Pediatric Liver Transplantation: Caregivers' Quality of Life.

Purpose: The survival rate of pediatric patients undergoing liver transplantation has increased considerably. Despite this, the period after transplantation is still complex and poses several challenges to the recipient's family, which is responsible for care management. Recently, more attention has been paid to the impact of this complex procedure on the quality of life of caregivers. Hence, this study is aimed at assessing the quality of life of caregivers of patients who have undergone liver transplantation and the aspects that influence it. Methods: This was an observational and cross-sectional study. From November 2020 to January 2021, short-form-36 questionnaires and additional questions were given to the main caregivers of children and adolescents who underwent pediatric liver transplantation. Results: Thirty-eight questionnaires were completed and the results revealed a lower quality of life in comparison to Brazilian standards, primarily in the mental domains (41.8±14.1 vs. 51.1±2.8; p<0.001). It did not show a significant association with socioeconomic or transplant-related factors, but it did show a negative impact on parents' perception of the child's health. Parents who reported worse health status for their children had a lower mental quality of life (44.1±13.8 vs. 33.3±12.6; p<0.05). Conclusion: The caregivers of transplanted children have a lower quality of life than those of the local population. Psychological assistance should be routinely provided to parents for long-term follow-up to mitigate potential negative effects on the transplanted child's care.

Protocols of Investigation of Neonatal Cholestasis-A Critical Appraisal.

Neonatal cholestasis (NC) starts during the first three months of life and comprises extrahepatic and intrahepatic groups of diseases, some of which have high morbimortality rates if not timely identified and treated. Prolonged jaundice, clay-colored or acholic stools, and choluria in an infant indicate the urgent need to investigate the presence of NC, and thenceforth the differential diagnosis of extra- and intrahepatic causes of NC. The differential diagnosis of NC is a laborious process demanding the accurate exclusion of a wide range of diseases, through the skillful use and interpretation of several diagnostic tests. A wise integration of clinical-laboratory, histopathological, molecular, and genetic evaluations is imperative, employing extensive knowledge about each evaluated disease as well as the pitfalls of each diagnostic test. Here, we review the difficulties involved in correctly diagnosing the cause of cholestasis in an affected infant.

Loss of FOCAD, operating via the SKI messenger RNA surveillance pathway, causes a pediatric syndrome with liver cirrhosis.

Cirrhosis is usually a late-onset and life-threatening disease characterized by fibrotic scarring and inflammation that disrupts liver architecture and function. While it is typically the result of alcoholism or hepatitis viral infection in adults, its etiology in infants is much less understood. In this study, we report 14 children from ten unrelated families presenting with a syndromic form of pediatric liver cirrhosis. By genome/exome sequencing, we found recessive variants in FOCAD segregating with the disease. Zebrafish lacking focad phenocopied the human disease, revealing a signature of altered messenger RNA (mRNA) degradation processes in the liver. Using patient's primary cells and CRISPR-Cas9-mediated inactivation in human hepatic cell lines, we found that FOCAD deficiency compromises the SKI mRNA surveillance pathway by reducing the levels of the RNA helicase SKIC2 and its cofactor SKIC3. FOCAD knockout hepatocytes exhibited lowered albumin expression and signs of persistent injury accompanied by CCL2 overproduction. Our results reveal the importance of FOCAD in maintaining liver homeostasis and disclose a possible therapeutic intervention point via inhibition of the CCL2/CCR2 signaling axis.

Progressive Familial Intrahepatic Cholestasis Type 2 and Recurrence After Liver Transplantation: A Case Report.

Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rare autosomal recessive disorder caused by mutations in the ABCB11 gene. Clinical manifestations include cholestasis with low γ-glutamyltransferase (GGT), hepatosplenomegaly, and severe pruritus. Liver transplantation is required for individuals with progressive liver disease or failure of the bypass procedure and has been considered curative. However, in the case of PFIC2, although bile salt excretory pump (BSEP) deficiency is a liver-specific condition rather than a systemic disease, evidence of recurrent BSEP disease has been shown in a small proportion of allografts. We describe an unusual case of a 21-year-old individual with PFIC2 and evidence of recurrent BSEP disease after liver transplantation, with clinical and laboratory improvement after pulse therapy with methylprednisolone for 3 days and adjustment of oral immunosuppression. This case report highlights the recurrence of PFIC2 in patients post liver transplant. It also emphasizes the importance of clinical suspicion, which should be considered in cases of posttransplant cholestasis in PFIC2 patients, especially those with low γ-glutamyltransferase (GGT) and without signs of acute graft rejection. Having knowledge of the condition favors a targeted diagnostic approach and contributes to early therapeutic management and a higher success rate.

CD40 Ligand Deficiency in Latin America: Clinical, Immunological, and Genetic Characteristics.

CD40 ligand (CD40L) deficiency is a rare inborn error of immunity presenting with heterogeneous clinical manifestations. While a detailed characterization of patients affected by CD40L deficiency is essential to an accurate diagnosis and management, information about this disorder in Latin American patients is limited. We retrospectively analyzed data from 50 patients collected by the Latin American Society for Immunodeficiencies registry or provided by affiliated physicians to characterize the clinical, laboratory, and molecular features of Latin American patients with CD40L deficiency. The median age at disease onset and diagnosis was 7 months and 17 months, respectively, with a median diagnosis delay of 1 year. Forty-seven patients were genetically characterized revealing 6 novel mutations in the CD40LG gene. Pneumonia was the most common first symptom reported (66%). Initial immunoglobulin levels were variable among patients. Pneumonia (86%), upper respiratory tract infections (70%), neutropenia (70%), and gastrointestinal manifestations (60%) were the most prevalent clinical symptoms throughout life. Thirty-five infectious agents were reported, five of which were not previously described in CD40L deficient patients, representing the largest number of pathogens reported to date in a cohort of CD40L deficient patients. The characterization of the largest cohort of Latin American patients with CD40L deficiency adds novel insights to the recognition of this disorder, helping to fulfill unmet needs and gaps in the diagnosis and management of patients with CD40L deficiency.

Estratégias da gestão estadual da atenção básica diante da pandemia de covid-19, Bahia, 2020/2021 / Primary care state management strategies for the covid-19 pandemic, Bahia, 2020/2021 / Estrategias de gestión estadual de atención primaria ante la pandemia del covid-19, Bahía, 2020/2021

A pandemia de Covid-19, maior acontecimento sanitário e com diversas implicações no cenário sociopolítico mundial nos últimos cem anos, trouxe consigo a necessidade de ir além das recomendações iniciais voltadas ao distanciamento físico, imposto como medida sanitária para passar a articular diferentes ações. Nesse sentido, este texto tem como objetivo apresentar a experiência da Diretoria de Atenção Básica (DAB), relativa ao desenvolvimento de estratégias com os municípios baianos para o enfrentamento da Covid-19. Algumas estratégias foram definidas, entre elas: ações de telessaúde; de apoio institucional; e de avaliação e monitoramento. O advento da pandemia tem exigido respostas rápidas e inovadoras da DAB diante do desafio de reestruturação desse nível de atenção, como o uso das tecnologias de informação e comunicação, que possibilitaram uma aproximação ainda maior entre a gestão estadual e municípios, assim como permitiram um melhor monitoramento e apoio às ações no nível local. As três estratégias têm se mostrado de grande relevância para o fortalecimento das ações da Atenção Básica.

The COVID ­ 19 pandemic, the biggest sanitary event and with several implications in the world  socio-political scenario in the last century, brought with in the need to go beyond the premise of social distancing imposed as a sanitary measure to start articulating different actions. In this context, the experience reported here had the general objective of describing the strategies  developed by the State Primary Care Directorate (DAB) with Bahian municipalities to face the COVID ­ 19 pandemic. Some strategies were defined, among them, the redefinition of the space of the Collegiate of Primary Care Coordinators (COCAB),which started to happen on virtual platforms for meetings and with greater frequency; the implementation of Telehealth/BA, with more web lectures focused on topics related to the pandemic and teleinterconsultation for higher education professionals in Primary Care om post ­ COVID-19 care and rehabilitation, and the third strategy comprised the implementation of thecnology for active search of cases of Flu Syndrome, COVID-19 and SRAG, for early identification and better monitoring of the epidemiological situation. This thecnology was developed through Sheet B ­ Flu Syndrome (SG) and the Indicator Base System. The onset of the pandemic has required and innovative responses from DAB to the challenge of restructuring at this level of care, such as the use of information and communication technologies, which allowed for an even greater approximation of state management and municipalities, as well as allowing for better monitoring and support for actions at the local level. The three strategies have been shown to be of great relevance to support actions at the local level, from the perspective of strengthening actions aimed at primary care.

La pandemia COVID ­ 19, el mayor evento sanitario y con varias implicaciones em el escenario sociopolítico mundial en el último siglo, trajo consigo la necesidad de ir más allá de la premisa del distanciamiento social impuesto como medida sanitaria para comenzar a articular diferentes acciones. En este contexto, la experiencia aquí reportada tuvo el objetivo general de describir las estrategias desarrolladas por la Dirección Estatal de Atención Primaria (DAB) con los municipios bahianos para enfrentar la pandemia COVID-19. Se definieron algunas estrategias, entre ellas, la redefinición del espacio del Colegiado de Coordinadores de Atención Primaria (COCAB), que comenzó a ocurrir en plataformas virtuales para reuniones y con mayor frecuencia; la implementación de Telesalud / BA, con más conferencias web enfocadas en temas relacionados con la pandemia y la teleinterconsulta para profesionales de educación superior en Atención Primaria en la atención y rehabilitación post-COVID-19, y la tercera estrategia comprendió la implementación de tecnología para la búsqueda activa de casos de Síndrome Gripal, COVID-19 y SRAG,  para una identificación temprana y un mejor seguimiento de la situación epidemiológica. Esta tecnología se desarrolló a través de la Hoja B - Síndrome de la gripe (SG) y el Sistema de base de indicadores. El inicio de la pandemia ha requerido respuestas rápidas e innovadoras por parte de la DAB al desafío de reestructurar en este nivel de atención, como el uso de tecnologías de la información y la comunicación, que permitieron una aproximación aún mayor de la gestión estatal y los municipios, así como permitiendo un mejor seguimiento y apoyo a las acciones a nivel local. Las tres estrategias se han mostrado de gran relevancia para apoyar acciones a nivel local, desde la perspectiva de fortalecer las acciones dirigidas a la atención primaria.

Autoimmune Hepatitis: Predictors of Native Liver Survival in Children and Adolescents.

OBJECTIVE: To determine predictors of native liver survival (NLS) in children and adolescents with autoimmune hepatitis (AIH). STUDY DESIGN: The medical records of children and adolescents with AIH were reviewed. A questionnaire was used to collect data on clinical presentation, biochemical and histologic findings, and treatment. RESULTS: A total of 819 patients were included, 89.6% with AIH-1 and 10.4% with AIH-2. The median age (months) at onset was 108 (min 6; max 210; IQR 59). The female sex was predominant (75.8%). The overall survival was 93.0%, with an NLS of 89.9%; 4.6% underwent liver transplantation. The risk of death or liver transplantation during follow-up was 3.2 times greater in patients with AIH-1 (P = .024). Greater levels of aspartate aminotransferase, alanine aminotransferase, serum albumin, platelet, and normal international normalized ratio at the initial presentation were associated with longer NLS (P = .046, P = .006, P < .001, P = .001, and P = .019, respectively). Normal C3 levels was associated with longer NLS (P = .017), with a chance of death or liver transplantation during follow-up being 3.4 times greater in patients with C3 below normal. Death or liver transplantation during follow-up was 2.8 times greater in patients with associated sclerosing cholangitis (P = .046). Complete remission favored NLS (P < .001), with a risk of death or liver transplantation 11.7 times greater for patients not achieving remission. CONCLUSIONS: The best predictors of NLS in children and adolescents with AIH were the AIH-2 subtype, a normal C3 at diagnosis, remission during treatment, and normal a cholangiogram during the disease course.

Relationship between beta-adrenergic agonists, calpain system activity and beef texture: A systematic review.

The objective was to conduct a systematic review to evaluate the effects of dietary supplementation with beta-adrenergic agonists on calpains and calpastatin activity in bovine muscle and changes in meat tenderness. A survey was conducted in June 2019 on Science Direct, Web of Science, Scopus, PubMed and Capes Periodicals, using four keyword combinations: agonist and calpain and cattle; agonist and calpain and bovine; agonist and calpain and heifers; agonist and calpain and steers. Thirteen studies were selected, 54% concluded that supplementation with beta-adrenergic agonists increases calpastatin activity, 23% observed increase in their gene expression and 23% reported no effect on activity or expression of this enzyme. Nine studies evaluated the influence of beta-adrenergic agonists supplementation on meat texture and all found an increase in shear force values. There is strong evidence that beta-adrenergic agonists may increase calpastatin activity in the muscle, causing damage to meat tenderness.

Foreign Body Granuloma in the Tongue by a Pequi Spine.

Substances considered foreign to the human organism can penetrate it due to local trauma, initially causing an acute inflammatory response against these substances, involving a neutrophilic infiltrate that, when it fails to deal with these foreign bodies, ends up generating a granulomatous inflammatory response. Granuloma formation has been associated with a variety of conditions. The correct clinical and imaging diagnoses are extremely important for the dentist to choose an appropriate therapeutic approach, aiming at the best possible treatment. This work is aimed at describing a case report of a foreign body granuloma, formed on the tongue, from the penetration of a pequi spine, in a 76-year-old patient, in whom, after an imaging diagnosis with ultrasound, surgical removal of the lesion was performed, and the piece was sent for histopathological examination, which confirmed the initial diagnostic hypothesis of a foreign body granuloma. The initial diagnosis of foreign body granulomas is challenging. For this reason, more sophisticated means of diagnosis such as tomography and magnetic resonance become important in the diagnosis, as they can show with greater clarity and reliability the nature of the lesion and its relationship with adjacent anatomical structures. In the case in question, an ultrasound examination was chosen, which was extremely important as an aid to diagnosis, considerably improving surgical planning. In addition, after surgical removal, the result of the histopathological analysis is essential to determine the definitive diagnosis, as it determines the granulomatous characteristic of the lesion.

Quality of Life in Children and Adolescents with Inflammatory Bowel Disease: Impact and Predictive Factors.

PURPOSE: Inflammatory bowel disease (IBD) in children and adolescents is associated with high morbidity and possibly has a significant negative impact on their quality of life. This study aimed to evaluate the quality of life of children and adolescents with IBD and define the variables that impact these individuals. METHODS: We administered the Pediatric Quality of Life Questionnaire (PedsQL) to 35 children and adolescents diagnosed with IBD and with available quantitative data from clinical records on epidemiology, clinical evolution, complementary tests, medical interventions, and disease activity. Data were evaluated according to the IBD type and compared with a control group of healthy children. RESULTS: The study group showed a significantly lower PedsQL score than the control group (p<0.01). Significant factors contributing to poor overall quality of life included female sex, Crohn's disease, surgery, and food restrictions. Symptoms such as diarrhea and the fear of using public toilets were associated with low physical scores. Feeling sick had a negative impact on the emotional PedsQL scores. Patients with a fear of using public toilets, anthropometric scores below the 3rd percentile, and greater disease activity scored lower in the social domain. Regarding school and psychosocial evaluations, younger children with symptom onset after the age of 2 years had lower scores than younger children with symptom onset before the age of 2 years. CONCLUSION: IBD negatively affects the quality of life of children and adolescents based on its impact on the physical, emotional, social, and psychosocial statuses of these patients.

Theoretical study of solvent effects on the hyperpolarizabilities of two chalcone derivatives / Estudio teórico de los efectos de solvente sobre la hiperpolarizabilidad de dos derivados de la chalcona / Estudo teórico dos efeitos do solvente nas hiperpolarizabilidades de dois derivados da chalcona

Abstract The use of organic as nonlinear optical materials has been intensively explored in the recent years due to the ease of manipulation of the molecular structure and the synthetic flexibility regarding the change of substituent groups. In the present work, the linear and nonlinear properties of two chalcones derivatives (E)-1-(4-methylphenyl)-3-phenylprop-2-en-1-one (4MP3P) and (E)-1-(4-Nitrophenyl)-3-phenylprop-2-en-1-one (4NP3P), that differ by the substituent position at the phenyl ring, were studied in the presence of protic and aprotic solvents simulated by the Polarizable Continuum Model (PCM) at DFT/B3LYP/6-311+G(d) level. The static and dynamic (1064 nm) molecular parameters as the dipole moment, linear polarizability, first and second hyperpolarizabilities were studied as function of the solvent dielectric constant value. The geometrical behavior as the chemical bond angles, torsion angles, and partial charges distribution of the compounds were studied, including calculations of gap energies in various solvents. The obtained results revealed that the substituent change of CH3 (4MP3P) to NO2 (4NP3P) benefits the nonlinear optical properties of the compounds in the presence of the solvent media, the absolute values of the parallel first hyperpolarizability were the ones that present the greater variation.

Resumen El uso de materiales orgánicos como materiales ópticos no lineales se ha explorado intensamente en los últimos años, debido a la facilidad de manipulación de estas estructuras moleculares y la flexibilidad de síntesis en relación con el cambio de grupos sustituyentes. En el presente trabajo, las propiedades lineales y no lineales de dos derivados de chalcona (E)-1-(4-metilfenil)-3-fenilprop-2-en-1-ona (4MP3P) y (E)-1-(4-nitrofenil)-3-fenilprop-2-en-1-ona (4NP3P), los cuales difieren en la posición del sustituyente en el anillo de fenilo, se estudiaron en presencia de disolventes próticos y apróticos simulados por el Modelo Continuo Polarizable a nivel DFT/B3LYP/6-311+G(d). Además, se estudiaron parámetros moleculares estáticos y dinámicos (1064 nm) como el momento dipolar, la polarización lineal y la primera y la segunda hiperpolarización en función del valor constante dieléctrico del disolvente. El comportamiento geométrico se estudió como ángulos de enlace químico, ángulos de torsión y distribución de carga parcial de compuestos, incluidos los cálculos de energía de huecos en varios solventes. Los resultados mostraron que el cambio del sustituyente CH3 (4MP3P) a NO2 (4NP3P) beneficia las propiedades ópticas no lineales de los compuestos en presencia del medio solvente, los valores absolutos de la primera hiperpolarizabilidad paralela fueron los que presentaron la mayor variación.

Resumo O uso de materiais orgânicos como materiais ópticos não lineares tem sido intensamente explorado nos últimos anos, devido à facilidade de manipulação dessas estruturas moleculares e à flexibilidade de síntese em relação à mudança de grupos substituintes. No presente trabalho, as propriedades lineares e não lineares de dois derivados de chalconas (E)-1-(4-metilfenil)-3-fenilprop-2-en-1-ona (4MP3P) e (E)-1-(4-nitrofenil)-3-fenilprop-2-en-1-ona (4NP3P), que diferem pela posição do substituinte no anel fenil, foram estudados na presença de solventes próticos e apróticos simulados pelo Modelo Continuo Polarizável (PCM) no nível DFT/B3LYP/6-311+G(d). Os parâmetros moleculares estáticos e dinâmicos (1064 nm) como momento dipolar, polarizabilidade linear, primeira e segunda hiperpolarizabilidades foram estudados em função do valor da constante dielétrica do solvente. Estudou-se o comportamento geométrico como ângulos de ligação química, ângulos de torção e distribuição parcial de cargas dos compostos, incluindo cálculos de energias de gap em vários solventes. Os resultados obtidos revelaram que a mudança do substituinte de CH3 (4MP3P) para NO2 (4NP3P) beneficia as propriedades ópticas não lineares dos compostos na presença do meio solvente, os valores absolutos da primeira hiperpolarizabilidade paralela foram os que apresentaram a maior variação.

Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes / Hepatite autoimune em 828 crianças e adolescentes brasileiros: achados clínicos e laboratoriais, perfil histológico, tratamentos e resultados

Abstract Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.

Resumo Objetivo: Este estudo com acompanhamento de longo prazo visou a avaliar o quadro clínico, os achados laboratoriais, o perfil histológico, os tratamentos e os resultados de crianças e adolescentes com hepatite autoimune. Métodos: Foram analisados os prontuários médicos de 828 crianças e adolescentes com HAI. Foi usado um questionário para coletar os dados anônimos sobre o quadro clínico, os achados bioquímicos e histológicos e os tratamentos. Resultados: De todos os pacientes, 89,6% tinham hepatite autoimune-1 e 10,4% hepatite autoimune-2. O sexo feminino foi predominante nos dois grupos. A idade média no início dos sintomas foi 111,5 (6; 210) e 53,5 (8; 165) meses nos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. Foi observado início clínico agudo em 56,1% e 58,8% e sintomas insidiosos em 43,9% e 41,2% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente. A probabilidade de insuficiência hepática foi 1,6 vezes maior para hepatite autoimune-2; 3,6% e 10,6% dos pacientes com hepatite autoimune-1 e hepatite autoimune-2, respectivamente, apresentaram insuficiência hepática fulminante; o risco foi 3,1 vezes maior para hepatite autoimune-2. Os níveis de gamaglobulina e imunoglobulina G foram significativamente maiores nos pacientes com hepatite autoimune-1, ao passo que os níveis de imunoglobulina A e C3 foram menores em pacientes com hepatite autoimune-2; 22,4% dos pacientes apresentaram cirrose e a remissão bioquímica foi atingida em 76,2%. A taxa de sobrevida atuarial foi de 93,0%. Um total de 4,6% pacientes foram submetidos a transplante de fígado e 6,9% morreram (hepatite autoimune-1: 7,5%; hepatite autoimune-2: 2,4%). Conclusões: Nesta grande série clínica de crianças e adolescentes brasileiros, a hepatite autoimune-1 foi mais frequente e os pacientes com hepatite autoimune-2 mostraram maiores taxas de remissão da doença com respostas mais rápidas aos tratamentos. Os pacientes com hepatite autoimune-1 apresentaram maior risco de óbito.

Differentially expressed mRNAs, proteins and miRNAs associated to energy metabolism in skeletal muscle of beef cattle identified for low and high residual feed intake.

BACKGROUND: Feed efficiency is one of the most important parameters that affect beef production costs. The energy metabolism of skeletal muscle greatly contributes to variations in feed efficiency. However, information regarding differences in proteins involved in the energy metabolism of the skeletal muscle in beef cattle divergently identified for feed efficiency is scarce. In this study, we aimed to investigate energy metabolism of skeletal muscle of Nellore beef cattle, identified for low and high residual feed intake using a proteomics approach. We further assessed the expression of candidate microRNAs as a one of the possible mechanisms controlling the biosynthesis of the proteins involved in energy metabolism that were differentially abundant between high and low residual feed intake animals. RESULTS: A greater abundance of 14-3-3 protein epsilon (P = 0.01) was observed in skeletal muscle of residual feed intake (RFI) high animals (RFI-High). Conversely, a greater abundance of Heat Shock Protein Beta 1 (P < 0.01) was observed in the skeletal muscle of RFI-Low cattle. A greater mRNA expression of YWHAE, which encodes the 14-3-3 protein epsilon, was also observed in the skeletal muscle of RFI-High animals (P = 0.01). A lower mRNA expression of HSPB1, which encodes the Heat Shock Protein Beta 1, was observed in the skeletal muscle of RFI-High animals (P = 0.01). The miR-665 was identified as a potential regulator of the 14-3-3 protein epsilon, and its expression was greater in RFI-Low animals (P < .001). A greater expression of miR-34a (P = 0.01) and miR-2899 (P < .001) was observed in the skeletal muscle of RFI-High animals, as both miRNAs were identified as potential regulators of HSPB1 expression. CONCLUSION: Our results show that Nellore cattle divergently identified for feed efficiency by RFI present changes in the abundance of proteins involved in energy expenditure in skeletal muscle. Moreover, our data point towards that miR-665, miR34a and miR-2899 are likely involved in controlling both 14-3-3 epsilon and HSPB1 proteins identified as differentially abundant in the skeletal muscle of RFI-High and RFI-Low Nellore cattle.

Clinical effect of Lactobacillus on the treatment of severe periodontitis and halitosis: A double-blinded, placebo-controlled, randomized clinical trial.

PURPOSE: To evaluate the clinical association between Lactobacillus and the efficacy of adjuvant treatment of severe periodontitis, periodontal parameters, and halitosis. METHODS: 60 healthy volunteers with severe periodontitis were randomized into two groups to receive periodontal therapy in a single session and lactobacillus or a placebo for 90 days. The test group received Lactobacillus reuteri, salivarius and acidophilus, and the control group received placebo with xylitol. RESULTS: There was a reduction in the depth levels of the probing pocket and the level of relative attachment after 90 days (P< 0.01). Regarding bleeding after pocket probing, the full-mouth with lactobacillus groups also showed more reduction than the placebo group (P< 0.01). The same results were observed with the halitosis parameters (P< 0.01). Oral administration of lactobacillus reduced periodontal parameters and halitosis and could contribute to the beneficial effects on periodontal conditions and halitosis. CLINICAL SIGNIFICANCE: Oral administration of lactobacillus reduced periodontal parameters and halitosis. At the dental office, dentists can consider using the beneficial effects of the probiotics on periodontal conditions and halitosis.

Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes.

OBJECTIVE: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. METHODS: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. RESULTS: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). CONCLUSIONS: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents.

PURPOSE: The objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis. METHODS: A cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations. RESULTS: Of the 77 patients, 51.9% were male, with a median age of 147 months (7.0-297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02). CONCLUSION: The pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.

Autoimmune Hepatitis in Children and Adolescents: Effect on Quality of Life.

OBJECTIVES: The aim of this study was to evaluate the effect of autoimmune hepatitis (AIH) on the quality of life of children and adolescents and to identify which variables effect health-related quality of life (HRQoL). METHODS: The Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was used to evaluate HRQoL. In addition, a questionnaire was applied which included signs, symptoms, and use of medications. RESULTS: A total of 43 patients (mean age, 15.0 ±â€Š3.9 years; 65.1% girls; 90.7% with type 1 AIH) was evaluated. Advanced liver disease was present in 30.2%, and 18.6% had sclerosing cholangitis. Treatment was effective in 93.1% of patients. The lowest HRQoL scores were associated with the school (67.7), emotional (68.2), and psychosocial (75.5) domains. Compared with healthy children, patients presented lower scores on the total, psychosocial, emotional, and school domains (P < 0.05). The presence of symptoms (ß = 0.39, P < 0.01), extrahepatic autoimmune diseases (ß = 0.27, P < 0.05), and a dislike of taking medication (ß = 0.40, P < 0.01) negatively affected the psychosocial PedsQL 4.0 score, and the presence of symptoms (ß = 0.40, P < 0.01) negatively affected the total PedsQL 4.0 score. CONCLUSIONS: AIH has a significant and negative effect on HRQoL among children and adolescents. The presence of symptoms, extrahepatic autoimmune diseases, and a dislike of taking medication were associated with the worsening of HRQoL. Providers should work with professionals trained to improve QoL to help improve treatment adherence and disease outcomes.

Effectiveness of muscle strengthening and description of protocols for preventing falls in the elderly: a systematic review

Background: Falls are a geriatric syndrome that is considered a significant public health problem in terms of morbidity and mortality because they lead to a decline in functional capacity and an impaired quality of life in the elderly. Lower limb muscle strengthening seems to be an effective intervention for preventing falls; however, there is no consensus regarding the best method for increasing lower limb muscle strength. Objectives: To analyze the effectiveness of lower limb muscle strengthening and to investigate and describe the protocols used for preventing falls in elderly subjects. Method: We performed a systematic review of randomized and controlled clinical trials published between 2002 and 2012 in the databases PubMed, EMBASE, Scopus, Web of Science, and PEDro that cited some type of lower limb muscle strengthening protocol and that evaluated the incidence of falls as the primary outcome exclusively in elderly subjects. Twelve studies met the inclusion criteria. Qualitative analysis was performed by independent reviewers applying the PEDro scale. Results: The data obtained from the selected studies showed lower fall rates in the intervention groups compared to controls. Six studies described the lower limb muscle strengthening protocol in detail. High methodological quality was found in 6 studies (PEDro score ≥7/10 points). Conclusions: The methodological quality of the studies in this area appears to leave little doubt regarding the effectiveness of lower limb strengthening exercises for preventing falls in elderly subjects, however the interventions in these studies were poorly reported. .

Effectiveness of muscle strengthening and description of protocols for preventing falls in the elderly: a systematic review.

BACKGROUND: Falls are a geriatric syndrome that is considered a significant public health problem in terms of morbidity and mortality because they lead to a decline in functional capacity and an impaired quality of life in the elderly. Lower limb muscle strengthening seems to be an effective intervention for preventing falls; however, there is no consensus regarding the best method for increasing lower limb muscle strength. OBJECTIVES: To analyze the effectiveness of lower limb muscle strengthening and to investigate and describe the protocols used for preventing falls in elderly subjects. METHOD: We performed a systematic review of randomized and controlled clinical trials published between 2002 and 2012 in the databases PubMed, EMBASE, Scopus, Web of Science, and PEDro that cited some type of lower limb muscle strengthening protocol and that evaluated the incidence of falls as the primary outcome exclusively in elderly subjects. Twelve studies met the inclusion criteria. Qualitative analysis was performed by independent reviewers applying the PEDro scale. RESULTS: The data obtained from the selected studies showed lower fall rates in the intervention groups compared to controls. Six studies described the lower limb muscle strengthening protocol in detail. High methodological quality was found in 6 studies (PEDro score ≥ 7/10 points). CONCLUSIONS: The methodological quality of the studies in this area appears to leave little doubt regarding the effectiveness of lower limb strengthening exercises for preventing falls in elderly subjects, however the interventions in these studies were poorly reported.