A Rare Case of Primary Gastric Signet Ring Cell Carcinoma: a Review of Guidelines for the Management of Gastric Cancer.

Gastric carcinoma is the fifth most common and the third leading cause of cancer deaths worldwide. The incidence of diffuse-type gastric cancer, of which signet ring cell carcinoma is a subtype, is rising in the world. Due to non-specific gastritis-like symptoms, difficulty in assessing true tumor characteristics owing to its horizontal spread, and non-distinguishable endoscopic appearance from other gastric pathologies, the diagnosis of this subtype is challenging. We present a case of a 67-year-old woman with progressively worsening abdominal pain who came for an endoscopic ultrasound evaluation of an incidentally noted pancreatic cyst on a previous MRI. During endoscopy, a 1-cm gastric ulcer was noted along the lesser curvature of the gastric body. Biopsy confirmed a diagnosis of gastric signet ring cell carcinoma (SRCC) with CDX-2 and keratin positivity. The patient underwent total gastrectomy with Roux-en-Y reconstruction. Gross specimen revealed a diffuse SRCC invading the muscularis propria, along with lymphovascular and perineural invasion. In the context of our case, we discuss the morphological features of SRCC and the effectiveness of treatment options based on existing literature. Early accurate diagnosis and staging play an important role in determining treatment options as well as the clinical course of gastric SRCC.

GC-MS analysis of organic fractions of Chrozophora tinctoria (L.) A.Juss. and their prokinetic propensity in animal models / Análise por GC-MS de frações orgânicas de Chrozophora tinctoria (L.) A.Juss. e sua propensão procinética em modelos animais

Chrozophora tinctoria (L.) A.Juss. is herbaceous, monecious annual plant used traditionally to cure gastrointestinal disorders. The present study was carried out to find the bioactive compounds by Gas Chromatography-Mass Spectrometry, the acetylcholinesterase inhibitory potential acute toxicity, and emetic activity present in the ethyl acetate fraction of Chrozophora tinctoria (EAFCT) and dichloromethane fraction of Chrozophora tinctoria (DCMFCT). The compounds detected in both fractions were mostly fatty acids, with about seven compounds in EAFCT and 10 in DCMFCT. These included pharmacologically active compounds such as imipramine, used to treat depression, or hexadecanoic acid methyl ester, an antioxidant, nematicide, pesticide, hypocholesterolemic, 9,12,15-Octadecatrienoic acid, ethyl ester, (Z,Z,Z)- is used as a cancer preventive, antiarthritic, antihistaminic, hepatoprotective, insectifuge, nematicide, Pentadecanoic acid, 14-methyl-, methyl ester have antifungal, antimicrobial and antioxidant activities, 10-Octadecanoic acid, methyl ester have the property to decrease blood cholesterol, Antioxidant and antimicrobial, 1-Eicosanol is used as an antibacterial, 1-Hexadecene has antibacterial, antioxidant, and antifungal activities. Both DCMFCT and EAFCT fractions inhibited acetylcholinesterase (AChE) activity with IC50 values of 10 µg and 130 µg, respectively. Both the fractions were found to be toxic in a dose-dependent manner, inducing emesis at 0.5g onward and lethargy and mortality from 3­5 g upwards. Both the fractions combined with distilled water showed highly emetic activity. The significant increase in the number of vomits was shown by EAFCT plus distilled water which are 7.50±1.29, 7.25±3.10, and 11.75±2.22 number of vomits at 1g, 2g, and 3g/kg concentration respectively, while DCMFCT plus distilled water showed 5.25±2.22, 7.50±2.52 and 10.25±2.22 number of vomits at 1g, 2, and 3g/kg correspondingly. The antiemetic standard drug metoclopramide has a higher impact against the emesis induced by both the fractions than dimenhydrinate. Metoclopramide decreases the number of vomits caused by EAFCT to 1.00±0.00, 2.00±0.00, 4.00±1.00 at 1g, 2, and 3g/kg sequentially, while dimenhydrinate decreases the number of vomits to 1.33±0.58, 2.33±1.15, 4.33±0.58 at 1g, 2, and 3g respectively. In the same way, Metochloprimide decreases the number of emesis caused by DcmCt from 5.25±2.22, 7.50±2.52, 10.25±2.22 to 1.33±0.58, 2.33±1.1, 4.33±0.58 at 1g, 2, and 3g/kg concentrations. The present study is the first documented report that scientifically validates the folkloric use of Chrozophora tinctoria as an emetic agent.

Chrozophora tinctoria (L.) A.Juss. é uma planta anual herbácea, monoica, usada tradicionalmente para curar distúrbios gastrointestinais. O presente estudo foi realizado para encontrar os compostos bioativos por Cromatografia Gasosa-Espectrometria de Massa (GC-MS), a toxicidade aguda do potencial inibitório da acetilcolinesterase e a atividade emética presente na fração acetato de etila de Chrozophora tinctoria (EAFCT) e fração diclorometano de Chrozophora tinctoria (DCMFCT). Os compostos detectados em ambas as frações foram principalmente ácidos graxos, com cerca de sete compostos em EAFCT e 10 em DCMFCT. Estes incluíam compostos farmacologicamente ativos, como a imipramina, usada para tratar a depressão, ou éster metílico do ácido hexadecanoico, um antioxidante, nematicida, pesticida, hipocolesterolêmico, ácido 9,12,15-octadecatrienoico, éster etílico, (Z,Z,Z)- é usado como preventivo do câncer, antiartrítico, anti-histamínico, hepatoprotetor, inseticida, nematicida, ácido pentadecanoico, 14-metil-, éster metílico tem atividades antifúngicas, antimicrobianas e antioxidantes, ácido 10-octadecanoico, éster metílico tem a propriedade de diminuir o colesterol no sangue, antioxidante e antimicrobiano, o 1-Eicosanol é usado como antibacteriano, o 1-Hexadeceno possui atividades antibacteriana, antioxidante e antifúngica. Ambas as frações DCMFCT e EAFCT inibiram a atividade da acetilcolinesterase (AChE) com valores de IC50 de 10µg e 130µg, respectivamente. Ambas as frações foram consideradas tóxicas de maneira dose-dependente, induzindo vômitos a partir de 0,5g e letargia e mortalidade de 3g a 5g para cima. Ambas as frações combinadas com água destilada apresentaram atividade altamente emética. O aumento significativo no número de vômitos foi demonstrado pelo EAFCT mais água destilada, que são 7,50±1,29, 7,25±3,10 e 11,75±2,22 número de vômitos nas concentrações de 1g, 2g e 3g/kg, respectivamente, enquanto DCMFCT mais água destilada mostrou 5,25±2,22, 7,50±2,52 e 10,25±2,22 número de vômitos em 1g, 2g e 3g/kg, respectivamente. A droga padrão antiemética metoclopramida tem um impacto maior contra a emese induzida por ambas as frações do que o dimenidrinato. A metoclopramida diminui o número de vômitos causados ​​por EAFCT para 1,00±0,00, 2,00±0,00, 4,00±1,00 a 1g, 2g e 3g/kg, sequencialmente, enquanto o dimenidrinato diminui o número de vômitos para 1,33±0,58, 2,33±1,15, 4,33± 0,58 a 1g, 2g e 3g, respectivamente. Da mesma forma, a metocloprimida diminui o número de vômitos causados ​​por DcmCt de 5,25±2,22, 7,50±2,52, 10,25±2,22 para 1,33±0,58, 2,33±1,1, 4,33±0,58 nas concentrações de 1g, 2g e 3g/kg. O presente estudo é o primeiro relato documentado que valida cientificamente o uso folclórico de Chrozophora tinctoria como agente emético.

Cytotoxic, phytotoxic and insecticidal potential of Achillea millefolium L. and Chaerophyllum villosum wall. ex dc / Potencial citotóxico, fitotóxico e insecticida de Achillea millefolium L. e Chaerophyllum villosum

The methanolic, chloroformic and aqueous extract of Achillea millefolium and Chaerophyllum villosum were investigated for cytotoxicity, phytotoxic and insecticidal activities. Cytotoxicity was investigated by brine shrimp lethality assay indicating that the crude methanolic extract of A.millefolium and chloroformic extract of C.villosum revealed highest mortality of brine shrimps with (LD50 of 52.60 µg/ml) and (14.81 µg/ml). Phytotoxicity was evaluated using the Lemna minor bioassay which revealed that the crude methanolic extract of A.millefolium and C.villosum extract has maximum inhibition of Lemna minor with (Fl50 6.60 µg/ml) and (0.67 µg/ml).The insecticidal activity showed that among all the insects studied it was observed that methanolic extract of A. millefoliumand C. villosum was highly toxic to Sphenoptera dadkhani with (LD50=4.17 µg/ml) and (0.34 µg/ml). From the present study it can be concluded that different extracts from A. millefolium and C. villosum showed good cytotoxic, phytotoxic and insecticidal activity in a dose dependent manner.

Neste estudo, os extratos metanólico, clorofórmico e aquoso de Achillea millefolium e Chaerophyllum villosum foram analisados em relação à citotoxicidade, atividade fitotóxica e inseticida. A citotoxicidade foi analisada através do ensaio de letalidade de artémia, indicando que o extrato metanólico bruto de A. millefolium e o extrato clorofórmico de C. villosum revelaram maior mortalidade de artêmias com DL50 de 52,60 µg/ml e 14,81 µg/ml. A fitotoxicidade foi avaliada utilizando o bioensaio de Lemna minor que revelou que o extrato metanólico bruto de A. millefolium e extrato de C. villosum têm inibição máxima de Lemna minor com Fl50 6,60 µg/ml e 0,67 µg/ml. A atividade inseticida mostrou que dentre todos os insetos estudados, o extrato metanólico de A. millefolium e de C. villosum foi altamente tóxico para Sphenoptera dadkhanicom DL50 = 4,17 µg/ml e 0,34 µg/ml . Por outro lado, diferentes extratos, como A. millefolium e C. villosum apresentaram boa atividade citotóxica, fitotóxica e inseticida de forma dose-dependente.

Cytotoxic, Phytotoxic and Insecticidal Potential of Achillea millefolium L. and Chaerophyllum villosum wall. ex dc.

The methanolic, chloroformic and aqueous extract of Achillea millefolium and Chaerophyllum villosum were investigated for cytotoxicity, phytotoxic and insecticidal activities. Cytotoxicity was investigated by brine shrimp lethality assay indicating that the crude methanolic extract of A.millefolium and chloroformic extract of C.villosum revealed highest mortality of brine shrimps with (LD50 of 52.60 µg/ml) and (14.81 µg/ml). Phytotoxicity was evaluated using the Lemna minor bioassay which revealed that the crude methanolic extract of A.millefolium and C.villosum extract has maximum inhibition of Lemna minor with (Fl50 6.60 µg/ml) and (0.67 µg/ml).The insecticidal activity showed that among all the insects studied it was observed that methanolic extract of A. millefoliumand C. villosum was highly toxic to Sphenoptera dadkhani with (LD50=4.17 µg/ml) and (0.34 µg/ml). From the present study it can be concluded that different extracts from A. millefolium and C. villosum showed good cytotoxic, phytotoxic and insecticidal activity in a dose dependent manner.

The Siberian pine growth dynamics in Altai Mountains, China / A dinâmica de crescimento do pinheiro siberiano nas montanhas de Altai, China

Climatic factors play an essential role in the growth of tree ring width. In this study, we aimed to evaluate the correlation between climatic variables and tree-ring growth characteristics of Pinus sibirica in Altai mountains, northwestern China. This study being is first of its kind on climate growth analysis of Pinus sibirica in northwestern China. The study showed great potential to understand the species growing under the specific climatic conditions. Total of 70 tree cores collected from three sites in the sampling area, out of which 63 tree cores considered for this study. The effect of climatic variables which was studied include precipitation, temperature and PDSI. Our results showed that Tree Ring Width chronology has a significantly positive correlation with the late winter (March) temperature and significant negative correlation with the July temperatures. A significant correlation was observed with the late summer precipitation whereas no significant relation found with the Palmer Drought Severity Index. These significant correlations with temperature and precipitation suggested that this tree species had the potential for the reconstruction of the past climate in the area.(AU)

Fatores climáticos desempenham papel essencial no crescimento da largura dos anéis das árvores. Neste estudo, objetivou-se avaliar a correlação entre variáveis climáticas e características de crescimento de anéis de árvores de Pinus sibirica nas montanhas de Altai, noroeste da China. Este estudo é o primeiro desse tipo na análise do crescimento climático de Pinus sibirica no noroeste da China. O estudo mostrou grande potencial para entender as espécies que crescem sob as condições climáticas específicas. Total de 70 testemunhos coletados em três locais na área de amostragem, dos quais 63 testemunhos considerados para este estudo. O efeito das variáveis climáticas estudadas incluem precipitação, temperatura e PDSI. Nossos resultados mostraram que a cronologia da Largura do Anel da Árvore tem uma correlação significativamente positiva com a temperatura do final do inverno (março) e uma correlação negativa significativa com as temperaturas de julho. Uma correlação significativa foi observada com a precipitação do final do verão, enquanto nenhuma relação significativa foi encontrada com o Índice de Severidade de Seca de Palmer. Essas correlações significativas com a temperatura e precipitação sugeriram que esta espécie de árvore tinha o potencial para a reconstrução do clima passado na área.(AU)

The Siberian pine growth dynamics in Altai Mountains, China / A dinâmica de crescimento do pinheiro siberiano nas montanhas de Altai, China

Climatic factors play an essential role in the growth of tree ring width. In this study, we aimed to evaluate the correlation between climatic variables and tree-ring growth characteristics of Pinus sibirica in Altai mountains, northwestern China. This study being is first of its kind on climate growth analysis of Pinus sibirica in northwestern China. The study showed great potential to understand the species growing under the specific climatic conditions. Total of 70 tree cores collected from three sites in the sampling area, out of which 63 tree cores considered for this study. The effect of climatic variables which was studied include precipitation, temperature and PDSI. Our results showed that Tree Ring Width chronology has a significantly positive correlation with the late winter (March) temperature and significant negative correlation with the July temperatures. A significant correlation was observed with the late summer precipitation whereas no significant relation found with the Palmer Drought Severity Index. These significant correlations with temperature and precipitation suggested that this tree species had the potential for the reconstruction of the past climate in the area.

Fatores climáticos desempenham papel essencial no crescimento da largura dos anéis das árvores. Neste estudo, objetivou-se avaliar a correlação entre variáveis climáticas e características de crescimento de anéis de árvores de Pinus sibirica nas montanhas de Altai, noroeste da China. Este estudo é o primeiro desse tipo na análise do crescimento climático de Pinus sibirica no noroeste da China. O estudo mostrou grande potencial para entender as espécies que crescem sob as condições climáticas específicas. Total de 70 testemunhos coletados em três locais na área de amostragem, dos quais 63 testemunhos considerados para este estudo. O efeito das variáveis climáticas estudadas incluem precipitação, temperatura e PDSI. Nossos resultados mostraram que a cronologia da Largura do Anel da Árvore tem uma correlação significativamente positiva com a temperatura do final do inverno (março) e uma correlação negativa significativa com as temperaturas de julho. Uma correlação significativa foi observada com a precipitação do final do verão, enquanto nenhuma relação significativa foi encontrada com o Índice de Severidade de Seca de Palmer. Essas correlações significativas com a temperatura e precipitação sugeriram que esta espécie de árvore tinha o potencial para a reconstrução do clima passado na área.

The Siberian pine growth dynamics in Altai Mountains, China

Abstract Climatic factors play an essential role in the growth of tree ring width. In this study, we aimed to evaluate the correlation between climatic variables and tree-ring growth characteristics of Pinus sibirica in Altai mountains, northwestern China. This study being is first of its kind on climate growth analysis of Pinus sibirica in northwestern China. The study showed great potential to understand the species growing under the specific climatic conditions. Total of 70 tree cores collected from three sites in the sampling area, out of which 63 tree cores considered for this study. The effect of climatic variables which was studied include precipitation, temperature and PDSI. Our results showed that Tree Ring Width chronology has a significantly positive correlation with the late winter (March) temperature and significant negative correlation with the July temperatures. A significant correlation was observed with the late summer precipitation whereas no significant relation found with the Palmer Drought Severity Index. These significant correlations with temperature and precipitation suggested that this tree species had the potential for the reconstruction of the past climate in the area.

RESUMO Fatores climáticos desempenham papel essencial no crescimento da largura dos anéis das árvores. Neste estudo, objetivou-se avaliar a correlação entre variáveis climáticas e características de crescimento de anéis de árvores de Pinus sibirica nas montanhas de Altai, noroeste da China. Este estudo é o primeiro desse tipo na análise do crescimento climático de Pinus sibirica no noroeste da China. O estudo mostrou grande potencial para entender as espécies que crescem sob as condições climáticas específicas. Total de 70 testemunhos coletados em três locais na área de amostragem, dos quais 63 testemunhos considerados para este estudo. O efeito das variáveis climáticas estudadas incluem precipitação, temperatura e PDSI. Nossos resultados mostraram que a cronologia da Largura do Anel da Árvore tem uma correlação significativamente positiva com a temperatura do final do inverno (março) e uma correlação negativa significativa com as temperaturas de julho. Uma correlação significativa foi observada com a precipitação do final do verão, enquanto nenhuma relação significativa foi encontrada com o Índice de Severidade de Seca de Palmer. Essas correlações significativas com a temperatura e precipitação sugeriram que esta espécie de árvore tinha o potencial para a reconstrução do clima passado na área.

GC-MS analysis of organic fractions of Chrozophora tinctoria (L.) A.Juss. and their prokinetic propensity in animal models.

Chrozophora tinctoria (L.) A.Juss. is herbaceous, monecious annual plant used traditionally to cure gastrointestinal disorders. The present study was carried out to find the bioactive compounds by Gas Chromatography-Mass Spectrometry, the acetylcholinesterase inhibitory potential acute toxicity, and emetic activity present in the ethyl acetate fraction of Chrozophora tinctoria (EAFCT) and dichloromethane fraction of Chrozophora tinctoria (DCMFCT). The compounds detected in both fractions were mostly fatty acids, with about seven compounds in EAFCT and 10 in DCMFCT. These included pharmacologically active compounds such as imipramine, used to treat depression, or hexadecanoic acid methyl ester, an antioxidant, nematicide, pesticide, hypocholesterolemic, 9,12,15-Octadecatrienoic acid, ethyl ester, (Z,Z,Z)- is used as a cancer preventive, antiarthritic, antihistaminic, hepatoprotective, insectifuge, nematicide, Pentadecanoic acid, 14-methyl-, methyl ester have antifungal, antimicrobial and antioxidant activities, 10-Octadecanoic acid, methyl ester have the property to decrease blood cholesterol, Antioxidant and antimicrobial, 1-Eicosanol is used as an antibacterial, 1-Hexadecene has antibacterial, antioxidant, and antifungal activities. Both DCMFCT and EAFCT fractions inhibited acetylcholinesterase (AChE) activity with IC50 values of 10 µg and 130 µg, respectively. Both the fractions were found to be toxic in a dose-dependent manner, inducing emesis at 0.5g onward and lethargy and mortality from 3-5 g upwards. Both the fractions combined with distilled water showed highly emetic activity. The significant increase in the number of vomits was shown by EAFCT plus distilled water which are 7.50±1.29, 7.25±3.10, and 11.75±2.22 number of vomits at 1g, 2g, and 3g/kg concentration respectively, while DCMFCT plus distilled water showed 5.25±2.22, 7.50±2.52 and 10.25±2.22 number of vomits at 1g, 2, and 3g/kg correspondingly. The antiemetic standard drug metoclopramide has a higher impact against the emesis induced by both the fractions than dimenhydrinate. Metoclopramide decreases the number of vomits caused by EAFCT to 1.00±0.00, 2.00±0.00, 4.00±1.00 at 1g, 2, and 3g/kg sequentially, while dimenhydrinate decreases the number of vomits to 1.33±0.58, 2.33±1.15, 4.33±0.58 at 1g, 2, and 3g respectively. In the same way, Metochloprimide decreases the number of emesis caused by DcmCt from 5.25±2.22, 7.50±2.52, 10.25±2.22 to 1.33±0.58, 2.33±1.1, 4.33±0.58 at 1g, 2, and 3g/kg concentrations. The present study is the first documented report that scientifically validates the folkloric use of Chrozophora tinctoria as an emetic agent.

The Siberian pine growth dynamics in Altai Mountains, China.

Climatic factors play an essential role in the growth of tree ring width. In this study, we aimed to evaluate the correlation between climatic variables and tree-ring growth characteristics of Pinus sibirica in Altai mountains, northwestern China. This study being is first of its kind on climate growth analysis of Pinus sibirica in northwestern China. The study showed great potential to understand the species growing under the specific climatic conditions. Total of 70 tree cores collected from three sites in the sampling area, out of which 63 tree cores considered for this study. The effect of climatic variables which was studied include precipitation, temperature and PDSI. Our results showed that Tree Ring Width chronology has a significantly positive correlation with the late winter (March) temperature and significant negative correlation with the July temperatures. A significant correlation was observed with the late summer precipitation whereas no significant relation found with the Palmer Drought Severity Index. These significant correlations with temperature and precipitation suggested that this tree species had the potential for the reconstruction of the past climate in the area.

Survival, causes of death, and prognostic factors in systemic sclerosis: analysis of 947 Brazilian patients.

OBJECTIVE: To analyze survival, prognostic factors, and causes of death in a large cohort of patients with systemic sclerosis (SSc). METHODS: From 1991 to 2010, 947 patients with SSc were treated at 2 referral university centers in Brazil. Causes of death were considered SSc-related and non-SSc-related. Multiple logistic regression analysis was used to identify prognostic factors. Survival at 5 and 10 years was estimated using the Kaplan-Meier method. RESULTS: One hundred sixty-eight patients died during the followup. Among the 110 deaths considered related to SSc, there was predominance of lung (48.1%) and heart (24.5%) involvement. Most of the 58 deaths not related to SSc were caused by infection, cardiovascular or cerebrovascular disease, and cancer. Male sex, modified Rodnan skin score (mRSS) > 20, osteoarticular involvement, lung involvement, and renal crisis were the main prognostic factors associated to death. Overall survival rate was 90% for 5 years and 84% for 10 years. Patients presented worse prognosis if they had diffuse SSc (85% vs 92% at 5 yrs, respectively, and 77% vs 87% at 10 yrs, compared to limited SSc), male sex (77% vs 90% at 5 yrs and 64% vs 86% at 10 yrs, compared to female sex), and mRSS > 20 (83% vs 90% at 5 yrs and 66% vs 86% at 10 yrs, compared to mRSS < 20). CONCLUSION: Survival was worse in male patients with diffuse SSc, and lung and heart involvement represented the main causes of death in this South American series of patients with SSc.

Undifferentiated spondyloarthritis: a longterm followup.

OBJECTIVE: To analyze the longterm followup of a series of Brazilian patients with undifferentiated spondyloarthritis (uSpA). METHODS: Prospective study analyzing a group of 111 patients with the diagnosis of uSpA, fulfilling the European Spondylarthropathy Study Group and the Amor criteria, who were followed for 5 to 10 years in a single university referral center. Patients had their outcome analyzed at 5, 7, and 10 years. RESULTS: There was a predominance of men (81.1%), white ethnicity (78.4%), and positive HLA-B27 (61.3%), with a mean age at onset of 27.2 years. Twenty-seven patients presented development to ankylosing spondylitis (AS; 24.3%) and 3 to psoriatic arthritis (PsA; 2.7%), while 25 patients (22.5%) went into remission during the followup. Univariate logistic regression analysis revealed that ethnicity, HLA-B27, buttock pain, inflammatory low back pain, ankle involvement, grade I sacroiliitis at the beginning of the study, and the use of sulfasalazine were statistically associated with progression to AS. Multivariate logistic regression analysis revealed that HLA-B27 (p = 0.035, OR 6.720, 95% CI 11.45-39.43) and buttock pain (p = 0.009, OR 6.211, 95% CI 1.591-24.25) were statistically associated with progression to AS. CONCLUSION: In a longterm followup of 111 Brazilian patients with uSpA, HLA-B27 and buttock pain were significant predictors of progression to a definite disease.

Frequency of HLA-B27 and its alleles in patients with Reiter syndrome: comparison with the frequency in other spondyloarthropathies and a healthy control population.

This retrospective study analyzed the HLA-B 27 alleles in a group of 20 consecutive patients with the diagnosis of Reiter syndrome (RS) followed in a tertiary referral university hospital in Brazil, during the period 1990-2006, and compared the data with that observed in other patients with spondyloarthropathies followed at the same institution. Eight cases were associated to gastrointestinal infection, eight cases to previous urethritis, and four cases presented no established preceding infection. HLA-B 27 alleles were typed by polymerase chain reaction-amplified DNA hybridized with sequence-specific oligonucleotide probes (HLA-B 2,701 to HLA-B 2,721). They were compared to a group of 108 patients with ankylosing spondylitis (AS), 40 with undifferentiated spondyloarthropathy (uSpA) and 111 healthy controls. Among the 20 patients, 17 were HLA-B 27 positive (85%). Two HLA-B 27 alleles were observed: HLA-B 2,705 (65%) and HLA-B 2,702 (35%). In the other spondyloarthropathies, the observed alleles were HLA-B 2,705 (90% in AS and 92.5% in uSpA), HLA-B 2,702 (8% in AS and 5% in uSpA), HLA-B 2,704 (1% in AS and 2.5% in uSpA) and HLA-B 2,713 (1% in AS). Among the 111 healthy controls, 80% presented HLA-B 2,705, followed by HLA-B 2,702 in 10%, HLA-B 2,703 in 6%, HLA-B 2,707 in 3% and HLA-B 2,713 in 1%. Concluding, in the HLA-B 27 positive patients with RS in this study there was predominance of HLA-B 2,705 allele, in a lower frequency than that observed in patients with other spondyloarthropathies and healthy controls.

Pulmonary involvement in ankylosing spondylitis.

This is a prospective study analyzing 52 asymptomatic, consecutive patients with ankylosing spondylitis (AS), who submitted to a pulmonary investigation that included plain chest radiography, pulmonary function test (PFT), and thoracic high-resolution computed tomography (HRCT). The results were compared according to sex, race, dorsal spine involvement, thoracic diameter, smoking status, and HLA-B27. There were four patients (8%) with an altered plain chest radiograph. PFT presented a restrictive pattern in 52% of the patients. Thoracic HRCT showed abnormalities in 21 patients (40%), predominantly nonspecific linear parenchymal opacities (19%), lymphadenopathy (12%), emphysema (10%), bronchiectasis (8%), and pleural involvement (8%). Linear parenchymal opacities were associated with a smoking history (p=0.026) and dorsal spine involvement (p=0.032). HLA-B27 was not associated with any abnormality. A lower thoracic diameter was observed in patients with dorsal spine involvement (p=0.0001), restrictive pattern at PFT (p=0.023), and linear parenchymal opacities (p=0.015). The study concluded that nonspecific subclinical pulmonary involvement is frequent in AS.

Characterization and outcome of uveitis in 350 patients with spondyloarthropathies.

This retrospective study analyzed 350 patients with the diagnosis of spondyloarthropathies (SPA) (207 with ankylosing spondylitis (AS), 80 with undifferentiated spondyloarthropathies (USPA) and 63 with psoriatic arthritis (PsA)) attended at a tertiary referral hospital for a minimum period of 5 years. All the patients presented complete clinical (axial and peripheral involvement, heel enthesopathies, extra-articular manifestations) and radiologic (sacroiliac, lumbar, dorsal and cervical spine) evaluation. HLA-B27 and respective alleles were searched. These data were compared with the occurrence of uveitis during the follow-up of the SPA patients. Thirty AS patients (14.5%) presented 55 episodes of acute anterior uveitis; there was statistical association between uveitis and juvenile-onset AS (P = 0.0094) and achillean (P = 0.0003) and plantar (P = 0.0067) enthesopathies; one AS patient presented a single episode of posterior uveitis, associated to tuberculosis. Seven USPA patients (8.8%) presented 13 episodes of acute anterior uveitis; it was not observed statistical association with any variable; one patient presented a single episode of posterior uveitis, associated to toxoplasmosis. Five HLA-B27 positive PsA patients (8%) presented 13 episodes of acute anterior uveitis. All the 26 positive HLA-B27 SPA patients with anterior uveitis tested for the HLA-B27 alleles were HLA-B*2705. No patient presented ophthalmologic severe sequelae of the anterior uveitis. Concluding, anterior uveitis was associated to the juvenile onset of the disease and to the enthesophatic involvement of the lower limbs in AS patients. The HLA-B*2705 allele was predominant in the anterior uveitis patients, whilst posterior uveitis was rare and associated to infectious disease.

Adult-onset Still disease in southeast Brazil.

BACKGROUND: Adult-onset Still disease (AOSD) has been described all over the world. Clinical presentations and prognosis have varied in different studies. OBJECTIVE: The objective of this study was to determine the clinical presentation and the evolution of AOSD at a tertiary referral center in southeast Brazil. METHODS: The clinical records of 16 patients were retrospectively studied to determine symptoms at diagnosis, follow up, and the medication prescribed. RESULTS: The mean age at onset was 30.8 years (range, 24-55 years; standard deviation [SD], 9.2 years) with a slight male prevalence (54.2%). All patients presented constitutional symptoms, fever, and skin rash. Liver involvement was observed in all cases, with hepatomegaly in 81.3%, increased liver enzymes in 50.0%, and hypergammaglobulinemia in 68.8%. Cardiac involvement was observed in 12.6%, pleuritis in 6.3%, and renal involvement in 25.0%. All patients presented leukocytosis with a predominance of neutrophils. Elevated ferritin levels were observed in 56.3%, and these levels were normalized after disease remission. Initial treatments included nonsteroidal antiinflammatory drugs and low-dosage corticosteroids in all patients; 43.8% also needed methotrexate. In 25.0% of cases, a monocyclic disease was observed; others had recurrent episodes. After a follow up of 6.9 years (SD, 1.2 years), carpal ankylosis was the main articular sequel, observed in 53.6% of the patients. CONCLUSION: AOSD is rare in southeast Brazil. Although less severe systemic manifestations, like serositis and pneumonitis, were observed, reversible liver involvement was common; the frequency of recurrent disease and carpal ankylosis was higher than in previous studies.

Hipoparatireoidismo primário manifestando-se com aumento de enzimas musculares / Primary hypoparathyreoidism with raised muscle enzymes

Os autores relatam o caso de um jovem de 18 anos de idade com fraqueza muscular generalizada, cãibras e crises convulsivas, tratado com corticosteróides por oito anos em decorrência de um diagnóstico de polimiosite, sem melhora clínica. Ao exame físico apresentava forca muscular normal, ausência de atrofias musculares, diminuicão de reflexos tendíneos profundos e presenca do sinal de Chvostek e Trousseau. A hipótese diagnóstica inicial foi de miopatia metabólica e a investigacão laboratorial revelou cálcio sérico e urinário diminuídos e dosagem de paratormônio (PTH) indetectável. O diagnóstico final foi de hipoparatireoidismo, tendo sido o paciente tratado com cálcio endovenoso. A tomografia de crânio demonstrou calcificacões difusas, características da síndrome de Fahr. O paciente recebeu alta assintomático com carbonato de cálcio e colecalciferol.

Camurati-Engelmann disease: failure of response to bisphosphonates: report of two cases.

Camurati-Engelmann disease is a rare bone disorder characterized by cortical thickening of the diaphysis of tubular bones, with sparing of the epiphysis. It has variable degrees of penetrance and expression, but may be very disabling for the affected individuals who manifest the painful symptoms. The authors report on two women with typical presentation of severe Camurati-Engelmann disease whose treatment with bisphosphonates failed to add any improvement beyond that elicited by corticosteroids alone.

Eficácia sintomática dos insaponificáveis de abacate e soja (IAS)* no tratamento de osteoartrose (OA) de quadril e joelho / Efficacy and tolerability of unsaponifiables of and soy in the treatment of symptomatic hip and knee osteoarthritis

Introdução: A osteoartrose (OA) é uma doença idiopática, multífatorial, caracteriza- da por uma lenta e progressiva degradação da cartilagem articular. Objetivo: Avaliar a eficácia e segurança dos insaponificáveis de abacate e soja (IAS) no tratamento de pacientes com osteoartrose sintomática (OA) do joelho e do quadril. Métodos: Foram incluídos 231 pacientes de ambos os sexos, com idade entre 45 e 75 anos, que apresentavam osteoartrose sintomática de joelho e/ou quadril confirmadas, clínica e radiologicamente, pelos Critérios do Colégio Americano de Reumatologia. Entraram em um estudo aberto, não randomizado, multicentrico, controlado, com um período de tratamento de seis meses. Um intervalo de 15 dias (Washout) para drogas antiinflamatórias não esteroidais (AINEs) precedeu o estudo. A eficácia foi julgada de acordo com: 1) índice Funcional de Lequesne (IFL); e 2) dor na Escala Visual Analógica (EVA; escala de 100 mm)" íngestão de AINElanalgésícos e escore de incapacidade total (pela EVA de 100 mm). Resultados: 231 pacientes receberam os IAS. Os dados de todos os pacientes encontravam-se disponíveis para avaliação no mês 6. A média +/- SEM no escore do IFL diminuiu de 11,88+/-0,24 para 6,84 +/- 0,29 no final de 6 meses (p < 0,001). A dor diminuiu de 6,65+/- 0, 11 mm para 3,21 +/- 0,17 mm (p < 0, 00 1). O consumo de A INES diminuiu de 1,95 +/- 0,10 para 0,67 +/- 0,14 (p <0,001) ao final de 6 meses. A taxa de sucesso foi de 42,3por cento no grupo de IAS ao final de 6 meses. A incapacidade funcional total foi signifícativamente reduzida no grupo IAS. A tolerância foi de boa a excelente para a maioria dos pacientes. Conclusão: Os IAS, neste estudo aberto, controlado e multícêntrico, mostrou ser seguro e eficaz, no controle sintomático da osteoartrose de quadril e de joelho. Estudos mais prolongados, com metodologias mais avançadas, na procura de desvendar seus mecanismos de ação ainda são necessários

Avaliação da pressão venosa periférica na esclerose sistêmica / Peripheral venous pressure in systemic sclerosis

Objetivo: Estabelecer a utilidade da medida indireta da pressão venosa periférica (PVP) na avaliação de pacientes com esclerose sistêmica (ES). comparando com uma população controle normal. Métodos: Estudo prospectivo avaliando 18 pacientes esclerodérmicas do sexo feminino (sendo oito delas com úlcera cutânea isquêmica) submetidas à medida indireta da PVP (por pletismografia). Os dados foram comparados a um grupo controle normal de 18 mulheres hígidas, pareadas para idade. Resultados: Os níveis de pressão arterial foram semelhantes nos dois grupos. Os valores de PVP estavam significativamente diminuídos nos pacientes com ES (58.9 + ou - 11.6 mmHg no grupo ES e 96.9 + ou - 7.1 mmHg no grupo controle; p < 0.0001); no grupo com ES, os pacientes com úlceras cutâneas isquêmicas apresentaram níveis de PVP significativamente diminuídos comparando-se com os pacientes com ES sem úlceras (50.6 + ou - 10.8 mmHg no grupo com úlceras e 65.5 + ou - 7.2 mmHg no grupo sem úlceras; p = 0.006). Conclusões: Pode-se inferir que a diminuição nos níveis de PVP na Es parece estar associada a uma diminuição do fluxo sangüíneo, predispondo ao aparecimento de úlceras isquêmicas nestes pacientes.

Crise renal como manifestação inicial de esclerose sistêmica sine scleroderma / Renal crisis as the initial clinical manifestation in systemic sclerosis sine scleroderma

A esclerose sistêmica (ES) sine scleroderma é uma doença rara, caracterizada pelo acometimento visceral (geralmente gastrintestinal, pulmonar ou vascular periférico) da ES, na ausência da característica fibrose cutânea. Os autores descrevem um caso da rara ocorrência da crise renal esclerodérmica como manifestação inicial em uma paciente com ES sine scleroderma.