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B G Russell

Publications and source records attributed to B G Russell.

At least 19 recordsLinked to original sources

Cranial and hand skeleton in fragile X syndrome.

The purpose of the present study was to track prenatally observed skeletal deviations in radiographs from fragile X syndrome children and young adults in a search for improvement of early diagnostics of fragile X syndrome. The material consisted of craniofacial profile radiographs and hand radiographs from six males age between 2 years 9 months and 20 years 3 months. Craniofacial radiographs showed normal morphology of the nasal bone in all cases. In five cases the sella turcica could be analyzed. In two cases the anterior wall of the sella was oblique, and in two cases the dorsum sellae appeared short. In one case the sella turcica had normal structure. In two cases, the cervical column was suitable for examination. In one, body fusion and short arcus occurred. Of the six hand radiographs analyzed, the hand skeleton appeared normal in the youngest male. In the other five cases there was a deviant location of the carpal bones in the developmental field corresponding to the first finger. Skeletal maturity was delayed in all cases. In conclusion, the prenatally registered morphological deviations in the skeletal development of fragile X syndrome fetuses were found in 5 of 6 fragile X males and young adults. We suggest that a skeletal analysis be considered in the phenotypic classification of children with fragile X syndrome.

Adolescent↗

Face, palate, and craniofacial morphology in patients with a solitary median maxillary central incisor.

The occurrence of a solitary median maxillary central incisor (SMMCI) is a very rare condition and might be a sign of a mild degree of holoprosencephaly. In this investigation, material from 10 patients, nine girls and one boy with a SMMCI (8-17 years of age) registered in orthodontic clinics was examined. The purpose was to evaluate the clinical characteristics and craniofacial morphology in this group of patients. Oral photographs, study casts, profile radiographs, and orthopantomograms were analysed. The study showed that this group of SMMCI patients were characterized by an indistinct philtrum, an arch-shaped upper lip, absence of the fraenulum of the upper lip, a complete or incomplete mid-palatal ridge, a SMMCI, and nasal obstruction or septum deviation. The craniofacial morphology of the nine girls, compared with normal standards for girls showed a short anterior cranial base, a short, retrognathic and posteriorly inclined maxilla, and a retrognathic and posteriorly inclined mandible. Furthermore, the sella turcica had a deviant morphology in five of the 10 subjects. The results indicate that the presence of a SMMCI should not be considered as a simple dental anomaly, since it may be associated with other clinical characteristics and more complex craniofacial malformations. It is therefore suggested that the SMMCI condition in future studies is classified according to clinical symptoms and craniofacial morphology.

Adolescent↗

Postnatal structure of the sella turcica in Down syndrome.

The purpose of this study was to analyze the shape of the sella turcica in a group of patients with Down syndrome and compare the findings with those made earlier in human fetuses with Down syndrome. Profile radiographs from 78 patients (age 4 months to 50 3/12 years) were analyzed. A tracing was made of each sella turcica, and the shape was compared with that of a normal sella, including the normal growth pattern from childhood to adulthood. Sella turcica structure could be classified into three morphological types, defined as: type I, almost normal appearance; type II, deviations in the anterior wall; and type III, deviations in the floor of the sella turcica. Compared with previously registered prenatal structural deviations in the sella turcica, it can be concluded that the postnatal radiographic material reflects the prenatal findings, because type I, both prenatally and postnatally, is by far the most common, whereas the remaining types are uncommon, both prenatally and postnatally. The study confirms the relevance of prenatal investigations for postnatal diagnostics as previously documented in sella turcica analyses of prenatal and postnatal myelomeningocele cases.

Adolescent↗

Surface structure of human mucin using X-ray photoelectron spectroscopy.

X-ray photoelectron spectroscopy (XPS) is a surface sensitive analytical technique that measures the binding energy of electrons in atoms and molecules on the surface of a material. XPS was used to determine the distribution of the oligosaccharide side chains in the glycoprotein, MUC1 mucin. Low-resolution XPS spectra provided elemental composition of MUC1 mucin (fully glycosylated), mucin polypeptide (nonglycosylated), and carbohydrates found in mucin. The nitrogen content of MUC1 mucin was determined to be intermediate between the mucin polypeptide and the carbohydrates. Assuming a uniform distribution of carbohydrate on MUC1 mucin, the average thickness of the carbohydrate layer was calculated to be 4.9 nm using the low-resolution N 1s signals. High-resolution XPS spectra give detailed information about the chemical bonding of the surface molecules. Calculations based on the high-resolution O 1s spectra showed a carbohydrate thickness of 6.6 nm. These experimentally determined values agree reasonably well with an estimated 5 nm of carbohydrate thickness from a simple model which assume that the core protein is a rodlike molecule approximately 5 nm in diameter. Although the carbohydrate coating on the MUC1 mucin appears to be thick enough to cover the core protein entirely, fully glycosylated breast milk MUC1 mucin is susceptible to proteolytic digestion without removal of any oligosaccharide side chain, suggesting areas of exposed core protein. A possible explanation is that the oligosaccharide side chains may form patches of carbohydrate along the core protein with regions of exposed core protein.

Amino Acid Sequence↗

Deciduous teeth in tuberous sclerosis.

Shed deciduous teeth from patients with tuberous sclerosis, cerebral palsy, Down syndrome, phenylketonuria and healthy persons were examined with a surface microscope. We found enamel pits in all 87 deciduous teeth from the 20 patients with tuberous sclerosis, but in none of the 253 deciduous teeth from 142 controls constituting patients with cerebral palsy, phenylketonuria and Down syndrome as well as healthy persons. Enamel pits always occurred in the facial surface of the central incisor, lateral incisor and canine, while the number of enamel pits in the other surfaces of the deciduous teeth varied from none to nine. Ground sections examined microscopically revealed an undisturbed pattern of incremental lines (Retzius striae) surrounding the pits. In five dental sacs from patients with tuberous sclerosis, microscopic examination showed that the inner surface of the operculum was remarkably more irregular than in control patients.

Adolescent↗

Ocular findings and sphenoid bone morphology in tuberous sclerosis.

The aim of the study was to focus on the correlation between the ocular findings and the morphology of the sphenoid bone supporting the optic nerve in patients with tuberous sclerosis. Ten patients participated in the investigation. The sphenoid bone morphology was analyzed from existing radiographs of the cranium. The results were compared to the individual ophthalmologic diagnosis. Changes in the midline osseous structure, the sella turcica and optic sulcus seem to be correlated with the severity of the ocular findings.

Adolescent↗

Tooth agenesis in Down syndrome.

We studied the frequency and pattern of tooth agenesis in a Danish population with Down syndrome, trisomy 21 (46 females and 54 males). The control group consisted of a normal Danish population (2424 females and 2431 males) [Rølling, 1980: Scand J Dent Res 88:365-369; Ravn and Nielsen, 1973: Tandlaaegebladet 77:12-22]. We found that individuals with Down syndrome have an occurrence of agenesis that is some 10 times greater that in the general population with a higher frequency in males than in females. Agenesis occurred more frequently in the mandible than in the maxilla and most often on the left side. The highly significant differences were primarily found in the occurrence of agenesis of the mandibular central incisors, followed by the maxillary lateral incisors and second premolars and the mandibular second premolars. The main components in the pattern of agenesis observed in Down syndrome are supposed to be related to the peripheral nervous system and abnormal cartilagenous tissue. The present study on Down syndrome suggests that the dentition, with its many different anomalies, from agenesis to malformation, can be used as an indicator in evaluating different aspects in the patheogenetic of aneuploidy conditions.

Adolescent↗

Oral use of chlorhexidine gluconate toothpaste in epileptic children.

The purpose of this clinical study was to evaluate the effect of a 3-min daily toothbrushing with 1% chlorhexidine gluconate toothpaste on dental plaque, gingivitis and gingival hyperplasia in 30 mentally retarded epileptic children treated with phenytoin. A double-blind cross-over technique was used. Brushing once daily for 2 months with 1% chlorhexidine gluconate toothpaste resulted in significantly lower Pl I and G I values. Gingival hyperplasia was not significantly reduced by the use of the chlorhexidine toothpaste.

Administration, Oral↗

Effect of chlorhexidine on dental plaque and gingivitis in mentally retarded children.

The purpose of the investigation was to determine the effect of chlorhexidine mouthrinses as a supplement to toothbrushing in the plaque control of mentally retarded children. Fifty-four mentally subnormal children 7-14 years of age took part in the investigation. During the first period, half of the children rinsed their mouths with 0.2% chlorhexidine gluconate solution twice a day. The rest of the children used a placebo mouthrinse. After a n 8-week interval a "cross-over" experiment was carried out during which a 0.1% chlorhexidine solution was used. Plaque and gingival indices were scored at the beginning of each period, after 3 weeks, and at the termination after 6 weeks. The results revealed that mouthrinses with 0.1% as well as 0.2% chlorhexidine gluconate reduced plaque and gingivitis in mentally retarded children. The results were statistically significant (P is less than 0.05) for both concentrations. Furthermore, the investigation confirmed the occurrence of certain side effects such as discoloration of teeth and tongues.

Adolescent↗

[Oral vaccinia].

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Child↗