Cleidocranial dysplasia (CCD) is an uncommon, generalized skeletal disorder characterized by delayed ossification of the skull, aplastic or hypoplastic clavicles, and serious, complex dental abnormalities. There are many difficulties in the early diagnosis of CCD because a majority of the craniofacial abnormalities becomes obvious only during adolescence. In the present case, a hypoplastic midface, a relative prognathia of the mandible, and close approximation of the shoulders in the anterior plane were the conspicuous extraoral findings. Prolonged exfoliation of the primary dentition, unerupted supernumerary teeth, and the irregularly and partially erupted secondary dentition produced occlusional anomalies. The presence of the second permanent molars together with the primary dentition and wide spacing in the lower incisor area were typical dental signs. Gradual extraction of the supernumerary teeth and over-retained primary teeth was the first step of oral surgery. This was followed by a surgical exposure of the unerupted teeth by thinning of the cortical bone. Orthodontic treatment was aimed at parallel growth of the jaws. Removable appliances were used to expand the narrow maxillary and mandibular arches, and a Delaire mask compensated for the lack of sagittal growth of the upper jaw. Temporary functional rehabilitation was solved by partial denture. When the jaws have been fully developed, implant insertions and bridges are the therapeutic measures. The reported case and the literature data support the importance of the early diagnosis and interdisciplinary treatment of CCD.
Our study showed that temporary absence of and changes in cardiovascular findings are frequent in Williams-Beuren syndrome. These results could contribute to the refinement of diagnostic criteria and recommendations for cardiovascular follow-up of patients with this syndrome.
A 24-year-old male with red tattoos developed ocular pain and blurred vision three years after having been tattooed. The patient also noticed redness and itching in the area of the tattooed skin (Fig. 1). The rest of the history and physical examination was unremarkable. Routine laboratory screening tests were within normal limits, with the exception of a mildly elevated white blood cell count (11,6 G/L). Chest X-ray examination and CT scan were within normal limits. Ophthalmological examination revealed panuveitis of the right eye with keratic precipitates, several firm posterior synechiae, infiltrated vitreous, and acute iridocyclitis of the left eye with ciliary congestion, flare and cells in the anterior chamber, and keratic precipitates (Figs. 2, 3). Histological examination of the lesion confirmed granulomatous reaction in the tattooed skin. Excision of the inflamed tattoo, administration of topical and systemic steroid (40 mg/day of prednisolone orally and 0,5% prednisolone acetate eye drops (2 drops twice daily) and cyclosporin (150 mg/day orally for 4 weeks) resulted in remarkable improvement in visual acuity and cure of the uveitis. All medications were discontinued, and the patient has been asymptomatic since. To our knowledge, the coexistence of uveitis and tattoo granulomas has only been reported once in the English language medical literature by Rorsman et al. [1]. According to their suggestion, tattoo granulomas and uveitis may be a special granulomatous hypersensitivity syndrome induced by cobalt [1]. As with their three cases, our patient also had no signs of systemic sarcoidosis and developed the ophthalmologic symptoms delayed after having had his skin tattooed.
Our data suggested that significant dose sparing is possible with the use of whole-virion vaccines and aluminium adjuvants, without compromising safety. This could have significant economic and public health impacts.
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