Early diagnosis of cystic fibrosis.
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Biomedical subjects
Publications and source records attributed to C A Boyle.
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OBJECTIVE: We sought to quantify the strength of associations between each of four specific developmental disabilities (DDs) and specific types of major birth defects. METHODS: We linked data from 2 independent surveillance systems, the Metropolitan Atlanta Congenital Defects Program and the Metropolitan Atlanta Developmental Disabilities Surveillance Program. Children with major birth defects (n = 9142; born 1981-1991 in metro Atlanta) and 3- to 10-year-old children who were born between 1981 and 1991 in metro Atlanta and identified between 1991 and 1994 as having mental retardation, cerebral palsy, hearing impairment, or vision impairment (n = 3685) were studied. Prevalence ratio (PR), which is the prevalence of a DD in children with 1 or more major birth defects divided by the prevalence of the same DD in children without major birth defects, was measured. RESULTS: Among the 9142 children who were born with a major birth defect, 657 (7.2%) had a serious DD compared with 0.9% in children with no major birth defect, yielding a PR of 8.3 (95% confidence interval: 7.6-9.0). In general, the more severe the DD, the higher was the PR. Birth defects that originated in the nervous system and chromosomal defects resulted in the highest PRs for a subsequent DD. For all other categories of birth defects, PRs were lowest when all major birth defects present were confined to a single category (ie, isolated defects). PRs for any DD increased monotonically with the number of coded birth defects per child or the number of different birth defect categories per child, regardless of the severity of the defect or whether defects of the nervous system, chromosomal defects, or "other syndromes" were counted. CONCLUSIONS: These data highlight the possible early prenatal origins of some DDs and suggest that both the number of coded birth defects present and the number of anatomic systems involved are strongly related to functional outcomes.
The authors examined the relation between intrapartum magnesium sulfate exposure and risk of cerebral palsy in a case-control study of low birth weight children designed to control for confounding by the clinical indications for magnesium in pregnancy. Case children (n = 97) included all singleton children with cerebral palsy who were born in 1985-1989 in Atlanta, Georgia with a birth weight less than 1,750 g and whose mothers had not had a hypertension-related disease during pregnancy. Control children (n = 110) were randomly selected from the infant survivors using identical selection criteria. Data on magnesium sulfate exposure, labor and delivery, and infant characteristics were abstracted from hospital records. The authors found no association between exposure to magnesium sulfate and cerebral palsy risk (odds ratio = 0.9; 95% confidence interval: 0.3, 2.6) either in all children or in subgroups with varying likelihoods for exposure to magnesium. However, the association did vary by birth weight, with a protective effect being seen in children born weighing less than 1,500 g and an elevated risk in children with birth weights of 1,500 g or more; all confidence intervals included 1.0 except for the combined <1,500 g group. Several ongoing randomized clinical trials of magnesium and cerebral palsy may shed more definitive light on this relation.
OBJECTIVE: Evaluation of a sedation technique which involved titrating intravenous midazolam to an 'ideal' sedation end point, followed by a continuous infusion of propofol. This technique might be a satisfactory alternative to general anaesthesia for implant surgery in anxious patients or when procedures exceed 60-min duration. MATERIAL AND METHODS: 20 patients were treated on 23 occasions. A 2-mg bolus of midazolam was injected intravenously followed after 90 s by 1 mg increments until the patient was adequately sedated. 30 min after this induction with midazolam, a propofol infusion was started at a rate which varied between 0-300 mg/h (0-30 ml/h). The actual infusion rate was adjusted in order to maintain the optimum sedation level which had been achieved using midazolam. The quality of sedation was assessed using Ellis and Sedation scores. RESULTS: Midazolam dosage varied between 5 and 14 mg. The initial propofol infusion rate was 200 mg/h (20 ml/h) for 21 of the 23 implant surgery treatments. CONCLUSION: The sedation technique described takes advantage of the differing pharmacokinetic properties of midazolam and propofol. Initial sedation was achieved using midazolam, this was then maintained using a variable propofol infusion. The shorter distribution and elimination half-lifes of the latter drug made matching the level of sedation to the patient's needs easier and also provided good short-term recovery.
Salivary gland inclusions in the mandible are relatively uncommon. If defects occur they are generally unilateral, although bilateral cases have been reported. This article describes an unusual case in which the dental panoramic tomogram revealed three radiolucent areas in the mandible. The diagnosis of the two posterior radiolucencies was confirmed as Stafne's bone cavities but a definite diagnosis for the parasymphyseal lesion remained elusive, even after surgery. However, Stafne's bone cavities are known to occur in this region and this diagnosis remains the most probable.
This paper demonstrates how oral midazolam can be employed as an alternative method of behaviour management to general anaesthesia for the dental treatment of people with learning disabilities. A range of treatments, from scaling to root canal therapy, can be carried out successfully using the sedation technique outlined. The advantages of sedation include reduced morbidity and mortality. Treatment outcomes are also likely to be improved as root canal therapy and periodontal care can be carried out over a number of visits rather than a single treatment session under general anaesthesia. Oral sedation with midazolam should improve the scope of dental treatment available to patients with disabilities.
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Migraines may occur at any time during the menstrual cycle but are commonly associated with the menses. Migraine-specific medications, such as the triptans, may be effective for acute management of menstrual migraine. However, it is important to recognize the relationship between migraines and the menstrual cycle because these headaches may not respond to the usual antimigraine medications. In that case, management may involve perimenstrual migraine prophylaxis, with migraine-specific medications used in addition for severe breakthrough migraines. Prostaglandin inhibitors started just before the time of headache vulnerability may prevent menstrual migraine attacks or reduce the severity of the headaches. Estrogen withdrawal has been shown to precipitate migraine headaches, and a sustained elevated level of estrogen will postpone the migraine. Transdermal estrogen started just before menstruation can provide a sustained low level of estrogen, decreasing the degree of estrogen decline, and thus may prevent induction of migraines. Ergotamine tartrate is usually taken only for acute migraine, but may also be effective for prevention of menstrual migraine when used regularly once or twice per day during the time of risk. By understanding the underlying pathophysiology of the relationship between migraines and the menstrual cycle, the physician can successfully treat migraines associated with menses.
OBJECTIVE: To examine trends in alcohol use among pregnant women in the United States and to characterize pregnant women who use alcohol, with an emphasis on frequent use (at least five drinks per occasion or at least seven drinks per week). METHODS: We used the Behavioral Risk Factor Surveillance System data from 1988 through 1995 to obtain the percentage of pregnant women who used alcohol. We used multiple logistic models to identify subgroups of pregnant women who are at increased risk for alcohol use. RESULTS: Overall, 14.6% (869 of 5983) of pregnant women consumed alcohol and 2.1% (133 of 5983) consumed alcohol frequently. Among pregnant women, alcohol use decreased from 22.5% (95% confidence interval [CI] 20.8, 23.9) in 1988 to 9.5% (95% CI 7.9, 11.8) in 1992 and then increased to 15.3% (95% CI 13.1, 17.2) by 1995. Among pregnant women, frequent alcohol use decreased from 3.9% (95% CI 2.4, 5.2) in 1988 to 0.9% (95% CI 0.4, 1.6) in 1991 and then increased to 3.5% (95% CI 2.0, 5.1) by 1995. Pregnant women who were at high risk for alcohol use were college educated, unmarried, employed, or students, had annual household incomes of more than $50,000, or were smokers. Pregnant women who were at high risk for frequent alcohol use were more likely to be unmarried, or smokers. CONCLUSION: The increasing prevalence of alcohol use among pregnant women calls for increased ascertainment of alcohol use among preconceptional and pregnant women. Brief interventions by clinicians, increased referral to alcohol treatment programs, and increased use of contraception by women of reproductive age who are problem drinkers should be considered as means of preventing alcohol-exposed pregnancies.
A peritoneal dialysis (PD) solution containing 1.1% amino acids as the osmotic agent was evaluated in a 3-month randomized, prospective, open-label study in malnourished PD patients. Patients in the treatment group (DAA) received one or two exchanges daily with the amino acid solution, depending on tolerance, in place of glucose solutions. Controls (DD) received their usual therapy with glucose dialysate. Fifty-four DAA and 51 DD patients completed the study. In DAA, but not in DD patients, there was a significant increase at month 3 in serum insulin-like growth factor-1 (IGF-1) levels and significant decreases in serum potassium (all 3 months) and inorganic phosphorus levels (months 1 and 3), indicating a general anabolic response. Prealbumin and transferrin levels were significantly increased in DAA but not in DD patients at month 1, but the groups did not differ at months 2 and 3. In patients with baseline albumin levels less than 3.5 g/dL (bromcresol green [BCG] method), DAA patients showed increases in albumin, transferrin (months 1 and 2), and prealbumin levels (all 3 months) relative to baseline values, whereas these serum protein levels were unchanged in DD patients, although the changes from baseline did not differ between groups. In this subgroup, midarm muscle circumference (MAMC) did not change in DD or DAA patients. In patients with baseline albumin levels of 3.5 g/dL or greater, DD patients had decreases in albumin and total protein levels at all 3 months and in prealbumin levels at months 1 and 2, relative to baseline. In DAA patients, there were fewer changes in serum proteins. MAMC increased significantly from baseline in DAA but not in DD patients, although changes from baseline did not differ between DAA and DD groups. DAA patients showed no changes in peritoneal membrane transport characteristics. The results indicate that treatment with one or two exchanges daily of this amino acid-based PD solution is safe and provides nutritional benefit for malnourished PD patients.
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We studied paternal exposure to Agent Orange and its dioxin contaminant (2,3,7,8-tetrachlorodibenzo-p-dioxin) and preterm birth, intrauterine growth retardation, or infant death in veterans of Operation Ranch Hand, the unit responsible for spraying herbicides during the Vietnam war. A Comparison group of Air Force veterans who served in Southeast Asia during the same time period and who were not occupationally exposed to herbicides was included. We studied children conceived during or after the father's service in Southeast Asia and based exposure on paternal dioxin measured in 1987 or 1992 extrapolated to the time of conception of the child. We assigned each child to one of four exposure categories: Comparison and three Ranch Hand categories (Background, Low, High). Children in the High (relative risk = 1.3) and Background (relative risk = 1.4) categories were at increased risk of preterm birth. The risk of intrauterine growth retardation was not increased in any exposure category. The risk of infant death was increased in all Ranch Hand children, with the greatest increases in the High (relative risk = 4.5) and Background (relative risk = 3.2) categories. These patterns indicate that the increases in the relative risk of preterm birth and infant death may not be related to paternal dioxin level.
DNA-based immunizations have been used to analyze the ability of DNA-expressed hemagglutinin (HA) and nucleoprotein (NP) to protect BALB/c mice against a homologous influenza virus, A/PR/8/34 (H1N1), challenge. The HA DNA, but not the NP DNA, protected mice against the lethal viral challenge. For the HA DNA, single gene gun inoculations of 0.04 microg and boosted inoculations of 0.004 microg of DNA raised complete protection. For the NP DNA, boosted gene gun immunizations of 0.4 microg of DNA and boosted intradermal or intramuscular injections of 50 microg of DNA failed to protect. The protection elicited by the HA DNA vaccine correlated with the titers of neutralizing antibody.
OBJECTIVE: To examine the relationship between prenatal magnesium sulfate exposure and the risk for cerebral palsy (CP) or mental retardation (MR) among very low-birth-weight (VLBW; <1500 g) children. Secondarily, to investigate the effect of prenatal magnesium sulfate exposure on VLBW infant mortality. DESIGN: Cohort study with follow-up to 1 year of age; a subset followed up to 3 to 5 years. SETTING: Twenty-nine Georgia counties, including the 5-county Atlanta metropolitan area. PARTICIPANTS: All VLBW births (N=1097) occurring during 2 years (1986-1988); all metropolitan Atlanta VLBW neonates who survived infancy (N=519). MAIN OUTCOME MEASURES: Infant mortality as determined from vital statistics records. Development of CP or MR by 3 to 5 years of age among metropolitan Atlanta VLBW survivors as determined from the Metropolitan Atlanta Developmental Disabilities Surveillance Program. RESULTS: For the entire cohort, there was no association between prenatal magnesium sulfate exposure and infant mortality (adjusted rate ratio, 1.02; 95% confidence interval [CI], 0.83-1.25). Among Atlanta-born survivors, those exposed to magnesium sulfate had a lower prevalence of CP or MR than those not exposed (CP: magnesium sulfate, 0.9%, no magnesium sulfate, 7.7%, crude odds ratio [OR], 0.11, 95% CI, 0.02-0.81; MR: magnesium sulfate, 1.8%, no magnesium sulfate, 5.8%, crude OR, 0.30, 95% CI, 0.07-1.29). Multivariable adjustment had no appreciable effect on the ORs for CP or MR, but the CIs included 1.0. CONCLUSIONS: A reduced risk for CP, and possibly MR, among VLBW children is associated with prenatal magnesium sulfate exposure. The reduced risk for childhood CP or MR does not appear to be due to selective mortality of magnesium sulfate-exposed infants.
PROBLEM/CONDITION: Serious developmental disabilities affect approximately 2% of school-age children and are lifelong conditions that incur substantial financial and societal costs. REPORTING PERIOD: January 1991-December 1991. DESCRIPTION OF SYSTEM: The Metropolitan Atlanta Developmental Disabilities Surveillance Program (MADDSP) monitors the prevalence of four serious developmental disabilities--mental retardation, cerebral palsy, vision impairment, and hearing impairment--among children 3-10 years of age in the five-county metropolitan-Atlanta area. Children who have at least one of the four developmental disabilities are ascertained through annual review of records at schools, hospitals, and other sources. RESULTS AND INTERPRETATION: During 1991, rates for mental retardation varied by age, race, and sex; rates ranged from 5.2 per 1,000 children to 16.6 per 1,000 children. Regardless of the absolute rate of mental retardation in each of the age-, race-, and sex-specific categories, severe mental retardation (i.e., an intelligence quotient of <50) accounted for one third of all cases. The overall crude rate of cerebral palsy was 2.4 per 1,000 children; however, the rate was higher among black children (3.1 per 1,000 children) than among white children (2.0 per 1,000 children). The rate of moderate to severe hearing impairment was 1.1 per 1,000 children, and the rate of vision impairment was 0.8 per 1,000 children. Rates of hearing impairment were higher among black males than among children in the other race and sex groups, whereas rates for vision impairment varied only slightly between these groups. The rates of the developmental disabilities were not adjusted for possible confounding factors (e.g., maternal education, family income, and various medical conditions). Consequently, the variation in rates may reflect social or other characteristics unique to the study population. ACTIONS TAKEN: MADDSP data will be used to direct early childhood intervention efforts to reduce the prevalence of these four developmental disabilities. MADDSP data also are being used to measure progress toward the year 2000 national objectives for the prevention of serious mental retardation.
Identification of surgical delays in an ambulatory care setting is essential for providing consumer-sensitive nursing care. One method of identifying and documenting surgical delays is described. This method, a time-flow study, allowed a freestanding ambulatory surgical center to identify problem areas within its scope of operations, and to provide a factual basis for implementing solutions as part of a continuous quality improvement and cost analysis program.
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Objective assessments of clinical teaching are difficult; in oral surgery students must acquire a wide range of clinical and operative skills before qualifying as independent dental practitioners. A student log book system was introduced in Manchester University to monitor student progress and assess the efficacy of clinical instruction in oral and maxillofacial surgery following the introduction of the new 5-year BDS curriculum. Initial appraisal of third- and fourth year students undertaking 10-week clinical attachments revealed generally high levels of attendance (greater than 80%), and reasonable clinical experience (over 80% of students acquiring regular experience of dentoalveolar surgery and extractions). However, individual variation in both attendance and achievement was wide, and a persistent 8% of students did not appear to gain any practical experience. Students achieving less than the average course performance marks were thus easily identified and measures initiated to identify and correct their difficulties. Log book assessment also facilitated effective audit of teaching staff, clinical sessions and overall course delivery. The use of student log books to record clinical performance is recommended as a versatile aid to clinical teaching practice.