Biomedical subjects

Samya Z Nasr

Publications and source records attributed to Samya Z Nasr.

10 recordsLinked to original sources

PubMed2006

Cystic fibrosis detection in high-risk Egyptian children and CFTR mutation analysis.

BACKGROUND: Knowledge about Cystic Fibrosis (CF) in Egypt is very limited. The objective of this study was to screen for CF in Egyptian children with suggestive clinical features and to identify causative genetic mutations. METHODS: Sixty-one patients from the Chest Unit, Cairo University Children's Hospital, Egypt, were included. Subjects presented with persistent or recurrent respiratory symptoms, failure to thrive, diarrhea and/or steatorrhea and unexplained persistent jaundice. Patients were screened using the CF Indicatortrade mark sweat test system (PolyChrome Medical, Inc., Brooklyn Center, MN). A quantitative sweat testing was conducted on 10 of the 12 positive patients. Seven probands and one sibling underwent molecular analysis by direct DNA sequencing of the coding region and of the intronic sequences adjacent to the 27 exons of the CFTR gene. RESULTS: Of 61 patients, 12 (20%) had positive sweat chloride screening. Ten of the 12 patients underwent quantitative sweat testing and were positive. Eight CFTR sequence changes were identified in seven affected probands and two were confirmed in one sibling by direct DNA sequencing. CONCLUSION: The study results suggest that CF is more common in Egypt than previously anticipated. Larger studies are warranted to identify the incidence, molecular basis and clinical pattern of CF in the Egyptian population.

Child, Preschool↗

PubMed2006

Inpatient health care use among adult survivors of chronic childhood illnesses in the United States.

OBJECTIVES: To describe the inpatient length of stay and related charges for adults in the United States with childhood-onset chronic disease and to examine patterns with respect to different hospital settings. DESIGN: We analyzed data from the 2002 Nationwide Inpatient Sample, a nationally representative data set of hospital discharges. We performed a case-mix-adjusted, sample-weighted regression analysis as well as descriptive statistics of hospital use among adults with childhood-onset chronic disease. SETTING: United States. PARTICIPANTS: We identified hospitalizations for persons aged 18 years or older with a diagnosis of complex congenital heart disease, cystic fibrosis, sickle cell disease, or spina bifida. Main Exposure Childhood chronic illness. MAIN OUTCOME MEASURES: Length of stay and total charges among pediatric, adult, and mixed hospitals. RESULTS: In multivariate adjusted analyses, patients with complex congenital heart disease and cystic fibrosis had a significantly longer length of stay in pediatric hospitals than in adult or mixed hospitals (P<.001); no similar difference was found for sickle cell disease and spina bifida. For all of the 4 conditions, hospital charges were significantly greater in pediatric hospitals than in adult or mixed hospitals (P<.001 for cystic fibrosis, complex congenital heart disease, and sickle cell disease, and P<.01 for spina bifida). CONCLUSIONS: The vast majority of persons who have survived to adulthood with complex congenital heart disease, cystic fibrosis, spina bifida, or sickle cell disease are hospitalized in hospitals that predominantly care for adults, where charges for care appear to be lower than in pediatric hospitals.

Adult↗

PubMed2006

High resolution computerized tomography of the chest and pulmonary function testing in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis patients.

To evaluate the sensitivity of high-resolution computerized tomography (HRCT) of the chest compared to spirometry measures in evaluating the effects of tobramycin solution for inhalation (TSI) in cystic fibrosis (CF) patients.Thirty-two subjects >/=6 years old with mild to moderate CF lung disease were enrolled in a randomized, double-blind, placebo-controlled pilot study. Duration was 28 days; 31 subjects completed the study.HRCT scores decreased 4.06 +/- 3.20 (mean +/- SD) for TSI and decreased 0.17 +/- 1.78 for placebo subjects (P = 0.13). Mean forced expiratory flow during middle half of forced vital capacity (FEF(25%-75%)) predicted increased 6.08 +/- 4.86 for TSI and decreased 0.60 +/- 2.34 for placebo (P = 0.23). Percentage forced expiratory volume in 1 s (FEV(1)) predicted increased slightly for both TSI and placebo (1.29 +/- 3.33 for TSI and 1.17 +/- 1.4 for placebo) (P = 0.97). Two of eight HRCT component scores (atelectasis and inhomogeneity) were observed to be highly discordant with observed HRCT global total score and other HRCT component scores. A modified total score was calculated by dropping them from the global total score. The modified HRCT total scores decreased 6.68 +/- 3.09 for TSI subjects and increased 0.02 +/- 2.0 for the placebo subjects (P = 0.07). Sample sizes were calculated to show statistical significance by differences in modified total HRCT scores, global total HRCT scores, FEF(25%-75%) predicted or FEV(1) % predicted. A total of 60, 100, 200, and over 800 patients would be necessary respectively.HRCT can be a useful measure of change in CF pulmonary disease, requiring a smaller sample size than that required to show treatment effect by pulmonary function testing (PFT) alone.

Administration, Inhalation↗

PubMed2006

Adherence to treatment in children and adolescent patients with cystic fibrosis.

PURPOSE: The purpose of this study was to monitor medication adherence in cystic fibrosis (CF) patients and its correlation with disease severity and patient age. METHODS: Children less than 12 years of age (group 1) and adolescents 12 years of age and older (group 2) were recruited from the University of Michigan CF Center. The study duration was 3 months. A total of 22 patients per group were enrolled. Adherence to ADEKs, an oral multivitamin, and dornase alfa, a nebulized mucolytic medication, was monitored. Adherence to ADEKs was monitored by using the Medication Event Monitoring System (MEMS) SmartCaps (APREX, AARDx, Inc., Union City, California). Dornase alfa adherence rate was monitored by counting empty medication vials. RESULTS: Thirty-three patients completed the study, 15 patients in group 1 and 18 patients in group 2. The overall mean adherence rates for ADEKs and dornase alfa were (+/- SD) 63.6% +/- 24.0% and 66.5% +/- 31.2%, respectively. The median ADEKs and dornase alfa adherence rate for group 1 was 84.6% and 79.1%, respectively (p = .08); and for group 2 was 56.7% vs. 78.4%, respectively (p = .07). There was a trend toward significance, suggesting that the adherence rate for ADEKs was higher than for dornase alfa (p = .08) in group 1. Group 2 showed a trend toward adherence to dornase alfa than to ADEKs (p = .07). There was a trend for ADEKs adherence between groups 1 and 2 (p = .09), but not for dornase alfa (p = .93). CONCLUSION: Parental supervision and disease severity are likely to play a major role in adherence to medical management. Partnership with patients and families about the treatment plan might be important for improving adherence rate. The MEMS SmartCaps is an electronic monitoring technology that should be used to measure drug adherence objectively both in further larger clinical trials and in the outpatient setting.

Administration, Oral↗

PubMed2005

Use of laryngeal mask airway in flexible bronchoscopy in infants and children.

In the past two decades, flexible bronchoscopy (FB) has gained increasing popularity among pediatric pulmonologists. The objective of this study was to review our experience with pediatric flexible bronchoscopy over the past 15 years, with special focus on route of bronchoscopy. This is a retrospective study. We reviewed our pediatric FB procedures performed at the University of Michigan, Mott Children's Hospital, from 1988-2003. The study included 1,947 procedures in 1,548 patients, with a mean of 1.3 procedures/patient. The male:female ratio was 1.66:1, and age was 4.9 +/- 5.6 years (mean +/- SD). Patients <2 years of age represented 46.6% of the study population. The laryngeal mask airway (LMA) was the most common route for flexible bronchoscopy in children 2 years of age and above. Complication rates were lower with the use of the LMA (1.9%) compared to the nasal route (3.5%). Stridor was the most common indication in the age group <2 years (20.8%), while persistent pulmonary infiltrates were more common in the older age groups (32.2-37%). Laryngomalacia was the most common finding in patients with stridor (31.5%), while inflammatory changes were more common with other indications. Procedure-related complications were reported in 2.3% of procedures. Bronchoalveolar lavage (BAL) samples were obtained and analyzed in 51.2% of FB procedures (n=1,000), of which 19.4% yielded positive microbiology cultures. In conclusion, FB is a safe procedure in pediatrics. Children less than 7 years of age represent the majority of FB subjects. The LMA offered a lower rate of procedure-related complications when compared to the nasal route or endotracheal tube. It also reduced procedure time and anesthesia time.

Adolescent↗

PubMed2005

Randomized, double-blind, placebo-controlled, dose-escalating study of aerosolized interferon gamma-1b in patients with mild to moderate cystic fibrosis lung disease.

Interferon gamma-1b (IFN-gamma1b) is a pleiotropic cytokine with immunomodulatory activities that could decrease bacterial burden, inflammation, and obstruction in patients with CF. Patients with CF (> or =12 years old, FEV1 > or =40% predicted) were randomly assigned to sequential dose cohorts inhaling 500 microg IFN-gamma1b, 1,000 microg IFN-gamma1b, or placebo by Respirgard II nebulizer thrice weekly for 12 weeks. Sputum bacterial density and spirometry were measured. Safety, antibiotic use, hospitalization, and sputum neutrophils, elastase, DNA, IL-8, and myeloperoxidase were also evaluated. Sixty-six patients (mean age, 24 years, with mean baseline FEV1 of 74 +/- 20 (SD) percent predicted) were studied. One patient had bronchospasm after the first dose of IFN-gamma1b; the overall withdrawal rate was 15% (5 in the placebo group, 2 in the 500-microg IFN-gamma1b group, and 3 in the 1,000 microg IFN-gamma1b group). The 500-microg IFN-gamma1b dose was well-tolerated, but the 1,000-mug dose cohort, who had a higher baseline bacterial density than placebo patients (mean difference, 1.2 log(10) CFU/g sputum, 95% confidence interval (CI), 0.1,2.8, P=0.04), had 24% more hospitalizations for exacerbation than placebo patients (95% CI, 2,45%, P=0.05). There was a 0.12-l difference between the 500-microg IFN-gamma1b and placebo groups with respect to the 12-week change in FEV1 (active group minus placebo group, 95% CI, -0.03,0.26, P=0.11), as compared to a 0.01-l difference between the 1,000-microg IFN-gamma1b and placebo groups (95% CI, -0.16,0.17, P=0.96). No effects of IFN-gamma1b were seen in sputum bacterial density or inflammatory biomarkers at 12 weeks. Aerosolized IFN-gamma1b did not improve pulmonary function, reduce sputum bacterial density, or affect inflammatory sputum markers in patients with mild-moderate lung disease.

Administration, Inhalation↗

PubMed2004

Treatment with tobramycin solution for inhalation reduces hospitalizations in young CF subjects with mild lung disease.

Our objective was to study the effect of tobramycin solution for inhalation (TSI; TOBI, Chiron Corp.) on lung function decline rate in 400 young persons with cystic fibrosis (CF) and mild lung disease. Effects on hospitalization, antibiotic use, school days missed, and nutritional status also were determined. This was an open-label, randomized (stratified by sex and age group, i.e., 6-10 and 11-15 years), parallel-group, multicenter study. Routine subject management (control group) was compared to routine management plus 28 days of twice-daily TSI inhalation, followed by 28 days off the drug (TSI group) for 56 weeks. Primary efficacy endpoints included rate of lung function decline (as measured by forced expiratory volume in 1 sec; FEV(1)), hospitalization, and concomitant antibiotic use. Safety was assessed by analysis of treatment-emergent adverse events. Only 184 of 400 planned subjects were recruited and randomized (93 to the TSI group, and 91 to the control group). Enrollment was ended after 2 years because of difficult recruitment. An interim safety review showed a 2.42-fold risk of respiratory hospitalization for control group subjects (P = 0.020), and the study was terminated. Sixty-three subjects (34.2%) completed the entire study (30 in the TSI group, or 32.3%; and 33 in the control group, or 36.3%). Significantly fewer TSI subjects were hospitalized for worsening of respiratory symptoms (11.0% vs. 25.6%; P = 0.011), and fewer TSI subjects were hospitalized overall (16.5% vs. 27.8%; P = 0.065). Fewer TSI subjects received antibiotics other than the study drug (78.0% vs. 95.6%), and significantly fewer received oral antibiotics (76.9% vs. 91.1%; P = 0.009). No other safety or adverse event differences were observed. In conclusion, significant reductions in respiratory hospitalizations, concomitant antibiotic use, and a trend towards improvement in percent predicted forced expiratory flow (FEF(25-75)) provide evidence of a clinical benefit of TSI use in young persons with CF and mild lung disease. An effect on lung function decline rate could not be evaluated as planned, due to inadequate enrollment and early study termination.

Administration, Inhalation↗

PubMed2004

Clinical redesign using all patient refined diagnosis related groups.

OBJECTIVE: Clinical redesign of processes in hospitals that care for children has been limited by a paucity of severity-adjusted indicators that are sensitive enough to identify areas of concern. This is especially true of hospitals that analyze pediatric patient care using standard Centers for Medicare and Medicaid Services (CMS) diagnosis-related groups (DRGs). The objectives of this study were to determine whether 1) utilization of all-patient refined (APR)-DRG severity-adjusted indicators (length of stay, cost per case, readmission rate) from the National Association of Children's Hospitals and Related Institutions (NACHRI) database could identify areas for improvement at University of Michigan Mott Children's Hospital (UMMCH) and 2) hospital staff could use the information to implement successful clinical redesign. METHODS: The APR-DRG Classification System (version 20) was used with the NACHRI Case Mix Comparative Database by severity level comparison from 1999 to 2002. Indicators include average length of stay (ALOS), case mix index, cost per case, and readmission rate for low acuity asthma (APR-DRG 141.1). UMMCH cases of 141.1 (n = 511) were compared with NACHRI 141.1 (n = 64,312). Although not part of the standard report, mortality rates were calculated by NACHRI for UMMCH and an aggregate of NACHRI member children's hospitals. RESULTS: Data from 1999 revealed that in noncomplicated asthma cases (level 1 severity), the UMMCH ALOS versus NACHRI ALOS was slightly longer (UMMCH 2.16 days vs NACHRI 2.14 days), and the cost per case was higher (UMMCH $2824 vs NACHRI 2738 dollars), whereas levels 2, 3, and 4 cases (moderate, major, and extreme severity) indicated the ALOS and cost per case were lower than the national aggregate. This showed that the APR-DRG system was sensitive enough to distinguish variances of care within a diagnosis according to severity level. After analysis of internal data and meeting with clinicians to review the indicators, 3 separate clinical processes were targeted: 1) correct documentation of comorbidities and complications, 2) standardized preprinted orders were created with the involvement of the pediatric pulmonologists, and 3) standardized automatic education for parents was started on the first day of admission. Yearly data were reviewed and appropriate adjustments made in the education of both residents and staff. In 2002, the UMMCH ALOS dropped to 1.75 +/- .08 days from 2.16 +/- .09. In 2002, the NACHRI ALOS was 2.00 days +/- 0.01 versus the UMMCH ALOS of 1.75 days +/- 0.0845, indicating that the UMMCH ALOS dropped significantly lower than the NACHRI aggregate database over the 3-year period. Cost per case of UMMCH compared with NACHRI after the 3 years indicated that UMMCH increased 12%, whereas the NACHRI aggregate increased 18%. These data show that length of stay and cost per case relative to the national database improved after clinical redesign. Improvements have been sustained throughout the 3-year period. Readmission rates ranged from 2.97% to 0.80% and were less than the national cohort by the third year. There were no mortalities in the UMMCH inpatient asthma program. This demonstrates that clinicians believed that the data from the APR-DRG acuity-adjusted system was useful and that they were then able to apply classical clinical redesign strategies to improve cost-effectiveness and quality that was sustained over 3 years. CONCLUSIONS: Severity-adjusted indicators were useful for identifying areas appropriate for clinical redesign and contributed to the improvement in cost-effective patient care without a detriment in quality indicators. This method of using a large comparative database, having measures of severity, and using internal analysis is generalizable for pediatric hospitals and can contribute to ongoing attempts to improve cost-effectiveness and quality in medical care.

Asthma↗

PubMed2002

Hypersensitivity to inhaled TOBI following reaction to gentamicin.

Cystic fibrosis (CF) is the most common autosomal-recessive disease in Caucasians. Colonization with Pseudomonas aeruginosa (P. aeruginosa) of the CF airways causes deterioration of pulmonary status. TOBI (Tobramycin solution for inhalation) is an inhaled antibiotic that can improve the pulmonary disease. We report on a 9-year old boy with CF who developed a rash following a course of IV gentamicin. The rash resolved after its discontinuation. However, the rash returned all over his body, with the start of inhalation of TOBI therapy. We desensitized the patient using escalating doses of inhaled TOBI. He tolerated the procedure well, and continues to be on TOBI 9 months after desensitization on a once-a-day regimen.

Administration, Inhalation↗

PubMed2002

Religious/spiritual coping in childhood cystic fibrosis: a qualitative study.

OBJECTIVE: To understand the role of religiousness/spirituality in coping in children with cystic fibrosis (CF). METHODS: Participants were a convenience sample of 23 patients with CF, ages 5 to 12 years, and their parent(s) in an ambulatory CF clinic. The design was a focused ethnography including in-depth interviews with children and parent(s), children's drawings, and self-administered written parental questionnaires. Analysis used grounded theory. RESULTS: Main outcome measures were participants' views on religion/spirituality in coping with illness. Data included 632 quotes organized into 257 codes categorized into 11 themes. One overarching domain emerged from analysis of the 11 themes: Religious/Spiritual Coping, composed of 11 religious/spiritual coping strategies. CONCLUSIONS: Children with CF reported a variety of religious/spiritual coping strategies they nearly always associated with adaptive health outcomes. A preliminary conceptual framework for religious/spiritual coping in children with CF is presented. More study is needed to assess how variability in age, disease type, disease severity, religious/spiritual preference, and religious/spiritual intensity affect religious/spiritual coping in children with chronic illness. Future studies should also investigate whether physician attention to religious/spiritual coping could assist patients in coping with CF and strengthen the doctor-patient relationship.

Adaptation, Psychological↗