Genetic transmission of tumoral calcinosis: autosomal dominant with variable clinical expressivity.

Tumoral calcinosis is a rare inherited metabolic disorder characterized by hyperphosphatemia, elevated serum 1,25-dihydroxyvitamin D levels and periarticular cystic and solid calcifications. Based on previous investigations, the inheritance of this disorder has been postulated to be autosomal recessive. This interpretation was based on finding clinically affected subjects in only single generations of kindreds. We investigated four generations of an affected kindred and found nine subjects with the disease. A unique dental lesion which is specific for this disorder and serves as a phenotypic marker was identified in two generations of the kindred. In all affected subjects, elevated serum 1,25-dihydroxyvitamin D levels were found, although each member did not have the classical clinical findings of tumoral calcinosis. The possibility that this disorder may be variably expressed and have multiple formes frustes has not been previously considered. Using the unique dental lesion as well as the classical clinical and biochemical abnormalities, we found that in this kindred, tumoral calcinosis is transmitted in an autosomal dominant mode, with variable clinical expressivity.

The concurrence of hypoparathyroidism provides new insights to the pathophysiology of X-linked hypophosphatemic rickets.

Controversy exists over the role that PTH and extracellular fluid calcium concentration may play in modulation of the renal phosphate transport defect in X-linked hypophosphatemic rickets. In previous studies, administration of PTH to affected subjects resulted in an increase or no effect on renal phosphate excretion, while calcium infusion increased renal tubular phosphate transport. In contrast, patients with X-linked hypophosphatemic rickets and hyperparathyroidism have no change in their renal phosphate wasting after parathyroidectomy. However, none of these were permanently hypoparathyroid postoperatively. We describe a patient with idiopathic hypoparathyroidism in whom we proved the coexistence of X-linked hypophosphatemic rickets using family history and dental abnormalities. Initially, the patient had a mean serum calcium level of 5.6 +/- 0.07 (+/- SE) mg/dl and a renal tubular maximum for reabsorption of phosphate per liter glomerular filtrate (TmP/GFR) of 6.5 +/- 0.46 mg/dl. Hypoparathyroidism was confirmed, and therapy with vitamin D (50,000 U/day) and calcium (1,000 mg/day) was begun. On this regimen, serum calcium rose to 8.1 +/- 0.2 mg/dl, and TmP/GFR declined to 2.59 +/- 0.12 mg/dl. Bone biopsy revealed the persistence of osteomalacia. Subsequently, therapy with 1,25-dihydroxyvitamin D3 (1.0 microgram/day) was initiated, and serum calcium rose to 9.6 +/- 0.07 mg/dl, and TmP/GFR declined to 1.79 +/- 0.16 mg/dl. The prevailing serum calcium level correlated inversely with the TmP/GFR (r2 = 0.91; P less than 0.001). These data indicate that calcium and/or PTH are involved in modulation of the renal phosphate transport defect in X-linked hypophosphatemic rickets.

Gingival telangiectases: an underappreciated physical sign of juvenile dermatomyositis.

BACKGROUND: MEDLINE searches (1966-June 1969) failed to identify references that give detailed descriptions of the oral manifestations of dermatomyositis (DM). However, several reports predating MEDLINE provided more complete descriptions of oral lesions associated with DM. OBSERVATIONS: We describe 5 cases of juvenile DM with oral manifestations, primarily in the form of gingival telangiectases. These findings are compared with those descriptions found in earlier reports. CONCLUSIONS: Oral lesions in juvenile DM have rarely been reported. Mucous membrane involvement associated with DM may include telangiectases, edema, erosions, ulcers, and leukoplakia-like areas. In cases of DM, gingival telangiectases likely represent an underappreciated diagnostic finding analogous to nail-fold telangiectases.

Peripheral ameloblastoma.

Peripheral ameloblastomas are rare odontogenic tumors presenting as tumoral masses, erythematous plaques, or papillary lesions on the attached gingiva. This study reviews the demographic, clinical, and microscopic features of 8 previously unreported cases. One patient had two separate peripheral ameloblastomas, one 7 years after the first in the opposite side and arch. The cases match those described previously with respect to age, gender, location, and radiographic and microscopic features. Because recurrence has been reported and second primary sites may be found, periodic follow-up is recommended after conservative surgical removal.

Botryoid odontogenic cyst: analysis of 33 cases.

The botryoid odontogenic cyst is considered to be a multiloculated variant of the lateral periodontal cyst. Fewer than 40 of these lesions have been described, many of which have recurred. This paper describes the demographic, clinical, microscopic, and radiographic features of 33 unreported cases from the files of the Oral Pathology Biopsy Service at the University of North Carolina School of Dentistry. Our findings are in agreement with previously published cases for an average age of 57 years and the most common site for occurrence in the lower premolar area. Follow-up information on 12 patients determined that 2 had recurrences.

Periodontal osseous defects associated with vitally submerged roots.

1. Vital roots associated with periodontal osseous defects can be clinically submerged beneath a soft tissue flap. 2. Connective tissue fibers filled the submerged osseous defects and were oriented parallel to the root surfaces in the majority of cases. 3. New cementum lined the root surface at the base of two successfully submerged defects, but was not apparent on the dentin amputation sites of any submerged roots. 4. Eight of the nine clinically submerged defects exhibited positive radiographic changes. The six control defects showed no positive radiographic changes. 5. All successfully submerged defects showed histologic evidence of new bone formation. New bone formation was not apparent in any of the control defects or nonsubmerged defects. 6. Either no epithelium or only a narrow band of epithelium was associated with the successfully submerged roots. A connective tissue inflammatory infiltrate was not present within the submerged defects. 7. The defects of all control teeth and nonsubmerged experimental roots contained epithelial downgrowth accompanied by a chronic inflammatory response in the connective tissue. 8. Pulpal tissues associated with submerged roots appeared vital without significant degenerative or inflammatory changes. Pulpal tissue was continuous with overlying connective tissue.

Blood-flow mapping of oral tissues by laser Doppler flowmetry.

The purpose of this study was to develop a rapid protocol for noninvasive quantification of blood flow at intraoral sites by laser Doppler flowmetry. For each flow measurement, the lowest flow observed over a 30-s period was recorded. Three flow readings at each site were averaged and recorded. Forehead and dorsal right-hand blood flows measured agreed with previous laser Doppler flow measurements. Flows measured with a hand-held probe were not different from flows measured with a stent-stabilized probe on the attached gingiva and an adhesive-stabilized probe on the dorsal hand. High flows were found in the tongue, buccal mucosa, and buccal vestibule. Medium flows were found in the attached gingivae. The lowest flows were found in the teeth. These results indicate that flow in intraoral tissues varies by site and can be noninvasively quantified with the laser Doppler flowmeter when a rigorous measurement protocol is used.

Radiographic manifestation of clear cell odontogenic tumor.

Clear cell odontogenic tumors are uncommon, only 20 cases having been reported in the literature. We report a case that presented with unique radiographic manifestations over a period of 12 years; no similar case has been reported to date. Documentation of the malignant nature of the condition and of the range of clinical and radiographic manifestations with which this neoplasm can present provides useful insight into its pathogenesis and progression. Early and timely recognition of the lesion, histopathologic examination, and aggressive interventional procedures are in order to successfully treat this condition and prevent a potentially fatal outcome.

Laser Doppler flowmetry: a clinical test of pulpal vitality.

PROBLEM: A rapid, accurate, noninvasive method of determining pulpal blood flow would be helpful in determining pulpal vitality. OBJECTIVE: The purpose of this study was to determine if laser Doppler flowmetry can measure induced changes in pulpal blood flow. STUDY DESIGN: Two percent lidocaine with epinephrine 1:100,000 was infiltrated into the labial vestibule to anesthetize five anterior teeth in healthy human volunteers. Stents were placed on the teeth to stabilize the laser Doppler probes. Measurements of pulpal blood flow were made along with electrocardiograms to record the cardiac cycle. RESULTS: Laser Doppler flowmetry demonstrated pulpal blood flow and pulse amplitude decreases under test conditions. These decreases were most significant at 10 minutes after the injection of anesthetic with vasoconstrictor. CONCLUSIONS: Laser Doppler flowmetry was able to measure pulpal blood flow and record changes in blood flow that occurred when epinephrine was used with infiltration anesthesia. The pulse width and the mean flow were dramatically affected as was synchronization with the cardiac cycle.

Pulpal response to cavity preparation with the Er:YAG and Mark III free electron lasers.

OBJECTIVE: The purpose of this investigation was to evaluate the pulp response to class V cavity preparation with the use of the Er:YAG laser and free electron laser. STUDY DESIGN: Class V cavities were prepared in 133 teeth of four beagle dogs by one of three methods: (1) Er:YAG laser, (2) free electron laser, (3) high-speed handpiece. Treatment occurred at 1 hour, 1 day, 7 days, and 28 days. The teeth were removed and the pulp evaluated. Histologically, the data were evaluated with Mantel-Haenszel analysis. RESULTS: The Er:YAG laser, free electron laser, and handpiece treatment groups resulted in specimens with normal or mild pulp reactions in 36, 46, and 42 teeth, respectively; moderate or severe reactions were observed in 7, 1, and 1 teeth, respectively. No statistically significant difference in the pulp response to the three treatment modalities was observed. CONCLUSION: The pulp response to Er:YAG laser and free electron laser application would appear to be similar to the response from high-speed handpiece application.

A peripheral giant-cell granuloma manifestation of primary hyperparathyroidism: report of case.

Profuse granulomatous masses of the gingiva frequently occur in patients with gingival and periodontal disease. A 33-year-old female was seen for followup of a previously treated peripheral giant-cell granuloma. Two years earlier, a mass had been removed from the gingiva between the maxillary right central and lateral incisors. Current laboratory test results indicated hyperparathyroidism, and led to the discovery of a parathyroid adenoma. After the gingival lesion, adjacent teeth, and parathyroid adenoma were removed, the patient had no further recurrence of the lesion or signs of hyperparathyroidism.

Lingual sebaceous glands: report of case.

A 28-year-old dentist noticed a 9X5-mm yellow submucosal plaque in the midlateral dorsum of his tongue. After a period of observation an excisional biopsy was performed. The histopathologic examination of the specimen revealed normal sebaceous glands opening onto the tongue surface. This occurrence of ectopic sebaceous glands in the tongue is notably rare in contrast with their frequent occurrence in the lips and buccal mucosa.

Erbium:YAG (2.94 mum) laser effects on dental tissues.

Past trials with soft and calcified tissues have demonstrated that long pulse train (2.5 mus) Er:YAG (2.94 mum) laser may be used to ablate tooth structure of human teeth. Determination of physical and thermal damage to surrounding tissue during removal of enamel and dentin is a primary objective of this study. Extracted human teeth with thermal probes imbedded in the pulp chambers were submitted to cavity preparation using an Erbium YAG laser with water mist. Wavelength selection as well as use of a water mist during the procedure resulted in efficient tissue removal without significant surrounding damage. Ground sections and SEM sections of teeth showed little or no melting or ash formation in adjacent dentin and enamel and no visible change in the pulp chamber. The surfaces produced by laser ablation were rough and irregular with craters and grooves. Average temperature change in the pulp chamber monitored during tooth preparation was 2.2 degrees Centigrade. These findings suggest that constantly available water aids vaporization and microexplosions, increasing the efficiency of tooth structure removal, and aids in cooling of the tooth structures. The long pulse Er:YAG (2.94 mum) laser may be an effective method for tooth reduction applications when used with a water mist.

Meeting the educational needs of patients with oral lichen planus.

Lichen planus is a chronic oral disorder that is often painful and annoying. The patients have been described as usually over 50 years old, with a high educational level, anxious and high-strung. In previous research studies, these patients have reported unusual and highly stressful life events. Because dentists are in a position to advise their patients about chronic illness, this study was designed to (1) determine what information was given to lichen planus patients by their dentists; (2) determine what questions the patients asked their dentists; and (3) assess what educational materials would be helpful for this group of patients. A survey was sent to 151 biopsy-confirmed lichen planus patients, with a response rate of 55 percent. The results indicated that the patients were concerned about the possibility of malignancy and of contagion, and that they were frustrated by the lack of available patient education.

Nifedipine-induced gingival hyperplasia.

Gingial hyperplasia is a complication which may be seen in a significant number of patients taking nifedipine (Procardia, Pfizer) a commonly used medication for cardiac vessel dilitation. This is a report of gingival changes in a 43-year-old hypertensive male who began using the calcium channel blocking agent during his active dental treatment. Gingival hyperplasia developed which was severe enough to alter the course of his dental treatment and prompt his changing to other medications to control his blood pressure. The recognition that the drug was responsible for the hyperplasia led to prompt and effective methods to resolve this problem.

Temporary silastic implantation following discectomy in the primate temporomandibular joint.

Silastic (Dow Corning, Midland, MI) has been used in the surgical treatment of a variety of temporomandibular joint (TMJ) disorders as either a permanent or temporary implant. In this study four Macaca fascicularis monkeys underwent bilateral TMJ discectomies and high condylar shaves. The experimental side was treated with a Silastic sheet implant which was removed at 3 months postdiscectomy. The contralateral side was left without an implant and served as a control. Animals were killed at 3, 4, 5, and 6 months postoperatively. A fibrous connective tissue capsule was found around the Silastic implant at all time intervals. This capsule formed a single joint space between the inferior surface of the glenoid fossa and articulating surface of the condyle. Three months following implant removal, the capsule was still present and there was no significant inflammatory cell infiltrate. The severity of the degenerative changes was decreased on the implant side.