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Biomedical subjects

H Eigen

Publications and source records attributed to H Eigen.

At least 37 records · Page 2Linked to original sources

Longitudinal evaluation of pulmonary function in infants and very young children with cystic fibrosis.

Thirty-two infants with cystic fibrosis (CF) had pulmonary function testing and chest radiographs at the time of diagnosis and on average 1 year later, when they had no acute respiratory symptoms. At diagnosis, 14 of 32 infants had respiratory symptoms (RESP) and 18 did not have respiratory symptoms (NRESP). There were no significant differences in age, weight, or length between the RESP and NRESP groups. At diagnosis, the RESP group had significantly lower forced expiratory flows compared to the NRESP group (41 +/- 32% vs. 98 +/- 48% predicted); however, there were no significant differences in functional residual capacity or chest radiographic scores. Between diagnosis and follow-up, the NRESP group had no significant change in pulmonary function but a decline in chest roentgenographic (CXR) scores (22 +/- 2 to 21 +/- 2). For infants in the RESP group, there were no significant changes in FRC or CXR score. Maximal expiratory flow at functional residual capacity (Vmax FRC) rose from diagnosis to 1 year follow-up (41 +/- 32% to 74 +/- 27% predicted; P < 0.002); however, at follow-up flows for the RESP group remained significantly lower than flows for the NRESP group (74% vs. 113% predicted; P < 0.0005). For the 32 infants with CF, there was significant correlation between percent predicted Vmax FRC at follow-up and at diagnosis (r = 0.47; P < 0.02). Those infants with lower percent predicted flows at diagnosis were more likely to have lower percent predicted flows 1 year later.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

Airway responsiveness in infants following bronchiolitis.

Airway responsiveness to inhaled methacholine was assessed in 18 infants, 4 and 10 months old, following bronchiolitis. Pulmonary function was measured from partial expiratory flow-volume curves generated by the rapid compression technique. Sleeping infants inhaled increasing concentrations of methacholine until maximal expiratory flows at functional residual capacity (VmaxFRC) decreased by 30% or 2.5 mg/mL was inhaled. Airway responsiveness was quantitated by: 1) the threshold concentration (log TC) required to decrease VmaxFRC by 2 standard deviations from baseline; 2) the concentration required to decrease VmaxFRC by 30% (log PC30); and 3) the slope of the dose-response curve between TC and PC30 (log SPC30). At both the first and second evaluation, the bronchiolitic infants had lower baseline VmaxFRC (% pred.) than 24 control infants. In addition, the bronchiolitic infants had heightened airway responsiveness compared to controls, demonstrating lower values for logTC and logPC30 and steeper slopes to their dose-response curves (logSPC30). After accounting for the relationship between airway responsiveness and age, the occurrence of bronchiolitis was found to be a significant independent factor 10 months but not 4 months following bronchiolitis. The bronchiolitic infants did not demonstrate the decline in airway responsiveness with increasing age that occurs in normal infants. We conclude that infants exhibit heightened airway responsiveness following bronchiolitis.

Acute Disease↗

Pediatric pulmonary function testing in asthma.

Pulmonary function testing is an important tool in the management of asthma. Lung function can be readily assessed in both the office and patient's home. This article reviews spirometry, peak flow meters, and bronchial challenge testing. Their interpretation and clinical use are presented as well.

Albuterol↗

Parental response to cystic fibrosis: a contextual analysis of the diagnosis phase.

A contextual framework guided the measurement of specific stressors encountered by parents of children recently diagnosed with cystic fibrosis (CF). Three variables were assessed within the context of the parenting role: illness-specific tasks, normal parenting tasks, and strains in family roles. These situation-specific stressors were contrasted with global measures of parenting stress in their ability to predict depression. Sixty-four parents (36 mothers, 28 fathers) of infants and toddlers recently diagnosed with CF completed a structured interview and standardized measures in the home. Parents reported elevations in both situation-specific and global parenting stress, and a greater number of depressive symptoms than a norm group. Mothers reported significantly greater strain in managing their caregiving role and higher levels of depression than fathers. Controlling for situation-specific parenting stress and marital satisfaction, regression analyses indicated that role strain related to CF was associated with greater depression in mothers, but not fathers. Furthermore, stressors measured contextually rather than globally accounted for substantially greater proportions of the variance in depression. The findings highlight the need to measure ongoing strains specific to the medical condition, and to assess role-related changes.

Adaptation, Psychological↗

Effectiveness of enteric coated pancreatic enzymes given before meals in reducing steatorrhea in children with cystic fibrosis.

In vitro data indicate that length of enzyme incubation with food critically affects enzyme dissolution and presumably effectiveness. This study compared the effectiveness of enteric coated (EC) pancreatic enzymes given before meals with those given during meals (15 minutes after beginning of meal) in reducing steatorrhea in well-nourished children with cystic fibrosis. Eight children (6 years 11 months old to 14 years 7 months old) were studied in the General Clinical Research Center at Indiana University Hospital, Indianapolis. A crossover study design randomly assigned enzymes before or during meals during two consecutive 7-day treatment periods for each child. No difference in fat excretion was documented for the total group when the children who took enzymes before meals were compared with those who took enzymes during meals. However, a stepwise multiple regression analysis of the difference (before minus during) in fat excretion on sex, age, enzyme number, and treatment order documented a positive correlation of age with fat excretion (r = .83). Mean fat excretion in younger children (less than 10 years old) decreased significantly when enzymes were given before meals (7.14 +/- 1.95%) rather than during meals (9.92 +/- 1.61%) (P = .004). The difference in fat excretion (mean = 2.78 +/- 0.55%, range = 1.4% to 4%) translates into a half to one full year's growth potential. Younger children with cystic fibrosis may benefit clinically from taking EC enzymes before meals.

Adolescent↗

Effectiveness and safety of small vs. large doses of enteric coated pancreatic enzymes in reducing steatorrhea in children with cystic fibrosis: a prospective randomized study.

Among cystic fibrosis (CF) centers, usual doses of enteric coated (EC) pancreatic enzymes vary from one to six capsules per meal based upon arbitrary criteria for stool and growth patterns. Large doses of non-EC enzymes are associated with increased serum urate (SU) and urinary uric acid (UUA) but data are unavailable for EC enzymes. This study compared the effectiveness and safety of a relatively large dose (patient's usual dose) versus a small dose (1/4 usual dose) of EC enzymes in nine nourished children with CF, regarding decreasing fecal fat and stool nitrogen losses and maintaining normal SU and UUA concentrations. A crossover study design randomly assigned large or small doses to two consecutive 7 day treatment periods within each child. Large doses of EC enzymes reduced steatorrhea and increased SU and UUA. SU was normal with both treatments and UUA was normal, i.e., 17 of 18 values were between the 10th and 95th percentiles for healthy children eating a normal diet. When fat excretion was greater than 10% with small doses of EC enzymes, large doses resulted in reduced fat excretion and normal UUA. These data suggest that large doses of EC enzymes reduce steatorrhea and are safe in patients who have malabsorbtion with small doses.

Celiac Disease↗

Platelet apheresis for extreme thrombocytosis in an 11-year-old girl with CML.

This report describes the dramatic reduction in the platelet count and ablation of symptoms by consecutive platelet apheresis in a young girl with CML who presented with an extremely high platelet count of 10,320 x 10(9)/l. Extreme thrombocytosis, although quite rare, can be symptomatic and life-threatening in paediatric patients and should be considered an emergency. The mechanical removal of platelets by apheresis is effective therapy and should begin as soon as possible.

Child↗

Assessment of airway responsiveness in infants with cystic fibrosis.

We compared the responses of cystic fibrosis (CF) (N = 14) and normal (N = 14) infants with inhaled methacholine. Airway function was assessed by forced expiratory flows at functional residual capacity (Vmax FRC) generated by the rapid compression technique, and methacholine responsiveness was quantitated as (1) TC: the threshold concentration to decrease Vmax FRC by 2 SD from baseline; (2) PC50: the provocative concentration to decrease Vmax FRC by 30%; and (3) SPC30; the slope of the dose-response curve between TC and PC30. There were no significant differences in age between CF and normal infants (16 +/- 8 versus 17 +/- 5 months, p greater than 0.3); however, the CF infants were shorter (74 +/- 10 versus 81 +/- 5 cm, p less than 0.05), had lower absolute Vmax FRC (241 +/- 103 versus 374 +/- 113 ml/s, p less than 0.001), and tended to have lower percentage of predicted flow values (87 +/- 13 versus 111 +/- 34%, p less than 0.10). Comparison of the indices of airway responsiveness revealed no difference in logTC; however, the CF infants had smaller, more negative values for logPC30 (-0.76 +/- 0.52 versus -0.22 +/- 0.53, p less than 0.02) and steeper slopes to their dose-response curves (logSPC30, 2.42 +/- 0.45 versus 1.88 +/- 0.74, p less than 0.025). Indices of airway responsiveness correlated significantly with baseline Vmax FRC (% of predicted). After the influence of baseline flow upon airway responsiveness was accounted for by multiple linear regression analysis, there was a tendency for CF infants to be more responsive than control infants.(ABSTRACT TRUNCATED AT 250 WORDS)

Bronchial Provocation Tests↗

A double-blind, randomized study of sodium cromoglycate versus placebo in patients with cystic fibrosis and bronchial hyperreactivity.

To evaluate the effects of sodium cromoglycate (SCG) on patients with cystic fibrosis (CF) and with bronchial hyperreactivity, a long-term, double-blind, placebo-controlled, crossover study was performed. Fourteen patients with CF and without asthma (aged 7 to 29 years) and with bronchial hyperreactivity entered the study. Each patient received 8 weeks of 1% SCG nebulizer solution three to four times daily and 8 weeks of placebo. Seven patients received the treatment in the order SCG/placebo and seven patients in the reverse order. Evaluation of SCG effect was performed every 4 to 8 weeks by (1) clinical assessment of symptoms, (2) clinician and patient/parent opinion, (3) pulmonary function tests, and (4) methacholine provocation tests. After two patients were withdrawn for lack of cooperation, the results were evaluated for treatment effect (SCG versus placebo), period effect (whether SCG was administered first or last), or combination of both. No significant difference was found for these parameters for the clinical assessment of symptoms, the patient/parent and clinician opinion, their subjective preferences, the metacholine challenges, or the pulmonary function tests. The study did not demonstrate any benefit from the use of SCG in patients with CF and with bronchial hyperreactivity and does not support the routine use of SCG in patients with CF.

Adolescent↗

Use of maximal expiratory flows to evaluate central airways obstruction in infants.

Partial expiratory flow-volume (PEFV) curves obtained by the rapid compression technique were employed to assess airway function in three infants with three different lesions of the central airways (vascular ring, congenital tracheal stenosis, subglottic polyp). Preoperatively, all three demonstrated severe flow limitation with a relatively constant forced expiratory flow over the tidal volume range. Postoperatively the PEFV curves changed to a normal convex shape, and the maximal expiratory flows at functional residual capacity (VmaxFRC) returned to normal. The PEFV curve adds to our physiologic assessment of the severity of the central airway obstruction in infants and to the efficacy of our therapeutic intervention.

Airway Obstruction↗

Day-night differences in steady-state theophylline pharmacokinetics in asthmatic children.

Potential day-night differences of theophylline absorption and disposition were examined in day-active asthmatic children. Theophylline was given orally as TheoDur tablets and Somophyllin-CRT capsules (random crossover) every 12 hr (0700 and 1900), and patients were studied during two consecutive dosing intervals. In addition, patients were studied during the last 24 hr of a 48-hr continuous, intravenous aminophylline infusion. Serum theophylline concentrations were essentially constant during the intravenous infusion for the day and night periods. Thus, day and night clearances were nearly identical. Following oral administration of Somophyllin-CRT or TheoDur, areas under the serum concentration-time curves were greater during the day than the night, with Somophyllin-CRT yielding greater areas than TheoDur for both dosing intervals. Theophylline was absorbed more rapidly during the day than the night, as evidenced by a time to maximum concentration that occurred earlier in the daytime dosing interval. We conclude that theophylline clearance is not characterized by a circadian rhythm and that absorption of theophylline from Somophyllin-CRT and TheoDur is more rapid and complete during the day than the night.

Administration, Oral↗