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1.
Prev Med ; 127: 105817, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31445113

RESUMO

Smoking during pregnancy is associated with a multitude of health behaviors and with the psychosocial and socio-economic circumstances of pregnant women. Limited research has so far been conducted on the clustering of these characteristics and on their effect on pregnancy outcomes. This study aimed to identify different groups of pregnant women based on their behavioral, psychosocial and socio-economic characteristics and their pregnancy outcomes. In total, 2455 women who were 12 weeks pregnant completed a questionnaire on smoking behavior, health behaviors and psychosocial and socio-economic characteristics. Neonatal and maternal outcomes were extracted from the Dutch perinatal registration. Subgroups were identified with latent class analysis and adverse pregnancy outcomes were compared between subgroups with logistic regression. Women were classified into four latent classes. Two classes represented the healthy higher-educated pregnant women who did not smoke: one group of multigravida women and one of primigravida women, also characterized by less pregnancy-specific knowledge and more pregnancy-related stress. The remaining women were grouped into two less healthy groups. One group frequently quit smoking, reported less healthy eating, less physical activity and comparable stress levels as the healthy higher-educated groups. The last group contained the most smokers, had the highest scores on psychosocial and pregnancy-related stress and the most adverse socio-economic circumstances. This group had an increased risk of adverse maternal outcomes, in particular developing diabetes during pregnancy. A comprehensive and integrated approach is needed to improve outcomes in pregnancies with a combination of adverse health, psychosocial, and socio-economic conditions.


Assuntos
Comportamentos Relacionados com a Saúde , Conhecimentos, Atitudes e Prática em Saúde , Resultado da Gravidez , Gestantes/psicologia , Fumar/efeitos adversos , Estresse Psicológico/psicologia , Adulto , Exercício Físico , Feminino , Humanos , Países Baixos , Gravidez , Fumar/psicologia , Fatores Socioeconômicos , Inquéritos e Questionários
2.
Health Educ Res ; 27(3): 459-69, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22350193

RESUMO

Preventing smoking initiation among adolescents of lower socio-economic groups is crucial for the reduction of socio-economic inequalities in health. The aim of the present study was to examine whether effective smoking prevention interventions in Europe are equally effective among adolescents of low- and high-socio-economic status (SES). As part of the European Union-funded TEENAGE project, three school-based smoking prevention intervention studies in Europe were selected for secondary analyses: (i) a Dutch class competition intervention, (ii) the European Smoking Prevention Framework (ESFA) study and (iii) the A Stop Smoking in Schools Trial (ASSIST) intervention. All three studies differed in effectiveness by SES. The Dutch class competition study only had a significant effect among higher SES adolescents. The results for the ESFA study and ASSIST study were mixed and depended on which SES indicator was used. The conclusion of the study is that stratified analyses provide important insights in differential intervention effects for higher and lower socio-economic groups. Although findings from the different studies were mixed, interventions that use a social network approach in which youngsters are allowed to deliver the intervention themselves may be a successful strategy in targeting adolescents from lower socio-economic groups.


Assuntos
Comportamento do Adolescente , Prevenção do Hábito de Fumar , Fumar/economia , Classe Social , Adolescente , Criança , Europa (Continente) , Feminino , Humanos , Masculino , Países Baixos , Ensaios Clínicos Controlados Aleatórios como Assunto , Instituições Acadêmicas , Resultado do Tratamento
3.
Prev Med ; 52(1): 53-9, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21078340

RESUMO

INTRODUCTION: A smoking prevention program was developed to prepare children in elementary school for secondary school. This study assessed the effects on smoking in secondary school. METHODS: In 2002, 121 schools in The Netherlands were randomly assigned to the intervention or control group. The intervention group received 3 lessons in 5th grade of elementary school and a second 3 lessons in 6th grade. The control group received "usual care". Students completed 5 questionnaires: before and after the lessons in 5th and 6th grade and in the first class of secondary school. At baseline, 3173 students completed the questionnaire; 57% completed all questionnaires. RESULTS: The program had limited effect at the end of elementary school. One year later in secondary school significant effects on behavioral determinants and smoking were found. The intervention group had a higher intention not to smoke (ß=0.13, 95% confidence interval=0.01-0.24) and started to smoke less often than the control group (odds ratio=0.59, 95% confidence interval=0.35-0.99): smoking increased from 2.5% to 3.6% in the intervention group and from 3.2% to 6.5% in the control group. Girls showed the largest differences in smoking between intervention and control condition. CONCLUSIONS: A prevention program in elementary school seems to be effective in preventing smoking.


Assuntos
Prevenção do Hábito de Fumar , Estudantes/psicologia , Criança , Análise por Conglomerados , Feminino , Promoção da Saúde , Humanos , Masculino , Avaliação de Programas e Projetos de Saúde , Instituições Acadêmicas , Inquéritos e Questionários
4.
Ned Tijdschr Geneeskd ; 151(46): 2566-9, 2007 Nov 17.
Artigo em Holandês | MEDLINE | ID: mdl-18074727

RESUMO

OBJECTIVE: To establish smoking prevalence of fertile-aged women; before and during pregnancy, and 6 months after delivery. DESIGN: Cross-sectional. METHOD: Yearly surveys by questionnaires handed out during 2001-2003 to mothers visiting a Well Baby Clinic with infants aged 0-6 months. RESULTS: Out ofa total of 14,540 questionnaires, 9133 (63%) were completed and returned. Before pregnancy 25% of all the women concerned smoked, 6% stopped 6 months before getting pregnant; this percentage rose between 2001 (5%) and 2003 (7%). During pregnancy, 14% of the women smoked. This percentage was significantly lower in 2002 and 2003 (13%) compared to 16% in 2001. 11% smoked while pregnant (average 5 cigarettes daily), and 3% stopped some time during pregnancy. Of the mothers who quitted smoking before or during pregnancy, two thirds did so permanently. Nevertheless 15% of all women smoked during the first half year after delivery. This percentage was lowest in 2003 (14%) and highest in 2001 (17%). 28% of all mothers with infants aged 0-6 months had partners who smoked (daily 10 cigarettes average), these women were more likely to start smoking again (RR: 2). CONCLUSION: Minor positive changes were observed within the study period. A smoking partner contributed to smoking relapse after delivery.


Assuntos
Período Pós-Parto , Complicações na Gravidez/prevenção & controle , Cuidado Pré-Natal/métodos , Abandono do Hábito de Fumar , Fumar/epidemiologia , Adulto , Estudos Transversais , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Países Baixos/epidemiologia , Gravidez , Resultado da Gravidez , Fumar/efeitos adversos , Abandono do Hábito de Fumar/psicologia , Abandono do Hábito de Fumar/estatística & dados numéricos , Inquéritos e Questionários
5.
Health Educ Behav ; 33(2): 178-96, 2006 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-16531512

RESUMO

This study assessed the antecedents of continued use of an education program to prevent passive smoking in infants. It consists of a booklet for parents and a manual for health professionals describing a five-step procedure for discussing passive smoking. A questionnaire was sent to 67 managers, 670 nurses, and 335 physicians working in well-baby clinics (response rate: 70%, 53%, 47% respectively). Questions concerned the completeness of use, level of institutionalization, and characteristics of the organization, the user, and the dissemination strategy. Seventy-one percent of nurses and 42% of physicians worked with the program. They foremost provided the first three steps of the five-step procedure. Physicians' completeness of use was related to their perceived responsibility in providing this education, and nurses' use was related to their perceived self-efficacy, responsibility, training attendance, participation in the adoption decision, and level of institutionalization. Diffusion efforts should focus on improving the completeness of use and level of institutionalization.


Assuntos
Atitude do Pessoal de Saúde , Proteção da Criança , Educação em Saúde/organização & administração , Bem-Estar do Lactente , Centros de Saúde Materno-Infantil/organização & administração , Pais/educação , Padrões de Prática Médica/estatística & dados numéricos , Poluição por Fumaça de Tabaco/prevenção & controle , Pessoal Administrativo , Adulto , Pré-Escolar , Educação em Saúde/métodos , Humanos , Lactente , Recém-Nascido , Manuais como Assunto , Pessoa de Meia-Idade , Países Baixos , Papel do Profissional de Enfermagem , Folhetos , Pais/psicologia , Papel do Médico , Autoeficácia , Prevenção do Hábito de Fumar , Inquéritos e Questionários , Materiais de Ensino
6.
Eur J Public Health ; 13(3): 269-74, 2003 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-14533732

RESUMO

BACKGROUND: Passive smoking is harmful to young children. A protocol has been developed to allow health care workers to communicate with parents about preventing passive smoking. The main message was to refrain from smoking in the presence of the child. The aim of the study was to assess the effectiveness of this education programme. METHOD: The prevalence of smoking in the presence of infants aged 0-10 months was compared before and after the implementation of the education programme. National samples of mothers completed questionnaires in 1996 (n = 1,129) and in 1999 (n = 2,534). Questions were asked about smoking in the living room in the presence of infants, and about parental smoking, and background characteristics. RESULTS: The prevalence of passive infant smoking decreased from 41% to 18%. The adjusted odds ratio for passive infant smoking in 1999 compared to 1996 was 0.34 (0.26-0.44) when none of the parents smoked, 0.19 (0.14-0.27) when one of the parents smoked, and 0.30 (0.20-0.44) when both parents smoked. CONCLUSION: The implementation of this health education programme seems to have been very successful in reducing passive smoking in children. Implementation of similar health education programmes in other countries is recommended.


Assuntos
Educação em Saúde/organização & administração , Programas Nacionais de Saúde/organização & administração , Pais , Pais/educação , Poluição por Fumaça de Tabaco/prevenção & controle , Adulto , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Países Baixos , Pais/psicologia , Prevalência , Avaliação de Programas e Projetos de Saúde , Fumar/epidemiologia , Inquéritos e Questionários
7.
J Epidemiol Community Health ; 57(9): 675-80, 2003 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-12933772

RESUMO

OBJECTIVE: To assess the effect of an antismoking intervention focusing on adolescents in lower education. Students with lower education smoke more often and perceive more positive norms, and social pressure to smoke, than higher educated students. An intervention based on peer group pressure and social influence may therefore be useful to prevent smoking among these students. DESIGN: Group randomised controlled trial. SETTING: 26 Dutch schools that provided junior secondary education. SUBJECTS: 1444 students in the intervention and 1118 students in the control group, all in the first grade, average age 13 years. INTERVENTION: Three lessons on knowledge, attitudes, and social influence, followed by a class agreement not to start or to stop smoking for five months and a class based competition. MAIN OUTCOME MEASURES: Comparison of smoking status before and immediately after and one year after the intervention, using multilevel analysis. RESULTS: In the intervention group, 9.6% of non-smokers started to smoke, in the control group 14.2%. This leads to an odds ratio of 0.61 (95% CI= 0.41 to 0.90) to uptake smoking in the intervention group compared with the control group. One year after the intervention, the effect was no longer significant. CONCLUSIONS: In the short-term, an intervention based on peer pressure decreases the proportion of adolescents with lower education who start smoking. Influencing social norms and peer pressure would therefore be a promising strategy in terms of preventing smoking among adolescents. The results also suggest that additional interventions in later years are needed to maintain the effect.


Assuntos
Comportamento do Adolescente , Educação em Saúde/métodos , Prevenção do Hábito de Fumar , Adolescente , Criança , Feminino , Seguimentos , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Masculino , Países Baixos , Razão de Chances , Grupo Associado , Serviços de Saúde Escolar , Estatística como Assunto
8.
Hum Mol Genet ; 12(11): 1241-52, 2003 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-12761039

RESUMO

Germline mutations of the fumarate hydratase (FH, fumarase) gene are found in the recessive FH deficiency syndrome and in dominantly inherited susceptibility to multiple cutaneous and uterine leiomyomatosis (MCUL). We have previously reported a number of germline FH mutations from MCUL patients. In this study, we report additional FH mutations in MCUL and FH deficiency patients. Mutations can readily be found in about 75% of MCUL cases and most cases of FH deficiency. Some of the more common FH mutations are probably derived from founding individuals. Protein-truncating FH mutations are functionally null alleles. Disease-associated missense FH changes map to highly conserved residues, mostly in or around the enzyme's active site or activation site; we predict that these mutations severely compromise enzyme function. The mutation spectra in FH deficiency and MCUL are similar, although in the latter mutations tend to occur earlier in the gene and, perhaps, are more likely to result in a truncated or absent protein. We have found that not all mutation-carrier parents of FH deficiency children have a strong predisposition to leiomyomata. We have confirmed that renal carcinoma is sometimes part of MCUL, as part of the variant hereditary leiomyomatosis and renal cancer (HLRCC) syndrome, and have shown that these cancers may have either type II papillary or collecting duct morphology. We have found no association between the type or site of FH mutation and any aspect of the MCUL phenotype. Biochemical assay for reduced FH functional activity in the germline of MCUL patients can indicate carriers of FH mutations with high sensitivity and specificity, and can detect reduced FH activity in some patients without detectable FH mutations. We conclude that MCUL is probably a genetically homogeneous tumour predisposition syndrome, primarily resulting from absent or severely reduced fumarase activity, with currently unknown functional consequences for the smooth muscle or kidney cell.


Assuntos
Fumarato Hidratase/genética , Neoplasias Renais/genética , Leiomiomatose/genética , Mutação , Neoplasias Cutâneas/genética , Neoplasias Uterinas/genética , Erros Inatos do Metabolismo dos Aminoácidos/genética , Sequência de Aminoácidos , Estabilidade Enzimática , Feminino , Fumarato Hidratase/química , Fumarato Hidratase/deficiência , Fumarato Hidratase/metabolismo , Predisposição Genética para Doença , Mutação em Linhagem Germinativa , Humanos , Neoplasias Renais/secundário , Leiomiomatose/patologia , Dados de Sequência Molecular , Conformação Proteica , Estabilidade de RNA , RNA Mensageiro/metabolismo , Homologia de Sequência de Aminoácidos , Neoplasias Cutâneas/patologia , Neoplasias Uterinas/patologia
10.
Prev Med ; 32(3): 209-17, 2001 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-11277677

RESUMO

BACKGROUND: The aim of this study was to assess the factors that influence smoking in the presence of the infant by mothers, partners, other family members, and friends. METHODS: An observational study using questionnaires was performed with smoking and nonsmoking parents of babies between 1 and 14 months old attending Dutch well-baby clinics between February and May 1996. The main measures were prevention of passive smoking in children by mothers and the relation with self-reported attitudes, social influence, and self-efficacy. RESULTS: A total of 1702 parents completed the questionnaire (63%). A total of 1551 questionnaires were completed by the mother. Sixty-five percent of the mothers prevented passive smoking by their child. This figure was 55% for smokers and 69% for nonsmokers. Attitude was the factor that most explained preventive behavior among both smokers and nonsmokers. Among the respondents, a lack of prevention of passive smoking was significantly related to (1) a negative attitude and 2) a negative social influence exerted by their partner, (3) lower self-efficacy in reducing passive smoking, and (4) increasing age of the child. (5) Finally, a lack of prevention is associated with the mother's self-efficacy in asking others not to smoke. This association strongly differs between smoking and nonsmoking mothers. CONCLUSION: The results suggest that health education efforts should focus on attitude and self-efficacy, assuming that these precede actual behavior, and in particular on the health consequences of the exposure of young children to tobacco smoke. The information should not be restricted to parents of newborn babies; it should also focus on parents with older children. Particular attention should be paid to smokers with a low educational level. The results also indicate that education should strengthen the ability of nonsmoking parents to deal with smokers and the ability of smoking parents to deal with their own smoking behavior.


Assuntos
Mães/psicologia , Poluição por Fumaça de Tabaco , Adulto , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Recém-Nascido , Masculino , Países Baixos , Análise de Regressão , Autoeficácia , Inquéritos e Questionários , Poluição por Fumaça de Tabaco/prevenção & controle
12.
Patient Educ Couns ; 39(2-3): 149-53, 2000 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-11040713

RESUMO

The objective of the study was to assess the prevalence of passive smoking in infancy. This was done by self-report questionnaires completed by parents who attended the well-baby clinic in the period February-May 1996. A total of 2720 questionnaires were spread among parents with babies between 1 and 14 months: smoking and non-smoking parents. The questionnaires contained questions on smoking habits, smoking at home, smoking in presence of the baby. A total of 1702 parents filled in and returned the questionnaire (63%); 24% of the mothers and 33% of their partners smoked. In 44% of the families, one or more persons smoked; 22% of the mothers and 26% of the partners smoked at home. In 39% of the families, one or both parents smoked at home; 42% of the babies were exposed to tobacco smoke in the living-room, 8% were exposed in the car, and 4% during feeding. In cases where only the mother smoked, 13% of the infants were exposed to tobacco smoke during feeding. In the families where only the partner smoked, the babies were predominantly exposed to smoke in the car (18%). If both parents smoked, the child was most frequently exposed to tobacco smoke in the living-room (73%). It can be concluded that health workers, nurses, pediatricians and family physicians should be advised to inform parents systematically of the harmful effects of passive smoking in infancy. If parents are unable or unwilling to stop smoking, it is important to advise them to refrain from smoking in the presence of the baby.


Assuntos
Fumar/epidemiologia , Poluição por Fumaça de Tabaco/estatística & dados numéricos , Adulto , Estudos Transversais , Saúde da Família , Feminino , Humanos , Lactente , Masculino , Avaliação das Necessidades , Países Baixos/epidemiologia , Pais/educação , Pais/psicologia , Prevalência , Inquéritos e Questionários
13.
Eur J Pediatr Surg ; 10 Suppl 1: 18-9, 2000 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-11214825

RESUMO

AIM: To establish the prevalence of Chiari malformations and hydrocephalus (HS) in adults with spina bifida (SB). INTRODUCTION: Adults with SB have recently been shown to be at risk from significant deterioration arising from the C/HS complex. Little is known about the prevalence of these malformations in adulthood, their natural history, risk factors for deterioration and optimum management. MATERIALS AND METHODS: Patients are recruited from a clinic for adults with SB and hydrocephalus, routinely questioned about recent changes in neurological symptoms and a full neurological examination performed. Functional assessments include: Barthel Index; Nottingham EADL; Nine Hole Peg Test; 10 metre timed walk test. Sagittal T1 and TSE (turbo spin-echo) magnetic resonance images (MRI) of the cranio-cervical junction and spinal cord are obtained using a 1.0-T MRI system with a phased-array surface coil. Scans are reported blindly. RESULTS: 76 patients have been assessed, 40 female, 36 male, mean age 27.5 years. Of these, 48 (63.2%) have symptoms +/- signs compatible with C/HS, but only 18 (23.7%) have reported new, potentially relevant, neurological symptoms over the previous 12 months. Of 25 patients undergoing MRI so far, 19 (76%) have had Chiari malformations and 12 (48%) have had HS. None of those with a negative scan have had signs/symptoms suggestive of the C/HS complex but a negative clinical evaluation has been unable to exclude such pathology. CONCLUSIONS: These early results suggest that the prevalence of the C/HS complex is high in adults with SB and cannot be predicted by neurological examination alone. Given past experience, the need for continued neurological follow up in a large proportion of the SB population throughout adulthood is likely to be essential.


Assuntos
Malformação de Arnold-Chiari/epidemiologia , Disrafismo Espinal/complicações , Siringomielia/epidemiologia , Atividades Cotidianas , Adulto , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Irlanda do Norte/epidemiologia , Prevalência , Siringomielia/complicações , Siringomielia/patologia
15.
J Bone Joint Surg Br ; 78(3): 488-91, 1996 May.
Artigo em Inglês | MEDLINE | ID: mdl-8636193

RESUMO

We report a 45-year follow-up of a patient with Engelmann's disease previously described in 1950, showing progression of the disease with unique involvement of the femoral capital epiphyses. The case is compared with others to add some information about the later stages of a disease which is not fully understood.


Assuntos
Síndrome de Camurati-Engelmann/diagnóstico por imagem , Idoso , Síndrome de Camurati-Engelmann/complicações , Síndrome de Camurati-Engelmann/patologia , Síndrome de Camurati-Engelmann/fisiopatologia , Cabeça do Fêmur/diagnóstico por imagem , Seguimentos , Marcha , Humanos , Masculino , Dor/etiologia , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X
16.
J Bone Joint Surg Br ; 75(1): 115-8, 1993 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-8421006

RESUMO

Seven patients with osteoid osteoma of the proximal femur were treated by percutaneous excision of the nidus. The combination of preoperative localisation by tomography and intraoperative localisation by image intensifier resulted in a curative procedure with minimal bone resection in all cases, although a second operation was required in one patient.


Assuntos
Neoplasias Femorais/cirurgia , Osteoma Osteoide/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Neoplasias Femorais/diagnóstico por imagem , Humanos , Masculino , Osteoma Osteoide/diagnóstico por imagem , Intensificação de Imagem Radiográfica , Tomografia por Raios X
17.
Cancer ; 67(4): 1070-5, 1991 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-1991254

RESUMO

The incidence, clinical characteristics, and outcome of hypersensitivity reactions to teniposide (VM-26), etoposide (VP-16), or both were determined in 108 children with acute lymphoblastic leukemia (ALL) treated with a contemporary regimen of intensive multiagent chemotherapy. Fifty (46%) of the 108 patients had one or more hypersensitivity reactions. The risk of any child having an initial reaction over the cumulative dose range studied was 52% (95% confidence limits, 41% and 63%) for VM-26, compared with 34% (95% confidence limits, 24% and 44%) for VP-16. The risk of having an initial reaction to VM-26 or VP-16 was clearly related to the cumulative dose. This risk peaked at 1500 to 2000 mg/m2 for VM-26 and at 2000-3000 mg/m2 for VP-16. All reactions were Type 1 reactions according to the Gell and Coombs classification, characterized by urticaria, angioedema, flushing, rashes, or hypotension, and 86% of reactions were of Grade 1 or 2 severity according to standard criteria. There was no evidence of increasing clinical severity on repeated rechallenge with premedication, and no deaths occurred. The findings suggested that hypersensitivity reactions to epipodophyllotoxins in children with ALL are more common than previously reported, but only rarely constitute dose-limiting toxicity.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Hipersensibilidade a Drogas/etiologia , Etoposídeo/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Teniposídeo/efeitos adversos , Adolescente , Corticosteroides/uso terapêutico , Espasmo Brônquico/induzido quimicamente , Criança , Pré-Escolar , Cianose/induzido quimicamente , Difenidramina/uso terapêutico , Relação Dose-Resposta a Droga , Hipersensibilidade a Drogas/prevenção & controle , Etoposídeo/administração & dosagem , Feminino , Humanos , Hipotensão/induzido quimicamente , Lactente , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Pré-Medicação , Teniposídeo/administração & dosagem
18.
Skeletal Radiol ; 19(4): 245-50, 1990.
Artigo em Inglês | MEDLINE | ID: mdl-2353208

RESUMO

The radiographic features of a unique autosomal dominant bone dysplasia are presented. The features are classified as generalised and/or focal. Generalised features are either altered trabecular pattern or modelling abnormalities. Focal features comprise lytic areas which progressively enlarge, producing expansion of the bone and eventual disintegration due to fibrous and finally fatty replacement of the normal medulla. Almost 90% of these lesions occur in the appendicular skeleton. Clinically, hearing loss is the earliest manifestation of the disease, presenting sometimes as early as 4 years of age. Apical and cervical resorption of teeth is extremely common, resulting in premature loss of teeth. Radiologically, the differential diagnosis refers to Paget's disease, polyostotic fibrous dysplasia, and osteofibrous dysplasia. The progressive destruction of the bone is similar to massive osteolysis (Gorham's disease). The radiographic features in combination with the histopathology render the condition unique.


Assuntos
Doenças do Desenvolvimento Ósseo/diagnóstico por imagem , Osteólise/genética , Adulto , Doenças do Desenvolvimento Ósseo/complicações , Doenças do Desenvolvimento Ósseo/genética , Osso e Ossos/diagnóstico por imagem , Surdez/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteólise/diagnóstico por imagem , Linhagem , Radiografia
19.
Blood ; 74(1): 409-15, 1989 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-2752120

RESUMO

To assess the frequency and significance of nonrandom abnormalities of chromosome 9p in childhood acute lymphoblastic leukemia (ALL), we analyzed our experience with 398 consecutive cases with completely banded karyotypes. Forty cases (10%) with abnormalities of 9p were identified: 26 with deletions, nine with unbalanced translocations resulting in the loss of 9p material, and five with apparently balanced reciprocal translocations. As compared with children with ALL lacking 9p abnormalities, these 40 cases were significantly older, had higher initial circulating WBC counts, more "lymphomatous" disease characteristics (including presence of a mediastinal mass in 15%. T-cell phenotype in 26%, splenomegaly greater than 8 cm in 25%), an increased failure rate in the first 2 to 3 years after diagnosis, and a higher incidence of extramedullary relapse. Conversely, lymphomatous ALL cases were twice as likely (19% v 8%) to have an abnormality of chromosome 9p than ALL cases lacking lymphomatous features (P = .01). The finding of an abnormal chromosome 9p, however, was not specific for lymphomatous ALL or T-cell lineage, because most cases were neither lymphomatous nor T-cell, and the overall Kaplan-Meier distribution of treatment failures for abnormal 9p cases was not statistically significantly different from control ALL cases receiving the same treatment who lacked abnormalities of 9p (P = .06, by log-rank test). We conclude that nonrandom abnormalities of chromosome 9p, especially a breakpoint in 9p21-22, occur with increased frequency in childhood ALL in association with some high-risk clinical features. Despite this association, the chromosome anomaly is nonspecific in its syndrome delineation and confers no major adverse consequence on long-term survival of childhood ALL treated with modern therapy. However, due to an apparently increased hazard of involvement of the CNS (eight of 17 failures), it may be inadvisable to lessen the intensity of CNS preventive therapy for this group of patients.


Assuntos
Cromossomos Humanos Par 9 , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Criança , Humanos , Cariotipagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Prognóstico , Fatores de Risco
20.
J Bone Joint Surg Br ; 70(5): 807-11, 1988 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-3192585

RESUMO

We reviewed 53 cases of synovial chondromatosis and compared their clinical, radiological and pathological features. A radiological diagnosis is possible with increasing frequency as the disease progresses; in the early phase arthrography is helpful. Radiologically the disease may be classified as either extra-articular, or intra-articular; the intra-articular variety may be localised or generalised. Recurrence after operation was seen in 11.5% and was much the same after either synovectomy or simple removal of loose bodies. A protocol for treatment is proposed.


Assuntos
Condroma/diagnóstico por imagem , Membrana Sinovial , Adolescente , Adulto , Idoso , Artrografia , Condroma/patologia , Condroma/cirurgia , Feminino , Humanos , Artropatias/diagnóstico por imagem , Corpos Livres Articulares/diagnóstico por imagem , Corpos Livres Articulares/etiologia , Masculino , Pessoa de Meia-Idade
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