Sickle Cell Disease in Brazil: Current Management.

Sickle cell disease (SCD) comprises inherited red blood cell disorders due to a mutation in the β-globin gene (c20A > T, pGlu6Val) and is characterized by the presence of abnormal hemoglobin, hemoglobin S, hemolysis, and vaso-occlusion. This mutation, either in a homozygous configuration or in compound states with other β-globin mutations, leads to polymerization of hemoglobin S in deoxygenated conditions, causing modifications in red blood cell shape, particularly sickling. Vaso-occlusive crisis (VOC) is the hallmark of the disease, but other severe complications may arise from repeated bouts of VOCs.

Real-World Evidence on the Use of Endocrine Therapy for Ductal Carcinoma In Situ in Patients Treated With Breast-Conserving Surgery Followed by Postoperative Radiation Therapy: A Brazilian Retrospective Cohort Study.

Introduction/background: This study aims to evaluate the reproducibility of findings from randomized controlled trials regarding adjuvant hormone therapy (HT) for breast ductal carcinoma in situ (DCIS) in a real-life scenario.

Materials/methods: This retrospective cohort study used Fundação Oncocentro de São Paulo database. It included DCIS patients DCIS who received breast-conserving surgery and postoperative radiation therapy.

Novel Insights into the Pathophysiology and Treatment of Sickle Cell Disease.

The polymerization of hemoglobin under deoxygenation is the main pathophysiological event in sickle cell diseases, described more than 70 years ago. The last two decades have seen a major increase in knowledge about the cascade of events that follow the polymerization of hemoglobin and the ensuing sickling of red blood cells. Several distinctive therapeutic targets have been discovered as a result, and a few drugs with innovative mechanisms of action are already on the market, while several others are the focus of ongoing trials.

Retrospective analysis of surgical and oncological results of laparoscopic surgeries performed by residents of coloproctology.

Introduction: with the improvement and wide acceptance of laparoscopy in colorectal operations, there was a need for specific training of surgeons in training. There are few studies evaluating the postoperative results of laparoscopic colectomies performed by resident physicians and their impact on patient safety.

Purpose: to analyze the surgical and oncological results of laparoscopic colectomies performed by coloproctology residents and compare them with data in the literature.

Reversible cerebral vasoconstriction syndrome associated with probable drug poisoning.

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical condition characterized by arterial involvement of the intracranial vessels, manifesting with vasospasm. The most common clinical manifestation related to the syndrome is the thunderclap headache, which consists of a severe headache that reaches the peak of pain within minutes. The imaging study assumes a leading role in the complementary investigation.

Three-Dimensional Dosimetry by Optical-CT and Radiochromic Gel Dosimeter of a Multiple Isocenter Craniospinal Radiation Therapy Procedure.

Craniospinal irradiation (CSI) is a complex radiation technique employed to treat patients with primitive neuroectodermal tumors such as medulloblastoma or germinative brain tumors with the risk of leptomeningeal spread. In adults, this technique poses a technically challenging planning process because of the complex shape and length of the target volume. Thus, it requires multiple fields and different isocenters to guarantee the primary-tumor dose delivery.

The Effect of Patient Position on Psoas Morphology and in Lumbar Lordosis.

Introduction: Among the interbody fusions, lateral lumbar interbody fusion allows access to the lumbar spine through the major psoas muscle, which offers several advantages to the spine surgeon. However, some of its drawbacks cause surgeons to avoid using it as a daily practice. Therefore, to address some of these challenges, we propose the prone transpsoas technique, differing mainly from the traditional technique on patient position-moving from lateral to prone decubitus, theoretically enhancing the lordosis and impacting the psoas morphology.

The histone deacetylase inhibitor PCI-24781 as a putative radiosensitizer in pediatric glioblastoma cell lines.

Background: Glioblastoma (GBM) is considered to be one of the most aggressive tumors of the central nervous system (CNS). Even with the use of modern treatment protocols, the prognosis remains reserved, with children with GBM having a mean survival of 12-15 months.

Methods: In the present study we investigated the potential radiosensitizing effect of PCI-24781, a potent pan-histone deacetylase inhibitor (HDACi), on the SF188 and KNS42 cell lines of pediatric GBM.

An Overview on the Search for Schizophrenia Biomarkers.

Unlabelled: Schizophrenia is a severe psychiatric disorder with a complex presentation comprising positive symptoms (e.g.: hallucinations and delusions), negative symptoms (e.

FLORENCE: a randomized, double-blind, phase III pivotal study of febuxostat versus allopurinol for the prevention of tumor lysis syndrome (TLS) in patients with hematologic malignancies at intermediate to high TLS risk.

Background: Serum uric acid (sUA) control is of key relevance in tumor lysis syndrome (TLS) prevention as it correlates with both TLS and renal event risk. We sought to determine whether febuxostat fixed dose achieves a better sUA control than allopurinol while preserving renal function in TLS prevention.

Patients And Methods: Patients with hematologic malignancies at intermediate to high TLS risk grade were randomized to receive febuxostat or allopurinol, starting 2 days before induction chemotherapy, for 7-9 days.

Functional analysis of the stomatognathic system in individuals infected with human immunodeficiency virus.

Purpose: To understand the effects of HIV type 1 on the function of the masticatory muscles.

Methods: Sixty individuals were selected from both genders, aged between 22 and 57years (mean 36.77±9.

Determinants of intensive insulin therapeutic regimens in patients with type 1 diabetes: data from a nationwide multicenter survey in Brazil.

Background: To evaluate the determinants of intensive insulin regimens (ITs) in patients with type 1 diabetes (T1D).

Methods: This multicenter study was conducted between December 2008 and December 2010 in 28 public clinics in 20 Brazilian cities. Data were obtained from 3,591 patients (56.

Effectiveness of heat and moisture exchangers in preventing ventilator-associated pneumonia in critically ill patients: a meta-analysis.

Background: Patients may acquire ventilator-associated pneumonia (VAP) by aspirating the condensate that originates in the ventilator circuit upon use of a conventional humidifier. The bacteria that colonize the patients themselves can proliferate in the condensate and then return to the airways and lungs when the patient aspirates this contaminated material. Therefore, the use of HME might contribute to preventing pneumonia and lowering the VAP incidence.

Inhibition of NF- κ B by Dehydroxymethylepoxyquinomicin Suppresses Invasion and Synergistically Potentiates Temozolomide and γ -Radiation Cytotoxicity in Glioblastoma Cells.

Despite advances in neurosurgery and aggressive treatment with temozolomide (TMZ) and radiation, the overall survival of patients with glioblastoma (GBM) remains poor. Vast evidence has indicated that the nuclear factor NF- κ B is constitutively activated in cancer cells, playing key roles in growth and survival. Recently, Dehydroxymethylepoxyquinomicin (DHMEQ) has shown to be a selective NF- κ B inhibitor with antiproliferative properties in GBM.

Prognostic factors in squamous cell carcinoma of the oral cavity.

Unlabelled: Researchers have been looking for factors that can influence the prognosis of oral cancer, because its outcome is highly uncertain.

Aim: To evaluate variables that can impact the survival rate of patients with squamous-cell carcinoma of the oral cavity.

Material And Methods: Data analysis of 45 patients from January, 2001 to January, 2006.

[Follow-up group for schizophrenic patients using Clozapine and their relatives: the participants' perception].

This study described the views of Schizophrenic patients as well as those of their relatives concerning the group follow-up. The population consisted of 11 patients participating in the group and of 11 relatives who most often accompanied them to group meetings. Interviews, which were tape-recorded and fully transcribed, were used for data collection.

Hair cell loss and regeneration in the chick cochlea after treatment with gentamicin.

Hair cells can be damaged by countless agents, among them aminoglycoside antibiotics. In the chick cochlea, the hair cell loss can be recovered by regeneration. The objectives of the present investigation were to study the time progression of injury caused by gentamicin and the regeneration process in chick hair cells.

[Hypertrophic cardiomyopathy treated by interventional technique of septal reduction].

A 75-year old woman with obstructive hypertrophic cardiomyopathy and class IV dyspnea refractory to medical management had relative contraindications for both pacemaker implantation and surgical approach. Percutaneous intervention was devised to perform the selective injection of absolute alcohol in the first septal branch of the left anterior descending coronary artery. This led to a limited septal infarction documented by ST elevation, enzymatic elevation and the appearance of complete right bundle branch block on the electrocardiogram.

[Reagudization of Chagas myocarditis inducing exclusive right ventricular failure].

Woman, 42 years-old, receiving immunosuppressive therapy for a lymphoma, presented reagudization of Chagas' disease, from its indeterminate phase. Intense inflammatory visceral aggression, due to extensive intracellular proliferation of the Trypanosoma cruzi, was the likely mechanism for acute myocarditis leading to severe right ventricular failure. Antiparasite chemotherapy was effective in the control of visceral involvement and for the remission of cardiac failure.

[Detection of myocardial ischemia in chronic Chagas disease patients with atypic precordial pain by exercise and Holter tests].

Purpose: To determine the incidence and characteristics of myocardial ischemia, as detected by stress electrocardiography and Holter monitoring in Chagas' patients whose main complaint was precordial pain.

Methods: Thirty-one consecutive patients with Chagas' disease diagnosed on the basis of clinical and serological tests, and precordial pain severe enough to warrant cardiac catheterization were studied. Mean age was 54.