Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

Ana Luiza Melo dos Santos,Helen de Melo Santos,Marina Bettiol Nogueira,Hugo Tadashi Oshiro Távora,Maria de Lourdes Jaborandy Paim da Cunha,Renata Belém Pessoa de Melo Seixas,Luciana de Freitas Velloso 대한소아소화기영양학회 2018 Pediatric gastroenterology, hepatology & nutrition Vol.21 No.4

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.

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

dos Santos, Ana Luiza Melo,de Melo Santos, Helen,Nogueira, Marina Bettiol,Tavora, Hugo Tadashi Oshiro,da Cunha, Maria de Lourdes Jaborandy Paim,de Melo Seixas, Renata Belem Pessoa,Monte, Luciana de Fr The Korean Society of Pediatric Gastroenterology 2018 Pediatric gastroenterology, hepatology & nutrition Vol.21 No.4

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.

Pediatric Liver Transplantation: Caregivers' Quality of Life

Tavares, Ana Paula Bastos,Seixas, Lucas Belem Pessoa de Melo Guerra,Jayme, Caren Lopes Wanderlei,Porta, Gilda,Seixas, Renata Belem Pessoa de Melo,Carvalho, Elisa de The Korean Society of Pediatric Gastroenterology 2022 Pediatric gastroenterology, hepatology & nutrition Vol.25 No.6

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.

Heat tolerance in Brazilian hair sheep

Luiza Seixas,Cristiano Barros de Melo,Candice Bergmann Tanure,Vanessa Peripolli,Concepta McManus 아세아·태평양축산학회 2017 Animal Bioscience Vol.30 No.4

Objective: The aim of this study was to evaluate heat tolerance using heat tolerance indices, physiological, physical, thermographic, and hematological parameters in Santa Ines and Morada Nova sheep breeds in the Federal District, Brazil. Methods: Twenty-six adult hair sheep, one and a half years old, from two genetic groups (Santa Ines: 12 males and 4 females; Morada Nova: 7 males and 3 females) were used and data (rectal temperature, respiratory rate, heart rate, skin temperatures; hematological parameters) were collected during three consecutive days, twice a day (morning and afternoon), with a total of six repetitions. Also physical parameters (biometric measurements, skin and hair traits) and heat tolerance indices (temperature-humidity index, Iberia and Benezra) were evaluated. The analyses included analyses of variance, correlation, and principal components with a significance level of 5%. Results: The environmental indices, in general, indicate a situation of thermal discomfort for the animals during the afternoon. Breed significantly influenced (p<0.001) physiological and physical characteristics of skin, hair, biometric measurements and Iberia and Benezra heat tolerance indices. Santa Ines animals were bigger and had longer, greater number and darker hair, thicker skin, greater respiratory rate and Benezra index and lower Iberia index compared with Morada Nova breed. Conclusion: Although both breeds can be considered adapted to the environmental conditions of the region, Morada Nova breed is most suitable for farming in the Midwest region. The positive correlation found between the thermographic temperatures and physiological parameters indicates that this technique can be used to evaluate thermal comfort. Also, it has the advantage that animals do not have to be handled, which favors animal welfare.

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

Silva, Larissa Caetano,Seixas, Renata B.P. Melo,de Carvalho, Elisa The Korean Society of Pediatric Gastroenterology 2020 Pediatric gastroenterology, hepatology & nutrition Vol.23 No.3

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.

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

Larissa Caetano Silva,Renata B.P. Melo Seixas,Elisa de Carvalho 대한소아소화기영양학회 2020 Pediatric gastroenterology, hepatology & nutrition Vol.23 No.3

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.