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Biomedical subjects

E Mass

Publications and source records attributed to E Mass.

At least 19 recordsLinked to original sources
PubMed2005

Case report: multiple endocrine neoplasia type 2B misdiagnosed as familial dysautonomia.

BACKGROUND: Familial dysautonomia (FD) is a rare autosomal recessive disorder of the peripheral nervous system, affecting exclusively Jewish children of Ashkenazi extraction. The typical clinical features consist of somatic abnormalities: failure to thrive, characteristic facies, excessive sweating, labile blood pressure, recurrent aspiration pneumonias, lack of tears, and diminished and later absent deep tendon reflexes with generalized reduction of pain sensation. Oro-dental features include a lack of tongue fungiform papillae, impairment of taste, oro-dental self-mutilation, dental crowding, excessive plaque and calculus accumulation, salivary over production and low caries experience. CASE REPORT: A child with multiple endocrine neoplasia type 2B (MEN 2B) received, at the age of 11 months, an incorrect diagnosis of familial dysautonomia (FD). At the age of 6 years, a paediatric dentist experienced with FD noticed a normal number and shape of tongue fungiform papillae, while expecting to find a smooth tongue lacking those structures. The presence of numerous submucosal neuromata initiated a meticulous neurological and endocrine work-up, which established the diagnosis of MEN 2B. This led to an early detection and appropriate treatment of asymptomatic medullary thyroid carcinoma (MTC).

Adolescent↗
PubMed2005

Treatment policies among Israeli specialists in paediatric dentistry.

AIM: This was to evaluate some suggested diagnostic procedures, treatment policies and professional attitudes of specialists in paediatric dentistry, in light of the periodically published guidelines by The American Academy of Pediatric Dentistry, The European Academy of Paediatric Dentistry and The British Society of Paediatric Dentistry. METHODS: Using a structured questionnaire, 67% of the Israeli specialists in paediatric dentistry, who agreed to participate in this study, were personally interviewed. RESULTS: Only 7.5% of the participants reported that they carry out pulp capping of primary teeth in cases of pulp exposure. Over 50% reported restoring teeth after pulpotomy with preformed crowns. Most indicated sealing pit and fissures after considering depth and morphology of the fissures and correlation with the patient's risk to caries. Cleaning teeth after eruption of the first tooth was suggested by 75.5% of the participants. A striking majority (96%) claimed that they restored permanent anterior teeth with composite resins and most used these materials for occlusal restoration in both primary and permanent posterior teeth. Most specialists advocated the use of amalgam in proximal posterior restorations. The presence of a parent in the operatory/surgery was preferred by 85% of the dentists. CONCLUSIONS: Israeli specialists in paediatric dentistry mostly comply with the mentioned guidelines. Further studies of this nature should also be encouraged in other countries to emphasize the importance of monitoring compliance with established and evidence based guidelines.

Adult↗
PubMed2001

Calcium hydroxide retention in wide root canals with flaring apices.

Calcium hydroxide (CH) is used to induce apexification in immature, pulpless teeth with wide root canals and flaring apices. Three placement methods of CH were compared in vitro, for their ability to enhance CH retention in the canal. Barium sulfate (BS), added as radiopacifier, was studied for its effects on the radiographic follow up of CH retention, as well as on the ability to detect voids in the CH. Calcium hydroxide filled teeth were kept in phosphate buffered saline-containing tubes in a shaker water bath. Radiographs were taken weekly to follow the retention or loss of the material; they were scanned and stored as digitized images. Quantitative evaluations were done on these images, using a computerized gray scale. Condensed CH was better retained in root canals than either lentulo-placed paste or commercial injected paste. Barium sulfate in the CH paste enabled better detection of voids in the paste filling the canal, however, it obscured the disappearance of CH from the canal, due to a residual radiopacity effect. The results supported (a) condensation of calcium hydroxide as a preferred method that improves its retention in wide root canals, and (b) discontinuing the use of barium sulfate as a radiopacifier in apexification procedures.

Analysis of Variance↗
PubMed1999

The effect of composite resin application and radiation techniques on dye penetration in Class II direct composite resin restorations.

This study examined the effect of the application of resin composite and radiation techniques on dye penetration in class II direct composite resin restorations. For the restorative procedure, the light-cone and the incremental layering techniques were used. The null hypothesis of this experiment was, that both techniques have the same influence on dye penetration. Evaluation of dye penetration in the restoration/tooth interface was performed in 20 extracted premolars and molars. On each tooth, two class II cavities were prepared. The cervical margin of the proximal boxes were located in dentin or in enamel. When the gingival margin of the cavity was located in enamel, a significant lower level of dye penetration of the the test group was found (Wilcoxon rank test, P = 0.0102). This study showed that the application of a transparent cone seems to be more effective than the three increments technique in the mechanical separation of the first composite increment. Transmitting the curing light into the proximal box, seems to have no positive effect on dye penetration in class II direct composite resin restorations.

Bicuspid↗
PubMed1998

A cephalometric evaluation of craniofacial morphology in familial dysautonomia.

OBJECTIVE: The purpose of this study was to delineate the craniofacial and dentoalveolar morphology of patients with familial dysautonomia (FD) in order to contribute to the understanding of the association between progressive sensory and autonomic neuropathy and the characteristic appearance of the dysautonomic face. PATIENTS: The study group comprised 32 patients with FD (15 females and 17 males; mean age 10.8 years, SD 3.5 years, range 5.8-19.8 years). DESIGN: Lateral cephalograms from each patient were traced twice. The means of the two measurements were compared with homologous cephalometric normal values of ethnic-specific and classical norms from the literature. RESULTS: In some parameters, the craniofacial morphology of the FD group was significantly different from the classical norms. There was a pronounced retrognathism in the mandible and a steep mandibular plane angle. The skeletal features of FD patients more closely resembled those of their ethnic group, although they were more retrognathic, and the mandibular growth axis was more horizontal. The incisors of these patients were more retropositioned and retroclined than were those of their healthy counterparts. CONCLUSIONS: The results suggest an insufficiency of the expected dentoalveolar compensatory mechanism that usually helps to bridge skeletal discrepancies. It is postulated that the neuropathy is probably the important factor in the lack of this compensatory mechanism.

Adult↗
PubMed1997

Taste and smell in familial dysautonomia.

Familial dysautonomia (FD) is one of the classic diseases characterised by taste and smell abnormalities. However, these typical features are based on data obtained from two separate crude studies published in 1964. In the present study psychophysical-cognitive and reflex-like facial-behavioral responses to taste and smell, in nine patients with FD and 15 healthy controls, were recorded. Five taste stimulants were presented to both study groups, while a selection of common household odors was used for FD patients only. The patients with FD showed a markedly higher incidence of recognition failures for salty, bitter, sweet, and water stimuli than the controls, but rate of recognition of sour stimuli was almost identical in the two groups. Estimates by the subjects on a hedonic scale of 0 to 10 and facial display in FD indicated a relatively normal sensitivity to sour stimuli and to a lesser extent to bitter stimuli. Water, sweet, and salty stimuli evoked non-discriminatory responses. These findings indicate specific dyageusia rather than general ageusia. Smell was found to be normal. In children with taste and smell impairment, a systematic evaluative approach may help in planning palatable diets for adequate and comfortable nutrition.

Adolescent↗
PubMed1996

Increased major salivary gland secretion in familial dysautonomia.

Familial dysautonomia (FD), an autosomal recessive peripheral nervous system disorder, affects almost exclusively children of Jewish Ashkenazi origin and causes profound generalized autonomic dysfunction. Excessive drooling is frequent and is traditionally attributed to swallowing difficulties. Although true hypersalivation has been postulated, no quantitative assessment of the salivary secretion rate has yet been reported. The authors determined this rate in 13 children with FD and 28 healthy controls. Resting parotid, submandibular/sublingual and unstimulated whole salivary secretion rates were significantly elevated in children with FD. The known relation of salivary function with age was found in controls only. This apparently major contribution of salivary hyperfunction to excessive drooling in FD may be attributable to salivary gland denervation supersensitivity, as this mechanism is present in the cardiovascular system and the pupil in FD.

Adolescent↗
PubMed1996

Abnormal enamel and pulp dimensions in familial dysautonomia.

In familial dysautonomia (FD), a hereditary autonomic and sensory neuropathy, somatic growth is impaired. This study was conducted to explore the possibility that tooth dimensions are altered as a consequence of neural crest dysfunction known to be present in FD. Enamel, dentin, pulp, and tooth size measurements of mandibular primary and permanent molars from FD patients were compared with those of healthy controls. It was found that although tooth size in the FD patients was smaller than normal, the enamel was thicker on the occlusal table, while the pulp chamber was smaller and disproportional to tooth size. Our results suggest distorted tooth dimensions rather than a generalized growth arrest as observed in other hereditary syndromes, such as Down's or Crouzon.

Adolescent↗
PubMed1995

A clinical and histopathological study of radicular cysts associated with primary molars.

Radicular cysts originating from primary teeth are considered rare. The study analyzed 49 primary molars with radiolucent lesions ranging from 4-15 mm in diameter. 73.5% of the lesions were diagnosed as radicular cysts, and 26.5% as granulomas. The lesions were more frequent in the mandible. All lesions were associated with severely decayed teeth and only 4 had previous pulp therapy. The present study and the survey of the literature suggest that radicular cysts associated with primary teeth are not rare.

Child↗
PubMed1995

Diseases with ocular and dental abnormalities.

This report gives an overall view on the aspect of numerous diseases and symptom complexes affecting both eyes and teeth. The knowledge of these disorders enables the ophthalmologist to suspect and recognize certain types of dental malformation and enables the dentist to discover certain eye defects. Moreover, it is important that clinicians be aware that simultaneous eye and tooth defects may be the indication of possible disturbances in other, apparently unrelated, organs or systems. Because such disorders are frequently hereditary conditions, their early detection will aid in the diagnosis and treatment of other affected family members. This may also help the parents to cope with the disease and be of benefit to the infants because a cure may be forthcoming.

Eye Abnormalities↗
PubMed1994

Periodontal ligament injection in the dog primary dentition: spread of local anaesthetic solution.

The spread of local anaesthetic solution administered with a pressure syringe has not been studied as extensively in young animals having primary or mixed dentitions as in adult animals. The purpose of this investigation was to study the distribution of local anaesthetic solution injected into the periodontal ligament of young dogs. India ink was added to carpules containing 2% lidocaine and 1:100,000 epinephrine, and the spread of solution was examined macroscopically and microscopically. Injections were made with a pressure syringe (Ligmaject) at 58 sites mesial and distal to primary teeth in five dogs aged 3-9 months. Three dogs were killed within 12 hours, the other two after 5 days. Spread of the ink was studied in non-decalcified slabs, in three-dimensional cleared specimens, and in histological sections. The solution usually reached the alveolar bone crest, seeped under the periosteum and alongside vascular channels into bone marrow, reaching natural cavities such as the crypts of tooth buds and the mandibular canal. The ink did not penetrate into the enamel organ or contact the permanent tooth buds. The solution appeared to spread along the path of least resistance, governed by the intricacies of anatomical structures and fascial planes. Therefore the risk of mechanical damage to permanent tooth germs appears to be minimal.

Anesthesia, Dental↗
PubMed1994

Oro-dental self-mutilation in familial dysautonomia.

Orodental self-mutilation (ODSM) has not gained sufficient recognition in familial dysautonomia (FD). Among 38 patients with FD, ODSM was found in 14 (36.8%). ODSM may be due to peripheral neuropathy with insensibility to pain, which is characteristic of FD. Elimination of the sharp edges of teeth was found to be helpful.

Adolescent↗
PubMed1993

Craniofacial morphology of children with Williams syndrome.

Facial dysmorphology is considered to be a major diagnostic feature in patients with Williams syndrome (WS). The dysmorphology is composed of soft tissue and skeletal components. In this study the skeletal component of the facial dysmorphology was examined by analyzing the lateral cephalometric radiographs of 8 children with WS. Seven anatomic areas were judged by means of the analysis of 48 cephalometric variables. Four skeletal features contributed to the facial appearance of children with WS: (1) the anterior cranial base was short, although the cranial base angle (N-S-Ba) was normal; (2) the angle of the mandibular plane was steep, although total facial height was normal; (3) despite the normal facial height, there was an unusual proportion of upper to lower anterior facial height and posterior to anterior facial height; and (4) the chin button (pogonion to N-B line) was deficient, although the mandible could not be classified as retrognathic. The common skeletal components were not dominant enough to characterize the facial dysmorphology completely.

Aortic Valve Stenosis↗
PubMed1992

Dental and oral findings in patients with familial dysautonomia.

Familial dysautonomia is an inherited autosomal recessive disease found almost exclusively in Ashkenazi Jews. It is characterized by selective damage to the sensory, motor, and autonomic peripheral nervous system. The main clinical features include decreased pain sensation, impaired temperature and blood pressure regulation, lack of tearing, absent tendon reflexes, and fungiform papillae on the tongue. The purpose of this study was to explore in depth and to verify the oral and dental status in familial dysautonomia. Twenty-two patients and 44 match-paired healthy persons of Ashkenazi descent were examined. Patients and parents had only little concern for their oral condition. Caries prevalence was lower than normal and plaque accumulation increased in all patients. Dental trauma was found in 59% of the patients, and 32% showed orodental self-mutilation. Dental age was within normal range, and dental arch measurements implied proportionally small jaws and little crowding. The low caries rate may be related to the known "hypersalivation" in familial dysautonomia and/or a possible change in the salivary composition and content, caused by chronic autonomic denervation.

Adolescent↗
PubMed1978

Talon cusp in deciduous dentition.

Talon cusp is a rare dental anomaly hitherto considered to affect permanent dentition only. A case of talon cusp in a deciduous maxillary incisor is described, suggesting that both dentitions may be affected by this anomaly.

Humans↗