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Biomedical subjects

R Hall

Publications and source records attributed to R Hall.

At least 55 records · Page 3Linked to original sources

Outcome at age 4 years in offspring of women with maternal phenylketonuria: the Maternal PKU Collaborative Study.

CONTEXT: Untreated maternal phenylketonuria (PKU) increases risk for developmental problems in offspring. The extent to which this risk is reduced by maternal dietary therapy at various stages of pregnancy is not known. OBJECTIVE: To determine whether dietary treatment during pregnancy of women with PKU affects developmental outcomes of offspring. DESIGN: The Maternal PKU Collaborative Study, an ongoing, longitudinal prospective study begun in 1984. SETTING: A total of 78 metabolic clinics and obstetrical offices in the United States, Canada, and Germany. PARTICIPANTS: A total of 253 children of women with PKU (n = 149), with untreated mild hyperphenylalaninemia (n = 33), or without known metabolic problems (comparison group; n = 71) were followed up to age 4 years. INTERVENTION: Women with PKU were offered a low-phenylalanine diet prior to or during pregnancy with the aim of maintaining metabolic control (plasma phenylalanine < or =10 mg/dL [< or =605 micromol/L]). Women with mild hyperphenylalaninemia, who had plasma phenylalanine levels of no more than 10 mg/dL (605 micromol/L) on a normal diet, were not treated. MAIN OUTCOME MEASURES: Children's scores on cognitive and behavioral assessments (McCarthy Scales of Children's Abilities, Test of Language Development, Achenbach Child Behavior Checklist, Vineland Adaptive Behavior Scales, and Home Observation for Measurement of the Environment), compared by maternal metabolic status at 0 to 10 weeks', 10 to 20 weeks', and after 20 weeks' gestation. RESULTS: Scores on the McCarthy General Cognitive Index decreased as weeks to metabolic control increased (r = -0.58; P<.001). Offspring of women who had metabolic control prior to pregnancy had a mean (SD) score of 99 (13). Forty-seven percent of offspring whose mothers did not have metabolic control by 20 weeks' gestation had a General Cognitive Index score 2 SDs below the norm. Overall, 30% of children born to mothers with PKU had social and behavioral problems. CONCLUSIONS: Our data suggest that delayed development in offspring of women with PKU is associated with lack of maternal metabolic control prior to or early in pregnancy. Treatment at any time during pregnancy may reduce the severity of delay.

Child Behavior↗

Does intrathecal morphine alter the stress response following coronary artery bypass grafting surgery?

PURPOSE: Intrathecal morphine administered prior to coronary artery revascularization (CABG) surgery was studied to determine its effects on the stress response. METHODS: In a single centre, open, randomized clinical trial, first time elective CABG surgery patients, < 75 yr, were studied. Control subjects (n=12) received a standardized anesthetic consisting of fentanyl (maximum cumulative dose of 35 microg x kg(-1)), propofol, and pancuronium. In addition, spinal subjects (n=13) received 1.0 mg (age > 60 yr) or 1.5 mg (age < or = 59 yr) intrathecal morphine prior to induction of anesthesia. Control subjects received continuous i.v. morphine at 2 mg x hr(-1) on arrival in the ICU with i.v. bolus morphine supplementation as required while spinal subjects received bolus i.v. morphine as required. Changes in plasma cortisol and catecholamine concentrations were measured preoperatively, poststernotomy, on admission to ICU, following tracheal extubation, at 0800 hr on the first postoperative day, and 24 and 48 hr after ICU admission. RESULTS: No differences between groups were detected for demographic variables. The percent change in cortisol concentration relative to preoperative values (control vs spinal; (38 (87) vs -41 (46)%: P < 0.05)) was lower in the spinal group on admission to ICU. The percent change in plasma epinephrine levels (control vs spinal) on admission to ICU (285 (337) vs -10 (37)%) and 0800 hr after surgery (314 (341) vs -4 (37)%) was also significantly different. CONCLUSION: Intrathecal morphine only partially attenuated the postsurgical stress response in CABG surgical patients.

Adult↗

The effect of Portuguese Man-of-war (Physalia physalis) venom on calcium, sodium and potassium fluxes of cultured embryonic chick heart cells.

Portuguese Man-of-war venom markedly increases calcium (45Ca2+) influx into primary, cultured, embryonic chick heart cells. This action is dose-dependent, but is unaffected by organic calcium blockers (diltiazem, verapamil, nifedipine, nimodipine and mibefradil). On the other hand, certain trivalent (La3+, Gd3+) and divalent (Zn2+, Ni2+, Cu2+, Mn2+) metals inhibit venom-induced calcium influx. Sodium (22Na+) influx into chick heart cells is also significantly increased by Man-of-war venom. Flecainide does not block venom-induced sodium influx. The efflux of the potassium analogue, 86Rb+, from heart cells is also significantly increased by the venom. The venom, however, has little or no effect on rubidium (86Rb+) or 2-deoxy-D-[2-3H] glucose influx.

Animals↗

Relapsing polychondritis, smouldering non-secretory myeloma and early myelodysplastic syndrome in the same patient: three difficult diagnoses produce a life threatening illness.

Multiple myeloma, relapsing polychondritis and myelodysplastic syndrome are all serious diseases in which making a clear diagnosis can be difficult. This case of a 72-year-old man found after extensive investigation to have all three of the above, demonstrates how difficult diagnosis and treatment can be, producing in this case a life threatening clinical syndrome. We also postulate that the association of these three diseases may be an immune-derived complication of myelodysplastic syndrome.

Aged↗

Reciprocal cross-protection induced by sporozoite antigens SPAG-1 from Theileria annulata and p67 from Theileria parva.

Theileria annulata and Theileria parva both possess a major surface antigen on the sporozoite stage of the life-cycle, called SPAG-1 and p67, respectively. In each case, these antigens are vaccine candidates and have been shown to induce a degree of homologous protection in earlier work. These antigens share sequence homology and are serologically cross-reactive. Here, we confirm that these antigens confer protection against homologous species challenge. More importantly, they mutually confer a degree of cross-species protection raising the prospect of a common vaccine in the future.

Animals↗

Pathology of early-onset Alzheimer's disease cases bearing the Thr113-114ins presenilin-1 mutation.

Most cases of familial presenile Alzheimer's disease are caused by mutations in the presenilin-1 (PSEN-1) gene, most of these mutations being missense mutations. A mutation in the splice donor site of intron 4 of PSEN-1 has been described recently which results in aberrant splicing of PSEN-1 mRNA, causing insertion of an additional amino acid, Thr113-114ins, into the protein. We studied the neuropathology of four cases bearing this mutation in an attempt to clarify the pathology of this hereditary form of Alzheimer's disease and to determine whether it differs from other familial forms of the disease. The disease presented as a progressive cognitive decline, myoclonus and seizures developing later in the disease, a feature common to PSEN-1-linked Alzheimer's disease. The course of the disease was relatively rapid, death occurring approximately 6 years after onset. Pathology in the intron 4 cases demonstrated a severe Alzheimer's disease pathology with abundant deposition of ss-amyloid (Ass) 1-42 senile plaques and the formation of neurofibrillary tangles. Amyloid angiopathy was present in these cases and was readily demonstrated by Ass 1-40 staining, particularly in the cerebellum. Cases with the intron 4 mutation appear clinically and pathologically similar to other cases of early-onset Alzheimer's disease bearing PSEN-1 mutations.

Adult↗

Assessment of parents' concerns and evaluation of outcomes.

BACKGROUND: Clinical and service evaluation often fails to accommodate sufficiently to parental perspectives and priorities concerning health interventions. Although parent satisfaction questionnaires are widely used, these assess issues chosen by the researcher. Quality of life research methods, however, can assess individual priorities. METHODS: A Schedule for Evaluation of Quality of Life was adapted to record the nature, and relative importance of parental concerns about their child before child psychiatric hospital admission. Level of concern or worry was assessed pre- and post-admission, and at 1 year follow-up, with a waiting-list control. Data were analysed qualitatively and quantitatively for individuals and groups of cases. RESULTS: The adapted instrument was feasible and clinically useful. It did not show repeated measurement effects but was sensitive to the effects of intervention (hospital admission). Effects (reduction in levels of concern) remained evident at 1 year follow-up. CONCLUSION: The instrument is brief, non-intrusive, and sensitive to change. It has utility for clinical case evaluation. It may complement satisfaction questionnaires, and has advantages over rating scales for the evaluation of treatment outcomes.

Adolescent↗

Effect of photodynamic therapy on revascularization of fasciocutaneous flaps.

OBJECTIVE: Photodynamic therapy (PDT) may be used as an adjuvant intraoperative treatment to improve locoregional control. PDT has been shown both to delay wound healing and to have a deleterious effect on flap survival after a primary ischemic insult. This delay in wound healing may make the flap dependent on its pedicled blood supply for a prolonged period. Long-term flap loss may be experienced. The effect of PDT on flap revascularization, with subsequent dependence on its vascular pedicle, is evaluated. STUDY DESIGN: Randomized controlled trial using a rodent model. METHODS: A rat fasciocutaneous flap was used. Study groups were as follows: group I received no treatment; group II received treatment with 630-nm light; groups IH and IV were given Photofrin (in group III, loupes without a fiberoptic light source were used for flap elevation, and in group IV, light source was employed); and group V was given Photofrin and 630-nm light. Primary ischemic times of 2 or 4 hours were used. Vascular pedicles were ligated on postoperative day (POD) 5, 6, or 7, and percentage of flap survival was evaluated 7 days later. RESULTS: With 2 hours of ischemia, revascularization was decreased in the PDT group on POD 6 (P < .05) and on day 7 (P < .005) when compared with the other groups. With 4 hours of ischemia, revascularization was decreased in the PDT group on PODs 5 (P < .001), 6 (P < .01), and 7 (P < .005). CONCLUSION: Intraoperative PDT decreases revascularization of a rat fasciocutaneous flap.

Animals↗

Charnley low-friction arthroplasty with an autograft of the femoral head for developmental dysplasia of the hip. The 10- to 15-year results.

Between 1983 and 1988 we carried out 45 Charnley low-friction arthroplasties with autografts from the femoral head in 41 patients for developmental dysplasia of the hip. The preoperative radiographs were assessed for the severity of DDH according to the classifications of Crowe et al, Hartofilakidis et al and Sharp. The postoperative and follow-up radiographs were analysed for coverage of the socket by the graft, for loosening and for the outcome of the fixation of the bone graft. Two patients died (two hips) at four and seven years after THR from causes unrelated to the surgery and were excluded from the final radiological analysis. The mean age of the patients at the time of operation was 46 years 3 months. The autograft of the femoral head covered a mean 26% (16 to 35) of the acetabular component. All the grafts united. Some degree of resorption of the bone graft occurred in 27 patients, and always involved the lateral part of the graft, which was beyond the margin of the socket. After a mean follow-up of 11 years there had been no revisions and 38 patients had no pain or only slight discomfort. One socket migrated and four others were fully demarcated. Our findings indicate that the Charnley LFA with an autograft of the femoral head for DDH remains successful at a follow-up of 15 years.

Adult↗

Aprotinin decreases exposure to allogeneic blood during primary unilateral total hip replacement.

BACKGROUND: Aprotinin, a hemostatic agent, regulates fibrinolysis, modulates the intrinsic coagulation pathway, stabilizes platelet function, and exhibits anti-inflammatory properties through inhibition of serine proteases, such as trypsin, plasmin, and kallikrein. Aprotinin has been used successfully for many years in cardiac operations, and there have been preliminary investigations of its use in hip replacement operations. The objectives of this multicenter, randomized, placebo-controlled, double-blind trial were to evaluate the efficacy and safety of aprotinin as a blood-sparing agent in patients undergoing an elective primary unilateral total hip replacement and to examine its effect on the prevalence of deep-vein thrombosis in this population. METHODS: Seventy-three patients received a placebo; seventy-six patients, a low dose of aprotinin (a load of 500,000 kallikrein inhibitor units [KIU]); seventy-five, a medium dose of aprotinin (a load of 1,000,000 KIU, with infusion of 250,000 KIU per hour); and seventy-seven patients, a high dose of aprotinin (a load of 2,000,000 KIU, with infusion of 500,000 KIU per hour). The end points for the determination of efficacy were transfusion requirements and blood loss. Patients received standard prophylaxis against deep-vein thrombosis and underwent compression ultrasonography with color Doppler imaging of the proximal and distal venous systems of both legs to evaluate for the presence of deep-vein thrombosis. RESULTS: Aprotinin reduced the percentages of patients who required any form of blood transfusion (47 percent of the patients managed with a placebo needed a transfusion compared with 28 percent of those managed with low-dose aprotinin [p = 0.02],27 percent of those managed with high-dose aprotinin [p = 0.008], and 40 percent of those managed with medium-dose aprotinin [p = 0.5]). Only 6 percent (twelve) of the 212 patients treated with aprotinin required allogeneic blood compared with 15 percent (ten) of the sixty-eight patients treated with the placebo (p = 0.03). Aprotinin decreased the estimated intraoperative blood loss (p = 0.02 for the low-dose group, p = 0.04 for the medium-dose group, and p = 0.1 for the high-dose group), the measured postoperative drainage volume (p = 0.4 for the low-dose group, p = 0.006 for the medium-dose group, and p = 0.000 for the high-dose group), and the mean reduction in the hemoglobin level on the second postoperative day (thirty-four grams per liter for the placebo group, twenty-eight grams per liter for the low-dose group [p = 0.000], twenty-six grams per liter for the medium-dose group [p = 0.000], and twenty-three grams per liter for the high-dose group [p = 0.0001). The rate of deep-vein thrombosis was similar for all groups. CONCLUSIONS: We concluded that aprotinin is safe and effective for use as a hemostatic agent in primary unilateral total hip replacements. In patients who are at high risk of receiving allogeneic blood, use of aprotinin may be of particular clinical and economic benefit.

Aprotinin↗

Surveillance of pneumococcal disease in Australian states and territories.

Information on pneumococcal disease, including immunisation programs, and optimum future surveillance in each Australian State and Territory were discussed at the Pneumococcal Disease in Australia Workshop on 26-27 March 1999. Workshop participants further expanded on the surveillance aspects of the Workshop in this report. Most participants favoured notification by laboratories of pneumococcal isolates from sterile sites, to provide baseline surveillance data before immunisation programs are fully implemented. It was also thought that trends in antimicrobial resistance should be notified.

Australia↗

Oregon's Toxic Household Products Law.

In 1991, Oregon became the first state in the U.S. to require the addition of an aversive agent to ethylene glycol-containing antifreeze and methanol-containing windshield wiper fluid. This new law, entitled "Toxic Household Products (THP) Act," was designed to reduce pediatric and animal poisonings from accidental ingestion of these two potentially lethal consumer automotive products. While not the stated intention of the law, addition of aversive agents to consumer automotive products could also reduce adult poisonings associated with intentional (suicides or alcoholics ingesting methanol-containing windshield wiper fluid) or accidental exposures. This law went into effect April 30, 1995, following settlement of a lawsuit brought by the Chemical Manufacturing Specialties Association (CSMA), a trade group representing the five largest manufacturers of ethylene glycol-based antifreeze in the U.S. This paper discusses the major policy issues that arose following the passage of Oregon's THP Act. Major provisions of the law are provided along with a discussion of CSMA's opposition to the Act's implementation. A description of the eventual settlement that was reached with CSMA as well as the major components of Oregon Health Division's (OHD) enforcement program are also highlighted. Data are presented for 1987 through 1998 on the number of exposures and severity of effects for pediatric cases (children < 6 years old) following exposure to both of these potentially lethal automotive products. However, because of the low incidence of exposures each year, these data are insufficient to draw any conclusions on the impact of the THP Act.

Adult↗

An outbreak of Salmonella typhimurium RDNC A045 at a wedding feast in South Australia.

In April 1998 an outbreak of salmonellosis amongst guests at a wedding feast was investigated. Of the 58 attendees interviewed 38 (66%) subsequently developed gastrointestinal symptoms. Stool cultures from 7 cases grew Salmonella Typhimurium RDNC A045. Food samples were culture-negative for Salmonella spp. A cohort study implicated spatchcock (RR 2.5, 95% CI 1.09-5.77) and scampi (RR 2.0, 95% CI 1.05-3.89). Temperature abuse and cross-contamination within the kitchen during preparation and cooking are likely to have been the main contributing factors to this outbreak. Control measures included staff education in safe food handling and improvements in poultry processing methods to minimise carcass contamination.

Cohort Studies↗