RESUMO

BACKGROUND: We present the first case to our knowledge of a spontaneous twin pregnancy in a 16-year-old Caucasian patient with cystic fibrosis and systemic lupus erythematosus. Cystic fibrosis is one of the most common autosomal recessive genetic disorders and primarily affects the respiratory and digestive systems. Systemic lupus erythematosus is a chronic inflammatory disease of unknown cause that affects nearly every organ. Patients with cystic fibrosis or systemic lupus erythematosus are progressively having longer life expectancy and better quality of life, which has led a greater number of female patients reporting the desire to become mothers. CASE PRESENTATION: We present a case of a Caucasian 16-year-old pregnant with twins being treated for both cystic fibrosis and systemic lupus erythematosus. She has two CFTR mutations: p.F508del and 1812_1G>A. In the second trimester, she was admitted for possible preterm labor, which was successfully stopped. The patient's nutritional status worsened, and she had a pulmonary exacerbation as well as a flare of systemic lupus erythematosus. At the 28th gestational week, she presented with a massive hemoptysis episode. The cesarean delivery had no complications, and there were no serious immediate postpartum complications. DISCUSSION AND CONCLUSIONS: While adolescent pregnancies in and of themselves are considered high risk for both the young mothers and their children, they are further complicated when the mother has two chronic diseases and a twin pregnancy. We achieved positive results using a multidisciplinary approach; however, the risks involved were so high that major efforts are to be taken by our medical community to prevent unplanned pregnancies in all patients with cystic fibrosis, especially when a serious comorbidity like the one in this case is present.

Assuntos

RESUMO

OBJECTIVE: Patients with cystic fibrosis (CF) have a high incidence of pubertal and growth delay. In girls with CF, pubertal delay has an important psychological impact. Still, only a few studies have explored the occurrence of pubertal delay in girls with CF. The aims of this study were to compare the pubertal development of girls with CF compared with healthy controls regarding Tanner staging and pelvic ultrasound and, in girls with CF, correlate the findings with those of spirometry, body mass index, Shwachman-Kulczycki score (SKS), and genotyping. METHODS: This was a cross-sectional, case-control study including 35 girls with CF aged 6-17 years and following up at the Pediatric Pulmonology Outpatient Clinic of a tertiary hospital. These patients were compared with 59 healthy controls who had undergone pelvic ultrasound as part of another study conducted by the same group. Girls with CF were consecutively enrolled in the study during their annual routine check-up visit. Data collected in the CF group included spirometry and anthropometric results, SKS values, bone age, occurrence of current cystic fibrosis-related diabetes (CFRD) and Pseudomonas aeruginosa colonization, history of meconium ileus, genotype, ultrasound parameters, and Tanner stage. RESULTS: Pelvic ultrasound findings and Tanner stage reflected less pubertal development in girls with CF compared with healthy controls. Pubertal stage in patients with CF who presented CFRD (3.17 ± 1.16), had chronic colonization by Pseudomonas aeruginosa (3.10 ± 1.10), or were homozygous for the F508del mutation (1.91 ± 1.30) was more delayed than in controls (3.41 ± 1.41). Tanner stage correlated with age at menarche, bone age, and anthropometric and ultrasound data. CONCLUSION: Girls with CF presented a delay in pubertal development evaluated by Tanner stage and ultrasound parameters, which was more evident in the presence of comorbidities.

Assuntos

RESUMO

ABSTRACT Objective: Patients with cystic fibrosis (CF) have a high incidence of pubertal and growth delay. In girls with CF, pubertal delay has an important psychological impact. Still, only a few studies have explored the occurrence of pubertal delay in girls with CF. The aims of this study were to compare the pubertal development of girls with CF compared with healthy controls regarding Tanner staging and pelvic ultrasound and, in girls with CF, correlate the findings with those of spirometry, body mass index, Shwachman-Kulczycki score (SKS), and genotyping. Subjects and methods: This was a cross-sectional, case-control study including 35 girls with CF aged 6-17 years and following up at the Pediatric Pulmonology Outpatient Clinic of a tertiary hospital. These patients were compared with 59 healthy controls who had undergone pelvic ultrasound as part of another study conducted by the same group. Girls with CF were consecutively enrolled in the study during their annual routine check-up visit. Data collected in the CF group included spirometry and anthropometric results, SKS values, bone age, occurrence of current cystic fibrosis-related diabetes (CFRD) and Pseudomonas aeruginosa colonization, history of meconium ileus, genotype, ultrasound parameters, and Tanner stage. Results: Pelvic ultrasound findings and Tanner stage reflected less pubertal development in girls with CF compared with healthy controls. Pubertal stage in patients with CF who presented CFRD (3.17 ± 1.16), had chronic colonization by Pseudomonas aeruginosa (3.10 ± 1.10), or were homozygous for the F508del mutation (1.91 ± 1.30) was more delayed than in controls (3.41 ± 1.41). Tanner stage correlated with age at menarche, bone age, and anthropometric and ultrasound data. Conclusions: Girls with CF presented a delay in pubertal development evaluated by Tanner stage and ultrasound parameters, which was more evident in the presence of comorbidities.

Assuntos

RESUMO

This study compared the electromyographic response, the blood lactate concentration (BLC), and the maximum number of repetitions (MNR) between protocols of different concentric/eccentric duration taken to muscle failure. This comparison may help to understand how different concentric/eccentric duration may influence performance and the central and metabolic responses in trained men. Seventeen strength-trained men performed two protocols in a counterbalanced design. Three sets of the Smith bench press exercise were performed to failure at 60% of the one-repetition maximum (1RM) using each protocol (4-s concentric/2-s eccentric [4 s: 2 s]; and 2-s concentric/4-s eccentric [2 s: 4 s]). The normalized root mean square (EMGRMS) and the mean frequency (EMGMF) of the electromyographic signals for the pectoralis major and the triceps brachii were compared in the first, middle, and last repetitions. The BLC was assessed at rest, during and after the test sessions. To compare the EMG and BLC, a 3-way ANOVA with repeated measures with a post hoc Tukey's test was used. To compare the MNR performed across the sets, an ANOVA-type rank test with the Dunn's post hoc test was used. The ANOVA indicated a greater EMGRMS for Protocol 4 s: 2 s in the pectoralis major and a lower EMGMF for Protocol 4 s: 2 s in the triceps brachii at the middle and last repetitions. Both protocols increased the EMGRMS and decreased the EMGMF across repetitions. Despite the results show different levels of activation and neuromuscular fatigue between protocols, the BLC and the MNR were similar.

RESUMO

Abstract Objective To evaluate height, sexual maturation, and the difference between final and expected height in girls with juvenile idiopathic arthritis and no glucocorticoid treatment for at least six months, as compared to a group of healthy girls. Methods This cross-sectional study involved 44 girls with juvenile idiopathic arthritis, diagnosed according to the International League of Associations for Rheumatology criteria, and 59 healthy controls aged between 8 and 18 (incomplete) years with no comorbid chronic diseases. Demographic data were collected from all participants, and disease and treatment variables were compiled for the patient group. Anthropometric measurements were converted into Z-scores based on World Health Organization standards. Sexual maturation was classified according to Tanner stages. Results Body mass index and height Z-scores were lower in girls with juvenile idiopathic arthritis as compared to control participants. These values differed significantly in Tanner stage II. Three (6.8%) girls with juvenile idiopathic arthritis had height-for-age Z-scores <−2 (short stature). Girls with polyarticular juvenile idiopathic arthritis and higher cumulative glucocorticoid doses were significantly more likely to present with short stature. The percentage of prepubertal girls in the juvenile idiopathic arthritis group was significantly higher than that observed in the control group, (p = 0.012). Age of menarche, adult height, and the difference between actual and expected height did not differ between groups. Conclusion These findings suggest that even six months after the suspension of glucocorticoid treatment, children with polyarticular/systemic juvenile idiopathic arthritis subtypes are still susceptible to low height and delayed puberty.

Resumo Objetivo Avaliar a estatura, maturação sexual e a diferença entre a estatura final e a esperada em meninas com artrite idiopática juvenil (AIJ) sem tratamento com glicocorticoides por pelo menos seis meses, em comparação com um grupo de meninas saudáveis. Métodos Este estudo transversal avaliou 44 meninas com artrite idiopática juvenil, diagnosticadas de acordo com os critérios da International League of Associations for Rheumatology e 59 controles saudáveis, entre oito e 18 anos (incompletos) sem comorbidades por doenças crônicas. Os dados demográficos foram coletados de todos os participantes e as variáveis de doença e tratamento foram compiladas para o grupo de pacientes. As medidas antropométricas foram convertidas em escores-z com base nos padrões da Organização Mundial da Saúde. A maturação sexual foi classificada de acordo com os estágios de Tanner. Resultados Índice de massa corporal e escores-z de estatura foram menores em meninas com artrite idiopática juvenil em comparação com os participantes-controle. Esses valores diferiram significativamente no estágio II de Tanner. Três (6,8%) meninas com artrite idiopática juvenil tinham escores-z de estatura para idade < -2 (baixa estatura). Meninas com artrite idiopática juvenil poliarticular e doses cumulativas de glicocorticoides foram significativamente mais propensas a apresentar baixa estatura. A porcentagem de meninas pré-púberes no grupo artrite idiopática juvenil foi significativamente maior do que a observada no grupo controle (p = 0,012). A idade da menarca, a estatura adulta e a diferença entre a estatura real e a esperada não diferiram entre os grupos. Conclusão Esses achados sugerem que, mesmo após seis meses da suspensão do tratamento com glicocorticoides, as crianças com os subtipos poliarticular/sistêmico de AIJ ainda são suscetíveis a baixa estatura e atraso na puberdade.

Assuntos

RESUMO

OBJECTIVE: To evaluate height, sexual maturation, and the difference between final and expected height in girls with juvenile idiopathic arthritis and no glucocorticoid treatment for at least six months, as compared to a group of healthy girls. METHODS: This cross-sectional study involved 44 girls with juvenile idiopathic arthritis, diagnosed according to the International League of Associations for Rheumatology criteria, and 59 healthy controls aged between 8 and 18 (incomplete) years with no comorbid chronic diseases. Demographic data were collected from all participants, and disease and treatment variables were compiled for the patient group. Anthropometric measurements were converted into Z-scores based on World Health Organization standards. Sexual maturation was classified according to Tanner stages. RESULTS: Body mass index and height Z-scores were lower in girls with juvenile idiopathic arthritis as compared to control participants. These values differed significantly in Tanner stage II. Three (6.8%) girls with juvenile idiopathic arthritis had height-for-age Z-scores <-2 (short stature). Girls with polyarticular juvenile idiopathic arthritis and higher cumulative glucocorticoid doses were significantly more likely to present with short stature. The percentage of prepubertal girls in the juvenile idiopathic arthritis group was significantly higher than that observed in the control group, (p=0.012). Age of menarche, adult height, and the difference between actual and expected height did not differ between groups. CONCLUSION: These findings suggest that even six months after the suspension of glucocorticoid treatment, children with polyarticular/systemic juvenile idiopathic arthritis subtypes are still susceptible to low height and delayed puberty.

Assuntos

RESUMO

INTRODUCTION: Tocilizumab (TCZ) is a recombinant humanized monoclonal antibody and IL-6 receptor antagonist, currently approved for the treatment of systemic juvenile idiopathic arthritis (sJIA) and polyarticular juvenile idiopathic arthritis (pJIA) in children aged 2 years or older refractory to conventional treatment. The most common adverse events in patients treated with TCZ were infections, especially in the respiratory tract. The most frequent laboratory abnormalities were altered liver function, neutropenia and elevated cholesterol levels. Areas covered: The safety of TCZ in the treatment of children with JIA was determined based on a review of published clinical trials, including two multicenter studies of patients with sJIA and pJIA (the TENDER and CHERISH trials, respectively). The frequency of adverse events (AEs), serious adverse events (SAEs) and deaths reported in these studies was analyzed and discussed. Expert opinion: TCZ was effective and well tolerated in the treatment of severe forms of sJIA and pJIA, and can be considered a treatment of choice for these conditions. The risk of infections and laboratory abnormalities, such as neutropenia, should be constantly monitored. There is still a need for comparative studies of the risks and benefits of biological agents in patients with refractory JIA.

Assuntos

RESUMO

This study analyzed the effect of different repetition durations on electromyographic and blood lactate responses of the bench press exercise. Fifteen recreationally trained male volunteers completed two training protocols, matched for intensity (% one-repetition maximum; 1RM), number of sets, number of repetitions, and rest intervals. One of the protocols was performed with a repetition duration of 4 s (2 s concentric: 2 s eccentric; 2:2 protocol), whereas the second protocol had a repetition duration of 6 s (2 s concentric: 4 s eccentric; 2:4 protocol). The results showed higher normalized integrated electromyography (pectoralis major and triceps brachii) for the 2:4 protocol. Blood lactate concentration was also higher in the 2:4 protocol across all sets. These results show that adding 2 s to the eccentric action in matched training protocols increases muscle activation and blood lactate response, which reinforces the notion that increasing repetition duration is an alternative load progression in resistance training.

Assuntos

RESUMO

OBJETIVO: Investigar o aleitamento materno exclusivo em bebês com fissura de lábio e/ou palato e sua associação com o tipo de fissura. MÉTODOS: Foram coletados dados de 137 prontuários, obtidos pela pediatra na primeira consulta. A amostra constituiu-se das fichas de pediatria de bebês de ambos os gêneros, com fissura de lábio e/ou palato, nascidos a termo e sem outras anomalias associadas, que chegaram para a primeira consulta entre zero e 12 meses. Os dados foram analisados estatisticamente. RESULTADOS: Houve predomínio da fissura transforame incisivo em bebês do gênero masculino. Os bebês apresentavam média de idade de 53 dias e mediana de 33 dias na primeira consulta. Quanto ao aleitamento materno exclusivo, este ocorreu em 7,3 por cento do total da amostra, sendo 6,57 por cento no grupo pré-forame incisivo e 0,73 por cento no grupo pós-forame incisivo, mas não ocorreu no grupo transforame incisivo. Houve associação significativa entre o tipo de fissura e o aleitamento. CONCLUSÃO: O aleitamento exclusivo está presente em pequena parte da amostra total de bebês com fissura de lábio e/ou palato, sendo frequente na fissura pré-forame incisivo.

PURPOSE: To investigate exclusive breastfeeding in infants with cleft lip and/or palate and its association with the type of cleft. METHODS: Data recorded by the pediatrician during the first consultation were obtained from 137 clinical charts. The sample consisted of the pediatric records of infants of both genders, with cleft lip and/or palate, born full term and without any other associated anomalies, who arrived for the first consultation between zero and 12 months of age. Data were statistically analyzed. RESULTS: There was predominance of complete cleft lip and palate in male infants. Subjects' mean age was 53 days, with median of 33 days on the date of first consultation. Exclusive breastfeeding occurred in 7.3 percent of the total sample - 6.57 percent in cleft lip group, 0.73 percent in the cleft palate, and none in the complete cleft lip and palate group. There was significant association between type of cleft and breastfeeding. CONCLUSION: Exclusive breastfeeding is present in only a small part of the total sample of infants with cleft lip and/or palate, and is frequent in infants with incomplete cleft lip.

Assuntos

RESUMO

Juvenile idiopathic arthritis (JIA) is the most prevalent chronic arthropathy in childhood and adolescence. The prevalence of metabolic syndrome, as well as obesity, is increasing rapidly in all age groups, including children. Metabolic syndrome is defined as a cluster of risk factors for cardiovascular disease and type 2 diabetes mellitus, including abdominal obesity, insulin resistance, dyslipidemia and hypertension. Besides those components, inflammation has been increasingly considered as a significant component of metabolic syndrome and obesity, and patients with diseases characterized by the presence of chronic inflammation, such as JIA, could represent special risk groups. Glucocorticoids are used routinely in the management of the inflammation of JIA, in high doses and long-term. Long-term use of the glucocorticoids can cause to insulin resistance, hypertension, and obesity, increasing the risk of metabolic syndrome. The aim of this study is to review the literature on the prevalence of different components of metabolic syndrome in patients with JIA. We observed that the data on metabolic syndrome and its components in those patients are very scarce and more studies needed, in view of the potential increased risk of cardiovascular disease.

Assuntos

RESUMO

This article verifies the consumption of salt and food rich in sodium and the blood pressure of schoolchildren of a private and a public school in the city of Barra do Ribeiro, Rio Grande do Sul State, Brazil. It is a cross-sectional study with standardized methods of anthropometric and blood pressure measures. Parents and tutors answered a questionnaire about socio-economic conditions, family risk factors regarding hypertension and feeding habits of the student (frequency questionnaire). 81 students were evaluated, from those 42 (51.90%) medium age were 8.3 +/- 3.2 years. 45 (55.60%) studied in the private school and 36 (44.40%) in the public school. Findings show two children with systolic hypertension, both from the public school (p = 0.194), yet this may be found by chance. Similar fact may have occurred with the four cases of diastolic hypertension; being three of them from the private school. But there was not significant difference between the groups (p = 0.625). The medium consumption of salt found in this population was of 7.66 g (3098.81 mg or 133.86 mEq), which is above the recommended in the present literature. Canned food was related to be rich in sodium and to be associated with high levels of systolic blood pressure. In conclusion, as higher the salt consumption, higher the systolic blood pressure.

Assuntos

RESUMO

O objetivo do estudo foi verificar o consumo de sal e alimentos ricos em sódio e a pressão arterial de escolares de uma escola da rede privada e de outra da rede pública, em Barra do Ribeiro, no Estado do Rio Grande do Sul. Trata-se de um estudo transversal. Foram realizadas medidas antropométricas e de pressão arterial, seguindo métodos padronizados. Os responsáveis pelos escolares responderam questionário com questões referentes às condições socioeconômicas, fatores de risco familiares para hipertensão e hábitos alimentares do escolar (questionário de frequência). Foram avaliados 81 escolares, 42 (51,90 por cento) com idade média 8,3 ± 3,2 anos. Dentre os escolares, 45 (55,60 por cento) eram da rede privada e 36 (44,40 por cento), da rede pública. Foram encontrados dois escolares com hipertensão arterial sistólica e ambos eram da escola da rede pública (p= 0,194); no entanto, o achado pode ter sido casual. Fato seme-lhante pode ter ocorrido com os quatro escolares com hipertensão arterial diastólica; desses, três eram da rede privada. Porém, não houve diferença significativa entre os grupos (p= 0,625). O consumo médio de sal foi de 7,66g (3098,81 mg da Na ou 133,86 mEq). O alimento rico em sódio correlacionado a níveis elevados de pressão arterial sistólica foi o enlatado. Constatamos que, quanto maior o consumo de sal, maior a pressão arterial sistólica. Nessa população, o consumo médio de sal encontra-se acima do recomendado pela literatura atualmente.

This article verifies the consumption of salt and food rich in sodium and the blood pressure of schoolchildren of a private and a public school in the city of Barra do Ribeiro, Rio Grande do Sul State, Brazil. It is a cross-sectional study with standardized methods of anthropometric and blood pressure measures. Parents and tutors answered a questionnaire about socio-economic conditions, family risk factors regarding hypertension and feeding habits of the student (frequency questionnaire). 81 students were evaluated, from those 42 (51.90 percent) medium age were 8.3 ± 3.2 years. 45 (55.60 percent) studied in the private school and 36 (44.40 percent) in the public school. Findings show two children with systolic hypertension, both from the public school (p = 0.194), yet this may be found by chance. Similar fact may have occurred with the four cases of diastolic hypertension; being three of them from the private school. But there was not significant difference between the groups (p = 0.625). The medium consumption of salt found in this population was of 7.66g (3098.81 mg or 133.86 mEq), which is above the recommended in the present literature. Canned food was related to be rich in sodium and to be associated with high levels of systolic blood pressure. In conclusion, as higher the salt consumption, higher the systolic blood pressure.

Assuntos

RESUMO

A Artrite Idiopática Juvenil (AIJ) é a artropatia crônica mais prevalente na infância e na adolescência. A prevalência da síndrome metabólica, assim como da obesidade, vem apresentando rápido aumento, atingindo todas as faixas etárias, inclusive a infância. A síndrome metabólica é caracterizada por um conjunto de riscos para doença cardiovascular e diabetes melito tipo 2, abrangendo adiposidade abdominal, resistência à insulina, dislipidemias e hipertensão arterial sistêmica. Além desses componentes, a inflamação tem sido reconhecida cada vez mais como um fator importante na síndrome metabólica e na obesidade, e pacientes com doenças caracterizadas por processos inflamatórios crônicos, como a AIJ, poderiam representar grupos de risco especiais. Os glicocorticoides são utilizados rotineiramente no controle da inflamação da AIJ, em doses elevadas e com uso prolongado. O uso crônico do glicocorticoide pode induzir resistência à insulina, hipertensão arterial sistêmica e obesidade, aumentando o risco de desenvolver síndrome metabólica. O objetivo deste artigo é revisar a literatura sobre a prevalência dos diversos componentes da síndrome metabólica em pacientes com AIJ. Observamos que, nesses pacientes, os dados sobre síndrome metabólica e seus componentes são muito escassos e mais estudos se fazem necessários, tendo em vista o potencial impacto no aumento do risco de doença cardiovascular.

Juvenile idiopathic arthritis (JIA) is the most prevalent chronic arthropathy in childhood and adolescence. The prevalence of metabolic syndrome, as well as obesity, is increasing rapidly in all age groups, including children. Metabolic syndrome is defined as a cluster of risk factors for cardiovascular disease and type 2 diabetes mellitus, including abdominal obesity, insulin resistance, dyslipidemia and hypertension. Besides those components, inflammation has been increasingly considered as a significant component of metabolic syndrome and obesity, and patients with diseases characterized by the presence of chronic inflammation, such as JIA, could represent special risk groups. Glucocorticoids are used routinely in the management of the inflammation of JIA, in high doses and long-term. Long-term use of the glucocorticoids can cause to insulin resistance, hypertension, and obesity, increasing the risk of metabolic syndrome. The aim of this study is to review the literature on the prevalence of different components of metabolic syndrome in patients with JIA. We observed that the data on metabolic syndrome and its components in those patients are very scarce and more studies needed, in view of the potential increased risk of cardiovascular disease.

Assuntos

RESUMO

Introdução: A nutrição tem papel essencial na sobrevida e qualidade de vida de pacientes com Fibrose Cística (FC). O objetivo desse estudo foi acompanhar a evolução do estado nutricional de pacientes com FC em uso de suplementação nutricional por gastrostomia. Métodos: Estudo longitudinal retrospectivo com todos os pacientes com FC e em uso de suplementação nutricional com dieta hipercalórica por gastrostomia em um centro de referência no sul do Brasil. Os pacientes foram acompanhados nos períodos de 6 meses antes, no momento da instalação da gastrostomia, 6 meses, 1 ano e 2 anos após o procedimento. Os parâmetros nutricionais utilizados foram escore Z para peso/idade e estatura/idade, percentil de índice de massa corpórea (PIMC), além de medidas de composição corporal. Foram obtidos dados de função pulmonar e de ingestão alimentar. Resultados: Foram avaliados dez pacientes, sendo sete (70%) do sexo masculino. A idade média da instalação da gastrostomia foi de 9,8 ± 3,8 anos. O ganho de peso foi de 8,35 Kg (P =0,007) e o de estatura de 16,2 cm (P <0,001). O PIMC passou de 14,2 para 27,1 no período de 2 anos e meio (P =0,282). Com relação à composição corporal houve aumento da reserva muscular e de gordura. Houve queda não significativa na função pulmonar ao longo do tempo. A média de calorias ofertadas comparada com a recomendação alimentar diária passou de 111,6 ± 24,6% para 157,7 ± 37,86% (P =0,048). Conclusão: A suplementação nutricional por gastrostomia resultou em significativo ganho de peso e estatura, bem como aumento da reserva muscular e de gordura, no entanto não houve melhora ou estabilização da função pulmonar nesse grupo de pacientes.

Background: Nutrition plays an essential role in the survival and quality of life of cystic fibrosis (CF) patients. The aim was to follow nutritional status of patients with CF in use of nutritional supplementation by gastrostomy. Methods: Retrospective longitudinal study including all patients with CF on nutritional supplementation by gastrostomy at a referral center in southern Brazil. Patients were followed up in periods of 6 months before the installation of gastrostomy, and then, 6 months, 1 year and 2 years after the procedure. The nutritional parameters used were Z scores for weight/age and height/age, percentile of body mass index (PBMI), and body composition measurements. Data of pulmonary function and food intake were recorded. Results: Ten patients were evaluated, of which seven (70%) were male. The average age when gastrostomy was made was 9.8 ±3.8 years. The weight earning was 8.35 Kg (P =0.007) and the height was 16.2 cm (P <0.001). The PBMI increased from 14.2 to 27.1 over two and a half years (P =0.282). In relation to body composition an increase of muscular and fat reserve was observed. There was no significant decline in pulmonary function over the time. Average calories offered compared with the Recommended Dietary Allowance increased from 111.6 ±24.6% to 157.7 ±37.86% (P =0.048). Conclusion: nutritional supplementation by gastrostomy resulted in significant weight and height gain, as well as increased muscle and fat reserves, however there wasn’t improvement or stabilization of pulmonary function in this group of patients.

Assuntos

RESUMO

OBJECTIVE: .To evaluate associations of growth velocity with inflammatory markers and cumulative dose of glucocorticoid in a cohort of patients with juvenile idiopathic arthritis (JIA) followed during 1 year. METHODS: Seventy-nine patients were evaluated. Disease activity was evaluated by a pediatric rheumatologist. Anthropometric data were classified according to the World Health Organization standards. Tanner growth velocity curves were used; values below the Z-score < or = -2 were considered low growth velocity. Serum concentrations of interleukin 6 (IL-6) were measured by ELISA, and values > 1 pg/ml were considered elevated. RESULTS: The prevalence of low growth velocity was 25.3%, and it was associated with active disease on followup visit, elevated IL-6, erythrocyte sedimentation rate and C-reactive protein, and higher cumulative glucocorticoid doses. In the multiple linear regression with growth velocity as the dependent variable, only elevated IL-6 level was independently and negatively associated with growth velocity. CONCLUSION: Low growth velocity is highly prevalent in children with JIA. Elevated IL-6 levels seem to have an important negative influence on growth in these children, while total glucocorticoid exposure appears to be a secondary factor.

Assuntos

RESUMO

OBJECTIVE: To assess nutritional status in patients with juvenile idiopathic arthritis (JIA) and the influence of inflammatory activity and glucocorticoid use. METHODS: One hundred sixteen patients were evaluated. Disease subtype and disease activity were defined by the attending physician, and the cumulative glucocorticoid dose was recorded from chart review. Percentiles of body mass index (BMI) and triceps skinfold (TSF) and the Z score for height were determined: low weight and low adiposity were diagnosed when BMI and TSF were below the 5th percentile. Short stature was defined by a Z score of height for age < -2. Serum concentration of insulin-like growth factor-I (IGF-I) was measured by radioimmunoassay. RESULTS: The prevalences of low weight, low adiposity, and short stature were 16.4%, 20.7%, and 10.4%, respectively. Low IGF-I serum level was found in 14 patients (12.1%). The factors negatively associated with the Z score of height in multivariable regression analysis were disease duration (partial correlation coefficient -0.370, 95% confidence interval: -0.527 to -0.188; p < 0.001), erythrocyte sedimentation rate (ESR) (-0.357, -0.516 to -0.174; p < 0.001), and polyarticular or systemic disease subtype (-0.290, -0.459 to -0.100; p = 0.003), while there was no significant correlation with the cumulative dose of glucocorticoids (0.086, -0.111 to 0.277; p = 0.391). None of these variables was significantly correlated with the percentiles of BMI and TSF, albeit confidence intervals for these correlation coefficients were relatively large. Patients with a systemic or polyarticular disease subtype tended to present lower percentiles of BMI (p = 0.051). CONCLUSION: Nutritional status is frequently compromised in patients with JIA. Duration and disease subtype and the ESR are factors independently associated with short stature. The cumulative dose of glucocorticoids was not independently associated with short stature or with other nutritional variables, although a relevant negative effect of glucocorticoid dose on BMI and TSF cannot be entirely excluded.

Assuntos

RESUMO

OBJECTIVES: To assess the presence of anti-cyclic citrullinated peptide antibodies in a cohort of patients with juvenile idiopathic arthritis. METHODS: Anti-cyclic citrullinated peptide antibodies was tested for with an enzyme linked immunoabsorbent assay (ELISA) in serum samples of patients from the Hospital de Clínicas de Porto Alegre, all less than 18 years old and with previous diagnosis for at least 6 months. IgMRF (rheumatoid factor) and antinuclear antibodies in Hep-2 cells were also assayed. RESULTS: Serum samples were analyzed from 45 patients. The presence of high levels of anti-cyclic citrullinated peptide antibodies was found in the serum of just one child (2%), who presented sero-positive polyarthritis. CONCLUSIONS: Anti-cyclic citrullinated peptide antibodies can be detected in children with juvenile idiopathic arthritis, but much less frequently than in adults with rheumatoid arthritis. It still remains to be determined whether anti-cyclic citrullinated peptide antibodies can identify a subset of juvenile idiopathic arthritis patients with the potential to progress to adult rheumatoid arthritis.

Assuntos

RESUMO

OBJECTIVES: To assess the presence of anti-cyclic citrullinated peptide antibodies in a cohort of patients with juvenile idiopathic arthritis. METHODS: Anti-cyclic citrullinated peptide antibodies was tested for with an enzyme linked immunoabsorbent assay (ELISA) in serum samples of patients from the Hospital de Clínicas de Porto Alegre, all less than 18 years old and with previous diagnosis for at least 6 months. IgMRF (rheumatoid factor) and antinuclear antibodies in Hep-2 cells were also assayed. RESULTS: Serum samples were analyzed from 45 patients. The presence of high levels of anti-cyclic citrullinated peptide antibodies was found in the serum of just one child (2%), who presented sero-positive polyarthritis. CONCLUSIONS: Anti-cyclic citrullinated peptide antibodies can be detected in children with juvenile idiopathic arthritis, but much less frequently than in adults with rheumatoid arthritis. It still remains to be determined whether anti-cyclic citrullinated peptide antibodies can identify a subset of juvenile idiopathic arthritis patients with the potential to progress to adult rheumatoid arthritis.

RESUMO

OBJETIVOS: Avaliar a presença de anticorpos contra peptídeos cíclicos citrulinados em uma coorte de pacientes com artrite idiopática juvenil. MÉTODOS: A presença de anticorpos contra peptídeos cíclicos citrulinados foi avaliada por ensaio imunoenzimático (ELISA) no soro de pacientes com artrite idiopática juvenil com idade inferior a 18 anos, acompanhados no ambulatório de reumatologia pediátrica do Hospital de Clínicas de Porto Alegre, com tempo de diagnóstico de doença de, no mínimo, 6 meses. Também foi estudada a presença do fator reumatóide IgM e do fator antinuclear em células Hep-2 RESULTADOS: Foram analisadas amostras séricas de 45 pacientes com artrite idiopática juvenil. A presença de títulos elevados de anticorpos contra peptídeos cíclicos citrulinados foi encontrada somente no soro de uma criança (2 por cento), a qual apresentava quadro de poliartrite com fator reumatóide reagente. CONCLUSÕES: O anticorpo contra peptídeos cíclicos citrulinados pode ser detectado em crianças com artrite idiopática juvenil, mas em freqüência muito inferior aos adultos com artrite reumatóide. Torna-se importante avaliar se anticorpos contra peptídeos cíclicos citrulinados podem identificar os pacientes com artrite idiopática juvenil com potencial de evolução para artrite reumatóide do adulto.

Assuntos

RESUMO

OBJETIVO: avaliar o estado nutricional na artrite idiopática juvenil (AIJ), a influência da atividade inflamatória e o uso de glicocorticóide. MÉTODOS: foram estudados 116 pacientes com AIJ diagnosticados segundo os critérios da ILAR/1997. Subtipo e atividade da doenca foram determinados por reumatologistas pediátricos, e a dose cumulativa de glicocorticóide foi revisada nos prontuários dos pacientes. Foram determinados os percentis do índice de massa corporal (IMC) e da prega cutânea tricipital (PCT) e o escore Z da estatura, de acordo com a OMS. Considerou-se baixo peso e baixa adiposidade quando o IMC e a PCT apresentavam-se abaixo do percentil 5. Baixa estatura foi definida por escore Z de estatura para idade menor do que -2. O nível sérico de IGF-1 foi medido por meio de radioimunoensaio. RESULTADOS: as prevalências de baixo peso, baixa adiposidade e baixa estatura foram 16,4 por cento, 20,7 por cento e 10,4 por cento, respectivamente. Níveis séricos reduzidos de IGF-1 foram observados em 14 pacientes (12,1 por cento). Os fatores negativamente associados com o escore Z da estatura na análise de regressão multivariável foram: duracão da doenca (coeficiente parcial de correlacão, intervalo de confianca de 95 por cento: -0,370, -0,527 a -0,188: p< 0,001), velocidade de hemossedimentacão (VHS) (-0,357, -0,516 a -0,174; p< 0,001), subtipo da doenca sistêmico ou poliarticular (-0,290, -0,459 a -0,100; p= 0,003), enquanto não houve associacão significante com dose cumulativa de glicocorticóide (0,086, -0,111 a 0,277: p= 0,391). Nenhuma dessas variáveis foi significativamente associada com os percentis de IMC e PCT, porém, pacientes com os subtipos sistêmico e poliarticular tenderam a apresentar percentis de IMC mais baixos (p= 0,051). CONCLUSÕES: pacientes com AIJ apresentam freqüentemente comprometimento do estado nutricional. A duracão e subtipo da doenca e o VSG são fatores independentemente associados à baixa estatura. A dose cumulativa de glicocorticóide não esteve associada de maneira independente com a baixa estatura ou a outras variáveis nutricionais.

Assuntos