Rose O. Wadenya

Dental and anaesthetic management of children with dystrophic epidermolysis bullosa

BACKGROUND Epidermolysis bullosa (EB) is a genetic disease associated with fragility and bullous lesions of the skin and mucous membranes. There are various patterns of inheritance and histopathology. The disease is associated with systemic and oral manifestations, among which may be dental decay necessitating oral rehabilitation. General anaesthesia is frequently required for oral rehabilitation in children with dystrophic EB. Paediatric dentists should be aware of the implications of dystrophic EB for dental and anaesthetic management. CASE REPORT Two siblings with moderate to severe systemic and oral manifestations of dystrophic EB requiring extensive oral rehabilitation for rampant decay are described, including special anaesthetic techniques required for airway management and maintenance of skin integrity. CONCLUSION Dystrophic EB is a rare genetic disorder in which vesiculobullous lesions occur with erosions and scarring of cutaneous and extracutaneous surfaces in the oral cavity. Poor oral hygiene results from efforts to avoid mucosal trauma, resulting in decay. The comprehensive care of children with dystrophic EB impacts not only dental management but anaesthesia administration as well.

Access Impediments to Health Care and Social Services Between Anglophone and Francophone African Immigrants Living in Philadelphia with Respect to HIV/AIDS

Objectives To describe the social and cultural differences between Anglophone and Francophone African immigrants which define the impediments that Francophone African immigrants face trying to access health and human services in Philadelphia, Pennsylvania. Methods Surveys and personal interviews were administered to participants in social events, community meetings, and health centers. A Chi-squared analysis was used to contrast the communities. Results Francophone Africans demonstrated less acculturation, education, English fluency, and more legal documentation problems, and thus face greater challenges accessing health care. Anglophone Africans had a higher level of acculturation, fewer language problems, and perceived fewer barriers in accessing health care than Francophone Africans. Conclusions Educating new immigrants, through a more culturally sensitive infectious disease treatment and prevention program, is integral to achieving a higher access and utilization rates of available services; especially in recent Francophone immigrants. A larger study is needed to extend the findings to other cities where immigrants with similar backgrounds or acculturation issues reside.

Hurler syndrome: a case report of a 5-year follow-up of dental findings after bone marrow transplantation.

Hurler syndrome is a rare autosomal recessive disorder of mucopolysaccharide metabolism. It results from a deficiency in lysosomal enzymes responsible for the breakdown of glycosaminoglycans. Affected individuals may show progressive physical and mental deterioration as glycosaminoglycans are deposited in the organs of the body. Bone marrow transplantation (BMT) is effective in improving some of the clinical manifestations of Hurler syndrome. Death is caused by cardiorespiratory failure and usually occurs before the second decade of life. In this case report, the course of dental development was followed over 5 years, from the primary dentition into the permanent dentition, of a child who was successfully treated with a bone marrow transplant in infancy. The timing of bone marrow therapy has significant and variable effect on the stages of tooth development with implications for the long-term maintenance of the dentition.

Dental calcification stages of the permanent M1 and M2 in U.S. children of African-American and European-American ancestry born in the 1990s

Reported here are the preliminary results of an ongoing study undertaken to determine if there are significant changes from the results obtained in the middle of the 20th Century in the range of variation in dental development in American children of European and African ancestry. Several thousand orthopanoramic radiographs, available from the Dental Clinics of Temple University and the University of Pennsylvania, in Philadelphia, Pennsylvania will eventually be incorporated into this study. The 170 radiographs that have thus far been analyzed document significant changes in the maturation and development of the first and second permanent lower molars. Children in this study have dentitions that are maturing earlier than those in the samples published by Moorrees et al.(1963)and (1973). If the results of this preliminary analysis are confirmed by the incorporation of additional teeth and a larger sample of children, as has been reported elsewhere, it will be necessary to reconsider the characterization of dental development in living humans. Confirmation would also require re-evaluation of the range of inherent plasticity in human dental development. These preliminary results, along with the work by zihlman et.al (2004), suggest that current concepts of what constitutes ‘‘normal’’ dental development in living humans and chimpanzees may have to be reconsidered; there are also marked implications for the way in which the developing dentition in fossil hominins is characterized.

A description of two surgical and anesthetic management techniques used for a patient with fibrodysplasia ossificans progressiva

Fibrodysplasia ossificans progressiva (FOP) is a rare and debilitating genetic disorder of skeletal malformations and progressive heterotopic ossification. Flare-ups are episodic, with bone formation in skeletal muscle and connective tissue leading to ankylosis of major joints of the axial and appendicular skeleton. This report outlines the management of a patient with FOP who had ankylosis of the temporomandibular joint and progressive ossification of the neck structures. The patient underwent two different surgical and anesthetic procedures within a 10-year period to manage his oral pain. The authors compare the surgical techniques, osteotomy versus the more conservative buccal approach, anesthesia techniques, and conventional intubation versus sedated fiberoptic intubation. This report emphasizes the importance of a less invasive surgical technique and an appropriate anesthetic management that reduces the risks, cost, and morbidity associated with routine surgical management of patients with FOP.

Effect of liquid rubber additions on physical properties of Bis-GMA based dental resins

OBJECTIVE The aim of this investigation was to determine the effect of an experimental liquid rubber (LR) toughening agent on fracture toughness, crosslink density and Barcol hardness of polymerized mixtures of 50/50 wt% Bis-GMA and TEGDMA. METHODS An experimental LR, was added to a solution of Bis-GMA and TEGDMA containing 1wt% dimethylamino ethyl methacrylate and 0.5 wt% camphoroquinone at concentrations 0-10%. The composites were polymerized by exposure to a visible light curing unit for 60s. Fracture toughness was determined according to ASTM. 399. Crosslink density was evaluated using a swell test in ethanol. RESULTS Mean fracture toughness values ranged from 0.39 MPa m(1/2) for samples containing 6% to 1.24 MPa m(1/2) for samples containing 8% LR. Fracture toughness of samples containing 2%, 4%, and 8% LR were significantly higher than controls. The highest degree of cross-linkage was obtained in controls and lowest for samples containing 2% LR. Barcol Hardness values ranged from 36.7 for the control and 4% LR composition to 31.2 for samples containing 10% LR. SIGNIFICANCE Addition of 2, 4 and 8% LR increases the fracture toughness of Bis-GMA/TEGDMA composites. Addition of LR shows promise as a method of improving the fracture toughness of Bis-GMA based dental composites.