Remifentanil for fetal immobilization and analgesia during the ex utero intrapartum treatment procedure under combined spinal-epidural anaesthesia.

Remifentanil undergoes extensive placental transfer and has been used to provide fetal immobilization and anaesthesia for in utero fetal endoscopic interventions. We report three cases of the ex utero intrapartum treatment performed under neuraxial anaesthesia where the maternal administration of remifentanil was used to provide fetal immobilization and analgesia. Fetal pathology included goiter and arthrogryposis, with one case requiring a tracheostomy. The longest time on placental circulation was 21 min. No clinically significant maternal sedation or respiratory depression was observed. In all cases, remifentanil provided adequate fetal immobilization and obviated the need to administer other analgesics or neuromuscular blocking agents. Remifentanil is a useful adjunct for ex utero fetal procedures.

Endemicity and inter-city spread of Burkholderia cepacia genomovar III in cystic fibrosis.

OBJECTIVES: We sought to determine whether the same Burkholderia cepacia complex strain has persisted as the dominant clonal lineage among patients in a large cystic fibrosis (CF) treatment center during the past 2 decades. STUDY DESIGN: The inter-city spread of B cepacia through transfer of a colonized patient and the impact of infection control measures in containing inter-patient transmission were investigated. We analyzed all available B cepacia complex isolates recovered from 1981 to 1987 and from 1996 to 2000 at one large CF treatment center (Center A) and from 1997 to 2000 at another center (Center B). Incidence of B cepacia complex infection and infection control measures in both centers were assessed. RESULTS: Seventeen (81%) of 21 Center A patients from whom B cepacia complex bacteria were recovered between 1981 and 1987 and 40 (97%) of 41 patients culture-positive between 1996 and 2000 were infected with the same genomovar III strain. Transfer of a colonized patient from Center A to Center B was associated with an increase in B cepacia complex infection in Center B, all of which was with the Center A dominant strain. This strain, designated PHDC, lacks both B cepacia epidemic strain and cblA markers. CONCLUSIONS: B cepacia complex strains may remain endemic in CF treatment centers for many years. Responsible bacterial and host factors and optimal infection control measures to prevent inter-patient spread remain to be identified.

Pediatric asthma: "Real world" measures of effectiveness.

In the treatment of pediatric asthma, measuring the effectiveness of an intervention in terms of cost or therapeutic value can be a challenging process. Parameters that should be considered in determining the cost effectiveness of an intervention vary considerably. In clinical therapeutic trials of pediatric asthma, subtle patient selection biases, difficulties in choosing and assessing treatment outcomes in young children, and the highly variable nature of asthma symptomatology can compromise valid measurements of the therapeutic effectiveness of an intervention. Ideally, outcome measurements in asthma should reflect "real world" conditions. They should be as broad-based as possible and include both objective measures of pulmonary function and subjective measures of quality-of-life evaluated over the long term. Patient adherence is thus a critical variable that influences the ultimate effectiveness of treatment. The rate of patient adherence is influenced not only by drug characteristics such as route of administration and dosing frequency, but also by the impact of treatment on a patient's quality of life. Because asthma is a chronic disease with variable symptomatology, clinical trials investigating the cost and therapeutic effectiveness of asthma interventions must evaluate real-world variables in long-term trials. This article will provide information regarding proper assessment of an intervention's cost and therapeutic value.

Stature as a prognostic factor in cystic fibrosis survival.

OBJECTIVE/DESIGN: This study provides a longitudinal analysis of the National Cystic Fibrosis Patient Registry to determine if height-for-age percentile would be a useful predictor of survival. SUBJECTS: All patients were selected from the national registry (n = 19,000) maintained by the Cystic Fibrosis Foundation's 115 accredited Cystic Fibrosis Care Centers in the United States. Inclusion in our analysis required that subjects were born between 1980 and 1989; had a minimum of 4 records each; the subject was alive at age 7; and the subject had a recorded height measurement at age 7 to 8 (n = 2,773). STATISTICAL ANALYSIS: The Cox proportional hazards model was used to compare height-for-age with survival. We recorded whether a subject was less than the 5th National Center for Health Statistics (NCHS) percentile at age 5 and then in a separate analysis at age 7. Cohort effect was coded as "1" if they were born before 1982 and "0" otherwise. RESULTS: Stature is a significant prognostic indicator of survival. The relative hazard associated with height below the 5th NCHS percentile for age was significant for both males and females. In males at age 5 the relative hazard was 2.9, [95% confidence interval (CI) 1.23, 6.91; P < .02] and at age 7 it was 6.3 (95% CI 2.1, 18.8; P < .001). The relative hazard in females at age 5 was 4.3 (95% CI 2.4, 7.3; P < .0001) and at age 7 was 5.8 (95% CI 2.5, 13.1; P < .0001). APPLICATION: These highly significant relative hazard values strongly suggest that shorter patients are much more likely to die before taller patients. The dietetic professional should consider using height-for-age as an effective screening tool to identify patients at risk. Based on these data, short stature should not be considered benign to patients with cystic fibrosis. The CF team, clinicians, family, and patients need work together to maximize linear growth through medical and nutritional intervention.

Inhaled corticosteroids in childhood asthma: growing concerns.

Inhaled corticosteroids (ICS) are an established treatment for asthma in childhood. Recent data bring to light growing concerns that ICS may have significant effects on growth velocity in children. The Food and Drug Administration (FDA) recently convened a joint meeting to review these data, and to release new class labelling for ICS that notes this potential adverse effect. Additional concerns regarding ICS are also discussed, including other potential adverse effects, difficulty of use, noncompliance, and patient and parental concerns with the safety of ICS. The aim of this article is as follows: to describe the rationale for the use of ICS in children with asthma; to delineate the association of ICS with potential growth suppression in children; to discuss recent FDA class labelling for use of ICS in children; to describe other potential long term effects of ICS in children; and to detail compliance issues in children with asthma treated with ICS.

Early feeding after elective open colorectal resections: a prospective randomized trial.

BACKGROUND: A period of starvation after colorectal resections to allow for resolution of the clinical evidence of ileus has been an unchallenged surgical doctrine until recent times. A prospective randomized trial comparing early feeding to traditional management in patients undergoing open elective colorectal resections is reported. METHODS: Patients undergoing elective intraperitoneal colorectal resections without stoma formation were randomized to either an early feeding or control group. The early feeding group were allowed free fluids from 4 h postoperatively progressing to a solid diet from the first postoperative day as they tolerated it. The control group remained nil orally until passage of flatus or bowel motion and were then commenced on fluids progressing to solids over 24-48 h. RESULTS: There were 40 patients in each group well matched for age, sex, type and duration of operation, method of analgesia and mobilization. Thirty-two patients (80%) in the early feeding group tolerated a diet within 48 h. There was no significant difference in the rate of vomiting, nasogastric reinsertion or complications. The early feeding group tolerated a diet, passed flatus, used their bowels, and were discharged from hospital significantly earlier than the control group. CONCLUSION: Early feeding after elective open colorectal resections is successfully tolerated by the majority of patients, leading to earlier resolution of ileus and hospital discharge.

Self-management of cystic fibrosis: short-term outcomes of the Cystic Fibrosis Family Education Program.

This study tested the efficacy of the Cystic Fibrosis Family Education Program, a cystic fibrosis self-management program, on improving participants' knowledge, self-efficacy, self-management behavior, health, and quality of life. A quasi-experimental pretest-posttest nonequivalent comparison group design was employed. Participants made up 104 patient-primary caregiver dyads from the intervention site cystic fibrosis center and 95 from the usual care comparison center. The intervention, a self-paced print curriculum based on social cognitive theory, targeted behavioral capability, self-efficacy, and outcome expectations and was implemented as an integral part of medical care. Parents, early childhood, middle childhood, and adolescents received separate materials on respiratory, nutrition and malabsorption, communication, and coping issues. Significant intervention effects were found on the knowledge scores for caregivers, adolescents, and children; caregiver and adolescent total self-management scores; Child Behavior Checklist total score; one parent coping scale score; the modified NIH score; NIH pulmonary factor 1; and the Brasfield total score. Significant interaction effects were evident in the self-efficacy scores for caregivers and children.

Effect of vitamin K1 supplementation on vitamin K status in cystic fibrosis patients.

BACKGROUND: Patients with cystic fibrosis are at risk for impaired vitamin K status due to fat malabsorption from pancreatic insufficiency. This study was designed to assess vitamin K status and measure the effect of vitamin K1 supplementation in cystic fibrosis patients. METHODS: Eighteen outpatients participated in a crossover study to determine the effect of vitamin K1 (phylloquinone) supplementation. After obtaining initial data, each subject was randomly assigned to either a 4-week study treatment of 5 mg oral vitamin K1 supplementation per week, or no supplementation and then crossed over to the other treatment for a second 4 week period. Plasma, serum and urine samples were collected and analyzed pre-study and at the end of each study period. RESULTS: The mean concentration of plasma vitamin K1 for the supplemented group was significantly higher than the unsupplemented group, [0.34 nmol/L and 0.21 nmol/L, respectively (p < 0.05)]. The percent of undercarboxylated osteocalcin increased on supplementation from 17% to 31%, (p < 0.005). Prothrombin induced in vitamin K absence (PIVKA-II) increased on supplementation from 5 ng/mL to 22 ng/mL, (p < 0.005). The ratio of urinary gamma-carboxyglutamic acid/creatinine was similar for both study periods. CONCLUSIONS: In contrast to other studies in cystic fibrosis, this study demonstrated a need for vitamin K1 supplementation. The carboxylation state of osteocalcin and PIVKA-II were the most sensitive indices of changes in vitamin K1 status. Although the 5 mg vitamin K1/week dose improved these vitamin K parameters, normal levels were not achieved.

A comparison of bronchial drainage treatments in cystic fibrosis.

We compared standard chest physical therapy and postural drainage (CPT/PD) with a new airway clearance therapy called high-frequency chest wall oscillation (HFCWO) in a group of stable cystic fibrosis (CF) patients. In this crossover trial, 29 CF patients (15 males, 14 females), aged 7-47 years that met the inclusion criteria were randomly assigned to alternate CPT/PD and HFCWO, on a daily basis, over a 4 day period. Each patient received 2 days of each form of therapy; treatment frequency and the length of treatment were the same for both techniques. Expectorated secretions were collected during each 30 minute therapy session and for 15 minutes following treatment. The wet and dry weights of collected secretions were determined gravimetrically, and the therapy methods were compared. Significantly more sputum was expectorated during HFCWO than during CPT/PD as determined by both the wet (P < 0.001) and the dry (P < 0.01) measurements. This study suggests that HFCWO is at least as effective as manual CPT/PD in clearing secretions from the airways in patients with cystic fibrosis.

Early experience with postoperative adjuvant chemoradiation for rectal carcinoma: focus on morbidity.

The morbidity of postoperative adjuvant chemoradiation for primary extraperitoneal rectal carcinoma is documented in this ongoing study. Patients who presented electively for resection with ACPS Dukes' B and C extraperitoneal rectal carcinoma during the period January 1990 to June 1993 were studied. Twenty patients received postoperative adjuvant chemoradiation. At a mean follow up of 32.6 months (s.d. 7.1), three of the 20 patients who received combined adjuvant chemoradiation reported no side effects. In 10 patients (50%) complications were classified as minor. In the remaining seven patients (35%) major complications of therapy occurred. There were no deaths. These early results highlight the morbidity of postoperative adjuvant chemoradiation that has been presumed but not documented.

Comparison of weight-based dosages of enteric-coated microtablet enzyme preparations in patients with cystic fibrosis.

Twenty-one stable hospitalized cystic fibrosis patients with malabsorption syndrome participated in an open-label crossover clinical trial to evaluate the efficacy of two-period dosing regimens of a pancreatic microtablet enzyme preparation in the treatment of steatorrhea. Standard dosing consisted of 500 U lipase/kg body weight/meal, 250 U lipase/kg body weight/snack; high dosing consisted of 1,500 U lipase/kg body weight/meal, 750 U lipase/kg body weight/snack. Doses were determined by units of lipase/kg body weight to provide dosing consistency among patients of varying size. Each patient was on a regular diet of approximately 100 g of fat per day. Two separate, 72-h stool collections were performed between markers. A significant difference in mean percentage fat absorbed between the standard dose and the high dose was found (86% versus 91%, p < 0.05). Subjects were then stratified into two groups, based on the grams of fecal fat eliminated (GFFE) as follows: Group 1 with < or = 7 GFFE/24 h on both dosages (n = 7) and Group 2 with > 7 GFFE/24 h on either dose (n = 14). A significant difference (p < 0.05) between Group 1 (96%) and Group 2 (88%) was noted in the percentage fat absorbed while on the high dose. Fat absorption improved from 81% to 88%, (p < 0.05) in Group 2. During the study period, the adverse reactions of constipation or elevated serum uric acid levels were not observed. The increased doses of pancreatic enzymes resulted in improved correction of steatorrhea.

Home apnea monitoring and risk factors for poor family functioning.

As part of a comprehensive study on the impact of home apnea monitoring on family functioning, we undertook an in-depth analysis of risk factors for poor family functioning in 93 families of monitored infants. A multistep correlation and regression analysis was used to examine the ability of 16 demographic, infant, family, and monitor-related variables to predict poor functioning in each of 12 different aspects of family life. Of note was the fact that monitor-related variables failed to be important predictors of poor family functioning. Rather, previous family problems and low satisfaction with social support were the most broad and powerful predictors of poor functioning. To enhance the capabilities of families to manage the added responsibility of home monitoring, clinicians might wisely assess both of these aspects of family life and then target extra support services to families identified to be at risk in these areas.

Use of home apnea monitors.

OBJECTIVE: To examine the frequency of use of home apnea monitors, reasons for not using them, and factors associated with their use among families of infants for whom home monitoring had been prescribed. DESIGN: Cross-sectional study, including a telephone interview to collect demographic data and a mailed questionnaire to obtain data on monitor use. SETTING: The apnea clinics in two tertiary-care centers. PARTICIPANTS: Ninety-three families (representing an 80.9% response rate) with infants considered at increased risk of sudden infant death syndrome and requiring home monitors. Infants with tracheostomies or bronchopulmonary dysplasia and families with monitored twins, a mother known to be drug addicted, or no home telephone were excluded. RESULTS: Of concern were that 23.1% of mothers reported using the monitor 12 or fewer hours per day and that 10.8% believed their infants did not need a monitor. Of 11 variables examined, only color change in the infant was associated with frequency of monitor use. CONCLUSIONS: Clear, consistent communication with families regarding the use of apnea monitors is essential to improve compliance with proper monitoring techniques.

Bronchoalveolar lavage for pneumocystis pneumonia in HIV-infected children.

Bronchoalveolar lavage (BAL) by flexible fiberoptic bronchoscopy is useful in the diagnosis of Pneumocystis carinii pneumonia (PCP) in adults with acquired immunodeficiency syndrome. To evaluate the safety and efficacy of this procedure in children with human immunodeficiency virus in whom PCP was considered, we reviewed the records of 15 consecutive procedures performed on eight patients by a pediatric pulmonologist during a 19-month period. Pneumocystis carinii pneumonia was identified after five of 15 BAL procedures. Other pathogens or multiple pathogens were found in some cases. A specific infectious diagnosis was obtained in ten of 15 procedures. No patient required subsequent open lung biopsy. Follow-up for a minimum of 6 weeks and response to therapy did not suggest PCP in any case where BAL failed to establish its diagnosis. No major complication was attributed to BAL. We conclude that BAL is safe and effective in the diagnosis of PCP in children with HIV infection. Guidelines are suggested to optimize its safety and utility.

Serum mucin-associated antigen levels of cystic fibrosis patients are related to their ages and clinical statuses.

Mucin levels are generally elevated in sera from many cystic fibrosis (CF) patients as measured by radioimmunoassay using monoclonal antibody 19-9, which is directed against the mucin-associated sialyl Lea antigen. Antibody 19-9 can only be used to measure mucin-associated antigen levels in those patients who are genetically able to make detectable levels of mucin-associated sialyl Lea epitope. Serial studies of 20 patients followed over 3-5 y showed that their serum mucin-associated antigen levels varied directly with respect to the severity of their disease and inversely with their Shwachman-Kulczycki clinical scores (p less than 0.001) and Brasfield chest roentgenographic scores (p less than 0.02). Serum mucin-associated antigen levels in samples from 89 CF patients were generally higher in the older patients (p less than 0.025). Serum mucin-associated antigen levels of CF patients who were colonized with Pseudomonas aeruginosa did not significantly differ from those of uninfected CF patients. The mean serum mucin-associated antigen level of CF patients colonized with Pseudomonas was higher than the mean mucin level of six non-CF bronchiectatic patients whose lungs were colonized with Pseudomonas (p = 0.053). Serum mucin-associated antigen levels are thus related to CF patients' ages and clinical statuses.

Intravenous immunoglobulin treatment of respiratory syncytial virus infections in infants and young children.

Respiratory syncytial virus (RSV)-infected cotton rats (Sigmadon hispidus) and owl monkeys (Aotus trivirgatus) showed significant reductions in RSV shedding from their respiratory tracts following parenteral therapy with human intravenous immunoglobulin (IVIG) containing high titers of RSV-neutralizing antibody. Because this therapy was well tolerated and appeared safe, a double-blind, placebo-controlled IVIG immunotherapy pilot study was performed on 35 hospitalized, RSV-infected infants and children. The treatment was well tolerated and resulted in significant reductions in nasal RSV shedding and in improvements in transcutaneous oximetry readings. However, the mean duration of hospitalization was not reduced by IVIG treatment. Followup to date has revealed no harmful effects resulting from immunotherapy of RSV infections. These studies appear to refute the hypothesis that passively acquired antibody may exacerbate RSV bronchiolitis or pneumonia in infants. Studies with larger numbers of seriously ill children will be required to determine if immunoglobulin G immunotherapy of RSV infections in infants is of clinical value.