Rett syndrome: Teenagers' and young adults' activities, usage of time and responses during an ordinary week - a diary study.

Background: Little is known about the everyday life of individuals with Rett syndrome.Aim/Objective: To describe ten participants' (teenagers/young adults) activities during a period of seven days, the time-use, where and with whom the activities were performed and the participants' responses in the form of visible/audible reactions during activities.Material and method: A time-geographic self-administered diary was filled in by 63 informants (parents/support staff) and analysed using the software, DAILY LIFE 2011.Results/Findings: The most frequently reported activities were hygiene/toilet, moving around indoors, eating and getting dressed. Most time was spent in sleeping, daily care, medical health care and travel/transportation. Little time remained for receptive activities, daytime rest, physical, social/creative, communication, school/daily work and domestic chore activities, especially for the young adults. Most time was spent with staff, thereafter with families and the least time was spent with friends. The most reported response was "interested", and "opposed" was the least reported.Conclusions: Daily and medical health care activities were time consuming. Improved communication between all parties may increase participation and well-being and provide solutions for handling unpleasant activities and sedentary time.Significance: A more varied range of activities may improve the everyday life for individuals with Rett syndrome.

Occupational life trajectories in the context of chronic pain and immigration.

BACKGROUND: Persons with chronic pain report a range of occupational problems. The specific health needs of immigrants are judged to be poorly understood, and health systems are not prepared to respond adequately. Being an immigrant is regarded as a risk factor for the progression of chronic widespread pain into a state of disability. OBJECTIVES: To explore occupational life trajectories among immigrant women with chronic pain. MATERIAL AND METHODS: A qualitative content analysis was used to capture the latent meaning of experiences in individual interviews of eight women with immigrant background and chronic pain. RESULTS: An overarching theme, 'making the best of a conditional situation', representing the women's occupational life trajectories, covers the content of two categories: 'being controlled', including affected self-perception, social relations, and future prospects, and 'trying to deal with challenges', comprising a focus on resources, having trust in one's own judgements and getting help from others. CONCLUSION AND SIGNIFICANCE: The experiences demonstrate an occupational life controlled by internal and external factors and may be understood as a disrupted occupational life trajectory. This knowledge may be helpful to occupational therapists supporting immigrant women's attempts to regain a structured life despite the constant presence of pain.

Evaluation of activity limitation and digital extension in Dupuytren's contracture three months after fasciectomy and hand therapy interventions.

STUDY DESIGN: Longitudinal Case Series. INTRODUCTION: Dupytren's contracture is thought to result in digital impairments and performance disabilities. No study to date has focused on how patients with Dupuytren's contracture experience limitations in daily activities and the results after surgery. PURPOSE OF THE STUDY: Describe which activities patients with Dupuytren's contracture defined as the most disabling, how they rated their activity limitations and determine the relationship between activity limitations and digital extension before and three months after surgery and postoperative hand therapy. METHODS: Self-reported rating of activity limitations, performance, and measures of total digital extension. RESULTS: The most disabling activities were with self-care (42%), though overall performance was significantly improved following surgery and postoperative hand therapy. The total digital extension was significantly improved 81 degrees and was positively related to performance. CONCLUSIONS: The results provide new information about activities that patients with Dupuytren's contracture experience as being difficult to perform and describes positive changes in performance and range of motion. LEVEL OF EVIDENCE: 4.

Women with fibromyalgia: work and rehabilitation.

PURPOSE: To explore disability in women with fibromyalgia with a focus on their work situation. METHOD: Review of literature on work status of women with fibromyalgia. RESULTS: Major differences exist between studies in reported disability and in the percentages of women working. Limitations caused by pain, fatigue, decreased muscle strength, and endurance influence work capacity. However, 34 - 77% of the women work. Individual adjustments in the work situation are reported. When the women find a level that matches their ability, they continue to work and find satisfaction in their work role. Many factors besides degree of impairment or disability influence whether clients with longstanding pain can remain in their work role or return to work after sickness leave. CONCLUSION: The total life situation, other commitments, type of work tasks, the ability to influence the work situation, and the physical and psychosocial work environment are important factors in determining whether a person can remain in a work role. More knowledge is needed about how to adjust work conditions for people with partial work ability to the benefit of society and the individual.

Factors of importance for work disability in women with fibromyalgia.

OBJECTIVE: To identify factors of importance for women with fibromyalgia (FM) to continue working despite the limitations imposed by the symptoms. METHODS: A mail questionnaire with questions regarding social background, symptoms, sickness benefits, work situation, work conditions and adjustments, opinions regarding own work ability, and satisfaction with the situation was sent to 218 consecutive women seen at a university pain or rheumatology clinic. Answers were obtained from 176 women. RESULTS: Pain, poor quality sleep, abnormal tiredness, muscle stiffness, and increased pain after muscle exertion were frequently reported symptoms. Fifty percent of the women were employed, 15% full-time. Twenty-three percent reported FM as the reason for not working. The work situation had been changed for 58% of the working women, and 80% counted on being able to continue working. CONCLUSION: Work disability is a serious concern in FM, and the majority of women with FM have limitations in their ability to work. Our results indicate that individual adjustments in the work situation need to be made and that women who have found a level matching their ability may continue to work and find it satisfactory. Early intervention in the work situation is recommended.

Hands-on endoscopy training: an evaluation of the SADE endoscopy course. Scandinavian Association of Digestive Endoscopy.

The Scandinavian Association of Digestive Endoscopy course in advanced practical endoscopy has been arranged on a regular basis since 1980, representing a joint Scandinavian effort to offer hands-on endoscopy training with expert supervision at major endoscopy centers. The course has been aimed at gastroenterologists with previous endoscopic experience, focusing on special techniques, practical hints and expert solutions in special cases. Three days of practical training throughout Scandinavia are completed with two days of review lectures, case reports and special techniques. An inquiry among previous course participants indicates that the course has indeed been of practical value for their daily work, and that personal training as well as watching "experts at work" were useful for their ensuing endoscopic practice. The course model does, however, require close co-operation with the endoscopy units hosting the practical part of the course.

Long-term follow-up of patients with gallbladder in situ after endoscopic sphincterotomy for choledocholithiasis.

Endoscopic sphincterotomy (EST) was performed on 148 patients with common bile duct stones and intact gallbladder. The duct was cleared in 130 cases (88%). Immediate complications occurred in 23 patients (15.5%), six of whom died. In 118 of the 139 patients discharged from the hospital, the bile ducts were cleared and the gallbladder in situ. The median observation time in these 118 cases was 42 (1-97) months, during which 13 (11%) were cholecystectomized because of symptoms or acute complications due to remaining gallbladder stones, and 49 (42%) died 2-87 (median 24) months after EST. The probability (life-table) of remaining free from cholecystectomy-requiring symptoms or complications was 0.87 after 42 months. There was no association between nonfilling of the gallbladder at ERC and subsequent problems with gallbladder stones. EST for choledocholithiasis with retained gallbladder is justified for elderly and frail patients. Indefinite postponement of cholecystectomy may be warrantable, restricting surgery to patients with symptoms from gallbladder stones. This most frequently means within 2 years after EST.

Long-term follow-up after endoscopic management of retained and recurrent common duct stones.

Endoscopic sphincterotomy (EST) was attempted on 128 patients because of retained or recurrent common bile duct stones. The duct was cleared in 118 cases (92%). Early complications arose after 27 (16.5%) of 164 sphincterotomies, with haemorrhage most common. Emergency laparotomy was performed in four cases. There were four deaths within a month of EST, two of them directly attributable to EST. In seven of the 118 patients with successful EST late complications (stenosis and/or recurrent stone) appeared during a median follow-up of 57 (range 24-101) months (after less than 2 years in 6 cases). Restenosis and new stones could usually be endoscopically treated. Ascending cholangitis was not a problem, provided that the bile flow was unobstructed. EST is relatively safe, and must now be considered the procedure of choice for postcholecystectomy choledocholithiasis especially in elderly or high-risk patients. EST in young, fit patients is more controversial, and requires further detailed longitudinal analyses.

Gallbladder cancer: current status in clinical practice.

A total series of 68 unselected patients with gallbladder cancer, diagnosed during 1972 to 1981, was studied retrospectively. In 61 cases (90%), the diagnosis was histologically verified. The cardinal symptoms were local pain (87%), loss of weight (53%), and jaundice (59%). The diagnosis was established after autopsy in 22 patients (32%), and exploratory laparotomy in 22 patients (32%). Palliative surgery was carried out in 26 patients of whom seven (27%) died postoperatively. Only one patient, with an incidentally detected cancer at routine cholecystectomy, was treated radically. Four out of nine patients died within one month after PTC-drainage. The mean survival time for the whole series was 2.9 months. The longest survival was 21 months. Considering the increasing incidence of gallbladder cancer in Sweden, as well as of other cancers of the biliary system, these findings emphasize the need for intensified research. An epidemiological approach studying the correlation of gallbladder cancer with the changing trend of gallstone disease and its treatment would perhaps be fruitful.

Drainage by endoscopic endoprosthesis in biliary obstruction. A report of initial experience.

Biliary drainage by endoscopic, transpapillary insertion of endoprosthesis was attempted in 38 patients with obstructive jaundice and was successful in 25. The procedure was palliative in 17 cases and preoperative in eight. Benefit was obtained by ten of the former and seven of the latter patients, while in eight the serum bilirubin did not fall despite adequate catheterization. In two intubated patients with recurrent nonterminal jaundice, the endoprostheses were repeatedly changed; the median time to clogging was 51 (8-77) days. Cholangitis developed in 15 of the total 38 patients. Antibiotic prophylaxis, though not routinely used, is recommended for all cases with biliary stasis. Unimpeded bile flow was not ensured with 7 F endoprostheses, whose patency time tended to be short. By using sizes 10-12 F the authors hope to improve bile flow, thereby diminishing obstruction, stagnation and infection and prolonging patency. In selected cases the method may offer an alternative to the percutaneous approach, and possibly also to surgical bypass.

Relations between circulating gastrin and endocrine cell proliferation in the atrophic gastric fundic mucosa.

It has been suggested that gastrin may be a causative factor in the proliferation of gastric fundic mucosal endocrine cells, as seen in the Zollinger-Ellison syndrome and in atrophic gastritis with hypergastrinemia of antral origin. In the present study, morphometrically determined densities of endocrine cells in fundic mucosal biopsy specimens were related to basal levels of serum gastrin in 10 normal controls and 60 patients with achlorhydric fundic atrophic gastritis, of which 45 had pernicious anemia (5 with fundic mucosal carcinoid) and 15 had atrophic gastritis without pernicious anemia. The densities of fundic mucosal endocrine cells were positively related to the levels of serum gastrin (atrophic gastritis, rs = 0.65; atrophic gastritis and normal controls, rs = 0.72). The highest levels of serum gastrin were found in patients with carcinoid tumors (mean, 1659.3 pmol/l), followed by those in patients with focal hyperplasias (cluster formation) of endocrine cells (mean, 503.2 pmol/l) and those in patients without focal hyperplasias (mean, 304.4 pmol/l) (p = 0.03 and p = 0.04, respectively).

Acid secretory tests in peptic ulcer disease before and after parietal cell vagotomy.

The aim of the present study was to evaluate the role of gastric acid secretory tests in predicting ulcer recurrence in a consecutive series of patients before and up to 5 years after parietal cell vagotomy (PCV). Included in the study are 405 patients with prepyloric or duodenal ulcer disease (57 recurrences). There were no differences in acid secretion in the group of patients with pyloric or prepyloric ulcer disease, with or without recurrences. Thus pre- and post-operative acid secretory tests are of no value in predicting the outcome of PCV in this group of patients. In the duodenal ulcer group both postoperative basal acid output and peak acid output after pentagastrin and after insulin were higher in patients with recurrences. The two best discriminatory values were the ratio between the preoperative and the 6-week value of basal acid output and the 6-week value of peak acid output after insulin. The results of the study suggests that the ratio of the basal output before surgery and 6 weeks after surgery is enough for judging the acid reduction after PCV and that only in the case of a recurrence should an acid stimulation test be used.

Endocrine cell proliferation and carcinoid development: a review of new aspects of hypergastrinaemic atrophic gastritis.

The accumulating evidence of an association between antrum-sparing hypergastrinaemic atrophic gastritis, frequently associated with pernicious anaemia, and the occurrence of gastric carcinoid tumours is briefly reviewed. The development of argyrophil cell carcinoid tumours in the atrophic fundic mucosa seems to be related to argyrophil cell hyperplasia caused by hypergastrinaemia. Epidemiologic considerations indicate that the gastric carcinoid generally is underdiagnosed and that the incidence of this tumour is higher than previously recognized. The clinical relevance of minute gastric carcinoids, or endocrine cell 'adenomas', is obscure. However, larger tumours should be regarded as potentially malignant. These findings are relevant to the aspect of long-term medically induced achlorhydria leading to hypergastrinaemia.

Pancreatic cancer.

During 1972-1981, a total of 177 individuals were diagnosed as having pancreatic cancer at the Central Hospital of Eskilstuna, Sweden. The mean crude incidence rate was 15.0 X 10(-5) year-1. Only 56% of the patients had the diagnosis made prior to death. 63% of the cancers had advanced beyond therapeutic range. Symptoms compatible with upper abdominal disease for more than six months prior to diagnosis occurred in 27% of the cases, and 15% had sought medical advice more than 6 months prior to diagnosis. Radical surgery was performed on 5 patients of whom one died postoperatively, and 61 patients (34%) underwent palliative treatment. The postoperative mortality rate after palliative treatment was 28%. The mean survival time for the whole series was 2.6 months, and 13% survived for one year. One patient survived for 2 years after palliative treatment. It is concluded that the only means by which the prognosis for patients with pancreatic cancer may be improved is to define populations at high risk and to develop more specific and sensitive diagnostic methods.

Ulcer healing and relapse prevention by ranitidine in peptic ulcer disease.

Ranitidine, 300 mg daily, was given to 92 patients with duodenal ulcer (DU), 38 with prepyloric ulcer (PPU), and 21 with gastric corporeal ulcer (GCU). The healing rates at 4 weeks differed for the different types of ulcers (P less than 0.01), being 91% for DU, 68% for PPU, and 81% for GCU. After established ulcer healing, maintenance treatment with either ranitidine, 100 mg twice daily or 150 mg at night, or placebo was given for 1 year or until ulcer relapse in a total of 108 patients--71 with DU, 24 with PPU, and 13 with GCU. There were no significant differences in relapse rates between the two groups treated with active drug or between the three ulcer groups. However, the overall relapse rate in the active drug groups was 16%, against 72% in the placebo group (P less than 0.001).

Recurrence after parietal cell vagotomy for peptic ulcer disease.

The value of parietal cell vagotomy as a safe surgical procedure in the treatment of chronic peptic ulcer disease has been seemingly limited by the high recurrence rate usually reported. In this 10 year experience of 405 consecutive parietal cell vagotomy operations, 57 patients with recurrent ulceration were evaluated to try to answer the questions of where and why recurrent ulceration appears and how to treat it. Patients with pyloric or prepyloric ulcer disease had recurrence of disease earlier than did patients with chronic duodenal ulcer disease. Consequently, pyloric or prepyloric ulcer patients had a higher recurrence rate in the early postoperative period than chronic duodenal ulcer patients, but this difference disappeared after the seventh postoperative year. Patients with recurrent chronic duodenal ulcer disease have high postoperative acid secretion levels, indicating incomplete vagotomy as a causative factor. Patients with recurrent pyloric or prepyloric ulcer disease have postoperative acid secretion similar to that of patients without recurrence, suggesting another etiologic factor. Thirty of 57 patients with recurrent ulcer had successful medical treatment. A conservative attitude towards recurrences is justified, particularly when symptoms are few and the postoperative acid secretion tests indicate complete or partial vagotomy. Surgical therapy consisting of truncal vagotomy and pyloroplasty in cases of incomplete vagotomy and antrectomy in cases of complete vagotomy should be reserved for patients with symptoms and a disease course that cannot be controlled by conservative treatment.

Parietal cell vagotomy in the surgical treatment of chronic duodenal, pyloric and prepyloric ulcer disease.

During the 1970s, parietal cell vagotomy (PCV) gradually became accepted as a suitable method in the surgical treatment of prepyloric, pyloric and duodenal ulcer disease. This study reports the data from a study of 405 consecutive patients with chronic ulcer disease treated with PCV. Mortality was low (0.5%) and there were few postoperative sequelae (periodic loose stools in 2% and mild dumping in 2%). Reduction in basal acid output was 75% and in pentagastrin stimulated acid secretion 50%. The ulcer recurrence rate was initially higher in pyloric-prepyloric (PU/PPU) than in duodenal ulcer disease but after the seventh postoperative year this difference was no longer statistically significant. The accumulated recurrence rate was 17.5%. Few side effects and a comparatively low recurrence rate makes PCV the method of choice in the surgical treatment of peptic ulcer disease.

Gastric endocrine cell hyperplasia and carcinoid tumors in pernicious anemia.

Endoscopic screening in 123 patients with pernicious anemia (PA) yielded 4 patients with solitary and 1 patient with multiple gastric carcinoid tumors. Quantitative histologic studies of multiple standardized biopsy specimens showed a significantly increased number of fundic mucosal argyrophil endocrine cells in 40 patients with PA when compared with 15 patients with simple fundic atrophic gastritis (p = 0.002) or 8 normal controls (p = 0.0001). Patients with simple atrophic gastritis had increased numbers of fundic mucosal argyrophil cells as compared with normal controls (p = 0.02). A significant difference was also noticed in the number of antral mucosal argyrophil cells between patients with PA and normal controls (p = 0.01), but not between patients with PA and patients with simple atrophic gastritis. It is concluded that, in addition to having hyperplasia of gastric mucosal argyrophil endocrine cells, patients with PA run an increased risk of developing gastric argyrophil cell carcinoid tumors, which should be regarded as potentially malignant.

Endoscopic sphincterotomy in the treatment of choledocholithiasis and ampullar stenosis. Experience with 202 patients.

Endoscopic sphincterotomy (EST) was performed on 202 patients (265 EST) in the 5-year period 1978-1983 because of choledocholithiasis or benign stenosis of the ampulla of Vater. The indications for EST in choledocholithiasis included residual or recurrent stone(s) in 80 cholecystectomized patients, and 96 had choledocholithiasis with the gallbladder in situ. Ductal calculi passed spontaneously after EST in 87 cases (50%) and were actively extracted in 73 (41%). Ductal clearance failed in 16 patients. The overall success rate thus was 91%. Ampullar stenosis was successfully treated in 26 patients. Sphincter stenosis after EST was diagnosed in one patient. Immediate complications of EST arose in 31 patients (11.7%), one of whom died. Emergency laparotomy was required in three cases (1.1%). EST with or without stone extraction is a relatively safe procedure for managing choledocholithiasis in high-risk patients before cholecystectomy is considered, and also for stones retained or reformed after cholecystectomy. Endoscopic treatment may offer appreciable clinical and financial benefits by reducing morbidity and mortality rates and shortening hospitalization and convalescence time.