Risco crescente de melanoma de pele no Brasil

A ocorrência de melonoma maligno de pele no Brasil é analisada a partir dos dados de mortalidade disponíveis no Ministério da Saúde e dos dados de incidência dos seis Registros de Câncer de Base Populacional, localizados em seis capitais brasileiras. Os coeficientes de incidência nessas capitais situam-se em padrões intermediários se comparadas às cifras mundiais. Para o Município de Porto Alegre, uma das capitais estudas, que apresentou os maiores coeficientes de incidência, é feita comparação entre os dados relativos ao período 1979-1982 e 1987, constatando-se que houve aumento relativo de 38% entre homens e de 11% entre mulheres. Concluiu-se pela necessidade de se conduzir estudos no Brasil entre comunidades de indivíduos de pele clara, os quais apresentam risco potencializado para o desenvolvimento de melanoma, para que sejam definidas medidas específicas e eficazes de controle.

Strength, muscle quality and functional capacity in liver transplanted familial amyloidotic polineuropathy patients

Liver transplantation is the unique treatment for several end stage diseases. Familial Amiloidotic Polineuropathy (FAP) is a neurodegenerative disease related with systemic deposition of amyloidal fibre mainly on peripheral nervous system, clinically translated by an autonomous sensitive-motor neuropathy with severe functional limitations in some cases. The unique treatment for FAP disease is a liver transplant with a very aggressive medication to muscle metabolism and force production. To our knowledge there are no quantitative characterizations of body composition, strength or functional capacity in this population. The purpose of this study was to compare levels of specific strength (isometric strength adjusted by lean mass or muscle quality) and functional capacity (meters in 6 minutes walk test) between FAP patients after a liver transplant (4.1±2 months after transplant surgery) (FAPT) and a healthy group (HG).

Bronzeamento e risco de melanoma cutâneo: revisão da literatura

Estudos epidemiológicos sugerem a relação entre comportamentos relacionados ao bronzeamento e risco elevado de melanoma. Nesse sentido, realizou-se revisão sobre essa temática que abrangeu o período correspondente aos anos de 1977 a 1998. Foram pesquisadas as bases de dados Medline e Embase (Excerpta Medica). A análise mostrou que entre os jovens, apesar do conhecimento sobre os riscos da exposição excessiva à radiação ultravioleta e sobre as práticas visando à proteção da pele, prevalece o hábito de expor-se intencionalmente ao sol. Esse hábito é alimentado por crenças e atitudes em relação ao bronzeado e estimulado por influência do grupo e de pessoas consideradas "referências". As práticas mais freqüentemente adotadas para bronzear a pele apresentam risco elevado para o desenvolvimento de melanoma. Conclui-se que a forma mais eficaz de prevenir o melanoma é divulgar nos meios de comunicação que a pele bronzeada não é saudável, pois foi danificada pela radiação ultravioleta solar; e iniciar campanhas com ações efetivas para mudar comportamentos, naquilo que os motiva e os alimenta.

The impact of exercise training on liver transplanted familial amyloidotic polyneuropathy (FAP) patients

Liver transplantation is nowadays the only effective answer to adjourn the outcome of functional limitations associated with familial amyloidotic polyneuropathy (FAP), a neurodegenerative disease characterized by sensory and motor polyneuropathies. Nevertheless, there is a detrimental impact associated with the after-surgery period on the fragile physical condition of these patients. Exercise training has been proven to be effective on reconditioning patients after transplantation. However, the effects of exercise training in liver transplanted FAP patients have not been scrutinized yet.

Intra-familial physical violence among Mexican and Egyptian youth

OBJECTIVE: To determine the prevalence of experiencing intra-familial violence among Mexican and Egyptian youth and to describe its associated risk factors. METHODS: Data from questionnaires applied to 12,862 Mexican and 5,662 Egyptian youth, aged 10 to 19, who attended public schools were analyzed. Biviarate and logistic regression analysis were used to determine the relationship between socio-demographics, the experience of intra-familial violence and violence perpetration. RESULTS: The prevalence of having experienced intra-familial violence was comparable across the Mexican and Egyptian populations (14% and 17%, respectively). In Mexico, young men were more likely to have experienced such violence (OR=2.36) than women, whereas in Egypt, young women were at slightly greater risk than young men (OR=1.25). Older age, male gender and urban residence were independent correlates of experiencing intra-familial violence among Mexican youth. For Egyptian adolescents, in contrast, younger age, female gender and having non-married parents were independent correlates of victimization. Intra-familial violence victims were also more likely than non-victims to perpetrate violence (Mexico: OR=13.13; Egypt: OR=6.58). CONCLUSIONS: Mexican and Egyptian youth experienced intra-familial violence at a relatively low prevalence when compared with youth of other countries. A strong association was found between experiencing intra-familial violence and perpetrating violence.

A perfusão isolada dos membros no melanoma maligno (a experiência do IPO-Porto)

Parte I - O contexto Neste capítulo faz-se a contextualização essencial do tema, dividindo-a em duas secções. A primeira secção apresenta conceitos relativos à patologia principal, o melanoma, apesar desta técnica não se aplicar só em doentes com esta patologia, mas considerando o peso da mesma na casuística da instituição, idêntico ao de outras instituições internacionais. A segunda secção apresenta o enquadramento da técnica e da terapêutica da Perfusão Isolada dos Membros, destacando as principais alterações ocorridas nos protocolos dos centros que a praticam, ao longo das últimas décadas. Parte II - A técnica e a terapêutica no IPOFG-Porto Neste capítulo é explicado passo a passo o procedimento da Perfusão Isolada dos Membros, segundo o protocolo do IPOFG-Porto, ilustrando cada passo com imagens de um caso real. Este procedimento apoia-se na técnica da circulação extracorporal, para aplicar uma terapêutica, a perfusão em dose elevada de uma dose pré-determinada de um ou mais citostáticos. São depois enumeradas as características essenciais de cada um dos citostáticos que foram e são usados na PIM ou associados a ela, terminando com a exposição da casuística da instituição, trabalhada apenas em contexto descritivo. Parte III - O seguimento dos doentes Neste capítulo elencam-se e explicam-se as variáveis em estudo no follow-up destes doentes, quer no pós-operatório imediato, quer no seguimento em ambulatório. O follow-up destes doentes pode ser muito longo, quase sempre dependendo do estadiamento prévio da doença antes da realização da PIM. O tipo de resposta clínica deve obter-se entre os 3 e os 6 meses pós PIM, mas pode obter-se antes ou depois. Todas as variáveis estão perfeitamente definidas internacionalmente e dão um contributo específico para o conhecimento da história natural da doença, mas para os doentes a que assume maior impacto é a necessidade, ou o evitar, da amputação do membro. São apresentados os dados destas variáveis relativos ao IPOFG-Porto. Parte IV - As reflexões Neste capítulo são feitas algumas reflexões sobre o custo, a sobrevivência e a qualidade de vida, e as perspetivas futuras da Perfusão Isolada dos Membros. Apesar da sua inegável eficácia terapêutica, a PIM é um procedimento de custo elevado e restrita a centros especializados, o que provoca assimetrias significativas. Os dados internacionais são claros, até agora não parece ter contribuído para um aumento da esperança média de vida destes doentes, mas o seu contributo para a qualidade de vida e manutenção da funcionalidade dos doentes é enorme. A Infusão Isolada dos Membros aparece como uma técnica standard promissora na quimioterapia regional.

Familial amyloid polyneuropathy (FAP): clinical features, pathophysiology and treatment

In the literature, concepts of “polyneuropathy”, “peripheral neuropathy” and “neuropathy” are often mistakenly used as synonyms. Polyneuropathy is a specific term that refers to a relatively homogenous process that affects multiple peripheral nerves. Most of these tend to present as symmetric polyneuropathies that first manifest in the distal portions of the affected nerves. Many of these distal symmetric polyneuropathies are due to toxic-metabolic causes such as alcohol abuse and diabetes mellitus. Other distal symmetric polyneuropathies may result from an overproduction of substances that result in nerve pathology such as is observed in anti-MAG neuropathy and monoclonal gammopathy of undetermined significance. Other “overproduction” disorders are hereditary such as noted in the Portuguese type of familial amyloid polyneuropathy (FAP). FAP is a manifestation of a group of hereditary amyloidoses; an autosomal dominant, multisystemic disorder wherein the mutant amyloid precursor, transthyretin, is produced in excess primarily by the liver. The liver accounts for approximately 98% of all transthyretin production. FAP is confirmed by detecting a transthyretin variant with a methionine for valine substitution at position 30 [TTR (Met30)]. Familial Amyloidotic Polyneuropathy (FAP) – Portuguese type was first described by a Portuguese neurologist, Corino de Andrade in 1939 and published in 1951. Most persons with this disorder are descended from Portuguese sailors who sired offspring in various locations, primarily in Sweden, Japan and Mallorca. Their descendants emigrated worldwide such that this disorder has been reported in other countries as well. More than 2000 symptomatic cases have been reported in Portugal. FAP progresses rapidly with an average time course from symptom onset to multi-organ involvement and death between ten and twenty years. Treatments directed at removing this aberrant protein such as plasmapheresis and immunoadsorption proved to be unsuccessful. Liver transplantation has been the only effective solution as evidenced by almost 2000 liver transplants performed worldwide. A therapy for FAP with a novel agent, “Tafamidis” has shown some promise in ongoing phase III clinical trials. It is well recognized that regular physical activity of moderate intensity has a positive effect on physical fitness as gauged by body composition, aerobic capacity, muscular strength and endurance and flexibility. Physical fitness has been reported to result in the reduction of symptoms and lesser impairment when performing activities of daily living. Exercise has been advocated as part of a comprehensive approach to the treatment of chronic diseases. Therefore, this chapter concludes with a discussion of the role of exercise training on FAP.

PET/CT com 18-fluor-fluorodeoxiglucose no seguimento do melanoma maligno cutâneo

Introdução – O melanoma maligno cutâneo (MMC) é considerado uma das mais letais neoplasias e no seu seguimento recorre-se, para além dos exames clínicos e da análise de marcadores tumorais, a diversos métodos imagiológicos, como é o exame Tomografia por Emissão de Positrões/Tomografia Computorizada (PET/CT, do acrónimo inglês Positron Emission Tomography/Computed Tomography) com 18fluor-fluorodeoxiglucose (18F-FDG). O presente estudo tem como objetivo avaliar a utilidade da PET/CT relativamente à análise da extensão e à suspeita de recidiva do MMC, comparando os achados imagiológicos com os descritos em estudos CT. Metodologia – Estudo retrospetivo de 62 estudos PET/CT realizados em 50 pacientes diagnosticados com MMC. Excluiu-se um estudo cujo resultado era duvidoso (nódulo pulmonar). As informações relativas aos resultados dos estudos anatomopatológicos e dos exames imagiológicos foram obtidas através da história clínica e dos relatórios médicos dos estudos CT e PET/CT. Foi criada uma base de dados com os dados recolhidos através do software Excel e foi efetuada uma análise estatística descritiva. Resultados – Dos estudos PET/CT analisados, 31 foram considerados verdadeiros positivos (VP), 28 verdadeiros negativos (VN), um falso positivo (FP) e um falso negativo (FN). A sensibilidade, especificidade, o valor preditivo positivo (VPP), o valor preditivo negativo (VPN) e a exatidão da PET/CT para o estadiamento e avaliação de suspeita de recidiva no MMC são, respetivamente, 96,9%, 96,6%, 96,9%, 96,6% e 96,7%. Dos resultados da CT considerados na análise estatística, 14 corresponderam a VP, 12 a VN, três a FP e cinco a FN. A sensibilidade, especificidade, o VPP e o VPN e a exatidão da CT para o estadiamento e avaliação de suspeita de recidiva no MMC são, respetivamente, 73,7%, 80,0%, 82,4%, 70,6% e 76,5%. Comparativamente aos resultados CT, a PET/CT permitiu uma mudança na atitude terapêutica em 23% dos estudos. Conclusão – A PET/CT é um exame útil na avaliação do MMC, caracterizando-se por uma maior acuidade diagnóstica no estadiamento e na avaliação de suspeita de recidiva do MMC comparativamente à CT isoladamente.

Pólipos Gástricos Como Rara Forma de Apresentação de Metástase Gástrica de Melanoma

Doente de 74 anos, sexo masculino, com dor epigástrica, associada a náuseas, anorexia e emagrecimento marcado (20 kg num mês). Antecedentes pessoais de melanoma excisado. No exame objectivo constatou-se: adenopatia supraclavicular esquerda, aderente aos planos profundos, de consistência pétrea, não dolorosa. No decurso da investigação realizou tomografia computorizada toraco-abdomino-pélvica que demonstrou: extensa disseminação hepática com pequenas imagens hipodensas e hipocaptantes. Realizou endoscopia digestiva alta que revelou: hérnia hiatal e nesta, pólipo gástrico, séssil, com 5 mm; mucosa do corpo e antro com múltiplos pólipos sésseis com dimensões entre 2 e 4 mm, erosionados que se biopsaram. O exame anatomopatológico das biópsias gástricas revelou: fragmentos de mucosa gástrica com infiltração compatível com metástase de melanoma.

Intraepidermal Epidermotropic Metastatic Melanoma: a Clinical and Histopathological Mimicker of Melanoma in Situ Occurring in Multiplicity

The distinction between primary melanoma and melanoma metastatic to the skin has major prognostic implications. We report a case of a 67-year-old male with a diagnosis of a superficial spreading melanoma (stage IB) rendered 6 years earlier who presented clinically with an atypical nevus on his left thigh. Histopathological examination showed an intraepidermal melanocytic proliferation that was interpreted as melanoma in situ. Subsequently, 45 additional pigmented macules appeared in crops over a 9-month period. Clinically and dermoscopically, these lesions were extremely polymorphic. Histopathological findings were compatible with melanoma in situ, as each lesion consisted of a wholly intraepidermal proliferation of markedly atypical melanocytes arranged singly and in nests. A complete gastrointestinal study showed multiple pigmented metastatic lesions throughout the stomach and small bowel, which supported a diagnosis of metastatic melanoma with gastrointestinal and epidermotropic skin involvement. Monosomy of chromosome 9 and a BRAF V600E mutation were detected in the primary tumor sample and in macro-dissected secondary lesions. No CDKN2A or CDK4 germline mutations were found. Intraepidermal epidermotropic metastases of melanoma have been rarely described in literature. In this case, histopathology alone was insufficient to distinguish metastatic melanoma from multiple in situ melanomas. The recognition of epidermotropic metastases should be based on the correlation between clinical, dermoscopic, histopathological and molecular findings.

Orthotopic Liver Transplantation in Familial Amyloidotic Polyneuropathy Is Associated with Long-Term Progression of Renal Disease

Orthotopic liver transplantation has become the treatment of choice for familial amyloidotic polyneuropathy. The aims of this study were to evaluate the renal complications post orthotopic liver transplantation in familial amyloidotic polyneuropathy and their impact. We retrospectively studied 185 recipients who underwent 217 orthotopic liver transplants. Mean age 36.8±9.5 years, 59% males, 14.3% with renal dysfunction pre orthotopic liver transplantation. Mean follow-up 3.6±3.7 years. Thirty-two patients died. Univariate and multivariate analysis were performed, and p<0.05 was considered significant. Acute kidney injury occurred in 57 patients and renal replacement therapy was needed in 16/57. In multivariate analysis, acute kidney injury was correlated with development of chronic kidney disease (p<0.001). Relating to development of chronic kidney disease, 23.5% had progress to stage 3, 6% to stage 4 and 5.1% to stage 5d. According to Spearmen correlation, risk factors for chronic kidney disease development were age (p<0.001), renal dysfunction pre orthotopic liver transplantation (p<0.001) and acute kidney injury post orthotopic liver transplantation (p<0.001). Mortality was correlated with age (p<0.001), retransplantation need (p=0.004), renal dysfunction pre orthotopic liver transplantation (p<0.001), acute kidney injury post orthotopic liver transplantation (p=0.04), and chronic kidney disease stage 5 (p<0.001). Using binary regression, mortality was correlated with chronic kidney disease development (p=0.02). In conclusion, familial amyloidotic polyneuropathy patients are disposed to renal complications that have a negative impact on the survival of these patients.

Attachment, Physiological and Familial Vulnerability in Childhood Obesity: an Interactive Multisystem Approach

The aims of the present study were to test the association between insecure attachment and basal cortisol and catecholamines levels in a sample of obese children. The role of familial vulnerability and gender was also investigated. Methods: Cortisol and catecholamines levels of 8- to 13-year olds obese children were measured. Self-report questionnaires were used to assess attachment pattern and current anxiety and depression, and parent-report questionnaires were used to assess attachment, current anxiety and depression and familial vulnerability. Linear regression analyses were performed for individuals that scored low versus high on parental internalizing problems, and for boys and girls, separately. Results: In the group with high parental internalizing problems, insecure attachment was significantly associated with reduced basal levels of cortisol, in boys (p=0.007, b= -0.861, R2= 73.0%). In the group with low parental internalizing problems, the association between insecure attachment and cortisol was not significant in either boys or girls, and it was negative in boys (p=0.075, b= -0.606, R2= 36.7%) and positive in girls (p=0.677, b= 0.176, R2= 3.1%) . Conclusions: Apparently, physiological risk factors for psicopathology in obesity are more evident in individuals with a high familial vulnerability. In addition, patterns of physiological risk for psicopathology in obesity are different in boys and girls. Therefore, it is important to take into account familial vulnerability and gender when investigating physiological risk factors for psycopathology in obesity. Insecure attachment in childhood may be a risk factor for obesity. Interventions to increase children's attachment security should examine the effects on children's weight.

The Risks of Self - Medication: Case Report of Familial Misuse of AM3 (Immunoferon®)

Over the last decades extended medical knowledge has been an important health care benefit in terms of disease prevention and management. However, probably with no exception, most pharmaceutical products are not devoid of adverse consequences. Immunomodulators are commonly considered a “benign” drug whose advantages bypass consequences. The immunomodulator AM3 (Immunoferon®) is a clinically used, orally administered compound whose active principle is stabilised in an inorganic matrix of calcium. We report the misuse of AM3 in three members of a family; father and two children. The drug was prescribed to the father who subsequently administered it to the children without seeking medical advice. Two months later, all subjects developed abdominal and/or flank colicky pain. Hypercalciuria was diagnosed in the children with different degrees of severity. It is likely that the calcium content of the inorganic matrix played an important role in the onset of symptoms. No adverse side effects related to the inorganic matrix of calcium of immunoferon® have been documented so far. This family case report calls attention to the risks of self -medication in a susceptible family. Paediatric patients are vulnerable as they rely on adults for the supply of medications. Concerning the use of drugs in family, especially nonprescription drugs, the quality of health care provided to the children depends on the health literacy of their parents.

Long-Term Evaluation of Recurrence after Photodynamic Therapy with Topical Methyl Aminolevulinate for Non-Melanoma Skin Cancer: a Hospital-Based Study

Introduction & Objectives: Several factors may influence the decision to pursue nonsurgical modalities for the treatment of non-melanoma skin cancer. Topical photodynamic therapy (PDT) is a non-invasive alternative treatment reported to have a high efficacy when using standardized protocols in Bowen’s disease (BD), superficial basal cell carcinoma (BCC) and in thin nodular BCC. However, long-term recurrence studies are lacking. The aim of this study was to evaluate the long-term efficacy of PDT with topical methylaminolevulinate (MAL) for the treatment of BD and BCC in a dermato-oncology department. Materials & Methods: All patients with the diagnosis of BD or BCC, treated with MAL-PDT from the years 2004 to 2008, were enrolled. Treatment protocol included two MAL-PDT sessions one week apart repeated at three months when incomplete response, using a red light dose of 37-40 J/cm2 and an exposure time of 8’20’’. Clinical records were retrospectively reviewed, and data regarding age, sex, tumour location, size, treatment outcomes and recurrence were registered. Descriptive analysis was performed using chi square tests, followed by survival analysis with the Kaplan-Meier and Cox regression models. Results: Sixty-eight patients (median age 71.0 years, P25;P75=30;92) with a total of 78 tumours (31 BD, 45 superficial BCC, 2 nodular BCC) and a median tumour size of 5 cm2 were treated. Overall, the median follow-up period was 43.5 months (P25;P75=0;100), and a total recurrence rate of 33.8% was observed (24.4 % for BCC vs. 45.2% for BD). Estimated recurrence rates for BCC and BD were 5.0% vs. 7.4% at 6 months, 23.4% vs. 27.9% at 12 months, and 30.0% vs. 72.4% at 60 months. Both age and diagnosis were independent prognostic factors for recurrence, with significantly higher estimated recurrence rates in patients with BD (p=0.0036) or younger than 58 years old (p=0.039). The risk of recurrence (hazard ratio) was 2.4 times higher in patients with BD compared to superficial BCC (95% CI:1.1-5.3; p=0.033), and 2.8 times higher in patients younger than 58 years old (95% CI:1.2-6.5; p=0.02). Conclusions: In the studied population, estimated recurrence rates are higher than those expected from available literature, possibly due to a longer follow-up period. To the authors’ knowledge there is only one other study with a similar follow-up period, regarding BCC solely. BD, as an in situ squamous cell carcinoma, has a higher tendency to recur than superficial BCC. Despite greater cosmesis, PDT might no be the best treatment option for young patients considering their higher risk of recurrence.