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BACKGROUND: A low prevalence of HIV in sickle cell disease (SCD) patients has been reported in the literature though mechanisms for this are not understood. METHODS: HIV risk behaviors were compared between SCD cases and non-SCD controls using a self-administered audio computer-assisted self-interview. SCD cases were recruited from a multi-center SCD cohort established in Brazil; controls were recruited from SCD social contacts. Categorical variables were analyzed using Chi-Square or Fisher exact test. Continuous variables were compared using the Mann-Whitney U test. RESULTS: There were 152 SCD cases and 154 age/location matched controls enrolled at three participating Brazilian centers during 2016-17. No significant differences in number of sexual partners (lifetime or previous 12 months), male-to-male sex partners or intravenous drug use were observed. Cases received more transfusions, surgeries, and acupuncture treatment. CONCLUSIONS: Besides the risk of transfusion-transmitted HIV, which is now exceedingly rare, SCD and non-SCD participants demonstrated similar HIV risk behaviors. Causes other than risk behaviors such as factors inherent to SCD pathophysiology may explain the reported low prevalence of HIV in SCD.

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BACKGROUND: Reducing risk of HIV window period transmission requires understanding of donor knowledge and attitudes related to HIV and risk factors. STUDY DESIGN AND METHODS: We conducted a survey of 7635 presenting blood donors at three Brazilian blood centres from 15 October through 20 November 2009. Participants completed a questionnaire on HIV knowledge and attitudes about blood donation. Six questions about blood testing and HIV were evaluated using maximum likelihood chi-square and logistic regression. Test seeking was classified in non-overlapping categories according to answers to one direct and two indirect questions. RESULTS: Overall, respondents were male (64%) repeat donors (67%) between 18 and 49 years old (91%). Nearly 60% believed blood centres use better HIV tests than other places; however, 42% were unaware of the HIV window period. Approximately 50% believed it was appropriate to donate to be tested for HIV, but 67% said it was not acceptable to donate with risk factors even if blood is tested. Logistic regression found that less education, Hemope-Recife blood centre, replacement, potential and self-disclosed test-seeking were associated with less HIV knowledge. CONCLUSION: HIV knowledge related to blood safety remains low among Brazilian blood donors. A subset finds it appropriate to be tested at blood centres and may be unaware of the HIV window period. These donations may impose a significant risk to the safety of the blood supply. Decreasing test-seeking and changing beliefs about the appropriateness of individuals with behavioural risk factors donating blood could reduce the risk of transfusing an infectious unit.

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Introduction: Diabetes insipidus (DI) is a syndrome characterized by polyuria and polydipsia secondary to a decreased secretion or action of the antidiuretic hormone (ADH). An early diagnosis is essential. Diagnosis is made by measuring plasma and urinary osmolarity and their changes under water deprivation and after DDAVP administration. Objective: Lo describe the clinical, radiological characteristics as well as the initial treatment of eight children with DI, 3 of them nephrogenic DI (DIN) and 5 with central DI. Methods: A Retrospective, descriptive study in DI patients under control at the Catholic University of Chile and Sotero del Rio Hospital between 1998-2008 is presented. Clinical files were evaluated collecting clinical, epidemiologic, biochemical and image data. Serum (Sosm) and urinary osmolarity (Uosm) were registered. DI was diagnosed with a Sosm > 300 and Usm < 600 mOsm/L. Central DI was defined as the inability to reach a Uosm > 600 or a 50 percent-increase after DDAVP treatment. Otherwise DI was classified as DIN. Results: Eight patients (5 males) were studied. Chief complaints were polydipsia/polyuria (5/8), hyperthermia (2/8), and failure to grow (1/8). MRI showed endocraneal lesion in all patients with Central DI. All of these utilized oral or inhalatory DDAVP treatment. Patients with Nephrogenic DI were trated with Hydrochlrothiazide. Conclusion: Polydipsia, polyuria, hyperthermia with hypernatremia are suggestive of DI in the first year of life. Water deprivation test is diagnostic in differentiating Central and Nephrogenic DI. MRI is an essential diagnostic tool in CDI. Manegement should be multidisciplinary, including a pediatician, nephrologist, endocrinologist and nutricionist.

Introducción: La diabetes insípida (DI) se caracteriza por poliuria y polidipsia, secundario a una disminución de la secreción o acción de la hormona antidiurética. Su diagnóstico precoz es fundamental. Objetivo: Describir las características clínicas, radiológicas y tratamiento inicial de una serie de ocho pacientes con DI. Diseño: Estudio descriptivo-restrospectivo. Universo: Pacientes con DI evaluados en la Universidad Católica de Chile y Hospital Dr. Sótero del Río entre 1998-2008. Pacientes y Métodos: Desde la ficha clínica se analizaron variables clínicas, epidemiológicas, bioquímicas e imágenes. Se determinó Osmolaridad sérica (OsmS) y urinaria (OsmU). Se consideró DI sí la OsmS > 300 mOsm con OsmU < 600 mOsm, Di-central (DIC) sí posterior a DDAVP la OsmU aumento > 50 por ciento ó > 600 mOsm, de los contrario se clasificó como nefrogénica (DIN). Resultados: Se reclutaron ocho pacientes con DI (5 varones), fueron DIN 3/8. El motivo de consulta fue: polidipsia-poliuria (5/8), hipertermia (2/8) y talla baja (1/8). La RNM mostró lesión intracraneana en todos los pacientes con DIC: nodulo hipofisiario, aracnoidocele selar, Histiocitosis X, germinoma y un paciente sin se±al de neurohipófisis. Los sujetos con DIC usaron DDAVP inhalatoria (4) y oral (1). Los sujetos con DIN usaron hidroclorotiazida. Conclusión: Polidipsia, poliuria, hipertermia con hipernatremia y falla de medro en lactantes son sugerentes de DI. La prueba de deprivación hídrica es fundamental en la diferenciación de DIC y DIN. La RNM cerebral es una herramienta diagnóstica imprescindible en la DIC. El tratamiento de estos pacientes debe ser multidiciplinario interactuando pediatra, nefrólogo, endocrinólogo y nutricionista.

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Introducción: Aproximadamente el 50 por ciento de los pacientes con Enfermedad de Crohn (EC) necesitarán de un tratamiento quirúrgico en algún momento de su evolución. La resección ileocecal (RIC) es una de las operaciones más frecuentes en pacientes con EC. Objetivo: Identificar las indicaciones quirúrgicas y determinar el porcentaje de recurrencia de la enfermedad a largo plazo de los pacientes sometidos a RIC por EC. Material y método: Se incluyeron todos los pacientes sometidos consecutivamente a RIC entre Enero 1970 y Diciembre 2006 y se analizaron características demográficas, indicación operatoria, variables intraoperatorias, complicaciones y, en el seguimiento, la recurrencia de la enfermedad. Resultados: 28 pacientes fueron operados en el periodo mencionado. 17 mujeres (60,7 por ciento), edad promedio del diagnóstico de EC: 34,8 años (i: 14-60) y de la RIC: 43,3 años (i: 16-68). Seis pacientes habían sido operados previamente por EC (3 sobre el periné y 3 resecciones parciales de intestino, no RIC). Una o más de las siguientes condiciones contribuyeron a la indicación quirúrgica: Obstrucción intestinal intermitente en 21 pacientes, refractariedad a tratamiento médico en 10, fístula enteral en 2 y hemorragia digestiva baja en 2. Nueve pacientes (32,1 por ciento) tuvieron una o más complicaciones postoperatorias, 3 (10 por ciento) de los cuales fueron reintervenidos (2 por filtración de la anastomosis, uno por hemoperitoneo). La mediana de estadía postoperatoria fue 9 días. No hubo mortalidad operatoria. En el seguimiento a largo plazo, 3 pacientes desarrollaron Íleo mecánico por bridas. Todos ellos resueltos quirúrgicamente. Cuatro pacientes (14 por ciento) fueron reintervenidos por recidiva de la EC con tiempo medio desde la RIC de 63 meses. La sobrevida a 5 años fue de 96 por ciento. Conclusión: La RIC por EC, se indica principalmente por obstrucción intestinal debida a estenosis. La estenosis en la recidiva de la enfermedad es baja.

Background: Approximately 50 percent of patients with Crohn's disease (CD) will need a surgical treatment in the long term course of the disease. The ileocaecal resection (ICR) is the most common operations in patients with CD. Aim: To identify the surgical indications and to determine the rate of recurrence in late follow up of the patients who underwent ICR for CD. Material and method: Consecutive inclusion of patients submitted to ICR between 1970 and 2006. Demographic characteristics, surgical indications, intraoperative findings, postoperative complications and recurrence of the disease were analyzed. Results: 28 patients had an ICR inthisperiod. Average age at diagnosis of CD 34.8 years (r: 14-60), ICR 43.3 years (r: 16-68). 60.7 percent female gender. Six patients were previously operated for CD (3 over perine and 3 partial resection of small bowel, no ICR). One or more of the following clinical conditions were considered surgical indications: Intermittent intestinal obstruction in 21 patients, failure of medical treatment in 10, enteral fistulae 2 and lower intestinal bleeding 2. Nine patients (32.1 percent) had one or more postoperative complications. 3 (10 percent) of them were re-operated (2 for leakage and fistulae, one for hemoperitoneum). The median postoperative stay was 9 days. There was no postoperative mortality (30 days). In the long term follow up, 3 (10 percent) patients developed intestinal obstruction due to adhesions. All of them needed an operation. Four patients (14 percent) were reoperated for CD with a mean time of 63 months since the ICR. Two of them for stenosis of ileocolic anastomosis and two for perianal abscess and fistulae. The 5 year survival was 96 percent. Conclusion: Stenosis and intestinal obstruction of the terminal ileum is the main indication for ICR.

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Objetivo: Comparar resultados y complicaciones inmediatas al realizar una sigmoidectomía laparoscópica (SL) versus abierta (SA) en pacientes con cáncer de colon sigmoides. Material y método: Inclusión prospectiva y consecutiva, mediante protocolo de estudio de pacientes operados mediante SL entre el 2000 y el 2006. Análisis comparativo caso-control, mediante pareo según edad, sexo y ASA entre pacientes operados mediante SL con SA. Análisis por intención de tratamiento, mediante test t de Student, chi cuadrado, Mann-Withney y exacto de Fischer; consideramos estadísticamente significativo p <0,05. Resultados: Treinta y dos pacientes por grupo fueron comparados. Los grupos (SL v/s SA) fueron similares estadísticamente en edad (65,6 v/s 67,3 años), sexo (femenino 31,25 por ciento), y ASA (p> 0,05). La mediana del tiempo operatorio fue mayor en SL (220 v/s 172 minutos, p <0,01). Un 9,3 por ciento se convirtió a técnica abierta en SL. El tiempo medio a la expulsión de gases y realimentación con líquidos fue menor en SL (2 v/s 4 días, p<0,01 y 3 v/s 5 días, p< 0,01). La estadía media postquirúrgica (5 v/s 8 días p<0,01) fue menor en SL. Las complicaciones postoperatorias médicas, fueron menores en SL (9,3 por ciento v/s 37,5 por ciento p<0,01), pero las quirúrgicas fueron iguales (6,25 por ciento cada grupo). Los linfonodos resecados y tamaños de piezas operatorias fueron similares (SL v/s SA: 22,7 v/s 22,1 linfonodos p= 0,9 y 21,8 v/s 19,3 cm. p= 0,2). Conclusión: El desarrollo de la cirugía laparoscópica colorrectal mediante un protocolo, permite realizar la SL por cáncer con resultados quirúrgicos similares a la SA.

Background: The laparoscopic surgery for colorectal cancer is an alternative to the laparotomy. The aim of this study is to compare results and early complications after a laparoscopic sigmoidectomy (LS) versus open surgery (OS) in patients with sigmoid cancer. Material and Method: Prospective and consecutive inclusion, by a study protocol of patients operated on for LS between 2000 and 2006. Comparative case-control design, the LS group was match with the OS group by age, sex, and ASA classification. Statistical analysis: Results were analyzed with intention to treat. The variables were analyzed with T Student, Chi-square, Mann-Withney and Fischer exact test, considering statistically significant a P value < 0.05. Results: Thirty-two patients by group were compared. The groups (LS vs OS) were statistically similar in age (65.6 v/s 67.3 years), gender (31.25 percent females each group), and ASA (p > 0.5). The mean operative time was higher in LS group (220 v/s 172 minutes, p <0.01). Conversion rate was 9.3 percent. The mean time to passing flatus and the time to reinitiate oral feeding were both shorter for LS group (2 v/s 4 days, p<0.01; and 3 v/s 5 days p <0.01). The mean total postoperative stay (5 v/s 8 days, p<0.01) was shorter for LS group. The postoperative medical complications were minor in LS group (9.3 v/s 27.5 percent, p <0.01), but the surgical complications were similar (6.25 percent each group). Lymph nodes resected and length of surgical specimens were similar for both groups (SL v/s OS: 22.7 v/s 22.1 lymphatic nodes p = 0.9 and 21.8 v/s 19.3 cm, p= 0.2). Conclusion: The development of laparoscopic colorectal surgery within a protocol, allows feasible the realization of laparoscopic sigmoidectomy for patients with sigmoid cancer with comparable surgical outcomes.

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The bottleneck for the complete understanding of the structure-function relationship of flexible membrane-acting peptides is its dynamics. At the same time, not only the structure but also the dynamics are the key points for their mechanism of action. Our model is PW2, a TRP-rich, cationic peptide selected from phage display libraries that shows anticoccidial activity against Eimeria acervulina. In this manuscript we used a combination of several NMR techniques to tackle these difficulties. The structural features of the membrane-acting peptide PW2 was studied in several membrane mimetic environments: we compared the structural features of PW2 in SDS and DPC micelles, that were reported earlier, with the structure properties in different lipid vesicles and the peptide free in water. We were able to unify the structural information obtained in each of these systems. The structural constraints of the peptide free in water were fundamental for the understanding of plasticity necessary for the membrane interaction. Our data suggested that the WWR sequence is the region responsible for anchoring the peptide to the interfaces, and that this same region displays some degree of conformational order in solution. For PW2, we found that affinity is related to the aromatic region, by anchoring the peptide to the membrane, and specificity is related to the N- and C-termini, which are able to accommodate in the membrane due to its plasticity.

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We studied the transmission routes of human T-cell lymphotropic virus type I (HTLV-I) within families of 82 Brazilian patients diagnosed with adult T-cell leukaemia/lymphoma (ATL). Diagnosis of ATL in 43 male and 39 female patients was based on clinical and laboratory criteria of T-cell malignancy and detection of HTLV-I monoclonal integration. Samples were tested for HTLV antibodies and infection was confirmed as HTLV-I by Western Blot and/or polymerase chain reaction (PCR) assays. Overall 26/37 (70%) of mothers, 24/37 (65%) of wives, 8/22 (36%) of husbands, 34/112 (30%) of siblings and 10/82 (12%) offspring were HTLV-I infected. In 11 ATL patients, mothers were repeatedly HTLV-I seronegative, but HTLV-I pol or tax sequences were detected in 2 out of 6 cases tested by PCR. ATL patients with seronegative mothers related the following risk factors for HTLV-I infection: 6 were breast-fed by surrogate mothers with unknown HTLV-I status, 4 had a sexually promiscuous behaviour and 1 had multiple blood transfusions at a young age. Familial aggregation of ATL and other HTLV-I associated diseases such as HTLV-I myelopathy (HAM/TSP) and or uveitis, ATL in sibling pairs or in multiple generations was seen in 9 families. There were 6 families with ATL and TSP sibling pairs. In 3 families at least one parent had died with lymphoma or presenting neurological diseases and 2 offspring with ATL. Further to the extent of vertical and horizontal transmission of HTLV-I infection within ATL families, our results demonstrate that mothers who provide surrogate breast-milk appear to be an important source of HTLV-I transmission and ATL development in Brazil.

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OBJECTIVE - To report the results of percutaneous occlusion of persistent ductus arteriosus with the Amplatzer prosthesis in 2 Brazilian cardiological centers. METHODS - From May 1998 to July 2000, 33 patients with clinical and laboratory diagnosis of persistent ductus arteriosus underwent attempts at percutaneous implantation of the Amplatzer prosthesis. The median age was 36 months (from 6 months to 38 years), and the median weight was 14kg (from 6 to 92kg). Sixteen patients (48.5%) were under 2 years of age at the time of the procedure. All patients were followed up with periodical clinical and echocardiographic evaluations to assess the presence and degree of residual shunt and possible complications, such as pseudocoarctation of the aorta and left pulmonary artery stenosis. RESULTS - The minimum diameter of the arterial ducts ranged from 2.5 to 7.0mm (mean of 4.0+/-1.0, median of 3.9). The rate of success for implantation of the prosthesis was 100%. Femoral pulse was lost in 1 patient. The echocardiogram revealed total closure prior to hospital discharge in 30 patients, and in the follow-up visit 3 months later in the 3 remaining patients. The mean follow-up duration was 6.4+/-3.4 months. All patients were clinically well, asymptomatic, and did not need medication. No patient had narrowing of the left pulmonary artery or of the aorta. No early or late embolic events occurred, nor did infectious endarteritis. A new hospital admission was not required for any patient. CONCLUSION - The Amplatzer prosthesis for persistent ductus arteriosus is safe and highly effective for occlusion of ductus arteriosus of varied diameters, including large ones in small symptomatic infants.

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We present a rare case of adult T cell leukemia/lymphoma (ATL) in which leukemic T cells expressed CD4 and CD25 surface antigens and infiltrated mammary glands during clinical course of the disease. A 40-year-old male was admitted with long-standing skin lesions and leukocytosis. Peripheral blood lymphocytes were highly pleomorphic and presented CD2, CD4, CD25, CD38 membrane surface antigens. The patient proved to be seropositive for human T-cell lymphotropic virus type I (HTLV-I) antibodies. Monoclonal expansion of lymphoid cells integrated with HTLV-I genome was observed, and the diagnosis of ATL chronic type was made. He underwent a chemotherapy regimen, and skin lesions and leukocytosis improved markedly. He progressed with an indolent clinical course of ATL, when he was admitted with bilateral hyperplasia of breast, recurrent skin lesions, and leukocytosis. Breast biopsy revealed bilateral gynecomasty, extensive leukemic infiltration of typical ATL cells in the mammary glands, and the presence of mammary epithelial cells productively infected with HTLV-I. This is the first report describing invasion of the mammary tissue with HTLV-I-transformed T-cells and HTLV-I-associated breast disease.

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We describe 195 cases of adult T-cell leukemia/lymphoma (ATLL) reported to the national registry of T-cell malignancies in Brazil between 1994 and 1998. We compared the effect of demographic differences and clinical features of 150 consecutive ATLL cases in different regions of this diverse country. At diagnosis, the predominant clinical sub-type was the acute type (60%), followed by lymphoma (22%), chronic (10%) and smoldering (8%) types. Although we expected that different sub-types would be present in different regions, on the basis of immunogenetic factors determined by ethnicity, we did not demonstrate these differences. There were no significant differences among ATLL subtypes by age or gender. No ethnic group predominated in the total population of patients, but significant differences were noted when examining ethnic distribution by region. Reflecting the general population distribution, white patients were seen more often in São Paulo and black patients in Bahia, than in other regions. In most regions, cases were equally distributed between blacks and mulattos, except in Pernambuco, where blacks were less frequent. The main clinical features were lymphadenopathy, skin lesions, hypercalcemia and hepatomegaly. Fourteen patients (9%) suffered from HTLV-I-associated myelopathy (HAM/TSP), either at diagnosis or during follow-up of ATLL. All cases but one had antibodies to HTLV-I, with concordant results with ELISA, WB and PCR analyses. For the antibody-negative case, pol and tax gene sequences were present in tumor cells when subjected to PCR analyses. The prognosis was generally poor, suggesting that the disease in Brazil behaves in similar fashion regardless of ethnic or geographical differences.

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Human T-cell lymphotropic Virus Type I (HTLV-I) is the etiologic factor for adult T-cell leukemia/lymphoma (ATL). HTLV-I infection can also lead to other diseases, such as HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP), uveitis, arthropathy and infectious dermatitis. Studies of the infectious mode of transmission of HTLV-I and risk factors for HTLV-I-related diseases have been conducted in several countries, and differences in the prevalence, age patterns, ethnic groups and clinical presentation of the related diseases have been described worldwide. Based on the geographical characteristics of Brazil and data from the literature, we have summarized the distribution of seroprevalence in blood donors in different states around the country, as well as the incidence of ATL in regards to the endemic foci. ATL in Brazil has the same characteristics as those described elsewhere, but is reported more frequently at a younger age. In order to better evaluate ATL in Brazil, a registry has been established at the several hematologic centers under the sponsorship of the instituto Nacional de Cancer and the Brazilian Society of Hematology and Hemotherapy, for the purpose of recording all cases originally diagnosed in Brazil.

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Molecular studies have demonstrated the existence of at least two major subtypes of human T-cell lymphotropic virus type 2 (HTLV-2), designated HTLV-2a and HTLV-2b. To further investigate the heterogeneity of this family of viruses, we have characterized the HTLV-2 subtypes present in several urban areas in Brazil. DNAs from peripheral blood mononuclear cells of a large number of infected individuals, the majority of whom were intravenous drug abusers, were analyzed by using PCR with restriction fragment length polymorphism and nucleotide sequencing analysis. Restriction fragment length polymorphism analysis of the env region suggested that all individuals were infected with the HTLV-2a subtype, and this was confirmed by nucleotide sequence analysis. In contrast, nucleotide sequence analysis of the long terminal repeat demonstrated that although the viruses were more related to the HTLV-2a than to the HTLV-2b subtype, they clustered in a distinct phylogenetic group, suggesting that they may represent a new and distinct molecular subtype of HTLV-2. This conclusion was supported by nucleotide sequence analysis of the pX region, which demonstrated that the Tax proteins of the Brazilian viruses differed from that of prototype HTLV-2a isolates but were more similar to that of HTLV-2b in that they would be expected to have an additional 25 amino acids at the carboxy terminus. In transient expression assays, the extended Tax protein of the prototype HTLV-2a subtype. The studies suggest that the Brazilian viruses analyzed in this study, while being phylogenetically related to the prototypic HTLV-2a seen in North America, are phenotypically more related to HTLV-2b and can be justifiably classified as a new molecular subtype, which has been tentatively designated HTLV-2c.

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T-cell malignancies in Brazil have a high seroprevalence rate of HTLV-I antibodies. We have analyzed the disease features in 188 Brazilian patients with a T-cell disorder. These included 40 with T-lymphoblastic leukaemia or lymphoma (T-ALL/T-LbLy) and 148 with mature T-cell diseases: 5 T-prolymphocytic leukaemia, 53 adult T-cell leukaemia/lymphoma (ATLL), 54 cutaneous T-cell lymphomas, 29 pleomorphic T-cell lymphomas and 7 large granular lymphocyte leukaemia. The diagnosis was based on clinical, morphological and immunological features and HTLV-I serology. ATLL in Brazil has the same diseases features as in other endemic regions, the only apparent differences being: age, Brazilian patients being younger than Japanese, and ethnic grouping, one third of Brazilians being white Caucasians of European descent. We applied a scoring system based on the presence or absence of typical features associated with ATLL; hypercalcaemia, cell morphology, immunophenotype, histopathology and HTLV-I status, to see whether it may help in diagnosing cases of ATLL. All had high scores, whereas all other T-cell diseases scored low. Only 5 ATLL cases were HTLV-I-negative by serology, but they had otherwise typical features of ATLL, and their cells did not have HTLV-I proviral sequences by DNA analysis. Such cases suggest that ATLL may develop in a minority of individuals living in regions where it is endemic, without evidence of HTLV-I infection, and that other factors may contribute to the pathogenesis of the disease.

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