Dental arch dimensions in subjects with beta-thalassemia major.

Aim: Thalassemia is a group of inherited hemoglobinopathies with thalassemia major representing the severe form of the disease characterized by craniofacial deformities. The aim of this study is to provide a detailed description of dental arch dimensions in subjects with thalassemia major.

Subjects And Methods: The sample consisted of 43 thalassemic subjects, 24 males and 19 females, aged 7.

Characteristics, clinical features and treatment of supernumerary teeth.

Aims: To determine the characteristics, clinical features and treatment of supernumerary teeth in a general district hospital in the North of Jordan.

Design: This retrospective study was conducted at Prince Rashid Al-Hassan Hospital in Irbid. The medical records of 139 patients who were diagnosed to have supernumerary teeth during the period April 1993 - June 2007 were reviewed.

Combined cutaneous findings with segmental odontomaxillary dysplasia: Review of the literature and proposal of a new clinical classification.

Segmental odontomaxillary dysplasia is characterized by variability of its clinical and radiological features, and may mimic other fibro-osseous lesions. We describe the case of a segmental odontomaxillary dysplasia in a 12-year-old male comprising of dermal, gingival, bony, and dental abnormalities. He presented with left midfacial diffuse hyperkeratotic erythematous lesion, ipsilateral hypopigmentation of upper lip with indistinct vermilion border, left-sided facial swelling, unilateral maxillary enlargement and ipsilateral failure of eruption of permanent teeth.

Multiple developmental dental anomalies and hypermobility type Ehlers-Danlos syndrome.

Concurrent existence of multiple developmental dental anomalies: hypodontia of permanent mandibular incisors, dentin dysplasia, transmigration, root dilaceration, ectopic eruption and delayed eruption combined with systemic abnormalities including joint hyperlaxity and skin hyperextensibility aided in diagnosis of a sporadic case of hypermobility type of Ehlers-Danlos syndrome in a Jordanian Arab male. In dental practice the presence of multiple developmental dental anomalies expressing simultaneous defects in different stages of tooth development should raise suspicion of possible of manifestation of an underlying systemic abnormality.

Phenotypic diversity and revision of the nomenclature for autosomal recessive amelogenesis imperfecta.

Purpose: The purpose of this study was to characterize the phenotype in 9 families with autosomal recessive amelogenesis imperfecta (ARAI), and to propose a classification system allowing inclusion and delineation of diverse ARAI phenotypes.

Study Design: Nine families with ARAI were evaluated clinically and radiographically. Exfoliated and extracted teeth were examined via light and scanning electron microscopy, with the enamel in one case evaluated by amino acid analysis.

Demonstration of altered splicing with the IVS3-1G --> a mutation of cathepsin C.

Papillon-Lefèvre syndrome is an autosomal recessive palmoplantar keratoderma caused by cathepsin C gene mutations. We present the second family segregating the IVS3-1G --> A mutation and demonstrate for the first time that altered splicing and decreased enzymatic activity occur. RNA analysis revealed two species in carriers, corresponding to wild-type and mutant transcripts, and only the mutant transcript in affected individuals.