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Objective The aim of this study was to describe the prevalence of syphilis by geographical region and the frequency of behaviours and symptoms related to sexually transmitted diseases (STD). Methods A cross-sectional study was performed with Brazilian conscripts in 2007. They answered a selfadministered questionnaire on demographic issues, sexual practices, condom use and STD symptoms. They had a blood sample collected for a syphilis test. Results A total of 35 460 conscripts answered the questionnaire and 75.5% reported previous sexual intercourse. Overall syphilis prevalence was 0.53% (95% CI 0.45% to 0.61%). By geographical region: northern (0.85%), northeast (0.82%), midwest (0.49%), southeast (0.34%) and southern region (0.26%). The final logistic regression model showed an association among having had syphilis and being 17 years old (OR 1.3; 95% CI 1.05 to 1.73), having up to 8 years of schooling (OR 1.5; 95% CI 1.03 to 2.22), living in the northern/ northeast region (OR 1.2; 95% CI 1.04 to 1.36), being men who have sex with men (OR 4.5; 95% CI 2.59 to 7.81), reporting a previous history of STD (OR 2.7; 95% CI 1.03 to 6.99) and genital ulcers (OR 2.6; 95% CI 1.59 to 4.26). Conclusions Addressing young people at the time of military enlistment may be a good time to consider new strategies for accessing and counselling this population, thus allowing the implementation of a more appropriate healthcare policy [ABSTRACT FROM AUTHOR]

Abstract: In this last decade, a deeper understanding of the pathophysiology of hereditary red cell disorders and the development of novel classes of pharmacologic agents have provided novel therapeutic approaches to thalassemias, sickle cell disease (SCD), and other red cell disorders. Here, we analyze and discuss the novel therapeutic options according to their targets, taking into consideration the complex process of erythroid differentiation, maturation, and survival of erythrocytes in the peripheral circulation. We focus on active clinical exploratory and confirmatory trials on thalassemias, SCD, and other red cell disorders. Beside β-thalassemia and SCD, we found that the development of new therapeutic strategies has allowed for the design of clinic studies for hereditary red cell disorders still lacking valuable therapeutic alternative such as α-thalassemias, congenital dyserythropoietic anemia, or Diamond-Blackfan anemia. In addition, reduction of heme synthesis, which can be achieved by the repurposed antipsychotic drug bitopertin, might affect not only hematological disorders but multiorgan diseases such as erythropoietic protoporphyria. Finally, our review highlights the current state of therapeutic scenarios, in which multiple indications targeting different red cell disorders are being considered for a single agent. This is a welcome change that will hopefully expand therapeutic option for patients affected by thalassemias, SCD, and other red cell disorders., (© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.)

Haemochromatosis (HC) encompasses a range of genetic disorders. HFE-HC is by far the most common in adults, while non-HFE types are rare due to mutations of HJV, HAMP, TFR2 and gain-of-function mutations of SLC40A1. HC is often unknown to paediatricians as it is usually asymptomatic in childhood. We report clinical and biochemical data from 24 paediatric cases of HC (10 cases of HFE-, 5 TFR2-, 9 HJV-HC), with a median follow-up of 9.6 years. Unlike in the adult population, non-HFE-HC constitutes 58% (14/24) of the population in our series. Transferrin saturation was significantly higher in TFR2- and HJV-HC compared to HFE-HC, and serum ferritin and LIC were higher in HJV-HC compared to TFR2- and HFE-HC. Most HFE-HC subjects had relatively low ferritin and LIC at the time of diagnosis, so therapy could be postponed for most of them after the age of 18. Our results confirm that HJV-HC is a severe form already in childhood, emphasizing the importance of early diagnosis and treatment to avoid the development of organ damage and reduce morbidity and mortality. Although phlebotomies were tolerated by most patients, oral iron chelators could be a valid option in early-onset HC., (© 2023 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)

Background: Women living with human immunodeficiency virus (HIV) (WLWH) are more likely to be infected with the oncogenic human papillomavirus (HPV). We assessed the prevalence of high-risk (HR) (16/18/31/33/35/39/45/51/52/56/58/59/68/73/82), probable high-risk (pHR) (26/53/66), and low-risk (LR) (6/11/40/42/43/44/54/61/70) HPV types and their associated risk factors., Methods: This cross-sectional study of WLWH aged 18-64 years included one laboratory and eight HIV-specialty healthcare facilities in the pilot network. Descriptive statistics were used to assess sociodemographic and behavioral characteristics. Adjusted analyses were conducted to evaluate risk factors associated with HR and/or pHR HPV infection in WLWH., Results: From May/2021 to May/2022, 1,914 (92.5%) WLWH participated in the pilot study and had valid HPV-DNA results of self-collected vaginal samples. The median age of the participants was 45 years, 60.1% had ≥ 9 years of schooling, 80.5% were ≤ 18 years at first sexual intercourse, and 51.7% had > 4 sexual partners throughout life. The prevalence of any HPV type, HR HPV, pHR HPV, and LR HPV was 65.8%, 49.6%, 16.7%, and 40.0%, respectively. Age was inversely associated with pHR and/or HR-HPV (p < 0.001), and education level was inversely associated with HR-HPV (p = 0.003) types. Any HR or pHR was associated with being single (p = 0.029) and exchanging sex for drugs (p = 0.037)., Conclusions: The prevalence of HPV, especially HR HPV, among WLWH is high in Brazil, highlighting the need for HPV screening in this population. Self-collection of vaginal samples is an important strategy for increasing testing access.

Objective: to describe the frequency of underreporting of unfavorable outcomes of congenital syphilis in the state of São Paulo, Brazil, 2007-2018., Methods: this was a descriptive study of cases of abortion, fetal and non-fetal deaths due to congenital syphilis reported on the Notifiable Health Conditions Information System (Sistema de Informação de Agravos de Notificação - SINAN), and those of congenital syphilis registered in any line in the Death Certificate, on the Mortality Information System (Sistema de Informações sobre Mortalidade - SIM), by means of probabilistic and deterministic linkage., Results: of the 27,713 cases of congenital syphilis reported, 1,320 progressed to death (871 fetal deaths, 449 infant deaths) and were matched to the SIM; 355 deaths (259 fetal deaths, 96 infant deaths) were not included on SINAN; there was an increase in unfavorable outcomes,11.4% for infant deaths due to congenital syphilis, 3.0% for fetal deaths and 1.9% for abortions., Conclusion: the use of different relationship techniques proved to be adequate to identify the frequency of underreporting of unfavorable outcomes of congenital syphilis in the state of São Paulo.

Gain-of-function mutations in PIEZO1 cause dehydrated hereditary stomatocytosis (DHS) or hereditary xerocytosis, an autosomal dominant hemolytic anemia characterized by high reticulocyte count, a tendency to macrocytosis, and mild jaundice, as well as by other variably penetrant clinical features, such as perinatal edema, severe thromboembolic complications after splenectomy, and hepatic iron overload. PIEZO1 mutations in DHS lead to slowed inactivation kinetics of the ion channel and/or facilitation of channel opening in response to physiological stimuli. To characterize the alterations of red blood cell proteome in patients with mutated PIEZO1, we used a differential approach to compare the proteome of patients with DHS (16 patients from 13 unrelated ancestries) vs healthy individuals. We identified new components in the regulation of the complex landscape of erythrocytes ion and volume balance mediated by PIEZO1. Specifically, the main impaired processes in patients with DHS were ion homeostasis, transmembrane transport, regulation of vesicle-mediated transport, and the proteasomal catabolic process. Functional assays demonstrated coexpression of PIEZO1 and band 3 when PIEZO1 was activated. Moreover, the alteration of the vesicle-mediated transport was functionally demonstrated by an increased vesiculation rate in patients with DHS compared with healthy controls. This finding also provides an explanation of the pathogenetic mechanism underlying the increased thrombotic rate observed in these patients. Finally, the newly identified proteins, involved in the intracellular signaling pathways altered by PIEZO1 mutations, could be used in the future as potential druggable targets in DHS., (© 2023 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

The number of individuals with the sickle cell trait exceeds 300 million worldwide, making sickle cell disease one of the most common monogenetic diseases globally. Because of the high frequency of sickle cell disease, reproductive counseling is of crucial importance. In addition, unlike other carrier states, Sickle Cell Trait (SCT) seems to be a risk factor for several clinical complications, such as extreme exertional injury, chronic kidney disease, and complications during pregnancy and surgery. This expert panel believes that increasing knowledge about these clinical manifestations and their prevention and management can be a useful tool for all healthcare providers involved in this issue.

We report data on survival and complications for a longitudinal cohort of 709 transfusion-dependent β-thalassemia major patients (51.1% males) born between 1970 and 1997 and followed through 2020 at seven major centers in Italy. Overall survival probability at 30 years was 83.6% (95%CI: 78.5-89.1) in the oldest birth cohort (1970-1974) compared with 93.3% (95%CI: 88.6-98.3) in the youngest birth cohort (1985-1997) (p = 0.073). Females showed better survival than males (p = 0.022). There were a total of 93 deaths at a median age of 23.2 years with the most frequent disease-related causes being heart disease (n = 53), bone marrow transplant (BMT) complication (n = 10), infection (n = 8), liver disease (n = 4), cancer (n = 3), thromboembolism (n = 2) and severe anemia (n = 1). There was a steady decline in the number of deaths due to heart disease from the year 2000 onwards and no death from BMT was observed after the year 2010. A progressive decrease in the median age of BMT was observed in younger birth cohorts (p < 0.001). A total of 480 (67.7%) patients developed ≥1 complication. Patients in younger birth cohorts demonstrated better complication-free survival (p < 0.001) which was comparable between sexes (p = 0.230). Independent risk factors for death in multivariate analysis included heart disease (HR: 4.63, 95%CI: 1.78-12.1, p = 0.002), serum ferritin >1000 ng/mL (HR: 15.5, 95%CI: 3.52-68.2, p < 0.001), male sex (HR: 2.75, 95%CI: 0.89-8.45, p = 0.078), and splenectomy (HR: 6.97, 95%CI: 0.90-54.0, p < 0.063). Survival in patients with β-thalassemia major continues to improve with adequate access to care, best practice sharing, continued research, and collaboration between centers., (© 2023 Wiley Periodicals LLC.)

Background: The correlation between thalassemia and malignancies other than hepatocellular carcinoma (HCC) and the possible relationship between other hemoglobinopathies and tumor risk have been poorly evaluated., Methods: Eight Italian specialized centers evaluated the incidence of malignant neoplasms in hemoglobinopathies as well as their sites and features. The study cohort included 4631 patients followed between 1970 and 2021 (transfusion-dependent β-thalassemia, 55.6%; non-transfusion-dependent thalassemia, 17.7%; sickle cell disease, 17.6%; hemoglobin H disease, 8.3%)., Results: A total of 197 diagnoses of cancer were reported (incidence rate, 442 cases per 100,000 person-years). The liver was the most frequent site of tumors in both sexes, with a higher incidence (190 cases per 100,000 person-years) in comparison with the general population found in all types of hemoglobinopathies (except hemoglobin H disease). In recent years, tumors have become the second cause of death in patients with transfusion-dependent thalassemia. A lower risk of breast and prostate cancer was observed in the whole group of patients with hemoglobinopathies. The first cancer diagnoses dated back to the 1980s, and the incidence rate sharply increased after the 2000s. However, although the incidence rate of cancers of all sites but the liver continued to show an increasing trend, the incidence of HCC showed stability., Conclusions: These findings provide novel insights into the relationship between cancer and hemoglobinopathies and suggest that the overall risk is not increased in these patients. HCC has been confirmed as the most frequent tumor, but advances in chelation and the drugs that have led to the eradication of hepatitis C may explain the recent steadiness in the number of diagnoses that is reported here., (© 2022 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.)

Neurodegeneration with brain iron accumulation (NBIA) comprises various rare clinical entities with brain iron overload as a common feature. Magnetic resonance imaging (MRI) allows diagnosis of this condition, and genetic molecular testing can confirm the diagnosis to better understand the intracellular damage mechanism involved. NBIA groups disorders include: pantothenate kinase-associated neurodegeneration (PKAN), mutations in the gene encoding pantothenate kinase 2 (PANK2); neuroferritinopathy, mutations in the calcium-independent phospholipase A2 gene (PLA2G6); aceruloplasminemia; and other subtypes with no specific clinical or MRI specific patterns identified. There is no causal therapy, and only symptom treatments are available for this condition. Promising strategies include the use of deferiprone (DFP), an orally administered bidentate iron chelator with the ability to pass through the blood-brain barrier. This is a prospective study analysis with a mean follow-up time of 5.5 ± 2.3 years (min-max: 2.4-9.6 years) to define DFP (15 mg/kg bid)'s efficacy and safety in the continuous treatment of 10 NBIA patients through clinical and neuroradiological evaluation. Our results show the progressive decrease in the cerebral accumulation of iron evaluated by MRI and a substantial stability of the overall clinical neurological picture without a significant correlation between clinical and radiological findings. Complete ferrochelation throughout the day appears to be of fundamental importance considering that oxidative damage is generated, above, all by non-transferrin-bound iron (NTBI); thus, we hypothesize that a (TID) administration regimen of DFP might better apply its chelating properties over 24 h with the aim to also obtain clinical improvement beyond the neuroradiological improvement.

Although numerous patient-specific co-factors have been shown to be associated with worse outcomes in COVID-19, the prognostic value of thalassaemic syndromes in COVID-19 patients remains poorly understood. We studied the outcomes of 137 COVID-19 patients with a history of transfusion-dependent thalassaemia (TDT) and transfusion independent thalassaemia (TIT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID-19 patients with no history of thalassaemia. The mean age of thalassaemia patients included in our study was 41 ± 16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 40% of thalassaemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID-19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age-, sex- and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in-hospital complications (supplemental oxygen use, admission to an intensive care unit for CPAP therapy or intubation) and all-cause mortality was significantly lower in the thalassaemia group compared to the matched cohort with no history of thalassaemia. Amongst thalassaemia patients in general, the TIT group exhibited a higher rate of hospitalization compared to the TDT group (p = 0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the TIT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in-hospital, all-cause mortality, whereas the presence of thalassaemia (either TDT or TIT) was found to be independently associated with reduced all-cause mortality. The presence of thalassaemia in COVID-19 patients was independently associated with lower in-hospital, all-cause mortality and few in-hospital complications in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models., (© 2022 The Authors. Journal of Cellular and Molecular Medicine published by Foundation for Cellular and Molecular Medicine and John Wiley & Sons Ltd.)

Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder characterized by recurrent acute vaso-occlusive crises (VOCs and anemia). Gold standard treatments are hydroxycarbamide (HC) and/or different red blood cell (RBC) transfusion regimens to limit disease progression. Here, we report a retrospective study on 1,579 SCD patients (median age 23 years; 802 males/777 females), referring to 34 comprehensive Italian centers for hemoglobinopathies. Although we observed a similar proportion of Caucasian (47.9%) and African (48.7%) patients, Italian SCD patients clustered into two distinct overall groups: children of African descent and adults of Caucasian descent. We found a subset of SCD patients requiring more intensive therapy with a combination of HC plus chronic transfusion regimen, due to partial failure of HC treatment alone in preventing or reducing sickle cell-related acute manifestations. Notably, we observed a higher use of acute transfusion approaches for SCD patients of African descent when compared to Caucasian subjects. This might be related to (i) age of starting HC treatment; (ii) patients' low social status; (iii) patients' limited access to family practitioners; or (iv) discrimination. In our cohort, alloimmunization was documented in 135 patients (8.5%) and was more common in Caucasians (10.3%) than in Africans (6.6%). Alloimmunization was similar in male and female and more frequent in adults than in children. Our study reinforces the importance of donor-recipient exact matching for ABO, Rhesus, and Kell antigen systems for RBC compatibility as a winning strategy to avoid or limit alloimmunization events that negatively impact the clinical management of SCD-related severe complications., Clinical Trial Registration: ClinicalTrials.gov, identifier: NCT03397017., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Graziadei, De Franceschi, Sainati, Venturelli, Masera, Bonomo, Vassanelli, Casale, Lodi, Voi, Rigano, Pinto, Quota, Notarangelo, Russo, Allò, Rosso, D'Ascola, Facchini, Macchi, Arcioni, Bonetti, Rossi, Sau, Campisi, Colarusso, Giona, Lisi, Giordano, Boscarol, Filosa, Marktel, Maroni, Murgia, Origa, Longo, Bortolotti, Colombatti, Di Maggio, Mariani, Piperno, Corti, Fidone, Palazzi, Badalamenti, Gianesin, Piel and Forni.)

Background: Iron-chelation therapy is life-saving in patients on a chronic transfusion regimen as it reduces organ damage related to iron deposition in the tissues. Deferasirox, an iron-chelator, is characterized by pharmacokinetics variability, and some patients may discontinue the treatment due to toxicities., Objective: Understanding whether deferasirox plasma levels are related to patients' specific characteristics could help to optimize DFX dosage., Methods: We analyzed deferasirox plasma concentration in 57 transfusion-dependent anemic patients using the HPLC method in this prospective-retrospective cohort study. All outpatients (3 to 98 years) were treated with deferasirox (film-coated tablet) for at least one year (median dose, 16.5 mg/Kg once a day). Deferasirox plasma concentration was normalized for dose/Kg (C/dose) and corrected with a linear regression model that relates C/dose and the time of blood sampling (C ref /dose)., Results: No significant differences in C ref /dose were found between males and females, either between different types of hemoglobinopathies or depending on the presence of the UGT1A1*28 polymorphism. C ref /dose has a positive and significant correlation with age, creatinine, and direct bilirubin. C ref /dose, instead, has a negative and significant correlation with Liver Iron Concentration (LIC), ferritin, and eGFR. C ref /dose was significantly different between three age categories <18yrs, 18-50yrs, and >50yrs, with C ref /dose median values of 1.0, 1.2, and 1.5, respectively., Conclusion: The study evidenced that to ensure the efficacy of deferasirox in terms of control over LIC and, at the same time, a lesser influence on renal function, the dose of the drug to be administered to an elderly patient could be reduced., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background: Hemoglobin A (Hb A) (α 2 β 2 ) in the normal adult subject constitutes 96-98% of hemoglobin, and Hb F is normally less than 1%, while for hemoglobin A 2 (Hb A 2 ) (α 2 δ 2 ), the normal reference values are between 2.0 and 3.3%. It is important to evaluate the presence of possible delta gene mutations in a population at high risk for globin gene defects in order to correctly diagnose the β-thalassemia carrier., Methods: The most used methods for the quantification of Hb A 2 are based on automated high performance liquid chromatography (HPLC) or capillary electrophoresis (CE). In particular Hb analyses were performed by HPLC on three dedicated devices. DNA analyses were performed according to local standard protocols., Results: Here, we described eight new δ-globin gene variants discovered and characterized in some laboratories in Northern Italy in recent years. These new variants were added to the many already known Hb A 2 variants that were found with an estimated frequency of about 1-2% during the screening tests in our laboratories., Conclusions: The knowledge recognition of the delta variant on Hb analysis and accurate molecular characterization is crucial to provide an accurate definitive thalassemia diagnosis, particularly in young subjects who would like to ask for a prenatal diagnosis or preimplantation genetic diagnosis.

Infections that cause cervicitis are a topic presented in the "Clinical Protocol and Therapeutic Guidelines for Comprehensive Care for People with Sexually Transmitted Infections", published by the Brazilian Ministry of Health in 2020. The document was developed based on scientific evidence and validated in discussions with experts. This article presents epidemiological and clinical aspects of infections that cause cervicitis and recommendations on screening, diagnosis, and treatment of affected people and their sexual partnerships. Also, it discusses strategies for surveillance, prevention, and control of these infections for health professionals and health service managers involved in the programmatic and operational management of sexually transmitted infections. Expanding access to diagnostic tests and early treatment are crucial for controlling the spread of pathogens that cause cervicitis. Associated factors to cervicitis: sexually active women younger than 25 years old, new or multiple sexual partners, partners with STI, previous history or presence of other STI, and irregular use of condoms.

This article aims to present concepts and clinical practices recommended to approach people with active sex life. These concepts are an integral part of the recommendations of the Clinical Protocol and Therapeutic Guidelines for Comprehensive Care for People with Sexually Transmitted Infections (STI), published by the Ministry of Health of Brazil in 2020. The article proposes a comprehensive approach to sexuality for health promotion. It presents significant aspects of the communication process that must develop, without prejudice and judgment, focusing on sexual and reproductive health. It also highlights relevant points about the exercise of sexuality at specific stages of life, recommending assessment of risks and vulnerabilities and screening for STI and condom use. In this way, it is possible to contribute to exercise their sexuality fully, responsibly, and safely. Sexual health is the physical, emotional, mental, and social wellbeing associated with the exercise of sexuality, with sexual and reproductive rights considered fundamental.

Thalassemia syndromes are characterized by the inability to produce normal hemoglobin. Ineffective erythropoiesis and red cell transfusions are sources of excess iron that the human organism is unable to remove. Iron that is not saturated by transferrin is a toxic agent that, in transfusion-dependent patients, leads to death from iron-induced cardiomyopathy in the second decade of life. The availability of effective iron chelators, advances in the understanding of the mechanism of iron toxicity and overloading, and the availability of noninvasive methods to monitor iron loading and unloading in the liver, heart, and pancreas have all significantly increased the survival of patients with thalassemia. Prolonged exposure to iron toxicity is involved in the development of endocrinopathy, osteoporosis, cirrhosis, renal failure, and malignant transformation. Now that survival has been dramatically improved, the challenge of iron chelation therapy is to prevent complications. The time has come to consider that the primary goal of chelation therapy is to avoid 24-h exposure to toxic iron and maintain body iron levels within the normal range, avoiding possible chelation-related damage. It is very important to minimize irreversible organ damage to prevent malignant transformation before complications set in and make patients ineligible for current and future curative therapies. In this clinical case-based review, we highlight particular aspects of the management of iron overload in patients with beta-thalassemia syndromes, focusing on our own experience in treating such patients. We review the pathophysiology of iron overload and the different ways to assess, quantify, and monitor it. We also discuss chelation strategies that can be used with currently available chelators, balancing the need to keep non-transferrin-bound iron levels to a minimum (zero) 24 h a day, 7 days a week and the risk of over-chelation.

Objective: To analyze how syphilis detection rates evolved from 2011 to 2017 according to sex, age and place of residence in the state of São Paulo., Methods: A historical series was organized with data from the Notification Disease Information System. The acquired syphilis detection rates (ASDR) per 100,000 inhabitants and the acquired syphilis detection rates including pregnant women with syphilis (PASDR) per 100,000 inhabitants were described. For a trend analysis of the rates in the studied period, the Poisson Jointpoint (inflection point) model was performed, and the annual percentage change (APC) per segment and the average annual percentage change (AAPC) were estimated, with respective 95% confidence intervals (95%CI)., Results: A total of 205,424 cases of acquired syphilis and syphilis in pregnant women in the period were reported. The ASDR per 100,000 inhabitants ranged from 26.0 to 84.6 between 2011 and 2017 and the PASDR per 100,000 inhabitants ranged from 33.7 to 108.9; the trend was increasing in both, and an inflection point was identified dividing the ASDR and PASDR curve into two periods: 2011 to 2013 and 2013 to 2017: the AAPC found for ASDR was 21.0% (95%CI 15.5 ‒ 26.4) and the PASDR was 21.2% (95%CI 16.4 ‒ 26.1), in the age groups up to 24 years old, there was a significant growth in both sexes. A heterogeneity in the evolution of rates by region of the state was observed between 2011 and 2017., Conclusions: The increasing trend in acquired syphilis detection rates can be attributed to better adherence to notification and disproportionate involvement of young people.

Manual erythroexchange (MEEX) was proven to be effective and safe in the management of sickle cell disease (SCD). The goal is to quickly reduce the percentage of hemoglobin S (HbS%). A national survey of the Italian Society for Thalassemia and Hemoglobinopathies (SITE) observed a great variability among MEEX protocols none of which were found to be predictive of the values of HbS% and hemoglobin (Hb) after the exchange. Two equations to estimate the HbS% and Hb values to be obtained after MEEX were developed based on the results of the MEEX procedures in place in the centers participating in the present study. A standard protocol was subsequently defined to evaluate the volumes to exchange to obtain the target values of HbS% and Hb. The protocol was tested in 261 MEEX performed in SCD patients followed in the 5 participating centers that belong to the Italian Hemoglobinopathy Comprehensive Care Network, with the support of the SITE. The results showed a correlation between the estimated and measured values of HbS% and Hb (R p 0.95 and 0.65 respectively, p < 0.001). A negligible bias was found for the prediction of HbS% and a bias of 1 g/dl for Hb. From consecutive MEEX, a rate of increase of HbS% between two exchanges of around 0.4% per day (p < 0.001) was measured. This protocol was shown to be effective and safe, as all patients reached the target value of HbS%. All the MEEX procedures were carried out with single venous access. No adverse events or reactions such as hypotension or electrolyte imbalance were reported nor were any complaints concerning the procedures received from patients.

Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Here, we carried out a retrospective study to evaluate sickle bone disease (SBD) in a cohort of adults with SCD. We assessed bone density, metabolism and turnover. We also evaluated the presence of fractures and the correlation between SCD severity and skeletal manifestations. A total of 71 patients with SCD were analyzed. The mean age of population was 39 ± 10 years, 56% of which were females. We found osteoporosis in a range between 7% and 18% with a high incidence of vertebral fractures. LDH and AST were predictive for the severity of vertebral fractures, while bone density was not. Noteworthy, we identified -1.4 Standard Deviations T -score as the cutoff for detecting the presence of fractures in patients with SCD. Collectively our data allowed us to develop an algorithm for the management of SBD, which may be useful in daily clinical practice to early intersect and treat SBD.

We here report the successful recovery from coronavirus disease-19 (COVID-19) pneumonia in a patient with β-thalassemia major (β-TM) and severe pulmonary arterial hypertension (PAH), focusing on the patient's comorbidities, therapeutic course and drug interaction.

Thalassemia is among the most common monogenic diseases worldwide. Stem cell transplantation can be curative but is reserved for young patients, as probably gene therapy will be in the future. Adult thalassemia patients are treated with transfusion therapy and iron chelation, and improvements in the safety of transfusion protocols, use of iron chelation, monitoring of iron overload, and management of comorbidities have substantially prolonged survival, increasing the proportion of adult patients in the thalassemic population. However, older patients are more likely to develop multiple disease-related morbidities, including osteoporosis, endocrine disorders, liver disease, renal dysfunction, and cancer. Thus, the main objective of this article is to describe new challenges posed by the increasing life expectancy of patients with thalassemia, focusing on data from Italy where there is a well-documented history of thalassemia management. It is hoped that the mortality and morbidity benefits already seen in patients with thalassemia will continue to improve with ongoing advances in the quality of treatment., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Sickle cell disease (SCD) is the most important hemoglobinopathy worldwide in terms of frequency and social impact, recently recognized as a global public health problem by the World Health Organization. It is a monogenic but multisystem disorder with high morbidity and mortality. Vaso-occlusion, hemolytic anemia and vasculopathy are the hallmarks of SCD pathophysiology. This review focuses both on "time-dependent" acute clinical manifestations of SCD and chronic complications commonly described in adults with SCD. The review covers a broad spectrum of topics concerning current management of SCD targeted at the internists and emergency specialists who are increasingly involved in the care of acute and chronic complications of SCD patients.

Introduction: This study aimed to identify the factors associated with condom use in the last sexual intercourse., Methods: A population-based survey with young people aged 15-24, in the city of São Paulo, which collected sociodemographic data referring to knowledge about sex and sexual behavior., Results: Among 821 sexually active young people interviewed in the last year, condom use in their last sexual intercourse was positively associated with: 1) not being married; 2) use of condom at sexual onset; and 3) receiving free condoms; additionally, among men: 4) casual partners in the previous year; and 5) partner of the same sex; and, amongwomen: 6) sexual onset after the age of 15. Having been tested for HIV was a negative association among women. Condoms are widely acknowledged, and there is a pattern of use for the first and last sexual intercourse. Access to free condoms is an important factor for use, and people use condoms according to standards that configure risk management., Conclusions: The strategy of primary prevention with the use of condoms is not yet exhausted. Based on this study, the city of São Paulo takes prevention as a public policy and allocates large condoms dispensers in 26 urban bus terminals, where 6million people circulate daily. In 2016, 75,546,720 free condoms were distributed, 30% in bus terminals alone.

We determined the prevalence of sexually transmitted infections (STIs), the factors associated with infection and types of counseling received by men and women from health professionals in the City of São Paulo. The investigation consisted of a cross-sectional study conducted with men and women aged between 15 and 64 years living in the City of São Paulo. Of 4,057 individuals who had engaged in sexual activity, 6.3% reported previous history of a STI: 4.3% of women and 8.2% of men. The factors associated with STI were being aged over 34 years and not using a condom during first sexual intercourse, among men, and being aged over 25 years among women. Protective factors included not having had sexual intercourse with someone from the same sex, among men, and having initiated sexual activity after the age of 15 years and not having a casual sex partner over the last 12 months, among women. Counseling about the importance of HIV and syphilis testing was received by 72.1% and 64.7% of women, respectively, while fewer than half of the men received this type of counseling (40.2% and 38.6 %, respectively). The prevalence of previous history of a STI was high among the population of the City of São Paulo. The findings of this study informed the development, implementation, and evaluation of STI policies, including those directed at HIV, leading to a reduction in the barriers that hinder access to and use of condoms and the creation of STI prevention app.

Objective: to estimate the prevalence of Kaposi's sarcoma (KS) in patients with AIDS and identify the associated factors to the occurrence of this neoplasm., Methods: this is a cross-sectional study with notification data from two AIDS reference centers in São Paulo-SP, Brazil, from January, 2003 to March, 2010; probabilistic linkage and multiple logistic regression methods were applied., Results: among 3,557 AIDS cases, 213 (6%) presented KS; 95.3% of them occurred in males; male sex (OR=3.1; 95%CI=1.4;6.6), age at the AIDS diagnosis >28 years old (OR=1.6; 95%CI=1.0;2.6), MSM (OR=3.2; 95%CI=2.0;4.9), prior use of HAART (OR=0.4; 95%CI=0.3;0.5), AIDS diagnosis between 2007-2010 (OR=0.3; 95%CI=0.2;0.4), and CD4+ T-cell counting under 200cells/mm3 (OR=16.0; 95%CI=6.0;42.7) and 200-500cells/mm³ (OR=2,5; 95%CI=1.1;6.4) were associated to the occurrence of KS., Conclusion: KS has a high prevalence in São Paulo-SP; strategies for early HIV diagnosis may reduce this prevalence.

Purpose: In magnetic resonance imaging (MRI) relaxometry, various software programs are available to perform R2* measurements and to estimate the liver iron concentration (LIC). The main objective of our study was to compare R2* LIC values, obtained with three different software programs based on specific decay models and calibration curves, with LIC estimates provided by R2-relaxometry (FerriScan)., Methods: This retrospective study included 15 patients with 15 baseline MRIs and 34 serial examinations. R2* LIC estimates were calculated using the FuncTool, CMRtools/Thalassemia Tools and Quanta Hematology programs. Longitudinal LIC changes (ΔLIC) were calculated using the subset of 34 serial MRIs., Results: After Bland-Altman analysis on baseline data, Quanta Hematology, which employs the monoexponential-plus-constant fit, produced the lowest mean difference [0.01 ± 0.14 log(mg/gdw)] with the closest limits of agreement. In the longitudinal setting, Quanta Hematology again gave the lowest mean difference between R2 and R2* LIC (0.1 ± 2.6 mg/gdw). Using FerriScan as reference, the value of concordant directional ΔLIC changes was the same for all programs (27/34, 85.7 %)., Conclusions: R2* LICs are higher than R2 LICs at iron levels <7 mg/gdw, while R2 LIC averages higher than R2* LIC with increasing iron load. The monoexponential-plus-constant model provided the best agreement with R2 LIC estimates.

Introduction: This study aimed to estimate the prevalence and risk factors associated with Chlamydia trachomatis (CT) infection among women with HIV in São Paulo., Methods: This cross-sectional study included women with HIV who were receiving care from sixteen public health services in São Paulo (October 2013 to March 2014). All participants answered a questionnaire regarding their sociodemographic, behavioral, and clinical characteristics. A urine sample was tested for CT and Neisseria gonorrhoeae (NG) using the polymerase chain reaction. The chi-square test and a logistic regression model were used to test the associations with CT or NG infections., Results: We evaluated 853 women and ultimately included 836 (98%) women. The mean age was 40.5 ± 0.34 years, and the prevalences of CT and NG infections were 1.8% and 0.5%, respectively. CT infection was associated with CD4+ T-cell counts of <350 cells/mm3 [adjusted odds ratio (ORadj): 24.5], age of 18-25 years (ORadj: 23.2), the non-use of condoms during the last 6 months (ORadj: 10.2), a self-reported history of a sexually transmitted infection (ORadj: 9.4), and having two or more sexual partners during the last year (ORadj: 6.1)., Conclusions: Although we observed a low prevalence of CT infection among women with HIV, younger age was associated with a high risk of infection. Therefore, it may be appropriate to include screening for CT as part of the routine care for this population.

Ion microprobe age determinations of 102 detrital zircon crystals from a sand extrudite, Cretaceous Paraná volcanic province, set limits on the origin of the numerous sand layers present in this major flood basalt province. The zircon U-Pb ages reflect four main orogenic cycles: Mesoproterozoic (1155-962 Ma), latest Proterozoic-early Cambrian (808-500 Ma) and two Palaeozoic (Ordovician- 480 to 450 Ma, and Permian to Lower Triassic- 296 to 250 Ma). Two additional small concentrations are present in the Neoarchean (2.8 to 2.6 Ga) and Paleoproterozoic (2.0 to 1.7 Ga). Zircon age peaks closely match the several pulses of igneous activity in the Precambrian Brazilian Shield and active orogeny in Argentina. A main delimitation of the origin of the sand is the absence of zircon ages from the underlying Cretaceous basalts, thus supporting an injectite origin of the sand as an extrudite that emanated from the paleoerg that constitutes the Botucatu Formation.

Our goal was to determine the prevalence of Trichomonas vaginalis and its associated risk factors in parturient women aged 15-24 years attending Brazilian public maternity units. Participants answered a demographic, behavioral, and clinical data questionnaire. A sample of urine was screened for T. vaginalis. A total 299 women participated in this study. The prevalence rate of T. vaginalis was 7.7% (95% CI: 4.7-10.7%). The factors associated with T. vaginalis were use of illicit drugs [OR=4.70 (95% CI: 1.63-13.56, p=0.004)] and not attending antenatal care [OR=5.15 (95% CI: 1.15-23.25, p=0.032)]. These data demonstrate that it is important to discuss how to include routine screening for T. vaginalis during antenatal care in Brazil., (Copyright © 2014 Elsevier Editora Ltda. All rights reserved.)

Objective: to describe the epidemiological profile, risk behaviors, frequency of prior history of syphilis in women living with AIDS and to investigate associated factors., Methods: a cross-sectional study conducted with women living with HIV attending at Reference Center for AIDS in São Paulo. Demographic, behavioral, and clinical data were analyzed based on medical records., Results: a total of 598 women were included in the study and the prevalence of previous syphilis was 6.2% (95% CI 4,3-8,1). Seventy-three percent of women were less than 40 years of age when diagnosed with AIDS and 49.6% had more than eight years of formal education. 67.2% were white and 65.9% were not married or living with a partner, 53.2% reported that their first sexual intercourse aged more than 15 years, 56.5% reported having only one partner in the last year and 13% reported drug use. Regarding laboratory data, 83.8% had CD4+ <500 cells/ mm3 upon diagnosis of AIDS. Previous syphilis was associated with the use of crack cocaine [AOR = 6.8 (95% CI 1.7 - 27.5)], >1 sexual partner in the last year [AOR = 6.6 (95% CI 1.2 - 37.1)], CD4 + <500 cells/mm3 [AOR = 3.8 (women 1.1 - 13.6)], HIV diagnosis > 8 years [AOR = 2.4 (95% CI 1.0 - 5.8)]., Conclusion: a high prevalence of previous syphilis was found in the population studied, and crack use was identified among the main associated risk factors. Interventions to reduce the risk of sexual transmission of HIV and syphilis must be strengthened, with the implementation of control actions, screening strategies, early diagnosis and treatment, preventing complications, reducing morbidity and improving sexual and reproductive health.

Introduction: Homeless people are a vulnerable group to sexually transmitted diseases (STD) with high prevalence of syphilis and hepatitis., Objectives: To estimate the prevalence of syphilis infection and its association with risky behaviors for STDs in a sample of homeless people, and to assess the feasibility of the use of rapid syphilis test (RST) in this population., Methods: Cross-sectional study, in a convenience sample of homeless people assisted in social support services of São Paulo, between 2006 and 2007. A structured questionnaire was applied and RST was performed. In addition, a blood sample for syphilis detection was also collected. The sensitivity and specificity of the RST was estimated using conventional laboratory diagnosis (VDRL + TPHA) as reference., Results: 1,405 volunteers were included in the study. The prevalence rate of syphilis was 7.0%, and was associated with homosexual practices (OR(adj) 4.9; 95%CI 2.6 - 9.4), prior history of STD (OR(adj) 2.6; 95%CI 1.7 - 4.0) and with self-referred non-white race (OR(adj) 1.9; 95%CI 1.1 - 3.4). The sensitivity and specificity of the RST for syphilis were, respectively, 81.4 and 92.1%., Conclusion: The high prevalence of syphilis infection among homeless people shows the need for actions for its control and the utilization of RST that can be considered an efficient strategy due to its sensitivity and specificity. Public Health policymakers must strengthen actions for syphilis control, with screening tests for syphilis and early treatment, decreasing morbidity with the improvement of sexual and reproductive health of the population in general and especially the most vulnerable.

Our goal was to estimate prevalence of HIV among young women in labor. A national, probability-based, cross-sectional study was performed among pregnant women, aged 15-24 years, who were attending Brazilian public hospitals. The study included 2,071 of 2,400 women selected (86.3 % participation). Mean age was 20.2 years (SD = 2.7). HIV prevalence was 0.7 % (95 % CI, 0.4-1.1 %). Living in the North region of the country and having previous sexually transmitted infections were associated with HIV infection. Our survey of young pregnant women found higher prevalence than expected for women of all ages in Brazil (0.42 %), indicating that the epidemic persists among heterosexuals.