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Biomedical subjects

C L James

Publications and source records attributed to C L James.

16 recordsLinked to original sources

Complications of temporary and definitive external fixation of pelvic ring injuries.

OBJECTIVES: To determine the incidence of complications of external fixation in pelvic ring injuries, comparing fixator use for temporary and definitive treatment. DESIGN: Retrospective case-note review. SETTING: A regional centre for pelvic trauma in the UK. PATIENTS: 100 consecutive patients. INTERVENTION: All patients were treated with pelvic external fixation for a pelvic ring injury. RESULTS: In 52 patients, external fixation was intended for use as the definitive treatment of the pelvic ring injury and was maintained for a mean duration of 60 days (17-113). In 48 patients, it was used temporarily for a mean duration of 8 days (1-20) before internal fixation of the pelvic ring. The complication rate for definitive and temporary fixators was 62 and 21%, respectively. Pin-site infection occurred in 50% of definitive fixators and 13% of temporary fixators but rarely led to more serious complications. In five patients, the definitive management was changed as a result of a complication of the external fixator. The commonest cause for revision of either fixator was aseptic pin loosening. Revision for loose pins in eight patients was associated with the use of two pins in each iliac crest rather than three. CONCLUSIONS: The temporary use of external fixation is safe and effective, but use for definitive treatment is associated with a high rate of infection and aseptic pin loosening.

Adolescent↗

Periocular keratoacanthoma: can we always rely on the clinical diagnosis?

AIM: To present a series of patients with a clinical diagnosis of periocular keratoacanthoma and assess the incidence of histologically proven invasive squamous cell carcinoma (SCC). METHODS: This retrospective case series included all patients with periocular tumours seen in the authors' unit between 1996 and 2004, and who were initially diagnosed with keratoacanthoma based on the clinical presentation. RESULTS: Twelve patients (eight males, four females) were clinically diagnosed with keratoacanthoma. The final histological diagnosis revealed two cases (16.7%) of invasive SCC, and 10 cases (83.3%) of keratoacanthoma. The lower lid was most commonly involved in cases of keratoacanthoma (50.0%). Six patients (60.0%) underwent Mohs surgery, and four (40.0%) were treated with excision under frozen section control. There were no cases of recurrence during a mean follow up period of 21 (SD 13) months. CONCLUSION: Although the clinical presentation of periocular keratoacanthoma is usually characteristic, a significant percentage of patients will prove to have invasive SCC. Complete excision with margin control offers a definitive diagnosis, as well as tissue conservation and a low recurrence rate.

Adult↗

Short-term metabolic responses of soybean root nodules to nitrate.

Soybean (Glycine max L. Merr.) plants exposed to 10 mM KNO(3) for a 4 d period were used to test the correlation between nitrogenase activity, gene expression and sucrose metabolism. Nitrate caused the down-regulation of sucrose synthase (SS) transcripts within 1 d, although a decline in nodule SS activity and an increase in nodule sucrose content only occurred after 3-4 d. In a second experiment, plants were exposed to (15)N-labelled nitrate for 48 h to determine the time period during which nitrate was taken up, and to relate this to the decline in apparent nitrogenase activity (H(2) production in air) and the reduction in SS gene transcript levels. The peak of nitrate uptake appeared to be between 8 h and 14 h whilst apparent nitrogenase activity began to decline at about 17.5 h. The SS mRNA signal declined markedly between 14 h and 24 h. The correlative association of these factors is clear. However, SS activity per se does not appear to be related to the initial decline in apparent nitrogenase activity as a result of nitrate uptake. These findings, therefore, do not support the hypothesis that the regulation of nodule function is mediated by the regulation of SS activity.

Blotting, Northern↗

Neonatal screening for hereditary fructose intolerance: frequency of the most common mutant aldolase B allele (A149P) in the British population.

Hereditary fructose intolerance (HFI) causes severe and sometimes fatal metabolic disturbances in infants and children but responds to dietary treatment. To determine the practicability of screening newborn infants for HFI, we have investigated the frequency of the most common and widespread mutant allele of aldolase B, A149P, in the neonatal population. The polymerase chain reaction was used to amplify aldolase B exon 5 genomic sequences in DNA present in dried blood specimens preserved on Guthrie cards. The A149P mutation was identified by discriminatory hybridisation to allele specific oligonucleotides and confirmed independently by digestion with the restriction endonuclease BsaHI. Twenty-seven A149P heterozygotes were identified by the molecular analysis of aldolase B genes in blood samples obtained from a random cohort of 2050 subjects born in 1994 and 1995, 1.32 +/- 0.49% (95% confidence level). Although no A149P homozygotes were identified, the data allow the frequency of 1 in 23,000 homozygotes for this allele to be predicted. Our findings have implications for establishing an interventional mass screening programme to identify newborn infants with HFI in the UK.

Alleles↗

Basal cell carcinoma with hyaline inclusions.

A 69 yr old man had a 4 mm basal cell carcinoma completely excised from the chin. Numerous hyaline cytoplasmic inclusions were contained within the tumor cells. The inclusions stained intensely red with Masson's trichrome, and immunocytochemically there was prominent rim labelling for keratins (bovine, callus and AE1/3) and muscle-specific actin, the latter more faintly decorating the centre of some inclusions. The inclusions were negative for antibodies to cytokeratin Cam5.2, epithelial membrane antigen (EMA), vimentin, S100, neurofilaments, glial fibrillary acidic protein (GFAP) and carcinoembryonic antigen (CEA) and there was no post Congo red apple green birefringence to indicate amyloid. Ultrastructure indicated the inclusions were composed of proteinaceous material surrounded by a defined rim of tonofilaments in cells showing no degenerative features. The findings suggested aberrant tumor cell keratinization. Familiarization with this rare variant of a common cutaneous carcinoma will alleviate diagnostic difficulties that may arise, particularly in superficial tumor curettage.

Aged↗

D1 dopamine agonist and antagonist effects on regional cerebral glucose utilization in rats with intact dopaminergic innervation.

The effects of stimulation and blockade of the D1 dopamine receptor on regional cerebral glucose utilization (RCGU) were studied using quantitative [14C]2-deoxyglucose autoradiography in naive rats. Systemic administration of the selective D1 antagonist, SCH 23390 (0.5 mg/kg), lowered glucose utilization by 24-28% in the globus pallidus, entopeduncular nucleus, subthalamic nucleus, substantia nigra pars reticulata (SNr), and motor cortex, suggesting that stimulation of the D1 receptor by endogenous dopamine contributes to basal metabolism in these regions. Administration of SCH 23390 increased RCGU in the lateral habenula, as do selective D2 antagonists. The selective D1 agonist, SKF 38393 (30 mg/kg), increased RCGU in the SNr (up 22%) without affecting the other brain regions which were examined. This modest increase contrasts with the large increase in RCGU (up 100-200%) in the SNr elicited by similar doses of SKF 38393 in rats with acute or chronic dopamine depletion. Systemic administration of amphetamine (5.0 mg/kg), a dopamine releasing agent, increased RCGU in the caudate-putamen (up 33%), globus pallidus (up 23%), subthalamic nucleus (up 46%), entopeduncular nucleus (up 78%), and SNr (up 72%) and lowered RCGU in the lateral habenula (down 43%). All of these amphetamine effects were blocked by pretreatment with either SCH 23390 (0.5 mg/kg) or eticlopride (2.0 mg/kg, a selective D2 antagonist). These results suggest that endogenous dopamine stimulates both D1 and D2 receptors in vivo and provide metabolic evidence to support the concept of a functional linkage of D1 and D2 receptor systems in animals with intact dopaminergic innervation.

2,3,4,5-Tetrahydro-7,8-dihydroxy-1-phenyl-1H-3-ben↗

Metastatic osteogenic sarcoma to the heart presenting as bacterial endocarditis.

A patient with metastatic osteogenic sarcoma involving the left atrium is described who presented with features of bacterial endocarditis. The source of infection was the adjacent esophagus into which the tumor had eroded. This case demonstrates that sarcomas metastasizing to the heart may result in a clinical condition indistinguishable from infective endocarditis. At post-mortem, careful dissection of cardiac metastases should be undertaken to check for possible esophageal involvement.

Adult↗

Myositis ossificans in childhood.

Myositis ossificans is a benign, localized, ossifying lesion of soft tissues that is rarely reported in young children. This paper describes two cases found in a search of the surgical biopsy files of the Adelaide Children's Hospital over the 30 yr period from 1962 to 1991, in boys both aged 7 yrs. Diagnosis was assisted by combined clinical, radiological and histopathological information (including an adequate well-orientated biopsy in Case 1 that demonstrated the characteristic growth pattern) enabling differentiation from other possibilities such as fibrodysplasia ossificans progressiva.

Child↗

Rapid development of dopaminergic supersensitivity in reserpine-treated rats demonstrated with 14C-2-deoxyglucose autoradiography.

Dopaminergic denervation supersensitivity has been implicated in the pathogenesis of levodopa-induced dyskinesias, the most common and limiting side effect in the drug treatment of Parkinson's disease, yet the mechanisms that mediate altered drug sensitivity remain poorly understood. In animals models, one key component of denervation supersensitivity is the enhanced efficacy of selective D1 agonists to stimulate locomotion. In rats with chronic dopamine depletion induced by 6-hydroxydopamine nigral lesion, the increased ability of D1 agonists to stimulate regional cerebral glucose utilization (RCGU) in the substantia nigra pars reticulata (SNr) has provided a metabolic correlate to the heightened motor response. In this study, we used the stimulation of RCGU in the SNr as a sensitive in vivo assay of D1 agonist effect to examine the time course of development of supersensitivity in rats following acute dopamine depletion with single doses of reserpine (5.0 mg/kg, i.p.) and alpha-methyl-p-tyrosine (AMPT; 100 mg/kg, i.p.). The stimulatory effect of the D1 agonist SKF 38393 (30 mg/kg) on RCGU in the SNr was first enhanced 6 hr after reserpine/AMPT injection and was maximally enhanced at 12-24 hr (relative 2-deoxyglucose uptake increased 32-51%; P less than 0.05). The response to SKF 38393 returned to control values 5 d after reserpine/AMPT injection. The single reserpine/AMPT injections depleted striatal dopamine to 1-2% of control values from 3-48 hr postinjection, whereas D1 and D2 dopamine receptor densities were unchanged at 24 hr.(ABSTRACT TRUNCATED AT 250 WORDS)

2,3,4,5-Tetrahydro-7,8-dihydroxy-1-phenyl-1H-3-ben↗

D1/D2 dopamine receptor stimulation by L-dopa. A [14C]-2-deoxyglucose autoradiographic study.

In rats with unilateral 6-hydroxydopamine substantia nigra lesions, the effects of selective D1 and D2 dopamine receptor antagonists on L-DOPA-induced rotation and regional cerebral glucose utilization (RCGU) changes were examined. Contralateral rotation induced by L-DOPA (25 mg/kg) was effectively blocked by D1 (SCH 23390, 1.0 mg/kg) and D2 (eticlopride, 2.0 mg/kg) antagonists, in combination, but not by either antagonist alone. This suggests that in the dopamine-depleted rat, L-DOPA administration results in the stimulation of both D1 and D2 receptor systems, each capable of independently eliciting a full motor response, L-DOPA altered RCGU in the following brain regions ipsilateral to the lesion: entopeduncular nucleus (EP, + 105%), substantia nigra pars reticulata (SNr, + 121%), subthalamic nucleus (STN, + 32%), deep layers of the superior colliculus (DLSC, + 35%), and lateral habenula nucleus (LHN, -52%). The effects in the EP and SNr were blocked completely by D1 antagonist pretreatment but only partially attenuated by D2 antagonist pretreatment, indicating the critical dependence of these changes on D1 stimulation. In contrast, combined D1 and D2 antagonist pretreatment, but neither drug alone, blocked the L-DOPA-induced increases in the STN and DLSC. The effects of L-DOPA in the LHN were attenuated by either SCH 23390 or eticlopride, and blocked completely by the antagonist combination. These results provide evidence that dopamine formed following the decarboxylation of L-DOPA stimulates both D1 and D2 receptors in vivo and that stimulation of each receptor contributes uniquely to its physiological effects. Neural mechanisms of action of L-DOPA are discussed in the context of these findings.

Animals↗

Cryptococcal epididymo-orchitis complicating steroid therapy for relapsing polychondritis.

Disseminated cryptococcosis is a known complication of steroid therapy. Infection within the genito-urinary tract is usually assumed to be part of generalized cryptococcosis complicating a primary pulmonary focus. A case of isolated testicular cryptococcal orchitis complicating steroid therapy for relapsing polychondritis is presented. To the authors' knowledge isolated cryptococcal orchitis has not been previously described.

Aged↗

Epithelioid leiomyosarcoma of the left atrium: immunohistochemical and ultrastructural findings.

Leiomyosarcomas of the heart are rare, with only 12 cases reported in the literature. An example of the epithelioid variant of leiomyosarcoma is described. The tumour cells expressed vimentin, desmin, muscle-specific actin and smooth muscle-specific actin. In addition, they were also labelled by monoclonal and polyclonal antibodies to cytokeratin intermediate filaments and displayed cell junctions at the ultrastructural level, features which highlight the potential pitfalls in the application of immunohistochemistry and electron microscopy in the diagnosis of poorly differentiated tumours.

Female↗

Total anomalous pulmonary venous drainage associated with fatal outcome in infancy and early childhood: an autopsy study of 52 cases.

Clinicopathological details of 52 cases of total anomalous pulmonary venous drainage (TAPVD) taken from pediatric autopsy files from hospitals in Adelaide (Australia) Oxford and Edinburgh (United Kingdom) between 1957 and 1990 are presented. The patients ranged in age from a stillborn girl to a 15-month-old boy, with 42 cases (81%) dying in the first 3 months of life. While many patients had signs of a congenital cardiovascular anomaly prior to death, including tachypnea, tachycardia, central cyanosis, cardiac failure, heart murmurs, and difficulty in feeding, it was noteworthy that eight patients (16%) presented as sudden and unexpected death in the absence of significant antemortem symptoms and signs. Anomalous pulmonary venous drainage was also unsuspected prior to death in a total of 26 cases (53%) of those where relevant history was available (49 cases). Twelve infants (23%) underwent surgical correction, none of whom survived more than several weeks. TAPVD was isolated in 30 cases (58%) and was associated with other cardiac or congenital anomalies in 22 patients (42%). Just under half of nonisolated cases comprised the asplenia-heterotaxy syndrome. The points of drainage of the anomalous pulmonary veins were to the infradiaphragmatic veins (n = 21, 40%), left innominate vein (n = 13, 25%), coronary sinus (n = 7, 13%), right superior vena cava (n = 4, 8%), inferior vena cava above the diaphragm (n = 2, 4%), right innominate vein (n = 2, 4%), mixed left innominate vein and coronary sinus (n = 1, 2%), azygos vein (n = 1, 2%), and mixed right superior vena cava and left hemiazygos vein (n = 1, 2%). Twenty-three of 47 cases (49%) that were specifically examined revealed obstruction of the pulmonary veins or pulmonary hypertensive vascular changes on histology. These results emphasize that TAPVD needs to be excluded at autopsy as a causal factor in cases of sudden infant death even in the absence of antemortem symptoms and signs. Clues at autopsy include abnormal mobility of the heart, visceral situs inversus, and polyasplenia. The diversity of pulmonary-systemic venous anastomoses necessitates careful in situ dissection above and below the diaphragm and consideration of postmortem angiography.

Autopsy↗