An Eiffel penetrating head injury.
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Accidental Falls↗
Biomedical subjects
J Round
Publications and source records attributed to J Round.
The natural history of developmental dysplasia of the hip after early supervised treatment in the Pavlik harness. A prospective, longitudinal follow-up.
Between June 1988 and December 1997, we treated 332 babies with 546 dysplastic hips in a Pavlik harness for primary developmental dysplasia of the hip as detected by the selective screening programme in Southampton. Each was managed by a strict protocol including ultrasonic monitoring of treatment in the harness. The group was prospectively studied during a mean period of 6.5 +/- 2.7 years with follow-up of 89.9%. The acetabular index (AI) and centre-edge angle of Wiberg (CEA) were measured on annual radiographs to determine the development of the hip after treatment and were compared with published normal values. The harness failed to reduce 18 hips in 16 patients (15.2% of dislocations, 3.3% of DDH). These required surgical treatment. The development of those hips which were successfully treated in the harness showed no significant difference from the normal values of the AI for the left hips of girls after 18 months of age. Of those dysplastic hips which were successfully reduced in the harness, 2.4% showed persistent significant late dysplasia (CEA <20 degrees) and 0.2% persistent severe late dysplasia (CEA <15 degrees). All could be identified by an abnormal CEA (<20 degrees) at five years of age, and many from the progression of the AI by 18 months. Dysplasia was considered to be sufficient to require innominate osteotomy in five (0.9%). Avascular necrosis was noted in 1% of hips treated in the harness. We conclude that, using our protocol, successful initial treatment of DDH with the Pavlik harness appears to restore the natural development of the hip to normal. We suggest that regular radiological surveillance up to five years of age is a safe and effective practice.
Acetabulum↗
Isolation and characterization of the activated B-cell factor 1 homolog in Caenorhabditis elegans.
Members of the basic helix-loop-helix (bHLH) family of transcription factors regulate a wide array of developmental processes in many cell types, including cell fate specification, differentiation and morphogenesis. Our studies describe the cloning of a gene from the nematode Caenorhabditis elegans that is closely related to the vertebrate-activated B-cell factor (ABF) gene. The nematode gene product CeABF-1 was detected by northern blot analysis from RNA isolated from pooled nematodes representing different developmental stages. The developmental expression profile of CeABF-1 was shown by RT-PCR analysis to be predominantly expressed in the larval stages L3 and L4, with lower levels observed in the L2 larval stage and adult. We also show that CeABF-1 is capable of forming heterodimers with E2A proteins and binding E-box target sites. Mammalian cells transfected with CeABF-1 expression plasmids were capable of blocking E2A-mediated gene transcription, but full repression activity required the presence of two conserved amino acid residues found within the first helix of the CeABF-1 bHLH domain. These results suggest a conserved mechanism of gene repression between certain class II bHLH and class I bHLH proteins found in vertebrates and invertebrates.
Amino Acid Sequence↗
Characterization of a basic helix-loop-helix protein, ABF-1: nuclear localization, transcriptional properties, and interaction with Id-2.
The activated B-cell factor (ABF)-1 cDNA was initially isolated from Epstein-Barr virus (EBV)-infected B cells and codes for a DNA-binding protein belonging to the basic helix-loop-helix (bHLH) family of transcription factors. In this study, we characterized the nuclear localization signal of ABF-1, mapped two distinct transcriptional repression domains, and identified one ABF-1-interacting protein, Id-2. By examining the subcellular location of deletion mutants of ABF-1 fused to green fluorescent protein (GFP), critical regions involved in nuclear localization were determined. Analysis of GFP-tagged ABF-1 deletion mutants revealed two separate regions capable of directing nuclear localization. One region mapped to the N-terminal amino acids 71 to 103, whereas the second region localized to the C-terminal bHLH domain. Transient transfection of ABF-1 deletion mutants demonstrated that the N-terminal amino acids 1 to 40 and the bHLH domain function together to achieve maximum repression of E2A activity. Taken together, these results indicate that ABF-1 is a nuclear transcriptional repressor with two distinct regions that function in a synergistic fashion to attenuate E2A-mediated gene activation.
Basic Helix-Loop-Helix Proteins↗
Clocks in delivery wards may not be sufficiently accurate to validate birth of "millennium babies".
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Female↗
A clinical evaluation of a direct radioimmunoassay of testosterone.
We report the evaluation of a commercial coated tube kit (Medgenix) to the measurement of testosterone in plasma/serum samples. The accuracy, precision, specificity and recovery of the assay and the affinity of antibody for ligand were established. The range of concentrations of testosterone providing satisfactory analyses was 0.48-48 nmol/l, which is appropriate for general clinical use. Results of analysis of male and female samples obtained from the routine hospital laboratory in endocrinologically-defined groups were also reviewed. In all groups of male subjects and in normal females, the kit provided results in close agreement to those of an extraction method. With samples from women with various reproductive disorders, as well as with some samples obtained from external quality controls, the kit showed a small but significant negative bias relative to the results of the extraction method. There was, however, no evidence of bias with samples from a separate group of volunteer women with the polycystic ovary syndrome. No interference due to SHBG was detected. Clinical sensitivities using the kit were equivalent to those obtained using the extraction RIA. There was no evidence of spuriously high results in samples from females which has been a common problem with direct radioimmunoassay of testosterone.
Adolescent↗
Studies to confirm the source of 11 beta-hydroxyandrostenedione.
In a longitudinal study of 82 children we found a gradual rise in median plasma concentrations of 11 beta-hydroxyandrostenedione (11 beta-OH-A4) from 2.5 to 6.4 nmol/l during childhood which was similar in both sexes. This could reflect changes in adrenal function during the adrenarche and sexual maturation. Plasma concentrations of 11 beta-OH-A4 in adults follow the patterns of cortisol secretion. In patients with diseases of the adrenal cortex, the plasma concentrations of 11 beta-OH-A4 were consistent with the pathology of each condition. In women with polycystic ovaries (PCO) undergoing gonadotrophic stimulation for in vitro fertilization and embryo transfer, 11 beta-OH-A4 (median = 3.8 nmol/l), testosterone and androstenedione, were raised when compared to women with normal ovaries (11 beta-OH-A4 median = 2.6 nmol/l). Follicular fluid has concentrations of 11 beta-OH-A4 six to twelve times greater than plasma levels and in women with PCO, 11 beta-OH-A4 concentrations were lower than in women with normal ovaries, which is consistent with an inhibition of ovarian 11 beta-hydroxylase. Granulosa cells in vitro demonstrated the production of 11 beta-OH-A4 by side chain cleavage of cortisol. These data support an adrenal source for 11 beta-OH-A4 but the raised plasma concentrations in women with polycystic ovary syndrome (PCOS) may reflect the excess androgen output from the ovary. 11 beta-OH-A4 may therefore be an additional marker for ovarian dysfunction.
Adult↗
Reduction in IgG galactose in juvenile and adult onset rheumatoid arthritis measured by a lectin binding method and its relation to rheumatoid factor.
Glycosylation changes in patients with juvenile chronic and adult onset rheumatoid arthritis have been studied using a novel binding method. Both these major types of arthritis showed decreased galactosylation of serum IgG, which confirms earlier studies using a different, more complex chemical method. No significant correlation between serum IgG, IgM, and IgA rheumatoid factors and age corrected G(o) (percentage of oligosaccharide chains lacking galactose) was found. The possibility that the less glycosylated IgG is preferentially confined to circulating IgM/IgG immune complexes cannot be excluded, however.
Adolescent↗
Muscle strength in girls with congenital adrenal hyperplasia.
To assess whether exposure to excessive adrenal androgens increases muscle strength in girls with congenital adrenal hyperplasia, eighteen girls with 21 hydroxylase type congenital adrenal hyperplasia (CAH), aged 4.3-12.1 years were studied and compared with 78 healthy control girls aged 4-16 years. Maximum voluntary isometric strength was measured using a muscle testing chair, and the highest value from at least three attempts with each leg was recorded. Height and weight were also measured in the patients and controls. When compared with normal girls of similar age, some of the girls with CAH seemed unusually strong, but this difference was less marked when the CAH girls were compared with controls of similar weights. However, our data suggest that girls diagnosed later in childhood may have unusual muscle strength.
Adolescent↗
Diaphragm paralysis causing ventilatory failure in an adult with the rigid spine syndrome.
A syndrome consisting of a rigid spine and myopathy predominantly affecting proximal limb muscles has been previously described in children, and as with most neuromuscular disorders, the respiratory muscles appear to be affected only at an advanced stage in the disease. We describe an adult male with this syndrome who presented with ventilatory failure caused by severe respiratory muscle weakness and who demonstrated profound nocturnal arterial oxygen desaturation, particularly during rapid eye movement sleep. Treatment with negative pressure ventilation initially resulted in only modest improvements in symptoms, blood gas tensions, and nocturnal desaturation. The cause of this only partial improvement was upper airway obstruction provoked by the mode of ventilatory support used. After tracheostomy there was a dramatic and sustained improvement in symptoms and blood gas tensions and complete abolition of nocturnal arterial oxygen desaturation. This is the first report of an adult with the rigid spine syndrome presenting with ventilatory failure and cor pulmonale due to severe respiratory muscle weakness.
Adult↗
Corticosteroid myopathy: a clinical and pathological study.
In six patients with Cushing's syndrome and three with steroid myopathy, the clinical, functional, biochemical and structural characteristics of myopathy are described. Proximal muscle weakness occurred in all the patients, preferentially affected the lower limbs and was accompanied by muscle wasting in all but one patient. Force measurements confirmed quadriceps weakness in every patient. Vastus lateralis muscle biopsies showed light microscopic abnormalities in two of three patients with steroid myopathy and one of five patients with Cushing's syndrome. Type II fibre atrophy was the commonest abnormality. Reduced type II mean fibre areas occurred in all the patients with steroid myopathy and were common in Cushing's syndrome patients. Type I mean fibre areas were also reduced in two of the former group and one of the latter group and two further patients in this group had areas at the lower end of the normal range. Abnormalities in electron microscopy, mitochondrial function tests and chemical content of skeletal muscle were frequent and are described and discussed. A plasma creatine kinase activity (CK) at the lower end of the normal range, a myopathic electromyogram (EMG) and a raised 24-h urinary 3-methylhistidine/creatinine ratio on a creatine free diet were other characteristic findings in both groups of patients.
Adolescent↗
Serum prolactin and oestradiol levels at different stages of puberty.
Serum prolactin and oestradiol levels were measured in 183 children who were staged for pubertal development. Mean serum prolactin levels are higher in girls than in boys at stage 3 of pubertal development. In girls prolactin levels rise at stage 2, and are higher after menarche; in boys there is no change in prolactin levels. In this study serum oestradiol correlates with prolactin in the girls, but not in the boys.
Adolescent↗