Oral features and dental health in Hurler Syndrome following hematopoietic stem cell transplantation.

BACKGROUND: Hurler Syndrome is associated with a deficiency of a specific lysosomal enzyme involved in the degradation of glycosaminoglycans. Hematopoietic stem cell transplantation (HSCT) in early infancy is undertaken to help prevent the accumulation of glycosaminoglycans and improve organ function. AIM: To investigate the oral features and dental health of patients with Hurler Syndrome who have undergone successful HSCT. MATERIALS AND METHODS: Twenty-five patients (median age 8.6 years) post-HSCT (mean age 9.4 months) underwent oral assessment (mean of 7.5 years post-HSCT). RESULTS: Dental development was delayed. Numerous occlusal anomalies were noted including: open-bite, class III skeletal base, dental spacing, primary molar infra-occlusion and ectopic tooth eruption. Dental anomalies included hypodontia, microdontia, enamel defects, thin tapering canine crowns, pointed molar cusps, bulbous molar crowns and molar taurodontism. Tooth roots were usually short/blunted/spindle-like in permanent molars. The prevalence of dental caries was low in the permanent dentition (mean DMFT 0.7) but high in the primary dentition (mean dmft 2.4). Oral hygiene instruction with plaque and or calculus removal was indicated in 71% of those that were dentate. CONCLUSION: Patients with Hurler Syndrome post-HSCT are likely to have delayed dental development, a malocclusion, and dental anomalies, particularly hypodontia and microdontia.

Dental health and management for children with congenital heart disease.

Congenital heart disease (CHD) is one of the most common developmental anomalies. Children with CHD are at increased risk of developing oral disease, and are at increased risk from the systemic effects of oral disease. Recent changes in guidelines related to prophylaxis against infective endocarditis have highlighted the importance of establishing and maintaining oral health for this group of patients. The management of children with CHD can be complex and, unfortunately, many of these children do not receive the care they require. The challenges that these children pose are discussed, and suggestions are made for the appropriate management of these patients and the key role that all those working in primary dental care have to play.

Alveolar lymphangioma in infants: report of two cases.

The alveolar lymphangioma is a benign but relatively rare condition found only in the oral cavities of black infants. Dentists practising in Ireland may be unaware of this condition due to its racial specificity. This paper presents two case reports of multiple alveolar lymphangiomas found in black infants in a children's hospital in Ireland. The epidemiology, aetiology, clinical presentation, histology, and management options are discussed. The photographs should aid the practitioner in recognising these lesions.

Sickle cell disease and dental treatment.

Sickle cell disease (SCD) and sickle cell trait (SCT) are found most frequently in individuals of African, Middle Eastern and Indian ethnicity. Population migration has made this disease more common worldwide, including Ireland. We present an overview of this disease, focusing on management and practical implications for dental practitioners.

Pharmaceutical prescribing for children. Part 6. The management of medical emergencies in children in dental practice.

This paper is the seventh in the series on prescribing medicines for child dental patients. Here, the authors aim to describe the medical emergencies that may occur in children presenting for dental treatment and to provide guidelines on their management, with particular reference to the use of emergency drugs.

Pharmaceutical prescribing for children. Part 6. Dental management and prescribing for the immunocompromised child.

This paper is the sixth in a series on the prescribing of medicines for children by dentists working in primary care. It deals with the dental management and prescribing of medicines for the immunocompromised child. It differs from previous papers in the series in that, apart from the prescription of medicaments such as fluoride supplements and chlorhexidine mouthwashes, most medication for this group of children is prescribed by specialists working in secondary care rather than by primary dental care practitioners.

Dentoalveolar trauma in a patient with chronic idiopathic thrombocytopenic purpura: a case report.

A case is presented of a 13-year-old boy with chronic idiopathic thrombocytopenic purpura (ITP) who sustained traumatic labial luxation of both lower central incisors, with partial alveolar fracture resulting in displacement of the labial alveolar plate. Intravenous immunoglobulin (Fleibogamma, 1 g/kg body weight x 2 days) was administered, resulting in the patient's platelet count rising from 15,000/mm3 to 70,000/mm3. Under general anesthesia, the displaced lower labial alveolus and luxated teeth were repositioned and splinted 2 days following trauma. Healing was uneventful. Subsequently, both lower central incisors became nonvital and were endodontically treated. The dental treatment of this patient with ITP is discussed in terms of emergency management, and subsequent care.

Congenital insensitivity to pain--review and report of a case with dental implications.

Pain is a protective mechanism for the body. Absence of pain is a symptom in several disorders, both congenital and acquired. The congenital types are present at birth and affect the number and distribution of types of nerve fibers. At present, 5 types of hereditary sensory and autonomic neuropathies have been identified. The various disorders within this group are classified according to the different patterns of sensory and autonomic dysfunction and peripheral neuropathy and the presence of additional clinical features such as learning disability. However, the field is currently moving away from classification based on clinical presentation toward classification based on underlying genetic abnormality. In the absence of pain, patients are at risk of late presentation with illnesses or injuries, and have an increased incidence of traumatic injury. Self-mutilation is an almost invariable feature of these disorders. We report the case of a patient with congenital insensitivity to pain that presented with self-mutilation injuries to his hands and oral tissues caused by biting. The severe nature of these injuries necessitated serial extraction of his primary teeth soon after eruption, which led to a cessation of the problem. The mutilation has not returned following the eruption of the first of his permanent teeth, suggesting that he has learned not to bite himself, even though to do so causes him no discomfort.

A prospective study of the oral manifestations of Crohn's disease.

BACKGROUND & AIMS: Recent studies suggest that the mouth may be involved frequently in patients with Crohn's disease (CD). The aim of this study was to document prospectively the proportion of children with oral lesions at diagnosis of CD, to describe the type of lesions found, and to examine the ability of gastroenterologists to identify correctly oral Crohn's manifestations. METHODS: In a prospective 3-year study, systematic dental examinations were performed on all children with suspected inflammatory bowel disease. Each child underwent upper endoscopy, colonoscopy, and barium follow-through radiography. RESULTS: Forty-eight of 49 children with CD were examined by the dentist. Oral CD was found in 20 patients (41.7%). Oral findings included mucogingivitis (12 patients), mucosal tags (4 patients), deep ulceration (4 patients), cobblestoning (3 patients), lip swelling (3 patients), and pyostomatitis vegetans (1 patient). Noncaseating granulomas were found in all 8 oral biopsy specimens from oral CD lesions (100%). Two patients with granulomas in oral biopsy specimens had no granulomas found in any other biopsy specimens. The presence of oral manifestations was associated with perianal disease. In only 9 patients (45%) with oral CD was the mouth found to be abnormal by the consultant gastroenterologists. Only nonspecific oral changes were seen in children with ulcerative colitis and indeterminate colitis. CONCLUSIONS: More than one third of all children presenting with CD had involvement of the mouth. The ability of physicians to recognize oral lesions was poor. Expert dental evaluation may be useful during the investigation of patients with suspected inflammatory bowel disease.