Unknown syndrome: peculiar face, severe hypodontia of permanent teeth, and precocious choroid calcifications.
نویسندگان
چکیده
We describe a mother and her twin daughters affected with severe hypodontia of the permanent teeth, precocious calcification of the choroid plexus, and minor digital anomalies. The presence of inner telecanthus, broad and flattened nasal bridge, mild ocular proptosis, small nose with anteverted nostrils, and slight microretrognathia gives them an unusual appearance. All three affected persons are of normal intelligence.
منابع مشابه
Craniofacial profile in Southern Chinese with hypodontia.
The association between craniofacial morphology and congenitally missing teeth is at present unclear. The aims of this study were to investigate whether hypodontia is associated with changes in the sagittal skeletal profile and to identify putative relationships between the skeletal profile and the severity of hypodontia. In a cross-sectional analytical study, the craniofacial structure and pro...
متن کاملBilateral hypodontia is more common than unilateral hypodontia in children with Down syndrome: a prospective population-based study.
BACKGROUND In individuals with simple hypodontia, congenital absence of teeth commonly affects just one tooth of a pair, not both. However, patterns of hypodontia have not been fully explored in children with Down syndrome (DS). OBJECTIVE We describe the frequency and left-right symmetry of hypodontia in the permanent dentition of 8- to 9-year-old Norwegian children with DS. MATERIALS AND M...
متن کاملIncontinenta pigmenti (Bloch-Sulzberger disease) در یک دختر 10 ساله
Abstract Incontinenta pigmenti or Bloch-Sulzberger syndrome is a rare x-linked dominant disease that mainly affects the skin, eye, hair, central nervous system and teeth. The disease is predominant among women. Dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome. Oral involvement characterized by hypodontia of deciduous and permanent teeth is i...
متن کاملAn orthopantomographic study of hypodontia in permanent teeth of Japanese pediatric patients.
Hypodontia of permanent teeth was evaluated from orthopantomograms of 2072 apparently healthy pediatric patients at The Hospital of Nihon University School of Dentistry at Matsudo. The prevalence of congenitally missing teeth (CMT) was 8.7% in boys and 10.8% in girls, and 9.4% for both sexes combined. Most cases (67.8%) involved either one or two missing teeth. There were in total 574 CMT, and ...
متن کاملIncontinentia pigmenti presenting as hypodontia in a 3-year-old girl: a case report
INTRODUCTION Incontinentia pigmenti or Bloch-Sulzberger syndrome is a rare X-linked dominant disease that mainly affects the skin, eyes, hair, central nervous system and teeth. The disease is predominant among women. Although dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome, they are less damaging to the patient and do not require treatment. How...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Journal of medical genetics
دوره 35 5 شماره
صفحات -
تاریخ انتشار 1998