad altre anomalie, come il coloboma."},"nl":{"name":"Loose anagen-syndroom","definition":"Loose anagen-syndroom is een zeldzame goedaardige aandoening van haar die vooral blonde vrouwen in hun kindertijd treft, en gekenmerkt wordt door de aanwezigheid van haar dat makkelijk en pijnloos kan uitgetrokken worden. De meeste haren bevinden zich in de anagene fase (groeifase) en heeft geen externe epitheelschede. Haar groeit snel terug en de aandoening verbetert spontaan met ouder worden. Loszittend anageen haar kan geassocieerd zijn met andere anomalieën, zoals coloboom."},"pt":{"name":"Síndrome do cabelo em fase anágena"},"pl":{"name":"Zespół luźnych włosów anagenowych","synonyms":["Coloboma - nieprawidłowe włosy"]},"cs":{"name":"Syndrom se snadno vypadávajícími vlasy v anagenní fázi"},"uk":{"name":"Синдром укороченого анагена","definition":"Loose anagen syndrome is a rare benign hair disorder affecting predominantly blond females in childhood and characterized by the presence of hair that can be easily and painlessly pulled out. Most of the hair is in the anagen phase and lacks an external epithelial sheath. Hair grows back quickly and the condition improves spontaneously with aging. Loose anagen hair can be associated with other anomalies, such as coloboma."},"zh":{"name":"生长期毛发松动综合征"},"tr":{"name":"Gevşek anajen sendromu"}},"xrefs":[{"source":"OMIM","code":"600628","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"L65.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EC21.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"547259783"},{"source":"MONDO","code":"0010908","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D058247","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0406468","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3287","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10085066","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79364,"name":"Alopecia","classifications":[187]},{"orpha":481771,"name":"Genetic alopecia","classifications":[156]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0010721","label":"Abnormal hair whorl","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001595","label":"Abnormality of the hair","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000612","label":"Iris coloboma","frequency":"Frequent (79-30%)","criteria":null}]}}