1/8. Simultaneous occurrence of unusual odontodysplasia and oligodontia in the permanent dentition: report of a case.odontodysplasia is an uncommon clinicopathological condition with a variety of expressions. Although it is generally recognized as a localized disorder of dental tissue, its aetiology has not yet been well explained. In the present case, odontodysplasia with oligodontia in the permanent dentition is reported. The patient was in good health with normal stature and no other physical abnormalities. His parents and siblings were dentally and medically normal. The primary teeth appeared to be normal except for the primary second molars, where the enamel was malformed. However, the permanent incisors that had erupted into the oral cavity showed rough and hypoplastic enamel. An orthopantomogram showed 17 congenitally missing permanent teeth and malformation of the other 11 permanent teeth and tooth-germs. Because these findings were caused by developmental disturbances of both the mesodermal and ectodermal dental components, we diagnosed the present case as odontodysplasia accompanied by oligodontia in the permanent dentition.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/8. Use of dental implants in patients with down syndrome: a case report.down syndrome is caused by trisomy of the 21st chromosome and is associated with well-described physical and systemic problems. Most people with down syndrome have some degree of mental retardation as well as malformation of head and neck. Oral structures that are commonly affected include the tongue (macroglossia), abnormalities in the number and shape of teeth, and poor quality (osteoporotic-like) of alveolar bone and jaw. These oral malformations as well as a tendency toward poor cooperation in the dental office contribute to the belief among dentists that people with down syndrome are not good candidates for oral rehabilitation with dental implants. This article describes the use of dental implants in the oral rehabilitation of a 16-year-old boy with down syndrome. Although more experience is needed before dental implants can be considered a suitable option during oral rehabilitation in people with down syndrome, this case report shows a promising beginning.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/8. Hurler's syndrome: dental findings in a case treated with bone marrow transplantation in infancy.Hurler's syndrome, also known as mucopolysaccharidosis i (MPS I-H), is a rare condition inherited as an autosomal recessive trait. It is caused by a deficiency in alpha-L-iduronidase, an enzyme that participates in the degradation of the glycosaminoglycans (GAGs) heparin sulphate and dermatan sulphate. Children with Hurler's syndrome appear nearly normal at birth but, left untreated, show a progressive mental and physical deterioration caused by a build-up of GAGs in all organs of the body. death is often caused by cardiac or respiratory failure and usually occurs before the second decade of life. In recent years, bone marrow transplantation (BMT) has been employed in the management of patients with Hurler's syndrome. However, the dental findings observed in these cases have not previously been reported in the dental literature. Here we report a patient aged 11 years and 6 months, presented to a Specialist Paediatric dentistry Unit, who was successfully treated by BMT at 18 months of age.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/8. Oligodontia in the primary dentition with permanent successors: report of case.A case of oligodontia in the primary dentition with corresponding permanent successors is described. A two-year-old Japanese boy was admitted to the pedodontic clinic with the chief complaint of congenitally missing primary teeth. The boy appeared to be normally developed and was in good physical health. There was no history of trauma, extraction, or exfoliation of primary teeth. Clinical examination showed that eight anterior teeth were missing. Also, a canine and three first molars were much smaller in size and were malformed. However, radiographic examination at age four years, ten months showed that all of the permanent tooth germs--including the corresponding successors of congenitally missing primary teeth--were evident.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/8. Oligodontia, short stature and small head circumference with normal intelligence.Complete absence of third molars and mandibular permanent second molars is reported in a 19-year-old female, with occipito-frontal head circumference and height at the 3rd centile. The patient's intelligence, appearance and physical examination were normal. Direct count of the patient's sweat pores in different areas of the palms and digits was normal. microcephaly, short stature and normal intelligence have been reported in two families as a distinct autosomal dominant syndrome. To the best of our knowledge, oligodontia, in addition to these findings, has not been previously reported.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/8. GAPO syndrome: report on the first case in japan.We studied a 3.5-year-old Japanese boy with growth retardation, alopecia, pseudoanodontia, and bilateral papilledema. He was born of nonconsanguineous parents, but his paternal grandparents were related. From his characteristic physical manifestations, we diagnosed him as the first known case of GAPO syndrome in japan and perhaps in the Mongoloid race. Our case had prominent dilatation of scalp veins and an audible intracranial bruit. Cranial angiography documented a narrowing of the sigmoid sinuses, with no flow to either jugular vein. We discuss here the relationships between optic atrophy and intracranial vascular changes in this syndrome.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/8. Features of oligodontia in three generations.Congenitally missing teeth of three generations of a family and the spousal influence on the oligodontia of the third generation is investigated. For clarification, a review of commonly used terms is included in the paper. Although there are a number of factors affecting the failure to develop permanent tooth buds, most researchers believe that an autosomal dominant trait is responsible for the oligodontia in this family. Many syndromes are associated with oligodontia, but none seem to be prevalent in the direct descendants of the first generation. A member of the second generation married a person with Witkop's (tooth-and-nail) syndrome. Subsequently, their child exhibits the same manifestations. A feature noted in this three generation pedigree is missing permanent first molars. This is considered rare, especially when few abnormal ectodermal findings are reported. Additional findings include an increased number of missing teeth, decreased tooth size and a prominent maxillary labial frenum. The dental treatment for patients affected will likely involve a multidisciplinary approach. The treatment is particularly important since the lack of a full complement of teeth impacts the emotional and physical well-being of the individual. Children who are missing permanent teeth should be screened for other ectodermal abnormalities to rule out syndromes associated with congenitally missing teeth. Oligodontia is predicted to affect fifty percent of the fourth generation. It may vary in penetrance and expression.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/8. Male cases of incontinentia pigmenti: case report and review.Male patients with Bloch-Sulzberger incontinentia pigmenti (IP type II) are rare and more severely affected than their female counterparts, with a significant occurrence of sex chromosome aneuploidy. This document introduces a new male IP type II patient and reviews 48 males reported with IP. Twenty-eight of the 49 patients meet current criteria for diagnosis of IP type II. The phenotype is variable and the incidence of documented developmental delay is 25%. Five patients had klinefelter syndrome (47,XXY). Most patients were reported prior to 1961 when chromosome analysis was not available. biopsy and laboratory reports considered to be "consistent with" the diagnosis of IP were seen in patients meeting criteria as well as those who would not currently be given the diagnosis. The histologic findings considered diagnostic are varied. This variability may be accounted for by differences in stage of disease, biopsy site, histologic technique, and reporting style. Conversely, this may indicate that the diagnostic weight given to the biopsy should be reconsidered. eosinophilia was not a consistent finding. overall, differences in reporting, ascertainment, and length of follow-up lead to difficulty in interpreting or predicting the natural history of males with IP type II. Based on the existing literature, they appear to have a higher rate of mental retardation than the general population, but there does not appear to be a correlation between severity of physical and mental involvement. The presence of sex chromosome aneuploidy documented in the more recent cases emphasizes the need for chromosome analysis in any male patient suspected of IP type II.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |