Mapping CushingQOL scores to EQ-5D utility values using data from the European Registry on Cushing's syndrome (ERCUSYN).

PURPOSE: To construct a model to predict preference-adjusted EuroQol 5D (EQ-5D) health utilities for CS using the disease-specific health-related quality of life measure (CushingQOL). METHODS: Data were obtained from the European Registry on CS (ERCUSYN). ERCUSYN is a web-based, multicenter, observational study that enrolled 508 CS patients from 36 centers in 23 European countries. Patients included in the study completed both the EQ-5D and the disease-specific CushingQOL questionnaire. Socio-demographic and clinical data were also collected. The UK tariff values were used to calculate EQ-5D utility scores. Various predictive models were tested, and the final model was selected based on four criteria: explanatory power (adjusted R-squared), consistency of estimated coefficients (sign and parameter estimation), normality of prediction errors (mean error, mean absolute error, root mean squared error), and parsimony. RESULTS: For the mapping analysis, data were available from a total of 129 patients. Mean (SD) age was 43.1 (13) years, and the sample was predominantly female (84.5 %). Patients had a mean (SD) CushingQOL score of 39.7 (17.1) and a mean (SD) 'tariff' value on the EQ-5D of 0.55 (0.3). The model which best met the criteria for selection included the intercept and 3 CushingQOL's questions and had an R(2) of 0.506 and a root mean square error of 0.216. CONCLUSIONS: It was possible to find a mapping function which successfully predicted the EQ-5D UK utilities from disease-specific CushingQOL scores. The function may be useful in calculating EQ-5D scores when EQ-5D data have not been gathered directly in a study.

Prevalence of hiatal hernia by blinded multidetector CT in patients with idiopathic pulmonary fibrosis.

Hiatal hernia (HH) is associated with gastro-oesophageal reflux (GOR) and/or GOR disease and may contribute to idiopathic pulmonary fibrosis (IPF). We hypothesised that HH evaluated by computed tomography is more common in IPF than in asthma or chronic obstructive pulmonary disease (COPD), and correlates with abnormal GOR measured by pH probe testing. Rates of HH were compared in three cohorts, IPF (n=100), COPD (n=60) and asthma (n=24), and evaluated for inter-observer agreement. In IPF, symptoms and anti-reflux medications were correlated with diffusing capacity of the lung for carbon monoxide (D(L,CO)) and composite physiologic index (CPI). HH was correlated with pH probe testing in IPF patients (n=14). HH was higher in IPF (39%) than either COPD (13.3%, p=0.00009) or asthma (16.67%, p=0.0139). The HH inter-observer κ agreement was substantial in IPF (κ=0.78) and asthma (κ=0.86), and moderate in COPD (κ=0.42). In IPF, HH did not correlate with lung function, except in those on anti-reflux therapy, who had a better D(L,CO) (p<0.03) and CPI (p<0.04). HH correlated with GOR as measured by DeMeester scores (p<0.04). HH is more common in IPF than COPD or asthma. In an IPF cohort, HH correlated with higher DeMeester scores, confirming abnormal acid GOR. Presence of HH alone was not associated with decreased lung function.

Alarmingly high prevalence of obesity in haemophilia in the state of Mississippi.

The state of Mississippi has consistently been ranked as the state with most number of obese people in the United States with prevalence rates of >30%. Our aims in this study were to estimate the prevalence of overweight and obesity in children and adults diagnosed with haemophilia in Mississippi, and to assess whether race/ethnicity and the severity of haemophilia are important risk factors. A retrospective chart review was performed for all haemophilic patients seen at the Mississippi Hemophilia Treatment Center. Patients were classified into two major age groups: age 2-19.9 years and > or =20 years. Body mass index (BMI) was calculated from the height and weight in kg m(-2) from the last clinic visit. Out of a total of 132 haemophilic patients, 61% were white and 37% were African American. Overall, 51% of the haemophilic patients were either obese or overweight. The prevalence of obesity in the adult (> or =20 years old) haemophilic patients was 36% and an additional 32% were overweight. A significantly greater proportion of patients >20 years old were overweight or obese as compared with the patients in the 2-19.9 year age range (P < 0.002). However, race/ethnicity and severity of haemophilia were not significant risk factors for overweight and obesity. There is a very high prevalence of obesity in the Mississippi haemophilic population, especially in adults. Particular attention at clinic visits should be paid to the BMI in order to identify patients that are overweight or obese to allow for early and appropriate intervention.

Steroid contraceptive use and cervical dysplasia: increased risk of progression.

In a prospective study of women with dysplasia of the cervix, there was an increase in severity of dysplasia and of conversion to cancer in situ in users of the contraceptive pill compared with users of other contraceptive methods. There was a delay in this adverse response. Nonreversal of dysplasia within the first 6 months of pill use is predictive of progression after prolonged exposure.

Structural correlates of seizure behavior in the mongolian gerbil.

Hippocampi of seizure-sensitive and seizure-resistant Mongolian gerbils were examined in search of structural correlates of seizure behavior. In animals with well-established seizure histories, differences were found in both presynaptic and postsynaptic structures. Seizing animals had less dense dendritic spines, a greater proportion of mossy tuft area devoted to presynaptic vesicles, and a smaller proportion devoted to spines. The possible relationship of these findings to epilepsy is discussed.

Evaluation of Papanicolaou smear and effect of sample biopsy in follow-up of cervical dysplasia.

In a prospective study of women with dysplasia, a superficial sampling biopsy of the cervix was carried out as a periodic check of the cytological findings. The relationship of smear and biopsy results was evaluated, as well as the effect of the biopsy procedure on the subsequent course of dysplasia. We found sufficiently close agreement between smear and biopsy results to conclude that smears and biopsies measure similar aspects of dysplasia. It would appear unwise, however, to rely on Papanicolaou smears exclusively in following women with a history of dysplasia, since negative smears in such women may occasionally show dysplasia in the corresponding biopsy. Periodic corroborative biopsy procedures are therefore indicated in the follow-up care of women with a history of dysplasia. There was no evidence that a superficial sampling biopsy significantly altered the short-term course of dysplasia. There was also no evidence of a cumulative effect of repeated sampling biopsies. These results do not rule out possible effects of other forms of biopsy procedures and schedules on the subsequent course of dysplasia.

Therapist characteristics and the outcome of treatment in schizophrenia.

The broad task of identifying and characterizing specific components of personality and behaviours of therapists that may be differentially helpful in the treatment of schizophrenia still remains to be addressed. This report presents data systematically collected in the course of a controlled study of the outcome of five different treatment methods in schizophrenia. Therapists seem to play a significant role in determining the outcome of the treatment of schizophrenia by drugs and by psychotherapy plus drugs. The salient therapist behaviors that seem to make a difference in outcome are yet to be identified and studied. The A-B dimension as customarily defined seems of little value for this task. The findings show a distinct need to identify cognitive and affective personality characteristics of the therapist relevant to eliciting patient cooperation, and the degree of knowledge and sophistication in the use of particular methods of treatment necessary for good results.

Somatization in the community.

We examined the prevalence of somatization disorder symptoms elicited with the Diagnostic Interview Schedule in 3132 community respondents interviewed in Los Angeles by the Epidemiologic Catchment Area program. The variables age, gender, ethnic background, and the presence of a psychiatric diagnosis significantly influenced the number of somatization symptoms reported. An introductory review on conceptual and nosological aspects of somatization phenomena led to the formulation of a less-restrictive operational definition of the somatizer. We found that 4.4% of the respondents met criteria for this abridged cutoff score of somatization, whereas only 0.03% of the respondents met criteria for the full DSM-III somatization disorder diagnosis. This abridged cutoff score was associated with sociodemographic factors and psychiatric diagnosis in the direction predicted.

The Spanish Diagnostic Interview Schedule. Reliability and comparison with clinical diagnoses.

The National Institute of Mental Health Diagnostic Interview Schedule (DIS) was translated into Spanish. The reliability of the Spanish instrument, its equivalence to the English version, and its agreement with clinical diagnoses were examined in a study of 90 bilingual (English-and Spanish-speaking) and 61 monolingual (Spanish-speaking only) patients from a community mental health center. The study design involved two independent DIS administrations and one independent clinical evaluation of each subject.

Treatment of Bell palsy with prednisone: a prospective, randomized study.

Two hundred thirty-nine patients with Bell palsy were randomly distributed into prednisone-treated and control groups. Patients were followed until complete recovery or for 1 year. In the steroid-treated and control groups, respectively, incomplete recovery of facial strength occurred in 12 percent and 20 percent; motor synkinesis in 9 percent and 15 percent; autonomic synkinesis in 1 percent and 10 percent; and electromyographic evidence of severe denervation in 9 percent and 17 percent. The differences reached statistical significance only for autonomic synkinesis. In the total series recovery of facial strength was incomplete in 16 percent. Residual weakness was mild in 14 percent and moderate in 2 percent. No patient remained with severe weakness. Age, hyperacusis, and severity of the initial palsy were associated with an increased risk of poor outcome.

Lipoamide dehydrogenase: rapid heat inactivation in platelets of patients with recessively inherited ataxia.

The activity of lipoamide dehydrogenase was abnormally heat-labile in homogenized platelets from seven patients with as recessive ataxia conforming to the syndrome of Friedreich ataxia or clinical variants. Taken together, the abnormality and previous findings of low activity and abnormal kinetic properties are compatible with a change in the conformation of the enzyme in these patients.