Revista Paulista de Pediatria (English Edition)最新文献

{"title":"Analysis of factors associated with excess weight in school children","authors":"Renata Paulino Pinto ,&nbsp;Altacílio Aparecido Nunes ,&nbsp;Luane Marques de Mello","doi":"10.1016/j.rppede.2016.04.005","DOIUrl":"10.1016/j.rppede.2016.04.005","url":null,"abstract":"<div><h3>Objective</h3><p>To determine the prevalence of overweight and obesity in schoolchildren aged 10 to 16 years and its association with dietary and behavioral factors.</p></div><div><h3>Methods</h3><p>Cross-sectional study that evaluated 505 adolescents using a structured questionnaire and anthropometric data. The data was analyzed through the T Test for independent samples and Mann–Whitney Test to compare means and medians, respectively, and Chi² Test for proportions. Prevalence ratio (RP) and the 95% confidence interval was used to estimate the degree of association between variables. The logistic regression was employed to adjust the estimates to confounding factors. The significance level of 5% was considered for all analysis.</p></div><div><h3>Results</h3><p>Excess weight was observed in 30.9% of the schoolchildren: 18.2% of overweight and 12.7% of obesity. There was no association between weight alterations and dietary/behavioral habits in the bivariate and multivariate analyses. However, associations were observed in relation to gender. Daily consumption of sweets [PR=0.75 (0.64–0.88)] and soft drinks [PR=0.82 (0.70–0.97)] was less frequent among boys; having lunch daily was slightly more often reported by boys [OR=1.11 (1.02–1.22)]. Physical activity practice of (≥3 times/week) was more often mentioned by boys and the association measures disclosed two-fold more physical activity in this group [PR=2.04 (1.56–2.67)] when compared to girls. Approximately 30% of boys and 40% of girls stated they did not perform activities requiring energy expenditure during free periods, with boys being 32% less idle than girls [PR=0.68 (0.60–0.76)].</p></div><div><h3>Conclusions</h3><p>A high prevalence of both overweight and obesity was observed, as well as unhealthy habits in the study population, regardless of the presence of weight alterations. Health promotion strategies in schools should be encouraged, in order to promote healthy habits and behaviors among all students.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 4","pages":"Pages 460-468"},"PeriodicalIF":0.0,"publicationDate":"2016-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2016.04.005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55275124","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Behavior change stages related to physical activity in adolescents from Santa Catarina: prevalence and associated factors","authors":"Jaqueline Aragoni da Silva,&nbsp;Kelly Samara da Silva,&nbsp;Adair da Silva Lopes,&nbsp;Markus Vinícius Nahas","doi":"10.1016/j.rppede.2016.03.017","DOIUrl":"10.1016/j.rppede.2016.03.017","url":null,"abstract":"<div><h3>Objective</h3><p>Verify the prevalence and sociodemographic and economic factors associated with behavior change stages for habitual physical activity practice in adolescents from Santa Catarina.</p></div><div><h3>Methods</h3><p>Secondary analysis of a study on the Behavior of Adolescents from Santa Catarina (CompAC 2). Cross-sectional school-based study of 6,529 high-school students (males, n=2,903) from the state of Santa Catarina public education system in 2011, aged 15 to 19 years. Multinomial logistic regression (crude and adjusted) was used to measure the association.</p></div><div><h3>Results</h3><p>The highest and lowest prevalence rates were found in the maintenance (43.9%) and precontemplation stages (7.0%), respectively. The stages of action, preparation and contemplation showed similar results: 16.2%; 17.0% and 15.6%; respectively. Male adolescents show higher prevalence in the maintenance stage in relation to females and these show a higher prevalence in preparation, contemplation and precontemplation. All the assessed variables (gender, age, area of residence, employment status, family income, maternal education and school grade), with the exception of school shift, were associated with at least one of the stages.</p></div><div><h3>Conclusions</h3><p>A large proportion of adolescents are in the pre-adoption stages and most of these have the intention to start regular physical activity. With the exception of school shift, the assessed variables were associated with stages in different ways, showing different profiles in relation to sociodemographic and economic characteristics in each subgroup.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 4","pages":"Pages 476-483"},"PeriodicalIF":0.0,"publicationDate":"2016-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2016.03.017","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55274841","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Consumption of fruits and vegetables associated with other risk behaviors among adolescents in Northeast Brazil","authors":"Fabiana Medeiros de Almeida Silva ,&nbsp;Aldemir Smith-Menezes ,&nbsp;Maria de Fátima da Silva Duarte","doi":"10.1016/j.rppede.2015.09.004","DOIUrl":"10.1016/j.rppede.2015.09.004","url":null,"abstract":"<div><h3>Objective</h3><p>To determine the prevalence of consumption of fruits and vegetables and identify the association with low level of physical activity, exposure to sedentary behavior, consumption of soft drinks and overweight/obesity in adolescents.</p></div><div><h3>Methods</h3><p>This is a cross-sectional school-based study with a representative sample of 3992 students aged 14–19 years from the state of Sergipe, Brazil. The outcome was low consumption of fruits and vegetables (&lt;5servings/day). Independent variables were: level of physical activity, sedentary behavior, consumption of soft drinks, and overweight/obesity. Global Student Health Survey questionnaire and body mass and height measurements were used, as well as chi-square test and crude and adjusted binary logistic regression. The significance level adopted was 5%.</p></div><div><h3>Results</h3><p>The prevalence of inadequate consumption of fruits and vegetables was high – 88.6% (95%CI=87.6–89.5). Higher likelihood of low consumption of fruits and vegetables was verified among boys who were exposed to sedentary behavior (OR=1.63; 95%CI=1.18–2.24), who consumed soft drinks (OR=3.04; 95%CI=2.10–4.40), with insufficiently physical activity (OR=1.98; 95%CI=1.43–2.73) and girls who consumed soft drinks (OR=1.88; 95%CI=1.43–2.47) and those with overweight/obesity (OR=1.63; 95%CI=1.19–2.23).</p></div><div><h3>Conclusions</h3><p>There is a need of public policies aimed at encouraging the consumption of healthy foods among adolescents.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 309-315"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2015.09.004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55270704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Influence of glycemic index and glycemic load of the diet on the risk of overweight and adiposity in childhood","authors":"Kellen Cristine Silva ,&nbsp;Luciana Neri Nobre ,&nbsp;Sofia Emanuelle de Castro Ferreira Vicente ,&nbsp;Lidiane Lopes Moreira ,&nbsp;Angelina do Carmo Lessa ,&nbsp;Joel Alves Lamounier","doi":"10.1016/j.rppede.2015.12.009","DOIUrl":"10.1016/j.rppede.2015.12.009","url":null,"abstract":"<div><h3>Objective</h3><p>To investigate the association between the glycemic index and the glycemic load of the diet with the risk of overweight and high adiposity in children with 5 years of age.</p></div><div><h3>Methods</h3><p>Cross-sectional study nested in a cohort of 232 children born and living in Diamantina (MG, Brazil). Parents and/or guardians provided the food intake data, using a semiquantitative food frequency questionnaire, past history and socioeconomic conditions. Anthropometric and fatness data were collected from the children. The dietary glycemic index and the glycemic load were calculated from the food intake. The glycemic index and glycemic load effect on overweight and adiposity in children was assessed by the Poisson regression (<em>p</em>&lt;0.05).</p></div><div><h3>Results</h3><p>The prevalence of overweight by body mass index was 17.3%, and high adiposity was observed in 3.4% and 6.9% by triceps skinfold and subscapular skinfold, respectively. No difference was reported between the mean body mass index, triceps skinfold and subscapular skinfold according to the glycemic index and glycemic load tertiles; however, the overweight group presented a higher carbohydrate intake (<em>p</em>=0.04). No association was found between glycemic index and glycemic load with overweight and adiposity among the children assessed.</p></div><div><h3>Conclusions</h3><p>The glycemic index and glycemic load of the diet were not identified as risk factors for overweight and adiposity in this cross-sectional study.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 293-300"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2015.12.009","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55271868","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Secular trends in infant mortality by age-group and avoidable components in the State of São Paulo, 1996–2012","authors":"Kelsy Catherina Nema Areco,&nbsp;Tulio Konstantyner,&nbsp;José Augusto de Aguiar Carrazedo Taddei","doi":"10.1016/j.rppede.2016.03.009","DOIUrl":"10.1016/j.rppede.2016.03.009","url":null,"abstract":"<div><h3>Objective</h3><p>To describe trends and composition of infant mortality rate in the State of São Paulo, from 1996 to 2012.</p></div><div><h3>Methods</h3><p>An ecological study was conducted, based on official secondary data of births and infant deaths of residents in São Paulo, from 1996 to 2012. The infant mortality rate was calculated by the direct method and was analyzed by graphs and polynomial regression models for age groups (early neonatal, late neonatal and post-neonatal) and for groups of avoidable causes of death.</p></div><div><h3>Results</h3><p>The mortality rate in the State of São Paulo tended to fall, ranging from 22.5 to 11.5 per thousand live births. Half of the infant deaths occurred in the early neonatal group. The proportion of avoidable infant deaths varied from 76.0 to 68.7%. The deaths which were avoidable by adequate attention to women during pregnancy and childbirth and newborn care accounted for 54% of infant deaths throughout the period.</p></div><div><h3>Conclusions</h3><p>The mortality rate levels are still far from those in developed countries, which highlight the need to prioritize access and quality of healthcare services during pregnancy, childbirth and newborn care, especially in the first week of life, aiming at achieving standards of infant mortality similar to those of developed societies.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 263-270"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2016.03.009","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55274447","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital central hypoventilation syndrome associated with Hirschsprung's Disease: case report and literature review","authors":"Renata Lazari Sandoval ,&nbsp;Carlos Moreno Zaconeta ,&nbsp;Paulo Roberto Margotto ,&nbsp;Maria Teresinha de Oliveira Cardoso ,&nbsp;Evely Mirella Santos França ,&nbsp;Cristina Touguinha Neves Medina ,&nbsp;Talyta Matos Canó ,&nbsp;Aline Saliba de Faria","doi":"10.1016/j.rppede.2015.10.009","DOIUrl":"10.1016/j.rppede.2015.10.009","url":null,"abstract":"<div><h3>Objective</h3><p>To report the case of a newborn with recurrent episodes of apnea, diagnosed with Congenital Central hypoventilation syndrome (CCHS) associated with Hirschsprung's disease (HD), configuring Haddad syndrome.</p></div><div><h3>Case description</h3><p>Third child born at full-term to a non-consanguineous couple through normal delivery without complications, with appropriate weight and length for gestational age. Soon after birth he started to show bradypnea, bradycardia and cyanosis, being submitted to tracheal intubation and started empiric antibiotic therapy for suspected early neonatal sepsis. During hospitalization in the NICU, he showed difficulty to undergo extubation due to episodes of desaturation during sleep and wakefulness. He had recurrent episodes of hypoglycemia, hyperglycemia, metabolic acidosis, abdominal distension, leukocytosis, increase in C-reactive protein levels, with negative blood cultures and suspected inborn error of metabolism. At 2 months of age he was diagnosed with long-segment Hirschsprung's disease and was submitted to segment resection and colostomy through Hartmann's procedure. A genetic research was performed by polymerase chain reaction for CCHS screening, which showed the mutated allele of PHOX2B gene, confirming the diagnosis.</p></div><div><h3>Comments</h3><p>This is a rare genetic, autosomal dominant disease, caused by mutation in PHOX2B gene, located in chromosome band 4p12, which results in autonomic nervous system dysfunction. CCHS can also occur with Hirschsprung's disease and tumors derived from the neural crest. There is a correlation between phenotype and genotype, as well as high intrafamilial phenotypic variability. In the neonatal period it can simulate cases of sepsis and inborn errors of metabolism.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 374-378"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2015.10.009","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55271479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dental pain and associated factors in Brazilian preschoolers","authors":"João Gabriel Silva Souza ,&nbsp;Andrea Maria Eleutério de Barros Lima Martins","doi":"10.1016/j.rppede.2016.03.002","DOIUrl":"10.1016/j.rppede.2016.03.002","url":null,"abstract":"<div><h3>Objective</h3><p>To describe the prevalence of dental pain in Brazilian preschoolers, as well as its associated factors, considering a representative sample of that population group in Brazil.</p></div><div><h3>Methods</h3><p>Cross-sectional study that used the analytical data from national oral health survey (SB Brazil) carried out in 2010. A representative sample of Brazilian preschoolers aged 5 years was considered. Interviews were carried out (answered by parents/tutors), as well as clinical examinations in children. Descriptive, bivariate and mutiple analyzes were performed, described in odds ratios and 95% confidence interval (OR/95%CI).</p></div><div><h3>Results</h3><p>7280 preschoolers were included. Of these, 1520 (21.1%) had dental pain in the last 6 months. The chance of the occurrence of dental pain was higher among those who used dental services (1.51/1.02–2.23), with tooth decay (3.08/2.08–4.56), that self-perceveid the need for dental treatment (3.96/2.48–6.34), whose parents reported dissatisfaction by children with their teeth and mouth (1.47/1.04–2.10) and those who reported impact of oral problems on quality of life (5.76/3.90–8.49).</p></div><div><h3>Conclusions</h3><p>The prevalence of dental pain among Brazilian preschool children was relatively high, being associated with the use of dental services and the normative and subjective oral health status.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 336-342"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2016.03.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55273944","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Insulin therapy in patients with cystic fibrosis in the pre-diabetes stage: a systematic review","authors":"Mariana Zorrón Mei Hsia Pu,&nbsp;Flávia Corrêa Christensen-Adad,&nbsp;Aline Cristina Gonçalves,&nbsp;Walter José Minicucci,&nbsp;José Dirceu Ribeiro,&nbsp;Antonio Fernando Ribeiro","doi":"10.1016/j.rppede.2016.03.006","DOIUrl":"10.1016/j.rppede.2016.03.006","url":null,"abstract":"<div><h3>Objective</h3><p>To elucidate whether insulin is effective or not in patients with cystic fibrosis before the diabetes mellitus phase.</p></div><div><h3>Data source</h3><p>The study was performed according to the Prisma method between August and September 2014, using the PubMed, Embase, Lilacs and SciELO databases. Prospective studies published in English, Portuguese and Spanish from 2002 to 2014, evaluating the effect of insulin on weight parameters, body mass index and pulmonary function in patients with cystic fibrosis, with a mean age of 17.37 years before the diabetes mellitus phase were included.</p></div><div><h3>Data synthesis</h3><p>Eight articles were identified that included 180 patients undergoing insulin use. Sample size ranged from 4 to 54 patients, with a mean age ranging from 12.4 to 28 years. The type of follow-up, time of insulin use, the dose and implementation schedule were very heterogeneous between studies.</p></div><div><h3>Conclusions</h3><p>There are theoretical reasons to believe that insulin has a beneficial effect in the studied population. The different methods and populations assessed in the studies do not allow us to state whether early insulin therapy should or should not be carried out in patients with cystic fibrosis prior to the diagnosis of diabetes. Therefore, studies with larger samples and insulin use standardization are required.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 367-373"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2016.03.006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55274248","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Substance misuse and sexual function in adolescents with chronic diseases","authors":"Priscila Araújo ,&nbsp;Márcio Guilherme Nunes Carvalho ,&nbsp;Marlon van Weelden ,&nbsp;Benito Lourenço ,&nbsp;Lígia Bruni Queiroz ,&nbsp;Clovis Artur Silva","doi":"10.1016/j.rppede.2015.10.008","DOIUrl":"10.1016/j.rppede.2015.10.008","url":null,"abstract":"<div><h3>Objective</h3><p>To evaluate alcohol/tobacco and/or illicit drug misuse in Chronic Diseases (CDs).</p></div><div><h3>Methods</h3><p>A cross-sectional study with 220 CDs adolescents and 110 healthy controls including: demographic/anthropometric data; puberty markers; modified questionnaire evaluating sexual function, alcohol/smoking/illicit drug misuse and bullying; and the physician-conducted CRAFFT (car/relax/alone/forget/friends/trouble) screen tool for substance abuse/dependence high risk.</p></div><div><h3>Results</h3><p>The frequencies of alcohol/tobacco and/or illicit drug use were similar in both groups (30% vs. 34%, <em>p</em><span>=</span>0.529), likewise the frequencies of bullying (42% vs. 41%, <em>p</em><span>=</span>0.905). Further analysis solely in CDs patients that used alcohol/tobacco/illicit drug versus those that did not use showed that the median current age [15 (11–18) vs. 14 (10–18) years, <em>p</em><span>&lt;</span>0.0001] and education years [9 (5–14) vs. 8 (3–12) years, <em>p</em><span>&lt;</span>0.0001] were significant higher in substance use group. The frequencies of Tanner 5 (<em>p</em><span>&lt;</span>0.0001), menarche (<em>p</em><span>&lt;</span>0.0001) and spermarche (<em>p</em><span>=</span>0.001) were also significantly higher in patients with CDs that used alcohol/tobacco/illicit, likewise sexual activity (23% vs. 3%, <em>p</em><span>&lt;</span>0.0001). A trend of a low frequency of drug therapy was observed in patients that used substances (70% vs. 82%, <em>p</em><span>=</span>0.051). A positive correlation was observed between CRAFFT score and current age in CD patients (<em>p</em><span>=</span>0.005, r<span>=+</span>0.189) and controls (<em>p</em><span>=</span>0.018, r<span>=+</span>0.226).</p></div><div><h3>Conclusions</h3><p>A later age was evidenced in CDs patients that reported licit/ilicit drug misuse. In CDs adolescent, substance use was more likely to have sexual intercourse. Our study reinforces that these patients should be systematically screened by pediatricians for drug related health behavioral patterns.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 323-329"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2015.10.008","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55271458","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hirschsprung's disease – Postsurgical intestinal dysmotility","authors":"Mariana Tresoldi das Neves Romaneli,&nbsp;Antonio Fernando Ribeiro,&nbsp;Joaquim Murray Bustorff-Silva,&nbsp;Rita Barbosa de Carvalho,&nbsp;Elizete Aparecida Lomazi","doi":"10.1016/j.rppede.2016.05.001","DOIUrl":"10.1016/j.rppede.2016.05.001","url":null,"abstract":"<div><h3>Objective</h3><p>To describe the case of an infant with Hirschsprung's disease presenting as total colonic aganglionosis, which, after surgical resection of the aganglionic segment persisted with irreversible functional intestinal obstruction; discuss the difficulties in managing this form of congenital aganglionosis and discuss a plausible pathogenetic mechanism for this case.</p></div><div><h3>Case description</h3><p>The diagnosis of Hirschsprung's disease presenting as total colonic aganglionosis was established in a two-month-old infant, after an episode of enterocolitis, hypovolemic shock and severe malnutrition. After colonic resection, the patient did not recover intestinal motor function that would allow enteral feeding. Postoperative examination of remnant ileum showed the presence of ganglionic plexus and a reduced number of interstitial cells of Cajal in the proximal bowel segments. At 12 months, the patient remains dependent on total parenteral nutrition.</p></div><div><h3>Comments</h3><p>Hirschsprung's disease presenting as total colonic aganglionosis has clinical and surgical characteristics that differentiate it from the classic forms, complicating the diagnosis and the clinical and surgical management. The postoperative course may be associated with permanent morbidity due to intestinal dysmotility. The numerical reduction or alteration of neural connections in the interstitial cells of Cajal may represent a possible physiopathological basis for the condition.</p></div>","PeriodicalId":101120,"journal":{"name":"Revista Paulista de Pediatria (English Edition)","volume":"34 3","pages":"Pages 388-392"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.rppede.2016.05.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"55275242","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}