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Oral manifestations of Ehlers-Danlos syndrome: report of case.

First described in the beginning of this century, Ehlers-Danlos syndrome is a hereditary connective tissue disorder. This case report describes an 11-year-old girl who had frequent unexplainable bruises. During an initial orthodontic evaluation, characteristic symptoms were spotted, and a subsequent diagnosis made.

Child↗

Chronic lymphocytic leukemia of B-cell origin: oral manifestations and dental treatment planning.

Chronic lymphocytic leukemia, or CLL, is the most common form of leukemia in the Western Hemisphere, accounting for approximately 30 percent of all cases. With patients having an expected life span of more than seven years, CLL is a relatively indolent hematologic malignant disease that, while incurable, often has a prognosis compatible with relatively normal dental treatment planning. The authors present four case reports of CLL in a dental setting, as well as an update on the diagnosis, prognosis and dental treatment of patients with CLL of B-lymphocyte origin.

Aged↗

[Study of the oral manifestations in diabetic children and their correlation variables].

Because children with type 1 diabetes (DM) are prone to diseases of the buccal cavity, in this transversal study we investigate the prevalence of dental caries, gingivitis and buccal alterations in 26 patients (16F/10M; average age: 10.3 years) from the Diabetic Children's Group at the Federal University Hospital of Santa Catarina, to verify possible correlations among the following variables: index of decayed, missing or filled permanent or deciduous teeth [DMFT or dmft], index of visible plaque [IVP], index of gum bleeding [IGB], dental calculi, gum recession, tongue abnormalities, xerostomia, burning sensation of the mucosa, age at DM diagnosis, administration of insulin; occurrence of DM-related complications; glycemia and glycosilated hemoglobin [GH]. The most frequent alterations were dental calculi (42.3%), xerostomia (38.5%), burning sensation (11.5%), and gum recession (7.7%). The median indices for DMFT/dmft, IVP and IGB were 2.0, 27.21% and 10.91%, respectively. Only age and the level of GH were significantly correlated to IVP (r2 = 0.61), whereas the IVP and hypoglycemia correlated to IGB (r2 = 0.63). We conclude that the older the patient and the higher the GH level, the higher the IVP; whereas the higher the IVP and the less frequent the crisis of mild hypoglycemia, the higher the IGB.

Child↗

Clinical treatment of oral manifestations of Beckwith-Wiedeman syndrome in a child.

Orthodontic treatment in a young patient with Beckwith-Wiedeman Syndrome (BWS) is reported and a multidisciplinary approach to the management of this syndrome is reviewed. The patient presented with a tendency to Class III malocclusion, an open-bite and a slight macroglossia, which was treated at an early age by glossotomy. It was decided to monitor growth without treatment and to wait for the best time to begin therapy. It was based only on functional rehabilitation, without any fixed appliances, in which optimum intercuspation of the teeth and the skeletal Class I relationship was achieved and maintained after the retention period of three years. In conclusion the treatment of BWS patients requires a multidisciplinary approach that includes orthodontics, orthopaedics and surgical intervention. It is also necessary to underline the significance of diagnosis at an early age and timely treatment to reduce the development of dento-skeletal alterations.

Adolescent↗

Oral manifestations of juvenile hyaline fibromatosis: a case report.

Hyaline fibromatosis is a rare autosomal recessive disease of connective tissue, characterised by an accumulation of hyaline in the skin as well as various organs. The clinical features include: multiple cutaneous nodules, joint contractures, osteolytic lesions and gingival hypertrophy. This paper reports the case of an 11-year-old boy, who was referred to our dental clinic complaining of pain in his mouth. On examination, the patient had gross maxillary and mandibular gingival hyperplasia, which caused severe feeding difficulties. He also had severe dental decay, mal-positioned teeth and limited mouth opening. Treatment was done under general anesthesia to remove excess gingival tissue and extract the severely decayed teeth. Histological examination confirmed the diagnosis of juvenile hyaline fibromatosis. It was concluded that patients with this condition have special dental needs. Early diagnosis of the affected children is important in order to start early preventive dental therapy.

Child↗

A variation from classic oral manifestations associated with acute myeloblastic leukemia. A case report.

A case representing previously misdiagnosed acute myeloblastic leukemia associated with an absence of classical intraoral manifestations is presented. Platelet count was less than 15,000, and hematocrit was 20.3, yet clinical signs were limited to malaise and extreme gingival and mucosal pallor. The typical initial signs of gingival enlargement or hemorrhage never appeared, probably due to excellent plaque control by this patient. Mucosal color changes dictated the need for laboratory studies leading to a rapid and relatively early diagnosis.

Adult↗

Oral manifestation of immunoproliferative small intestinal disease. A case report.

A patient with a previous medical history of immunoproliferative small intestinal disease (IPSID) presented with persistent red and ulcerated lesions on her attached gingiva. Despite the obvious long standing gingival inflammation, minimal destruction of alveolar bone had occurred. Following failure of the tissues to respond to routine periodontal therapy, a gingival biopsy was taken. Histological assessment of the specimen revealed several features in common with the intestinal lesions seen in IPSID. In particular, a large plasma cell infiltrate subjacent to the gingival epithelium was noted. There was a marked loss of normal connective tissue morphology and evidence of epithelial degeneration. Treatment has since consisted of palliative measures only. While this is a relatively rare condition, it (as well as other disorders of the immune system) provides a useful insight into the role that the immune system plays in the inflammatory periodontal diseases.

Adult↗

Oral manifestations and dental management of immunocompromised patients.

Successful management of dental problems in immunocompromised patients requires an understanding of the diverse causes of immunodeficiency so that patients may be protected from potential hazards. Consultation with the patient's physician, oncologist, or haematologist is mandatory before any invasive dental procedure if there is concern about the disease process itself or doubt about adequate levels of leukocytes or platelets. Particular attention must be paid to maintain the intact mucosal barriers in the provision of dental care.

Dental Care for Chronically Ill↗