Resumo: O objetivo do presente estudo foi descrever a frequência de constipação intestinal (CI) em pacientes com fibrose cística (FC) e os sintomas associados a essa condição. Estudo descritivo, prospectivo, observacional e transversal incluiu pacientes com idade superior a 6 meses, de ambos os sexos, com diagnóstico de FC e acompanhados num Centro Especializado de Referência em Fibrose Cística (CERFC), no Ambulatório de Pediatria e no de Triagem Neonatal, num Hospital Universitário. Foram registradas características demográficas, clínicas e sintomas gastrointestinais para o diagnóstico de CI, de acordo com os critérios estabelecidos pela European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN). O instrumento para coleta de dados consistiu de um questionário desenvolvido pelos autores. Foram entrevistados 105 pacientes, idade: 11,7 ± 6,1 anos (média±DP). A prevalência de CI foi de 41%. Dor abdominal foi referida por 11 dos 43 pacientes constipados e por 4 dos 62 não constipados (p=000,8). Diminuição da frequência das evacuações (1 a 2 evacuações/semana) foi referida apenas por pacientes constipados (N=6), p=0,005. Consistência fecal endurecida (pedaços duros separados e banana com rachos profundos) foi mais frequente nos pacientes constipados (p<000,1), sendo o sintoma mais prevalente nesse grupo: relatado por 33/43 e seguido por dor/dificuldade para evacuar: 19/43 e dor/desconforto abdominal e dor abdominal: 11/43. As classes mais prevalentes no Alelo I foram a II (F508del; 80%) e a I (R1162X; 7,5%). No Alelo II foram a II (F508del; 21%), a I (G542X; 18,4%) e a II (A561E; 10,5%), ambas classificadas como variantes graves. O antecedente de síndrome de obstrução intestinal (SOID) foi registrado em 14% dos pacientes constipados e em 4,8% dos pacientes não constipados, p=0,1553 e o antecedente de Íleo Meconial em 16% dos constipados e 17% dos não constipados, p=0,8449. A insuficiência pancreática foi identificada em 62,8% dos constipados, sem diferença significativa com a frequência em não constipados, p=0,7478. Observou-se elevada frequência de CI entre pacientes com FC, evacuação de fezes ressecadas e duras foi a queixa mais comum entre os pacientes com essa condição. Constipação intestinal não se associou a genótipo, função pancreática e antecedentes de SOID ou Íleo meconial Ver menos

Abstract: The objective of this study was to describe constipation prevalence in Brazilian patients with Cystic Fibrosis (CF) and also to report the gastrointestinal symptoms informed by patients. A descriptive, prospective, observational and transversal study was conducted including patients over six-months old, both sexes, with diagnosis of CF and followed in a Reference Center with two outpatient clinics: pediatrics and neonatal screening, both set in a University Hospital. Demographic characteristics and gastrointestinal symptoms were recorded using a questionnaire developed by the authors and applied to patients and/or caregivers. Constipation diagnosis was defined in accordance to the criteria defined by the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN). One hundred and five patients were included, mainly female 55%, aged 11.7 ± 6.1 years. Forty-three patients were diagnosed as constipated 41% (N=43). Hardened feces (separate hard pieces and "banana" shaped with deep cracks) manifested itself in higher prevalence in constipated patients compared to non-constipated (p = < 000.1). Reduced frequency of bowel movements (1 to 2 bowel movements/week) was detected only in patients with constipation (N=6), p = 0.005. Abdominal pain was reported by 11/43 constipated patients and by 4/62 non-constipated (p = 000.8). Hard fecal consistency was the most common complaint among constipated patients, reported by 33/43 patients, followed by pain or difficulty during evacuation 19/43 and abdominal pain 11/43. The most prevalent classes in Allele II were the II (80%) F508del; and I (R1162X; 7.5%). The Allele II were the II (21%), F508del; I (G542X; 18.4%) and the II (A561E; 10.5%), both classified as severe diseases. Previous diagnosis of intestinal obstruction syndrome (DIOS) was found in 14% of constipated patients and in 4,8% of non-constipated, p = 0.1553, previous history of meconium ileus was found in16% of constipated patients and in 17% of non-constipated, p = 0.8449. Pancreatic insufficiency was present in 62.8%, there was no difference between constipated patients and non-constipated, p = 0.7478. A high prevalence of constipation was observed in pediatric patients with CF, hardened fecal consistency was the most common complaint in patients diagnosed as constipated, according to ESPGHAN criteria. Constipation was not related to genotype, pancreatic function and previous history of DIOS or meconium ileus Ver menos