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Biomedical subjects

H Levison

Publications and source records attributed to H Levison.

At least 109 records · Page 6Linked to original sources

Malnutrition: a cause of elevated sweat chloride concentration.

In order to document the frequency and causes of elevated sweat chlorides we reviewed all sweat chloride determinations performed over a 2-year period. Seven hundred and thirty-five quantitative tests were performed. Three hundred and three positive or borderline results were obtained from 133 patients. Thirty-three of these patients did not have clinical evidence of CF. Fourteen of them (42%) suffered from malnutrition or growth stunting when tested. The remaining patients who were normally nourished, represented a very heterogeneous group with no unifying diagnosis. Initial sweat chlorides in 11/14 malnourished children were in the abnormal range (greater than 60 mmol/l). These tended to revert to normal coincident with improvement in nutritional status. Less remarkable elevations in sweat chloride levels were noted in the normally nourished patients, since almost 50% (9/19) were in the intermediate range (50-60 mmol/l). We conclude that malnutrition can be the cause of elevated sweat chlorides, which may lead to an erroneous diagnosis of cystic fibrosis. Clinicians should be aware of this association and adhere to strict criteria for the diagnosis of cystic fibrosis. Also, repeated sweat chloride determinations should be obtained, preferably following re-nutrition.

Adolescent↗

Effects of nutritional status on exercise performance in advanced cystic fibrosis.

Initial evaluation of 22 patients with cystic fibrosis (CF) on entry into a trial of home oxygen therapy was used to elucidate the possible effects of poor nutritional status on exercise performance in CF. The patients had advanced lung disease (mean FEV1, 36 percent predicted) and all had a stable resting PaO2 less than or equal to 65 mm Hg. Nutritional status was determined by calculating weight as a percentage of ideal for height (Wt/Ht) for each subject. Exercise testing consisted of a progressive exercise test on a cycle ergometer to measure maximum work capacity (Wmax), and a steady state test at 50 percent of baseline Wmax. During the steady state test, cardiac output (Q) and stroke volume (SV) were computed by the indirect Fick (CO2) method. Wmax, SV, Q and lung function results are expressed as percent predicted. Mean (+/- SD) Wmax was 58 +/- 15 percent predicted. Wmax correlated with both FEV1 and Wt/Ht, but FEV1 and Wt/Ht were not related. During steady state exercise, 12 of 22 patients had a SV less than 80 percent predicted. SV correlated with Wt/Ht, but not with lung function. Thirteen of the 22 patients had a Wt/Ht less than or equal to 90 percent and were considered malnourished. When compared with the well-nourished patients (Wt/Ht greater than 90%), these malnourished subjects had significantly lower mean values for Wmax%, SV% and Q% predicted, but not for lung function parameters. We conclude that: in patients with CF and advanced lung disease, nutritional status plays a significant role in determining exercise capacity; lower exercise tolerance of malnourished patients is an independent effect, as nutritional status and lung function were not related; and malnourished patients with CF have an altered cardiac performance on exercise testing which is due to a reduced SV rather than an impaired heart rate response.

Adolescent↗

Methacholine inhalational challenge in the evaluation of chronic cough in children.

The medical records of 58 children (age range, 7 to 16 years) who presented with chronic cough were retrospectively analyzed to determine the value of methacholine (MCH) bronchial challenge in reaching a specific diagnosis. Baseline lung function tests were normal in all subjects, apart from an elevated residual volume/total lung capacity ratio (RV/TLC) in 11 patients, and a reduced midexpiratory flow rate (FEF25-75) in five of these patients. Thirty-one patients had a positive response to MCH, but these patients could not be identified on the basis of clinical criteria or spirometric parameters. MCH-positive patients did have a significantly higher RV/TLC than did MCH-negative patients. Twenty-seven of the 31 MCH-positive patients responded to a trial of bronchodilator therapy (albuterol/theophylline), confirming the diagnosis of cough variant asthma in these patients. We conclude that children with chronic cough should be considered for methacholine challenge in order to identify patients who are likely to benefit from specific bronchodilator therapy.

Adolescent↗

Psychologic adjustment of the family with a member who has cystic fibrosis.

Seventy-eight percent of the 384 patients 2 years of age and older who were enrolled in the Cystic Fibrosis Clinic at the Hospital for Sick Children participated in this study of family adjustment to cystic fibrosis. Residence with at least one parent at the time of the survey was a condition for inclusion in the study. The effect of the disease on the family was assessed using three questionnaires: an inventory of family problems as perceived by the parents and two standardized measures of family interaction. Measures of behavior and self-concept in each patient and sibling, physical disease parameters for each patient, and demographic characteristics of the families were examined and analyzed in relation to the responses to the questionnaires on family interaction. Families of patients with cystic fibrosis were found to function very well, with normal mean scores on both standardized profiles. Children with cystic fibrosis showed more behavior problems than expected from the test norms, and healthy siblings of children with cystic fibrosis had behavior problem scores midway between those with the disease and norm scores. Measures of self-concept were remarkably similar for patients and siblings and showed levels at or above those for children and young adults in the general population. Parents psychosocial scores were influenced by the patients' physical parameters, but the severity of the disease generally was unrelated to psychosocial scores of patients and siblings. A notable exception was the correlation of emotional disturbance with amount of hospitalization in patients older than 12 years of age.

Adaptation, Psychological↗

Evaluation of serum theophylline concentrations following administration of sustained-release beads in applesauce to asthmatic preschool children.

A sustained-release theophylline preparation (Theo-Dur Sprinkle) was evaluated in young asthmatic patients aged 1 to 6 years and receiving a daily dose of 23.4 +/- 2.0 mg/kg (mean +/- SD) to determine, on the basis of serial serum concentrations obtained over a 12-hour dosing interval at steady state, the suitability of such a product in patients likely to metabolize the drug very rapidly. Peak theophylline concentrations of 15.1 +/- 4.1 mg/L were achieved 5.5 +/- 1.5 hours after dosing. The mean maximum to minimum concentration difference was 6.9 +/- 2.2 mg/L for the dosing interval studied. Fluctuations in theophylline concentration less than 100% were achieved in nine of the 12 study patients. Use of the "sprinkle-technique" with Theo-Dur Sprinkle appears to be a simple and effective method of maintaining acceptable fluctuations in serum theophylline concentrations in preschool asthmatic children.

Asthma↗

Benefit of intravenous IgG replacement in hypogammaglobulinemic patients with chronic sinopulmonary disease.

Seven patients with hypogammaglobulinemia and chronic sinopulmonary infections were treated with a preparation of intravenous gammaglobulin. In order to maintain levels of serum IgG at greater than 500 to 750 mg/dl four weeks after infusion, 0.6 g/kg was administered every month. Stable serum levels were achieved after three to eight months. After six to 12 months of this regimen, there was significant reduction in acute infections requiring hospitalization, amelioration of clinical and radiographic evidence of chronic maxillary sinusitis, and improvement in pulmonary symptoms and pulmonary function test results. The administration of increased amounts of IgG intravenously is of benefit in patients with chronic sinopulmonary infections.

Adolescent↗

Family adaptation and psychosocial adjustment to cystic fibrosis in the preschool child.

The parents of 80% (41 of 51) of preschoolers with cystic fibrosis (CF) diagnosed at least 1 year prior to the study and attending the Hospital for Sick Children (HSC) CF Clinic completed the Problem Inventory (PINV), Preschool Behavior Questionnaire (PBQ) and Family Assessment Measure (FAM). The mean age of the CF children was 3.7 years. Parents of a control group of 31 healthy daycare children with a mean age of 3.6 years completed the same questionnaires. Parents of healthy preschoolers reported more child-related problems for 2-5 year olds than did parents of CF children (P less than 0.001) suggesting that parents who have confronted the CF diagnosis go on to minimize the normal stresses of the developmental period. Considerable agreement was seen between PINV scores for mothers and fathers in each group, revealing that parents in a given family perceive similarly the impact their child has upon them. The mean PBQ for CF preschoolers was not significantly different from that of the control group, although there was some tendency toward hostile aggressive behavior in the CF group. Surprisingly, total FAM scores of all samples showed no significant differences with the exception of a better total FAM score for fathers of CF children when compared to control fathers revealing that the CF family is not, during the early years of relative health stability, adversely affected. Two subscales were significantly elevated, social desirability (for CF mothers and fathers) and denial (for CF mothers only), describing an important response style which may enhance mastery of long-term stress.(ABSTRACT TRUNCATED AT 250 WORDS)

Adaptation, Psychological↗

Comparison of cold air, ultrasonic mist, and methacholine inhalations as tests of bronchial reactivity in normal and asthmatic children.

The sensitivity and specificity of cold air, ultrasonically nebulized distilled water mist (USM), and standard methacholine (MCH) challenges were studied in 21 children with asthma (mean age 11.5 years) and 12 normal children (mean age 14.2 years). The cold air challenge consisted of successive 3-minute periods of hyperventilation during which incremental volumes of subfreezing air (mean temperature -16 degrees C) were inhaled. To perform the USM challenge, subjects inhaled increasingly larger volumes of nebulized distilled water while breathing tidally. The specificity of both nonpharmacologic challenges was found to be 100%, whereas that of MCH was only 83%. The sensitivity of the cold air and USM tests was 57% and 71%, respectively, compared with 95% obtained with MCH challenge. We conclude that cold air and USM challenges are promising alternatives to the MCH challenge, and may be superior to it if optimal standard testing protocols are defined.

Adolescent↗

The immotile cilia syndrome: radiological manifestations.

The immotile cilia syndrome (ICS) is an uncommon disorder characterized by specific and genetically determined defects of cilia that cause upper and lower respiratory disease. We reviewed the radiographic patterns in 30 patients who had ICS (15 females, 15 males) and ranged in age from newborn to 26 years. Except for two neonates, sinusitis and otitis were present in all patients. Chest radiographic abnormalities, universally present, included bronchial wall thickening, hyper-inflation, segmental atelectasis or consolidation, and segmental bronchiectasis. Situs inversus, present in 50% (7 females, 8 males), was not an essential part of this disorder. Radiologically, the disease progresses from bronchial wall thickening with or without hyperinflation, to increasing hyperinflation plus parenchymal changes including segmental atelectasis, consolidation, and bronchiectasis. There is also a predilection for anatomic middle lobe abnormalities. The radiological appearance and clinical state have similarities to cystic fibrosis, although they are less severe and less progressive. ICS should be considered in the differential diagnosis of slowly progressive chronic lung disease, sinusitis, and otitis.

Adolescent↗

Maximal inspiratory and expiratory pressures are reduced in hyperinflated, malnourished, young adult male patients with cystic fibrosis.

We measured maximal inspiratory and expiratory pressures (MIP and MEP, respectively) in 23 male patients with cystic fibrosis (CF), 16 to 35 yr of age (22.1 +/- 3.7), and in a control group of 33 male volunteers, 17 to 39 yr of age (22.5 +/- 6.8), to evaluate the effects of chronic hyperinflation and malnutrition on MIP and MEP in the patients with CF. Routine pulmonary function tests and skeletal muscle indexes, such as the force generated by the adductor pollicis muscle with supramaximal ulnar nerve stimulation at a frequency of 10 Hz as percentage of force at 100 Hz (F10/100) and midarm muscle circumference as a percentage of predicted (MAMC), were also measured in the patients with CF. Severe hyperinflation in this study was defined as a ratio of residual volume to total lung capacity above 50% and malnutrition as a ratio of actual weight to the ideal weight for the patient's age and height of 90% or less. The severely hyperinflated subgroup of patients with CF had significantly reduced MIP values in comparison with those in the other patients with CF. The malnourished subgroup, which was also severely hyperinflated, differed from the well-nourished one in both skeletal muscle indexes; MAMC was reduced, whereas F10/100 was elevated, and respiratory muscle pressure generation, MIP, and MEP were reduced. We conclude that patients with CF who are malnourished and/or severely hyperinflated have reduced maximal respiratory pressures.

Adolescent↗