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Radiosurgery for residual or recurrent nonfunctioning pituitary adenoma.

OBJECT: Nonfunctioning pituitary adenomas comprise approximately 30% of all pituitary tumors. The purpose of this retrospective study is to evaluate the efficacy and role of gamma knife radiosurgery (GKS) in the management of residual or recurrent nonfunctioning pituitary adenomas. METHODS: A review was conducted of the data obtained in 42 patients who underwent adjuvant GKS at the University of Pittsburgh between 1987 and 2001. Prior treatments included transsphenoidal resection, craniotomy and resection, or conventional radiotherapy. Endocrinological, ophthalmological, and radiological responses were evaluated. The duration of follow-up review varied from 6 to 102 months (mean 31.2 months). Fifteen patients were observed for more than 40 months. The mean radiation dose to the tumor margin was 16 Gy. Conformal radiosurgery planning was used to restrict the dose to the optic nerve and chiasm. Tumor control after GKS was achieved in 100% of patients with microadenomas and 97% of patients with macroadenomas. Gamma knife radiosurgery was equally effective in controlling adenomas with cavernous sinus invasion and suprasellar extension. No patient developed a new endocrinological deficiency following GKS. One patient's tumor enlarged with an associated decline in visual function. Another patient experienced a deterioration of visual fields despite a decrease in tumor size. CONCLUSIONS: Gamma knife radiosurgery can achieve tumor control in virtually all residual or recurrent nonfunctioning pituitary adenomas. Dose sparing facilitates tumor management even when the adenoma is close to the optic apparatus or invades the cavernous sinus.

Adenoma↗

A model of disease-specific worry in heritable disease: the influence of family history, perceived risk and worry about other illnesses.

Disease-related worry is associated with family history and perceived risk of that disease; however, the influences of general risk perceptions and tendencies to worry about diseases have been neglected in the literature. This study investigates a model of disease-specific worry which includes family history, disease-specific perceived risk, and perceived risk for and worry about other diseases. Participants completed a survey assessing these variables in relation to several heritable diseases. Structural equation modeling found that family history predicted disease-specific perceived risk but not perceived risk for other diseases. Disease-specific perceived risk predicted disease-specific worry and worry about other diseases. Perceived risk for other diseases predicted worry about other diseases and disease-specific perceived risk but not disease-specific worry. Disease-specific worry predicted worry about other diseases. This model was supported across several diseases and indicates that disease-specific and general considerations of risk influence worry about a disease and should be considered in interventions.

Adult↗

Is coeliac disease more prevalent in young adults with coexisting Type 1 diabetes mellitus and autoimmune thyroid disease compared with those with Type 1 diabetes mellitus alone?

AIM: It is known that patients with Type 1 diabetes mellitus are more prone to develop coeliac disease and that autoimmune thyroid disease occurs more frequently in patients with coeliac disease. We therefore assessed whether coeliac disease, either known or occult, occurs more frequently in young/middle aged adults with Type 1 diabetes and coexisting autoimmune thyroid dysfunction than in adults with Type 1 diabetes alone. METHODS: The prevalence of known coeliac disease was assessed in 509 (301 males, aged 16-55 years) patients with Type 1 diabetes, 28 (5.5%) of whom had treated autoimmune thyroid disease. In a second study 38 patients with Type 1 diabetes and coexisting autoimmune thyroid disease along with 112 patients with Type 1 diabetes alone were then screened for coeliac disease using serum IgA endomysial antibodies and IgA gliadin antibodies. RESULTS: Seven of the 509 patients (1.4%) had been diagnosed with coeliac disease and two of these had later developed autoimmune thyroid disease (both hypothyroid). The subsequent screening exercise found that one of the 38 patients with both Type 1 diabetes and thyroid disease had positive endomysial antibodies on screening. However, duodenal biopsy was negative for coeliac disease. There were two patients with positive endomysial antibodies in the group of 112 patients with diabetes only. Both had duodenal biopsy but only one was consistent with coeliac disease. CONCLUSION: The prevalence of known coeliac disease in this young adult Type 1 diabetes clinic in North-west England was 7/509 (1.4%). Two of these seven patients with coeliac disease were from the group of 28 who had autoimmune thyroid disease as well. Therefore we suggest that patients with known coeliac disease and Type 1 diabetes should be screened for autoimmune thyroid disease. The second screening study then found 3/150 (2%) to have a serological marker for coeliac disease. However, patients with both Type 1 diabetes and autoimmune thyroid disease were not more likely to have occult coeliac disease compared with those with Type 1 diabetes only.

Adolescent↗

[Interstitial lung disease in mixed connective tissue disease].

INTRODUCTION: The authors analyzed the incidence of interstitial lung disease in mixed connective tissue disease. They were seeking an answer to the following problems: the nature of the pathological course of mixed connective tissue disease complicated by and the therapy to be used in interstitial lung disease. PATIENTS AND METHODS: 179 patients were followed up during a period of 15.9 +/- 6.1 years. Interstitial lung disease was diagnosed using high resolution computed tomography. The diagnosis of interstitial lung disease was not obvious in 5 patients thus open lung biopsy was performed, which confirmed common interstitial pneumonitis. The patients were followed-up, and the data of computed tomography and respiratory function tests were detected 6 months, and then 4 years after the acute lung disease complicated by mixed connective tissue disease. RESULTS: Out of the 179 mixed connective tissue disease patients 96 (53.6%) had interstitial lung disease. The onset of interstitial lung disease was the most frequent in the 2-4 years of the disease. Four years after the first appearance of interstitial lung disease severe fibrosis was diagnosed in 24 patients (25%). A honey comb formation in the lung developed only in one patient. For the treatment of interstitial lung disease, corticosteroid treatment had to be combined with cyclophosphamide in 51 cases. In 4 patients (24%), pulmonary arterial hypertension evolved 2-4 years following interstitial lung disease. The high pulmonary arterial pressure decreased using pulsed corticosteroid treatment, cyclophosphamide, prostacyclin analogue, anticoagulants therapy and the 4 patients stay alive. The pulmonary arterial hypertension was caused by obliterative vasculopathy. CONCLUSION: Pulmonary involvement is found in more than half of the patients with mixed connective tissue disease. Early diagnosis of interstitial lung disease is possible by computed tomography. Interstitial lung disease can be treated by the combination of corticosteroids and cyclophosphamide. The authors were the first to detect the coexistence of interstitial lung disease and pulmonary arterial hypertension in mixed connective tissue disease. Subsequent respiratory alterations in these patient necessitate regular patient follow up.

Adult↗

Autoimmune thyroid diseases and coeliac disease.

BACKGROUND: Coeliac disease may be associated with a wide variety of diseases of known or suspected immunological aetiology. OBJECTIVE: To screen for both (a) the prevalence of coeliac disease in adults with autoimmune thyroid diseases, and (b) thyroid impairment among adults with coeliac disease, as compared to sex- and age-matched controls. DESIGN: Prospective cohort study. SETTING: University teaching hospital. PATIENTS: A total of 152 consecutive adults with autoimmune thyroid diseases, 185 consecutive coeliac disease patients (53 newly diagnosed and 132 already on a gluten-free diet) and 170 sex- and age-matched controls. METHODS: Screening for coeliac disease was done by means of IgA anti-endomysium antibodies, detected by indirect immunofluorescence on monkey oesophagus. Patients with positive sera underwent duodenal biopsy for diagnostic confirmation. Thyroid function was assessed by measuring the levels of serum thyroid-stimulating hormone, free T3, free T4, thyroperoxidase and thyroid microsome antibodies. Autoimmune thyroid diseases were classified according to the American Thyroid Association guidelines. RESULTS: Anti-endomysium antibodies were positive in five of 152 autoimmune thyroid disease patients (3.3%) and coeliac disease was histologically confirmed in all: this prevalence is 10-fold higher than expected. Only one patient presented with gastrointestinal complaints, but iron deficiency was found in three and alterations at bone mineralometry in all. The overall prevalence of autoimmune thyroid diseases was significantly higher (38/185, 20.5%) in coeliac patients than in controls (19/170, 11.2%). The prevalence of both hypo- and hyperthyroidism was not different from that of controls, while the prevalence of autoimmune thyroid disease with euthyroidism was 13% in patients and 4.7% in controls. CONCLUSIONS: The association of coeliac disease with autoimmune thyroid disease is not surprising as they share common immunopathogenetic mechanisms. It is advisable to screen autoimmune thyroid disease patients for coeliac disease as there is an increased risk for gluten intolerance. In contrast, thyroid function assessment in coeliac disease patients is probably less justified, although the need for a strict clinical follow-up of those patients with euthyroidism and autoimmune thyroid disease, who could develop overt thyroid impairment, remains an open question.

Adolescent↗

Measuring loss of life, health, and income due to disease and injury: a method for combining morbidity, mortality, and direct medical cost into a single measure of disease impact.

The impact of disease on a population includes illness, death, and medical care cost. Information on all three may be combined in a disease impact scale. The disease impact for a given condition can be defined as the sum of (a) the years of life lost before age 75 per 100,000 population (adjusted to reflect causes of death up to age 100); (b) the person-years of complete disability per 100,000 population, and (c) the direct medical costs in years of average annual personal income per 100,000 population.The sum of (a), (b), and (c)-disease impact in person years per 100,000 population-can be used to compare one disease with another, to estimate the potential effect of programs for risk alteration, and to measure the outcome of planned or accidental changes in society. The data necessary to calculate disease impact are becoming available in many States.In Minnesota, the total disease impact in 1978 was approximately 26,000 person-years per 100,000 population per year. The disease catgories in the International Classification of Diseases, Adapted, Eighth Revision, with the highest disease impact in the State were circulatory diseases (23.7 percent), injury and poisoning (10.9 percent), respiratory system (9.3 percent), neoplasms (9.0 percent); musculoskeletal system and connective tissue (8.8 percent), digestive system diseases (7.5 percent), and nervous system and sense organ diseases (5.8 percent). Circulatory diseases ranked first in morbidity, mortality, and cost, but the rankings for several other categories varied according to the parameter being considered.Use of a disease impact scale such as the one developed in Minnesota avoids dependence on a single parameter such as mortality or cost in making program decisions. In contrast to economic analyses of disease impact, it does not require estimates of discount rates, future rates of inflation, or salaries for homemakers, students, and children. Although the results of present calculations are only approximate, they provide a methodological framework within which correctable deficiencies in data collection methods are readily apparent. The disease impact scale is intended to be a component of a comprehensive disease surveillance system that includes measures of disease impact, the prevalence of risk factors for diseases, and the availability of health resources.

Costs and Cost Analysis↗

Criteria for development of animal models of diseases of the respiratory system: the comparative approach in respiratory disease model development.

Advances in the understanding of human respiratory disease can come from careful clinical studies of the diseases as they occur in man, but such studies are naturally limited in terms of experimental manipulation. In the last 2 decades, an increasingly complex plethora of experimental respiratory disease models has been developed and utilized by investigators, but relatively less attention has been paid to the naturally occurring pulmonary diseases of animals as potential models. This paper is aimed at presenting selected examples of spontaneous pulmonary disease in animals that may serve as exploitable models for human chronic bronchitis, bronchiectasis, emphysema, interstitial lung disease, hypersensitivity pneumonitis, hyaline membrane disease, and bronchial asthma. Chronic bronchitis in dogs is characterized by chronic cough, excessive mucus production, and chronic inflammatory changes in bronchial walls. The disease affects mainly smaller-breed dogs of middle age or older. Equine chronic bronchitis tends to be a small airway disease with marked goblet cell proliferation and excessive mucus production, which may be accompanied by alveolar emphysema. Many animals develop bronchiectasis or bronchiolitis obliterans secondary to chronic suppurative bronchopneumonia, but chronic respiratory disease (CRD) of rats may be the most useful model of bronchiectasis. Models for emphysema must include actual alveolar destruction and ideally should be accompanied by appropriate pathophysiologic decrements. Many animals occasionally develop emphysema, but the disease has not been well documented, except possibly in horses. The interstitial lung diseases of man represent a complicated and poorly understood group of entities and near-entities. The same is true for animals, although interstitial lung disease in animals is much less common than bronchopneumonia. Cattle seem prone to develop interstitial lesions. Proliferative interstitial pneumonia of cattle includes many morphologic similarities to the spectrum of human interstitial pneumonitides. Fibrosing alveolitis of cattle is a morphologic end point that may have its origins in different forms of interstitial injury. Hypersensitivity pneumonitis has been best detailed in cattle and in horses and is clinically, etiologically, immunologically, and morphologically similar to the disease in man. Hyaline membrane disease has been poorly documented in animals, with the possible exception of the neonatal respiratory distress syndromes of foals and piglets. Bronchial asthma is similarly not well established as a spontaneous disease in animals, although experimental models exist. Eosinophilic bronchiolitis of cattle may represent a useful asthma model but has been poorly detailed. In order to make them useful as models, more attention should be paid to detailing the clinical, morphologic, and etiologic aspects of these naturally occurring animal pulmonary diseases.

Animals↗

Prevalence of coeliac disease in Italian patients affected by Addison's disease.

OBJECTIVE: It is well known that coeliac disease is associated with autoimmune endocrine diseases, such as autoimmune thyroid disease and insulin-dependent diabetes mellitus. Recently, coeliac disease has been shown in approximately 10% of patients with autoimmune Addison's disease. Addison's disease is the most common cause of primary adrenocortical insufficiency and it shares several clinical features with coeliac disease. Although hyperpigmentation and hypotension are the most specific signs, gastrointestinal symptoms are common and can be the first complaints of the patients. The aim of our study was to investigate the prevalence of coeliac disease in Italian patients with Addison's disease. MATERIAL AND METHODS: Seventeen consecutive patients affected by Addison's disease (14 F, mean age 53.9 years, range 26-79 years) were enrolled in the study. Eleven of them were affected by Addison's disease associated with autoimmune thyroid disease and/or insulin-dependent diabetes mellitus; the other 6 patients were suffering from isolated Addison's disease. Diagnosis had been performed at the age of 40.5 years (range 23-55). Steroid treatment had already been started in 16 of the patients. Endomysial antibodies were tested in all of them and a duodenal biopsy was taken in those found to be positive for antiendomysial antibody (EMA). RESULTS: One out of 17 patients was found to be EMA positive. Duodenal biopsy confirmed the diagnosis of coeliac disease by showing subtotal villous atrophy. CONCLUSIONS: Although we studied only a small sample, our preliminary results confirmed that Addison's disease is associated with coeliac disease, being present in 5.9% of patients with Addison's disease. Since the symptoms can be similar and treatment of Addison's disease can mask coeliac disease, this association should always be actively investigated.

Addison Disease↗

A taxonomy for disease management: a scientific statement from the American Heart Association Disease Management Taxonomy Writing Group.

BACKGROUND: Disease management has shown great promise as a means of reorganizing chronic care and optimizing patient outcomes. Nevertheless, disease management programs are widely heterogeneous and lack a shared definition of disease management, which limits our ability to compare and evaluate different programs. To address this problem, the American Heart Association's Disease Management Taxonomy Writing Group developed a system of classification that can be used both to categorize and compare disease management programs and to inform efforts to identify specific factors associated with effectiveness. METHODS: The AHA Writing Group began with a conceptual model of disease management and its components and subsequently validated this model over a wide range of disease management programs. A systematic MEDLINE search was performed on the terms heart failure, diabetes, and depression, together with disease management, case management, and care management. The search encompassed articles published in English between 1987 and 2005. We then selected studies that incorporated (1) interventions designed to improve outcomes and/or reduce medical resource utilization in patients with heart failure, diabetes, or depression and (2) clearly defined protocols with at least 2 prespecified components traditionally associated with disease management. We analyzed the study protocols and used qualitative research methods to develop a disease management taxonomy with our conceptual model as the organizing framework. RESULTS: The final taxonomy includes the following 8 domains: (1) Patient population is characterized by risk status, demographic profile, and level of comorbidity. (2) Intervention recipient describes the primary targets of disease management intervention and includes patients and caregivers, physicians and allied healthcare providers, and healthcare delivery systems. (3) Intervention content delineates individual components, such as patient education, medication management, peer support, or some form of postacute care, that are included in disease management. (4) Delivery personnel describes the network of healthcare providers involved in the delivery of disease management interventions, including nurses, case managers, physicians, pharmacists, case workers, dietitians, physical therapists, psychologists, and information systems specialists. (5) Method of communication identifies a broad range of disease management delivery systems that may include in-person visitation, audiovisual information packets, and some form of electronic or telecommunication technology. (6) Intensity and complexity distinguish between the frequency and duration of exposure, as well as the mix of program components, with respect to the target for disease management. (7) Environment defines the context in which disease management interventions are typically delivered and includes inpatient or hospital-affiliated outpatient programs, community or home-based programs, or some combination of these factors. (8) Clinical outcomes include traditional, frequently assessed primary and secondary outcomes, as well as patient-centered measures, such as adherence to medication, self-management, and caregiver burden. CONCLUSIONS: This statement presents a taxonomy for disease management that describes critical program attributes and allows for comparisons across interventions. Routine application of the taxonomy may facilitate better comparisons of structure, process, and outcome measures across a range of disease management programs and should promote uniformity in the design and conduct of studies that seek to validate disease management strategies.

Cardiology↗