RESUMO
Antisynthease syndrome (ASSD) is a rare, complex and understudied autoimmune disease. Internet-based studies can overcome barriers of traditional on-site research and are therefore very appealing for rare diseases. The aim of this study was to investigate patient-reported symptoms, diagnostic delay, symptoms, medical care, health status, working status, disease knowledge and willingness to participate in research of ASSD patients by conducting an international web-based survey. The multilingual questionnaire was created by an international group of rheumatologists and patients and distributed online. 236 participants from 22 countries completed the survey. 184/236 (78.0%) were female, mean age (SD) was 49.6 years (11.3) and most common antisynthetase antibody was Jo-1 (169/236, 71.6%). 79/236 (33.5%) reported to work full-time. Median diagnostic delay was one year. The most common symptom at disease onset was fatigue 159/236 (67.4%), followed by myalgia 130/236 (55.1%). The complete triad of myositis, arthritis and lung involvement verified by a clinician was present in 42/236 (17.8%) at disease onset and in 88/236 (37.3%) during the disease course. 36/236 (15.3%) reported to have been diagnosed with fibromyalgia and 40/236 (16.3%) with depression. The most reported immunosuppressive treatments were oral corticosteroids 179/236 (75.9%), followed by rituximab 85/236 (36.0%). 73/236 (30.9%) had received physiotherapy treatment. 71/236 (30.1%) reported to know useful online information sources related to ASSD. 223/236 (94.5%) were willing to share health data for research purposes once a year. Our results reiterate that internet-based research is invaluable for cooperating with patients to foster knowledge in rare diseases.
Assuntos
Autoanticorpos , Miosite , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Doenças Raras , Diagnóstico Tardio , Miosite/diagnóstico , Miosite/terapia , Síndrome , Aceitação pelo Paciente de Cuidados de SaúdeRESUMO
BACKGROUND: The feasibility, safety, and efficacy of prolonged use of an artificial beta cell (closed-loop insulin-delivery system) in the home setting have not been established. METHODS: In two multicenter, crossover, randomized, controlled studies conducted under free-living home conditions, we compared closed-loop insulin delivery with sensor-augmented pump therapy in 58 patients with type 1 diabetes. The closed-loop system was used day and night by 33 adults and overnight by 25 children and adolescents. Participants used the closed-loop system for a 12-week period and sensor-augmented pump therapy (control) for a similar period. The primary end point was the proportion of time that the glucose level was between 70 mg and 180 mg per deciliter for adults and between 70 mg and 145 mg per deciliter for children and adolescents. RESULTS: Among adults, the proportion of time that the glucose level was in the target range was 11.0 percentage points (95% confidence interval [CI], 8.1 to 13.8) greater with the use of the closed-loop system day and night than with control therapy (P<0.001). The mean glucose level was lower during the closed-loop phase than during the control phase (difference, -11 mg per deciliter; 95% CI, -17 to -6; P<0.001), as were the area under the curve for the period when the glucose level was less than 63 mg per deciliter (39% lower; 95% CI, 24 to 51; P<0.001) and the mean glycated hemoglobin level (difference, -0.3%; 95% CI, -0.5 to -0.1; P=0.002). Among children and adolescents, the proportion of time with the nighttime glucose level in the target range was higher during the closed-loop phase than during the control phase (by 24.7 percentage points; 95% CI, 20.6 to 28.7; P<0.001), and the mean nighttime glucose level was lower (difference, -29 mg per deciliter; 95% CI, -39 to -20; P<0.001). The area under the curve for the period in which the day-and-night glucose levels were less than 63 mg per deciliter was lower by 42% (95% CI, 4 to 65; P=0.03). Three severe hypoglycemic episodes occurred during the closed-loop phase when the closed-loop system was not in use. CONCLUSIONS: Among patients with type 1 diabetes, 12-week use of a closed-loop system, as compared with sensor-augmented pump therapy, improved glucose control, reduced hypoglycemia, and, in adults, resulted in a lower glycated hemoglobin level. (Funded by the JDRF and others; AP@home04 and APCam08 ClinicalTrials.gov numbers, NCT01961622 and NCT01778348.).
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Diabetes Mellitus Tipo 1/tratamento farmacológico , Hipoglicemiantes/efeitos adversos , Sistemas de Infusão de Insulina , Insulina/efeitos adversos , Adolescente , Adulto , Algoritmos , Criança , Estudos Cross-Over , Diabetes Mellitus Tipo 1/sangue , Desenho de Equipamento , Feminino , Hemoglobinas Glicadas/análise , Humanos , Hipoglicemia/induzido quimicamente , Hipoglicemiantes/administração & dosagem , Bombas de Infusão Implantáveis , Insulina/administração & dosagem , Sistemas de Infusão de Insulina/efeitos adversos , Masculino , Pessoa de Meia-IdadeRESUMO
The perineuronal net (PN) is a subtype of extracellular matrix appearing as a net-like structure around distinct neurons throughout the whole CNS. PNs surround the soma, proximal dendrites, and the axonal initial segment embedding synaptic terminals on the neuronal surface. Different functions of the PNs are suggested which include support of synaptic stabilization, inhibition of axonal sprouting, and control of neuronal plasticity. A number of studies provide evidence that removing PNs or PN-components results in renewed neurite growth and synaptogenesis. In a mouse model for Purkinje cell degeneration, we examined the effect of deafferentation on synaptic remodeling and modulation of PNs in the deep cerebellar nuclei. We found reduced GABAergic, enhanced glutamatergic innervations at PN-associated neurons, and altered expression of the PN-components brevican and hapln4. These data refer to a direct interaction between ECM and synapses. The altered brevican expression induced by activated astrocytes could be required for an adequate regeneration by promoting neurite growth and synaptogenesis.
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Núcleos Cerebelares/fisiopatologia , Degeneração Neural/fisiopatologia , Rede Nervosa/fisiopatologia , Plasticidade Neuronal/fisiologia , Células de Purkinje/patologia , Sinapses/fisiologia , Animais , Núcleos Cerebelares/patologia , Modelos Animais de Doenças , Camundongos , Degeneração Neural/patologia , Rede Nervosa/patologia , Sinapses/patologiaRESUMO
AIM OF STUDY: Working in second half of life is individually and socially important. The aim of this study is to multidimensionally evaluate the subjective health situation of older people, in particular those beyond the legal age of retirement who are working or assisting in family run businesses, using the example of Bavarian rural economics. METHODS: A 3 stage random sample stratified proportionally to regional population numbers was selected for a cross-sectional questionnaire survey (personal delivery, postal return n=3 176, 39.1%). Sample results can be generalised (men 59.8%, at least 65 years old 60.8%, smallest/small enterprises 61.8%). RESULTS: Life satisfaction is similar to that of the German age cohort population. In comparison to commercial and technical fields, physic and psychological complaints are lower and work pleasure is much higher. Health indicators are not dependent on enterprise structures, but on age and gender - on age partly non-linearly. CONCLUSION: Our results indicate that working in older age can be a source of subjective health. Process of family business succession may effect health. RESULTS might be applicable to other structurally similar agriculture regions and to craft sector. They promote individual confrontation with and social debate on a positive image of old age and aging.
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Emprego/estatística & dados numéricos , Nível de Saúde , Satisfação no Emprego , Qualidade de Vida/psicologia , População Rural/estatística & dados numéricos , Empresa de Pequeno Porte , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Emprego/economia , Emprego/psicologia , Feminino , Alemanha/epidemiologia , Inquéritos Epidemiológicos , Humanos , Masculino , Pessoa de Meia-Idade , Modelos Econômicos , Empresa de Pequeno Porte/economia , Recursos HumanosRESUMO
PURPOSE: The aim of this study was to evaluate the duration of the preoperative examination and patient comfort in measurements with an Image Guided System (Verion, Alcon), which is used for the calculation and intraoperative alignment of toric intraocular lenses, in comparison to established keratometry devices. PATIENTS AND METHODS: In a prospective, monocentric, open, ethics committee controlled study, 150 eyes of 150 ophthalmologically healthy volunteers were examined by a single examiner. Three measurements were performed with the Verion Image Guided System and - for comparison - one measurement each with IOLMaster 500, Lenstar LS900 and Pentacam HR. The measurement time was recorded with a stopwatch. For the analysis, patients were divided into three age groups (young, middle, old). Patient comfort was assessed using a questionnaire, which focussed on grading the whole measurement, as well as brightness of light, head posture and subjective duration. RESULTS: The mean age of the volunteers was 40.5 years (18-78 years). The mean duration of measurement was as follows: first Verion measurement 54.0 ± 42.7 seconds (n = 149), second Verion measurement 42.0 ± 20.5 s (n = 144), third Verion measurement 44.7 ± 25.2 s (n = 143), IOLMaster 500 46.3 ± 22.4 s (n = 147), Lenstar LS900 46.6 ± 14.4 s (n = 146) and Pentacam HR 46.6 ± 25.5 s (n = 147). Only the first and second Verion measurements were statistically different (p < 0.01). There were no statistical differences between the age groups, with the single exception of young versus old with the Pentacam (p < 0.01). Subjective patient comfort (n = 143) was very high for all devices and rated as "not uncomfortable" or "slightly uncomfortable". CONCLUSION: The duration of the preoperative examination with the Verion Image Guided System is comparable to established keratometry devices. However, IOL calculation with the Verion requires measurement of axial length and anterior chamber depth with another biometric device, which requires additional time. No age dependent differences were found. The examination can be easily integrated into clinical routine and is well tolerated by patients.
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Topografia da Córnea/instrumentação , Implante de Lente Intraocular/instrumentação , Conforto do Paciente , Ajuste de Prótese/instrumentação , Cirurgia Assistida por Computador/instrumentação , Carga de Trabalho , Adolescente , Adulto , Idoso , Biometria/instrumentação , Desenho de Equipamento , Análise de Falha de Equipamento , Feminino , Alemanha , Humanos , Implante de Lente Intraocular/métodos , Masculino , Pessoa de Meia-Idade , Monitorização Intraoperatória , Satisfação do Paciente , Cuidados Pré-Operatórios , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Cirurgia Assistida por Computador/métodos , Fatores de Tempo , Adulto JovemRESUMO
PURPOSE: The purpose of this study was to evaluate the functional results and quality of vision after implantation of an aspheric, aberration-neutral, monofocal toric intraocular lens (IOL). PATIENTS AND METHODS: 18 eyes of 13 patients were enrolled in this prospective clinical study. The T-flex aspheric, aberration-free toric IOL (Rayner Intraocular Lenses Limited, U.âK.) was implanted after femtosecond or standard phacoemulsification. Follow-up examinations were performed two to four months after surgery; these included subjective refraction, ETDRS uncorrected (UDVA) and corrected distance visual acuity (CDVA), wavefront analysis, measurements of stray light (C-Quant), and a questionnaire. RESULTS: Median UDVA was 0.06 logMAR (range: 0.30 to - 0.18 logMAR) postoperatively. Median CDVA increased from 0.20 logMAR (range: 0.64 to 0.00 logMAR) preoperatively to - 0.07 logMAR (range: 0.16 to - 0.26 logMAR) postoperatively. The median difference between achieved versus intended spherical equivalent was - 0.08 D (range: - 1.25 to + 0.65 D). Median subjective cylinder improved from - 2.00 D (range: - 6.50 to - 0.25 D) preoperatively to - 0.50 D (range: - 1.25 to 0.00 D) postoperatively. The median spherical aberrations (6 mm pupil size) were - 0.38 µm (range: - 0.69 to - 0.10 µm) postoperatively. Measurements of stray light (C-Quant) revealed a median value of 1.28 log(s) (range: 0.92 to 1.61 log[s]). CONCLUSION: The T-flex aspheric toric IOL provided beneficial and predictable functional results after surgery. The implantation of the T-flex is an effective method to correct a large range of corneal astigmatism.
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Astigmatismo/diagnóstico , Astigmatismo/cirurgia , Extração de Catarata/métodos , Implante de Lente Intraocular/métodos , Lentes Intraoculares , Idoso , Extração de Catarata/instrumentação , Análise de Falha de Equipamento , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Desenho de Prótese , Resultado do Tratamento , Acuidade VisualRESUMO
PURPOSE: The aim of this study was to perform a clinical evaluation of the functional results and quality of vision after implantation of an aspheric, aberration-neutral, monofocal intraocular lens (IOL). PATIENTS AND METHODS: 47 eyes of 34 patients (median age: 68 years) with cataract were enrolled in this prospective clinical study that had Ethics Committee approval. The C-flex or Superflex aspheric IOL (Rayner, UK) was implanted after phacoemulsification. Follow-up examinations were performed two to four months after surgery including subjective refraction, ETDRS uncorrected (UDVA) and corrected distance visual acuity (CDVA), wavefront analysis, analysis of stray light (C-Quant), contrast sensitivity under different lighting conditions (F.âA.âC.âT.) and a questionnaire. RESULTS: Two to four months after surgery, median UDVA was 0.08 logMAR (range: 0.64 to - 0.18 logMAR, n = 41). Median CDVA increased from 0.30 logMAR (range: 1.00 to - 0.02 logMAR) preoperatively to - 0.08 logMAR (range: 0.16 to - 0.22 logMAR) postoperatively (n = 47). Median difference between achieved vs. intended (Holladay 1-formula) spherical equivalent was + 0.06 D (range: - 1.06 to + 0.87 D). Median total HOA RMS (6 mm pupil size) was 0.66 µm (range: 0.41 to 1.19 µm). The median spherical aberrations were - 0.36 µm (range: - 0.70 to - 0.17 µm). Analysis of stray light (C-Quant) revealed a median value of 1.21 log(s) (range: 0.79 to 1.57 log[s]). CONCLUSION: The C-flex and Superflex aspheric IOLs provide good and predictable functional results. Patients are not negatively influenced by stray light and show slightly negative spherical aberrations.
Assuntos
Implante de Lente Intraocular , Lentes Intraoculares , Recuperação de Função Fisiológica , Erros de Refração/diagnóstico , Erros de Refração/terapia , Acuidade Visual , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Falha de Equipamento , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Desenho de Prótese , Resultado do TratamentoRESUMO
Successor establishment in familiy business may lead to interpersonal and intrapsychological conflicts and is mostly a transition to retirement. This may have a negative impact on the senior's health. The "Sozialversicherung für Landwirtschaft, Forsten und Gartenbau (SVLFG)" reacts with an intervention of several days duration. Evaluation aim is measuring participants changes in specific activities and confidence on successor establishment and retirement indicating mental health stabilisation. The method comprises a panel of intervention group (I, n=61) and comparison group (V, n=28) randomly selected, structurally similar and parallelised to point of transfer. Retirement activities significantly rise in I (p=0.001, Cohen's d=0.48) and are steady in V (p=0.54) after one year. Level of confidence is equal in both groups and steady but strongly indicating response shift in I. Intervention activates targeted activities and improves confidence at least qualitatively in a field that has hardly been researched.
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Comércio/organização & administração , Terapia Familiar/métodos , Família/psicologia , Promoção da Saúde/organização & administração , Transtornos Mentais/prevenção & controle , Transtornos Mentais/psicologia , Aposentadoria/psicologia , Alemanha , Promoção da Saúde/métodos , HumanosRESUMO
Photoemission electron microscopy performed on a well-prepared surface of BaTiO3 reveals the persistence of surface domains at temperatures well above the bulk Curie temperature. Their patterns follow the ferroelectric domain structure observed at 300 K. The contrast between formerly outward polarized domains and in-plane polarized domains is preserved across the transition, while the contrast of inward polarized domains changes sign. The work functions of different possible structures are compared by first-principles calculations. The domain contrast in photoemission above the bulk Curie temperature is associated with a remaining tetragonal distortion of the topmost unit cells which is stabilized by an ionic surface relaxation.
RESUMO
BACKGROUND: Alzheimer´s disease (AD) is characterized by a progressive neuronal degeneration caused by two pathological hallmarks, hyperphosphorylated tau protein aggregated into tau filaments and amyloid precursor protein derived beta amyloid peptides aggregated into extracellular amyloid plaques. All attempts so far to find effective drugs failed in clinical trials. AD is a multifactorial disease, so that selective drugs to target one AD-relevant structure alone may not be sufficient. OBJECTIVE: We built novel furopyridines with various substitution patterns to evaluate them as protein kinases inhibitors of enzymes related to tau pathology. METHODS: Furopyridine derivatives were synthesized and purified using column chromatography. The protein kinase inhibitory properties were determined in ATP-competition assays with determined affinity constants for the most active compounds. RESULTS: The compounds were prepared in simple two-component reactions of substituted 1,4- dihydropyridines and respective quinones to obtain various substitutions of the molecular furopyridine scaffold. The substituent effects on the determined kinase inhibitory properties of cdk1, cdk2, Fyn, JNK3 and gsk-3ß are discussed. CONCLUSION: Various 3-substitutions were found most sensitive for the protein kinase inhibition depending on the length, nature and a substituent positioning within. We identified compounds as inhibitors of several kinases as a tool to potentially combat the disease progress in a multitargeting approach.
Assuntos
Doença de Alzheimer/tratamento farmacológico , Inibidores de Proteínas Quinases/química , Inibidores de Proteínas Quinases/farmacologia , Proteínas Quinases/metabolismo , Piridinas/química , Piridinas/farmacologia , Proteínas tau/metabolismo , Glicogênio Sintase Quinase 3 beta/metabolismo , Humanos , Fosforilação/efeitos dos fármacos , Inibidores de Proteínas Quinases/uso terapêutico , Piridinas/uso terapêutico , Relação Estrutura-AtividadeRESUMO
Juvenile Idiopathic Arthritis (JIA) patients living in areas with high prevalence of tick-borne-encephalitis-virus-(TBEV)-infection are recommended for administration of inactivated TBE-vaccination. However, there are serious concerns regarding protective vaccine-induced immune responses against TBEV in immunocompromised patients. The present study aimed to analyze the humoral and cellular immune response to TBE-vaccination in previously TBE-vaccinated JIA patients compared to healthy controls (HC) including investigation of IgG-anti-TBEV avidity, neutralization capacity, cellular reactivity by IFNgamma-ELISPOT and cytokine secretion assays. Similar IgG-anti-TBEV antibody concentrations, neutralization titers and cellular reactivity were found between JIA and HC. The number and the early timing of booster vaccinations after primary vaccination had the most prominent effect on neutralizing antibodies in JIA and on IgG-anti-TBEV concentrations in both JIA and HC. Administration of booster vaccinations made it more likely for JIA patients to have IgG-anti-TBEV concentrations ≥165 VIEU/ml and avidities >60%. TNF-alpha inhibitors had a positive and MTX administration a negative effect on humoral immune responses. In conclusion, irrespective of having JIA or not, vaccinated children showed similar humoral and cellular immunity against TBEV several years after primary TBE-vaccination. However, in JIA, booster vaccinations mounted a significantly higher humoral immune response than in JIA without boosters. Our results highlight the need for timely administration of boosters particularly in JIA. Although immunosuppressive treatment at vaccinations in diagnosed JIA had a negative effect mainly on TBEV-specific cellular immunity, most JIA patients mounted a favorable humoral immune response which was maintained over time. Thus, successful TBE-vaccination seems highly feasible in JIA patients with immunosuppressive regimens.
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Artrite Juvenil , Encefalite Transmitida por Carrapatos , Carrapatos , Vacinas Virais , Animais , Anticorpos Antivirais , Criança , Encefalite Transmitida por Carrapatos/prevenção & controle , Humanos , Imunidade Celular , VacinaçãoRESUMO
The family Chlamydiaceae currently comprises a single genus Chlamydia, with 11 validly published species and seven more taxa. It includes the human pathogens Chlamydia (C.) trachomatis, C. pneumoniae and C. psittaci, a zoonotic agent causing avian chlamydiosis and human psittacosis, as well as other proven or potential pathogens in ruminants, birds, snakes, reptiles and turtles. During routine testing of 15 apparently healthy captive flamingos in a zoo in 2011, an atypical strain of Chlamydiaceae was detected by real-time PCR of cloacal swab samples. Sequence analysis of the 16S rRNA gene revealed high similarity to the uncultured Chlamydiales bacterium clone 122, which previously had been found in gulls. As more samples were collected during annual campaigns of the flamingo ringing program in southern France from 2012 to 2015, Chlamydiaceae-specific DNA was detected by PCR in 30.9% of wild birds. From these samples, three strains were successfully grown in cell culture. Ultrastructural analysis, comparison of 16S and 23S rRNA gene sequences, whole-genome analysis based on de novo hybrid-assembled sequences of the new strains as well as subsequent calculation of taxonomic parameters revealed that the relatedness of the flamingo isolates to established members of the family Chlamydiaceae was sufficiently distant to indicate that the three strains belong to two distinct species within a new genus. Based on these data, we propose the introduction of Chlamydiifrater gen. nov., as a new genus, and Chlamydiifrater phoenicopteri sp. nov. and Chlamydiifrater volucris sp. nov., as two new species of the genus.
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Aves/microbiologia , Chlamydiaceae , Filogenia , Animais , Animais de Zoológico , Chlamydiaceae/classificação , Chlamydiaceae/isolamento & purificação , DNA Bacteriano/genética , RNA Ribossômico 16S/genética , Análise de Sequência de DNARESUMO
BACKGROUND: To investigate the feasibility and efficacy of earlier induction of hypothermia already during the 'no-flow' period of cardiac arrest with non-invasive surface cooling or invasive aortic flush cooling. METHODS: This was a prospective randomized experimental study that included 14 pigs, Large White breed (30-38 kg), with ventricular fibrillation cardiac arrest plus blanket surface and an invasive cold saline flush cooling. The endpoint was a decline in brain temperature (T(br)) at 35 min after cardiac arrest. RESULTS: With surface cooling, T(br) decreased from 38.7+/-0.2 degrees C to 37.4+/-0.8 degrees C (P=0.02) and with invasive cooling T(br) decreased from 38.8+/-0.13 degrees C to 19.0+/-2.8 degrees C within 216+/-23 s (P=0.02) and increased back to 33.0+/-0.6 degrees C at 35 min of cardiac arrest (P=0.02 vs. T(br) at 15 min, P=0.002 vs. T(br) at 35 min in the surface cooling groups). CONCLUSION: Invasive cooling by aortic flush with cold saline rapidly induces deep cerebral hypothermia, whereas non-invasive surface cooling only marginally decreases brain temperature.
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Parada Cardíaca/terapia , Hipotermia Induzida/métodos , Animais , Aorta Torácica , Roupas de Cama, Mesa e Banho , Glicemia/análise , Pressão Sanguínea/fisiologia , Temperatura Corporal/fisiologia , Encéfalo/fisiologia , Reanimação Cardiopulmonar , Cateterismo/instrumentação , Cardioversão Elétrica , Estudos de Viabilidade , Feminino , Parada Cardíaca/etiologia , Frequência Cardíaca/fisiologia , Hemoglobinas/análise , Hipotermia Induzida/instrumentação , Infusões Intra-Arteriais , Potássio/sangue , Estudos Prospectivos , Distribuição Aleatória , Respiração Artificial , Sódio/sangue , Suínos , Fatores de Tempo , Resultado do Tratamento , Fibrilação Ventricular/complicações , Fibrilação Ventricular/terapiaRESUMO
OBJECTIVE: The VAC system (vacuum-assisted wound closure) is an established noninvasive active therapy to promote the healing of difficult wounds that fail to heal with conventional treatment after cardiac surgery. We report our initial experience of the intrathoracic application of the VAC system after extended thoracic surgery. METHODS: Thirteen patients (11 men, 2 women) with a median age of 60 years (range 41 to 82 years) with deep wound infections after thoracotomy (empyema = 3; lobectomy = 5; Pancoast = 1; pneumonectomy = 4) were treated primarily with the VAC system after initial surgical debridement. All patients had an increased risk for impaired wound healing (e.g., diabetes, obesity, empyema, steroids). The VAC system was removed when systemic signs of infection resolved and quantitative cultures were negative. RESULTS: After a mean period of 64 +/- 45 days (range 7 to 134 days) the VAC system was removed in all patients. It was used as a bridge to reconstructive surgery with a latissimus dorsi muscle flap in 2 patients (15 %), while surgical wound closure could be achieved in the remaining 11 patients (85 %). Complete healing without recurrence was achieved in 11/13 (85 %) patients to date. Hemodynamic or respiratory complications (e.g., air leakage) during VAC system application were not observed in any case. Survival was 100 % after 16 +/- 9 months. Duration of hospital stay varied from 16 to 110 days (mean 44 +/- 34 days). CONCLUSION: Intrathoracic vacuum therapy after extended thoracic surgery seems to be an effective and safe adjunct to conventional treatment modalities for the therapy of intrathoracic infections or deep wound infections.
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Tratamento de Ferimentos com Pressão Negativa , Infecção da Ferida Cirúrgica/terapia , Toracotomia/efeitos adversos , Cicatrização , Adulto , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Terapia Combinada , Desbridamento , Feminino , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Infecção da Ferida Cirúrgica/tratamento farmacológico , Infecção da Ferida Cirúrgica/microbiologia , Infecção da Ferida Cirúrgica/cirurgia , Fatores de Tempo , Resultado do TratamentoRESUMO
We report on a 23-year-old woman who presented with elevated serum ferritin values at our department. She had undergone cataract surgery at the age of 14 and her family pedigree showed hereditary autosomal-dominant cataract. The combination of isolated hyperferritinemia with autosomal-dominant hereditary cataract led to the diagnosis of the hereditary hyperferritinemia cataract syndrome (HHCS) which we now describe in a German family for the first time. HHCS was confirmed by detection of a causal mutation at position 32 within the iron responsive element (IRE) of L-ferritin leading to a guanine to adenine exchange and the pathognomonic star-shaped cataract. This mutation interrupts the post-transcriptional control of L-ferritin. It prevents binding of the iron regulatory protein 1 (IRP1) to the 5alpha untranslated region of L-ferritin resulting in uncontrolled L-ferritin synthesis and high serum ferritin levels independent of the body iron stores. Premature cataract is eventually caused by deposition of L-ferritin crystals in the lens of the eye. Our family shows the typical autosomal-dominant inheritance of HHCS over four generations affecting a total of 17 family members. The causal mutation, star-shaped cataract and typical laboratory configuration were confirmed in five patients. Thus, in gastroenterological practice, HHCS should be added as a differential diagnosis of hyperferritinemia in Germany. Importantly, patients with HHCS can be spared from invasive diagnostics such as liver biopsy.
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Apoferritinas/genética , Catarata/diagnóstico , Catarata/genética , Distúrbios do Metabolismo do Ferro/diagnóstico , Distúrbios do Metabolismo do Ferro/genética , Adulto , Feminino , Predisposição Genética para Doença/genética , Heterozigoto , Humanos , Polimorfismo de Nucleotídeo Único/genética , SíndromeRESUMO
Chlamydiaceae are obligate intracellular bacterial pathogens for humans and animals. A recent study highlighted that a Chlamydiaceae intermediary between C. psittaci and C. abortus can infect hawks. Here, an isolate was obtained upon passage of cloacal and conjunctival sac material collected from a female hatch-year red-shouldered hawk (Buteo lineatus) in cultured cells. The diseased bird, one of 12 birds housed in a rehabilitation center, developed conjunctivitis and later died. Swabs from both sites tested positive for Chlamydia using the QuickVue Chlamydia test. The isolate, named RSHA, tested negative in qPCR assays specific for C. psittaci and C. abortus, respectively. Analysis of the 16S rRNA, 23S rRNA and whole genome sequences as well as MLST, ANIb and TETRA values reveal that C. psittaci and C. abortus are the closest relatives of RSHA. However, the overall results strongly suggest a phylogenetic intermediate position between these two species. Therefore, we propose the introduction of a new species designated Chlamydia buteonis with RSHAT as the type strain.
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Doenças das Aves/microbiologia , Chlamydia/classificação , Falcões/microbiologia , Filogenia , Animais , Linhagem Celular , Chlamydia/genética , Chlamydia/ultraestrutura , DNA Bacteriano/genética , Feminino , Genes Bacterianos/genética , Tipagem de Sequências Multilocus , RNA Ribossômico 16S/genética , RNA Ribossômico 23S/genética , Análise de Sequência de DNA , Especificidade da EspécieRESUMO
AIM: Whether time of day influences survival after out-of-hospital cardiac arrest (OHCA) remains controversial. We compared outcomes after OHCA between day and night and explored whether characteristics of pre-hospital advanced life support (ALS)-quality varied by time of day. METHODS: We conducted a prospective cohort study of individuals that suffered a non-traumatic OHCA in the city of Vienna between August 2013 and August 2015 and who received resuscitative efforts by EMS. We compared clinical outcomes between day and night, defined as 7:00 pm-7:00 am based on EMS shift time including rates of sustained return of spontaneous circulation (ROSC), 30-day survival and favourable neurologic outcome (cerebral performance category 1 or 2). ALS quality measures included time to first medical contact, time to first shock, total dose of epinephrine, and multiple ALS performance measures. RESULTS: We included 1811 patients (37% female) with a mean age of 67 ± 16 years in our analyses. Rates of ROSC and 30-day survival with favourable neurological outcome did not differ between day or night (30% vs 28%, p = â0.33; 12% vs. 11%, p = â0.51, respectively). These results remained unchanged after multivariate adjustment for ROSC (RR, 1.1; 95% CI, 1.0-1.3, p = 0.19) and 30-day survival with favourable neurological outcome (RR, 1.2; 95% CI, 1.0-1.5, p = â0.10). The quality of ALS did not differ between day and night. CONCLUSIONS: In contrast to previous studies, there was no significant difference in sustained ROSC rates and 30-day survival with favourable neurological outcome after OHCA between day and night in the city of Vienna. This is likely due to nearly identical high bystander CPR rates and identical ALS performance provided by EMS personnel irrespective of time of the day.
Assuntos
Suporte Vital Cardíaco Avançado , Serviços Médicos de Emergência , Parada Cardíaca Extra-Hospitalar , Tempo para o Tratamento/estatística & dados numéricos , Suporte Vital Cardíaco Avançado/métodos , Suporte Vital Cardíaco Avançado/normas , Suporte Vital Cardíaco Avançado/estatística & dados numéricos , Idoso , Áustria/epidemiologia , Estudos de Coortes , Serviços Médicos de Emergência/métodos , Serviços Médicos de Emergência/normas , Serviços Médicos de Emergência/estatística & dados numéricos , Feminino , Humanos , Masculino , Parada Cardíaca Extra-Hospitalar/mortalidade , Parada Cardíaca Extra-Hospitalar/terapia , Avaliação de Processos e Resultados em Cuidados de Saúde , Estudos Prospectivos , Análise de SobrevidaRESUMO
BACKGROUND: The aim of this study was to analyze the distribution of total, corneal, and mainly internal human third- to sixth-order aberrations (HOA) and investigate possible correlations between wavefront errors and patient age. PATIENTS AND METHODS: For this study 140 eyes of 140 subjects (age range 17-77 years) were measured three times each using Nidek Optical Path Difference scan. Mean value and standard deviation for each Zernike polynomial was computed for a 5-mm pupil diameter, and six different age groups were formed. RESULTS: There was wide individual variation in the internal wavefront aberrations. The mean root-mean-square value for HOA was 0.4563 microm+/-0.296 microm. No positive correlation was found between Zernike polynomials and increasing age. In total spherical aberration however a significant difference was found between the 26-35-year and > or =56-year age groups (p=0.0002), as well as to internal and total coma (Z8) (p=0.0487 and p=0.0117, respectively). CONCLUSIONS: The internal, corneal, and total wavefront aberrations varied widely among patients. Some significant differences were found between the age groups. No correlation between age and the internal, corneal, or total aberrations was found.
Assuntos
Córnea/fisiopatologia , Cristalino/fisiopatologia , Refração Ocular , Erros de Refração/diagnóstico , Adolescente , Adulto , Fatores Etários , Idoso , Topografia da Córnea , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Erros de Refração/fisiopatologia , Estatísticas não ParamétricasRESUMO
Multifocal intraocular lenses (MIOLs) of different designs have been used for more than 20 years in modern cataract surgery. The first IOLs were either refractive 2-3-zone designs or of diffractive concept. All MIOL types, regardless of manufacturer, show reduced contrast sensitivity and increased glare for years. Nevertheless, even first-generation MIOL patients had very good functional results when patient selection, IOL calculation, and surgical techniques were appropriate. Today's gold standard is foldable, aspherical diffractive, or refractive MIOLS. Advancements in technology allow the possibility that MIOLs will be used for aphakia correction as well as presbyopia correction.