Children with Functional Motor Limitations: The Effects on Family Strengths

Sixty children with functional motor limitations (age range from 15months to 7years 3months) and their parents participated in the study. The objective was to explore the relationship of the severity of their restrictions on family strengths. Functional motor abilities of the children were assessed using Autti-Ramos Scale. Cognitive functions were assessed using the Swedish standardized version of the Griffiths Scales of Mental Development. Family strengths were indexed using the Family Functioning Style Scale. The social-economical status, childrens age and caretakers age were taken into account. Overall, the results indicated that family strengths were rather strong. Only families rearing a child with severe participation limitations (functional motor limitations and cognitive difficulties) showed less strengths concerning family identity and internal coping relative to families with a child with milder participation limitations.This research has been made possible by the grants of theTampere University Foundation and Medical Research Fund of Tampere University Hospital, Finland. We thank Matti Koivikko, MD, PhD, and Markku Ojanen, PhD, for their critical comments.     

The relationship between job-related burnout and depressive disorders--results from the Finnish Health 2000 Study

BACKGROUND: Depression and burnout are common health problems in working populations today. They appear to be interrelated, and the need for their differential diagnosis has been highlighted in many reviews. We analysed the overlap of job-related burnout and depressive disorders, i.e., major depressive disorder, dysthymia, and minor depressive disorder. METHODS: We used the population-based 'Health 2000 Study' in Finland. Our nationally representative sample comprised 3276 employees aged 30-64 years. Burnout was assessed with the Maslach Burnout Inventory-General Survey. Diagnoses of depressive disorders were based on the Composite International Diagnostic Interview. RESULTS: Burnout and depressive disorders were clearly related. The risk of depressive disorders, especially major depressive disorder (12-month prevalence), was greater when burnout was severe. Half of the participants with severe burnout had some depressive disorder. Those with a current major depressive episode suffered from serious burnout more often than those who had suffered a major depressive episode earlier. LIMITATIONS: This study was cross-sectional. CONCLUSIONS: The concepts of burnout and depression complement each other and cover partly overlapping phenomena. Depressive disorders are related to job-related burnout, particularly when it is severe. A current major depressive episode is likely to be associated with the experience of burnout. When encountering working patients, it is recommended to assess both the occurrence of burnout and of depressive disorders.     

A mouse model for alpha-methylacyl-CoA racemase deficiency: adjustment of bile acid synthesis and intolerance to dietary methyl-branched lipids

alpha-Methylacyl-CoA racemase (Amacr) deficiency in humans leads to sensory motor neuronal and liver abnormalities. The disorder is recessively inherited and caused by mutations in the AMACR gene, which encodes Amacr, an enzyme presumed to be essential for bile acid synthesis and to participate in the degradation of methyl-branched fatty acids. To generate a model to study the pathophysiology in Amacr deficiency we inactivated the mouse Amacr gene. As per human Amacr deficiency, the Amacr(-/-) mice showed accumulation (44-fold) of C27 bile acid precursors and decreased (over 50%) primary (C24) bile acids in bile, serum and liver, however the Amacr(-/-) mice were clinically symptomless. Real-time quantitative PCR analysis showed that, among other responses, the level of mRNA for peroxisomal multifunctional enzyme type 1 (pMFE-1) was increased 3-fold in Amacr(-/-) mice. This enzyme can be placed, together with CYP3A11 and CYP46A1, to make an Amacr-independent pathway for the generation of C24 bile acids. Exposure of Amacr(-/-) mice to a diet supplemented with phytol, a source for branched-chain fatty acids, triggered the development of a disease state with liver manifestations, redefining the physiological significance of Amacr. Amacr is indispensable for the detoxification of dietary methyl-branched lipids and, although it contributes normally to bile acid synthesis from cholesterol, the putative pMFE-1-mediated cholesterol degradation can provide for generation of bile acids, allowing survival without Amacr. Based upon our mouse model, we propose elimination of phytol from the diet of patients suffering from Amacr deficiency.     

Type C botulism due to toxic feed affecting 52,000 farmed foxes and minks in Finland

The largest reported outbreak of type C botulism in fur production animals is described. Epidemiological investigation of 117 out of 157 (response rate, 74.5%) farms revealed that 44,130 animals died or were euthanized, while 8,033 animals with milder symptoms recovered. The overall death rate in all animals at risk was 21.7%. The death rates were significantly higher in blue and shadow foxes (24.2 and 27.8%, respectively) than in silver and blue silver foxes and minks (below 4%). All minks had been immunized against botulinum toxin type C. Deaths were associated with feed manufactured by a local processor, 83 of whose customer farms (70.9%) reported dead or sick animals. Five feedlots out of 19 delivered to the farms on the day preceding the onset of the outbreak (day 2) were associated with a death rate higher than 40%. These feedlots consisted of fresh feed processed on day 2 and feed processed 1 day earlier (day 1). In laboratory analysis, the day 2 feed contained botulinum toxin type C (>600 minimum lethal doses/g), while the day 1 feed did not contain toxin. Toxin was not detected in feed raw-material samples. Clostridium botulinum type C was detected by PCR in some feed components and in feed. However, as the feed temperature was continuously 8 degrees C or below and the pH was continuously 5.6 or below according to the manufacturer, it seems unlikely that spore germination and toxin formation occurred during overnight storage. Hence, the events leading to toxin formation were not determined.     

Expression of the thioredoxin system in interstitial lung disease

The thioredoxin system containing thioredoxin (Trx) and thioredoxin reductase (TrxR) has profound effects on cell proliferation and protection against exogenous oxidants. The significance of the Trx system in human lung and lung diseases is, however, largely unresolved. Altogether, 66 specimens of human lung were investigated by immunohistochemistry for their expression of Trx and TrxR. The diseases included interstitial pneumonias such as usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), and UIP associated with collagen vascular diseases (CVD-ILD), and granulomatous diseases such as sarcoidosis and allergic alveolitis. The ultrastructural localization of Trx and TrxR was analysed by immunoelectron microscopy. In healthy lung, Trx and TrxR were expressed in bronchial epithelium and alveolar macrophages. Trx and TrxR were highly concentrated in areas of metaplastic epithelium in UIP and in alveolar macrophages in DIP, though fibrotic areas in UIP were mainly negative. The expression of both enzymes was clearly weaker in CVD-ILD than in UIP. Granulomas of sarcoidosis showed moderate to intense Trx immunoreactivity. Ultrastructurally, Trx and TrxR were expressed diffusely in the cytosolic compartment and plasma membrane of metaplastic type II pneumocytes, macrophages, and bronchial epithelial cells. This study highlights the importance of Trx and TrxR in primary defence in bronchial epithelium, alveolar epithelium, and macrophages in human lung, but also indicates that elevated expression of these proteins may serve as markers of ongoing cell regeneration and inflammation.     

Long-term outcome of patients with claudication after balloon angioplasty of the femoropopliteal arteries

PURPOSE: To report the long-term outcome of patients with lifestyle-limiting claudication after percutaneous transluminal angioplasty (PTA) of the femoropopliteal arteries. MATERIALS AND METHODS: Between 1989 and 1992, 173 consecutive claudicant patients (mean age, 65 years; age range, 41-90 years) underwent PTA in 218 limbs; all interventions included femoral and/or popliteal arterial segments, and additional iliac (n = 27) and infrapopliteal (n = 11) arterial lesions were also treated. Patients were followed up for 7-10 years. Altogether, 37 (17%) limbs were classified as Fontaine class 2A, and 181 (83%) were class 2B. Average length of the primary lesion was 5.2 cm. Reinterventions were analyzed. Patency rates and patient survival were assessed by means of life table analysis. Cox-Mantel tests and Cox proportional hazards models were used to define associated independent determinants. Development of chronic critical ischemia (CCI) and its determinants was assessed by using the Pearson chi(2) test and multiple logistic regression analysis. RESULTS: The primary and secondary patencies (+/- standard error of the estimate), respectively, were 46% +/- 3 and 63% +/- 3 at 1 year, 25% +/- 3 and 41% +/- 4 at 5 years, and 14% +/- 3 and 22% +/- 4 at 10 years. One-third (71 of 218) of the limbs required repeat interventions, including surgical revascularization in 35 limbs. Fourteen (6.4%) limbs developed CCI, resulting in a 0.8% incidence per year. In multivariate analysis, poor postinterventional peripheral runoff was an indicator of increased risk of CCI development (P =.03). CONCLUSION: Although the long-term patency rates of PTA of the femoropopliteal arteries in claudicant patients were poor, the acceptable number of reinterventions and the low frequency of development of CCI imply the long-term benefits achievable with this treatment.     

Cognitive functions in relation to MRI findings 30 years after traumatic brain injury

OBJECTIVE: The aim of the study was to relate cognitive effects of a remote traumatic brain injury (TBI) to MRI findings and severity of injury. METHOD: Sixty-one patients were assessed on average 30 years after a TBI of variable severity. A comprehensive cognitive test battery was used to evaluate memory, executive functions and cognitive overall impairment. Multiple regression analyses were used to examine the relationships between cognitive variables and MRI volumetric findings (the volumes of the hippocampus and the lateral ventricles) and local contusions on MRI. Also, the effect of injury severity on cognitive outcome was evaluated. RESULTS: Reductions in hippocampal volumes and lateral ventricular enlargement were significantly associated with impaired memory functions, memory complaints and executive functions. Of the MRI parameters used, the best predictor for cognitive outcome was the volume of the lateral ventricle. There was only a modest relationship between severity of injury and cognitive performance. CONCLUSIONS: The results show that long-term memory impairments after TBI are associated with MRI volumetric measures. This suggests that the degree of diffuse injury leading to atrophic changes is prognostically more important than the initial severity of TBI.