The association between oil spill cleanup-related total hydrocarbon exposure and diabetes.

BACKGROUND: Although evidence suggests relationships between some crude oil components and glycemic dysregulation, no studies have examined oil spill-related chemical exposures in relation to type 2 diabetes mellitus (DM) risk. This study examined the relationship between total hydrocarbon (THC) exposure among workers involved in the 2010 Deepwater Horizon (DWH) oil spill and risk of DM up to 6 years afterward. METHODS: Participants comprised 2660 oil-spill cleanup or response workers in the prospective GuLF Study who completed a clinical exam and had no self-reported DM diagnosis prior to the spill. Maximum THC exposure was estimated with a job-exposure matrix based on interview data and personal measurements taken during cleanup operations. We defined incident DM by self-reported physician diagnosis of DM, antidiabetic medication use, or a measured hemoglobin A1c value ≥ 6.5%. We used log binomial regression to estimate risk ratios (RRs) for DM across ordinal categories of THC exposure. The fully adjusted model controlled for age, sex, race/ethnicity, education, employment status, and health insurance status. We also stratified on clinical body mass index categories. RESULTS: We observed an exposure-response relationship between maximum daily ordinal THC exposure level and incident DM, especially among overweight participants. RRs among overweight participants were 0.99 (95% CI: 0.37, 2.69), 1.46 (95% CI: 0.54, 3.92), and 2.11 (95% CI: 0.78, 5.74) for exposure categories 0.30-0.99 ppm, 1.00-2.99 ppm, and ≥3.00 ppm, respectively (ptrend = 0.03). CONCLUSION: We observed suggestively increasing DM risk with increasing THC exposure level among overweight participants, but not among normal weight or obese participants.

Novel homozygous RARS2 mutation in two siblings without pontocerebellar hypoplasia - further expansion of the phenotypic spectrum.

BACKGROUND: Pontocerebellar hypoplasia type 6 (PCH6) is a mitochondrial disease caused by mutations in the RARS2 gene. RARS2 encodes mitochondrial arginyl transfer RNA synthetase, an enzyme involved in mitochondrial protein translation. A total of 27 patients from 14 families have been reported so far. Characteristic clinical features comprise neonatal lactic acidosis, severe encephalopathy, intractable seizures, feeding problems and profound developmental delay. Most patients show typical neuroradiologic abnormalities including cerebellar hypoplasia and progressive pontocerebellar atrophy. METHODS: We describe the clinical, biochemical and molecular features of 2 siblings with a novel homozygous mutation in RARS2. Both patients presented neonatally with lactic acidosis. While the older sibling had severe neurological symptoms with microcephaly, seizures and developmental delay, the younger patient was still neurologically asymptomatic at the age of 2 months. RESULTS: MRI studies in both children lacked pontocerebellar involvement. The expression of the OXPHOS complex proteins was decreased in both patients, whereas oxygen consumption was increased. CONCLUSIONS: Characteristic neuroradiological abnormalities of PCH6 such as vermis and cerebellar hypoplasia and progressive pontocerebellar atrophy may be missing in patients with RARS2 mutations. RARS2 testing should therefore also be performed in patients without pontocerebellar hypoplasia but otherwise typical clinical symptoms.

[Primary liver tumours in childhood]. / Primäre Lebertumoren im Kindesalter.

The reported prevalence of focal liver lesions in adult patients and children is different. The article discusses pediatric liver tumors under the criteria of histopathology and contrast enhanced ultrasound (CEUS) features. Aim of this article is also to support the already established Pediatric Registry of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) to collect data on safety and applications of ultrasound contrast agents in children (www.efsumb.org).

Autonomic dysreflexia: a possible trigger for the development of heterotopic ossifications after traumatic spinal cord injury? : A clinical longitudinal study.

PURPOSE: The aim of this study was to investigate the influence of the initial American Spinal Injury Association Impairment Scale (AIS) category and the conversion rate in acute traumatic tetraplegic patients on the development of heterotopic ossifications (HO). The second objective was to prove the hypothesis that tetraplegic patients with autonomic dysreflexia (AD) develop HO more often than patients without AD. METHODS: A retrospective analysis from 2002 to 2009 of 330 patients with spinal cord injuries was performed and led to the inclusion of 77 traumatic tetraplegic patients. Clinical data was reviewed to determine the appearance of HO (n = 8) and its possible coincidence with AD during urodynamics. Spearman's correlation coefficient was calculated to test the relationship between HO and initial AIS category or the change in AIS category within 6 weeks. A matched pair (age, neurological level of injury) analysis of two samples (n = 8 with/without appearance of HO; total n = 16) was performed. RESULTS: The appearance of HO was significantly correlated with an initial AIS A compared to incomplete tetraplegia at baseline (p < 0.017). The conversion of AIS A into incomplete tetraplegia was highly correlated with the incidence of HO (p < 0.003). AD showed a positive correlation with HO (r = 0.97, p = 0.001). CONCLUSIONS: An initial AIS A that converts early into an incomplete tetraplegia constitutes a risk factor for the development of HO. Additionally, AD constitutes an important trigger in the development of HO in acute traumatic tetraplegic patients.

[Five-year survival rate of the Allofit titanium press-fit cup]. / 5-Jahres-Ergebnisse der zementfreien Allofit(R)-Press-fit-Pfanne.

BACKGROUND: The Allofit titanium press-fit cup has become the best-selling cementless acetabular implant in Germany, with approximately 30,000 implants sold in 2007. However, only a limited number of scientific publications - mostly from the development centres in Austria - support this success on the market. METHODS: At our institution in 1999-2000, 174 total hip replacements were performed using the Allofit cementless press-fit cup. At a mean follow-up of 5.3 years (range 40-85 months), 154 patients were evaluated clinically and radiographically, representing a follow-up rate of 89%. RESULTS: The 5-year survival rate was 98.1% (95% CI: 95.8-100%), with revision for any reason as an end point. Three cups were revised (aseptic loosening with one, and two septic complications). The average Harris hip score was 89.1+/-13.7 in all unrevised patients. CONCLUSION: We confirm the excellent survival rates of the Allofit cup as reported by the development centres, supporting the ongoing use of the cup at our institution.

[Hypophosphatasia]. / Hypophosphatasie.

Hypophosphatasia (HP) is an inborn error of bone metabolism transmitted predominantly as an autosomal-recessive trait. It is characterized by a reduced activity of the tissue-nonspecific isoenzyme of alkaline phosphatase (TNSAP) and elevated concentrations of its substrates, including pyrophosphates. Clinical symptoms include defective bone mineralisation with bone deformities, fractures and as recently defined chronic non-bacterial osteomyelitis. Renal damage due to calcification, craniosynostosis and dental abnormalities with premature loss of dentition are further symptoms, which have been described as characteristic in the ESPED inquiry of 2004. Knowledge about the mechanisms underlying cell activation leading to inflammation and tissue destruction is still limited in HP. Recent investigations have provided evidence that calcium pyrophosphate crystals are essentially involved in activating inflammatory signal transduction pathways via different receptors of the innate immune system. Laboratory assays, genetic counselling and testing, and radiologic imaging can confirm the diagnosis. Because symptoms are highly variable in their clinical expression, patients should be followed by a HP-experienced multidisciplinary team (paediatrician, radiologist, orthopedist, neurosurgeon, dentist). At the moment symptomatic support and treatment is most important because a causative therapy, e. g. enzyme replacement therapy, is not yet available.

[Training program for radiologic technologists for performing chest X-rays at inspiration in uncooperative children]. / Trainingsprogramm für MTRA zur Anfertigung von Thoraxübersichtsaufnahmen in Inspiration bei unkooperativen Kindern.

PURPOSE: A computer program was created to train technologists to perform chest X-rays in crying infants at maximum inspiration. MATERIALS AND METHODS: Videos of 4 children were used. Using a computer program, the moment of deepest inspiration was determined in the video in the single frame view. During the normal running video, 14 technologists (3 with significant experience, 3 with little experience and 8 with very little experience in pediatric radiography) simulated a chest radiograph by pushing a button. The computer program stopped the video and the period of time to the optimal moment for a chest x-ray was calculated. Every technologist simulated 10 chest X-rays in each of the 4 video clips. The technologists then trained themselves to perform chest X-rays at optimal inspiration like playing a computer game. After training, the test was repeated. Changes were evaluated by t-test for unpaired samples (level of significance p < 0.05). RESULTS: Although the differences improved in all children, minimal deviation from the optimal moment for taking an X-ray at inspiration occurred in the periodically crying child (0.21 sec before and 0.13 sec after training). In a non-periodically crying infant, the largest differences were shown. The values improved significantly from 0.29 sec to 0.22 sec. The group with substantial experience in pediatric radiology improved significantly from 0.22 sec to 0.15 sec. The group with very little experience in pediatric radiology showed worse results (improvement from 0.29 sec to 0.21 sec). CONCLUSION: The ability of a technologist to take a chest X-ray at optimal inspiration in a crying child is improved by the tested computer program.

[No sports = no exercise?--sports, overweight and physical activity in the 50-70 age group in Baden-Württemberg]. / Sind Bewegungsmuffel auch Sportmuffel? Sport, Ubergewicht und körperliche Mobilität in der Gruppe der 50- bis 70-jährigen baden-württembergischen Bevölkerung.

Sporting activity is proven to decline with advancing age. But do older adults replace sporting activity with physical activity like walking and cycling for transportation purposes, and are people with a history of sporting activity more likely to integrate physical activity in their day-today lives? There is a proven negative correlation between sporting activity and overweight, a major risk factor for numerous diseases. The purpose of this study was to investigate correlations between walking/cycling for transportation and sporting activity/overweight, while controlling other variables. This representative study, which includes 50 to 70 year old people from Baden-Württemberg, is sponsored by the Landesstiftung Baden-Württemberg (N=2002). People who say they use a bicycle for transportation on a regular basis, and those who go for walks on a regular basis, are significantly more likely to engage in sports than people who do not go for walks or cycle regularly. The correlation applies after controlling sociodemographic and lifestyle relevant variables. With regard to the correlation between physical activity and overweight, the results show that older adults who go for walks regularly are more likely to be overweight than those who do not. In contrast, those who use a bicycle for transportation on a regular basis are significantly less likely to be overweight. Older adults who do not work out do not compensate for this by physical activity in their day-today lives, i.e., no work out = no physical activity. In recognition of the biological and medical role of sporting activity and exercise for preserving health, exercising and doing sports is of special importance especially for older adults.

How can calcium pyrophosphate crystals induce inflammation in hypophosphatasia or chronic inflammatory joint diseases?

Hypophosphatasia (HP) is a rare inborn error of bone and mineral metabolism characterized by a defect in the tissue non-specific alkaline phosphatase (TNSALP) gene. Calcium pyrophosphate dihydrate (CPPD) crystals are known to accumulate as substrates of TNSALP in tissues and joints of patients with HP. In CPPD-induced arthritis these crystals are known to induce an inflammatory response. HP patients do suffer from pain in their lower extremities. However, it is not clear whether CPPD crystals contribute to these musculoskeletal complaints in HP. As long as there is no curative treatment of HP, symptomatic treatment in order to improve clinical features, especially with regard to pain and physical activity, is of major interest to the patients. Knowledge of the mechanisms underlying crystal-induced cell activation, however, is limited. Here we describe recent advances in elucidating the signal transduction pathways activated by CPPD crystals as endogenous "danger signals". Recent investigations provided evidence that Toll/interleukin-1 receptor (TIR) domain containing receptors including Toll-like receptors (TLRs) and interleukin-1 receptor (IL-1R), as well as the triggering receptor expressed on myeloid cells 1 (TREM-1) and the NACHT-leucin rich repeat and pyrin-domain-containing protein (NALP3) containing inflammasome are essentially involved in acute CPPD crystal-induced inflammation. These receptors are considered in part as components of the innate immune system. Further studies are needed to improve our understanding of the pathophysiological mechanisms leading to inflammation and tissue destruction associated with deposition of microcrystals. They might support the development of new therapeutic strategies for crystal-induced inflammation. Eventually, patients with HP might as well profit from such strategies addressing these metabolic disorders secondary to the gene defect.

[Spectroscopic imaging of the human liver using 3D CSI: optimization and application in patients with metastatic uvea melanoma]. / Spektroskopische bildgebung der menschlichen leber mittels 3D-CSI: etablierung und anwendung bei patienten mit metastasiertem aderhautmelanom.

PURPOSE: (31)P MR spectroscopy (MRS) allows the noninvasive assessment of metabolic alterations in tumors. Due to physical as well as technical limitations, mostly large and single voxels are used. We used a spatially resolved (31)P MRS technique to characterize metabolic abnormalities inside and adjacent to liver metastases of patients with uvea melanoma. MATERIALS AND METHODS: Optimization of 3D chemical shift imaging (3D CSI) was performed in healthy volunteers (n = 19; voxel size 25 ml). Patients (n = 8) with liver metastases were then examined. Cross sectional imaging was available for all patients. RESULTS: Compared to healthy volunteers, the PME/PDE ratios of patients with liver metastasis were significantly higher (0.56 +/- 0.30 vs. 0.39 +/- 0.21; p < 0.05). A trend towards increased PME/beta ATP ratios (2.07 +/- 1.83 vs. 1.02 +/- 0.45; p = 0.12) and decreased Pi/PME ratios (0.57 +/- 0.29 vs. 1.06 +/- 0.58; p = 0.06) was also observed. Patients with metastases > or = 5 cm showed significantly higher PME/PDE ratios (0.68 +/- 0.17 vs. 0.45 +/- 0.03; p < 0.05). Liver parenchyma adjacent to metastases did not show any significant changes compared to non-diseased tissue. CONCLUSION: 3D CSI allows the simultaneous analysis of metabolic alterations in diseased as well as in healthy human liver. Metastases show significant metabolic alterations. Thus, (31)P MRS opens new possibilities for therapeutic monitoring.

[Total knee arthroplasty - GPs' views in Germany: a questionnaire-based study]. / Beurteilung des Verfahrens der Knieendoprothetik durch deutsche Hausärzte: Eine Fragebogenstudie.

To assess general knowledge and individual views of general practitioners (GPs) on total knee arthroplasty (TKA), we conducted a questionnaire-based survey involving 170 GPs in Germany. Eighty-one GPs returned the questionnaire. They treat a mean of 10.6+/-8.3 patients with TKA. General knowledge can be estimated as good. Compared with the data in the literature, GPs assessed the rate of satisfied patients as lower and the risk for revision surgery as higher. The mean risk of potential complications (infection, instability, persistent pain) in association with TKA was estimated correctly. Seventy-eight percent of GPs consider an allergic reaction to the implant or bone cement as problematic. The number of cases per year, personal experience with the surgeon, and - less important - local accessibility are important factors for GPs when recommending an operating centre to a patient.A broad spectrum of individual responses indicates the need to improve the information transfer between orthopaedic surgeons and referring GPs.

[Whole-body MR imaging in children with suspected osteonecrosis after intensive chemotherapy: preliminary results]. / Ganzkörper-MRT bei Kindern mit Verdacht auf Osteonekrose nach intensiver Chemotherapie: Erste Ergebnisse.

PURPOSE: Use of multidrug chemotherapy poses the risk of avascular osseous necroses in children. Depiction of the whole body, including clinically non-apparent sites is mandatory for starting early and proper treatment, including surgical approaches in lesions near the joints. We analyzed the value of whole-body MRI in the detection of osteonecrosis, (1) in relation to conventional X-ray imaging and clinical symptoms, (2) using different MRI sequences, (3) with follow-up examinations. MATERIALS AND METHODS: 5 patients suffering from an oncological disease, 13 to 16 years old (3 x ALL, 1 x medulloblastoma, 1 x CML), and recently developing bone pain were examined with X-ray imaging of the particular region and a whole-body MRI (T2w TIRM, T 1w TSE sequences, pre- and post-contrast GD-DTPA, including fat suppression techniques). Neck/thorax/abdomen/pelvis, and upper and lower extremities were acquired in the coronal plane, and the feet in sagittal orientation. 4 of 5 patients had at least one follow-up examination (in the mean after 10 +/- 4 months). RESULTS: None of the initial X-ray images revealed an abnormal finding. The whole-body MRI showed in 4 of 5 children bone marrow lesions compatible with osteonecrosis. The locations were around the knee joints (n = 3) and the tibiae/ankle joints (n = 4). In addition to the symptomatic sites, MRI revealed additional lesions at the following sites: humerus (n = 5), hip joints (n = 4), knee joints (n = 6), ankle joints (n = 4). The size varied from small focal lesions to lesions measuring 90 % of the whole transverse diameter of the bone. The lesions were able to be detected most easily with heavily T 2-weighted (TIRM) sequences, and the diagnosis was most easily established using the non-enhanced TSE T 1-weighted sequences. As a consequence of the results of the whole-body MRI, all patients with lesions compatible with osteonecrosis received symptomatic (n = 2) or specific (n = 2) therapy. In the follow-up examinations, a higher number of patients showed no changes in the lesions as to size and distribution. 2 patients showed partial resolution of the osteonecroses. CONCLUSION: Whole-body MR imaging allows early diagnosis of symptomatic as well as clinically non-apparent osteonecroses. It can be used in planning and monitoring surgical and pharmacological therapies.

Proangiogenic effects of ionizing irradiation on squamous cell carcinoma of the hypopharynx.

OBJECTIVE: There is experimental evidence that ionizing irradiation affects a proangiogenic response. However, the relevance of this effect on tumour growth in vivo is not in detail investigated yet. The present objectives were to examine the influence of ionizing radiation on the expression of the vascular endothelial growth factor (VEGF) and its receptors (flt-1 and flk-1), the microvessel density and the tumour proliferation, in head and neck squamous cell carcinoma (HNSCC). METHODS: We used a HNSCC-cell line, derived from a hypopharyngeal tumour, for subcutaneous injection in 16 athymic nude mice. After reaching an average diameter of 12-14 mm the xenografts were randomised and 8 out of the 16 animals (therapy group) were irradiated with a single fraction of 6 Gy while the control group remained without any intervention. The irradiated and the respective control tumours were prepared after 7 (T7) and 70 days (T70) for immunohistochemical analysis. The expression of VEGF, its receptors flk-1 and flt-1, the vessel density (CD31) and the proliferation rate (Ki67) were quantified. RESULTS: At the point of time T7 we observed a reduction of the tumour growth rate, of the proliferative activity and of the VEGF- as well as of the VEGF-R-expression. At the point of time T70 we found increased values for proliferation, microvessel density, VEGF- and flk-1 expression in the therapy group compared to the therapy group at T7 as well as to the control group at T70. CONCLUSION: These changes might suggest a long-term proangiogenic effect of irradiation, which might result in growth promotion of the remaining tumour after the end of therapy.

["Living an active life"--sports, exercise and health in middle-aged and older adults. An empirical database on physical activity, health behavior and lifestyle in the 50- to 70-year-old residential population of Baden-Wuerttemberg]. / "Ein aktives Leben leben"--Sport, Bewegung und Gesundheit im mittleren und höheren Erwachsenenalter. Konzeption, Datenerhebung und erste Ergebnisse eines repräsentativen Basis-Survey für die 50- bis 70-jährige baden-württembergische Wohnbevölkerung.

In a society with an aging population, the preventive healthcare importance of physical activity in middle-aged and older adults is growing. The purpose of the study "Living an active life - age and aging in Baden-Wuerttemberg" is to acquire generalisable data of practical relevance for the 50- to 70-year-old population of Baden-Wuerttemberg. The main themes of this study are sports, exercise, and health-related and lifestyle aspects--from both a current and a biographical perspective. Following conceptualization and a test run, the survey was conducted by means of a computer-assisted telephone interview (CATI) in the period from May to October 2006. 982 men and 1,020 women responded. The percentage of respondents with a high level of education was disproportionately high, as is frequently the case for telephone surveys. Due to the resultant bias, the data was weighted by age, gender and education on the basis of the German Microcensus 2004. In addition to serving as a health report on the middle-aged and older adult population, this basic survey generated representative data on physical/sporting activity among older people in Baden-Wuerttemberg, and can be used as a reliable basis for designing future preventive measures. First analyses show that people with higher educational levels and good health, and non-smokers, people with balanced diet and normal weight are more likely to do sporting activities.

[Total hip replacement--GPs' views in Germany. A questionnaire-based study]. / Beurteilung des Verfahrens Hüftendoprothetik durch deutsche Hausärzte. Eine Fragebogenstudie.

To assess the general knowledge and individual views of general practitioners (GPs) on total hip arthroplasty (THA), we performed a questionnaire-based survey involving 200 GPs in Germany.Ninety-four GPs returned the questionnaire. They had treated a mean of 37.7+/-38.6 patients (range 0-300) with THA. Their general knowledge can be estimated as good. They assessed the potential for complications in association with THA as higher than that reported in the literature. If the GPs had been exposed to more complications in their practices, they estimated a lower patient satisfaction rate 10 years after surgery and were less satisfied with the indications given by their operating colleagues.A broad spectrum of individual responses indicates the need to improve information transfer between orthopaedic surgeons and referring GPs.

Gender, age and seasonal effects on IgA deficiency: a study of 7293 Caucasians.

BACKGROUND: The frequency of serum IgA deficiency (SIgAD) differs between populations. We examined the prevalence of SIgAD in healthy Caucasians. MATERIALS AND METHODS: Serum immunoglobulin A (SIgA) was measured in 7293 volunteers (2264 women, 5029 men) aged 30 +/- 14.2 years (mean +/- SD; range: 12-66). Serum immunoglobulin A and subnormal SIgA levels were defined by a SIgA level < 0.07 g L(-1), and between 0.07 and 0.7 g L(-1), respectively. Means were compared by analysis of variance (anova) and analysis of covariance (ancova); frequencies by the chi(2) test. RESULTS: Fifteen subjects (0.21%; one woman, 14 men) had SIgAD. Subnormal SIgA levels were found in 155 persons (2.13%): 21 females (0.93% of the females) and 134 males (2.66% of the males; difference: 1.74%; 95% CI: 1.12-2.33%; P < 0.001). Males were more likely to have subnormal SIgA levels or SIgAD (odds ratio 3.09, 95% CI: 1.97-4.85). The prevalence of SIgAD and subnormal SIgA was lowest in winter (chi(2) = 14.8; P = 0.002; 3 d.f.; and chi(2) = 43.2; P < 0.001; 3 d.f., respectively). Serum immunoglobulin A concentrations were significantly higher during winter. Serum immunoglobulin A levels increased with age on average by 0.2 +/- 0.06 g L(-1) per decade of life (P < 0.001). Taking into account the influence of age, SIgA concentration was lower in females as compared with males. CONCLUSION: The prevalence of SIgAD and subnormal SIgA levels is increased in males. There exists a significant influence of gender, age and seasons on SIgA levels.