1/101. Familial cerebellar hypoplasia and pancytopenia without chromosomal breakages.Two siblings manifested a neuro-haematologic syndrome characterised by low birth weight, failure to thrive, chronic persistent tongue ulceration, severe truncal ataxia and pancytopenia without either telangiectasia or chromosomal instability. One sibling died from sepsis and the cerebellum demonstrated reduced cellularity of the molecular and granular layers with relative preservation of purkinje cells and minimal gliosis. A surviving sibling has shown haematologic progression to a myelodysplastic disorder. There was no evidence of any chromosomal instability following exposure of fibroblasts and lymphocytes to irradiation. monosomy-7 was not present in the surviving sibling. We suspect that these two patients represent another example of the rare Hoyeraal-Hreidarsson syndrome and we are currently engaged in very close monitoring of the surviving sibling for evidence of any karyotypic abnormality.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
2/101. Familial occurrence of congenital hypothyroidism due to lingual thyroid gland.Two sisters who presented with midline neck masses proved to be biochemically hypothyroid. Thyroid scintigraphy supplemented with perchlorate discharge testing showed lingual thyroid glands and ruled out the expected autosomal recessive organification defect. The related literature is reviewed.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
3/101. Manifestations of factor viii inhibitor in the head and neck.hemophilia a is the most severe of the inherited bleeding disorders. Otolaryngologists are frequently asked to assist in the care of a patient with this disorder who has intractable bleeding from sites in the head and neck. This disorder is transmitted in an X-linked recessive manner and results from factor viii deficiency. Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae in patients with hemophilia. A consequence of factor replacement therapy that occurs in as much as 16% of patients is the development of antibodies to the exogenous factor. These antibodies inactivate any supplemental factor viii, resulting in continued bleeding despite attempts at replacement therapy. This poses an extremely challenging clinical problem because other standard therapies have had limited usefulness. Activated prothrombin complex has been shown to be efficacious in the treatment of patients with inhibitors. This has led to the production of factor viii inhibitor bypassing activity (FEIBA), which contains activated forms of factors II, VII, IX, and X. The mechanism of action of this preparation remains unknown, but it seems to bypass the need for factor viii in the clotting cascade. factor viii inhibitor bypassing activity is not as effective as factor viii concentrates in patients with normally responsive hemophilia, and patients with low inhibitor levels may be given larger doses of factor viii. disseminated intravascular coagulation is most often mentioned as a potential complication of the administration of factor viii inhibitor bypassing activity, although it has been rarely reported. Otolaryngologists should be familiar with this clinical phenomenon and understand its implications. Two patients with bleeding diatheses were recently treated at our institution.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
4/101. tongue necrosis provoked by ergotamine tartrate and disclosing a giant cell arteritis.A case of tongue necrosis induced by ergotamine tartrate is reported in a patient who was suffering from an unknown giant cell arteritis (GCA). The role of ergotamine in provoking tongue necrosis in temporal arteritis has only infrequently been considered. The hypothesis concerning ergotamine-induced vasospasm potentially being able to trigger a tongue necrosis in GCA is supported by the present case. This unusual complication warns us against uncritical prescription of this drug for elderly people suffering from migraine without considering GCA.- - - - - - - - - - ranking = 5keywords = rate (Clic here for more details about this article) |
5/101. Congenital thyroid disease revisited: migrational anomalies and dyshormonogenesis.nuclear medicine scintigraphy, in conjunction with other diagnostic tools, plays a vital role in assessing patients with congenital migrational anomalies. We present 5 patients, 4 of whom were hypothyroid and 1 patient who was euthyroid. Scintigraphic and ultrasonographic images were examined and correlated with laboratory results. These patients demonstrate the spectrum of migrational anomalies of the thyroid. nuclear medicine scintigraphy was a valuable aid in establishing the location of the ectopic thyroid as well as its function.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
6/101. Delayed and immediate hypersensitivity reactions associated with the use of amalgam.hypersensitivity to the constituents of dental amalgam is uncommon. When it occurs it typically manifests itself as a lichenoid reaction involving a delayed, type IV, cell-mediated hypersensitivity response. Rarely, a more acute and generalised response can occur involving both the oral mucosa and skin. We describe two cases that illustrate the presentation and management of these two types of reaction.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
7/101. Critical review of lymphomatoid papulosis of the oral cavity with case report.A 60-year-old woman was referred to the Department of Dental Medicine at Long Island Jewish Medical Center for evaluation of multiple lesions of the tongue. She reported a long history of recurrent papular cutaneous eruptions that waxed and waned. A biopsy specimen of one of the cutaneous lesions was diagnosed as lymphomatoid papulosis. Sporadic, recurrent oral ulcers that resolved spontaneously were noted 10 to 14 days before the initial visit. These ulcers had recurred for the past 17 years. The most recent oral lesion was an erythematous, irregular, solitary ulcerated area on the middle third dorsum of tongue. The area quickly enlarged, ultimately developing extensive surface necrosis. Shortly after, a similar lesion on the posterior dorsum of the tongue developed. biopsy specimens of the former lesion showed numerous, large, atypical, pleomorphic, and hyperchromatic cells with abundant mitoses. The large, atypical cells were immunohistochemically proven to be T lymphocytes. A diagnosis of lymphomatoid papulosis was made. Two weeks later, the tongue lesions had spontaneously and totally resolved. The clinical, histomorphologic, and immunohistochemical features, as well as gene rearrangement studies of this rare entity, are presented.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
8/101. alendronate-related oral mucosa ulcerations.alendronate is widely used in the treatment of osteoporosis and other bone diseases. Although it is considered a well-tolerated drug, there are numerous reports of adverse effects on the mucosa in the upper aerodigestive tract, with oesophagitis as the most common complication. The strict regulations for the proper administration of the drug indicate that these side effects might well be the result of a direct, irritant mechanism on the upper aerodigestive tract. We present two clinical cases of patients who developed extensive palatal ulcers as a result of taking alendronate. We discuss possible mechanisms implicated in the production of the ulcers and some clinical factors of interest.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
9/101. Emergency tracheostomy following life-threatening hemorrhage in the floor of the mouth during immediate implant placement in the mandibular canine region.BACKGROUND: The edentulous interforaminal mandibular area is frequently the preferred area for implant placement. methods: A case of emergency tracheostomy following life-threatening hemorrhage in the floor of the mouth during immediate implant placement in the mandibular canine region is described. The probable cause was bleeding from the sublingual artery or a branch of that artery following implant perforation of the lingual cortex. RESULTS: Healing was uneventful and the patient was released from the hospital after 11 days. Three years later, CT showed a well-osseointegrated implant with a severe buccolingual inclination. CONCLUSIONS: It is stressed that short implants (14 mm or less) should be used in the mandibular canine region and that effective treatment of this complication is essential.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
10/101. diagnosis of acromegaly in orofacial pain: two case reports.acromegaly is an uncommon condition, with an annual incidence in the UK of three per million. The gradual onset of the clinical features mean that often friends and relatives are unaware of the underlying pathology. In view of the morbidity, and indeed mortality, arising from undiagnosed cases, general dental practitioners and other healthcare workers should routinely take note of systemic as well as intra-oral changes occurring in their patients when seen on review. The association of paraesthesia, anaesthesia and pain with acromegaly is well documented. However, there appear to be few reports linking acromegaly with orofacial pain or dysaesthesia. This paper describes two such cases.- - - - - - - - - - ranking = 3.3888620139567keywords = mortality (Clic here for more details about this article) |
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