Search PubMedSearch

Biomedical subjects

D M Orenstein

Publications and source records attributed to D M Orenstein.

At least 19 recordsLinked to original sources

Validity of self-assessment of sexual maturation in adolescent male patients with cystic fibrosis.

PURPOSE: Self-assessment of sexual maturity by healthy male adolescents has been found to correlate closely with physician ratings. Data are lacking, however, in adolescents with cystic fibrosis (CF), where an altered body image may affect self-assessment. Delayed sexual maturation occurs in many patients with CF, particularly those with severe disease. We hypothesized that self-assessment of sexual maturation by adolescents with CF would agree with physician ratings. METHODS: Using Tanner's standard photographs for pubic hair (PH) and genital (G) development, we compared self-assessment of sexual maturation to physician rating in 34 adolescent male patients with CF and 27 healthy male controls (C). RESULTS: The two groups did not differ in age. All subjects were initially examined and Tanner-staged by a physician (SRB), and instructed in self-assessment using the Tanner photographs; they then performed a self-assessment. Scores by physician and subjects were assessed for inter-observer agreement by Kappa analysis. For the CF group, 29 of 34 PH assessments and 21 of 34 G assessments demonstrated exact inter-observer agreement between physician and subject ratings. The Kappa coefficient, kappa, (weighted for the degree of closeness between two observers) was 0.946 for PH and 0.840 for G and the C group, kappa was 0.905 for PH and 0.737 for G. Repeat analysis combining stages 3 and 4 PH and G development yielded higher inter-observer agreement in the CF group (33 of 34 PH assessments and 26 of 34 G assessments) and in the C group (24 of 27 PH assessments and 18 of 27 G assessments). CONCLUSIONS: Self-assessment is a valid method to assess sexual maturity in clinical evaluation and as a research tool in the study of patients with CF.

Adolescent

Chronic pancreatitis in a patient with cystic fibrosis and clinical pancreatic insufficiency.

Pancreatitis occurs in up to 15% of patients with cystic fibrosis and pancreatic sufficiency, but the possibility of its occurrence in patients with pancreatic insufficiency has not been recognized. We describe a patient with homozygous delta F508 cystic fibrosis and typical symptoms of pancreatic insufficiency (greasy, fatty stools) in whom pancreatitis developed.

Child, Preschool

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

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

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

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

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

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

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

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

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

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

Oxygen supplementation during exercise in cystic fibrosis.

Fourteen female and 22 male patients with cystic fibrosis (CF), 8 to 29 yr of age, performed two progressive exercise tests to exhaustion on a cycle ergometer, breathing normoxic air (21% O2) for one test, and hyperoxic air (30% O2) for the other test. The order of gas administration was randomized. Minute ventilation (VE), oxygen uptake (VO2), end-tidal CO2 tension (PETCO2), work rate, oxyhemoglobin saturation (SAO2), and heart rate (HR) were measured throughout the tests. The SaO2 of 11 patients at peak exercise was 90% or less ("Low Sat" group). The SaO2 of 23 patients remained above 90% throughout the exercise ("High Sat" group). Hyperoxic air minimized desaturation during exercise in the Low Sat group to 2 +/- 2% compared to a decrease of 10 +/- 5% with normoxic air. The decrease in saturation was not significant for the High Sat group (1 +/- 1% for both 21% and 30% O2). Peak work rate and VO2 did not differ significantly between normoxic and hyperoxic conditions. However, VE and HR at peak exercise tended to be lower, and PETCO2 was higher during peak exercise with 30% O2 than 21% O2 for both groups. During submaximal exercise, O2 desaturation was diminished and HR was significantly lower with supplemental O2, specifically in the Low Sat group. VE was significantly lower for both groups during submaximal exercise with hyperoxic air. The results suggest that O2 supplementation minimizes O2 desaturation and enables patients with CF to exercise with reduced ventilatory and cardiovascular work.

Adolescent

Quality of well-being before and after antibiotic treatment of pulmonary exacerbation in patients with cystic fibrosis.

General quality of life has only recently been measured with an objective tool in patients with cystic fibrosis (CF), and there have been no reported attempts to document changes in patients' overall well-being over time, as patients deteriorate or respond to intervention. We applied the Quality of Well-Being scale (QWB) in 28 patients with CF before and after a two-week course of oral ciprofloxacin used to treat pulmonary exacerbations. There were significant correlations between changes in QWB and various pulmonary function test results; QWB vs FEV1: r = 0.4, p less than 0.03; QWB vs FVC: r = 0.5, p less than 0.01; and QWB vs SaO2: r = 0.4, p less than 0.05. Thus, the QWB can track changes in general well-being in CF patients over a brief time and detect changes associated with pulmonary exacerbation and its treatment.

Attitude to Health

The ocular surface in cystic fibrosis.

Seventeen patients with cystic fibrosis and 17 age-, race-, and sex-matched controls were examined under standardized conditions. Testing included slit-lamp biomicroscopy, fluorescein staining, rose bengal staining, Schirmer's basic tear test, tear film break-up time, tear pH, tear lysozyme, tear protein, lid and conjunctival cultures, and conjunctival impression cytology. Cystic fibrosis patients showed a statistically significant increase in the incidence of fluorescein staining and clinical blepharitis, as well as significantly decreased Schirmer testing and tear lysozyme. Ocular surface abnormalities in these patients may be attributable to aqueous and lipid tear film deficiencies. Cystic fibrosis patients showed normal conjunctival epithelial cell morphology, grew no pathogenic organisms, and had a decreased incidence of conjunctival bacterial colonization.

Adolescent

Depression and anxiety in pediatric inflammatory bowel disease and cystic fibrosis.

The lifetime and current prevalence of depression and anxiety disorders was determined in 41 children with Crohn's disease, 12 children with ulcerative colitis, and 52 children with cystic fibrosis, using the Kiddie-Schedule for Affective Disorders and Schizophrenia interview. The lifetime prevalence of depression was 29% in Crohn's disease, 21% in ulcerative colitis, and 11.5% in cystic fibrosis. The difference in the prevalence of depression between Crohn's disease and cystic fibrosis was significant (p less than 0.05). The lifetime and current prevalence of dysthymia was significantly greater in ulcerative colitis than Crohn's disease (p less than 0.01) or cystic fibrosis (p less than 0.01). The lifetime prevalence of atypical depression was significantly greater in Crohn's disease than cystic fibrosis (22% versus 5.8%, p less than 0.05) and was also greater in ulcerative colitis than cystic fibrosis (21% versus 5.8%, p = 0.1). There was no difference between the groups in the current prevalence of major depression or atypical depression, or in the lifetime or current prevalence of anxiety disorders.

Adolescent