Family-centred care and health-related quality of life of patients in paediatric neurosciences.

BACKGROUND: Little is known about the influence of contextual factors such as health services characteristics on health-related quality of life (HRQL) for children with a neurological condition. To address this gap, we conducted an exploratory study of the relationship between family-centred care (FCC) and HRQL outcomes in children from neurosciences clinics in a large acute care hospital. METHODS: A total of 187 family caregivers completed questionnaires regarding their socio-demographic status, the severity of their children's condition (FIM), perceptions of their children's HRQL (PedsQL 4.0) and their experiences of FCC (MPOC-20). Hierarchical regression analyses explored the hypothesis that FCC is a significant predictor of children's HRQL, independent of illness severity. RESULTS: Illness severity and FCC jointly explained one-third of the variance in children's total HRQL. When FCC was controlled for illness severity, it remained a significant predictor of physical, psychosocial and total HRQL scores. CONCLUSIONS: This study provides evidence that the level of FCC is positively related to paediatric HRQL independent of neurological illness severity. The implication is that the uptake of FCC practices by service providers can positively impact the quality of life of children with neurological disorders.

Ru2(ap)4(sigma-oligo(phenyleneethynyl)) molecular wires: synthesis and electronic characterization.

Reported in this contribution are the synthesis, characterization, and charge transport properties of wire-like Ru2(ap)4(OPEn), where ap is 2-anilinopyridinate and OPE is -(CCC6H4)nSCH2CH2SiMe3 with n = 1 (1) and 2 (2). Scanning tunneling microscopy (STM) measurements of compound 2 inserted into a SAM of C11 thiol reveal that molecule 2 exhibits (i) the stochastic switching characteristic of wire molecules embedded in insulating SAMs and (ii) higher conductivity than the C11 thiol SAM. More importantly, analysis of the molecular electronic decay constant (beta) exhibits a decrease of at least 15% as compared to purely organic molecular analogues. Hence, the transport characteristics of molecules can be significantly improved for nanoscale electronics through the incorporation of a Ru2 fragment into conjugated backbone.

Metal-molecule contacts and charge transport across monomolecular layers: measurement and theory.

Charge transport studies across molecular length scales under symmetric and asymmetric metal-molecule contact conditions using a simple crossed-wire tunnel junction technique are presented. It is demonstrated that oligo(phenylene ethynylene), a conjugated organic molecule, acts like a molecular wire under symmetric contact conditions, but exhibits characteristics of a molecular diode when the connections are asymmetric. To understand this behavior, we have calculated current-voltage (I-V) characteristics using extended Huckel theory coupled with a Green's function approach. The experimentally observed I-V characteristics are in excellent qualitative agreement with the theory.

Mid- and far-infrared spectroscopic studies of the influence of temperature, ultraviolet photolysis and ion irradiation on cosmic-type ices.

Infrared (IR) studies of laboratory ices can provide information on the evolution of cosmic-type ices as a function of different simulated space environments involving thermal, ultraviolet (UV), or ion processing. Laboratory radiation experiments can lead to the formation of complex organic molecules. However, because of our lack of knowledge about UV photon and ion fluxes, and exposure lifetimes, it is not certain how well our simulations represent space conditions. Appropriate laboratory experiments are also limited by the absence of knowledge about the composition, density, and temperature of ices in different regions of space. Our current understanding of expected doses due to UV photons and cosmic rays is summarized here, along with an inventory of condensed-phase molecules identified on outer solar system surfaces, comets and interstellar grains. Far-IR spectra of thermally cycled H2O are discussed since these results reflect the dramatic difference between the amorphous and crystalline phases of H2O ice, the most dominant condensed-phase molecule in cosmic ices. A comparison of mid-IR spectra of products in proton-irradiated and UV-photolyzed ices shows that few differences are observed for these two forms of processing for the simple binary mixtures studied to date. IR identification of radiation products and experiments to determine production rates of new molecules in ices during processing are discussed. A new technique for measuring intrinsic IR band strengths of several unstable molecules is presented. An example of our laboratory results applied to Europa observations is included.

Late presentation of upper airway obstruction in Pierre Robin sequence.

A retrospective review was carried out of 11 consecutive patients with the Pierre Robin sequence referred to a tertiary paediatric referral centre over a five year period from 1993 to 1998. Ten patients were diagnosed with significant upper airway obstruction; seven of these presented late at between 24 and 51 days of age. Failure to thrive occurred in six of these seven infants at the time of presentation, and was a strong indicator of the severity of upper airway obstruction. Growth normalised on treatment of the upper airway obstruction with nasopharyngeal tube placement. All children had been reviewed by either an experienced general paediatrician or a neonatologist in the first week of life, suggesting that clinical signs alone are insufficient to alert the physician to the degree of upper airway obstruction or that obstruction developed gradually after discharge home. The use of polysomnography greatly improved the diagnostic accuracy in assessing the severity of upper airway obstruction and monitoring the response to treatment. This report highlights the prevalence of late presentation of upper airway obstruction in the Pierre Robin sequence and emphasises the need for close prospective respiratory monitoring in this condition. Objective measures such as polysomnography should be used, as clinical signs alone may be an inadequate guide to the degree of upper airway obstruction.

CT-determined intracranial volume for a normal population.

Intracranial volume comparisons of patients with craniosynostosis and normal have been contrary to expectations, leading to questioning of the validity of the current normal reference material. Computed tomography-determined intracranial volume is presented for a white normal population. Specifically, intracranial volumes for 157 subjects (82 female and 75 male) were measured from computed tomography data using the Cavalieri estimator: volume determination was based on measuring the area in each computed tomography section. Monomolecular and Gompertz models were applied to find curves of best fit to the intracranial volume as a function of the age. The best fit was obtained using the monomolecular model when the response variable was the logarithmically transformed intracranial volume, and the independent variable was the logarithm of the age from conception. For example, the mean (standard deviation) for male subjects at 1 year and 20 years were 1,125.6 (89.6) ml and 1,472.9 (117.2) ml, respectively, and for female subjects 1,024.9 (84.0) ml and 1,321.7 (108.3) ml, respectively. Although the shape and rate of increase of the female and male curves is similar, the female mean is 1.3 standard deviations below the male mean at 20 years. These curves were compared with the commonly referenced curves of Blinkov (1941), Lichtenberg (1960), and Dekaban (1977). Our male curve is substantially higher than these curves in the age range 8 months to 4 years. Our female curve, however, is approximately 1 standard deviation below Lichtenberg's curve from birth to 7 months. There are then only minor differences between our female curve and Lichtenberg's curve until his curve crosses ours at 41 months, where they significantly diverge from approximately 4.5 years. Our curves indicate that 95% of the final intracranial volume has been attained by 42 months for girls and 46 months for boys.

Cerebral cortical dysplasia and digital constriction rings in Adams-Oliver syndrome.

Adams-Oliver syndrome (AOS) is characterised by aplasia cutis congenita of the scalp and variable degrees of terminal transverse limb defects. Short fingers and hypoplastic nails also occur in this predominantly autosomal dominant syndrome which displays marked variability of expression and lack of penetrance in some cases. We describe a boy with AOS whose sister is also mildly affected. Their mother has hypoplastic fifth toenails which may represent very mild expression of the syndrome. Brain (computed tomography) imaging to investigate mild left hemiparesis in the boy demonstrated severe cortical dysplasia of central, occipital and anterior regions of the right cerebral hemisphere. A variety of brain and cranial malformations has been reported in AOS but dysplasia of the cerebral cortex has not been noted previously. In addition, the boy and his sister have apparent constriction rings present on the toes which are uncommon in AOS.

Carbamic acid: molecular structure and IR spectra.

Infrared absorption spectra of mixed H2O, NH3 and 12CO2/13CO2 ices subjected to 1 MeV proton irradiation were investigated. The results of analyses of the spectra suggest formation of carbamic acid at low temperatures. The stability of this compound in the solid phase is attributed to intermolecular hydrogen bonding of the zwitter-ion (NH3+ COO-) structure.

Tissue expansion in the reconstruction of Tessier craniofacial clefts: a series of 17 patients.

Tessier craniofacial clefts are among the most surgically challenging examples of craniofacial dysmorphology. These clefts are characterized by hypoplasia of soft-tissue and skeletal elements throughout the three-dimensional extent of the cleft. Whereas bone grafting and craniofacial osteotomies have been successful toward correcting the underlying skeletal abnormalities, the ultimate success of these reconstructions has been limited by the deficiency of skin and soft tissue. This deficiency demands reconstruction ideally with tissue of like texture, consistency, and, especially in the face, color. Craniofacial tissue expansion was used toward reconstructing these facial clefts with like-quality tissue, allowing for tension-free reconstruction after osteotomy and bone grafting. Seventeen patients with Tessier craniofacial clefts underwent preoperative craniofacial soft-tissue expansion in the surgical management of their clefts. Tissue expansion was used in the primary correction of facial clefts in eight patients, with nine patients undergoing expansion before secondary surgery. In this series, tissue expansion has evolved as an important element in overcoming the skin and soft-tissue deficiency associated with these clefts, allowing for tension-free closure and improved aesthetic results in these surgically challenging patients.

Outcome measurement of the treatment of maxillary fractures: a prospective analysis of 100 consecutive cases.

We performed a prospective study of 100 consecutive patients presenting to our unit with complex maxillary fractures. Fracture severity was assessed according to the alpha-numeric scoring system of Cooter and David and correlated with outcome. Regional maxillary and total fracture scores demonstrated a positive correlation with complication rates, with correlation coefficients of 0.89 and 0.98 respectively. Ocular and orbital complications were most commonly seen with these complex injuries, but occlusal problems were much less common. Despite close consultant-led supervision in a specialised centre, these injuries frequently result in long term problems.

Coincident metopic synostosis and deformational plagiocephaly.

A male infant is described in whom coincident pathologies of metopic synostosis and deformational plagiocephaly were observed. The role in causation of localized pressure (in particular, extreme constraint) is addressed.

A thymine-like base analogue forms wobble pairs with adenine in a Z-DNA duplex.

The DNA hexamer d(CACGPG), in which dP is the ambivalent pyrimidine nucleoside analogue 2'-deoxy-beta-d-ribofuranosyl-(6H,8H-3, 4-dihydropyrimido[4,5-c][1,2]oxazin-7-one), crystallises as a left-handed Z-DNA duplex. X-ray analysis at 1.5 A shows that both P. A base-pairs are of the wobble type. This result appears inconsistent with other evidence from hybridisation and NMR studies of P-containing oligonucleotides, which suggests that, while P can form stable base-pairs with either A or G, thymine-like properties are more pronounced. Thermal denaturation experiments over a range of solution pH values indicate that protonation of the P.A base-pairs is unlikely to be responsible for the anomalous behaviour. No specific crystal packing effects can be identfied as an explanation, and it is concluded that base stacking and other interactions between nucleotide residues in Z-DNA are responsible.

Bilambdoid and posterior sagittal synostosis: the Mercedes Benz syndrome.

A consistent pattern of craniosynostosis in the sagittal and bilateral lambdoid sutures is described in three patients. The external cranial ridging associated with fusion of these sutures produces a characteristic triradiate, or "Mercedes Benz," appearance to the posterior skull. Locally marked growth restriction is evident in the posterior fossa with compensatory secondary expansion of the anterior fossa manifesting a degree of frontal bossing which mimics bicoronal synostosis. Although this appearance could lead to inadvertent surgery in the frontal region, attention to the occipital region with wide early suture excision and vault shaping is indicated.

Growing skull fracture after cranial vault reshaping in infancy.

Growing skull fractures are reported in three infants undergoing cranial vault surgery for craniosynostosis. Although primary dural injury was overt in only one patient, an extensive dural defect with spatially coincident bony absence was identified in all patients at late exploration. The coincidence of dural injury, deficient overlying bone, and a pulsatile growing brain are necessary conditions for the development of growing skull fractures. With the advent of increasingly radical cranial vault procedures during infancy, the potential for this complication to arise may increase. Definitive correction requires autogenous tissue reconstruction of the dural and bony defect.

Amniotic band syndrome: the association between rare facial clefts and limb ring constrictions.

The pathologic cause of both rare craniofacial clefts and congenital limb ring constrictions is the subject of some debate. Uncommon though these two conditions are, they have often been anecdotally reported in association. This study sought to determine the frequency of congenital limb anomalies in general and limb ring constrictions in particular amongst a population of patients with rare craniofacial clefts. Eighty-five cases of nonsyndromal, rare, craniofacial cleft were identified from a population of 280 patients assessed at the Women's and Children's Hospital, North Adelaide, with a diagnosis of rare craniofacial cleft classifiable by the Tessier system. Twenty-two patients (25.9 percent) displayed congenital limb anomalies. Eleven of these (12.9 percent) showed evidence of limb ring constrictions, a frequency much greater than in the general population. The group with limb ring constrictions demonstrated a significantly greater complexity of involvement with craniofacial clefting than the non-limb ring constriction group (4.27 clefts/patient versus 2.3 clefts/patient, p < 0.01). The distribution of craniofacial cleft locations in patients with evidence of limb ring constrictions was found to differ significantly from those with other or no limb anomalies (p < 0.01). The clefts in cases in which limb ring constrictions coexist are largely restricted to the paramedian 2-12, 3-11, 412 axes. This study confirms an association between rare craniofacial clefts and limb ring constrictions. The two conditions may therefore possess common etiology.

Structure of d(TGCGCA)2 and a comparison to other DNA hexamers.

The X-ray crystal structure of d(TGCGCA)2 has been determined at 120 K to a resolution of 1.3 A. Hexamer duplexes, in the Z-DNA conformation, pack in an arrangement similar to the 'pure spermine form' [Egli et al. (1991). Biochemistry, 30, 11388-11402] but with significantly different cell dimensions. The phosphate backbone exists in two equally populated discrete conformations at one nucleotide step, around phosphate 11. The structure contains two ordered cobalt hexammine molecules which have roles in stabilization of both the Z-DNA conformation of the duplex and in crystal packing. A comparison of d(TGCGCA)2 with other Z-DNA hexamer structures available in the Nucleic Acid Database illustrates the elusive nature of crystal packing. A review of the interactions with the metal cations Na+, Mg2+ and Co3+ reveals a relatively small proportion of phosphate binding and that close contacts between metal ions are common. A prediction of the water structure is compared with the observed pattern in the reported structure.

Computer tomography determined intracranial volume of infants with deformational plagiocephaly: a useful "normal"?

Over the last 10 years, children with deformational (nonsynostotic) plagiocephaly have undergone computed tomography scans using the same protocols as children with lambdoid craniosynostosis because of their similarly distorted head shapes. These children are believed to have normal intracranial volume. Given the recent questioning of what is the normal range of intracranial volume in human populations, the authors have undertaken a comparison of the intracranial volume of children with deformational plagiocephaly and Lichtenberg's normal population (Lichtenberg R. Radiographie du crane de 226 enfants normaux de la naissance a 8 ans: Impressions digitiformes, capacite, angles et indices [thesis]. Paris: University of Paris, 1960). The intracranial volume was determined for 20 females and 46 males with deformational plagiocepahly ranging in age from 2.5 to 20.7 months using computed tomography scan data. Although no significant differences were found for the females, the authors found that the intracranial volume of the males with deformational plagiocephaly were significantly larger than Lichtenberg's population. The authors conclude that this is because Lichtenberg's data do not adequately reflect the normal range of intracranial volume for males ranging in age from 2.5 to 20.7 months, rather than gender differences associated with deformational plagiocephaly. Further, until a more suitable normal becomes available, the deformational (nonsynostotic) plagiocephaly data could be used as substitute normal reference material in the measured age range for assessment of the intracranial volumes of children with craniosynostosis.

Laboratory assessment of tooth whitening by sodium bicarbonate dentifrices.

The increasing emphasis on dental aesthetics has made tooth whitening an important function of a dentifrice. This laboratory study investigated the whitening effect of toothbrushing with sodium bicarbonate-based dentifrices compared with standard dentifrices that do not contain sodium bicarbonate. Six dentifrices and a distilled water control were tested for their ability to whiten teeth with intrinsic stain. The dentifrices contained different abrasive systems: (1) 45% NaHCO3; (2) 65% NaHCO3 (Arm and Hammer Dental Care); (3) 94% NaHCO3; (4) 94% NaHCO3 + 1.5% H2O2; (5) silica (Crest Regular Toothpaste); and (6) dicalcium phosphate (Colgate Regular Toothpaste). After a thorough rubber cup cleaning with a pumice slurry, the intrinsic color of the test teeth with a Vita shade of A3 or darker was measured with a spectrophotometer using the standard L*a*b* color scale. Measurements were taken on a total of 12 teeth per test dentifrice before treatment, and after 30, 60, 90 and 120 minutes of mechanical toothbrushing. No changes in L* (lightness/brightness) or a* (red-green hue) occurred, but significant differences in b* (yellow-blue hue) were observed over time. Compared to baseline tooth color, all four sodium bicarbonate-based dentifrices were significantly effective in removing the yellow intrinsic tooth stain, while the water control, silica dentifrice, and dicalcium phosphate dentifrice demonstrated no significant change. Between-group comparisons showed that the four dentifrices containing sodium bicarbonate were significantly more effective than the water and dicalcium phosphate dentifrice groups. The commercial dentifrice containing 65% sodium bicarbonate was also more effective than the commercial silica dentifrice. Although continued toothbrushing from 30 to 120 minutes resulted in additional stain removal, most of the tooth whitening by the sodium bicarbonate-based dentifrices occurred in the first 30 minutes of brushing. In the studies conducted, dentifrices containing high concentrations of sodium bicarbonate were more effective at removing intrinsic tooth stain than dentifrices that do not contain sodium bicarbonate.

Imaging of the Apert syndrome hand using three-dimensional CT and MRI.

Imaging of the Apert syndrome hand is usually done with plain x-rays. This gives only a limited assessment of the grossly abnormal anatomy. Computed tomography (CT) and magnetic resonance imaging (MRI) have the ability to comprehensively demonstrate the spatial arrangement of the bones as well as the anatomy of the soft-tissue structures. This paper demonstrates the use of these two imaging modalities in the complex Apert syndrome hand to improve surgical planning and treatment.

Localized vitiligo and frontoethmoidal meningoencephalocele.

A child with frontoethmoidal meningoencephalocele in whom localized facial vitiligo developed after surgical correction of the encephalocele is presented. The potential role of the ocular disease accompanying the encephalocele in the development of the hypopigmentation is discussed.