A Rapp-Hodgkin like syndrome in three sibs: Clinical, dental and dermatoglyphic study


Atasu M., Akesi S., Elcioglu N., Yatmaz P., Ertas E.

CLINICAL DYSMORPHOLOGY, cilt.8, sa.2, ss.101-110, 1999 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 8 Sayı: 2
  • Basım Tarihi: 1999
  • Dergi Adı: CLINICAL DYSMORPHOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.101-110
  • Anahtar Kelimeler: ectodermal dysplasia, Rapp-Hodgkin syndrome, hypodontia, camptodactyly, cleft palate, dermatoglyphic pattern, ECTODERMAL DYSPLASIA SYNDROME, CLEFT-LIP, PILI TORTI, PALATE, ASSOCIATION, HAIR
  • Marmara Üniversitesi Adresli: Hayır

Özet

Rapp-Hodgkin ectodermal dysplasia is an autosomal dominant disorder characterized by distinctive craniofacies, cleft lip or palate, oligodontia or anodontia, hypoplasia of the nails, and a decrease in or absence of the sweat glands and hair follicles. We have identified a family in which three children display clinical features similar to Rapp-Hodgkin syndrome. The father and two other sisters of the patient had normal facial features, but had short stature and had dental anomalies, the latter suggestive of ectodermal dysplasia. The overall clinical, dental, and dermatoglyphic findings of these patients are discussed in relation to reports of families with Rapp-Hodgkin syndrome. Clin Dysmorphol 8: 101-110 (C) 1999 Lippincott Williams & Wilkins.