Abstract Hallux valgus (HV) decreases the forces under the first ray during the propulsive phases of gait and transfers loading to the lesser metatarsals. Biomechanical factors contribute to the development of HV through various mechanisms; however, the flexibility of HV has not been considered to be a major determinant when planning surgery for HV. The aims of this thesis were to develop a clinical test and a new surgical technique for flexible HV, to report the mid-term follow-up results of this new correction method, and to compare the metatarsal load distribution after the new technique to that achieved with distal chevron osteotomy in a matched-pair analysis. The new surgical technique, first tarsometatarsal joint derotational arthrodesis (FTJDA), includes rotational correction of the first metatarsal without surgery to the first metatarsophalangeal joint (I MTPJ). It is indicated for patients who are able to reduce HV with peroneus longus (PL) function (PL activation test). Between 2003 and 2009, a total of 88 flexible HV underwent FTJDA operations. Seventy-six operated feet were re-examined after an average of 5.1 (range 3.0 to 8.3) years of follow-up. Outcome measures included the American Orthopaedic Foot and Ankle Society (AOFAS) score and pre- and postoperative radiological analysis. Gait analysis was used to compare the metatarsal load distribution after the FTJDA with a distal chevron cohort in 30 pairs matched by HV angle and follow-up time. The mean HV and intermetatarsal correction angles between preoperative and six-week follow-up radiographs were 19.8 degrees and 8.9 degrees, respectively. HV and intermetatarsal angle (IMA 1-2) correction were satisfactorily maintained (13.4 degrees [95% CI 11.6 to 15.1] and 4.5 degrees [95% CI 3.7 to 5.2], respectively) at mid-term follow-up. AOFAS score was excellent or good in 72% of the operated feet. In gait analysis, the FTJDA group had 8% higher relative impulses under the first metatarsal head (MTH), whereas chevron group had central loading pattern. In conclusion, FTJDA corrects flexible HV effectively without intervention to the I MTPJ. The correction is satisfactorily maintained at the late follow-up and the clinical results are good. In the gait analysis, the FTJDA produces better postoperative dynamic loading capacity of the first ray in comparison to distal chevron osteotomy. / Tiivistelmä Vaivaisenluu heikentää voimantuottoa ensimmäisen säteen alla kävelyn ponnistusvaiheiden aikana ja kuormitus siirtyy ulommille jalkapöytäluille. Biomekaanisilla tekijöillä arvellaan olevan osuutta vaivaisenluun kehittymiseen. Vaivaisenluun joustavuutta ei ole pidetty ratkaisevana tekijänä kirurgisen hoidon suunnittelussa. Tämän tutkimuksen tavoitteina oli kehittää kliininen testi ja uusi kirurginen korjausmenetelmä joustavalle vaivaisenluuvirheasennolle, raportoida uuden korjausmenetelmän keskipitkän seuranta-ajan tulokset ja verrata jalkapöytäluiden kuormitusjakaumaa uuden korjausmenetelmän jälkeen distaaliseen chevron -osteotomiaan kaltaistettujen parien analyysillä. Uusi kirurginen toimenpide, ensimmäisen tarsometatarsaalinivelen derotaatioartrodeesi (FTJDA), sisältää metatarsaalin rotaatiokorjauksen avaamatta ensimmäisen varpaan tyviniveltä. Menetelmä on tarkoitettu potilaille, joilla vaivaisenluuvirheasento korjaantuu peroneus longuksen toiminnalla (PL-aktivaatiotesti). Vuosina 2003–2009 operoitiin yhteensä 88 joustavaa vaivaisenluuvirheasentoa ensimmäisen tarsometatarsaalinivelen derotaatioartrodeesillä (FTJDA). 76 leikattua jalkaa tutkittiin uudelleen 5,1 vuoden (vaihteluväli 3,0 – 8,3) keskiseuranta-ajan jälkeen. Tulosten arvioinnissa käytettiin AOFAS-pisteytystä ja pre- ja postoperatiivisia radiologisia mittauksia. Kävelyanalyysillä verrattiin jalkapöytäluiden kuormitusta FTJDA- ja distaalisen chevron -osteotomiaryhmän välillä 30 parilla, jotka kaltaistettiin vaivaisenluukulman ja seuranta-ajan suhteen. Keskimääräinen vaivaisenluu- ja intermetatarsaalikulmien korjautuminen oli preoperatiivisten ja kuuden viikon kontrollikuvien välillä 19,8 ja 8,9 astetta. Korjautumistulokset vaivaisenluu- ja intermetatarsaalikulmien osalta säilyivät hyvänä,13,4 astetta (95% luottamusväli 11,6-15,1) ja 4,5 astetta (95% luottamusväli 3,7-5,2), keskipitkänajan seurannassa. AOFAS pisteytys antoi erinomaisen tai hyvän tuloksen 72%:lle leikatuista jaloista. Kävelyanalyysissä FTJDA -ryhmän relatiivinen impulssi oli 8% korkeampi ensimmäisen jalkapöytäluun pään alla, kun taas chevron -ryhmällä todettiin päkiän keskiosan kohonnut kuormitusjakauma. Yhteenvetona voidaan todeta, että FTJDA korjaa joustavan vaivaisenluun tehokkaasti ilman I-varpaan tyvinivelen toimenpiteitä. Korjautumistulos säilyy pitkäaikaisseurannassa ja kliiniset tulokset ovat hyviä. Kävelyanalyysissä FTJDA tuottaa paremman postoperatiivisen, ensimmäisen säteen dynaamisen kuormituskapasiteetin, distaaliseen chevron -osteotomiaan verrattuna.

Abstract In the Nordic countries, event-free survival (EFS) exceeds 80% in certain groups of children treated for acute lymphoblastic leukaemia (ALL). With the improved cure rates, however, there are more children suffering from neurological late effects, especially due to therapy directed at the central nervous system (CNS). The aim of this study is to examine the changes taking place in the nervous system after leukemia treatment and to evaluate the role of treatment in these changes in patients and in an animal model. Twenty-seven ALL survivors and healthy controls were examined by means of motor evoked potentials (MEPs). ALL survivors were also examined clinically. The children with ALL continued to show decreased motor nerve conduction in the peripheral nerves, but not within the CNS, five years after the cessation of treatment. Clinical neurological findings were obtained in 33% of the cases. The MEP results indicated reversibility of the motor injury due to CNS effects. Nineteen patients underwent perfusion magnetic resonance imaging (MRI) at the cessation of treatment or 4-8 years after the treatment. Seventeen of them also underwent single-photon emission computed tomography (SPECT). The studies showed small perfusion defects in SPECT, which were not visible by perfusion MRI. Methotrexate (Mtx) neurotoxicity was studied in a swine model using functional MRI, brain perfusion SPECT, iodine-123 labelled 2β-carbomethoxy-3β-(4-iodophenyl) tropane ([123I]β-CIT) SPECT and whole-hemisphere autoradiography with [125I]β-CIT in ten Mtx-treated animals and five control animals. Mtx-related changes in the brain could be detected as reduced or negative blood-oxygen-level-dependent (BOLD) responses to somatosensory activation in BOLD contrast MRI, which indicates changes in flow metabolism coupling. Perfusion defects in brain SPECT were seen in the Mtx group and the control group, which suggests that the perfusion defects seen in brain SPECT are probably multifactorial. The change in dopamine transporter (DAT) density in the Mtx group was not different from that in the controls. The abnormalities in nerve conduction after treatment in survivors of ALL were partly reversible years after the treatment. The patients had perfusion defects in SPECT imaging which were not seen in perfusion MRI. The clinical significance of these defects remains obscure. The animal model suggested perfusion defects to be multifactorial.

Master of Public Health - MPH / Two quantitative studies were carried out in randomly-selected facilities within all nine provinces of South Africa. First, a situational assessment of these randomly selected facilities was undertaken using key informant (health care personnel) interviews and record reviews to ascertain guidelines and procedures for early identification of HIV-exposed infants (HEI), the coverage of early infant diagnosis services, the human resource capacity of the health system, and existing linkage and referral system for antenatal and postnatal PMTCT services. This was followed by the South African national PMTCT survey (SAPMTCTE) which involved a collection of infant blood samples and maternal interview data from mother-infant pairs (infants age 4-8weeks) attending six weeks immunisation service points in the selected facilities. Interviews were conducted with mothers to assess antenatal and peripartum PMTCT services received and maternal intention to request for infant HIV testing at six weeks immunisation visits. Data on gestational age at birth, infant birth weight and HIV status was extracted from the road-to-health-card (RtHC). The HIV status of mothers was determined from maternal report or enzyme immunoassay (EIA) test conducted on infants dried blood spots (DBS). A weighted analysis (weighted for sample size realisation and population live births) was performed to assess uptake of services along the PMTCT cascade. Mothers who either self-reported an HIV-positive status or had an EIA positive infant were classified as HIV-positive mothers. Perinatal ARV regimen coverage was calculated from the total number of HIV-positive mothers who received maternal azidothymidine (AZT) or HAART for any duration during pregnancy plus infant nevirapine (NVP)/AZT received at birth. Descriptive methods were used to analyse national availability of EID services and approaches for identifying HEI at the six weeks immunisation visit. Logistic regression assessed key factors influencing maternal intention to receive EID. Logistic regression was also used to explore individual, health facility and provincial level factors that explain variability in mother-to-child-transmission rates.

Magister Curationis - MCur / Background: Health care workers who sustain needle stick injuries are at risk of contracting blood-borne pathogens, e.g. Human Immunodeficiency Virus,Hepatitis B virus or Hepatitis C virus. Needle stick injuries are viewed as occupational hazards that can lead to health care workers developing acute or chronic diseases, which may lead to disability or death. Due to these healthrelated risks, health care workers are encouraged to adhere to universal precautions and standard operating procedures. In South Africa, the Occupational Health and Safety Act promulgated in 1993 required institutions draw up protocols in line with the regulations of the Act. However, if the health care workers do not comply with the protocols they may not be compensated for contracting a disease, e.g. Human Immunodeficiency Virus infection, following needle stick injuries. Aim: The aim of the study was to explore the health care workers’ perceptions of the needle stick injury protocol at a level 2 hospital in Cape Town. Research design: A qualitative approach was used to make sense of health care workers’ compliance to the protocols when sustaining a needle stick injury. An exploratory descriptive, contextual design was used to carry out an in-depth investigation of the phenomenon. Sample: The study was done at Mowbray Maternity Hospital, a level 2 obstetric hospital in Cape Town. The researcher made use of convenience, purposive sampling. Semi-structured interviews were used to collect the research data. Data collection: During the data collection phase, ethical considerations towards participants were ensured to include, among others, anonymity, autonomy and confidentiality of information. Data analysis: It included the following steps: reading and rereading,coding, displaying, reducing and interpreting the data. Findings: Some health care workers do not view sustaining a needle stick injury as risky enough to report the injury or even go for follow-up testing. This risky behaviour can have detrimental effects on their health. There is also a lack of knowledge about the institutional needle stick injury protocol. Recommendations: It is recommended to have educational and training sessions for all health care workers and new employees to familiarise them with the needle stick injury protocol and policies of the institution; to provide adequate management support 7 following work related injuries and to make health care workers aware of the consequences of non-compliance to institutional protocol.

Abstract Salla disease (SD) is a rare lysosomal storage disorder, belonging to the Finnish disease heritage. The condition leads to intellectual disabilities. Two main categories of the disease have been identified – a conventional subtype and a severe subtype. The gene locus of SD has been assigned to a restricted region on the long arm of chromosome 6. The gene SLC17A5 is responsible for lysosomal-membrane sialic acid transport. The objective of this study was to describe the neurocognitive developmental spectrum of SD in a long follow-up study. In the original study (1997–1999), the sample consisted of 41 Finnish patients with Salla disease. They were examined by a paediatric neurologist, a psychologist and a speech therapist. The follow-up study (2010–2012) concerned of 27 (66%) patients from the original SD patient sample. The study included neurological and neuropsychological investigations. A case study of a mildly affected female patient was also reported. In the first study, the typical neurocognitive profile of SD was outlined and the different phenotypes confirmed. The neurocognitive profile of SD consisted of a strong motor handicap, but also well-developed skills in verbal comprehension and interaction. In the follow-up study, the main finding was that the verbal skills related to comprehension did not diminish over time. However, the skills that demanded productive speech were worsened by both dyspraxia and dysarthria, markers of dysfunction of the cerebellum. The neurocognitive and neurological status of the mildly affected female patient remained stable during the long follow-up time. In addition the MRI findings revealed mild dysfunction. The results indicate that the neurocognitive deficits related to SD are clear in childhood, but the illness does not have a rapid progressive nature after teenage years. The motor handicap is strong but the cognitive skills related to verbal comprehension, and interactive skills, do not deteriorate in adulthood. Four different neurodevelopmental periods can be outlined. / Tiivistelmä Tämän tutkimuksen tavoitteena oli kuvata Sallan tautiin liittyvä neurokognitiivisen kehityksen kulku pitkän seurantatutkimuksen aikana. Sallan tauti, erittäin harvinainen lysosomaalinen kertymäsairaus, kuuluu suomalaiseen tautiperimään. Nämä perinnölliset sairaudet ovat Suomessa yleisempiä kuin muissa maissa. Sallan tauti etenee älylliseen kehitysvammaisuuteen. Kaksi taudin päätyyppiä, tavanomainen ja vakava-asteinen fenotyyppi, on tunnistettu. Sallan taudin aiheuttavan geenin sijainti on paikallistettu kromosomiin 6. SLC17A5-geeni vastaa sialihapon kuljetuksesta solujen lysosomeissa. Ensimmäisen tutkimuksen (1997–1999) aineisto koostui 41 suomalaisesta Sallan tautia sairastavasta potilaasta. Neurologi, psykologi sekä puheterapeutti tutkivat jokaisen potilaan. Seuranta-aineisto (2010–2012) koostui 27 (66 %) potilaasta. Tutkimukseen kuului neurologin sekä neuropsykologin tutkimus. Lieväoireisen naispotilaan kehityskulku julkaistiin erillisenä tapaustutkimuksena. Ensimmäisessä tutkimuksessa selvitettiin Sallan taudille ominainen neurokognitiivinen profiili, lisäksi vahvistettiin kahden eri fenotyypin olemassaolo. Neurokognitiivisiin tyyppioireisiin kuuluivat vahvat motoriset defektit, mutta toisaalta hyvin kehittyneet kielelliset taidot puheen ymmärtämisen osalta. Myös vuorovaikutustaidot olivat vahvat. Seurantatutkimuksen päätulos oli puheen ymmärtämisen taitojen säilyminen taudin edetessä. Puheen tuottamiseen liittyvien vaikeuksien osalta sekä dyspraksia että dysartria heikensivät kielellistä toimintakykyä. Nämä kielelliset defektit liittyvät pikkuaivojen toimintahäiriöihin. Lieväoireisen naispotilaan neurologiset ja neurokognitiiviset löydökset eivät olleet edenneet pitkän seurantatutkimuksen aikana. Myös aivojen kuvantamistutkimuksen tulokset olivat lievät. Sallan tautiin liittyvät neurokognitiiviset muutokset ovat selkeät lapsuusiässä, mutta sairauden luonne aikuisiällä ei ole nopeasti etenevä. Motorisen toimintakyvyn defektit ovat vahvat, mutta kielellisen ymmärtämisen ja vuorovaikutuksen taidot eivät heikkene aikuisilla potilailla. Taudista voidaan erotella neljä erilaista kehityksellistä vaihetta.

[pt] Esta pesquisa buscou analisar o chamado de Jesus ao rico notável, conforme o relato do Evangelho segundo Lucas, e perceber em que medida tal convite não se destina somente ao personagem em questão, mas pode ser estendido a todos aqueles que se aproximem de Jesus reconhecendo nele o caminho para a vida eterna. Concluiu-se que o núcleo do chamado não é o cumprimento dos mandamentos e nem mesmo a distribuição dos bens aos pobres, mas o seguimento de Jesus. Como decorrência, esta adesão a Cristo se traduz na acolhida da vida eterna como graça, e não conquista pessoal; além disso, aquele que acolhe tal chamado é incorporado à Igreja, à comunidade dos seguidores de Jesus. Em oposição à frustrante hesitação do rico notável, outros casos de vocação e discipulado em Lc e At mostram como a acolhida do chamado de Jesus era possível, e seria fonte de alegria duradoura. A pesquisa combinou o método histórico-crítico com métodos de caráter sincrônico, como a análise retórica. / [en] This survey sought to analyze the call of Jesus to the rich young ruler, as narrated in the Gospel according to Luke, and realize to what extent such an invite is not destinated only to the character in case, but can be extended to all those who approach Jesus recognizing in him the path to eternal life. It was concluded that the center of the call is not the accomplishment of the Commandments, and not even the distribution of the goods to the poor, but the follow of Jesus. As a consequence, this adhesion to Christ signifies the reception of eternal life as grace, and not as self achievement; besides, the one who accepts such call is incorporated to the church, the community of Jesus followers. In opposition to the frustrating hesitation of the rich young ruler, other cases of vocation and discipleship in Luke and Acts show how the acceptance of the call of Jesus was possible, and would be a source of lasting joy. The survey combined the historical-critical method with synchronic character methods, such as the rhetorical analysis.

INTRODUÇÃO: O Lúpus Eritematoso Sistêmico (LES) é uma doença inflamatória crônica que pode acometer qualquer órgão ou sistema. O acometimento do coração pode ocorrer em até 50% dos casos e não existem estudos de prevalência de eventos arrítmicos (EA) em pacientes com LES, nem de correlações laboratoriais preditoras de sua ocorrência. OBJETIVOS: Estabelecer a taxa de ocorrência de EA e identificar variáveis laboratoriais preditoras de sua ocorrência em pacientes com LES em seguimento em ambulatório de hospital terciário; estabelecer a associação entre o uso de cloroquina com a ocorrência de EA e óbitos (tipo, número e tempo de seguimento). MÉTODOS: Foi realizado um estudo clínico descritivo, observacional e aberto com pacientes em seguimento ambulatorial no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo que foram submetidos a avaliação clínica, exames laboratoriais, ECG de repouso e Holter de 24h. A associação entre as variáveis e os EA foi avaliada por meio dos testes qui-quadrado, razão de verossimilhança, teste exato de Fisher, teste t-Student, teste não-paramétrico de Mann-Whitney, regressão logística múltipla e curva ROC. RESULTADOS: Entre agosto/2005 e agosto/2006 foram estudados 325 pacientes consecutivos, sendo 8 excluídos. A idade média foi de 40,25 anos, 91% mulheres. O tempo médio do diagnóstico de LES foi de 11,36 anos e apenas 6 pacientes apresentaram critérios para atividade do LES (escore SLEDAI). Duzentos e vinte e um pacientes estavam em uso de cloroquina. Alterações ao ECG ocorreram em 66 pacientes (20,82%): 5 bloqueios atrioventriculares de 1º grau; 4 bradicardias sinusais; 4 taquicardias sinusais e 1 supraventricular; 6 bloqueios do ramo direito (BRD); 2 bloqueios do ramo esquerdo (BRE); 45 QT prolongados. Ao Holter foram identificados 4 pacientes com pausas > 2,0 segundos; 45 com FC mínima < 50bpm; 90 com extrassístoles supraventriculares (ESV); 26 com taquiarritmias supraventriculares (FA/TA); 65 com extrassístoles ventriculares (EV). Foram registrados 7 óbitos (2,47%). Idade acima de 40 anos foi preditora da ocorrência de EA (p=0,002; OR=2,523; IC 95%= 1,389-5,583). A presença do anticorpo anticardiolipina foi preditora da ocorrência de BRD/BRE (p = 0,005; OR 3,989; IC 95% = 1,615-9,852). Títulos de C3 abaixo de 105mg% foram preditores de menor probabilidade de ocorrência de FC mínima < 50bpm (p=0,016; OR=1,018; IC 95%=1,003-1,033). Os preditores para a ocorrência de EV foram a idade (p=0,002; OR=1,051; IC95%=1,018-1,085) e a duração do QRS (p=0,005; OR=1,061; IC95%=1,018-1,106); quanto mais avançada a idade e quanto mais largo o QRS, maior a probabilidade de ocorrência de EV. Para a ocorrência de TA/FA, os preditores foram a idade (p<0,001; OR=1,100; IC95%=1,050-1,154) e o tempo de uso cloroquina (p=0,035; OR=0,921; IC95%=0,853-0,994); quanto mais avançada a idade e quanto menor o tempo de uso de cloroquina, maior a probabilidade de ocorrência de TA/FA. Pacientes com mais de 50 anos e tempo de uso de cloroquina inferior a 8 anos tiveram mais TA/FA. CONCLUSÕES: Neste estudo, que avaliou pacientes com LES em seguimento ambulatorial em hospital terciário, a taxa de ocorrência de EA foi elevada; a sua correlação com variáveis laboratoriais identificou como preditores de maior ocorrência: idade acima de 40 anos, título de C3 abaixo de 105mg% e presença de anticorpo anticardiolipina. A cloroquina demonstrou efeito protetor cardíaco sobre a evolução da doença. / INTRODUCTION: Systemic Lupus Erythematosus (SLE) is a chronic inflammatory illness that can affect any organ and system. Up to 50% of patients have their heart affected and there are no prevalence studies of arrhythmic events (AE) in SLE patients and laboratory predictors are also unknown. OBJECTIVES: To establish the rate of occurrence of AE and to identify laboratory predictors in outpatients with SLE; to establish the association between chloroquine use and the occurrence of AE and death (type, number and time of follow-up). METHODS: A descriptive, observational and opened clinical study was carried out with SLE oupatients selected from the Rheumatology clinic of São Paulo University Medical School, Brazil. They were submitted to clinical evaluation, laboratory exams, resting-ECG and 24-hour Holter monitoring. Statistics: The association between the variables and the occurrence of AE was assessed by chi-square, likelihood ratio, Fishers test, t-Student, Mann-Whitney, ROC curve and logistic regressions. RESULTS: Between august/05-august/06, 325 consecutive patients were studied. Resting-ECG abnormalities were found in 66 patients, rate of 20.82%. The average age was 40.25yo, 91% female. The average time of SLE diagnosis was of 11.36y and only 6 presented criteria for diseases activity (SLEDAI score). There were 221 patients using chloroquine. ECG disturbances found: 5 1st degree AV-block; 4 sinus bradycardia; 4 sinus tachycardia and 1 supraventricular tachycardia; 6 RBBB; 2 LBBB; 45 long QT. At Holter monitoring: 4 pauses>2.0s; 45 HR<50bpm; 90 atrial ectopies; 26 atrial tachyarrhythmia; 65 ventricular ectopies. Seven death were registered (2.47%). Age above 40yo was predictor of AE (p=0.002; OR=2.5; 95%IC=1.4-5.6). Presence of anticardiolipine antibody was predictor of QRS>120ms occurrence (p = 0.005; OR 3.989; IC 95% = 1.615-9.852). C3 level bellow 105mg% was predictor of non-occurrence of HR<50bpm (p=0.02; OR=1.02;95% IC=1.003-1.03). The predictor for ventricular ectopies (VE) occurrence were age (p=0,002; OR=1,051; IC95%=1,018-1,085) and QRS duration (p=0,005; OR=1,061; IC95%=1,018-1,106); advanced age and longer QRS predicted greater probability of VE. For supraventricular tachyarrhythmia (AT/AF) the predictors were age (p<0,001; OR=1,100; IC95%=1,050-1,154) and time of Chloroquine use (p=0,035; OR=0,921; IC95%=0,853-0,994); advanced age and short time of Chloroquine use are related to greater probability of AT/AF. Patients older than 50y and using chloroquine for less than 8y had more AT/AF. CONCLUSIONS: The rate of AE occurrence was high (20%) and the correlation with laboratory variables identified predictors of occurrence of AE: age above 40 years, C3 level below 105mg% and anticardiolipin antibody. Chloroquine demonstrated cardiac protection effect.

Com o objetivo de avaliar a sobrevida e os fatores prognósticos da cardiomiopatia hipertrófica estudamos, de forma prospectiva, 214 pacientes matriculados no ambulatório de Cardiopatias Gerais do Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, entre 1980 e 1997. A idade variou de 3 a 76 anos, com média de 37±16; 102 pacientes eram do sexo masculino e 112 do sexo feminino. Os pacientes foram submetidos a eletrocardiografia, eletrocardiografia dinâmica de 24 horas, ecocardiografia e estudo hemodinâmico. A média do tempo de seguimento foi de 88±56 meses com variação de 13 a 299. Observamos 22 óbitos, sendo 14 (6%) relacionados à doença e destes, 11/14 (78%) faleceram subitamente. A probabilidade de sobrevida em cinco anos foi de 93,8%, 87,7% em dez anos e 76,4% em 15 anos. A taxa de mortalidade anual foi de 0,4%. A análise estatística univariada de cada uma das 23 variáveis clínicas e das 20 obtidas pelos exames complementares mostrou que apenas a forma familiar foi fator de risco de óbito. As variáveis forma familiar, idade menor que 20 anos, síncope e classe funcional foram selecionadas para análise multivariada pelo modelo de Cox e método stepwise. Novamente, a forma familiar foi identificada como fator . de risco independente de óbito. Podemos concluir, com base em nossos dados de casuística de centro de referência que, a longo prazo, a sobrevida dos pacientes portadores de cardiomiopatia hipertrófica foi benigna e que a forma familiar é fator de risco de óbito. / To evaluate the prognostic factors related to long-term survival in hypertrophic cardiomyopathy, 214 outpatients were prospectively studied from 1980 to 1997 at the Equipe de Cardiopatias Gerais do Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. There were 102 male and 112 female patients, aged 37±16 years, ranging from 3 to 76 years. They underwent electrocardiography, 24-h ambulatory electrocardiographic recording, echocardiography and hemodynamic study. There were 22 deaths, 14 (6%) of them as a direct consequence of the disease, after a mean follow up of 88±56 months (13 - 299). Eleven of these deaths (78%) were sudden and unexpected. The cumulative survival rates were 93.8% in 5 years, 87.7% in 10 years and 76.4% in 15 years. The annual mortality rate was 0.4%. Univariate analysis was performed taking into account 23 clinical variables and 20 variables obtained from laboratory tests. Only family history was shown to be associated with cardiac mortality. In addition, four known adverse factors such as family history, young age, syncope, and functional class were chosen for entering a Cox's multivariate stepwise model. Again, only family history was identified as an independent risk factor for cardiac death. We concluded, based on this selected population from a referral center, that long-term survival of patients with hypertrophic cardiomyopathy is benign and related to family history.

The purpose of this project was to create a booklet useful to teachers, one that would increase their technological competence and so encourage them to use new technologies.

Background and problems: ​In a market with increased competitiveness, it's important for companies to achieve superior performance. Thus by being quick at meeting new market conditions and customer needs. To achieve superiority, kaizen is a suitable tool to use. The kaizen-philosophy comprises to constantly make improvements in everyday work and require commitment from all parties in a company. Some improvements work advantageously occasionally while others give good and long lasting effects. It may be difficult for organizations to maintain the results of kaizen improvements and the effects may diminish some time after implementation. Generally for companies that work according to the philosophy is that some improvements are implemented without adequate guidance and resources. This results in frustration since the improvements do not remain successful. Whether a kaizen-improvement implementation is successful has many influencing factors. In order to investigate this problem, Scania has been studied in regard of the company's good experience in applying the kaizen-philosophy. Previous research and literature have been investigated in order to support and analyze the topic. Thus, this study's research question is "What factors affect success and survival in implementation of a kaizen-improvement?". 5 Purpose:​ The purpose with this case study is to create a profound understanding of underlying factors which are critical for implementing kaizen-improvements and its further survival. Thus to understand what characterizes an successful implementation of kaizen-improvements. By successful kaizen improvement is meant an improvement that is standardized and becomes a beneficial part of the company. Method: ​This thesis is based on a qualitative research strategy. The research design is a single case study with semi-structured interviews as a method for the empirical material that has been collected. Conclusion: ​In the implementation process ten successfactors were identified in three different areas where Scania experienced difficulties with kaizen. In the first area, difficulties were due to cheating in the preparation stage. Here, the following successfactors were identified: An accurate and favourable current situation analysis, communication of the current situation and a good competence assessment. The second area was due to difficulties in implementing new working methods. Here, the following successfactors were identified: Communication of goals, employee training and favourable follow-up. The last area was due to difficulties in motivation employees. Here, the following successfactors were identified: Commitment and participation, responsibility, avoiding guilt and good leadership.