Biomarkers of endothelial glycocalyx damage are associated with microvascular dysfunction in resuscitated septic shock patients.

BACKGROUND: Microvascular dysfunction plays a central role in organ dysfunction during septic shock. Endothelial glycocalyx (eGC) damage could contribute to impaired microcirculation. The aim was to assess whether several eGC-damaged biomarkers are associated with microvascular dysfunction in resuscitated septic shock patients. METHODS: This cross-sectional study included resuscitated septic shock patients (N = 31), and a group of healthy individuals (N = 20). The eGC damage biomarkers measured were syndecan-1 (SDC-1), soluble CD44 (CD44s), hyaluronic acid (HYAL) in blood sample; sulfated glycosaminoglycans (GAGs) in urine sample; and thrombomodulin (TBML) in blood sample as biomarker of endothelial cell damage. Microcirculation was assessed through sublingual videocapillaroscopy using the GlycoCheck™, which estimated the perfused vascular density (PVD); the perfused boundary region (PBR), an inverse parameter of the eGC thickness; and the microvascular health score (MVHS). We defined a low MVHS (<50th percentile in septic patients) as a surrogate for more impaired microvascular function. RESULTS: The SDC-1, CD44s, TBML and GAGs levels were correlated with impaired microvascular parameters (PVD of vessels with diameter < 10 µm, MVHS and flow-adjusted PBR); p < 0.05 for all comparisons, except for GAGs and flow-adjusted PBR. The SDC-1 [78 ng/mL (interquartile range (IQR) 45-336) vs. 48 ng/mL (IQR 9-85); p = 0.052], CD44s [796ρg/mL (IQR 512-1995) vs. 526ρg/mL (IQR 287-750); p = 0.036], TBML [734ρg/mL (IQR 237-2396) vs. 95ρg/mL (IQR 63-475); p = 0.012] and GAGs levels [0.42 ρg/mg (IQR 0.04-1.40) vs. 0.07 ρg/mg (IQR 0.02-0.20); p = 0.024]; were higher in septic patients with more impaired sublingual microvascular function (low MVHS vs. high MVHS). CONCLUSION: SDC-1, CD44s, TBML and GAGs levels were associated with impaired microvascular function in resuscitated septic shock patients.

Cerebral palsy and sleep: nonpharmacological treatment and impact on the life of caregivers - an integrative review.

BACKGROUND: Children with cerebral palsy have a higher prevalence of sleep disorders, with numerous factors associated with a negative impact on the quality of life of caregivers. OBJECTIVE: To identify factors related to sleep disorders, nonpharmacological treatment, and the impact on the lives of caregivers. METHODS: The present literature review was carried out in the Latin American and Caribbean Center on Health Sciences Information (BIREME), the Cochrane Library, Scopus, PubMed, the Cumulative Index to Nursing and Allied Health Literature (CINAHL), PsycInfo, WorldCat, Web of Science, Latin American Literature on Health Sciences (LILACS), and Excerpta Medica Database (EMBASE), with the descriptors sleep, child, cerebral palsy, parents, and nursing. Studies available in Portuguese, English, or Spanish, published between 2010 and 2020, were our inclusion criteria. A total of 29 articles were included in the present review. RESULTS: We considered nonpharmacological interventions effective support measures to drug-based treatments. The main sleep disorders in children with cerebral palsy are insomnia, parasomnias, nightmares, sleep bruxism, sleepwalking, sleep talking, disorders of initiation and maintenance of sleep, and sleep hyperhidrosis. Most studies point to a reduction in the quality of life of caregivers whose children have sleep disorders. CONCLUSION: Our review suggests the effectiveness of nonpharmacological treatments combined with the use of medications. Measures such as changes in sleep environment and routine are favorable strategies to improve sleep quality. In addition, children with sleep disorders negatively impact the quality of life of their caregivers.

ANTECEDENTES: Crianças com paralisia cerebral apresentam maior prevalência de distúrbios do sono, com inúmeros fatores associados a um impacto negativo na qualidade de vida dos cuidadores. OBJETIVO: Identificar fatores relacionados aos distúrbios do sono, o tratamento não farmacológico e o impacto na vida dos cuidadores. MéTODOS: Esta revisão da literatura foi realizada no Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde (BIREME), Biblioteca Cochrane, Scopus, PubMed, Cumulative Index to Nursing and Allied Health Literature (CINAHL), PsycInfo, WorldCat, Web of Science, Literatura Latino-Americana em Ciências da Saúde (LILACS) e Excerpta Medica Database (EMBASE), com os descritores sono, criança, paralisia cerebral, pais e enfermagem. Estudos disponíveis em português, inglês ou espanhol, publicados entre 2010 e 2020, foram nossos critérios de inclusão. Ao todo, 29 artigos foram incluídos nesta revisão. RESULTADOS: Consideramos as intervenções não farmacológicas medidas eficazes de apoio aos tratamentos medicamentosos. Os principais distúrbios do sono em crianças com paralisia cerebral são: insônia, parassonias, pesadelos, bruxismo do sono, sonambulismo, falar dormindo, distúrbios de iniciação e manutenção do sono e hiperidrose do sono. A maioria dos estudos aponta redução na qualidade de vida de cuidadores de crianças com distúrbios do sono. CONCLUSãO: Nossa revisão sugere a eficácia de tratamentos não farmacológicos combinados com o uso de medicamentos. Medidas como mudanças no ambiente e na rotina do sono são estratégias favoráveis para melhorar a qualidade do sono. Além disso, crianças com distúrbios do sono provocam impactos negativos na qualidade de vida de seus cuidadores.

The effect of constraint-induced movement therapy assessed by accelerometry: the impact on daytime activity and sleep in children with cerebral palsy / O efeito da terapia por contensão induzida avaliada por meio de acelerômetros: o impacto na atividade diurna e no sono em crianças com paralisia cerebral

Abstract Introduction Spastic hemiparetic cerebral palsy (CP) is the most prevalent type of CP. Children with spastic hemiparesis experience difficulties when using their affected upper extremities, and one effective treatment is the Constraint-Induced Movement Therapy (CIMT). The study of rest-activity patterns provides information on children's daily activities with spastic hemiparetic CP during the day and sleep. Objective To investigate the effect of CIMT on the rest-activity patterns in children with spastic hemiparetic CP vs in a healthy group. Methods Nonrandomized controlled trial was conducted at the Neuropediatric Center of the Hospital de Clínicas Complex, in Curitiba, Brazil. Children with spastic hemi-paretic CP between 5 and 16 years old participated in the study group and receive the CIMT. The healthy group was composed of children between 5 and 15 years old. Both groups used accelerometer to record rest-activity patterns, that may be studied through nonparametric variables of accelerometer: M10 (an individual's most active 10h); L5 (an individual's least active 5h); and RA (relative amplitude of the circadian rest-activity patterns). Results Forty-five children were recruited, and 38 were included in the analyses (19 allocated to each group). In the study group, there was a significant increase in M10 and L5 (p < 0.001) after CIMT. The values of M10 and L5 were significantly higher (p < 0.001) in the healthy group compared to the study group after CIMT. Conclusion Our results showed that children with spastic hemiparetic CP became more active and participant in their daily life during the day as well as more efficient sleeping.

Resumo Introdução A paralisia cerebral (PC) hemiparética espástica é o tipo de PC mais prevalente. Crianças com hemiparesia es-pástica apresentam dificuldades ao usar as extremidades superiores afetadas e um tratamento eficaz é a Terapia por Contensão Induzida (TCI). O estudo dos padrões de atividade-repouso fornece informações sobre as atividades diárias de crianças com PC hemiparética espástica durante o dia e o sono. Objetivo Investigar o efeito da TCI nos padrões de repouso-atividade em crianças com PC hemiparética espástica versus um grupo saudável. Métodos Realizou-se um ensaio controlado não randomizado no Centro de Neuropediatria do Complexo do Hospital de Clínicas, Curitiba, Brasil. Crianças com PC hemi-parética espástica entre 5 e 16 anos participaram do grupo de estudo e receberam a TCI. O grupo saudável foi composto por crianças entre 5 e 15 anos. Ambos os grupos utilizaram um acelerômetro para registrar padrões de atividade-repouso, os quais podem ser estudados através de variáveis não paramé-tricas do acelerômetro: M10 (10h mais ativas de um indivíduo); L5 (5h menos ativas de um indivíduo); e AR (amplitude rela-tiva dos padrões de atividade-repouso). Resultados Foram recrutadas 45 crianças e 38 foram incluídas nas análises (19 alocadas em cada grupo). No grupo de estudo, houve aumento significativo de M10 e L5 (p < 0,001) após TCI. Os valores de M10 e L5 foram significativamente maiores (p < 0,001) no grupo saudável em comparação ao grupo de estudo após TCI. Conclusão Os resultados do presente estudo mostraram que crianças com PC hemiparética espástica tornaram-se mais ativas e participantes de sua vida diária durante o dia, bem como dormiram mais eficientemente.

Adherence to PKU guidelines among patients with phenylketonuria: A cross-sectional national multicenter survey-based study in Argentina, Brazil, and Mexico.

Objective: To characterize adherence to Phenylketonuria (PKU) management practices among PKU patients treated at reference sites around Argentina, Brazil, and Mexico. Methods: This is a retrospective, observational, multicenter, and multinational survey-based study using aggregate data. From an initial list of 40 sites, 22 clinicians expressed interest in completing the survey, with 20 clinicians from 20 unique sites fulfilling all the study criteria. The Survey contained 28 questions, including respondent's clinic characteristics, clinic PKU treatment recommendations, and patient adherence to clinic recommendations. Survey was available in local languages, and the respondents were asked to consult their clinic records to complete their responses. Adherence was assessed by target blood phenylalanine (Phe), target blood testing frequency, and clinic visits. Results: A total of 1077 (out of 1377) actively managed PKU patients (seen in the clinic in the last 3 years) from 13 clinics in Brazil, six in Argentina, and one in Mexico were analyzed. Upper blood Phe target was set over 360 µMol/L in 70% of the clinics for adult patients. Around 40% of the patients >30 years old had Phe blood tests done twice a year or less, with 60% of the clinics recommending semestral visits for adults <30 years old. Twice a month was the most common frequency of visits for <1 year old. The COVID-19 pandemic was a disruptor for frequency of visits and exams. Conclusions: These results show that there is still room for improvement in terms of adherence, namely in adults and older children. More efforts must be made to educate patients and healthcare professionals about the importance of treatment adherence, accompanied by public policies that expand access to pharmacological and dietary treatment with diversity and quality to improve adherence to adequate blood Phe levels.

Coronary Artery Bypass Grafting: A Comparative Exercise between Patients from the BYPASS Registry and Patients from a University Hospital.

INTRODUCTION: The coronary artery bypass grafting (CABG) data provided by the Brazilian Registry of Cardiovascular Surgeries in Adults (BYPASS) Registry is a Brazilian reality. OBJECTIVE: To carry out a comparative exercise between the BYPASS Registry published data and data from patients operated on in a randomly chosen period (2013-2015) at Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (HCFMRP-USP). METHODS: This is a retrospective study reviewing 173 electronic medical records of CABG patients from the HCFMRP-USP. These data were compared with the BYPASS Registry published data. Chi-square test was used to verify the changes within the prevalence of adequate/inadequate biochemical tests before and after surgery. The sample was divided into groups consistent with cardiopulmonary bypass (CPB) time (CPB ≤ 120 minutes and CPB > 120 minutes). For the complications, prevalence by the chi-square test was adopted. Significant P-values are < 0.05. RESULTS: The comparative operative data of the BYPASS Registry and the HCFMRP-USP patients were quite similar, except for the isolate use of only arterial grafts, which was more frequent on HCFMRP-USP patients (30.8% vs. 15.9%), and the use of radial artery, also more frequent on HCFMRP-USP patients (48.8% vs. 1.1%). CONCLUSION: The comparative study suggested that the BYPASS Registry should be a reference for CABG quality control.

Burnout and depression among anesthesiology trainees in the United States: An updated National Survey.

STUDY OBJECTIVE: A high prevalence of burnout, depression and suicidal ideation has been reported among anesthesiology trainees. Over the last decade, there has been a significant emphasis on the development of wellness programs in academic departments to mitigate the prevalence and effects of burnout during anesthesiology training. Therefore, we aimed to reevaluate the prevalence of burnout and depression in anesthesiology trainees in the United States. DESIGN: Cross-sectional survey. SETTING: Anesthesiology Department. PATIENTS: A nationally representative sample of 1000 anesthesiology trainees in the United States. MEASUREMENTS: A 33-item questionnaire consisting of 1) burnout, 2) depression, 3) job satisfaction, 4) family support, 5) work characteristics, 6) demographic factors, 7) self-reported errors, and 8) impact of COVID-19 pandemic. RESULTS: We received 384 responses. Twenty-four percent (91/384) of the respondents met the criteria for high burnout risk and 58/384 (15%) of the respondents screened positive for depression. Multivariable analysis revealed that: (1) hours per week > 70, (odds ratio [OR; 95% confidence interval {CI}] = 3.1 [1.4-6.8], P=0.005 and (2) overnight calls per month > 7 (OR [95% CI] = 2.5 [1.0- 6.0], P=0.03 were independent factors for increased odds of burnout whereas the presence of (3) married/domestic partnership (OR [95% CI} = 0.52 [0.32-0.85], P=0.01 was associated with lower odds of burnout and/or depression. Ten percent (4/40) of high burnout/depression residents reported that they often times fall short in the quality of care provided to patients compared to 0.36% (1/275) residents with low burnout/depression scores, P < 0.001. Similarly, 22% (9/40) of high burnout/depression residents reported that they often times did not have enough attention to their patients compared to 4% (11/275) residents with low burnout/depression scores, P < 0.001. CONCLUSIONS: We report a still concerning but significantly lower rate of burnout and depression in anesthesiology trainees than previously reported a decade ago. This suggests that efforts on wellness implemented by academic programs have positively impacted the work experience of anesthesia trainees.

Translation, Cross-Cultural Adaptation, and Validation to Brazilian Portuguese of the Cerebellar Cognitive Affective/Schmahmann Syndrome Scale.

Cerebellar cognitive affective syndrome (CCAS) is characterized by deficits in executive functions, language processing, spatial orientation, and affect regulation in patients with cerebellar disease. The symptoms can occur isolated or along with motor and coordination symptoms. The aim of our study was to translate and culturally adapt the CCAS scale to Brazilian Portuguese and validate the scale in our population. We performed a cross-sectional study with patients with primary and secondary ataxia. The study included 111 individuals, aged between 20 and 80 years, of both genders, 20 without cognitive and/or affective complaints who participated in the pre-test phase, 40 with cerebellar disease (hereditary/neurodegenerative ataxia or acquired/secondary cerebellar ataxia), and 51 healthy controls with no evidence of cognitive impairment and no affective symptoms matched for sex, age, and educational level. The scale was translated, culturally adapted, and validated. Statistical analysis of the data was performed, with association tests, mean comparison, and ROC curve analysis. Based on the analysis of the ROC curve, optimal cutoff values ​were found for each subitem of the scale. The translated and adapted scale has good internal consistency, is reproducible, has good reliability, and has the potential to be a reliable tool for screening cognitive symptoms in patients with cerebellar disease.

Coronary Artery Bypass Grafting: A Comparative Exercise between Patients from the BYPASS Registry and Patients from a University Hospital

ABSTRACT Introduction: The coronary artery bypass grafting (CABG) data provided by the Brazilian Registry of Cardiovascular Surgeries in Adults (BYPASS) Registry is a Brazilian reality. Objective: To carry out a comparative exercise between the BYPASS Registry published data and data from patients operated on in a randomly chosen period (2013-2015) at Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (HCFMRP-USP). Methods: This is a retrospective study reviewing 173 electronic medical records of CABG patients from the HCFMRP-USP. These data were compared with the BYPASS Registry published data. Chi-square test was used to verify the changes within the prevalence of adequate/inadequate biochemical tests before and after surgery. The sample was divided into groups consistent with cardiopulmonary bypass (CPB) time (CPB ≤ 120 minutes and CPB > 120 minutes). For the complications, prevalence by the chi-square test was adopted. Significant P-values are < 0.05. Results: The comparative operative data of the BYPASS Registry and the HCFMRP-USP patients were quite similar, except for the isolate use of only arterial grafts, which was more frequent on HCFMRP-USP patients (30.8% vs. 15.9%), and the use of radial artery, also more frequent on HCFMRP-USP patients (48.8% vs. 1.1%) Conclusion: The comparative study suggested that the BYPASS Registry should be a reference for CABG quality control.

Serious mental illness in women.

PURPOSE OF REVIEW: Psychiatric illnesses are very prevalent in the United States and impact women and men differently. In this review, we will explore some gender differences in the expression of psychopathology, discuss the most common serious mental illnesses (SMI) affecting women, and review treatment options according to specific life stages. We hope to raise awareness of these issues and consequently improve outcomes for women with serious mental illness. RECENT FINDINGS: SMI have different rates and are manifested differently in women and men because of biological, psychological, social, and cultural factors. Some SMI are more prevalent in women whereas others uniquely affect them during particular life stages. Even in disorders that have a similar prevalence in men and women or are more prevalent in men, the presentation, course, management, and repercussions can vary significantly between the two genders. Medical and psychiatric comorbidities, which directly influence treatment, prognosis, and disability, are more common in women. SUMMARY: Several differences in the gender expression of SMI have not yet been fully described. It is important to become familiar with important characteristics of SMI in women, including biological determinants, treatment differences, and psychosocial aspects. Recognizing gender biases, cultural considerations, and adaptive responses can help identify women at risk, promote early recognition of symptoms, and prompt interventions that may lead to improved outcomes.

Editorial Perspective: The mental health impact of school closures during the COVID-19 pandemic.

School closures were one of the earlier actions taken as the world tried to contain the COVID-19 outbreak, a decision impacting nearly three-fourths of the learners worldwide. One year into the pandemic, over 200 million students still faced disruptions to their education. Nearing the end of another academic year that has occurred entirely during the pandemic, many schools remained fully or partially closed for in-person education, altering routines and representing potential hardships to the next generation and their families. This article will explore the potential impacts of school closures on children and adolescents related to increased screen time, irregular sleep patterns, less balanced diets, learning difficulties, and changes in social interaction. In addition, we note broader familial difficulties likely accompanying the loss of the mitigating effects of schools and their associated support systems, such as parental stress and work responsibilities, loss of employment, intimate partner violence, child abuse and neglect, and parental substance abuse, which appear to have increased during the pandemic. Considering what is known about the impact from previous natural catastrophes, the populations at risk, and the early but not yet definitive data from the current pandemic, we stress the urgent need for robust data as we prepare for a new academic year in the Northern Hemisphere. Although we currently lack consistent and complete data, signs for concern include a rise in pediatric emergency room visits for mental health and substance use issues in many parts of the world. This paper aims to stimulate a discussion about the potential mental health effects of school closures for children and adolescents in the context of a pandemic.

Sapropterin dihydrochloride therapy in dihydropteridine reductase deficiency: Insight from the first case with molecular diagnosis in Brazil.

Tetrahydrobiopterin (BH4) is a cofactor that participates in the biogenesis reactions of a variety of biomolecules, including l-tyrosine, l-3,4-dihydroxyphenylalanine, 5-hydroxytryptophan, nitric oxide, and glycerol. Dihydropteridine reductase (DHPR, EC 1.5.1.34) is an enzyme involved in the BH4 regeneration. DHPR deficiency (DHPRD) is an autosomal recessive disorder, leading to severe and progressive neurological manifestations, which cannot be exclusively controlled by l-phenylalanine (l-Phe) restricted diet. In fact, the supplementation of neurotransmitter precursors is more decisive in the disease management, and the administration of sapropterin dihydrochloride may also provide positive effects. From the best of our knowledge, there is limited information regarding DHPRD in the past 5 years in the literature. Here, we describe the medical journey of the first patient to have DHPRD confirmed by molecular diagnostic methods in Brazil. The patient presented with two pathogenic variants of the quinoid dihydropteridine reductase (QDPR) gene-which codes for the DHPR protein, one containing the in trans missense mutation c.515C>T (pPro172Leu) in exon 5 and the other containing the same type of mutation in the exon 7 (c.635T>C [p.Phe212Ser]). The authors discuss their experience with sapropterin dihydrochloride for the treatment of DHPRD in this case report.

A Glass Ceiling Was Shattered in American Politics: When Will Medicine Follow?

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The relationship between gross motor function impairment in cerebral palsy and sleeping issues of children and caregivers.

AIM: To investigate, among children and adolescents with cerebral palsy (CP), the relationship between impairment of the gross motor function and: (i) child sleep disorders; (ii) the need for nocturnal support; and (iii) the quality of sleep of their caregivers. METHODS: For children, we considered their scores on the gross motor function measure (GMFM-88) and on the sleep disturbance scale for children (SDSC), besides analyzing qualitative features about their sleep. For caregivers, we considered their scores in the Pittsburgh sleep quality index (PSQI). RESULTS: Our sample was comprised of 87 participants with mean age of 11.4 years old (±3.4). We observed correlations between GMFM-88 and disorders of initiating and maintaining sleep (DIMS) (r = -0.22; p = 0.039), sleep-wake transition disorders (SWTD) (r = 0.26; p = 0.017) and disorders of arousal (DA) (r = 0.23; p = 0.033). Children receiving nocturnal support presented lower scores in the GMFM-88 (p = 0.001) and higher scores in the SDSC (p = 0.029). For the caregivers, we found no correlation between GMFM-88 and PSQI. Nonetheless, their PSQI scores correlated with the SDSC scores (r = 0.24; p = 0.027). CONCLUSION: Impairment of the gross motor function correlated with DIMS and the need for nocturnal support but might not have an impact on the caregivers' sleep, which in turn correlated with child sleep disorders.