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Epoetin biosimilars are an alternative to originator erythropoietic agents in the treatment of chemotherapy-induced anaemia; however, their effects in patients with lymphoproliferative disorders remain unclear. This analysis examined the response of patients with lymphoproliferative disorders experiencing chemotherapy-induced anaemia to 4- or 8-week treatment with the biosimilar epoetin alpha. Treatment was initiated at first occurrence of haemoglobin (Hb) < 10 g/dL during chemotherapy and was stopped when Hb was >11 g/dL, when chemotherapy was completed, or in case of transfusion dependency. Response to epoetin alpha was defined as an increase in Hb of >1 g/dL or as an Hb > 11 g/dL. Stability was defined as change in Hb of ±1 g/dL, and no response was indicated by a decrease in Hb of >1 g/dL or acquired transfusion dependence. Overall, 65 patients were enrolled (median age 69 years; 47.7% ≥ 70 years old). Mean Hb levels at the initiation of epoetin alpha was 9.3 ± 0.5 g/dL. Mean Hb levels reached 10.7 ± 1.4 and 10.6 ± 1.5 g/dL at weeks 4 and 8, respectively, in patients on first-line chemotherapy and 11.4 ± 1.6 and 9.7 ± 1.3 g/dL in those on a second- or higher-line regimen. Overall, 70.8% of patients responded, 26.1% had stable Hb, and 3.1% did not respond. Delays or interruption of any chemotherapy cycle due to anaemia occurred in 18 patients. The biosimilar epoetin alpha was well tolerated and allowed patients with non-Hodgkin lymphoma or chronic lymphoproliferative disorders to continue their course of chemotherapy by effectively increasing and maintaining adequate concentrations of Hb., (Copyright © 2017 John Wiley & Sons, Ltd.)

Background: Celiac disease is an autoimmune enteropathy triggered by the ingestion of gluten in genetically susceptible individuals. Almost all celiac patients carry immune recognition genes coding for HLA-DQ2.5 and DQ8 heterodimers. Over the last few years, great importance has been given to HLA-DQ2.2 as probable predisposing variant, although controversies still exist regarding its relevance., Objective: The aim of our study was to determine the possible existence of an association between HLA-DQ2.2 and celiac disease in Brazilian children by analyzing the prevalence of the predisposing variants for celiac disease in a representative group of children of a population in which this determination is still missing., Methods: HLA-DQ typing was performed in samples from a group of celiac (n=100) and non-celiac children (n=110). All samples were tested for the presence of the following variants: DQA1*05-DQB1*02 (DQ2.5), DQA1*03-DQB1*03:02 (DQ8) and DQA1*02:01-DQB1*02:02 (DQ2.2). Fisher`s exact test was used for statistical analysis., Results: In the group of 100 celiac children, 78 (78%) were positive for DQ2, 13 (13 %) were DQ2/DQ8 and 6 (6%) were DQ8 positives. The HLA-DQ pattern in the 110 non-celiac children was as follows: positive for DQ2 in 33 (29.9%) samples, in 2 (1.8 %) was positive for DQ2/DQ8 and in 15 (13.6%) was positive for DQ8. We found significant differences between the distribution of some but not all of the analyzed alleles when comparing celiac and non-celiac children., Conclusion: The genotyping of celiac disease HLA-DQ predisposing alleles showed similarities with HLA-DQ patterns found in both European and non-European populations, which may be a reflection of the miscegenation, which gave origin to the current Brazilian population. No significant association was found between DQ2.2 variant and celiac disease in the studied population.

Rubella is an acute viral disease that usually does not generate sequels; however, in pregnant women the infection can cause serious abnormalities to fetuses, which are collectively called congenital rubella syndrome. In Brazil, population immunization was started in 1992, but few epidemiological studies have been conducted to assess vaccination coverage and seroconversion since then. The aim of this work is to evaluate the seropositivity of pregnant women to rubella virus after vaccination campaign was carried out in 2008. Serological tests for rubella diagnosis were performed in 87 pregnant women who attended the University of Brasilia Hospital, Federal District, Brazil. Antirubella IgG antibodies were detected in 83 out of 87 pregnant women (95.4%), with an age-independent seroprevalence. Only one woman was positive in IgM serological tests. Our data suggest high levels of vaccination coverage and antirubella immunization in the Brazil Federal District population.

Objective: The purpose of this study was to verify the presence of endothelial dysfunction and initial structural atherosclerotic changes in children with Type 1 diabetes mellitus (T1DM)., Subjects and Methods: The study population comprised 31 diabetic children aged 6 to 12 years, divided into two subgroups according to the duration of the T1DM diagnosis: subgroup 1, with less than 5 years elapsed since diagnosis, and subgroup 2, with more than 5 years elapsed since diagnosis. The control group comprised 58 age-matched healthy children. Ultrasonographic techniques were used to measure the flow-mediated dilatation (FMD) of the brachial artery and the intima-media thickness (IMT) of the carotid arteries., Results: Children with T1DM with longer disease duration showed significantly decreased mean values of FMD compared with those in the control group. No significant differences between the groups were found in relation to IMT. The FMD percentage presented a moderate negative correlation with glycated hemoglobin (HbA1c) and fasting glucose levels., Conclusion: Our findings suggest that endothelial dysfunction may be already present in children with 5 years or more elapsed since diagnosis, even in the absence of atherosclerotic structural changes. The decreased vasodilation response correlated with hyperglycemia.

Objective: The present study aimed to assess the safety of gluten-free bakery products for consumption by coeliac patients. Design/setting In the current exploratory cross-sectional quantitative study, a total of 130 samples were collected from twenty-five bakeries in Brasilia (Brazil). For the quantification of gluten, an ELISA was used. The threshold of 20 ppm gluten was considered as the safe upper limit for gluten-free food, as proposed in the Codex Alimentarius., Results: The results revealed a total of 21·5 % of contamination among the bakery products sampled. Sixty-four per cent of the bakeries sold at least one contaminated product in our sample., Conclusions: These findings represent a risk for coeliac patients since the ingestion of gluten traces may be sufficient to adversely impact on their health.

Conditions associated to the consumption of gluten have emerged as a major health care concern and the treatment consists on a lifelong gluten-free diet. Providing safe food for these individuals includes adapting to safety procedures within the food chain and preventing gluten cross-contamination in gluten-free food. However, a gluten cross-contamination prevention protocol or check-list has not yet been validated. Therefore, the aim of this study was to perform the content validation and semantic evaluation of a check-list elaborated for the prevention of gluten cross-contamination in food services. The preliminary version of the check-list was elaborated based on the Brazilian resolution for food safety Collegiate Board Resolution 216 (RDC 216) and Collegiate Board Resolution 275 (RDC 275), the standard 22000 from the International Organization for Standardization (ISO 22000) and the Canadian Celiac Association Gluten-Free Certification Program documents. Seven experts with experience in the area participated in the check-list validation and semantic evaluation. The criteria used for the approval of the items, as to their importance for the prevention of gluten cross-contamination and clarity of the wording, was the achievement of a minimal of 80% of agreement between the experts (W-values ≥ 0.8). Moreover, items should have a mean ≥4 in the evaluation of importance (Likert scale from 1 to 5) and clarity (Likert scale from 0 to 5) in order to be maintained in the instrument. The final version of the check-list was composed of 84 items, divided into 12 sections. After being redesigned and re-evaluated, the items were considered important and comprehensive by the experts (both with W-values ≥ 0.89). The check-list developed was validated with respect to content and approved in the semantic evaluation., Competing Interests: The authors declare no conflict of interest.

Background: Brentuximab vedotin (BV) has shown high overall response rate in refractory/relapsed Hodgkin lymphoma (HL) and systemic anaplastic large cell lymphoma (sALCL) with reported long-term response duration in clinical trials, but few data are available regarding its role in long-term outcomes in real life., Patients and Methods: A single-center observational study was conducted on patients treated with BV in daily clinical practice to evaluate the long-term effectiveness of BV in HL and sALCL patients and to check whether clinical trial results are confirmed in a real-life context., Results: The best response rate in the treated 53 patients (43 HL and 10 sALCL) was 69.8% (with 46.5% complete response [CR]) in HL and 100% (80% CR) for sALCL, respectively. With a median patient follow-up of 36.8 months, the estimated median duration of response was 31.5 months for HL and 17.8 for sALCL, respectively. At the latest available follow-up, 75% of patients were still in response, with 43% without any consolidation. Toxicity was primarily neurological and it was rarely so serious to require dose reduction or interruption. In addition, it always reversed completely after the end of treatment., Conclusion: Our data showed that 51% of patients treated with BV can be regarded as "long-term responders." Among these cases, for all patients who underwent stem cell transplantation immediately after BV, the procedure was consolidative. For patients who have remained in continuous CR without any consolidation after therapy, BV can induce prolonged disease control., Implications for Practice: Brentuximab vedotin (BV) has shown a high overall response rate in refractory/relapsed Hodgkin lymphoma and systemic anaplastic large cell lymphoma, with reported long-term response duration in clinical trials, whereas few data are available regarding its role in long-term outcomes in real life. The data reported in this study suggest that BV can induce the same results in daily clinical practice. The data showed that 51% of patients treated with BV can be regarded as "long-term responders." Among these cases, BV can induce prolonged disease control in patients who have remained in continuous complete response without any consolidation after the drug., Competing Interests: of potential conflicts of interest may be found at the end of this article., (©AlphaMed Press.)

The personal care industry is focused on developing safe, more efficacious, and increasingly milder products, that are routinely undergoing preclinical and clinical testing before becoming available for consumer use on skin. In vitro systems based on skin reconstructed equivalents are now established for the preclinical assessment of product irritation potential and as alternative testing methods to the classic Draize rabbit skin irritation test. We have used the 3-D EpiDerm™ model system to evaluate tissue viability and primary cytokine interleukin-1α release as a way to evaluate the potential dermal irritation of 224 non-ionic, amphoteric and/or anionic surfactant-containing formulations, or individual raw materials. As part of our testing programme, two representative benchmark materials with known clinical skin irritation potential were qualified through repeated testing, for use as references for the skin irritation evaluation of formulations containing new surfactant ingredients. We have established a correlation between the in vitro screening approach and clinical testing, and are continually expanding our database to enhance this correlation. This testing programme integrates the efforts of global manufacturers of personal care products that focus on the development of increasingly milder formulations to be applied to the skin, without the use of animal testing., (2016 FRAME.)

Background: Autoimmune manifestations may occur in patients with hairy cell leukemia (HCL), and some rare cases of polyarteritis nodosa and leukocytoclastic vasculitis have been reported. However, data regarding the treatment of these cutaneous manifestations are lacking, given the rarity of the concomitance of HCL and vasculitic syndromes., Case Presentation: We present a 37-year-old man with paraneoplastic leukocytoclastic vasculitis complicating newly diagnosed HCL. The vasculitis completely resolved after the first 3 weekly administrations of cladribine, which is regarded as the gold-standard treatment for this disease. The underlying leukemia showed refractoriness to the same agent, thus requiring a second line of treatment., Conclusions: The clinical picture we have observed is of interest for the following reasons: i) it confirms an existing pathogenetic relationship between this lymphoproliferative disorder and its cutaneous manifestations, as suggested by the prompt resolution of the purpuric lesions upon cladribine administration; ii) it indicates that cladribine is an effective treatment for HCL-related paraneoplastic syndromes, including leukocytoclastic vasculitis; iii) the evolution and the outcomes of the paraneoplastic manifestations may be independent of those of the underlying leukemia, which showed less than a partial response to its initial treatment.

Introduction:: The impact of gestational toxocariasis is an understudied topic on female reproductive health. We estimated anti-Toxocara IgG prevalence among pregnant women in Brasília, Brazil, and investigated the association of the infection with history of abortion and contact with pets., Methods:: Infection was diagnosed using ELISA with excretory/secretory antigens. Participant information was obtained via questionnaires., Results:: Of 311 pregnant women, 23 were anti-Toxocara IgG positive. Twenty-two percent of anti-Toxocara IgG-positive participants and 26% had previously miscarried. Previous contact with pets was associated with higher toxocariasis prevalence., Conclusions:: A direct relationship between toxocariasis and contact with pets was observed, but there was no relationship with the miscarriage prevalence.

Background: Medicinal plants have traditionally been used in many parts of the world as alternative medicine. Many extracts and essential oils isolated from plants have disclosed biological activity, justifying the investigation of their potential antimicrobial activity. In this study, the in vitro antifungal activity of six Brazilian Cerrado medicinal plant species were evaluated against clinically relevant Candida species., Methods: The crude extract plants were evaluated against American Type Culture Collection (ATCC) standard strains of Candida spp. using disk diffusion method and determining the minimum inhibitory concentration (MIC). The chemical study results were confirmed by HPLC method., Results: All six plant species showed antifungal activity. Among the species studied, Eugenia dysenterica and Pouteria ramiflora showed significant inhibitory activity against C. tropicalis at lowest MIC value of 125 and 500 μg/disc, respectively. The Eugenia dysenterica also disclosed MIC value of 125 μg/disc against C. famata, 250 μg/disc against C. krusei and 500 μg/disc against C. guilliermondii and C. parapsilosis. Pouteria torta, Bauhinia rufa, Erythroxylum daphnites and Erythroxylum subrotundum showed activity against the yeast strains with MIC value of 1000 μg/disc. The chemical study of the most bioactive extracts of Eugenia dysenterica and Pouteria ramiflora revealed catechin derivatives and flavonoids as main components., Conclusions: All six evaluated plant species showed good antifungal potential against several Candida strains. However, E .dysenterica and P. ramiflora showed the higher inhibitory effect against the non-albicans Candida species. Our results may contribute to the continuing search of new natural occurring products with antifungal activity.

Radioimmunotherapy (RIT) after an induction phase with conventional chemoimmunotherapy became an attractive strategy of consolidation for patients with advanced follicular lymphoma: in particular, in many studies RIT was represented by yttrium-90-ibritumomab tiuxetan ((90) Y-IT). Independently by the different front-line treatment, updates on the long-term follow-up of these studies are needed because the disease course of follicular lymphoma is characterised by multiple relapses and progressively shorter durations of response. We report updated long-term efficacy and toxicity results of a multicenter phase II study on sequential treatment with four cycles of fludarabine, mitoxantrone, and rituximab followed by (90) Y-IT as front-line therapy for untreated patients with intermediate/high-risk follicular lymphoma. With a median follow-up of 84 months, only 19/49 (38.8%) complete response patients relapsed, yielding an estimated long-term disease-free survival of 62.6%. The 7-year overall survival was 72.7%. Four (7.3%) second acute myeloid leukemia occurred, with a median time following RIT of 42 months. A relevant patients' responsiveness to subsequent therapies occurred: approximately 65% of relapsed patients obtained a good clinical response after the second-line treatment. These data represented the first evidence of a real role even in the long period of 90Y-IT after a fludarabine-containing regimen plus rituximab in the treatment of high-risk follicular lymphoma., (© 2016 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Objective: To comprehend the psychosocial effects that Coeliac Disease diagnosis entails., Method: Qualitative study, achieved through semi-structured interviews, analyzed in accordance to the Association of ideas map Technique. A total 12 recently-diagnosed patients from the Centro de diagnóstico, tratamento e apoio ao paciente com doença celíaca (Coeliac Disease Prevention, Support and Treatment Diagnose Centre) from the Hospital Universitário de Brasília (University Hospital of Brasilia) were enrolled for the study, between the years of 2013 and 2014., Results: The interviewed patients presented negative impacts in three categories: psychoaffective, family and social relationships, indicating issues with social readaptation once the treatment had started, as well as difficulty coping with a gluten free diet., Conclusion: Coeliac Disease holds substantial impact on psychological functions, family and social relationships to diagnosed patients, requiring a clinical biopsychological assistance for better adherence to treatment and patients quality of life.

Background. Celiac disease (CD) is a genetically determined immune-mediated disorder in which gluten immunogenic peptides are presented to CD4 T cells by HLA-DQ2.5, DQ8, DQ2.2, and their combinations. Our aim is to establish a risk gradient for celiac disease based on HLA-DQ profile in a brazilian representative population and the relevance of DQ2.2 in celiac disease development. Materials and Methods. 237 celiac patients and 237 controls (both groups with 164 females and 73 males) were included. All samples were tested for the presence of predisposing HLA-DQ alleles using the PCR-SSP method. Results were considered significant when p < 0.05. Disease risk was expressed as 1 :  N for each HLA-DQ category described at this study. Results. DQ2.5 and/or DQ8 were detected in 224 celiac patients (94.5%) and 84 controls (35.4%). Eight celiac patients (3.4%) and 38 controls (16%) disclosed only DQ2.2. Even though DQ2.2 ( β 2/ β 2 or β 2/x) showed a low CD risk of 1 : 251 and 1 : 550, respectively, the genotype DQ2.5/DQ2.2 ( β 2/ β 2) showed high CD risk of 1 : 10 ( p < 0.0001). The disease risk gradient ranged from 1 : 3014 to 1 : 7. Conclusion. Our study allowed the determination of a risk gradient for celiac disease development in at-risk population, showing that DQ2.2 variant was relevant when associated with DQ2.5., Competing Interests: The authors declare no competing interests regarding the publication of this paper.

Regarding primary mediastinal large B-cell lymphoma (PMLBCL), there are several controversial topics that warrant further investigation: the superiority of third-generation regimens, the impact of rituximab, the use of involved field radiotherapy (RT) and the assessment of clinical response by positron emission tomography (PET). We report our experience on 74 PMLBCL patients treated with a combination of a third-generation chemotherapy regimen (MACOP-B) and rituximab: an observational retrospective single-centre study was conducted on patients diagnosed and treated between February 2002 and July 2011. All patients were evaluated by computed tomography scan and PET scan; after the final PET evaluation, PET-negative patients were observed, whereas PET-positive patients underwent mediastinal RT. Sixty-one (82.4%) patients achieved a complete response after the MACOP-B plus rituximab regimen; 68.9% presented a positive final PET and were treated with local RT, whereas 31.1% had a negative PET. Five patients relapsed within 12 months. At 10 years, overall survival was 82%, progression-free survival was 87.6% and disease-free survival (DFS) was 90.5% (median follow-up 4 years). No statistically significant differences were observed in DFS between the patients treated also with RT (PET positive) and patients only observed (PET negative): 90.7% vs 90% (p = 0.85), respectively. In our experience, adding rituximab does not change the final results in terms of complete response and DFS utilizing third-generation regimen. Furthermore, the introduction of the PET-guided RT approach leads to a patient-tailored treatment, which preserves the outcome and, at the same time, allows reducing the use of RT., (Copyright © 2014 John Wiley & Sons, Ltd.)

Background: Adult Langerhans cell histiocytosis (LCH) is an orphan disease. Chemotherapy is usually reserved to patients presenting with single system multifocal (SS-m) or multisystem (MS) disease but due to the lack of randomized studies no standard first line therapy has been defined yet. Pediatric regimens based on the vinblastine/prednisone backbone are not well tolerated in adults and probably less effective. We previously demonstrated high efficacy of the dose dense polichemotherapy regimen MACOP-B in 7 adult patients with SS-m or MS-LCH, in terms of high response rate and durable responses. Here we report an update of these data with the purpose of evaluating the long term efficacy of MACOP-B in adult LCH., Methods: Clinical data of all adult LCH patients (n = 17) diagnosed and treated at our Institution during the past 20-year period were retrospectively reviewed., Results: A total of 11 patients (6 with SS-m and 5 with MS-LCH) were treated with MACOP-B from 1995 to 2014. The overall response rate was confirmed to be 100 %, with a complete response of 73 % and a partial response rate of 27 %. Overall progression free survival was 64 %, and disease free survival after achievement of initial CR was 87 %. Overall survival rate was 82 % after 6.7 years of median follow-up., Conclusions: These data confirm high activity of MACOP-B in adult LCH, indicating that a substantial fraction of patients achieve long lasting responses and can be cured with this therapeutic approach.

Indolent non-follicular lymphomas (inFLs) are generally regarded as incurable, apart from extranodal mucosa-associated lymphatic tissue lymphomas, which can be partially cured by surgery, local radiotherapy, or antibiotic treatment. The aim of the present study was to test the degree of effectiveness and the safety of the regimen containing fludarabine, mitoxantrone, and rituximab (FMR) in inFL patients considering all the different entities belonging to this group. An observational retrospective study was conducted on 143 inFL patients providing that their first chemoimmunotherapy performed was FMR regimen and diagnosis from September 2000 to March 2011. There were 32 small lymphocytic lymphomas and 111 marginal zone lymphomas. At the end of treatment, overall response rate was 96.5% with 88% of complete responses (CR) and 8.5% of partial responses. With a median follow-up of 48 months, 10 out of 125 (8%) CR patients had disease relapse, yielding an estimated 9-year disease-free survival (DFS) of 74.9% and an estimated 10-year overall survival of 92.8%. The estimated 9-year progression free survival was 70.5%. The 10 relapsed patients showed lymphoma recurrence within 52 months: after this time, the DFS curve presented a plateau configuration. Only two (1.4%) patients developed a secondary hematological neoplasia. This study showed promising findings for the use of a fludarabine-based regimen in combination with rituximab in the front-line treatment of symptomatic inFL with a noteworthy high percentage of CR associated to an interesting long-term DFS and favorable acute and long-term safety profile., (Copyright © 2014 John Wiley & Sons, Ltd.)

Langerhans cell histiocytosis (LCH) is a rare proliferative disease with a wide spectrum of clinical presentations and, as a consequence, the treatment choice is unclear. Recently, detection of the BRAF V600E mutation changed the perspective of this disease, suggesting a possible use for BRAF inhibitors in its treatment. Herein, a case is presented of a patient with LCH undergoing treatment with vemurafenib after several lines of therapy. After 4 months of vemurafenib treatment, skin lesions associated with cranial involvement were reduced in size at physical evaluation and nuclear imaging assessment showed a very good partial response, with the resolution of multiple lesions. Based on this very good partial response and because the patient tolerated treatment well, the patient was able to continue treatment with vemurafenib until disease progression nearly 10 months later. This approach should be considered for patients with severe and multiresistant LCH with a BRAF mutation. However, more studies are needed to evaluate the efficacy and duration of response in a larger patient population., (Copyright © 2015 by the National Comprehensive Cancer Network.)

Background: Celiac disease is an autoimmune enteropathy triggered by the ingestion of gluten in genetically susceptible individuals. Genetic susceptibility is associated with two sets of alleles, DQA1*05 - DQB1*02 and DQA1*03 - DQB1*03:02, which code for class II MHC DQ2 and DQ8 molecules, respectively. Approximately 90%-95% of celiac patients are HLA-DQ2 positive, and half of the remaining patients are HLA-DQ8 positive. In fact, during a celiac disease diagnostic workup, the absence of these specific DQA and DQB alleles has a near perfect negative predictive value., Objective: Improve the detection of celiac disease predisposing alleles by combining the simplicity and sensitivity of real-time PCR (qPCR) and melting curve analysis with the specificity of sequence-specific primers (SSP)., Methods: Amplifications of sequence-specific primers for DQA1*05 (DQ2), DQB1*02 (DQ2), and DQA1*03 (DQ8) were performed by the real time PCR method to determine the presence of each allele in independent reactions. Primers for Human Growth Hormone were used as an internal control. A parallel PCR-SSP protocol was used as a reference method to validate our results., Results: Both techniques yielded equal results. From a total of 329 samples the presence of HLA predisposing alleles was determined in 187 (56.8%). One hundred fourteen samples (61%) were positive for a single allele, 68 (36.3%) for two alleles, and only 5 (2.7%) for three alleles., Conclusion: Results obtained by qPCR technique were highly reliable with no discordant results when compared with those obtained using PCR-SSP.

Hairy cell leukemia (HCL) patients could have an excellent prognosis with adequate treatment. Treatments are not generally curative but are extremely effective in inducing long-lasting clinical remissions. An observational retrospective study was conducted on a single-center registry of 144 patients with a median follow-up of 11.5 years, focusing on long-lasting continuous first complete remissions (CR) wondering if patients can be cured only with front-line approach. CR for more than 5 years after first-line therapy were found in 22.2 % cases. The median duration of response was 9.8 years, while for relapsed patients, the first response had a median duration of 2.4 years. Three different subsets of long-lasting first CR were identified: 15 patients are between 5 and 10 years with a median duration of CR of 6.5 years; 7 patients are between 10 and 15 years with a median duration of CR of 12.3 years; and 10 patients present a follow-up superior to 15 years with a median duration of CR of 20.0 years. There is a need for continuous study in this field to better define the optimal therapeutic regimen and, in particular, the biological issues since at least 20-25 % of HCL patients can be cured with only one treatment.

The definition of the role of positron emission tomography (PET) in peripheral T-cell lymphomas (PTCLs) is still under investigation. The purpose of the present observational retrospective study was to assess the early prognostic value of PET after the first three cycles of therapy (PET+3), evaluating visual data in de novo PTCL patients treated in first line with standard chemotherapy and followed by both PET and computed tomography scan. Of 27 PET+3-negative patients, 19 also had a negative PET at the end of treatment (PET+6), whereas 8 of 27 had a positive final one; 6 of 7 PET+3-positive patients had a positive PET+6, whereas only 1 patient had a negative PET+6. Estimated overall survival plotted according to PET+3 results showed 78.6% for negative patients and 21.4% for positive patients at 88.7 months with a significant difference. Patients with negative PET+3 had superior progression-free survival of 72.6% compared with 16.7% of PET+3-positive patients. At the time of this analysis, 17 of 19 (89.5%) patients with negative PET+3 are in continuous complete response (CCR) and only 1 of 7 (14.2%) patients with positive PET+3 is still in CCR. In conclusion, our results indicate that positive PET+3 is predictive of a worse outcome in PTCL, and this significant statistical difference between the two curves could be clinically informative. Larger and prospective studies and harmonization of PET reading criteria are needed., (©AlphaMed Press.)

Background: Although it is known that first degree relatives of celiac patients have an increased risk for celiac disease few studies are available on its incidence. We investigated the incidence of serologic conversion and of new cases of celiac disease among first degree relatives with negative results at a first screening., Methods: From a total of 634 first degree relatives of 186 biopsy-proven celiac disease patients diagnosed between October 2000 and October 2010, 450 subjects agreed to participate in the study (Group I), and underwent serologic screening. Between January 2010 and October 2012, out of the initial group of 450, 205 previously sero-negative subjects consented to participate in a second stage of the study and undergo new serologic testing (Group II). All serologically positive individuals of both groups (I and II) were genotyped for celiac disease-predisposing alleles (HLA-DQ2/DQ8)., Results: 19 subjects (4.2%) out of the 450 subjects of Group I disclosed positive serologic results, presence of DQ2 and/or DQ8 alleles and celiac disease-compatible mucosal abnormalities. The 205 previously negative first degree relatives from Group II that underwent new serologic testing disclosed eight sero-converted subjects. Mucosal abnormalities in five of these patients confirmed the diagnosis of celiac disease. During the 10-year period of the study the incidence of sero-conversion was 8/205 and the incidence of biopsy-proven celiac disease cases was 5/205., Conclusions: Our data are coincident with other works on this subject and confirm once again that relatives of celiac patients, especially first degree relatives are at high risk of developing celiac disease. In view of the relatively low incidence further studies are needed to try to establish a useful and cost-effective algorithm for follow-up of relatives of celiac patients.

Current treatments for non-Hodgkin lymphomas are not optimally effective. Among new agents, bortezomib seems to play a pivotal role in the regulation of several cell pathways involved in the development of lymphomas. After results were obtained with clinical trials, we aimed to observe treatment with bortezomib in everyday clinical practice. We performed a multicenter retrospective analysis to assess the efficacy of bortezomib in heavily pretreated (median number of previous therapies 4, range 2-6) lymphoma patients in an off-label setting. Bortezomib therapy was scheduled for 4-6 cycles (1.3 mg/m(2) biweekly). Data from 50 patients were collected: 22% had a complete remission, 26% obtained a partial response and the remaining 52% was non-responder. According to histotype, we observed an overall response rate (ORR) of 51.6% in mantle cell lymphomas, an ORR of 60% among follicular lymphoma patients, and an ORR of 50% in the indolent nonfollicular lymphomas. None of diffuse large B-cell lymphoma patients obtained a response. Extra-hematological toxicity was really mild, and peripheral neuropathy occurred in only 5 patients; hematological toxicity was grades 3-4 thrombocytopenia in nine patients and grades 3-4 neutropenia in only three patients. In conclusion, treatment with bortezomib as single agent resulted safe and effective in a subset of heavily pretreated lymphoma patients with usually poor outcome. New future hypotheses of investigation are indicated., (Copyright © 2012 John Wiley & Sons, Ltd.)

Bronchial-associated lymphoid tissue (BALT) lymphoma is an extranodal primary pulmonary lymphoma. The optimal therapy for this rare disease is still debated, and few heterogeneous data are available in literature. The aim of our study was to critically review data of patients with BALT lymphoma treated in first-line therapy with fludarabine and mitoxantrone-containing regimens (with or without rituximab) to investigate the effectiveness and the safety of this approach and patients' survival. An observational retrospective study was performed on homogenous clinical data from 17 patients with biopsy-proven diagnosis of BALT. All the patients were treated with fludarabine and mitoxantrone-containing regimen therapy. Radiological findings were also reviewed to assess the role of (18) fluoro-deoxyglucose positron emission tomography in the initial assessment and in the monitoring of this extranodal lymphoma. A high percentage of response was observed: 82.3% of patients achieved a complete response, 11.8% a partial response. Furthermore, a very remarkable progression-free survival (71%) and overall survival (100%) were estimated at 14 years. No relevant toxicities were registered. Our results support the use of fludarabine and mitoxantrone-containing regimens as first-line therapy in the treatment of BALT lymphoma even if further data are necessary to consolidate our findings. Positron emission tomography scanning may provide additional valuable information in the assessment of BALT lymphoma., (Copyright © 2012 John Wiley & Sons, Ltd.)

Aim: To evaluate the prevalence of celiac disease in a group of Brazilian individuals over 60 years of age and compare it with the previously known prevalence in a pediatric group living in the same geographical area., Methods: The research protocol was approved by the Ethics Committee of the University of Brasilia School of Medicine, Brasilia, Brazil. Blood samples from 946 individuals (295 male and 651 female) aged 60 years or older were collected between May 2010 and July 2011. The study subjects' mean and median ages were 68.1 and 67 years, respectively, ranging from 60 to 92 years. That age distribution closely corresponded to the age distribution of the Brazilian population according to the Brazilian 2010 census. The participants were consecutive and unselected outpatients undergoing blood tests at the University of Brasilia Hospital's Clinical Pathology Laboratory. All sera were tested for immunoglobulin A anti-transglutaminase antibodies (IgA-tTG) by enzyme- linked immunosorbent assay, and those that were positive were further tested for immunoglobulin A anti-endomysium antibodies (IgA-EMA). Human leukocyte antigen (HLA) genotyping was performed for all individuals who exhibited positive serologic results for IgA-tTG and/or IgA-EMA., Results: Out of the 946 studied patients, only one previously diagnosed case of biopsy-proven celiac disease was detected. For the remaining subjects, nine serum samples tested positive for IgA-tTG antibodies; however, none of them tested positive for IgA-EMA antibodies. The HLA genotyping of those nine subjects revealed that one was carrying DQA1*0501 and two were carrying DQB1*0201 alleles. These data showed that, among those 946 elderly individuals, the prevalence of celiac disease (CD) was 0.1% (95%CI: 0.00-0.59). The prevalence of CD for the elderly group was compared with that observed for the group of 2034 children younger than 15 years (age range, 1-14 years; mean age, 8 years) who took part in our previous CD prevalence screening study. All the children came from the same geographical region and shared a similar ethnic and low-income background. As in the elderly group in the current study, the younger group was made up of consecutive outpatients who underwent blood evaluation at the University of Brasilia Hospital's Clinical Laboratory. The prevalence of biopsy-proven CD among those children was 0.54% (95%CI: 0.27-0.57). The comparative analysis between the two groups resulted in the following values: odds ratio = 0.19 (95%CI: 0.01-1.45) Fisher test P = 0.06., Conclusion: The prevalence of CD among the children of our previous study was 5.4 times higher than that found in the present elderly group.

Background: Vitamin D is a secosteroid hormone with important roles in the control of bone and mineral metabolism of vertebrates and in the maintenance of systemic homeostasis. This study aimed (i) to evaluate the serum concentrations of 25-hydroxy-vitamin D levels [25(OH)D], parathyroid hormone (PTH) and ionized calcium (iCa) of wild Callithrix penicillata (black-tufted marmosets) and (ii) to propose reference ranges for those analytes for free-living marmosets., Methods: Blood samples were collected from 15 wild animals and analyzed for 25(OH)D, PTH and iCa. Reference values were calculated following standard analytical criteria., Results: The observed mean serum levels (±standard deviation) were 25(OH)D, 61.7 (±20.8) ng/ml; PTH, 275.2 (±34.1) pg/ml; iCai 4.0 (±0.6) mg/dl., Conclusions: For free-living marmosets, the proposed physiological range for 25(OH)D is 20.1-103.3 ng/ml and for PTH is 207.0-343.3 pg/dl, with a confidence interval of 95%., (© 2012 John Wiley & Sons A/S.)

Background: Celiac disease is an autoimmune disorder that occurs in genetically susceptible individuals in whom the ingestion of dietary gluten induces intestinal mucosa inflammation. Previous studies suggest that celiac disease may either be very rare or underdiagnosed in African and/or African-derived population., Aim: Determine the prevalence of celiac disease in Sub-Saharan African-derived Brazilian communities using serological screening., Subjects and Methods: Inhabitants from 10 African-derived communities from Northeastern of Brazil were screened for celiac disease. All sera were tested for endomysial class IgA antibody using indirect immunofluorescence., Results: No positive test for IgA-endomysial was observed in the 860 individuals tested., Conclusion: Our data suggests a low prevalence of celiac disease in African-derived Brazilian populations., (Copyright © 2012 Wiley Periodicals, Inc.)

The present report describes the severe evolution of Kawasaki disease in a three-month-old infant. The ailment was initially atypical in its presentation, with the patient exhibiting only persistent fever in association with a progressive lethargy and maculopapular rash on the face, trunk and limbs erroneously diagnosed as roseola infantum. On the 10th day of the condition, mainly due to the unexplained persistence of fever, the infant was admitted to a local hospital. The typical features of KD appeared only on the 14th day of illness with the relapse of the maculopapular rash in association with non-purulent conjunctivitis; dry, reddish and fissured lips; tongue with reddish and hypertrophic papillae; erythema and edema of the palms and soles. During the following days, the ailment rapidly evolved to a catastrophic clinical picture characterized by generalized vasculitis, splenic infarction, pulmonary thrombosis, giant right and left coronary aneurysms, dilatation of common and internal iliac arteries and progressive ischemia of the distal third of the feet resulting in necrotic lesions of both halluces. Appropriate therapy was initiated, but repeated administration of intravenous immunoglobulin G (IVIG) followed by three days of administration of methylprednisolone did not abate the intense inflammatory activity. The remission of inflammation and regression of vascular lesions were only achieved during the following five weeks after the introduction of methotrexate associated with etanercept. The report of this case aims to draw attention to severe forms of KD that exhibit an unfavorable evolution and can be extremely refractory to the conventional therapy.

The objective of this study was to develop and analyze a gluten-free pasta made with green banana flour. The study was divided into five steps: preparation/selection, chemical, sensory, technological, and statistical analysis. The modified sample presented greater acceptance (84.5% for celiac individuals and 61.2% for nonceliac) than standard samples (53.6% for nonceliac individuals). There was no significant difference between the modified and the standard samples in terms of appearance, aroma, flavor, and overall quality. The modified pastas presented approximately 98% less lipids. Green bananas are considered a subproduct of low commercial value with little industrial use. The possibility of developing gluten-free products with green banana flour can expand the product supply for people with celiac disease and contribute to a more diverse diet., (Copyright © 2012 Academy of Nutrition and Dietetics. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate the possible association between celiac disease (CD) and/or gluten sensitivity (GS) and autism spectrum disorder (ASD)., Methods: Occurrences of CD were determined in a group of children and adolescents affected by ASD and, conversely, occurrences of ASD were assessed in a group of biopsy-proven celiac patients. To detect the possible existence of GS, the levels of antigliadin antibodies in ASD patients were assessed and compared with the levels in a group of non-celiac children., Results: The prevalence of CD or GS in ASD patients was not greater than in groups originating from the same geographical area. Similarly the prevalence of ASD was not greater than in a group of biopsy-proven CD patients., Conclusion: No statistically demonstrable association was found between CD or GS and ASD. Consequently, routine screening for CD or GS in all patients with ASD is, at this moment, neither justified nor cost-effective.

Background: Atherosclerosis is the leading cause of coronary heart disease and ischemic stroke, which can cause sudden death in adulthood. In general, the clinical manifestations of cardiovascular diseases are caused by atherosclerosis, which is a process that starts during middle age. More recent studies indicate that the atherosclerotic process begins during childhood., Methods: To evaluate the extent of atherosclerotic disease in young Brazilians, we conducted a study of the pathological alterations in the major arteries of victims of violent death. Samples of the right carotid artery, left coronary artery, and thoracic aorta of young victims of violent death were analyzed and graded in accordance with the histological atherosclerotic lesion types proposed by the American Heart Association. Samples were collected from 100 individuals who had died from external causes, aged from 12 to 33 years., Results: The majority of cases (83%) were male, and 66% of deaths were homicides caused by firearms. The median age was 20.0 years and mean body mass index was 20.9 kg/m2. Of the right carotid artery specimens, 3% were normal, 55% had type I, 40% had type II, 1% had type III, and 1% had type IV atherosclerotic lesions. Of the left coronary artery specimens, 5% were normal, 48% had type I, 41% had type II, 3% had type III, and 3% had type IV lesions. Of the thoracic aorta specimens, none were normal, 13% had type I, 64% had type II, 22% had type III, and 1% had type IV lesions. Overall, 97.34% of arteries examined had some degree of atherosclerosis. The most common histological type was type II (foam cells). No thoracic aorta specimens were normal, and the coronary artery specimens had the most atherosclerosis., Conclusions: Our results show a high prevalence of atherosclerotic lesions among young people in Brazil. Intervention should be undertaken to decrease the rate of sudden cardiac death in the adult population.

Objective: To evaluate the efficacy of horse ridding simulator on the sitting postural control of children with spastic diplegia., Method: Forty children were randomly divided in a group using the simulator (RS) and a group performing conventional physical therapy (CT). FScan/Fmat equipment was used to register maximal displacement in antero-posterior (AP) and medio-lateral (ML) directions with children in sitting position. At the pre and post intervention stage both groups were classified according to the Gross Motor Function Classification System (GMFCS) and, after intervention, by the AUQEI questionnaire (Autoquestionnaire Qualité de vie Enfant Image)., Results: Comparison between groups disclosed statistically significant pos-intervention improvement both in the AP (p<0.0001) as in the ML (p<0.0069) direction in the RS group., Conclusion: The horse ridding simulator produced significant improvement in the postural control of children in sitting position, additionally showing a higher motor functionality and a better acceptance of the therapeutic intervention.

Background: We performed a retrospective investigation to assess the efficacy of chemotherapy and rituximab as front-line treatment for primary bone lymphoma (PBL)., Patients and Methods: Between 1999 and 2009, 21 previously untreated patients received a diagnosis of PBL. All the patients were treated with anthracycline-containing chemotherapeutic regimens, with the addition of rituximab; 11 patients received consolidative radiation therapy after induction treatment., Results: Patients' median age was 34 years (range, 18-82 years); all presented with diffuse large B-cell lymphoma. Complete responses were seen in 95.2% of the patients treated. No relapses were observed at a median follow-up of 43.9 months. Eight-year overall survival and disease-free survival were 95.2% and 100.0%, respectively., Conclusion: These data indicate that the combined chemotherapy plus rituximab treatment may represent a suitable front-line approach in PBL, with a high rate of responses and an excellent long-term survival., (Copyright © 2011. Published by Elsevier Inc.)

Objective: To draw attention to complications that might arise in any Kawasaki disease (KD) stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae., Methods: Prospective study (clinical cohort) conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied., Results: Twenty-five patients (21.7%) had coronary aneurisms. Thirty eight patients (33%) had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3%) maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9%), ataxia in acute and subacute phases in 11 (9.5%); 15 patients had ophthalmologic complications (13.2%), with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9%), with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9%) and another patient had a necrotizing vasculitis progressing to peripheral gangrene and tongue tip loss (0.9%). Behavioral changes over convalescence were observed in 23 children., Conclusion: KD may progress with several complications even within months of the disease acute phase, eventually resulting in permanent sequelae. The earlier the diagnosis and therapeutic intervention with IV IgG administration are, the lower will be the occurrence of complications; the presence of thrombocytosis, anemia and elevated and extended inflammatory activity are risk factors for complication arising.

The research reported here attempted to answer the question, "is the foam important in delivering coacervates from shampoos." In order to answer this question, we have measured the amount of polymer in the foam and in the liquid phases of several cationic polymer/anionic surfactant systems by gravimetry and by FTIR techniques. In all cases studied, we discovered that the concentration of solids and, especially the polymer, in the liquid phase and in the foam phase were essentially the same. We conclude that the foam is unlikely to be an important factor in the topical delivery of polymer/surfactant coacervates.

Background: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology in which the main complication is coronary arteritis. Sensorineural hearing loss (SNHL) has also been described as a complication of this disorder. The objective of this study was to evaluate the prevalence of SNHL in patients with KD and to determine the possible causative abnormality, correlating the findings with the prevalence of cardiac complications, with the use of gammaglobulin, and with the results of laboratory tests., Methods: A clinical cohort of 40 patients with KD seen between 2005 and 2007 was evaluated by acoustic immittance measurement and brainstem-evoked response audiometry that were performed within the first 30 days of disease and 6 months after the first evaluation. Patients with hearing disability after this period underwent further testing through otoacoustic emissions analysis. All patients were treated with intravenous administration of immunoglobulin., Results: Ten (25%) of the 40 patients developed coronary aneurysm, and 22 (55%) disclosed hearing loss within the first 30 days, although this problem was noticed by the parents in only 3 children. In 12 (30%), the hearing loss persisted after 6 months. Ten (83.3%) of these 12 patients also showed thrombocytosis (platelets, >500,000), 8 (66%) had anemia (hemoglobin, <10 mg/dL), and all had high erythrocyte sedimentation rate (>50 mm/hr) persisting more than 30 days., Conclusion: SNHL was more prevalent than coronary complications. A significant association was found between persistent SNHL, although often not initially noted, with a prolonged presence of thrombocytosis, anemia, and high erythrocyte sedimentation rate and the delayed use of intravenous administration of immunoglobulin after the first 10 days of illness.

Background: Based on historical data on the role of radioimmunotherapy (RIT) in pretreated non-Hodgkin lymphoma, we reviewed our hospital's clinical database., Patients and Methods: Between 2005 and 2008, 57 patients previously treated with at least 1 rituximab-containing chemotherapy were treated with Yttrium-90-labeled ibritumomab tiuxetan ((90)Y-IT). The median number of pretreatments was 3 (range, 1-9 pretreatments). A total of 46 patients had stage III/IV disease (31 with bone marrow involvement); 6 had bulky disease. According to histology, 53 were follicular lymphoma (FL), 2 were marginal zone lymphoma, and 2 were small lymphocytic lymphoma., Results: Overall response rate was 93% (53 of 57); complete response (CR) rate was 70% (40 of 57). Twenty-six of 40 patients (65%) who obtained a CR are in continuous CR (CCR) with a median follow-up of 20 months (range, 10-42 months); 4 of them still maintain their CCR after 36 months. All patients achieving a CCR had FL, and 21 of them with stage III/IV disease; 12 of 26 had been heavily pretreated (>or= 3 previous treatments), and 2 had had autologous stem cell transplantation. Toxicity was primarily hematologic and mostly transient; no grade 4 extrahematologic toxicity was observed., Conclusion: This study confirms the safety and high efficacy of (90)Y-IT RIT in heavily pretreated FL patients, with the possibility of having a subset of long-term responders.

Context: Several studies have demonstrated a higher prevalence of celiac disease (CD) among females with Turner syndrome when compared to the general population. Nevertheless, there is no record in literature concerning this investigation among Brazilian patients., Objective: To assess the prevalence of CD among a group of Brazilian patients with Turner syndrome., Methods: Fifty-six females with Turner syndrome and on gluten-containing diet were screened for CD utilizing immunoglobulin A antiendomysium (IgA-EMA) and immunoglobulin A anti-tissue transglutaminase (IgA-tTG) antibody assays. Additionally, they were genotyped for CD human leukocyte antigen (CD-HLA) predisposing alleles. Patients showing positivity in serological testing were offered to perform small intestine biopsy for histological confirmation., Results: Mean age at diagnosis of Turner syndrome was 5.5 ± 4.4 years; mean age at screening for CD was 17.0 ± 9.3 years (from 10 months of age to 52 years). Two girls were positive for IgA-EMA and IgA-tTG, presented predisposing HLA-DQ2 alleles and both had the diagnosis of CD confirmed by jejunal biopsy., Conclusion: The 3.6% prevalence of biopsy-proven CD among this group of females with Turner syndrome is 10 times higher than the one among females from the general population of the same geographical area. This result provides additional support to an association between these two disorders and restates that girls and women with Turner syndrome represent a high risk population for developing CD.

Context: Celiac disease susceptibility has been shown to be associated with the HLA alleles DQA1*0501 and DQB1*0201 (together encoding the DQ2 heterodimer) that are present in practically all celiac disease patients. The DQ8 heterodimer (coded by DQA1*03-DQB1*0302), which is carried on a DRB1*04 (DR4) haplotype, is commonly encoded for by the few celiacs who do not carry the DQ2 heterodimer. Only a few celiac disease patients have been reported without these known risk alleles., Objective: To assess the prevalence of celiac disease in a group of first degree relatives of celiac patients, and the frequency of HLA predisposing alleles both in the group of celiac patients and in their first degree relatives, identifying those first degree relatives who would need further screening for celiac disease., Methods: Ninety celiac disease patients and 207 first degree relatives underwent serologic screening for celiac disease (endomysial and transglutaminase antibodies) followed by intestinal biopsy in positive patients. The HLA-DQA1*0501, DQB1*0201 and DRB1*04 frequencies of celiac patients and their first degree relatives were determined utilizing the PCR method., Results: All the celiac disease patients (n = 90) with the exception of one (1.1%) carried at least one of the alleles investigated. Altogether 11 (5.3%) of the investigated first degree relatives did not carry any of the alleles studied. Fourteen (6.7%) new cases of celiac disease were found among the 207 celiac disease patients first degree relatives., Conclusions: Considering the cost-benefit of the HLA typing of all the first degree relatives of celiac patients, their HLA status should be decided on an individual basis, taking account of their profile and preferences, and the existence of other medical conditions.

Background: Adult Langerhans cell histiocytosis (LCH) is a rare disease. The combination of vinblastine and prednisone, given in a 6-month course, is the standard of care but prospective randomized trials are lacking., Patients and Methods: We report our monocentric experience in the treatment of seven adult patients with multisystem (MS) LCH (n = 3) or single-system multifocal (SS-m) LCH (n = 4) with the short-course intensive chemotherapy regimen methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone and bleomicin (MACOP-B)., Results: The overall response rate was 100% [five complete response (CR), two partial response (PR)]. After a median follow-up of 6.5 years, four patients are in first continuous CR and three patients relapsed after 5, 8 and 62 months, respectively. Four patients were evaluated with positron emission tomography (PET) scan: all three PET-negative patients at the end of treatment had a long-lasting response with only one patient relapsing after 5 years. PET scan detected additional bone lesions at diagnosis in two of four patients, changing the treatment program in one of them., Conclusions: MACOP-B regimen seems to be very active in the treatment of adult MS or SS-m LCH, with long-lasting responses in five of seven patients. PET scan merits further evaluation in the initial staging and in the evaluation of the response to chemotherapy.

The objective of the present study was to evaluate the integrity of the peripheral and central auditory systems of sickle cell disease (SCD) patients, through electrophysiological evaluation utilizing auditory evoked potentials, and comparing the results obtained in SCD patients with individuals without SCD. A total of 80 individuals were evaluated: 40 SCD patients; and 40 healthy age- and sex-matched controls. Brainstem auditory evoked response (BAER) was used to check neural integrity and electrophysiological thresholds, and cognitive potential (P300) to analyse the auditory selective attention. Despite the exclusion of individuals with comorbidities typical of SCD, the predominance of hearing loss among the patients was detected in 16 ears (20%). The absolute latencies of the BAER were within the expected range but the SCD group showed a small but statistically significant reduction of the interpeaks I-V, indicative of cochlear alteration. P300 latency and amplitude were adequate for both groups suggesting the absence of central auditory system abnormalities. The present findings suggest that SCD causes variable degree of cochlear abnormalities without evidence of neural problems.

Background: Peripheral T-cell lymphoma unspecified (PTCLU) and mycosis fungoides (MF) often show resistance to conventional chemotherapy. Gemcitabine should be considered a suitable option. We report the long-term update of 39 pretreated T-cell lymphoma patients treated with gemcitabine., Patients and Methods: From May 1997 to September 2007, 39 pretreated MF and PTCLU patients received gemcitabine. Inclusion criteria were as follows: histologic diagnosis of MF or PTCLU; relapsed/refractory disease; age > or =18 years; and World Health Organization performance status of two or less. Nineteen patients had MF and 20 PTCLU. All patients with MF had a T3-T4, N0, and M0 disease and patients with PTCLU had stage III-IV disease. Gemcitabine was given on days 1, 8, and 15 on a 28-day schedule (1200 mg/m(2)/day) for a total of three to six cycles., Results: Overall response rate was 51% (20 of 39 patients); complete response (CR) and partial response (PR) rates were 23% (9 of 39 patients) and 28% (11 of 39 patients), respectively. Patients with MF had a CR rate of 16% and a PR rate of 32% compared with a CR rate of 30% and a PR rate of 25% of PTCLU patients. Among the CR patients, 7 of 9 are in continuous complete response with a variable disease-free interval (15-120 months)., Conclusion: In our experience, gemcitabine proved to be effective in pretreated MF and PTCLU patients, even in the long term.

Background: Animals in captivity should receive adequate sunlight exposure for sufficient generation of vitamin D [25(OH)D]. In the present study, 25(OH)D serum levels of 84 Callithrix penicillata primates were evaluated., Objectives: To determine 25(OH)D levels of those animals; to evaluate the influence of gender and period of sunlight exposure on their 25(OH)D levels., Methods: Three groups were evaluated: group 1 (n = 29) on free sunlight exposure; group 2 (n = 34) on partial sunlight exposure; group 3 (n = 21) without sunlight exposure., Results: The obtained 25(OH)D values were: group 1, 121.2 +/- 33.3 ng/ml; group 2, 115.2 +/- 32.2 ng/ml; group 3, 53.3 +/- 10.4 ng/ml. Significant statistical differences were obtained between groups 1 and 3 (p < 0.001) and groups 2 and 3 (p < 0.001); no statistical difference was found between genders., Conclusion: Direct sunlight exposure is essential for 25(OH)D sufficiency and it is proposed that the 25(OH)D normal range for captive Callithrix penicillata would be from 104.8 to 137.1 ng/ml (CI = 95%).

Context: The correct diagnosis of celiac disease in environmentally deprived children is frequently hindered by the common presence of other causes for the classical celiac disease symptoms: malnutrition, failure to thrive and frequent diarrheas., Objectives: To determine the prevalence of celiac disease in a group of 12 to 36 month-old children using immunoglobulin antibodies against gliadin (IgG and IgA-AGA), against endomysium (IgA-EMA), and against human tissue transglutaminase (IgA-tTG) as screening method., Methods: A total of 214 children (114 boys), aged 12 to 36 months, on gluten-containing diet, were admitted to the study. IgG and IgA-AGA, IgA-tTG and IgA-EMA tests were performed in all sera. Biopsy was obtained from all children showing positive result in one or more of the serologic tests, excluding those in which IgG-AGA had been the only positive result. In those cases, polymerase chain reaction (PCR) HLA genotyping for the identification of celiac disease predisposing alleles was applied. HLA genotyping was also performed to confirm the diagnosis in children identified as celiac by means of positive serologic testing and compatible biopsy results., Results: Normal results were obtained in 131 children. Ten children out of 68 identified as positive exclusively on the IgG-AGA test disclosed the presence of celiac disease predisposing alleles on PCR and underwent jejunal biopsy with normal results. All serologic tests were positive in four children. A fifth child showed positive IgG and IgA-AGA and IgA-tTG results but disclosed a negative IgA-EMA test. Jejunal biopsy of these five children revealed characteristic lesions of celiac disease., Conclusion: A prevalence of 2.3% was found among symptomatic 12- to 36-month-old children that had not been previously diagnosed as celiac.