Das Trichorhinophalangealsyndrom
| العنوان: | Das Trichorhinophalangealsyndrom |
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| المؤلفون: | V Schacht, Max A. Spycher, D. Tsambaos, Ralph M. Trüeb, S Borelli |
| المصدر: | Der Hautarzt. 52:51-55 |
| بيانات النشر: | Springer Science and Business Media LLC, 2001. |
| سنة النشر: | 2001 |
| مصطلحات موضوعية: | chemistry.chemical_classification, Brittle hair, Pathology, medicine.medical_specialty, integumentary system, biology, business.industry, Hair shaft, Dermatology, medicine.disease, biology.organism_classification, Langer–Giedion syndrome, Variable Expression, chemistry, Keratin, Trichorhinophalangeal syndrome, otorhinolaryngologic diseases, medicine, sense organs, Differential diagnosis, business, Cabello |
| الوصف: | In 1956 Klingmuller first described the trichorhinophalangeal syndrome (TRPS), which was named by Giedion ten years later. The syndrome includes a combination of typical hair, facial and bone abnormalities with variable expression allowing the further distinction of three subtypes. In a 37-year old patient with TRPS type I who reportedly had reduced hair growth length, clinically fine and brittle hair were found. Scanning electron microscopy revealed widely spaced cuticular scales. Quantitative measurement of the biomechanical properties of the hair showed a significant increase in the viscous parameter. This could be a result of decreased disulfide bridges and increased halogen bonds in the keratin matrix of the hair. In dermatological practice patients with TRPS often present because of hair abnormalities. Because of premature arthrosis due to skeletal abnormalities, occupational counseling is advised.Congenital heart problems, kidney abnormalities and endocrinological problems are rare, but should be sought in the symptomatic individual. Apart from mild hair care and avoidance of additional physical or chemical injuries due to hair cosmetic procedures,there is no treatment for the hair defects. |
| تدمد: | 1432-1173 0017-8470 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::780a0b9aa760bc89a9c1038f8f8df7c5 https://doi.org/10.1007/s001050051262 |
| حقوق: | CLOSED |
| رقم الأكسشن: | edsair.doi...........780a0b9aa760bc89a9c1038f8f8df7c5 |
| قاعدة البيانات: | OpenAIRE |
Find this article in full text from Springer Verlag. | تدمد: | 14321173 00178470 |
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