Myelopathy in sickle cell disease: a case-oriented review.

INTRODUCTION: Although neurological complications are well recognized in patients with sickle cell disease, myelopathy has been rarely described, with few reported cases of compressive and ischemic myelopathy. We present the first case report of longitudinally extensive myelitis (LETM) in SCD and review the differential diagnosis of myelopathy in these patients. CASE PRESENTATION: We report the case of a 29-year-old African-Brazilian man with SCD, who experienced a subacute flaccid paraparesis, with T2 sensory level and urinary retention. Cerebrospinal fluid analysis showed a lymphocytic pleocytosis and increased protein levels. MRI disclosed a longitudinally extensive spinal cord lesion, with a high T2/STIR signal extending from C2 to T12. We searched Medline/PubMed, Embase, Scopus, and Google Scholar databases for myelopathy in SCD patients. DISCUSSION: Spinal cord compression by vertebral fractures, extramedullary hematopoietic tissue, and Salmonella epidural abscess have been reported in SCD. We found only three case reports of spinal cord infarction, which is unexpectedly infrequent compared to the prevalence of cerebral infarction in SCD. We found only one case report of varicella-zoster myelitis and no previous report of LETM in SCD patients. Specific and time-sensitive causes of myelopathy should be considered in SCD patients. In addition to compression and ischemia, LETM is a possible mechanism of spinal cord involvement in SCD patients.

Polymorphisms in Inflammatory Genes Modulate Clinical Complications in Patients With Sickle Cell Disease.

Sickle cell disease (SCD), the most common monogenic disease worldwide, is marked by a phenotypic variability that is, to date, only partially understood. Because inflammation plays a major role in SCD pathophysiology, we hypothesized that single nucleotide polymorphisms (SNP) in genes encoding functionally important inflammatory proteins might modulate the occurrence of SCD complications. We assessed the association between 20 SNPs in genes encoding Toll-like receptors (TLR), NK cell receptors (NKG), histocompatibility leukocyte antigens (HLA), major histocompatibility complex class I polypeptide-related sequence A (MICA) and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4), and the occurrence of six SCD clinical complications (stroke, acute chest syndrome (ACS), leg ulcers, cholelithiasis, osteonecrosis, or retinopathy). This study was performed in a cohort of 500 patients. We found that the TLR2 rs4696480 TA, TLR2 rs3804099 CC, and HLA-G, rs9380142 AA genotypes were more frequent in patients who had fewer complications. Also, in logistic regression, the HLA-G rs9380142 G allele increased the risk of cholelithiasis (AG vs. AA, OR 1.57, 95%CI 1.16-2.15; GG vs. AA, OR 2.47, 95%CI 1.34-4.64; P = 0.02). For SNPs located in the NKG2D loci, in logistic regression, the A allele in three SNPs was associated with a lower frequency of retinopathy, namely, rs2246809 (AA vs. GG: OR 0.22, 95%CI 0.09-0.50; AG vs. GG: OR 0.47, 95%CI 0.31-0.71; P = 0.004, for patients of same origin), rs2617160 (AT vs. TT: OR 0.67, 95%CI 0.48-0.92; AA vs. TT: OR 0.45, 95%CI 0.23-0.84; P = 0.04), and rs2617169 (AA vs. TT: OR 0.33, 95%CI 0.13-0.82; AT vs. TT: OR 0.58, 95%CI 0.36-0.91, P = 0.049, in patients of same SCD genotype). These results, by uncovering susceptibility to, or protection against SCD complications, might contribute to a better understanding of the inflammatory pathways involved in SCD manifestations and to pave the way for the discovery of biomarkers that predict disease severity, which would improve SCD management.

Cisticercose em lingua: relato de caso / Cysticercosis in stock: case report

A cisticercose é uma doença causada pelo parasitismo da larva da tênia do porco (Cysticercus cellulosae) em tecidos humanos. É mais comum no cérebro, tecido subcutâneo, músculos e olhos. Relatamos aqui uma apresentação rara desta doença na margem lateral esquerda da língua, com diagnóstico histopatológico em biópsia excisional e retirada total da lesão. A criança acometida tinha 7 anos de idade, era do sexo masculino e previamente hígida...

Tongue cysticercosis: Case reportCysticercosis is a parasitic disease caused by the larva (Cysticercus cellulosae) of pork tapeworm. Itis more common in the brain, subcutaneous tissue, muscles and eyes. We report a rare presentation ofthis disease, affecting a previously healthy 7-year-old boy...

Desigualdades associadas à não realização de mamografia na zona urbana de Teresina-Piauí-Brasil, 2010-2011 / Inequalities associated with lack of mammography in Teresina-Piauí-Brazil, 2010-2011

FUNDAMENTOS: Estudos demonstram que a realização da mamografia em programas de rastreamento diminui a mortalidade do câncer de mama. Entretanto, há indícios de grande desigualdade no acesso à realização deste exame no Brasil. OBJETIVOS: Analisar o percentual de mulheres que não realizaram mamografia segundo variáveis socioeconômicas e demográficas em mulheres de 40 a 69 anos de Teresina-PI. MÉTODOS: Estudo transversal realizado em 2010/2011, com amostragem multifásica em cinco etapas, realizando-se entrevista face a face com cada mulher sorteada. Processou-se os dados com programa SPSS 19.0, realizando-se análise uni e multivariada. RESULTADOS: Dentre as 433 mulheres que responderam o questionário, a taxa de realização de mamografia foi de 75,3%,sendo que, destas, 17,2% a haviam realizado há mais de dois anos. A não realização de mamografia esteve relacionada com raça parda/negra (p = 0,030), ausência de companheiro (p = 0,041), menor grau de instrução (p = 0,010), menor renda (p < 0,001), tabagismo (p = 0,006), não possuir plano ou seguro de saúde (p < 0,001). O Sistema Único de Saúde (SUS) foi responsável por realizar 56,3% das mamografias. CONCLUSÃO: 24,7% das mulheres teresinenses nunca haviam realizado mamografia e 37,9% não a haviam realizado nos últimos dois anos. A não realização deste exame foi associada à existência de desigualdades racial e socioeconômica.

INTRODUCTION: Randomized studies have shown that screening for breast cancer with mammography reduces the breast cancer mortality. However there are signs of a great inequality in access to mammography in Brazil. OBJECTIVES: To analyze the percentage of women who did not undergo mammography according to socioeconomic and demographic variables in women aged from 40 to 69 years in Teresina, Piauí State, Brazil. METHODS: This was a cross-sectional population-based study in women aged 40-69 years in Teresina-Piauí in 2010/2011. The sampling was randomly conducted in five stages. The data were processed by SPSS 19.0 and it was performed univariate and multivariate analysis. RESULTS: Among 433 women who answered the questionnaire, 75,3% had a mammography and 17,2% of these women had not a mammography over the last two years. The lack of breast cancer screening was associated with non-white skin color (p = 0,030), never being married (p = 0,041), low levels of education (p = 0,010), low family income (p < 0,001), smoking (p = 0,006), having no private health insurance (p < 0,001). The Unified National Health System (SUS) performed 56,3% of reported mammograms. CONCLUSION: About 24.7% of women in the sample never had a mammography. According to the findings, the lack of breast cancer screening is associated with social and racial inequalities.

Inequalities associated with lack of mammography in Teresina-Piauí-Brazil, 2010-2011.

INTRODUCTION: Randomized studies have shown that screening for breast cancer with mammography reduces the breast cancer mortality. However there are signs of a great inequality in access to mammography in Brazil. OBJECTIVES: To analyze the percentage of women who did not undergo mammography according to socioeconomic and demographic variables in women aged from 40 to 69 years in Teresina, Piauí State, Brazil. METHODS: This was a cross-sectional population-based study in women aged 40-69 years in Teresina-Piauí in 2010/2011. The sampling was randomly conducted in five stages. The data were processed by SPSS 19.0 and it was performed univariate and multivariate analysis. RESULTS: Among 433 women who answered the questionnaire, 75,3% had a mammography and 17,2% of these women had not a mammography over the last two years. The lack of breast cancer screening was associated with non-white skin color (p = 0,030), never being married (p = 0,041), low levels of education (p = 0,010), low family income (p < 0,001), smoking (p = 0,006), having no private health insurance (p < 0,001). The Unified National Health System (SUS) performed 56,3% of reported mammograms. CONCLUSION: About 24.7% of women in the sample never had a mammography. According to the findings, the lack of breast cancer screening is associated with social and racial inequalities.