Renal urate hypoexcretion in Polynesian women is not as severe as in United Kingdom (UK) women with familial juvenile hyperuricaemic nephropathy (FJHN).
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Adult↗
Biomedical subjects
M Jackson
Publications and source records attributed to M Jackson.
Toward an unnatural history of immunology.
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Allergy and Immunology↗
Gamekeeper's thumb: a quantitative evaluation of acute surgical repair.
A consecutive series of patients who underwent operation for acute rupture of the ulnar collateral ligament of the thumb during a 3 year period from 1985 to 1988 at the Royal Infirmary of Edinburgh were identified. In total 44 patients sustained this injury of whom 19 were available for clinical assessment. The minimum period of follow-up was 17 months (mean 32 months). All patients were satisfied although six had minor symptoms of discomfort. There were no failures of repair; all repaired ligaments were considered to be clinically taut. A quantitative evaluation demonstrated all had good restoration of pinch grip.
Acute Disease↗
Revision total knee arthroplasty. Comparison of outcome following primary proximal tibial osteotomy or unicompartmental arthroplasty.
Forty-three patients who had undergone revision total knee arthroplasty following either primary, medial unicondylar arthroplasty (23 patients) or valgus tibial osteotomy (20 patients) for medial compartment osteoarthritis were reviewed. Although the two groups had similar knee scores and range of motion at review, six patients (30%) who had undergone prior tibial osteotomy suffered serious postoperative complications. Of these, four patients had a deep infection. It was felt that several factors were responsible, resulting largely from difficulties in gaining access to the lateral tibial plateau and subsequently causing impairment of wound vascularity and healing. In contrast, those patients undergoing revision of unicondylar prostheses did not experience wound healing problems, but it was noted that in half of the cases, there was significant bone loss from the medial tibial plateau.
Humans↗
A prospective randomised study of continuous warm versus intermittent cold blood cardioplegia for coronary artery surgery: preliminary report.
Between October 1991 and March 1993, 281 consecutive patients underwent non-emergency isolated coronary artery surgery under the care of one surgeon (A.R.). They were prospectively randomised to receive either intermittent cold (Group I-144 patients) or continuous warm (Group II-137 patients) blood cardioplegia for myocardial protection. There were no significant differences in clinical outcome between the two groups, as judged by operative mortality, rates of peri-operative myocardial infarction, blood loss, need for circulatory support, post-operative neurological deficit, or duration of intensive care or hospital stay. However, sinus rhythm returned spontaneously with greater frequency (91.2% vs 45.8%, P < 0.001) in Group II patients. There was greater transmyocardial oxidative stress in Group I patients, as evidenced by a significant rise in oxidised glutathione in coronary sinus blood on myocardial reperfusion. Also, the serum CKMb isoenzyme level 2 h post-operatively was significantly raised in Group I patients, although this difference had disappeared by the day after surgery. In conclusion this preliminary report suggests that continuous warm blood cardioplegia provides comparable myocardial protection to that achieved with standard hypothermic techniques in patients undergoing coronary artery surgery.
Coronary Angiography↗
Kinetics and affinity of reactions between an antigen-specific T cell receptor and peptide-MHC complexes.
We show here that the net rate of accumulation of complexes formed by the antigen-specific receptor of T cells (TCR) of a T cell clone with its natural ligand, an octapeptide in association with Ld, a class I protein of the major histocompatibility complex (MHC), approaches the maximal value determined by the affinity of the TCR for this peptide-MHC ligand in 1-2 min, which is well within the lifetime of transient T cell-target cell conjugates. Consistent with this finding, we also found that the widely divergent affinity values (equilibrium constants) of this TCR for six related peptide-MHC complexes correlate well with the extent of specific lysis of target cells bearing various level of these complexes.
Amino Acid Sequence↗
The hazards of prescribing from serum levels.
Clinical audit at a mental handicap hospital suggested that doses of antiepileptic drugs were being altered in response to suspiciously high or low serum level results. In order to assess the reliability of such results, sequential serum levels for individual patients were examined for consistency. Correlation between results which should have been the same was found to be so low as to call into question the value of drug serum level sampling in this hospital. Investigation of the source of the variability continues. In the meantime, clinical staff are reminded that it is unsafe to make any decision on the basis of a single drug serum level.
Anticonvulsants↗
Variation in the arterial to end-tidal PCO2 difference during one-lung thoracic anaesthesia.
We have measured the arterial to end-tidal PCO2 difference (PaCO2-PE'CO2) in 22 patients undergoing pulmonary resection in the lateral thoracotomy position during two-lung ventilation (TLV) and after transition to one-lung ventilation (OLV). With OLV for each patient, the practice of correcting the estimate by an initial measurement of (PaCO2-PE'CO2) was evaluated by subtracting the initial (PaCO2-PE'CO2) from subsequent values measured at 10-min intervals. Net (uncorrected) and corrected differences during OLV were analysed using ANOVA. (PaCO2-PE'CO2) values during TLV and OLV were similar: mean (SD) 1.3 (0.6) kPa and 1.2 (0.7) kPa, respectively (ns). Mean (PaCO2-PE'CO2) varied in the range 0.2-2.5 kPa, while maximum (PaCO2-PE'CO2) was 0.3-2.8 kPa. The mean (SD) of 133 pairs of measurements with OLV was 1.1 (0.7) kPa. Even after correction, mean (PaCO2-PE'CO2) varied in the range -0.7 to 0.8 kPa; individual extreme values were from -1.3 to 1.7 kPa. Variation between patients was found to be greater than variation within patients for both net and corrected differences (F ratio = 37.0 and 10.9, respectively), although calculating a corrected difference did reduce variation between patients from a mean square value of 2.44 to 0.61. The wide variation in (PaCO2-PE'CO2) suggests that the accuracy of estimation of PaCO2 by monitoring PE'CO2, although improved by the use of a corrected difference, remains questionable during OLV.
Adult↗
Polynesian women are also at risk for hyperuricaemia and gout because of a genetic defect in renal urate handling.
The prevalence of asymptomatic hyperuricaemia among Polynesian women (Maoris, Cook Islanders, Samoans, Tongans) was high--44%. This hyperuricaemia resulted from a reduced fractional uric acid clearance (FEur: uric acid clearance factored by creatinine clearance x 100--6.7 +/- 1.5%) compared with the FEur in healthy UK women (12.8 +/- 2.9%). This reduction in FEur was not as great as that in young UK women with familial juvenile hyperuricaemic nephropathy (FJHN: 5.1 +/- 1.5%) and was not associated with impaired renal function. The FEur in the normouricaemic Polynesians (9.7 +/- 1.9%) was also lower than that in healthy UK women (12.8 +/- 2.9%). The reduced FEur in these Polynesian women supports the hypothesis that indigenous Pacific races share a similar genetic defect in renal urate handling to that reported as the basis for the susceptibility to hyperuricaemia in Maori men. Neither alcohol nor hypertension contributed to this. This study also confirmed that, compared with their European counterparts, Polynesian women have a high purine intake and a strong tendency to obesity which increases with age. These factors, together with the reduced FEur, put them at added risk for gout. However, the reduction in FEur was not as great as that reported for the normouricaemic or asymptomatic hyperuricaemic Maori male (4.9 +/- 1.5% and 3.9 +/- 1.4%, respectively), confirming the same sex difference in renal urate handling in adult Polynesians as in caucasians.
Adult↗
Paediatric road accidents in two health districts.
For a 1-year period from 1 January 1990 all children seen, as a result of road traffic accidents (RTAs), in two accident and emergency departments (A&E), one in Liverpool and one in Chester were studied. Simple data were collected and injury severity scores (ISS) were used to assess injuries. Progress was monitored at 3 months post-injury by a review of hospital case notes. A total of 710 children fulfilled the study criteria in the year with a male:female ratio of 1.7:1. Only 37% of children were accompanied by an adult at the time of their accident. Overall 29% of children in vehicles were restrained and only three of 75 cyclists wore cycle helmets. Most injuries were minor with ISS of less than 9 in 96% of cases. Two children died after admission as a result of severe head injuries. The results were similar to those from other centres but highlight a need for continuing public education on child supervision, safe driving habits and the need for environmental changes.
Accidents, Traffic↗
Balloon dilatation of the aortic valve after previous surgical valvotomy: immediate and follow up results.
OBJECTIVE: To evaluate the immediate and long-term results of transcatheter balloon dilatation of the aortic valve for restenosis after previous surgical valvotomy. DESIGN: Prospective follow up by clinical examination and cross sectional Doppler echocardiography of all patients fulfilling the above criteria. SETTING: Tertiary paediatric cardiology referral centre. PATIENTS AND METHODS: 22 patients (18 male, 4 female)--median (range) age 157.5 (12-254) months--underwent 25 balloon dilatation procedures at a median of 72 (8-155) months after surgery. The median age at surgical valvotomy was 82.5 (0.5-230) months and the systolic gradient across the aortic valve immediately after surgery was 31 (0-49) mm Hg. The indication for dilatation was a Doppler derived peak instantaneous gradient of > 60 mm Hg with grade 2 or less aortic regurgitation. A single balloon was used, and the median balloon to annulus ratio was 1 (0.9-1). RESULTS: After dilatation the catheter pullback gradient decreased acutely from 55 (35-75) to 30 (0-75) mm Hg (p < 0.01) and the Doppler gradient from 74 (52-92) to 40.5 (30-96) mm Hg (p < 0.01). In three patients who underwent a second dilatation of the aortic valve eight months after the first procedure, the pullback gradient decreased from 50 (50-60) to 15 (15-16) mm Hg. Aortic regurgitation grade increased from 1 (0-2) to 2 (1-3); only one patient had grade 3 regurgitation. Over a median follow up of 33 (2-67) months seven patients had aortic valve replacement for recurrent stenosis (six patients) or severe regurgitation (one patient with grade 3 regurgitation after dilatation, who had partial detachment of one of the valve leaflets). There was no significant difference for the pullback gradient (median of 19 v 32.5 mm Hg), Doppler gradient 24 hours after dilatation (33.5 v 50.5 mm Hg; p = 0.03), or the duration of follow up (27.5 v 18 months) between the 12 patients who did not require further dilatation or surgery and the 10 patients who did. CONCLUSIONS: Balloon dilatation of the aortic valve is a safe and feasible option for palliation of restenosis after surgical valvotomy for congenital aortic valve stenosis. In many patients, however, stenosis progressed and a further intervention was required.
Adolescent↗
Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960-91): a study of 313 patients.
OBJECTIVE: To determine the incidence of the various types of obstruction of the left ventricular outflow tract in patients born in the five health districts of Liverpool and to compare their prognosis into early adult life. DESIGN: Notes of all patients with obstruction of the left ventricular outflow tract born in the study area between 1960 and 1991 were reviewed. Patients with hypoplastic left ventricle, mitral valve atresia, and those with discordant atrioventricular or ventriculoarterial connections were excluded. Survivors were traced and assessed clinically; eight were lost to follow up. RESULTS: Obstruction of the left ventricular outflow tract occurred in 313 patients (67% male), giving an incidence of 6.1/10,000 live births. The median (range) age at presentation was 13.9 months (0-20 yr). Aortic valve stenosis occurred in 71.2%: subvalve in 13.7%, supravalve in 7.7%, and multilevel in 7.4%. The median (range) duration of follow up was 10.0 (1-29) yr. Aortic regurgitation at presentation occurred more often (p < 0.001) in patients with subvalve stenosis than in those with other types of obstruction, but there was an increased incidence (p < 0.001) at follow up in patients with valve stenosis. Ninety eight patients (31.3%) underwent operation. The reoperation rate was 27% for valve stenosis and 9% for subvalve obstruction. No patients with supravalve stenosis underwent reoperation. The median duration from first operation to aortic valve replacement (17 patients) was 12.3 years. Hazard analysis confirmed that the risk of death was higher in patients presenting at a younger age, with more severe stenosis, and those with subaortic, multilevel obstruction or a syndrome. Hazard analysis also showed that the risk of a clinical event (surgery, balloon dilatation, or endocarditis) was greater in patients who presented at a younger age, with more severe stenosis or aortic regurgitation, and in those with subvalve or multilevel obstruction. CONCLUSIONS: Aortic valve stenosis was the most common type of obstruction. Hazard analysis indicates that the age and severity of obstruction at presentation have a significant effect on survival and freedom from a clinical event. The risk of premature death in patients presenting with moderately severe valve stenosis is reasonably small, but increases considerably in those with subvalve, supravalve, and multilevel obstruction. Patients who present with mild valve stenosis have a good prognosis. The risk of sudden death is less than previous predictions. Patients with subvalve and multilevel obstruction, even when mild at presentation, are more likely to undergo intervention or develop endocarditis than those with valve or supravalve stenosis. Follow up into adult life is essential.
Adolescent↗
Morphology of left ventricular outflow tract structures in patients with subaortic stenosis and a ventricular septal defect.
OBJECTIVE: To compare the incidence and prognosis of subaortic stenosis associated with a ventricular septal defect and to define the morphological basis of subaortic stenosis. DESIGN: Presentation and follow up data on 202 patients with subaortic stenosis seen at the Royal Liverpool Children's Hospital between 1 January 1960 and 31 December 1991 were reviewed. Survivors were traced to assess their current clinical state. Necropsy specimens of 291 patients with lesions associated with subaortic stenosis were also examined. RESULTS: In the clinical study; 65 (32.1%) of the 202 patients with subaortic stenosis had a ventricular septal defect (excluding an atrioventricular septal defect). 32 of these patients had a short segment (fibromuscular) subaortic stenosis. 33 had subaortic stenosis produced by deviation of muscular components of the outflow tracts. In 17 patients (51.5%) this was caused by posterior deviation or extension of structures into the left ventricular outflow tract, resulting in obstruction above the ventricular septal defect. In the other 16 patients (48.5%) there was over-riding of the aorta with concordant ventriculoarterial connections, (without compromise to right ventricular outflow) producing subaortic stenosis below the ventricular septal defect. Additional fibrous obstruction occurred in 39% of the patients with deviated structures. The age at presentation was lower (P < 0.01) in patients with deviated structures (median (range) 0.4 (0 to 9.2) months) than in those with short segment obstruction (median (range) 4.2 (0 to 84.9) months). The incidence of aortic arch obstruction was higher (P < 0.002) in patients with deviated structures than in those with short segment obstruction (38%). In the morphological study 35 pathological specimens showed obstructive muscular structures in the left ventricular outflow tract either above or below the ventricular septal defect. 16 had either posterior deviation of the outlet septum or extension of the right ventriculoinfundibular fold, or both of these together into the left ventricle. 19 had anterior deviation of the outlet septum into the right ventricle with overriding of the aorta (without compromise to right ventricular outflow). The earliest age at which additional fibrous obstruction was seen was 9 months. The aortic valve circumference was small in 18% of specimens. FOLLOW UP: The median (range) duration of follow up in survivors from the clinical study was 6.6 (1 to 25.7) years. 16 patients with deviated musculature (49%) and 16 with short segment fibromuscular stenosis (50%) underwent operation for subaortic stenosis. Patients with deviated structures were younger at operation than those with short segment stenosis (P < 0.005). Patients with posterior deviation or extension of structures into the left ventricular outflow tract underwent operation for subaortic stenosis more frequently (P < 0.05) than those with anterior deviation of the outlet septum and aortic override. The ventricular septal defect required surgical closure more frequently (P < 0.005) in patients with deviation (93.9%) than in those with short segment obstruction (21.9%). There was no significant difference in the mortality between patients with deviation (27%) and those with short segment obstruction (12%). CONCLUSIONS: 32% of patients in the clinical study with subaortic stenosis had a ventricular septal defect. Only 51% of these had obstructive and deviated muscular structures in the left ventricular outflow tract. These patients had a significantly higher incidence of aortic arch obstruction and required surgery for subaortic stenosis at a younger age than those with short segment obstruction. The ventricular septal defect also required surgical closure more frequently in those patients with deviation. The morphological study defined the two sites of obstruction. The presence or absence and type of deviation should be clearly defined in all patients with a ventricular septal defect,
Aortic Stenosis, Subvalvular↗
Long term non-invasive domiciliary assisted ventilation for respiratory failure following thoracoplasty.
BACKGROUND: Ventilatory failure is a well recognised complication of patients who have had a thoracoplasty for tuberculosis, but there are few data regarding the value of long term non-invasive assisted ventilation in this situation. METHODS: Thirty two patients who had had a thoracoplasty 20-46 years previously and who had developed respiratory failure were treated with nocturnal cuirass assisted ventilation or nasal positive pressure ventilation. Their survival and changes in arterial blood gases, nocturnal oximetry, and pulmonary function tests were assessed. RESULTS: The actuarial survival rates at one, three, five, and seven years after starting treatment were 91%, 74%, 64%, and 55%, respectively. Only seven of the 13 deaths were directly attributable to chronic respiratory or cardiac failure. The arterial PO2, PCO2, mean nocturnal oxygen saturation, vital capacity, and maximal inspiratory and expiratory pressures had all improved at the time of the initial post-treatment assessment (mean 12 days after starting treatment), but no subsequent improvements were seen after up to 48 months of follow up. Neither survival nor physiological improvements were correlated with the patients' age, the interval since thoracoplasty, or the pretreatment arterial blood gas tensions or results of pulmonary function tests. CONCLUSIONS: These results show that, even when ventilatory failure has developed, the prognosis with non-invasive assisted ventilation is good and the physiological abnormalities can be partially reversed. Patients who develop respiratory failure after a thoracoplasty should be considered for this type of long term domiciliary treatment.
Adult↗
The 'register of toxicologists'.
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Accreditation↗
Altromycins E, F, G, H and I; additional novel components of the altromycin complex.
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Actinomycetales↗
MLR-52, (4'-demethylamino-4',5'-dihydroxystaurosporine), a new inhibitor of protein kinase C with immunosuppressive activity.
In the course of screening with the mixed lymphocyte reaction, a new inhibitor of protein kinase C with immunosuppressive activity was isolated from the fermentation broth and mycelia of Streptomyces sp. AB 1869R-359. Although certain similarities exist, this strain is morphologically and physiologically distinct from other reported producers of staurosporine-related compounds. We have found that this strain produces relatively high levels of staurosporine and the new minor compound MLR-52, which possesses the indolo[2,3-a]carbazole chromophore of staurosporine, but differs in the substitution pattern of the sugar moiety. Their structures have been elucidated by mass and NMR spectra. MLR-52 has been shown to inhibit the enzymatic activity of protein kinase C and the murine mixed lymphocyte reaction.
Alkaloids↗
Aselacins, novel compounds that inhibit binding of endothelin to its receptor. I. The producing organism, fermentation and biological activity.
A radioligand test to detect inhibitors of endothelin-1 binding to its receptors in bovine atrial and porcine cerebral membranes was used to screen fungal metabolites from stationary fermentations. Inhibitory activity, observed in culture extracts of two Acremonium species, led to the discovery of aselacins A, B and C. Aselacin A inhibits binding to both membrane fractions with IC50s of approximately 20 micrograms/ml.
Acremonium↗