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Biomedical subjects

D M Orenstein

Publications and source records attributed to D M Orenstein.

At least 37 records · Page 2Linked to original sources
PubMed1995

Nutrition in the pediatric double lung transplant patient with cystic fibrosis.

Cystic fibrosis (CF) is the most common lethal genetic disease in the white population. The pulmonary infections and pancreatic insufficiency make CF a medically challenging disease. Although the importance of nutrition in the CF patient is known, approximately 50% of CF patients are in less than the 10th percentile for weight and height as reported by the 1991 CF Foundation Registry of 114 CF Centers in the United States. This paper addresses the nutritional status of 10 pediatric CF patients who underwent double lung transplant at Children's Hospital of Pittsburgh between August 1991 and May 1993. Patients who survived beyond 1 year gained a significant amount of weight sooner after transplant than those who survived less than 1 year. Gastrostomy tube feedings were more effective than oral intake for weight gain after transplant. CF patients with pancreatic insufficiency have more difficulty with adjustment of doses of immunosuppressive agents for reasons that are not clearly understood.

Adolescent↗
PubMed1995

Exercise testing in pediatric heart, heart-lung, and lung transplant recipients.

Cardiorespiratory responses to progressive exercise were examined in 38 children who had undergone heart (n = 16), heart-lung (n = 13), or double-lung (n = 9) transplantation, and in 41 healthy controls. The four groups were similar in age, but the control subjects and heart transplant recipients were significantly larger than the heart-lung and lung recipients as assessed by body mass index (BMI). Time since transplant was significantly longer in the heart (601 days) compared with heart-lung (146 days) and lung (125 days) transplant groups. Physical work capacity and peak oxygen uptake were significantly reduced (43 to 64% of predicted) in the three transplant groups compared with the control group. Peak heart rate (percent predicted) was significantly higher in the control subjects (94%) compared with the heart (66%), heart-lung (70%), and lung (77%) transplant recipients. Peak minute ventilation was significantly higher in the control (72.9 L/min) and heart transplant (51.0 L/min) groups than the heart-lung (37.4 L/min) and lung (41.3 L/min) transplant groups. The control group had a higher peak tidal volume than the three transplant groups, and a higher peak respiratory rate than the lung transplant recipients. Correlational analysis revealed that physical work capacity (PWC) was significantly related to heart rate at peak exercise (HRpeak) and minute ventilation at peak exercise (VE-peak) in the heart transplant recipients, BMI, VEpeak, and FEV1 in the heart-lung transplant recipients, and BMI, HRpeak, VEpeak, FEV1, and number of days posttransplant in the lung transplant recipients. In addition to these variables, physical deconditioning and factors related to pharmacotherapy, infection, and rejection may also contribute to the decreased PWC observed in the transplant recipients.

Adolescent↗
PubMed1994

Ear disease in children with cystic fibrosis.

OBJECTIVE: To assess otitis media in children with cystic fibrosis. DESIGN: Prospective prevalence survey. SETTING: Otolaryngology and cystic fibrosis/pulmonology outpatient clinics, Children's Hospital of Pittsburgh (Pa), a regional referral center. PATIENTS: Seventy patients, aged 4 months to 17 years, with documented cystic fibrosis who presented to the cystic fibrosis/pulmonary clinic with scheduled appointments were asked to volunteer for the study. The 70 patients recruited represent approximately one fourth of the children younger than 17 years with cystic fibrosis who were followed up by the cystic fibrosis/pulmonary clinic. INTERVENTION: Ear, nose, and throat examination including pneumatic otoscopy, with tympanometry when indicated; relevant history taking. MAIN OUTCOME MEASURE: Presence or absence of otitis media. RESULTS: Seven (10%) of the 70 patients had unilateral or bilateral otitis media. CONCLUSIONS: Children with cystic fibrosis have a low prevalence of otitis media compared with normal children.

Acute Disease↗
PubMed1994

Lipid levels in adults with cystic fibrosis.

OBJECTIVE: To examine the hypothesis that an energy-dense, high-fat diet, which is necessary to maintain weight in adults with cystic fibrosis, does not result in high serum cholesterol levels. DESIGN: Dietary, anthropometric, and biochemical data were correlated. SETTING: A cystic fibrosis center in Pittsburgh, Pa. SUBJECTS: Thirty-one adults with cystic fibrosis, 50 obligate carriers of the cystic fibrosis gene, and 26 controls who did not have cystic fibrosis. MAIN OUTCOME MEASURES: Adults with cystic fibrosis had a lower mean serum cholesterol level and higher mean intakes of energy and fat than controls. STATISTICAL ANALYSES PERFORMED: Student's t test was used to determine the statistical significance between two means. Univariate correlation coefficients were determined to measure the relative intensity of association between two variables. RESULTS: Mean total serum cholesterol levels in men with cystic fibrosis was 3.1 mmol/L vs 4.7 mmol/L in male controls (P < .001). Mean total serum cholesterol levels in women with cystic fibrosis was 3.2 mmol/L vs 4.3 mmol/L in female controls (P < .001). Three adults with cystic fibrosis and no signs of pancreatic insufficiency had serum cholesterol levels in the high normal range. Carriers had serum lipid levels in the same range as the controls. CONCLUSIONS/APPLICATIONS: The findings indicate that a high-energy, high-fat diet does not raise serum lipid levels in those patients with cystic fibrosis and pancreatic insufficiency. However, those individuals with cystic fibrosis and normal pancreatic function may be at the same risk as the general population for developing high serum lipid levels. They should have their serum lipid levels monitored and be given appropriate dietary recommendations.

Adult↗
PubMed1994

Experience with pediatric lung transplantation.

Heart-lung transplantation and lung transplantation have become accepted techniques in adult patients with end-stage cardiopulmonary disease. We report here our experience between July 1985 and March 1993 with 34 children (< 20 years) who underwent heart-lung (n = 18) or lung transplantation (n = 17). Indications for transplantation included cystic fibrosis (n = 9), congenital heart disease with Eisenmenger complex (n = 9), primary pulmonary hypertension (n = 8), pulmonary arteriovenous malformations (n = 2), desquamative interstitial pneumonia (n = 2), Proteus syndrome with multicystic pulmonary disease (n = 1), graft-versus-host disease (n = 1), rheumatoid lung disease (n = 1), and bronchiolitis obliterans and emphysema (n = 1). Twenty-six patients (76%) have survived from 1 to 88 months after transplantation; most patients have returned to an active lifestyle. Of the eight deaths, four were due to infections, two to multiorgan failure, 1 to posttransplant lymphoproliferative disease, and one to donor organ failure. Four of the patients who died had cystic fibrosis. Despite considerable morbidity related to infection, rejection, and function of the heart-lung and lung allograft in some patients, our results with this potentially lifesaving procedure in the pediatric population have been encouraging.

Adolescent↗
PubMed1994

Pseudomonas cepacia empyema necessitatis after lung transplantation in two patients with cystic fibrosis.

Lung transplantation is an accepted modality for patients with cystic fibrosis (CF) who have end-stage respiratory failure. The postoperative course of these patients is often complicated by serious infections with organisms such as Pseudomonas aeruginosa and Pseudomonas cepacia that may be multiply resistant to conventional antimicrobial agents. We describe two patients with CF who, after double lung transplantation, developed the unusual complication of empyema and empyema necessitatis due to P cepacia that was resistant to all tested antibiotics.

Adolescent↗
PubMed1994

Complications of pediatric lung and heart-lung transplantation.

As more children receive lung and heart-lung transplants, understanding posttransplantation complications becomes more important. Complications can be categorized as 1) anatomic and surgical, 2) infectious, 3) rejection, 4) posttransplantation lymphoproliferative disease (PTLD), and 5) drug-related. In the early posttransplantation period, reimplantation injury and vascular obstruction with thrombus formation may occur, whereas stenosis at tracheal or bronchial anastomotic sites develops more slowly. Infections in the transplanted lung are common, and can be caused by bacteria, viruses, fungi, or protozoans. Both acute and chronic rejection are potentially serious. Frequent or severe episodes of acute rejection increase the risk of chronic rejection, characterized by obliterative bronchiolitis. Differentiating rejection from infection requires tissue, obtained through transbronchial or open lung biopsy. PTLD, related to Epstein-Barr virus infection, is common in children, and difficult to treat. Finally, drug toxicity from immunosuppressive agents causes considerable morbidity. Increased understanding will continue to improve the outcome for children undergoing lung and heart-lung transplantation.

Acute Disease↗
PubMed1993

Neonatal screening for cystic fibrosis: addition of molecular diagnostics to increase specificity.

Newborn screening for cystic fibrosis (CF) has been carried out on approximately 106,000 neonates in western Pennsylvania since 1987 using the immunoreactive trypsinogen (IRT) assay on dried filter paper blood specimens (DBS). Molecular analysis utilizing a duplicate DBS from the same sample was implemented in November 1989 for newborns having elevated IRT levels. DNA is amplified directly from the DBS and the amplified products are tested for the delta F508 deletion and several common exon 11 mutations. Substituting dUTP for dTTP in the PCR reaction and an initial treatment with uracil N-glycosylase (UNG) virtually eliminates PCR carryover contamination. The number of confirmed cases of CF is 20, giving an estimated incidence of 1:5287 in the western Pennsylvania population. Eight of the CF patients are homozygous and 12 are compound heterozygotes for the delta F508 deletion and a second mutation. Two of the compound heterozygotes carry the G551D mutation and one has the R553X mutation. Twenty-one additional neonates that are heterozygous for the delta F508 mutation are normal carriers for CF. In approximately 55% of the cases, molecular analysis of the CF gene confirmed the diagnosis of CF prior to sweat testing. The incorporation of molecular analysis into our CF screening program increases the specificity of the screening strategy and has the potential to decrease the false positive and sweat test referral rate, reduce parental anxiety, and bring CF infants to the attention of physicians more rapidly.

Cystic Fibrosis↗
PubMed1993

Cystic fibrosis.

The past few years have seen amazing progress in our understanding of the underlying cellular and molecular bases of CF. This new understanding has raised hopes for improved treatments in the very near future. These new treatments are expected to improve even further the prognosis for survival in this disorder, which has improved nearly 1 year for every passing year over the past two decades. The future for patients with CF is brighter than ever.

Burkholderia cepacia↗
PubMed1993

Bronchoalveolar lavage and transbronchial biopsy in children following heart-lung and lung transplantation.

Between July 1985 and March 1992, 20 children received either heart-lung (11), double lung (8), or single lung (1) transplants at our center. Since 1988, flexible fiberoptic bronchoscopy with bronchoalveolar lavage and transbronchial biopsy have been carried out to monitor for rejection or infection in these patients. As of March 31, 1992, we have performed a total of 112 transbronchial biopsies in our patients, who ranged from 6.8 to 18 years of age and 19.3 to 67.3 kg in weight. All but two of these procedures were carried out using conscious sedation and a transnasal approach. Four to seven biopsy samples were obtained at each procedure. One patient had hemorrhage (< 100 ml) and no patient had pneumothorax as a complication. Of the biopsy samples, 72.4 percent had a surface area of greater than 2 mm2, and 89.5 percent of the biopsy samples were deemed adequate for pathologic interpretation. We believe that for the majority of pediatric lung or heart-lung recipients, flexible bronchoscopy and transbronchial biopsy using conscious sedation and a transnasal approach is safe and permits the recovery of adequate tissue for pathologic evaluation. The avoidance of general anesthesia, endotracheal intubation, and mechanical ventilation at the time of bronchoscopy and transbronchial biopsy probably decreases the likelihood of pneumothorax as a complication of the procedure.

Biopsy↗
PubMed1993

Accuracy of three pulse oximeters during exercise and hypoxemia in patients with cystic fibrosis.

To evaluate the reliability of noninvasive oximeters during exercise in patients with cystic fibrosis (CF), we studied 9 patients during exercise tests, using 3 devices (Hewlett-Packard 47201A, Biox-Ohmeda 3700, Nellcor 100), comparing oximetry readings with arterial blood co-oximetry. Fifty samples from preexercise, exercise, and recovery were collected; and paired measurements were analyzed for bias (arterial blood gas SaO2--noninvasive SaO2) and precision (1 SD of the bias). Values were available for the Hewlett-Packard and the Biox-Ohmeda at all 50 collection times. The Nellcor failed to read on four occasions, all during exercise. The accuracy of the Biox-Ohmeda readings was significantly worse during exercise than rest, while the Nellcor and the Hewlett-Packard performance did not differ significantly between exercise and rest. During hypoxemia (SaO2 < 90 percent as determined by co-oximetry on arterial blood), the Biox-Ohmeda and Nellcor consistently overestimated saturation, with mean bias of -6.1 percent and -3.0 percent, respectively. The Hewlett-Packard was more reliable and valid during hypoxemia, with a mean bias of -1.0 percent. The Nellcor read significantly more accurately in the six patients with severe digital clubbing than in the three patients with mild digital clubbing. It is important to be aware of possible limitations of noninvasive oximeters before accepting them as accurate.

Adolescent↗
PubMed1993

Cystic fibrosis. Clinical update for radiologists.

The clinical features and current management of cystic fibrosis are reviewed, with emphasis on pulmonary manifestations. Pulmonary radiographic findings in cystic fibrosis are illustrated. The role of transplantation as a therapeutic avenue is discussed. Recent discoveries--the specific genetic mutation in cystic fibrosis, the elucidation of the cellular defect, and the development of gene-transfer techniques to correct the cellular defect in vitro--have generated fresh excitement in the search for an ultimate cure for cystic fibrosis.

Child↗
PubMed1992

The prognostic value of exercise testing in patients with cystic fibrosis.

BACKGROUND: Previous studies have shown female sex, impaired pulmonary function, older age, malnutrition, and colonization of the respiratory tract with Pseudomonas cepacia to be associated with a poor prognosis in patients with cystic fibrosis. We sought to determine the prognostic value of exercise testing in addition to the other prognostic factors. METHODS: A total of 109 patients with cystic fibrosis, 7 to 35 years old, underwent pulmonary-function and exercise testing in the late 1970s. They were followed for eight years to determine the factors associated with subsequent mortality. Survival rates were calculated with standard life-table methods. Cox proportional-hazards regression models were used to determine crude relative risks of mortality and relative risks adjusted for age, sex, body-mass index, forced expiratory volume in one second (FEV1) end-tidal partial pressure of carbon dioxide (PCO2) at peak exercise, and oxygen consumption at peak exercise (VO2 peak). RESULTS: Patients with the highest levels of aerobic fitness (VO2 peak, > or = 82 percent of predicted) had a survival rate of 83 percent at eight years, as compared with rates of 51 percent and 28 percent for patients with middle (VO2 peak, 59 to 81 percent of predicted) and lowest (VO2 peak, < or = 58 percent of predicted) levels of fitness, respectively. After adjustment for other risk factors, patients with higher levels of aerobic fitness were more than three times as likely to survive than patients with lower levels of fitness. Colonization with P. cepacia was associated with a risk of dying that was increased fivefold. Age, sex, body-mass index, FEV1, and end-tidal PCO2 at peak exercise were not independently correlated with mortality. CONCLUSIONS: Higher levels of aerobic fitness in patients with cystic fibrosis are associated with a significantly lower risk of dying. Although better aerobic fitness may simply be a marker for less severe illness, measurement of VO2 peak appears to be valuable for predicting prognosis. Further research is warranted to determine whether improving aerobic fitness through exercise programs will result in a better prognosis.

Adolescent↗
PubMed1992

Pancreatitis in young children with cystic fibrosis.

Five young children with cystic fibrosis and abdominal pain were found to have pancreatitis. Diagnosis was delayed in four patients because of the belief that pancreatitis occurs only in older patients with cystic fibrosis. In one patient pancreatitis was diagnosed before cystic fibrosis and diagnosis of cystic fibrosis was delayed. Pancreatitis should be considered as a possible cause of abdominal pain in pancreatic-sufficient children with cystic fibrosis and cystic fibrosis should be considered as a possible cause of pancreatitis, even in the young child.

Abdominal Pain↗
PubMed1991

Exercise performance and breathing patterns in cystic fibrosis: male-female differences and influence of resting pulmonary function.

Despite improved prognosis for patients with cystic fibrosis (CF), they continue to suffer from progressive decline in pulmonary function and exercise tolerance. Beyond puberty, female patients have greater loss of pulmonary function and higher mortality than males, yet no one has studied male-female differences in exercise tolerance among patients. We therefore compared the responses to progressive exercise in 58 male and 52 female patients with CF and analyzed the influence of resting pulmonary function and gender on these responses and on breathing patterns during exercise. Within each of three pulmonary function groups (group 1: FEV1 greater than or equal to 65% predicted; group 2: FEV1 = 50-64% predicted; and group 3: FEV1 less than 50% predicted), female patients had lower exercise tolerance than males. This was reflected in the overall group for each gender: males' peak work load was 130 +/- 64 W, females' 111 +/- 32 W (P = 0.04); males' peak oxygen consumption, 36.9 +/- 11.4 mL/kg/min and females', 31.0 +/- 7.4 mL/kg/min (P = 0.0006). There was no interactive effect between gender and pulmonary function. Neither respiratory rate nor tidal volume differed between sexes. Male and female patients, irrespective of disease severity, employed similar proportions of their ventilatory capacity at exhaustion; group mean minute ventilation to maximum voluntary ventilation ratio ranged from 62.9 to 78.3%, with no apparent relationship to disease severity.

Adolescent↗
PubMed1991

Measuring the quality of well-being in cystic fibrosis and lung transplantation. The importance of the area under the curve.

The quality of well-being scale (QWB) has been used to measure overall life quality in a number of different clinical conditions, including cystic fibrosis and chronic obstructive pulmonary disease. Evidence supports its validity and reliability in both cross-sectional and longitudinal studies. One of the strengths of the QWB is that it is not organ-specific, so that it can take into account the broad impact of a disease and/or intervention, including unanticipated effects. The well-year of life is a concept that includes both morbidity and mortality: if a disease reduces quality of life by one-half, over a period of two years, that person can be said to have lost one full well-year. As we examine the impact of diseases such as cystic fibrosis, and of interventions such as lung transplantation, it is important to consider changes in overall well-being over time to help individual patients and families make difficult therapeutic choices and to help formulate health policy.

Adult↗