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While person-centred care has gained increasing prominence in recent decades as a goal for healthcare systems, mainstream implementation remains tentative and there is a lack of knowledge about how to develop person-centred care in practice. This study therefore aimed to explore what may be required in order for person-centred care programmes to be successful. The study used an ethnographic method of data collection. This consisted of closely following an implementation programme on a medical emergency ward in a Swedish hospital. Data consisted of participant observation and informal interviews with healthcare providers and their management leaders while they were in the process of training to use person-centred care. These interlocutors were using action learning methods under the guidance of facilitators. Our findings revealed that although the programme resulted in some of the processes that are central for person-centred care being developed, organisational factors and a lack of attention to ethics in the programme counteracted these positive effects. The study highlights the importance of facilitating mechanisms to produce desired results. These include management leaders' learning about the dynamic and collective nature of learning processes and change. They also include allowing for inter-professional dialogue to enable managers and professionals to reflect deeply on professional boundaries, disciplinary knowledge and power relations in their teams. Teamwork is essential for the development of person-centred care and documentation, in accordance with this specific implementation programme, is also indispensable. The space for inter-professional dialogue should also accommodate their various perspectives on the aims of care and organizational reality.

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Quantitative microbial risk assessments (QMRAs) often lack data on water quality leading to great uncertainty in the QMRA because of the many assumptions. The quantity of waste water contamination was estimated and included in a QMRA on an extreme rain event leading to combined sewer overflow (CSO) to bathing water where an ironman competition later took place. Two dynamic models, (1) a drainage model and (2) a 3D hydrodynamic model, estimated the dilution of waste water from source to recipient. The drainage model estimated that 2.6% of waste water was left in the system before CSO and the hydrodynamic model estimated that 4.8% of the recipient bathing water came from the CSO, so on average there was 0.13% of waste water in the bathing water during the ironman competition. The total estimated incidence rate from a conservative estimate of the pathogenic load of five reference pathogens was 42%, comparable to 55% in an epidemiological study of the case. The combination of applying dynamic models and exposure data led to an improved QMRA that included an estimate of the dilution factor. This approach has not been described previously.

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The hereditary spastic paraplegias (HSP) are heterogeneous neurodegenerative disorders characterized by progressive spasticity and weakness in the lower limbs. Axonal loss in the long corticospinal tracts has been shown. Supraspinal symptoms and findings in the most common dominant HSP type, SPG4, support the theory that the disease also causes cerebral neuronal damage in specific parts of the brain. To investigate whether SPG4-HSP is associated with neuronal biochemical changes detectable on MR spectroscopy (MRS), single-voxel proton MRS of the brain was performed in eight subjects from four families with genetically confirmed SPG4-type HSP and eight healthy age-matched controls. Volumes of interest (VOI) were located in the frontal white matter and motor cortex. N-acetyl-aspartate-to-creatine ratio (NAA/Cr), N-acetyl-aspartate-to-choline (NAA/Cho), cholin to creatin (Cho/Cr) and myo-inositol-to-creatine (Ins/Cr) ratios were calculated for both locations. Neuropsychological tests were performed to support the neuroradiological findings. The Cho/Cr ratio in motor cortex (MC) of SPG4-HSP subjects was significantly lower than in controls. This reduction of the Cho/Cr ratio in SPG4 subjects was significantly associated with age-related verbal learning- and memory (CVLT) reduction. Our findings support involvement of motor cortex in SPG4-HSP. Proton MRS could be a useful tool for detecting metabolite abnormalities in areas of brain that appear normal on MRI. Cho/Cr ratio may be a marker of neurodegenerative process in SPG4-HSP.

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BACKGROUND: Hereditary spastic paraplegias (HSP) are neurodegenerative diseases mainly characterized by lower limb spasticity with additional neurological symptoms and signs in complicated forms. Among the many autosomal recessive forms, SPG11 appears to be one of the most frequent. OBJECTIVE: Our objective was to select potential SPG11 patients based on phenotypes in our material, identify eventual disease-causing variants with the collaboration of laboratories abroad, estimate the frequency and spectrum of SPG11-mutations and describe their associated phenotypes. MATERIAL AND METHODS: Two isolated cases and two affected members of one family with cognitive impairment and confirmed thin corpus callosum on magnetic resonance imaging were selected from our database for inclusion into a multicenter study. Results - Mutations were found in the two isolated cases but not in the proband of the family. CONCLUSION: We present the first SPG11-HSP in the Norwegian population. SPG11 should be suspected in patients with isolated or recessive HSP, thin corpus callosum and mental retardation.

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To establish the phenotypic variation and frequency of SPAST mutations or deletions in Norwegian patients with hereditary spastic paraplegia (HSP), we examined 59 unrelated patients with HSP and screened for DNA point mutations and microdeletions in SPG4. Forty-one had a familial history, 35 had a clear dominant inheritance, six had other affected sibs and 18 were sporadic. We found 12 mutations in SPG4, seven of them novel, and four different heterozygous exon deletions, two of them novel. Mutations were found in 16 families showing autosomal dominant (AD) inheritance, and in one sporadic case. In two non-SPG4 families the S44L polymorphism/modifier was found in both affected and unaffected individuals. This is the first study of Norwegian patients with HSP since the 1970s, and the first report on SPG4 in Norway. Our results show that SPG4 mutations and deletions are a significant cause of HSP in our population and warrant SPG4 screening in AD families and selected sporadic cases.

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AIMS: Although pulmonary involvement is a known cause of morbidity in Niemann-Pick disease, histological features in the lung are not well characterized. The purpose of this study is to document the histological features seen in pulmonary involvement by types B and C Niemann-Pick disease and to correlate them with clinical and imaging data. METHODS AND RESULTS: Surgical lung biopsies from six patients (four with type B and two with type C disease) were reviewed and all showed diffuse endogenous lipid pneumonia, with lesser involvement of the interstitium by fibrosis and foamy macrophage accumulation. In type B disease only, there was also fine cytoplasmic vacuolation within the cytoplasm of ciliated epithelial cells. Neither disease showed foamy changes within pneumocytes. One patient had a bronchial cast removed on whole lung lavage. Electron microscopy showed abnormal lamellar inclusions within lysosomes of affected cells in type B disease. In patients with type C disease, biopsies were undertaken as part of investigations into acute respiratory failure in the context of multiorgan systemic presentation. Three patients with type B disease had clinical disease limited to the lung, all adults (mean age of 40 years) with unexplained diffuse parenchymal lung disease and mainly ground-glass shadowing on high-resolution computed tomography. CONCLUSIONS: Niemann-Pick disease should be considered for any patient with unexplained diffuse endogenous lipid pneumonia, even when disease is limited to the lungs and presentation is during adulthood.

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We determined whether short-term, posthatch oral exposure to estradiol benzoate (EB) or the industrial surfactant octylphenol (OP) could impair the reproductive performance of zebra finches. If so, naturally occurring phytoestrogens and xenoestrogens might influence reproduction in wild populations. Chicks were given oral administration of 10 or 100 nmol EB per gram of body mass (earlier work showed the latter to be the minimum oral dose required to maximally masculinize female song nuclei) or an equimolar amount of OP daily from 5 through 11 days of age. Canola oil was used as a vehicle and control. Reproductive testing was done either in individual pair cages or in communal cages that permitted self-selection of mates, N = 10 pairs per group. Pairs consisted of EB-treated males and females, EB-treated males paired with canola-treated females, vice versa, and canola-treated males and females. Posthatch EB treatment produced sex-specific impairments in reproduction that, in some instances, were additive when both sexes were treated. Egg production was reduced and egg breakage was increased in 100 nmol/g EB-treated male and female pairs. The incidence of missing eggs was increased in 10 nmol/g EB-treated male and female pairs. Candled fertility was reduced in both groups containing 100 nmol/g EB-treated males. The number of hatched chicks was severely reduced in all EB-treated groups. No adverse effects of OP treatment were detected. These significant treatment effects (all P < 0.05) show that posthatch EB treatment profoundly disrupts the reproductive performance of zebra finches, suggesting that exposure to estrogens in the wild could impair the reproductive performance of wild populations.

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The objective of the study was to assess the 10-year cumulative risk and clinical risk factors for the development of a contralateral cancer and to compare the tumours histopathologically. Among 1980 consecutive radically treated breast carcinoma patients a separate malignant breast tumour was diagnosed in 90 and 74 could be histopathologically compared with the primary tumour. The 10-year cumulative risk was 6.5% (95% CI: 5-8%). There was no difference in 10-year cumulative risk in developing a second breast tumour comparing premenopausal (7.1%) with postmenopausal women (6.1%). The cumulative risk among premenopausal tamoxifen-treated women (19.3%) or among patients with relapse (13.8%) was significantly increased as compared to similar patients without tamoxifen or without relapse. Sixty-six percent of the tumours displayed different histopathology. Morphologically similar and different tumours developed almost equally among patients with synchronous tumours and in those with or without relapse. We conclude that a radically treated breast cancer patient has a 10-year cumulative risk of 6.5% to develop a new malignant breast tumour. In premenopausal women the tumour-protective effect of two years tamoxifen application seems questionable. Histopathological comparison of the bilateral breast tumours enables discrimination of bilateral breast tumours as two primaries in 2/3 of the patients with morphologically different tumours.

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PURPOSE: To compare CT and MR findings of the paranasal sinuses in patients with cystic fibrosis (CF) with microbiology and histopathology. Further, to compare microbiology from the maxillary sinuses, nasopharynx and sputum. MATERIAL AND METHODS: CT and MR imaging of the paranasal sinuses were performed in 10 CF patients. Endoscopy and maxillary sinus aspirates were obtained (guided by the MR findings) and analyzed microbiologically and histologically. Samples from the nasopharynx and sputum were analyzed microbiologically. RESULTS: CT and MR were equal in displaying the extent of soft tissue masses, which at CT were homogeneous, while MR showed heterogeneous signals. MR images also demonstrated circumscribed areas with signal void at the STIR sequence with corresponding high to intermediate signal at the T1-weighted sequence. P. aeruginosa was frequently cultured from these areas which we named the "black hole sign". Maxillary sinus cultures revealed the same bacteria as nasopharynx and sputum cultures combined. CONCLUSION: MR images were superior to CT in differentiating soft tissue masses in the paranasal sinuses in CF patients. Bacteria with potential for specialized iron uptake mechanisms were present in areas with signal void at the STIR sequence. Our hypothesis is that the MR "black hole sign" can be explained by paramagnetic properties related to bacterial agents.

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The incidence of bronchial carcinoma is increasing. The only curative treatment so far has been surgery. The correlation between preoperative CT thorax and peroperative/pathologic findings was evaluated in order to assess the value of preoperative CT thorax in patients with bronchial carcinoma. The thoracic CTs of 96 patients with bronchial carcinoma who were referred to our outpatient clinic from January 1992 to September 1994 were reviewed. Only 19 patients (20%) underwent surgery (seven pulmectomies, nine lobectomies, two wedge resections, one explorative thoracotomy). The remaining 77 patients received either irradiation, chemotherapy or other palliative treatment. The tumours were classified according to the TNM system (T = tumour, N = node, M = metastases) of UICC (Union Internationale Centre le Cancer). In 11 of the 19 patients who underwent surgery a correct TN classification was assessed at CT, while in four the T classification and in another four the N classification was changed after peroperative and pathologic assessment. CT showed a slight tendency to overestimate T classification. As for the N classification, overestimation and underestimation were equal. We conclude that CT thorax is valuable in the preoperative staging of bronchial carcinoma.

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The preservation of morphological details in frozen sections subjected to microwave-assisted fixation was compared with that in frozen sections subjected to snap fixation with either formalin/ethanol or ethanol alone. Especially nuclear details were better preserved after short microwave fixation. Ethanol alone gave the poorest results, with loss of nuclear details in the majority of the cases. In this study there was no significant difference between the morphological results obtained with a cheap domestic oven and that with a specialized microwave processor. Microwave-assisted fixation in frozen sections is a simple, rapid method. It is recommended for use in routine laboratories.

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The article reviews recent findings in the carcinogenesis of male germ cell tumours. Histologic, cytogenetic and cell molecular studies indicate that the embryonal germ cell-the gonocyte-is the precursor cell. A step-wise modification of the gonocyte with gradual loss of genetic material, and participation of different oncogenes like myc and ras, are probably of great significance, but the modes and extent are unknown. The histogenesis of the different subtypes of germ cell tumours is still obscure.

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An increasing incidence of testicular germ cell tumours has been observed in many white populations in this century. Ethnic genetic implications and lifestyle seem to influence the development of testicular cancers, whereas the influence of intrauterine conditions is under debate. Epidemiologic studies cannot explain the increasing incidence among young men. The age variation of incidence and histologic type indicates at least two different aetiological mechanisms. Testicular cancers among young men are most probably due to gonadal dysgenesis, whereas in old men they reflect the general increased risk of cancer with old age. The increased risk of developing testicular germ cell tumours among cryptorchid young men supports the gonadal dysgenesy hypothesis.

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Lines of sugarbeet (Beta vulgaris L.) tolerant of multiple salts was accomplished by an in vitro multiple salt challenge. Petioles were placed on RV medium amended with 5 different salts along with Murashige and Skoog base salts for one month. Surviving shoots were cultured on RV medium to obtain petioles for subsequent challenges. During the first, second and third challenges, organogenically regenerated shoots developed from 5%, 46%, and 80% of the petioles, respectively. After the third multiple salt challenge, tolerant shoots were rooted and transplanted in soil. Salt was added to this soil at 1.0% by weight and plants were observed for 2 months. The ten most salt tolerant plants were vernalized to obtain seed. The R1 seed and controls were planted in soil containing 0%, 0.61% or 0.77% multiple salts per dry soil weight. Emergence of R1 seedlings was significantly greater than the controls under salt stress. Multiple salt tolerant R1 plants were maintained in salt amended soil to the 8-10 leaf stage and appeared as healthy and vigorous as the control growing in salt free soil.

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Two cases are reported in which old anoxic/ischemic damage to the Ammon's horns was associated with atrophy and sponginess of the mammillary bodies but with sparing of the neuronal perikarya. The lesions were unilateral in one case and bilateral in the other. The changes in the mammillary bodies closely resembled those seen in inactive Wernicke's encephalopathy but they were considered to be due to transneuronal atrophy following loss of hippocampal afferent fibres. Consequently, this condition may present a problem in diagnostic neuropathology. However, there was no reactive astrogliosis in these cases and this may serve to distinguish this condition from inactive Wernicke's encephalopathy in which gliosis is always present and often prominent.

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Ten patients with an ileoanal anastomosis had conversion to permanent ileostomy 13 (range, 6-29) months after the primary procedure. Causes for reoperation were incontinence in seven patients, unacceptable stool frequency without incontinence in two patients, and atypia in the mucosal remnant with perfect continence in one patient. Stool frequency, continence function, anal canal resting pressure, external anal sphincter (EAS) EMG/pressure relationship (in terms of slope, m), EAS fiber density (FD), and pressure in the distal ileum were registered, and the mucosa and the anal sphincter muscles were examined histologically. There were significant correlations between continence function and EAS changes in terms of both neurophysiologic tests (m and FD) and the histologic picture. The abnormalities in six incontinent patients were consistent with denervation of the EAS. The main reason for fecal leakage in one patient was the high amplitude of pressure waves in the distal ileum. Preservation of mucosal epithelium proximal to the dentate line per se did not seem essential to maintain continence.

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In the present paper we have examined the properties of the prostaglandin E (PGE) receptor in a transplantable rat Leydig cell tumour (H-540). It appears that PGE1 and PGE2 share a common receptor in membrane particles from this Leydig cell tumour. From saturation analysis and modified Hofstee plots, the specific binding sites for PGE1 can be divided into a high (25%) and low affinity state (75%) with apparent equilibrium constants of dissociation (Kd) of 2.4.10(-7) mol/l and 4.4.10(-6) mol/l, respectively. Association rate kinetics at different temperatures employing 5.10(-9) mol/l [3H]PGE1 showed that specific binding was time- and temperature-dependent. At 37 degrees C an apparent steady state was achieved after approximately 4 h incubation. The binding of [3H]PGE1 was very tight and no dissociation was observed at 20 degrees C during the first 20 h. The free PGE1 receptor appears to be very unstable. Binding was reduced rapidly by storage at 0 degrees C, by freezing and thawing of membrane particles, and by incubation of concentrated membrane particles. Specificity curves showed that PGA1 and PGA2 displaced [3H]PGE1 from receptor to a somewhat lesser degree than PGE2 and PGE1, whereas PGs of the B, D, I and F series had little or no effect. The fact that inhibition of [3H]PGE1 binding by cold PGE1 occurred in the same concentration range as PGE1 activation of adenylate cyclase, indicates that the specific binding of PGE observed here represents functional receptors coupled to the adenylate cyclase.

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The localized dilatation below the confluence of the bile and pancreatic ducts is known as the ampulla of Vater. The purpose of the present study was to delineate the radiological features of the choledochopancreaticoduodenal junction, with special reference to ampullar formation in this region. This was done by specimen radiography and review of the films from 255 examinations with endoscopic retrograde cholangiopancreatography. Full evaluation was possible in 200 cases. Ampullar dilatation was observed in only 9 (5.1%) of the 175 patients with a common terminal opening. No ampullar dilatation was observed in the 25 patients with separate openings of the duct. The appearance in the presence of ampullar dilatation is described. The distal end of the common bile duct, below its confluence with Wirsung's duct, has been known as the ampulla of Vater for nearly 130 years. This is a most important diminutive region of the human body, but there is little in the literature concerning its radiological presentation. In this report a radiological study is presented on the choledochopancreaticoduodenal junction.

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