Sex and body mass index impact on digit circumference for Leeds Dactylitis Index calculation.

OBJECTIVES: To estimate digit circumference and the impact of sex and body mass index (BMI) for the calculation of the Leeds Dactylitis Index (LDI) in psoriatic arthritis (PsA) patients with bilateral dactylitis. METHODS: Digit circumference of the hands and the foot were measured with a dactylometer and were studied according to sex and BMI (divided in 4 weight categories) in healthy Portuguese subjects, using Student's t-test and One-way ANOVA, respectively. The effect size of sex and BMI were calculated using Cohen's d test and Eta squared, respectively. Multiple linear regression was used to calculate the effect of sex and BMI, as well as their interaction, to create a formula to predict digit circumference. RESULTS: Fifty-nine participants (33 women, 26 men) with a mean BMI of 24.8 were included. Men's mean digit circumferences were statistically higher than those of women (p<0.001), with a large sex effect size in most of the digits. Differences in the mean circumference between the four BMI categories were statistically significant (p<0.05) for all digits, with a large BMI effect size. Sex and BMI were independent variables to predict mean digit circumference (p<0.001). A new tool (based on regression analysis) allowing to estimate the circumference of digits for males and females of different BMIs is presented. CONCLUSIONS: Our data allows the calculation of digit circumference for males and females of different BMIs in the Portuguese population; and shows that BMI influences digital circumference supporting BMI inclusion in LDI references tables.

Patient satisfaction with ultrasound, whole-body CT and whole-body diffusion-weighted MRI for pre-operative ovarian cancer staging: a multicenter prospective cross-sectional survey.

BACKGROUND: In addition to the diagnostic accuracy of imaging methods, patient-reported satisfaction with imaging methods is important. OBJECTIVE: To report a secondary outcome of the prospective international multicenter Imaging Study in Advanced ovArian Cancer (ISAAC Study), detailing patients' experience with abdomino-pelvic ultrasound, whole-body contrast-enhanced computed tomography (CT), and whole-body diffusion-weighted magnetic resonance imaging (WB-DWI/MRI) for pre-operative ovarian cancer work-up. METHODS: In total, 144 patients with suspected ovarian cancer at four institutions in two countries (Italy, Czech Republic) underwent ultrasound, CT, and WB-DWI/MRI for pre-operative work-up between January 2020 and November 2022. After having undergone all three examinations, the patients filled in a questionnaire evaluating their overall experience and experience in five domains: preparation before the examination, duration of examination, noise during the procedure, radiation load of CT, and surrounding space. Pain perception, examination-related patient-perceived unexpected, unpleasant, or dangerous events ('adverse events'), and preferred method were also noted. RESULTS: Ultrasound was the preferred method by 49% (70/144) of responders, followed by CT (38%, 55/144), and WB-DWI/MRI (13%, 19/144) (p<0.001). The poorest experience in all domains was reported for WB-DWI/MRI, which was also associated with the largest number of patients who reported adverse events (eg, dyspnea). Patients reported higher levels of pain during the ultrasound examination than during CT and WB-DWI/MRI (p<0.001): 78% (112/144) reported no pain or mild pain, 19% (27/144) moderate pain, and 3% (5/144) reported severe pain (pain score >7 of 10) during the ultrasound examination. We did not identify any factors related to patients' preferred method. CONCLUSION: Ultrasound was the imaging method preferred by most patients despite being associated with more pain during the examination in comparison with CT and WB-DWI/MRI. TRIAL REGISTRATION NUMBER: NCT03808792.

Promoting clinical reasoning in undergraduate Family Medicine curricula through concept mapping: a qualitative approach.

Clinical reasoning is a crucial skill for physicians, enabling them to bridge theoretical knowledge with practical application. The gap between basic sciences and clinical practice persists as a challenge, with traditional teaching methods yet to effectively bridge it. Concept maps (CMs), visual tools for organizing and connecting knowledge, hold promise for enhancing clinical reasoning in the undergraduate medical curriculum. However, further research is required to ascertain if CMs facilitate clinical reasoning development in medical students transitioning from basic sciences to clinical practice. This study aims to delineate how CMs can facilitate clinical reasoning in patients with multimorbidity within undergraduate Family Medicine curricula, as perceived by students and tutors, and to understand the implementation process and resources required. This exploratory qualitative study formed a part of an action research project. While introducing an educational intervention to 5th-year medical students, we conducted a qualitative evaluation. Subsequently, semi-structured group interviews were conducted with students, and a focus group was conducted with tutors. Three main educational impacts were identified: integration of clinical information, support for patient management and care plan, and collaborative learning. Key aspects for successful CM implementation included clear instructions for map construction, using user-friendly software, allocating sufficient time for the task, encouraging group discussion of CMs, and incorporating tutor feedback. CMs are pedagogical tools that facilitate clinical information integration and support management and treatment plans, helping students better understand multimorbidity patients and promoting some components of clinical reasoning in undergraduate medical education.

Sex moderates the association between quantitative sensory testing and acute and chronic pain after total knee/hip arthroplasty.

OBJECTIVES: Acute postsurgical pain (APSP) may persist over time and become chronic. Research on predictors for APSP and chronic postsurgical pain (CPSP) has produced inconsistent results. This observational study aimed to analyze psychological and psychophysical variables associated with APSP and CPSP after total knee or hip arthroplasty, and to explore the role of sex. METHODS: Assessments were conducted before surgery, 48 h, and 3 months postsurgery, including questionnaires (sociodemographic, pain related, and psychological) and quantitative sensory testing (QST). Hierarchical linear regression models analyzed potential predictors of APSP and CPSP, and moderation analyses evaluated the role of sex. RESULTS: The study included 63 participants undergoing total knee (34, 54%) or hip (29, 46%) arthroplasty. Thirty-one (49.2%) were female and 32 (50.8%) were male. APSP (48 h) was associated with impaired conditioned pain modulation (CPM) (ß = 0.301, p = 0.019). CPSP (3 months) was associated with being female (ß = 0.282, p = 0.029), longer presurgical pain duration (ß = 0.353, p = 0.006), knee arthroplasty (ß = -0.312, p = 0.015), higher APSP intensity (ß = 373, p = 0.004), and impaired CPM (ß = 0.126, p = 0.004). In multivariate analysis, these clinical variables were significant predictors of CPSP, unlike sex, and CPM (adj. R 2 = 0.349). Moderation analyses showed that wind-up ratio (WUR) was a significant predictor of APSP in men (WUR × sex: b = -1.373, p = 0.046) and CPM was a significant predictor of CPSP in women (CPM × sex: b = 1.625, p = 0.016). CONCLUSIONS: Specific QST parameters could identify patients at risk for high-intensity APSP and CPSP, with sex as a moderator. This has important clinical implications for patient care, paving the way for developing tailored preventive pain management strategies.

Uniparental Disomy as a Mechanism for Combined Oxidative Phosphorylation Deficiency Associated with MRPS34 Gene.

INTRODUCTION: Mitochondrial oxidative phosphorylation (OXPHOS) is a cellular process that generates most of the cellular energy required by the body. Disorders affecting OXPHOS are multisystem diseases caused by pathogenic variants in more than 50 genes. In 2017, biallelic variants in the MRPS34 gene were shown to cause combined oxidative phosphorylation deficiency type 32 (COPD32) (OMIM#617664); however, only 7 patients have been reported in the literature up to this moment. COPD32 is characterized mainly by a severe Leigh-like syndrome. METHODS: Whole-exome sequencing identified a homozygous pathogenic variant in the MRPS34 gene, c.322-10G>A. Only the mother was heterozygous for this variant. SNP-array analysis was performed, which revealed a region of absence of heterozygosity in variant 16q with 9.8Mb, compatible with maternal uniparental disomy. RESULTS/CASE REPORT: We report the case of an 18-year-old female with unremarkable family history. The pregnancy was complicated by oligohydramnios, and the neonatal period was unremarkable. She evolved with low weight, mild-moderate developmental delay/intellectual disability, and hypogonadotropic hypogonadism. On examination, she had slender habitus, joint laxity, and kyphoscoliosis. The cardiac evaluation was normal, and the head MRI showed bilateral olivary nucleus degeneration that was not confirmed subsequently. Extensive metabolic studies documented only mild lactate and pyruvate elevation, and the chromosomal microarray was normal. CONCLUSION: We have reported the case of the first patient with COPD32 due to partial maternal uniparental disomy of chromosome 16, being first in Portugal and seventh in the literature. Contrarily to previous patients, who died in the first months of life or survived with severe DD/ID, and had a Leigh-like syndrome, this case is significantly milder, contributing to a better characterization of the phenotypic spectrum. Recurrence risk is unexpectedly low in this instance. This case illustrates the importance of segregation analysis in patients with homozygous recessive mutations.

Clinical Management and Neurosurgical Approach of Pott's Puffy Tumor: A Case Report.

Pott's puffy tumors are assumed to be infrequent concomitant intra- and extracranial abscesses, mainly secondary to complicated frontal sinusitis during infancy. Due to the close proximity to the superior sagittal sinus, there is a risk of developing venous infections, thrombosis, and morbidity. In this case report, we present a case of an 11-year-old girl who presented with headache and face edema. After recognizing the Pott's puffy tumor pattern on the CT scan and brain MRI, the neurosurgical approach involved pus evacuation and frontal sinus blockage, and the patient received antibiotic therapy and was evaluated for total recovery. To our knowledge, the prompt diagnosis and treatment of such conditions are paramount to avoid complications, and differential diagnosis should be encouraged in medical practice.

The Dicentrarchus labrax estrogen screen test: A relevant tool to screen estrogen-like endocrine disrupting chemicals in the aquatic environment.

In response to the need for the diversification of regulatory bioassays to screen estrogen-like endocrine disrupting chemical (EEDC) in the environment, we propose the use of a reporter gene assay involving all nuclear estrogen receptors from Dicentrarchus labrax (i.e., sbEsr1, sbEsr2a, or sbEsr2b). Named DLES test (D. labrax estrogen screen), it aims at complementing existing standardized in vitro tests by implementing more estrogen receptors notably those that do not originate from humans. Positive responses were obtained with all three estrogen receptors, and-consistently with observations from other species-variations in sensitivity to E2 were measured. Sensitivity and EC50 values could be classified as follows: sbEsr2b < sbEsr2a < sbEsr1. The pharmacological characterization with a human estrogen receptor antagonist (fulvestrant) successfully validated the specific involvement of each sbEsr and evidenced the capacity of the DLES test to highlight antagonist interactions. The DLES test was applied to WWTP contaminant extracts. A positive response was detected in the inflow sample in accordance with the YES test, but not in the outflow sample. Notwithstanding, the DLES test (sbEsr2b) exhibited greater sensitivity for the screening of those samples. This study demonstrates the need for more comprehensive testing including representatives of marine species for a better detection of EEDCs. The DLES test appears as a pertinent tool to predict adverse effects and to widen the scope of screening and hazard assessment of EEDCs in the environment.

Molecular Profiling of Axial Spondyloarthritis Patients Reveals an Association between Innate and Adaptive Cell Populations and Therapeutic Response to Tumor Necrosis Factor Inhibitors.

This study aims at identifying molecular biomarkers differentiating responders and non-responders to treatment with Tumor Necrosis Factor inhibitors (TNFi) among patients with axial spondyloarthritis (axSpA). Whole blood mRNA and plasma proteins were measured in a cohort of biologic-naïve axSpA patients (n = 35), pre and post (14 weeks) TNFi treatment with adalimumab. Differential expression analysis was used to identify the most enriched pathways and in predictive models to distinguish responses to TNFi. A treatment-associated signature suggests a reduction in inflammatory activity. We found transcripts and proteins robustly differentially expressed between baseline and week 14 in responders. C-reactive protein (CRP) and Haptoglobin (HP) proteins showed strong and early decrease in the plasma of axSpA patients, while a cluster of apolipoproteins (APOD, APOA2, APOA1) showed increased expression at week 14. Responders to TNFi treatment present higher levels of markers of innate immunity at baseline, and lower levels of adaptive immunity markers, particularly B-cells. A logistic regression model incorporating ASDAS-CRP, gender, and AFF3, the top differentially expressed gene at baseline, enabled an accurate prediction of response to adalimumab in our cohort (AUC = 0.97). In conclusion, innate and adaptive immune cell type composition at baseline may be a major contributor to response to adalimumab in axSpA patients. A model including clinical and gene expression variables should also be considered.

mRNA therapy corrects defective glutathione metabolism and restores ureagenesis in preclinical argininosuccinic aciduria.

The urea cycle enzyme argininosuccinate lyase (ASL) enables the clearance of neurotoxic ammonia and the biosynthesis of arginine. Patients with ASL deficiency present with argininosuccinic aciduria, an inherited metabolic disease with hyperammonemia and a systemic phenotype coinciding with neurocognitive impairment and chronic liver disease. Here, we describe the dysregulation of glutathione biosynthesis and upstream cysteine utilization in ASL-deficient patients and mice using targeted metabolomics and in vivo positron emission tomography (PET) imaging using (S)-4-(3-18F-fluoropropyl)-l-glutamate ([18F]FSPG). Up-regulation of cysteine metabolism contrasted with glutathione depletion and down-regulated antioxidant pathways. To assess hepatic glutathione dysregulation and liver disease, we present [18F]FSPG PET as a noninvasive diagnostic tool to monitor therapeutic response in argininosuccinic aciduria. Human hASL mRNA encapsulated in lipid nanoparticles improved glutathione metabolism and chronic liver disease. In addition, hASL mRNA therapy corrected and rescued the neonatal and adult Asl-deficient mouse phenotypes, respectively, enhancing ureagenesis. These findings provide mechanistic insights in liver glutathione metabolism and support clinical translation of mRNA therapy for argininosuccinic aciduria.

Consensus of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) and the Brazilian Ministry of Health - General management of blood and blood products on the tests necessary for the release of exceptional medicines for sickle cell disease.

To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea. This consensus aims to reduce the number of mandatory exams needed to access the drug, prioritizing the requesting physician's report, without affecting patient safety.

O armário: Fruiting phenology data for 4,462 plant taxa in Portugal (1926-2013).

Species phenology - the timing of key life events - is being altered by ongoing climate changes with yet underappreciated consequences for ecosystem stability. While flowering is generally occurring earlier, we know much less about other key processes such as the time of fruit ripening, largely due to the lack of comprehensive long-term datasets. Here we provide information on the exact date and site where seeds of 4,462 taxa were collected for the Index Seminum (seed exchange catalogue) of the Botanic Garden of the University of Coimbra, between 1926 and 2013. Seeds were collected from spontaneous and cultivated individuals across Portugal, including both native and introduced taxa. The database consists of 127,747 curated records with information on the species, or infraspecific taxa (including authority), and the day and site where seeds were collected. All records are georeferenced and provided with a confidence interval for the collection site. Taxonomy was first curated manually by in-house botanists and then harmonized according to the GBIF backbone taxonomy.

Consensus of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) and the Brazilian Ministry of Health - General management of blood and blood products on the tests necessary for the release of exceptional medicines for sickle cell disease

Abstract To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea. This consensus aims to reduce the number of mandatory exams needed to access the drug, prioritizing the requesting physician's report, without affecting patient safety.

Comparison of three intraoperative analgesic strategies in laparoscopic bariatric surgery: a retrospective study of immediate postoperative outcomes

Abstract Introduction and objectives Multimodal Analgesia (MMA) has shown promising results in postoperative outcomes across a broad spectrum of surgeries, including bariatric surgery. We compared the analgesic effect immediately after Laparoscopic Bariatric Surgery (LBS) of the combined effect of MMA and methadone against two techniques that were based mainly on the use of high-potency medium-acting opioids. Methods Two hundred seventy-one patients were retrospectively reviewed. The primary outcome was postoperative pain score > 3/10 measured by the Verbal Numeric Scale (VNS) during the Postanesthetic Care Unit (PACU) stay. The three protocols of intraoperative analgesia were: (P1) sufentanil at anesthetic induction followed by remifentanil infusion; (P2) sufentanil at induction followed by dexmedetomidine infusion; and (P3) remifentanil at induction followed by MMA including dexmedetomidine, magnesium, lidocaine, and methadone. Only P1 and P2 patients received morphine toward the end of surgery. Poisson regression was used to adjust confounding factors and calculate Prevalence Ratio (PR). Results Postoperative VNS > 3 was recorded in 135 (49.81%) patients, of which 93 (68.89%) were subjected to P1, 25 (18.56%) to P2, and 17 (12.59%) to P3. In the final adjusted model, both anesthetic techniques (P3) (PR = 0.10; 95% CI [0.03-0.28]), and (P2) (PR = 0.42%; 95% CI [0.20-0.90]) were associated with lower occurrence of VNS > 3, whereas age range 20-29 was associated to higher occurrence of VNS > 3 (PR = 3.21; 95% CI [1.22-8.44]) in PACU. Postoperative Nausea and Vomiting (PONV) was distributed as follows: (P1) 20.3%, (P2) 31.25% and (P3) 6.77%; (P3 < P1, P2; p< 0.05). Intraoperative hypotension occurred more often in P3 (39%) compared to P2 (20.31%) and P1 (17.46%) (p< 0.05). Conclusion MMA + methadone was associated with higher incidence of intraoperative hypotension and lower incidence of moderate/severe pain in PACU after LBS.

Is Botulinum Toxin Useful in Systemic Sclerosis Related Peripheral Vasculopathy? A Literature Review / ¿Es la toxina botulínica útil en la vasculopatía periférica de la esclerosis sistémica? Una revisión sistemática

Introduction: In systemic sclerosis (SSc), peripheral vasculopathy presents typically as Raynaud Phenomenon (RP) and Digital Ulceration (DU). Over the last decade, botulinum toxin (BT) has been reported effective in this scenario. Our goal was to review existing literature evaluating the efficacy of BT on RP/DU in SSc. Materials and methods: We performed a search in Pubmed with the MeSH terms “systemic sclerosis” and “botulinum toxin”. Original studies evaluating BT in the treatment of SSc-associated RP/DU were considered for inclusion. Results were screened by title, abstract and full-text. Results: We identified 30 results, of which 5 original papers were included: 2 randomized controlled trials (RCT), 2 case series and 1 case control study, from a total of 133 patients. Only one RCT showed negative results, with worse blood flow in treated arm, but with lower dose of BT. Despite this, all 5 included studies reported improvement of at least 1 RP/hand function outcome measure. Concerning DU healing, resolution of baseline DU at the end of follow-up was reported in 75–100% of the patients, with 1 RCT showing superiority over placebo. The only reported adverse effect was transient hand weakness, affecting only 0–16.7% of patients. BT injection protocols were highly heterogeneous. Conclusion: Despite conflicting results in 1 RCT, evidence points BT as an option in the treatment of SSc-related peripheral vasculopathy. However, future larger prospective trials are necessary to corroborate this hypothesis.(AU)

Introducción: En la esclerosis sistémica (ES), la vasculopatía periférica se presenta normalmente como fenómeno de Raynaud (FR) y ulceración digital (UD). En el último decenio se ha reportado la efectividad de la toxina botulínica (TB) en este escenario. Nuestro objetivo fue revisar la literatura existente que evalúa la eficacia de la TB en el FR/UD en la ES. Materiales y métodos: Realizamos una búsqueda en Pubmed con los términos MeSH «esclerosis sistémica» y «toxina botulínica». Se consideraron para inclusión los estudios originales que evaluaban la TB en el tratamiento del FR/UD asociados a ES. Se cribaron los resultados por título, resumen y texto completo. Resultados: Identificamos 30 resultados, de los cuales se incluyeron 5 documentos originales: 2 ensayos controlados aleatorizados (ECA), 2 series de casos y un estudio de control de caso, de un total de 133 pacientes. Únicamente un ECA reflejó resultados negativos con peor flujo sanguíneo en el brazo tratado, aunque con menor dosis de TB. A pesar de ello, los 5 estudios incluidos reportaron una mejora de al menos una medida del resultado FR/función de la mano. En cuanto a la sanación de la UD, la resolución de la UD basal al final del seguimiento se reportó en el 75-100% de los pacientes, y un ECA reflejó superioridad con respecto al placebo. El único efecto adverso reportado fue debilidad transitoria en la mano, que afectó únicamente al 0-16,7% de los pacientes. Los protocolos de inyección de la TB fueron altamente homogéneos. Conclusión: A pesar de los resultados conflictivos en un ECA, la evidencia apunta a la TB como opción para el tratamiento de la vasculopatía periférica asociada a la ES. Sin embargo, son necesarios ensayos prospectivos futuros más amplios para corroborar esta hipótesis.(AU)

Contratualização de resultados: implantação da gestão regionalizada na Secretaria de Saúde do Distrito Federal / Results contracts: implementation of regionalized management in the Health State of the Federal District

Neste artigo relata-se a experiência da implantação da gestãoregionalizada na Secretaria de Estado da Saúde do Distrito Federal,com a finalidade de reestruturação das ações e serviços de saúdecom vistas à melhoria do acesso e qualidade e criar espaços degestão compartilhada e solidária. O Programa de Gestão Regionalda Saúde definiu os Acordos de Gestão Regional e Local comoinstrumentos de pactuação de metas entre a Administração Central eas Superintendências Regionais de Saúde e destas com suas unidadesassistenciais. Estes instrumentos de contratualização objetivaminstituir um modelo de gestão por resultados nessa Secretaria.

This article reports on the experience of the implementation of regionalized management in the State Department of Health of the Federal District (SES-DF), with the purpose of restructuring health actions and services with a view to improving access and quality and creating spaces for shared and supportive management. The Regional Health Management Program defined the Regional and Local Management Agreements as instruments of goal agreement between the Central Administration and the Regional Superintendencies of Health and of these with its health care units. These contractual instruments aim to establish a results-based management model at SES-DF.

Literacia Pré-Escolar e Desempenho na Leitura na Instrução Primária / Preschool Literacy and Reading Performance in Primary School

RESUMO A literacia pré-escolar tem recebido uma crescente atenção. Neste estudo, participaram 70 crianças que iniciaram o 1.º ano de escolaridade no ano letivo 2009/10 e que, na educação pré-escolar, frequentaram três instituições com abordagens diferenciadas de leitura e de escrita. O estudo inclui quatro medidas repetidas no tempo nas áreas da fonologia, reconhecimento de letras, palavras e velocidade de leitura. Os resultados sugerem que a abordagem específica à leitura na educação pré-escolar condiciona o desempenho da leitura na instrução primária. Contudo, essa influência parece ser atenuada com o tempo. Por outro lado, verificou-se que sujeitos que receberam instrução direta de leitura em idade pré-escolar (mas não treino fonológico) obtêm resultados significativamente superiores em tarefas fonológicas aos de sujeitos que receberam treino fonológico prolongado (mas não de leitura).

ABSTRACT The debate regarding kindergarten literacy has received increasing attention and social visibility. In this study, participants are 70 children who attended first grade in 2009/2010. Participants attended three kindergartens with unique approaches to reading. A four wave repeated measure design was conducted to investigate the growth of the children in phonological awareness, letter recognition, word recognition, and reading speed. Results suggest that the approach towards reading and writing in kindergarten affects children's performance in grades 1 and 2 of elementary school. This influence, however, seems to fade over time. Moreover, results show that children who received formal reading instruction in kindergarten, perform significantly better in phonological tasks than children who received direct and extended phonological instruction.

Imunodeficiência comum variável: relato de caso e revisão da literatura / Common variable immunodeficiency: case report and literature review

A Imunodeficiência comum variável (ICV) é a segunda mais frequente das imunodeficiências primárias e tem como critérios diagnósticos: infecções recorrentes; idade acima de 4 anos; níveis reduzidos de IgG, de IgA e/ou IgM; exclusão de outras causas de hipogamaglobulinemias; ausência de isohemaglutininas e de resposta vacinal. O artigo visa relatar o caso de um paciente com ICV associada a infecções/infestações recorrentes e esplenomegalia. Trata-se de menino de 8 anos com antecedente de oito pneumonias, giardíase recorrente e esplenomegalia com pesquisa de anticorpos anti-HIV negativa. Apresentava IgG=614mg/dl, IgM= 19mg/dl e IgA=24mg/dl; CD4=24% (742 células/mm3), CD8= 70% (2150 células/mm3), CD19 = 247células/mm3 e linfócito B total = 9,2%. A ICV pode se manifestar através de cinco fenótipos clínicos: infecções, autoimunidade, infiltração linfocítica, malignidade e enteropatia. Neste caso, o paciente apresentava infecções/infestações recorrentes, as dosagens de imunoglobulinas encontram-se abaixo do percentil 3 para idade e o de linfócitos B, no limiar mínimo. Reiteramos a importância do diagnostico de ICV em paciente com infecções/infestações recorrentes e esplenomegalia pela importante prevalência na população.

Platelet-rich plasma in diabetic dry eye disease / Plasma rico em plaquetas no olho seco diabético

ABSTRACT Objective: To evaluate the effectiveness of autologous Plasma- rich platelet (PRP) in symptomatic dry eye of diabetic patients. Design A prospective single group interventional study. Participants: Twelve diabetic patients with refractory dry eye disease. Methods Patients were treated with autologous PRP eye drops four times a day for a month. Dry eye symptoms and its frequency, according to DEWS studies, tear film breakup time, improvement of visual acuity lines, and Schirmer test. Results: All the patients had some symptomatic improvement regarding dryness, itching, burning and redness (p=0.002). Five patients, 41.66% (5/12) had improvement of 1 or more lines of visual acuity in both eyes, 50% and 58.33% had no alteration in right and left eye, respectively (p=0.14). Considering Schirmer test, 66.66% (8/12) had improvement in the test value, 25% (3/12) had no alteration in this test value and 8.33% (1/12) had a reduced value in the test after treatment.(p=0.04). Considering the value of BUT test 58.33% (7/12) had improvement in the test value and 41.66% (5/ 12) had no alteration in this test value (p=0.018). Conclusions PRP is safe and an interesting alternative therapy in symptomatic diabetic dry eye. More clinical trials are required to create specific protocols to this treatment.

RESUMO Objetivo: Avaliar a eficácia do colírio de concentrado de plaquetas (CCP) autólogo no olho seco sintomático de pacientes diabéticos. Projeto Um estudo de intervenção único grupo prospectivo. Participantes Doze pacientes diabéticos com doença do olho seco refratário. Métodos Os pacientes foram tratados com colírio de PRP autólogo quatro vezes por dia durante um mês. Sintomas de olho seco e sua frequência, seguindo os critérios de DEWS, tempo de ruptura do filme lacrimal, a melhoria das linhas de acuidade visual e teste de Schirmer. Resultados Todos os pacientes tiveram alguma melhora dos sintomas de ressecamento, coceira, ardor e vermelhidão (p = 0,002). Destes, 41,66% (5/12) tiveram melhora de uma ou mais linhas de acuidade visual em ambos os olhos; 50% e 58,33% não tinham alteração no olho direito e esquerdo, respectivamente (p = 0,14) . Considerando o teste de Schirmer, 66,66% (8/12) tiveram melhora no valor do teste, 25% (3/12) não apresentaram alteração neste valor de teste e de 8,33% (1/12) tiveram um valor reduzido no teste após tratamento. (p = 0,04). Considerando o valor de teste BUT 58,33% (7/12) apresentaram melhora no valor de teste e 41,66% (12/05) não apresentaram alteração neste valor de teste (p = 0,018). Conclusões O CCP é seguro e uma terapia alternativa interessante no olho seco diabético sintomático. Mais ensaios clínicos são necessários para criar protocolos específicos para este tratamento.

The current state of calcium silicate cements in restorative dentistry: a review / Revisión del estado actual de cementos de silicato de calcio en odontología restauradora

ABSTRACT Calcium silicate cements have been used as dental materials for more than twenty years; however, their use in restorative dentistry is more recent. Better mechanical properties and shorter curing times make them suitable for a variety of applications in which they are used as a substitute of dentin, including direct/indirect pulp capping and as cavity base/liner. These materials may also be used to restore enamel temporarily. This article seeks to review the available scientific evidence with a focus on their applications in restorative dentistry. The information was gathered by reviewing original scientific research articles and literature reviews published in journals available in databases such as Medline/Pubmed and Scielo, along with technical information provided by the manufacturers of these cements. This article describes the composition, instructions for use, and curing reaction of calcium silicate cements, as well as the scientific evidence on their applications in restorative dentistry.

RESUMEN Los cementos de silicato de calcio se han aplicado como materiales dentales desde hace más de veinte años; sin embargo, su uso en el área de la odontología restauradora es más reciente. Mejores propiedades mecánicas y menores tiempos de endurecimiento le permiten ser indicados para una variedad de aplicaciones en las que este material se utiliza como sustituto dentinario, entre ellas el recubrimiento pulpar directo/indirecto y como base/liner cavitario. A su vez, también se podría utilizar como material para restaurar esmalte de manera temporal. El presente artículo busca revisar la evidencia científica disponible, enfocándola a sus aplicaciones en odontología restauradora. La información se obtuvo a partir de artículos originales de investigación científica y revisiones de literatura, publicados en revistas disponibles en bases de datos como Medline/Pubmed y Scielo, junto a la información técnica otorgada por los fabricantes de estos cementos. El presente trabajo describe la composición, el modo de empleo, la reacción de fraguado y la evidencia científica sobre las aplicaciones de los cementos de silicato de calcio en odontología restauradora.