1/164. Case studies: psychological test findings for two children with X-linked adrenoleukodystrophy.Psychological evaluations were performed on two children with X-linked adrenoleukodystrophy. Case histories are presented that describe the natural history of the disorder and its symptom patterns, some of which mirror psychiatric disorders of childhood. Psychological evaluation test scores for both boys are displayed in a table, and commonalities and differences are discussed. Findings are reviewed to expedite the referral process for a definitive diagnosis. Guidelines are provided regarding the differential diagnosis of adrenoleukodystrophy and other neurodegenerative disorders of childhood from the more prevalent disorders of behavior and learning that may present in a developmental clinic. Finally, recommendations for psychological interventions with the affected child and family members are provided.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
2/164. The development of a dementia process within the family context: the case of Alice.Qualitative analysis was used to analyse a diary written over a period of two years by the sister of a dementia patient. The analysis is directed at the question of how a patient and a social network respond to each other during the dementia process. The diary highlights features in the development of the dementia process which receive scant attention in empirical studies: changes in the interaction process between patient and social network; a patient's residual capacities; a caregiver's perceived rewards of caregiving. We designated three phases in the interaction process: the phase of recognition, the stable phase and the phase of destabilization. This diary illustrates how a stable phase in the interaction between patient and primary caregiver can be established. The caregiver derived rewards by noticing and using the patient's residual capacities and by a feeling of being useful. In this case caregiving is not unidirectional. The quality of future support programmes may be enhanced by combining programmes aimed to influence patient's behaviour and to support caregivers.- - - - - - - - - - ranking = 8keywords = process (Clic here for more details about this article) |
3/164. A patient with hypertrophic cardiomyopathy accompanied by right ventricular dilation of unknown cause.Hypertrophic cardiomyopathy (HCM) is a disease characterized by an unknown cause of hypertrophy in the left or right ventricle. The dilated phase of HCM shows disease conditions resembling dilated cardiomyopathy, such as ventricular dilation, thin ventricular wall, and reduction of the ejection fraction. A patient presented with left ventricular concentric hypertrophy accompanied by right ventricular dilatation of unknown cause. Right ventricular endomyocardial biopsy specimens showed characteristic myocardial disarray. Therefore, there is the possibility that the patient had right and left ventricular HCM in the process toward the dilated phase, in which dilatation first occurred in the right ventricle.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
4/164. A retired shipyard worker with rapidly progressive pulmonary interstitial fibrosis.We present a case of progressive interstitial fibrosis in a retired shipyard worker who was exposed to asbestos during the postwar era of the late 1940s and 1950s, when asbestos exposures in the workplace were not regulated. Forty years later, at 63 years of age, the patient presented with restrictive lung disease. The patient was diagnosed with asbestos-related pleural disease and parenchymal asbestosis. He remained stable for the next 7 years, but then he began to manifest rapid clinical progression, which raised the possibility of an unusual variant of asbestosis, a concomitant interstitial process, or an unrelated disease. lung biopsy was not undertaken because of the patient's low pulmonary reserve and limited treatment options. An empiric trial of oral steroids was initiated, but his pulmonary status continued to deteriorate and he died of pulmonary failure at 72 years of age. Many diseases result in pulmonary interstitial fibrosis. Ideally, open lung biopsy should be performed, but this procedure inevitably causes complications in many patients with end-stage restrictive lung disease. Furthermore, while the presence of asbestos bodies in tissue sections is a sensitive and specific marker of asbestos exposure, neither this finding nor any other charge is a marker indicative of asbestosis or the severity of asbestosis. With the enactment of the Asbestos Standard in the united states, asbestos exposures have been decreasing in this country. However, industries that produce asbestos products and wastes continue to expand in developing countries. Prevention of asbestos-related lung disease should be a global endeavor, and asbestos exposures should be regulated in both developed and developing countries.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
5/164. Changing diagnostic and therapeutic approaches to the 'Ogilvie syndrome'.The only thing that has remained unchanged about the genuinely described 'Ogilvie syndrome' is its name. Recently it was considered to be an acute colonic pseudoobstruction, a clinical entity mimicking the mechanic ileus of the distal large intestine, without organic obstruction. It is almost always secondary to other diseases. Not all details of the pathogenesis are known, but it has become clear that the direct factor leading to the disturbance of the motility is a vegetative imbalance. X-ray findings are highly characteristic and critical in the planning of treatment. The danger for the patients is the progression of the state or the long duration of the process. Conservative treatment is suitable only for early cases, without complications. In case of failure non-invasive endoscopic or endoscopically assisted minimally invasive procedures may be mandatory. These methods have seen rapid advance in recent years. Uncertain diagnoses or complications call for open surgery. cecostomy is the solution of choice anyway. The mortality is high in this group of elderly polymorbid patients. Authors compare six of their cases with data collected from the literature.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
6/164. pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia.We describe the case of a 53-year-old philadelphia-chromosome-positive woman with chronic myelogenous leukemia, who developed pulmonary alveolar proteinosis (PAP). The possible mechanism involved in the pathogenesis of PAP are discussed based on the clinical and laboratory data for this patient as well as on experimental and clinical data reported in the literature.- - - - - - - - - - ranking = 49.046176021765keywords = alveolar (Clic here for more details about this article) |
7/164. Triggering of acute alcoholic hepatitis by alpha-interferon therapy.BACKGROUND/AIMS: Alcohol may induce autoimmunity by recognition of acetaldehyde-modified proteins which may be implicated in the pathogenicity of acute alcoholic hepatitis. We report here the potential role of alpha-interferon, a potent inducer of the autoimmunity process, in inducing alcoholic hepatitis. methods: We analyzed clinical, biological, virological and histological features in two cases where alpha-interferon treatment for HCV-related hepatitis led to a marked increase in aminotransferase activities. RESULTS: alpha-interferon as treatment of HCV-related hepatitis seemed to exacerbate acute alcoholic hepatitis despite moderate alcohol consumption. In Case 1, moderate daily alcohol intake of 40 g during therapy led to biopsy-proven acute alcoholic hepatitis, while the same consumption before therapy did not. In Case 2, before treatment, the liver biopsy showed mild acute alcoholic hepatitis; aminotransferases increased during alpha-interferon therapy, although no increase in alcohol intake was observed. CONCLUSION: alpha-interferon therapy by its immunomodulatory properties could be implicated in alteration of the course of acute alcoholic hepatitis. These observations emphasize that the decision to treat with alpha-interferon when there is even moderate alcohol consumption should be carefully weighted in HCV-infected patients.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
8/164. Pituitary involvement by Wegener's granulomatosis: a report of two cases.We describe two cases of pituitary involvement by Wegener's granulomatosis. At initial presentation, or during subsequent disease "flares," a pattern of pituitary abnormality was suggested. During periods of remission, we found the pituitary returned to a nearly normal appearance. Loss of the normal posterior pituitary T1 hyper-intensity matched a clinical persistence of diabetes insipidus, suggesting there is permanent damage to this structure by the initial disease process.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
9/164. Focal, steroid responsive myositis causing dropped head syndrome.The dropped head syndrome, which occurs in a variety of neuromuscular disorders, is usually not due to an inflammatory process and generally either self-limited or nonresponsive to therapy. We present an 80-year-old woman who developed progressive neck weakness over a few months due to a focal and restricted inflammatory process involving the neck extensor muscles. She responded dramatically to treatment with immunosuppressive therapy.- - - - - - - - - - ranking = 2keywords = process (Clic here for more details about this article) |
10/164. Pulmonary sarcoidosis: calcified micronodular pattern simulating pulmonary alveolar microlithiasis.A case of sarcoidosis demonstrating an unusual pattern of profuse micronodular calcification is presented. The striking similarity with the so-called pathognomonic appearance of pulmonary alveolar microlithiasis and the progressive deterioration of pulmonary function are emphasized.- - - - - - - - - - ranking = 49.046176021765keywords = alveolar (Clic here for more details about this article) |
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