Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 158
Filter
1.
J. pediatr. (Rio J.) ; 98(1): 33-38, Jan.-Feb. 2022. tab
Article in English | LILACS | ID: biblio-1360554

ABSTRACT

Abstract Objective: In this present study, the authors evaluated the predictive factors for adverse maternal-fetal outcomes in pregnancies of women with cystic fibrosis (CF). Patients were followed up by a referral center for adults in southern Brazil. Methods: This is a retrospective cohort study that used data from electronic medical records regarding pregnancies of women diagnosed with CF. Results: The study included 39 pregnancies related to 20 different women. The main adverse outcomes were high prevalence rates of premature birth (38.5%) and maternal respiratory exacerbation (84.6%). Lower body mass index (BMI) values (< 20.8) and younger ages of CF diagnosis increased the risk of premature birth. The presence of methicillin-resistant and absence of methicillin-sensitive Staphylococcus aureus, as well as a younger age of diagnosis, increased the risk of maternal respiratory exacerbation during pregnancy. Conclusions: Conception in women with CF is often associated with maternal and fetal complications. Continuous monitoring by a multidisciplinary team should emphasize appropriate nutritional status, investigation of bacterial colonization, and immediate attention to respiratory exacerbations.


Subject(s)
Humans , Female , Pregnancy , Adult , Pregnancy Complications/epidemiology , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Premature Birth/etiology , Premature Birth/epidemiology , Pregnancy Outcome , Nutritional Status , Retrospective Studies
2.
Einstein (Säo Paulo) ; 20: eRW5686, 2022. tab
Article in English | LILACS | ID: biblio-1364796

ABSTRACT

ABSTRACT Objective To develop a scientific consensus on nutrition in cystic fibrosis. Methods Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed®/MEDLINE database, after training and standardization of search strategies, to write the best level of evidence for the questions elaborated. Issues related to disagreement were discussed until a consensus was reached among specialists, based on the current scientific literature. Results Forty-two questions were prepared and objectively answered, resulting in a consensus of nutritional therapy in cystic fibrosis. Conclusion This work enabled establishing a scientific consensus for nutritional treatment of cystic fibrosis patients.


Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/therapy , Brazil , Nutrition Assessment , Nutritional Status , Nutritional Support
3.
Rev. pediatr. electrón ; 18(2): 10-20, ago.2021. tab, graf
Article in Spanish | LILACS | ID: biblio-1370818

ABSTRACT

INTRODUCCIÓN. La diabetes es la complicación extrapulmonar más frecuente en adultos con fibrosis quística. Existen escasas publicaciones de diabetes relacionada a la fibrosis quística en preescolares a nivel mundial. En Chile se desconoce su prevalencia. MÉTODO. Reportamos una serie de tres casos de niños con fibrosis quística (FQ) y diagnóstico de diabetes a muy temprana edad. RESULTADOS. Caso 1: Niño de 8 años, con diagnóstico de fibrosis quística a los 3 meses de vida por test de sudor y estudio genético p.Phe508del /-. Presenta hiperglicemia no cetósica desde los 6 meses de edad, con colonización traqueal de Staphylococcus Aureus (SA) y Pseudomona Aeruginosa (PA) y debut de diabetes a los 2 años 1 mes. Caso 2: Niño de 16 años, a los 7 meses de vida se diagnostica FQ por test de sudor y estudio genético p.Phe508del /-. Presenta colonización traqueal por SA y múltiples infecciones por PA. A los 5 años 7 meses se diagnostica diabetes presentando cetosis al debut. Caso 3: Niño de 13 años, con diagnóstico de FQ a los 7 meses de vida mediante test de sudor y estudio genético p.Phe508del/-. Presenta colonización traqueal por SA y múltiples infecciones por PA, se realiza diagnóstico de diabetes a los 2 años 7 meses de edad. DISCUSIÓN: La diabetes asociada a fibrosis quística es una complicación frecuente en adultos con fibrosis quística, pero puede presentarse desde edades tempranas. Se debe tener alto nivel de sospecha para el diagnóstico oportuno y óptimo manejo.


INTRODUCTION: Diabetes is the most common extra pulmonary complication in adults with cystic fibrosis (CF). There are few reports of diabetes related to (CF) in preschool children worldwide. Prevalence in Chile is unknown. MÉTODO: We report ta serie of three cases of children with CF and diagnosis of diabetes at an early age. Case 1: Boy 8 year old, CF diagnosed at the age of 3 months by sweat test and genetic study p.Phe508del/-. He presented non-ketotic hyperglycemia since he was 6 months old, with tracheal colonization of Staphylococcus Aureus (SA) and Pseudomona Aeruginosa (PA) , and diagnosis of diabetes at the age of 2 years 1 month. Case 2: Boy patient, 16 years old, with diagnosis of CF at the of age 7 months by sweat test and genetic study p.Phe508del/-. He presents tracheal colonization by SA and multiple PA infections. At the age 5 years 7 months, diabetes is diagnosed, presenting ketosis at the beginning. Case 3: Boy 13 years diagnosed with CF at the age of 7 months, presented sweat test and genetic study p.Phe508del/-. He presents tracheal colonization by SA and multiple infections. DISCUSSION: CF related diabetes is common in adults with cystic fibrosis, but it can be diagnosed in early childhood. A high level of suspicious is required for a proper and timely diagnosis


Subject(s)
Humans , Male , Child , Adolescent , Cystic Fibrosis/complications , Diabetes Mellitus/etiology , Prevalence , Age of Onset , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Cystic Fibrosis/epidemiology , Diabetes Mellitus/diagnosis , Diabetes Mellitus/therapy , Diabetes Mellitus/epidemiology
4.
Rev. chil. endocrinol. diabetes ; 14(4): 171-177, 2021. tab
Article in Spanish | LILACS | ID: biblio-1348218

ABSTRACT

Los avances en el campo de la fibrosis quística han aumentado la esperanza de vida de estos pacientes, por lo que cada vez es más prevalente la Diabetes Relacionada con la Fibrosis Quística (DRFQ) y sus complicaciones. La DRFQ se asocia a mayor morbimortalidad, deterioro de la función pulmonar y del estado nutricional. Por lo mismo, el manejo óptimo de esta patología depende de un diagnóstico precoz, tratamiento individualizado y vigilancia de las complicaciones diabéticas. El screening de DRFQ debe realizarse anualmente a partir de los 10 años, mediante una Prueba de Tolerancia a la Glucosa Oral (PTGO), lo cual permite el diagnóstico. El manejo de esta patología tiene por objetivo estabilizar y mejorar la función pulmonar y el estado nutricional y metabólico de los pacientes. Actualmente, la insulina es el tratamiento farmacológico de elección para controlar la hiperglicemia y el esquema de uso debe ser individualizado para cada persona. En caso de enfermedades agudas pueden existir mayores requerimientos de insulina. Además, se deben tener consideraciones especiales en cuanto a la dieta y la insuficiencia pancreática exocrina que presentan estos pacientes. Para la vigilancia de complicaciones microvasculares se debe realizar una monitorización anual a partir de los 5 años desde el diagnóstico de DRFQ. Debido a la complejidad de estos pacientes, para alcanzar el mejor cuidado posible se necesita un enfoque multidisciplinario con distintos profesionales de la salud coordinados, incluyendo en la toma de decisiones al paciente y su familia.


Advances made in the field of cystic fibrosis have increased the life expectancy of these patients, which is why Cystic Fibrosis-Related Diabetes (CFRD) and its complications are becoming more and more prevalent. CFRD is associated with increased morbidity and mortality, lower lung function and inadequate weight maintenance. Therefore, the optimal management of this pathology depends on an early diagnosis, individualized treatment and monitoring of diabetic complications. For CFRD, routine screening with an Oral Glucose Tolerance Test (OGTT) should be carried out yearly from the age of 10, which allows to diagnose it. The treatment goals in CFRD are to stabilize and improve lung function and obtain adequate weight gain. Currently, insulin is the pharmacological treatment of choice to control hyperglycemia and the insulin regimen must be personalized for each person. In acute illnesses, there may be higher insulin requirements. In addition, special considerations must be taken regarding diet and exocrine pancreatic insufficiency that these patients present. For the surveillance of microvascular complications, annual monitoring should be carried out 5 years after the diagnosis of CFRD. Due to the complexity of these patients, in order to achieve the best possible care, a multidisciplinary approach is needed with different coordinated health professionals, including the patients and their family in the decision-making process.


Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Diabetes Mellitus/etiology , Diabetes Mellitus/therapy , Patient Care Team , Mass Screening , Cystic Fibrosis/physiopathology , Nutrition Therapy , Diabetes Mellitus/diagnosis , Diabetes Mellitus/physiopathology , Insulins/therapeutic use , Glucose Tolerance Test , Hypoglycemic Agents/therapeutic use
5.
J. bras. pneumol ; 47(4): e20200557, 2021. tab, graf
Article in English | LILACS | ID: biblio-1286951

ABSTRACT

ABSTRACT Objective: Massive hemoptysis is one of the most serious complications in patients with cystic fibrosis (CF). This study aimed to evaluate the hemoptysis-free period following bronchial and non-bronchial artery embolization (BAE/non-BAE) in CF patients and to investigate predictors of recurrent bleeding and mortality by any cause. Methods: This was a retrospective cohort study of CF patients ≥ 16 years of age undergoing BAE/non-BAE for hemoptysis between 2000 and 2017. Results: We analyzed 39 hemoptysis episodes treated with BAE/non-BAE in 17 CF patients. Hemoptysis recurrence rate was 56.4%. Of the sample as a whole, 3 (17.6%) were hemoptysis-free during the study period, 2 (11.8%) underwent lung transplantation, and 3 (17.6%) died. The median hemoptysis-free period was 17 months. The median hemoptysis-free period was longer in patients with chronic infection with Pseudomonas aeruginosa (31 months; 95% CI: 0.00-68.5) than in those without that type of infection (4 months; 95% CI: 1.8-6.2; p = 0.017). However, this association was considered weak, and its clinical significance was uncertain due to the small number of patients without that infection. Conclusions: BAE appears to be effective in the treatment of hemoptysis in patients with CF.


RESUMO Objetivo: A hemoptise maciça é uma das complicações mais graves em pacientes com fibrose cística (FC). O objetivo deste estudo foi avaliar o período livre de hemoptise após a embolização arterial brônquica/não brônquica (EAB/não EAB) em pacientes com FC e investigar preditores de sangramento recorrente e mortalidade por qualquer causa. Métodos: Trata-se de um estudo retrospectivo de coorte de pacientes com FC com idade ≥ 16 anos submetidos a EAB/não EAB para o tratamento de hemoptise entre 2000 e 2017. Resultados: Foram analisados 39 episódios de hemoptise tratada por meio de EAB/não EAB em 17 pacientes com FC. A taxa de recidiva da hemoptise foi de 56,4%. Do total de pacientes, 3 (17,6%) permaneceram sem hemoptise durante o estudo, 2 (11,8%) foram submetidos a transplante de pulmão e 3 (17,6%) morreram. A mediana do período sem hemoptise foi de 17 meses. A mediana do período sem hemoptise foi maior em pacientes com infecção crônica por Pseudomonas aeruginosa (31 meses; IC95%: 0,00-68,5) do que naqueles sem esse tipo de infecção (4 meses; IC95%: 1,8-6,2; p = 0,017). No entanto, essa associação foi considerada fraca, e sua importância clínica foi considerada incerta em virtude do pequeno número de pacientes sem essa infecção. Conclusões: A EAB parece ser eficaz no tratamento de hemoptise em pacientes com FC.


Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/therapy , Embolization, Therapeutic , Bronchial Arteries , Retrospective Studies , Treatment Outcome , Hemoptysis/etiology , Hemoptysis/therapy
6.
Neumol. pediátr. (En línea) ; 16(2): 81-84, 2021. ilus, tab
Article in Spanish | LILACS | ID: biblio-1293292

ABSTRACT

La aspergilosis broncopulmonar alérgica (ABPA) es una reacción de hipersensibilidad secundaria al Aspergillus fumigatus (Af) que complica la evolución en fibrosis quística (FQ). Existen pocos estudios pediátricos de su prevalencia publicados en el mundo y en Chile se desconoce. El objetivo de este trabajo fue estimar la prevalencia de ABPA en niños con FQ en un hospital de referencia, explorar factores de riesgo y describir los criterios diagnósticos, tratamiento y evolución. Se incluyeron retrospectivamente los niños con FQ atendidos en un hospital terciario en Santiago de Chile (Hospital Roberto del Río) entre los años 2011 a 2019, se identificaron aquellos con diagnóstico de ABPA. Se registraron criterios diagnósticos según la Cystic Fibrosis Foundation, presencia de factores de riesgo, tratamientos recibidos y efectos adversos. De 65 pacientes con FQ atendidos en este período, la prevalencia de ABPA fue del 12%. El promedio de edad al diagnóstico fue ± 11 años (5-17 años), predominando la edad adolescente y el género masculino. El 50% cumplieron con los criterios clásicos, el 87,5% usaron antibióticos y el 62,5% corticoides inhalados. La respuesta favorable al tratamiento inicial con corticoides y antifúngico vía oral fue 62,5%, con una exacerbación al momento del estudio. El 25% se comportaron como refractario y el 12,5% respondieron a tratamiento con pulsos de metilprednisolona. El 37,5% presentaron eventos adversos relacionados a corticoides. La prevalencia de ABPA observada es comparable a las series publicadas. Se necesitan trabajos prospectivos para conocer la prevalencia nacional y su tendencia a lo largo de los años, identificando factores de riesgo.


Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity response to Aspergillus fumigatus (Af) and worsens outcome in children with cystic fibrosis (CF). Its prevalence varies in the literature, but we do not know it in Chile. The aim of the study was to know the prevalence of ABPA in children with CF and to describe risk factors, diagnostic criteria, treatment and outcome. We included all patients with CF seen in a tertiary hospital in Santiago, Chile (Hospital Roberto del Río), between 2011 and 2019; ABPA cases (CF Foundation diagnostic criteria) were identified for the estimation of the prevalence. Risk factors, diagnostic criteria and treatment were recorded, as proposed by the Cystic Fibrosis Foundation. A total of 65 patients with CF were identified in the study period, with a prevalence of 12% (8 cases). Mean age at diagnosis ± 11 years (5-17), more frequent in adolescence and male. CF Foundation criteria diagnostic were identified in 50% of cases, with high frequency of antibiotic use (87,5%) and inhaled steroids (62,5%). Positive oral steroids and antifungal treatment response was 62,5%. Refractary response was 25% and 12,5% needed intravenous metilprednisolone pulses. A 37,5% of cases presented adverse effects to steroids. Prevalence of ABPA is comparable to literature. A prospective study is needed to identified national prevalence and trends, identifying risks factors.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Aspergillosis, Allergic Bronchopulmonary/epidemiology , Cystic Fibrosis/epidemiology , Aspergillosis, Allergic Bronchopulmonary/diagnosis , Aspergillosis, Allergic Bronchopulmonary/drug therapy , Aspergillus fumigatus , Chile , Prevalence , Risk Factors , Cystic Fibrosis/complications , Hospitals, Pediatric/statistics & numerical data , Antifungal Agents/therapeutic use
7.
J. pediatr. (Rio J.) ; 96(6): 686-692, Set.-Dec. 2020. tab, graf
Article in English | LILACS, ColecionaSUS, SES-SP | ID: biblio-1143196

ABSTRACT

Abstract Objective: To systematically revise the literature in search of data about the prevalence of constipation in patients with cystic fibrosis according to the publications in this field, which partly refer to guidelines defined in 2010 by the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. Sources: Systematic review selecting articles based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses, including Cystic Fibrosis patients of all ages. Sources of information were selected to identify the articles without period limitation: CADTH - Canadian Agency for Drugs and Technologies in Health, CINAHL Complete, Clinical Trials US NIH, Cochrane Library, Embase, MEDLINE via Ovid, Scopus, Web Of Science, PubMed, SciELO, MEDLINE and LILACS , Health Systems Evidence, PDQ Evidence, CRD Canadian Agency for Drugs and Technologies in Health, INAHTA - International Network of Agencies for Health Technology Assessment, and PEDro. Findings: The prevalence of constipation was reported in eight observational studies. Only two studies assessed the frequency of constipation as a primary objective; in the others, constipation was quoted along with the prevalence of the spectrum of gastrointestinal manifestations. Altogether, the publications included 2,018 patients, the reported prevalence varied from 10% to 57%. Only two of the six articles published after 2010 followed the definition recommended by the European Society. Conclusions: Constipation is a frequent but still insufficiently assessed complaint of Cystic Fibrosis patients. The use of diverse diagnostic criteria restricts comparison and epidemiological conclusions, future studies should compulsorily apply the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition definition.


Resumo Objetivo Revisar sistematicamente a literatura em busca de dados sobre a prevalência de constipação em pacientes com fibrose cística (FC), de acordo com as publicações nesse campo, que se referem parcialmente às diretrizes definidas pela European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN, 2010). Fontes de dados Revisão sistemática, selecionaram-se artigos com base no Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), incluindo todos os pacientes com FC de todas as faixas etárias. As fontes de informação foram selecionadas para identificar os artigos sem limitação de período para a pesquisa: CADTH (Canadian Agency for Drugs and Technologies in Health), CINAHL Complete, Clinical Trials US NIH, Cochrane Library, Embase, Medline via Ovid, Scopus, Web of Science, PubMed, SciELO, Medline e Lilacs por meio da Biblioteca Virtual em Saúde (BVS), Health Systems Evidence, PDQ Evidence, CRD (Canadian Agency for Drugs and Technologies in Health), INAHTA (International Network of Agencies for Health Technology Assessment) e PEDRO. Achados A prevalência de constipação em pacientes com FC foi relatada em oito estudos observacionais. Apenas dois estudos avaliaram a frequência de constipação como objetivo primário; nos outros, a constipação foi citada juntamente com a prevalência do espectro de manifestações gastrointestinais. No total, as publicações incluíram 2.018 pacientes e a prevalência relatada variou amplamente, de 10 a 57%. Apenas dois dos seis artigos publicados após 2010 seguiram a definição recomendada pela ESPGHAN. Conclusões A constipação é uma queixa frequente, mas ainda insuficientemente avaliada, dos pacientes com FC. O uso de diversos critérios diagnósticos restringe as comparações e declarações epidemiológicas, de modo que futuros estudos deveriam aplicar a definição ESPGHAN de maneira compulsória.


Subject(s)
Humans , Child , Constipation/etiology , Constipation/epidemiology , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Canada , Nutritional Status , Prevalence , Observational Studies as Topic
8.
J. pediatr. (Rio J.) ; 96(6): 732-740, Set.-Dec. 2020. tab
Article in English | LILACS, ColecionaSUS, SES-SP | ID: biblio-1143191

ABSTRACT

Abstract Objective: To evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. Methods: Case series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. Results: All patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. Conclusion In this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time.


Resumo Objetivo: Avaliar os dados demográficos, o genótipo e o quadro clínico de pacientes pediátricos que apresentam síndrome da obstrução intestinal distal (DIOS) e os fatores associados à recidiva da DIOS. Métodos: Casuística de 10 pacientes (média de 13,2 anos) monitorados em um centro de referência e avaliados de forma retroativa. Os dados analisados incluíram idade, sexo, genótipo da fibrose cística, íleo meconial no nascimento, estado de hidratação, exacerbação pulmonar, colonização por Pseudomonas aeruginosa, insuficiência pancreática (IP), IMC nos episódios, manifestações clínicas da DIOS, estudos de diagnóstico por imagem realizados, manejo agudo da DIOS, terapia de manutenção e recidiva no acompanhamento. Resultados: Todos os pacientes apresentaram dois exames de cloreto no suor positivos e 09/10 também apresentaram estudo do genótipo. O genótipo mais comum identificado foi a homozigose da mutação delta F508. Em sete casos foi mencionado um histórico de íleo meconial. Todos os pacientes apresentaram insuficiência pancreática. O diagnóstico da DIOS teve como base achados clínicos e de imagem; 85% do número total de episódios foram tratados com sucesso com laxantes osmóticos orais e/ou terapia retal (enema de glicerina ou irrigação salina). A recidiva foi observada em 5 de 10 pacientes. Conclusão: Neste primeiro relatório da DIOS pediátrica na América do Sul, a presença de dois fatores de risco na ocorrência da DIOS foi universal: insuficiência pancreática e genótipo associado a doença grave. O histórico de íleo meconial no nascimento esteve presente na maioria dos pacientes, bem como no subgrupo com recidiva da DIOS. O diagnóstico dependeu principalmente do quadro clínico e do diagnóstico por imagem abdominal. As práticas de manejo de episódios variaram, provavelmente refletiram as mudanças no tratamento dessa síndrome ao longo do tempo.


Subject(s)
Humans , Child , Adolescent , Exocrine Pancreatic Insufficiency/diagnosis , Exocrine Pancreatic Insufficiency/etiology , Exocrine Pancreatic Insufficiency/therapy , Cystic Fibrosis/complications , Cystic Fibrosis/diagnosis , Cystic Fibrosis/genetics , South America , Retrospective Studies , Intestinal Obstruction/diagnosis , Intestinal Obstruction/etiology , Intestinal Obstruction/therapy
9.
J. bras. pneumol ; 46(4): e20180294, 2020. tab, graf
Article in English | LILACS | ID: biblio-1134879

ABSTRACT

ABSTRACT Objective: Although various strategies have been proposed for eradicating Pseudomonas aeruginosa in patients with cystic fibrosis (CF), only a few employ multistep treatment in children colonized by that pathogen for the first time. The aim of this study was to describe the effectiveness of a three-phase eradication protocol, initiated after the first isolation of P. aeruginosa, in children with CF in Brazil. Methods: This was a retrospective real-life study in which we reviewed the medical records of pediatric CF patients in whom the eradication protocol was applied between June of 2004 and December of 2012. The three-phase protocol was guided by positive cultures for P. aeruginosa in airway secretions, and the treatment consisted of inhaled colistimethate and oral ciprofloxacin. Success rates were assessed after each phase, as well as cumulatively. Results: During the study period, 47 episodes of P. aeruginosa colonization, in 29 patients, were eligible for eradication. Among the 29 patients, the median age was 2.7 years, 17 (59%) were male, and 19 (65%) had at least one F508del allele. All 29 patients completed the first phase of the protocol, whereas only 12 and 6 completed the second and third phases, respectively. Success rates for eradication in the three treatment phases were 58.6% (95% CI: 40.7-74.5), 50.0% (95% CI: 25.4-74.6), and 66.7% (95% CI: 30.0-90.3), respectively. The cumulative success rate was 93.1% (95% CI: 78.0-98.1). Treatment failure in all three phases occurred in only 2 patients. Conclusions: In this sample of patients, the multistep eradication protocol was effective and had a high success rate.


RESUMO Objetivo: Embora várias estratégias de erradicação de Pseudomonas aeruginosa tenham sido propostas para pacientes com fibrose cística (FC), apenas algumas usaram um tratamento em fases e incluíram crianças na primeira colonização por esse patógeno. O objetivo deste estudo foi descrever a eficácia de um protocolo de erradicação em três fases em crianças com FC a partir do primeiro isolamento de P. aeruginosa no Brasil. Métodos: Estudo retrospectivo de vida real que avaliou prontuários de pacientes pediátricos com FC submetidos ao protocolo de erradicação entre junho de 2004 e dezembro de 2012. O protocolo em três fases foi orientado pela cultura positiva para P. aeruginosa de secreções das vias aéreas, utilizando-se colistimetato inalatório e ciprofloxacina oral no tratamento. As taxas de sucesso após cada fase e a de sucesso acumulado foram avaliadas. Resultados: Durante o período do estudo, 47 episódios de colonização por P. aeruginosa, em 29 pacientes, foram elegíveis para erradicação. Todos os 29 pacientes foram submetidos à primeira fase do protocolo (mediana de idade de 2,7 anos, 17 pacientes (59%) do sexo masculino e 19 (65%) com pelo menos um alelo F508del), sendo que 12 e 6 pacientes foram submetidos a segunda e terceira fases, respectivamente. As taxas de sucesso de erradicação nas três fases de tratamento foram de 58,6% (IC95%: 40,7-74,5), 50,0% (IC95%: 25,4-74,6) e 66,7% (IC95%: 30,0-90,3), respectivamente. A taxa de sucesso acumulado foi de 93,1% (IC95%: 78,0-98,1). Apenas 2 pacientes apresentaram falha do tratamento de erradicação. Conclusões: O primeiro isolamento de P. aeruginosa ocorreu em crianças de baixa idade. O protocolo de erradicação em fases foi efetivo com alta taxa de sucesso.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Pseudomonas aeruginosa/drug effects , Pseudomonas Infections/drug therapy , Cystic Fibrosis/complications , Anti-Bacterial Agents/therapeutic use , Brazil , Clinical Protocols , Retrospective Studies
11.
Rev. Paul. Pediatr. (Ed. Port., Online) ; 37(4): 435-441, Oct.-Dec. 2019. tab, graf
Article in English | LILACS | ID: biblio-1041361

ABSTRACT

ABSTRACT Objective: To determine the prevalence of hepatic steatosis (HS) in children and adolescents with cystic fibrosis (CF) and associate it with nutritional status. Methods: Cross-sectional study with children and adolescents with CF diagnosis. Weight and height were used to calculate the body mass index (BMI) and subsequent classification of the nutritional status. The midarm circumference (MAC), triceps skinfold thickness (TSF) and midarm muscle circumference (MAMC) were used to evaluate body composition. Abdominal ultrasonography was performed for diagnosis of HS. The statistical tests used were Student's t test, Mann-Whitney test and chi-square test with significance level of 5%. Results: 50 patients with CF were evaluated, 18 (36%) were diagnosed with HS (Group A) and 32 (64%) without HS (Group B). The mean age of Group A was 13,2±4,9 years old and Group B 11,7±4,9; for BMI, the value for Group A was 18,0±4,1 and Group B was 15,7±3,8; the TSF of Group A was 8,4±3,5 mm and Group B was 7,0±2,5 mm. For these variables, there was no significant difference between the groups. The mean of MAC and MAMC differed significantly between the groups, being higher in the HS group, with p values of 0,047 and 0,043. Conclusions: The frequency of HS in patients with CF is high and it is not related to malnutrition, according to the parameters of BMI, TSF and MAMC. The values of MAC and MAMC indicated a greater reserve of muscle mass in patients with HS.


RESUMO Objetivo: Determinar a prevalência de esteatose hepática (EH) em crianças e adolescentes com fibrose cística (FC) e associá-la com o estado nutricional. Métodos: Estudo transversal com crianças e adolescentes com diagnóstico de FC. Foram aferidos o peso e a altura para o cálculo do índice de massa corpórea (IMC) e classificação do estado nutricional. A circunferência do braço (CB), a dobra cutânea tricipital (DCT) e a circunferência muscular do braço (CMB) foram empregadas para avaliação da composição corporal. A ultrassonografia abdominal foi realizada para o diagnóstico de EH. Os testes estatísticos empregados foram o teste t de Student, o teste de Mann-Whitney e o teste do qui-quadrado, com nível de significância de 5%. Resultados: Dos 50 pacientes avaliados, 18 (36%) apresentaram EH (Grupo A) e 32 (64%) não (Grupo B). Para as médias de idade (Grupo A: 13,3±5,0 anos; e Grupo B: 11,7±5,0 anos), IMC (Grupo A: 18,0±4,1; e Grupo B: 15,7±3,8) e DCT (Grupo A: 8,4±3,5 mm; e Grupo B: 7,0±2,5 mm), não houve diferença significativa entre os grupos. A média da CB e da CMB diferiram significativamente entre os grupos, sendo mais elevada no grupo com EH, com valores p respectivos de 0,047 e 0,043. Conclusões: É alta a frequência de EH em pacientes com FC e ela não está relacionada com a desnutrição, segundo os parâmetros de IMC, DCT e CMB. Os valores de CB e CMB indicaram maior reserva de massa muscular nos pacientes com EH.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Young Adult , Nutritional Status , Cystic Fibrosis/complications , Malnutrition/complications , Non-alcoholic Fatty Liver Disease/ethnology , Risk Management , Prevalence , Cross-Sectional Studies , Cystic Fibrosis/physiopathology , Malnutrition/diagnosis , Malnutrition/physiopathology , Non-alcoholic Fatty Liver Disease/diagnosis , Non-alcoholic Fatty Liver Disease/epidemiology
12.
Rev. argent. cir ; 111(2): 99-103, jun. 2019. tab
Article in English, Spanish | LILACS | ID: biblio-1013352

ABSTRACT

Debido a la falta de órganos para trasplantes se han desarrollado diferentes alternativas quirúrgicas, como la bipartición hepática (BH) y los trasplantes hepáticos con donantes vivos. En la BH clásica, de la división de un hígado de donante cadavérico se obtienen dos injertos, uno correspondiente a los segmentos 2-3 y otro a los segmentos 1, 4-8. Para poder utilizar los injertos de una BH, en pacientes adultos, se puede realizar una BH derecha/izquierda típica, donde se obtienen un injerto derecho (segmentos 5-8) y otro izquierdo (segmentos 1-4). La BH se puede realizar en el momento de la ablación (BH in situ) o en la cirugía de banco (BH ex situ). En este trabajo informamos el primer caso de BH in situ derecha/izquierda típica de la Argentina, resaltando los detalles de la cirugía del donante y del receptor.


Due to the shortage of organs for transplantation, different surgical alternatives have been developed, as split liver transplantation (SLT) and living-donor liver transplantation. In classical SLT, the liver of a cadaveric donor is divided and two allografts are obtained, one corresponding to segments 2-3 and the other to segments 1, 4-8. In order to produce two grafts from one liver for two adult recipients, splitting of the liver can create a right graft including segments 5-8 and a left graft with segments 1-4. Splitting of the liver can be performed during procurement (in situ) or on the bench (ex situ). The aim of our study is to describe the first case of in situ full-right full-left split liver transplantation, with focus on donor and recipient surgery.


Subject(s)
Humans , Male , Female , Child , Middle Aged , Liver Transplantation/instrumentation , Hepatectomy/methods , Cholangiography/methods , Neuroendocrine Tumors , Cystic Fibrosis/complications , Liver Neoplasms/surgery , Neoplasm Metastasis
13.
Rev. cuba. anestesiol. reanim ; 18(1): e475, ene.-abr. 2019.
Article in Spanish | LILACS, CUMED | ID: biblio-1093098

ABSTRACT

Introducción: La fibrosis quística es una enfermedad genética que constituye causa frecuente de neumopatía crónica grave en adultos jóvenes. Se asocia a cirrosis de vías biliares y cálculos vesiculares, por lo que el anestesiólogo debe estar familiarizado con las características clínicas de la enfermedad a fin de disminuir el índice de complicaciones perioperatorias que pueden presentarse. Objetivo: Presentar las características clínicas y la conducta perioperatoria en una paciente con diagnóstico de fibrosis quística anunciada para colecistectomía videolaparoscópica. Caso clínico: Paciente femenina de 19 años de edad, raza negra, con múltiples infecciones respiratorias durante la infancia, que se diagnosticó como caso nuevo de fibrosis quística y durante los estudios se observó litiasis vesicular sintomática por lo que se anunció para colecistectomía videolaparoscópica. Se describe la evaluación y preparación preoperatoria, así como la conducta anestésica intraoperatoria. Conclusiones: Los resultados anestésicos dependen de la atención a una enfermedad compleja que afecta a múltiples órganos y el control estrecho y el tratamiento oportuno de la enfermedad pulmonar previa(AU)


Introduction: Cystic fibrosis is a genetic disease that is a frequent cause of severe chronic lung disease in young adults. It is associated with biliary tract cirrhosis and gallstones, a reason why the anesthesiologist must be familiar with the clinical characteristics of the disease in order to reduce the rate of perioperative complications that may occur. Objective: To present the clinical characteristics and perioperative behavior in a patient diagnosed with cystic fibrosis announced for videolaparoscopic cholecystectomy. Clinical case: Female patient of 19 years of age, black race, with multiple respiratory infections during childhood, who was diagnosed as a new case of cystic fibrosis. During the studies, symptomatic vesicular lithiasis was observed, that's why the patient what was announced for videolaparoscopic cholecystectomy. The evaluation and preoperative preparation, as well as the intraoperative anesthetic behavior are described. Conclusions: Anesthetic outcomes depend on the attention to a complex disease that affects multiple organs and the close control and timely treatment of previous lung disease(AU)


Subject(s)
Humans , Female , Young Adult , Cholecystectomy, Laparoscopic/methods , Cystic Fibrosis/complications , Urinary Bladder Calculi/surgery , Anesthetics/therapeutic use
14.
Rev. chil. infectol ; 36(1): 112-114, feb. 2019. graf
Article in Spanish | LILACS | ID: biblio-1042652

ABSTRACT

Resumen Las exacerbaciones pulmonares de causa infecciosa son una de las mayores complicaciones en los pacientes con fibrosis quística (FQ). Estas se asocian a un progresivo aumento en la morbilidad y mortalidad. El tratamiento antimicrobiano se realiza dependiendo del microorganismo aislado. Con frecuencia se utilizan antimicrobianos β-lactámicos, los cuales no están exentos de reacciones adversas. A continuación, se describen dos casos de neutropenia tras el uso prolongado de cefepime en pacientes con FQ.


Pulmonary exacerbations of infectious cause are one of the major complications in patients with cystic fibrosis (CF). These are associated with a progressive increase in morbidity and mortality. The treatment depending on the isolated microorganism. The β-lactam antibiotics are generally used which are not exempt from adverse reactions. Next, two report of neutropenia cases are described after prolonged use of cefepime in CF patients.


Subject(s)
Humans , Male , Female , Child, Preschool , Cystic Fibrosis/drug therapy , Cefepime/adverse effects , Anti-Bacterial Agents/adverse effects , Neutropenia/chemically induced , Time Factors , Risk Factors , Cystic Fibrosis/complications , Leukocyte Count
15.
J. bras. pneumol ; 45(1): e20170280, 2019. tab, graf
Article in English | LILACS | ID: biblio-990106

ABSTRACT

ABSTRACT Objective: Bone disease is a common comorbidity in patients with cystic fibrosis (CF). We sought to determine risk factors and identify potential biochemical markers for CF-related bone disease (CFBD) in a unique cohort of CF patients with end-stage lung disease undergoing lung transplantation (LTx) evaluation. Methods: All of the CF patients who were evaluated for LTx at our center between November of 1992 and December of 2010 were included in the study. Clinical data and biochemical markers of bone turnover, as well as bone mineral density (BMD) at the lumbar spine and femoral neck, were evaluated. Spearman's rho and multivariate logistic regression analysis were used. Results: A total of 102 adult CF patients were evaluated. The mean age was 28.1 years (95% CI: 26.7-29.5), and the mean body mass index was 17.5 kg/m2 (95% CI: 17.2-18.2). Mean T-scores were −2.3 and −1.9 at the lumbar spine and femoral neck, respectively, being lower in males than in females (−2.7 vs. −2.0 at the lumbar spine and −2.2 vs. −1.7 at the femoral neck). Overall, 52% had a T-score of < −2.5 at either skeletal site. The homozygous Phe508del genotype was found in 57% of patients without osteoporosis and in 60% of those with low BMD. Mean T-scores were not particularly low in patients with severe CFTR mutations. Although the BMI correlated with T-scores at the femoral neck and lumbar spine, serum 25-hydroxyvitamin D and parathyroid hormone levels did not. Conclusions: CFBD is common in CF patients with end-stage lung disease, particularly in males and patients with a low BMI. It appears that CF mutation status does not correlate with CFBD. In addition, it appears that low BMD does not correlate with other risk factors or biochemical parameters. The prevalence of CFBD appears to have recently decreased, most likely reflecting increased efforts at earlier diagnosis and treatment.


RESUMO Objetivo: A doença óssea é uma comorbidade comum em pacientes com fibrose cística (FC). Nosso objetivo foi determinar os fatores de risco e identificar possíveis marcadores bioquímicos de doença óssea relacionada à FC (DOFC) em uma coorte única de pacientes com FC e doença pulmonar terminal submetidos a avaliação para transplante de pulmão (TxP). Métodos: Todos os pacientes com FC avaliados para TxP em nosso centro entre novembro de 1992 e dezembro de 2010 foram incluídos no estudo. Foram avaliados dados clínicos e marcadores bioquímicos de remodelação óssea, bem como a densidade mineral óssea (DMO) na coluna lombar e colo do fêmur. Foram usados rô de Spearman e análise de regressão logística multivariada. Resultados: Foram avaliados 102 pacientes adultos com FC. A média de idade foi de 28,1 anos (IC95%: 26,7-29,5), e a média do índice de massa corporal foi de 17,5 kg/m2 (IC95%: 17,2-18,2). A média do escore T foi de −2,3 e −1,9 na coluna lombar e colo do fêmur, respectivamente, sendo menor nos homens que nas mulheres (−2,7 vs. −2,0 na coluna lombar e −2,2 vs. −1,7 no colo do fêmur). No geral, 52% apresentaram escore T < −2,5 em um dos dois sítios esqueléticos. O genótipo homozigoto para Phe508del foi encontrado em 57% dos pacientes sem osteoporose e em 60% daqueles com DMO baixa. A média do escore T não foi particularmente baixa em pacientes com mutações graves do gene CFTR. Embora o IMC tenha se correlacionado com o escore T no colo do fêmur e coluna lombar, os níveis séricos de 25-hidroxivitamina D e paratormônio não o fizeram. Conclusões: A DOFC é comum em pacientes com FC e doença pulmonar terminal, particularmente em homens e pacientes com IMC baixo. O estado de mutação da FC aparentemente não se correlaciona com a DOFC. Além disso, aparentemente não há correlação entre DMO baixa e outros fatores de risco ou parâmetros bioquímicos. A prevalência de DOFC parece ter diminuído recentemente, o que provavelmente é reflexo do aumento dos esforços para antecipar o diagnóstico e tratamento.


Subject(s)
Humans , Male , Female , Adult , Osteoporosis/etiology , Cystic Fibrosis/complications , Lung Diseases/complications , Osteoporosis/epidemiology , Parathyroid Hormone/blood , Switzerland/epidemiology , Vitamin D/analogs & derivatives , Vitamin D/blood , Body Mass Index , Bone Density , Logistic Models , Multivariate Analysis , Retrospective Studies , Lung Transplantation , Critical Illness , Bone Remodeling , Sex Distribution , Statistics, Nonparametric , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Cystic Fibrosis/epidemiology , Lung Diseases/epidemiology , Mutation
16.
Clin. biomed. res ; 39(1): 69-74, 2019.
Article in Portuguese | LILACS | ID: biblio-1026176

ABSTRACT

Evidências mostram que o exercício físico gera efeito positivos em indivíduos com fibrose cística, melhorando o prognóstico, a qualidade de vida e reduzindo o número de internações. O estudo se caracteriza como qualitativo e de revisão sistemática. A partir dos descritores "fibrose cística", "criança" e "exercício físico" realizou-se uma busca por artigos nas bases de dados do Portal de Periódicos da CAPES e Medline/PubMed. Desta busca originaram-se inicialmente 125 artigos. Após refinamento, foram selecionados 14 artigos que se adequaram aos critérios propostos pela pesquisa. Utilizou-se como critérios de inclusão a leitura prévia dos resumos, nos quais deveriam conter informações referentes à utilização do exercício físico como tratamento da fibrose cística, ser um estudo com população alvo ≤ 20 anos de idade e que utilizassem protocolo validado de exercícios físicos. Os artigos encontrados trazem relatos dos benefícios da prática regular de exercícios físicos no tratamento efetivo de pacientes com fibrose cística na infância e adolescência. Os achados são referentes à melhora da capacidade cardiorrespiratória, da postura, além de outros benefícios como aumentos na autoestima e na qualidade de vida destes indivíduos. Grande parte dos trabalhos avaliam o efeito do exercício físico a curto prazo, demonstrando que os dados oriundos dos estudos atuais são escassos e que são necessários mais estudos para maior esclarecimento do tema. (AU)


Evidence shows that physical exercise produces positive effects on individuals with cystic fibrosis, improving their prognosis and quality of life and reducing the number of hospitalizations. This study was qualitative and consisted of a systematic review. Using the descriptors "cystic fibrosis," "child" and "physical exercise," a search for articles was carried out in the databases of CAPES Journals Portal and MEDLINE/PubMed. This initial search yielded 125 articles. After narrowing, 14 articles meeting the study criteria were selected. Abstracts were screened in search of the following inclusion criteria: having information on the use of physical exercise as treatment of cystic fibrosis, having a target population aged ≤ 20 years, and using a validated exercise protocol. The articles report the benefits of regular physical exercise in the effective treatment of patients with cystic fibrosis in childhood and adolescence. The findings show improved cardiorespiratory capacity and posture, as well as other benefits such as increased self-esteem and quality of life of these individuals. Most studies evaluated short-term effects of physical exercise, demonstrating that current data are still incipient and further studies are needed to investigate the subject. (AU)


Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Adult , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Exercise/physiology , Child Health , Adolescent Health
17.
Rev. bras. enferm ; 71(6): 2891-2898, Nov.-Dec. 2018.
Article in English | LILACS, BDENF | ID: biblio-977593

ABSTRACT

ABSTRACT Objective: To understand the experience of adults living with cystic fibrosis. Method: A qualitative study based on the social phenomenology by Alfred Schütz, carried out with 12 adults interviewed in 2016. The statements were analyzed and organized into concrete categories. Results: The following categories were evidenced: "The biopsychosocial impact of the disease on daily life", "Social prejudice as a generator of embarrassment", "Coping strategies" and "Fear, uncertainties and the desire to carry out life projects". Final considerations: The understanding of the experience lived by adults with cystic fibrosis allowed unveiling intersubjective aspects experienced by this public that should be considered by health professionals in the care of this group. It is up to the professionals involved in assisting these people to develop care strategies aimed at completeness, respect for the world of meanings of each individual, their life history, and intersubjectivity that is specially built in the relationship between professionals and people with cystic fibrosis.


RESUMEN Objetivo: Comprender la experiencia de personas adultas que conviven con la fibrosis quística. Método: Estudio cualitativo fundamentado en la fenomenología social de Alfred Schütz, realizado con 12 adultos entrevistados en 2016. Los testimonios fueron analizados y organizados en categorías concretas. Resultados: Fueron evidenciadas las categorías: "Impacto biopsicosocial de la enfermedad en el día a día", "Preconcepto social como generador de bochorno", "Estrategias de enfrentamiento" y "Miedo, incertidumbres y deseos de realizar proyectos de vida". Consideraciones finales: La comprensión de la experiencia atravesada por adultos con fibrosis quística permitió develar aspectos intersubjetivos experimentados por ese grupo, que deben considerar los profesionales de salud para su cuidado. Corresponde a los profesionales involucrados en la atención de estas personas elaborar estrategias de atención que apunten a la integralidad, el respeto al mundo de significaciones de cada uno, su historia de vida y la intersubjetividad especialmente construida en la relación profesional/persona con fibrosos quística.


RESUMO Objetivo: Compreender a experiência de pessoas adultas que convivem com a fibrose cística. Método: Estudo qualitativo fundamentado na fenomenologia social de Alfred Schütz, realizado com 12 adultos entrevistados em 2016. Os depoimentos foram analisados e organizados em categorias concretas. Resultados: Foram evidenciadas as categorias: "O impacto biopsicossocial da doença no cotidiano", "O preconceito social como gerador de constrangimento", "Estratégias de enfrentamento" e "Medo, incertezas e desejo de realizar projetos de vida". Considerações finais: A compreensão da experiência vivida por adultos com fibrose cística possibilitou desvelar aspectos intersubjetivos vivenciados por esse público que devem ser considerados pelos profissionais da saúde no cuidado a esse grupo. Cabe aos profissionais envolvidos na assistência a essas pessoas elaborar estratégias de cuidado que visem à integralidade, ao respeito ao mundo de significações de cada um, à sua história de vida e à intersubjetividade especialmente construída na relação profissional/pessoa com fibrose cística.


Subject(s)
Humans , Male , Female , Adult , Quality of Life/psychology , Adaptation, Psychological , Cystic Fibrosis/complications , Quality of Health Care/standards , Cost of Illness , Cystic Fibrosis/psychology , Qualitative Research
18.
Rev. Assoc. Med. Bras. (1992) ; 64(11): 1032-1037, Nov. 2018. tab, graf
Article in English | LILACS | ID: biblio-976796

ABSTRACT

SUMMARY OBJECTIVE: to assess the progression of pediatric cystic fibrosis (CF) patients' nutritional status during the first 12 months after diagnosis and to establish its association with neonatal screening and clinical variables. Patients were recruited from two reference centers in Southern Brazil. METHODS: Retrospective cohort study was carried out with all the patients diagnosed between 2009 and 2014. Anthropometric, clinic and neonatal screening were collected from medical files. Analysis of anthropometric markers over time was performed by generalized estimating equations. A multivariate regression analysis model to predict the Δ percentile body mass index (BMI) (BMI percentile difference between one year after the treatment and BMI percentile at diagnosis) was done. RESULTS: Forty-seven patients were included in the study. Analysis of nutritional data over the period between six months and one year after diagnosis showed significant improvement of BMI, weight/age and weight/height percentiles and Z scores. The neonatal screening was associated with a significant increase of 31.2 points in ΔBMI percentile at the one-year evaluation (p<0.05). On the other hand, a one-point increase of initial BMI percentile was associated with a reduction of 0.6 points in ΔBMI percentile. CONCLUSION: This study demonstrated the role of neonatal screening in the nutritional status of patients diagnosed with CF in the first year after diagnosis. Early diagnosis can significantly contribute to the achievement of appropriate anthropometric indicators and important nutritional recovery of CF patients.


RESUMO OBJETIVO: Avaliar a evolução do estado nutricional de pacientes pediátricos com fibrose cística (FC), provenientes de dois centros de referência do sul do Brasil, durante os 12 primeiros meses após o diagnóstico e estabelecer associação com a triagem neonatal e com variáveis clínicas. MÉTODOS: Estudo de coorte retrospectivo realizado com todos os pacientes diagnosticados entre 2009 e 2014. Foram coletados dados antropométricos, clínicos e de realização da triagem neonatal a partir dos prontuários dos pacientes. A análise dos indicadores antropométricos ao longo do tempo foi realizada por equações de estimativas generalizadas. Utilizou-se o modelo de análise de regressão multivariada para predizer o D percentil índice de massa corporal - IMC/I (diferença entre percentil de IMC/I um ano após o tratamento e percentil de IMC/I no momento do diagnóstico). RESULTADOS: Participaram do estudo 47 pacientes. A análise dos dados antropométricos ao longo do período de seis meses e um ano após o diagnóstico demonstrou melhora significativa dos parâmetros de percentil e escore Z de IMC/I, peso/idade e peso/estatura em cada período analisado. A realização da triagem neonatal foi associada com um aumento significativo de 31,2 pontos no Δ percentil de IMC/I durante o período de um ano (p<0,05). Por outro lado, um ponto a mais de percentil de IMC/I inicial foi associado com uma redução de 0,6 ponto no Δ percentil de IMC/I (p<0,01). CONCLUSÃO: O presente estudo evidencia o papel da triagem neonatal na evolução antropométrica de pacientes com FC no primeiro ano após o diagnóstico. O diagnóstico precoce pode contribuir significativamente para a recuperação nutricional desses pacientes.


Subject(s)
Humans , Male , Female , Infant, Newborn , Child, Preschool , Nutritional Status , Neonatal Screening/methods , Cystic Fibrosis/diagnosis , Nutrition Disorders/diagnosis , Body Height , Body Weight , Brazil , Body Mass Index , Anthropometry , Retrospective Studies , Cystic Fibrosis/complications , Nutrition Disorders/etiology , Nutrition Disorders/prevention & control
19.
J. pediatr. (Rio J.) ; 94(5): 504-510, Sept.-Oct. 2018. tab
Article in English | LILACS | ID: biblio-975994

ABSTRACT

Abstract Objectives: The aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis and to describe the sociodemographic, clinical, and laboratory profile of these patients. Methods: This was a retrospective, descriptive, and analytical study of 55 patients diagnosed with cystic fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered. Results: Hepatobiliary disease was diagnosed in 16.4% of the patients and occurred as an initial manifestation of cystic fibrosis in 55.6% of these cases. The diagnosis of hepatopathy occurred before or concomitantly with the diagnosis of cystic fibrosis in 88.9% of the children. All patients with hepatobiliary disease were considered non-white, with a predominance of females (77.8%) and median (IQR) of 54 (27-91) months. Compared with the group without hepatobiliary disease, children with liver disease had a higher frequency of severe mutations identified in the CFTR gene (77.8% vs. 39.6%, p = 0.033) and severe pancreatic insufficiency (88.9% vs. 31.6%, p = 0.007). Conclusion: The frequency of hepatobiliary disease was high, with a very early diagnosis of the disease and its complications in the studied series. A statistical association was observed between the occurrence of hepatobiliary disease and the presence of pancreatic insufficiency and severe mutations in the CFTR gene. It is emphasized that cystic fibrosis is an important differential diagnosis of liver diseases in childhood.


Resumo Objetivos: Os objetivos do estudo foram determinar a frequência da doença hepatobiliar em pacientes com fibrose cística e descrever o perfil sociodemográfico, clínico e laboratorial destes. Métodos: Estudo retrospectivo, descritivo e analítico de 55 pacientes com diagnóstico de fibrose cística, entre três meses e 21 anos, acompanhados de janeiro de 2008 a junho de 2016 em um centro de referência. Foi realizada consulta aos prontuários médicos, registrando-se os dados sociodemográficos, clínicos e laboratoriais, incluindo-se alterações hepatobiliares, exames de imagem, estudos genéticos, biópsias hepáticas e endoscopias digestivas altas. Resultados: A doença hepatobiliar foi diagnosticada em 16,4% dos pacientes e ocorreu como manifestação inicial da fibrose cística em 55,6% destes casos. O diagnóstico da hepatopatia ocorreu antes ou concomitante ao diagnóstico da fibrose cística em 88,9% das crianças. Todos os pacientes com doença hepatobiliar foram considerados não brancos, havendo predominância do sexo feminino (77,8%) e mediana (I.I.Q) de idade de 54 (27-91) meses. Em comparação com o grupo sem doença hepatobiliar, as crianças com hepatopatia tiveram maior frequência de mutações graves no gene CFTR identificadas (77,8% vs 39,6%; p = 0,033) e de insuficiência pancreática grave (88,9% vs 31,6%; p = 0,007). Conclusão: A frequência de doença hepatobiliar foi elevada, observando-se um diagnóstico muito precoce da mesma e de suas complicações na casuística estudada. Houve associação estatística entre a ocorrência de doença hepatobiliar e a presença de insuficiência pancreática e de mutações graves do gene CFTR. Enfatiza-se que a fibrose cística represente um importante diagnóstico diferencial de hepatopatias na infância.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Exocrine Pancreatic Insufficiency/etiology , Cystic Fibrosis/complications , Liver Diseases/etiology , Mutation/genetics , Exocrine Pancreatic Insufficiency/genetics , Socioeconomic Factors , Retrospective Studies , Cystic Fibrosis/genetics , Genotype , Liver Diseases/genetics
20.
Rev. Paul. Pediatr. (Ed. Port., Online) ; 36(3): 322-328, jul.-set. 2018. tab, graf
Article in Portuguese | LILACS | ID: biblio-977067

ABSTRACT

RESUMO Objetivo: Analisar a associação do exercício físico e da atividade física com a percepção da qualidade do sono em adolescentes. Métodos: Trata-se de um estudo com abordagem quantitativa que integra o levantamento epidemiológico transversal de base escolar e abrangência estadual cuja amostra foi constituída por 6.261 adolescentes (14 a 19 anos), selecionados por meio de uma estratégia de amostragem aleatória de conglomerados. Os dados foram coletados a partir do questionário Global School-based Student Health Survey. O teste do qui-quadrado e a regressão logística binária foram utilizados nas análises dos dados. Resultados: Na amostra, 29% dos adolescentes não faziam exercício e não foram classificados como fisicamente ativos. Os adolescentes que não praticavam exercício físico tinham mais chances de apresentar uma percepção negativa da qualidade do sono (OR 1,13, IC95% 1,04-1,28; p=0,043). Não foi encontrada associação entre o nível de atividade física e a percepção da qualidade do sono (OR 1,01, IC95% 0,89-1,14; p=0,868). Ao serem avaliadas as práticas de forma isolada ou simultânea, constatou-se que aqueles que praticavam exercício físico apresentavam menor chance de terem uma percepção negativa da qualidade do sono (OR 0,82, IC95% 0,71-0,95) e, ao praticarem exercício e, paralelamente, terem uma vida fisicamente ativa, essas chances diminuíam ainda mais (OR 0,79, IC95% 0,68-0,93). Conclusões: Ser classificado como fisicamente ativo, por si só, não foi suficiente para uma melhor percepção da qualidade do sono, pois apenas a prática de exercício físico apresentou tal associação.


ABSTRACT Objective: To analyze the association of exercises and physical activity with the perception of sleep quality by adolescents. Methods: This is a cross-sectional epidemiological survey with statewide coverage, whose sample was composed of 6,261 adolescents (14-19 years old) who were selected by random sampling of conglomerates. The Global School-Based Student Health Survey questionnaire was used for data collection. The chi-square test and the binary logistic regression were applied for data analyses. Results: In the sample, 29% of adolescents did not exercise and were not classified as physically active. Adolescents who did not exercise were more likely to present a negative perception of sleep quality (OR 1.13, 95%CI 1.04-1.28, p=0.043). No association between the level of physical activity and the perception of sleep quality was found (OR 1.01, 95%CI 0.89-1.14, p=0.868). Those who practiced exercises only had less chance of perceiving sleep quality as poor (OR 0.82, 95%CI 0.71-0.95). However, those who practiced exercise and had a physically active life had less chances of having a negative perception of their sleep (OR 0.79, 95%CI 0.68-0.93). Conclusions: Practicing physical activity alone was not enough to increase the chances of positive sleep quality perception. Only physical exercise had a positive association with sleep quality perception.


Subject(s)
Humans , Child , Aspergillosis, Allergic Bronchopulmonary/immunology , Aspergillus fumigatus , Immunoglobulin E , Cystic Fibrosis/complications
SELECTION OF CITATIONS
SEARCH DETAIL