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Biomedical subjects

E Kerem

Publications and source records attributed to E Kerem.

At least 73 records · Page 4Linked to original sources
PubMed1992

Controlled trial of nebulized albuterol in children younger than 2 years of age with acute asthma.

To determine the response to nebulized beta 2 agonist, 28 children younger than 2 years of age who visited the emergency department during an episode of acute asthma were studied. Each subject had a previous history of recurrent wheezing episodes. They were randomly assigned to receive two administrations of either nebulized albuterol (0.15 mg/kg per dose) or placebo (normal saline) with oxygen, 1 hour apart. After two nebulizations, the albuterol-treated patients had a greater improvement in clinical status (respiratory rate, degree of wheezing and accessory muscle use, total clinical score, and arterial oxygen saturation) than the placebo group. None of the patients in the albuterol group experienced a decrease of arterial oxygen saturation of greater than or equal to 2%. It is concluded that a trial of nebulized beta 2 agonists is warranted in the treatment of acute asthma in infants and young children.

Acute Disease↗
PubMed1991

Bronchopulmonary dysplasia: improvement in lung function between 7 and 10 years of age.

To evaluate the natural history of bronchopulmonary dysplasia, we studied the same 32 patients at a mean age of 7 and 10 years. The group as a whole had normal height and weight percentiles, and each child grew along his or her established somatic growth curve. Although some children had abnormal values, the group maintained a normal mean total lung capacity and functional residual capacity. The mean residual volume and the residual volume/total lung capacity ratios were elevated at both ages. At age 7 years the 19 patients (59%) who had a forced expiratory volume in 1 second (FEV1) of less than 80% had "catch up" improvement by 10 years of age (65 +/- 11% to 72 +/- 16% of predicted value; p less than 0.05). All the children who had a normal FEV1 at 7 years of age continued to have a normal FEV1 at age 10 years. Resting single-breath carbon monoxide uptake by the lung was normal when measured at age 10 years. The majority of patients had a positive methacholine challenge test result at both ages, although there was a low incidence of clinically diagnosed asthma. This study demonstrates that patients with bronchopulmonary dysplasia who have normal lung function at age 7 have had normal lung growth and that those with evidence of mild to moderate lung disease have continued lung growth or repair, or both, during their school years.

Bronchopulmonary Dysplasia↗
PubMed1991

Clinical-physiologic correlations in acute asthma of childhood.

Seventy-one patients who presented to the emergency room with acute asthma were evaluated to determine the relationship between common clinical signs and spirometric and transcutaneous arterial oxygen saturation (SaO2) measurements. Prior to treatment, a physical examination was performed, a clinical score assigned, and pulmonary function and SaO2 were measured. Although forced expiratory volume in 1 second (FEV1) and SaO2 had strong correlation with the overall clinical score (r2 = .47, .49 respectively), many patients with low clinical scores and apparent mild clinical disease had low FEV1 values (as low as 20% predicted). Of the individual components of the clinical score (ie, heart rate, respiratory rate, pulsus paradoxus, accessory muscle use, dyspnea, and wheezing), the degree of accessory muscle use correlated most closely with lung function followed by the degree of dyspnea and wheezing. Similarly, the degree of accessory muscle use correlated most closely with SaO2 followed by dyspnea and respiratory rate. Significant correlation (r2 = .59) was found between SaO2 and FEV1, although the range of SaO2 value for a given FEV1 was wide and some patients with low FEV1 values had normal SaO2 values. These results show that although clinically apparent severe disease and hypoxemia were always associated with low FEV1, their absence does not exclude the presence of airflow obstruction. It is concluded that for the optimal evaluation of acute asthma in children in the emergency room, clinical evaluation should be used in conjunction with objective laboratory measurements.

Acute Disease↗
PubMed1990

The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508).

BACKGROUND AND METHODS: Both the clinical manifestations of cystic fibrosis and the genotypes of patients are heterogeneous, but the associations between the two are not known. We therefore studied blood samples from 293 patients with cystic fibrosis for the presence of the most common disease-causing mutation (delta F508) on chromosome 7 and compared the results with the clinical manifestations of the disease. RESULTS: The prevalence of the delta F508 allele in the cohort was 71 percent; 52 percent of the patients were homozygous for the mutation, 40 percent were heterozygous, and 8 percent had other, undefined mutations. The patients who were homozygous for the mutation had received a diagnosis of cystic fibrosis at an earlier age and had a greater frequency of pancreatic insufficiency; pancreatic insufficiency was present in 99 percent of the homozygous patients, but in 72 percent of the heterozygous patients and only 36 percent of the patients with other genotypes. The patients with pancreatic insufficiency in all three genotype groups had similar clinical characteristics, reflected by an early age at diagnosis, similar sweat chloride values at diagnosis, similar severity of pulmonary disease, and similar percentiles for weight. In contrast, the patients in the heterozygous-genotype and other-genotype groups who did not have pancreatic insufficiency were older and had milder disease. They had lower sweat chloride values at diagnosis, normal nutritional status, and better pulmonary function after adjustment for age. CONCLUSIONS: The variable clinical course in patients with cystic fibrosis can be attributed at least in part to specific genotypes at the locus of the cystic fibrosis gene.

Adolescent↗
PubMed1990

Effect of endotracheal suctioning on arterial blood gases in children.

The occurrence of hypoxia during endotracheal tube suctioning and its prevention was examined in 25 hemodynamically stable and non-cyanosed pediatric patients. In each patient 4 suction and treatment protocols were studied: 1. pre- and post-suction arterial blood gases (ABG) with no treatment (control). 2. ABG with pre-suction oxygenation. 3. ABG with presuction hyperinflation. 4. ABG with postsuction hyperinflation. With no presuction treatment the PO2 and saturation fell significantly after suctioning from control level of 116.6 +/- 9.4 mmHg to 93 +/- 9.3 mmHg post-suction and 97.2 +/- 0.4% to 92.8 +/- 1.4% (p less than 0.001) respectively. In 6 patients with low but adequate pre-suction PO2, hypoxic levels (PO2 less than 60 mmHg) were found post-suction. The significant fall in PO2 was completely prevented by pre-suction oxygenation. Post-suction hyperinflation induced a rapid return of the PO2 to control levels. These results suggest that severe hypoxia might occur during endotracheal suctioning and can be prevented by pre-oxygenation. We recommend 1 min oxygenation with FiO2 1.0 prior to suctioning procedures and intermittent hyperinflation with 100% oxygen during repeated endotracheal suction passes to prevent hypoxia, especially in children in respiratory failure who already have low or borderline pre-suction PO2.

Blood Gas Analysis↗
PubMed1990

Nebulized albuterol in acute bronchiolitis.

In a double-blind, placebo-controlled trial, 40 infants between 6 weeks and 24 months of age who had a first episode of wheezing and other signs and symptoms of bronchiolitis were randomly assigned to receive either nebulized albuterol (0.15 mg/kg/dose) or placebo (saline solution) for two administrations 1 hour apart. The albuterol therapy resulted in a significantly greater improvement in the accessory muscle score (decreases 0.70 vs decreases 0.30; p = 0.03), oxygen saturation (increases 0.71% vs decreases 0.47%; p = 0.01) after one dose, and in the accessory muscle score (decreases 0.86 vs decreases 0.37; p = 0.02), respiratory rate (decreases 19.6% vs decreases 8.0%; p = 0.016), and oxygen saturation (increases 0.76% vs decreases 0.79%; p = 0.015) after two doses of the drug. The response to therapy was similar in infants younger and those older than 6 months of age. The heart rate rose slightly more in the albuterol group (increases 7.76 from baseline) versus the placebo group (decreases 6.79). There were no other side effects of the treatment. Of the 34 children from whom nasal specimens were obtained by swab for viral identification, 24 had positive test results (21 for respiratory syncytial virus, 1 for parainfluenza, 1 for paramyxovirus, and 1 for influenza A). We conclude that nebulized albuterol constitutes a safe and effective treatment of infants with bronchiolitis.

Acute Disease↗
PubMed1990

Renal calcium handling in cystic fibrosis: lack of evidence for a primary renal defect.

Because cystic fibrosis (CF) epithelia have ion transport abnormalities that may in part be regulated by intracellular calcium metabolism, and the kidney is actively involved in both ion transport and calcium homeostasis, we have investigated renal calcium handling in CF. Twenty-four-hour urine collections were analyzed in 34 CF patients (age 5 to 35 years) and kidney ultrasound studies were performed in 17 CF patients (age 6 months to 23 years). Renal histologic findings at postmortem examination of 14 CF patients (age 4 months to 23 years) were compared with those of 12 patients (age 11 months to 17 years) with other chronic illnesses (6 congenital heart disease, 6 malignancy). In 30 of the 34 CF patients urinary calcium excretion was normal (less than 4 mg (0.1 mmol)/kg/24 hr). Four CF patients had hypercalciuria (calcium excretion 4.4 to 8.8 mg (0.11 to 0.22 mmol)/kg/24 hr). However, these patients had other possible explanations for hypercalciuria, such as immobilization (n = 2), increased dietary sodium load (n = 1), and glucocorticoid therapy (n = 1). None of the 17 patients examined by renal ultrasonography had nephrocalcinosis. Five CF patients had histologic evidence of sparse nephrocalcinosis at autopsy. However, 6 of 12 autopsy kidney specimens from patients with other chronic illnesses and similar preterminal events also showed nephrocalcinosis. The hypercalciuria and nephrocalcinosis in CF and other chronic debilitating diseases may be explained by factors known to affect calcium handling. Our evidence does not support a primary renal defect as the basis of hypercalciuria and nephrocalcinosis in CF.

Adolescent↗
PubMed1990

Sequential pulmonary function measurements during treatment of infantile chronic interstitial pneumonitis.

Three infants with histologically confirmed chronic interstitial pneumonitis were treated with monthly intravenously administered high doses of methylprednisolone with or without daily hydroxychloroquine therapy. We applied the multiple occlusion technique to measure the static respiratory system compliance, and the end-inspiratory occlusion technique to measure passive respiratory system compliance, resistance, and time constant. When assessed by clinical criteria and pulmonary function measurements, all three patients showed improvement with this treatment. Clinical improvement was associated with an increase in respiratory system compliance as measured by both techniques (60% to 100% increase in all patients). The passive respiratory resistance and the time constant did not closely reflect the clinical course. We conclude (1) that high doses (pulses) of methylprednisolone and daily oral doses of hydroxychloroquine are effective in the treatment of infantile chronic interstitial pneumonitis and (2) that the respiratory system compliance, measured by both pulmonary function techniques, correlates well with the response to treatment and change in clinical status.

Airway Resistance↗
PubMed1990

Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa.

To evaluate the relationship between Pseudomonas aeruginosa colonization and the development of lung disease, we studied 895 patients who attended our cystic fibrosis clinic between 1975 and 1988. The prevalence of P. aeruginosa colonization was 82%. Patients who acquired P. aeruginosa in the first year of life had a similar 10-year survival rate (85%) to that in patients who were colonized between the ages of 1 and 7 years (87%), and to that in patients colonized after the age of 7 years (78%). One year before colonization, mean age, forced expiratory volume in 1 second (FEV1), forced vital capacity, and forced expiratory flow in the mid-expiratory phase were similar to those in a group of patients who remained free of P. aeruginosa. No significant change in pulmonary function variables could be demonstrated 1 year and 2 years after the colonization. The rate and duration of hospitalization did not increase in the years after P. aeruginosa colonization compared with the years before colonization. By the age of 7 years, the mean percentage of predicted FEV1 was lower by 10% in patients who were already colonized by P. aeruginosa compared with those who were not colonized (p less than 0.01). A similar reduction in FEV1 was observed at all ages from 7 to 35 years, but no precipitate rate of decline in FEV1 could be associated with P. aeruginosa colonization. We conclude that although P. aeruginosa colonization is associated with 10% lower lung function, it does not cause an immediate and rapid reduction, as has been previously reported. The clinical course and the pulmonary deterioration in cystic fibrosis after P. aeruginosa colonization is a gradual and variable process.

Adolescent↗
PubMed1990

Disappearance of IgM antibodies to hepatitis A virus after an acute infection in children and adolescents.

The kinetics of IgM antibodies to hepatitis A virus (HAV) following an acute infection, were studied in 17 children. Antibodies disappeared in two patterns, one group at 113 +/- 18 days after the acute infection in the children, and the second group at 283 +/- 90 days. The same two patterns of kinetics were seen in adults. We conclude that IgM anti-HAV antibodies can be found in the sera of children for over 6 months after the acute infection, as was observed in adults.

Acute Disease↗
PubMed1990

Risk factors for Pseudomonas aeruginosa colonization in cystic fibrosis patients.

In an attempt to study the association between Pseudomonas aeruginosa colonization and clinical factors present before colonization, we studied 502 patients who attended our cystic fibrosis (CF) clinic between 1975 and 1988 and who were not colonized with P. aeruginosa before the study period. Twelve percent of the patients became colonized with P. aeruginosa before the age of 1 year and 44% by the age of 7 years. In a birth cohort followed from diagnosis during the study period, 23% were colonized with P. aeruginosa before age 1 year and 67% before age 7 years. Early P. aeruginosa colonization was associated with early diagnosis of CF. Presence of meconium ileus, gastrointestinal symptoms and pancreatic insufficiency at the time of diagnosis was also associated with early colonization. There was no association between the presence of respiratory symptoms at the time of diagnosis and age at first P. aeruginosa colonization. In families in which more than one sibling had CF, the age of P. aeruginosa acquisition was similar in the first colonized and the subsequently colonized siblings. In the year before the colonization patients with P. aeruginosa who were older than 1 year had higher rates of hospitalization and spent more days in hospital compared with patients without P. aeruginosa. In conclusion most P. aeruginosa colonization occurs at a younger age than previously reported. Early age at diagnosis and presence of CF-associated gastrointestinal abnormalities increase the likelihood of earlier colonization of P. aeruginosa.

Age Factors↗
PubMed1990

Predicting the need for hospitalization in children with acute asthma.

In an attempt to identify factors which influence the decision of physicians to admit patients with acute asthma to the hospital, we studied prospectively 200 children (age 5.6 +/- 3.1 years, mean +/- SD) presenting to our emergency room with acute asthma. The children were assessed on arrival, and on disposition from the Emergency Room by one of the investigators. After obtaining historic data, a clinical score was assigned, and oxygen saturation and pulmonary function were measured. Of the 134 (67 percent) children who were discharged home from the Emergency Room, five returned within seven days and one was subsequently admitted. The clinical score on disposition was the sole variable found to best predict the decision for hospitalization (sensitivity 73 percent, specificity 95 percent). Of the variables obtained at presentation, the resulting decision tree found the clinical score to predict the decision for hospitalization (sensitivity 79 percent, specificity 75 percent). When the individual components of the clinical score were analyzed, the degree of dyspnea, as assessed by the investigator, was chosen as the rule to predict the hospitalization decision (sensitivity 88 percent, specificity 71 percent). We conclude that the decision with respect to the need for hospitalization in acute childhood asthma, is in practice based mainly on careful clinical evaluation. Pulmonary function and SaO2 measurements, although helpful adjuncts in the assessment of acute asthma, do not appear to contribute to the identification of patients who need hospital admission.

Asthma↗
PubMed1990

Response of acute asthma to a beta 2 agonist in children less than two years of age.

The management of the young asthmatic child is still controversial and it has been questioned whether the infant and very young child with asthma respond to beta 2 agonists. We studied the response to nebulized beta 2 agonists in 43 children under the age of 2 years who presented to the emergency room with acute asthma. Clinical score and oxygen saturation were determined on admission to the emergency room and at least 30 minutes after each inhalation. The clinical score was defined as the sum of five variables (heart rate, respiratory rate, dyspnea, accessory muscle use, and wheezing), each graded as 0 or 1. The mean clinical score improved significantly after nebulized albuterol (mean +/- SD, 3.75 +/- 1.2 versus 2.80 +/- 1.65, P less than .01). Mean oxygen saturation did not change significantly (mean +/- SD, 94.8% +/- 2.85 versus 95.2% +/- 2.54). Only three patients had a decrease in arterial oxygen saturation of greater than 2% (3% in each one of them) following bronchodilator therapy. Our results suggest beta 2 inhalation to be beneficial to the majority of asthmatic children younger than 2 years of age and safe to administer. The combination of clinical score and oxygen saturation provides a simple noninvasive method of monitoring the response to therapy in young children with acute asthma.

Acute Disease↗
PubMed1989

A double staining method for measuring DNA content of specific cell populations utilizing computerized microscopy.

A method for identifying specific cell populations with the computerized microscope is described. This method utilizes double staining techniques using immunofluorescent antibodies and the Feulgen technique. It was applied to measure the area and DNA content of the nuclei of beta-cells in patients with Persistent Neonatal Hypoglycemia with Hyperinsulinism (PNHH). A significant increase in the area and DNA content was found in nuclei of patients compared to a control group. This method may be applied to sample cells from other tissues as well.

Coloring Agents↗
PubMed1989

Glycogen storage disease, Fanconi nephropathy, abnormal galactose metabolism and mitochondrial myopathy.

We present a 4-year-old male suffering from profound muscular weakness, enzymatically undefined glycogen storage disease. Fanconi nephropathy and impaired galactose utilization. Distorted mitochondria, intramitochondrial fat droplets and partial deficiencies of pyruvate dehydrogenase complex, succinate: cytochrome c oxidoreductase, and cytochrome c oxidase have been found in muscle tissue. The causal relationship between mitochondrial myopathy, glycogen storage disease, Fanconi nephropathy and impaired utilization of galactose is discussed.

Child, Preschool↗
PubMed1989

Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus.

We set out to determine if the clinical course or genetic profiles of patients with cystic fibrosis who had meconium ileus differed from those of other patients with cystic fibrosis. Since 1950 we have followed 158 patients with meconium ileus among 1175 patients with cystic fibrosis (13.4%). Patients with meconium ileus had lower birth weight (3026 +/- 610 gm) than patients with no meconium ileus (3169 +/- 534 gm; p less than 0.008); the deficit was especially evident in female patients. Survival in the first year of life increased from 55% in those born between 1958 and 1972 to 96% in those born between 1973 and 1987. Since 1973 the median survival of male and female patients with meconium ileus was similar to that in female patients with no meconium ileus (21 years), whereas 78% of males with no meconium ileus survived to this age (p less than 0.0001). Patients with meconium ileus born before 1972 had lower weight and height percentiles at age 13 years compared with patients with no meconium ileus, but this difference was not as apparent in patients born after 1973. There were no differences between the two groups in forced vital capacity, forced expiratory volume in 1 second, or forced expiratory flow in the middle half of forced vital capacity. Patients with meconium ileus acquired Pseudomonas aeruginosa at a younger age than did patients with no meconium ileus (4.20 +/- 4.67 vs 7.18 +/- 5.19 years), but there was no difference in age of acquisition of P. cepacia. In families in which the first child had meconium ileus, 29% of subsequent siblings with cystic fibrosis had meconium ileus, compared with 6% of siblings born to families in which the first child did not have meconium ileus. Allelic frequencies and haplotypic variants for cystic fibrosis chromosomes with respect to DNA markers closely linked to the cystic fibrosis locus were similar in families with cystic fibrosis with meconium ileus and those with no meconium ileus. These findings suggest that patients with cystic fibrosis and those without meconium ileus do not have major intrinsic differences and that the previously poor outlook in patients with meconium ileus has improved greatly.

Adolescent↗