title: Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis.
abstract: OBJECTIVE
  To investigate the long-term follow-up of distal intestinal obstruction syndrome (DIOS) in Israeli cystic fibrosis (CF) patients.
  
  
  METHODS
  This is a multi-center, comparative, retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012. Patients diagnosed with DIOS were defined as the study group. The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan. For the control group, CF patients with no DIOS were matched to the patients in the study group for age, sex, and cystic fibrosis transmembrane conductance regulator (CFTR) mutations. For both groups, the collected data included age, sex, CFTR genotype, weight, height, and body mass index. Clinical data included respiratory function tests in the last five years prior to the study, respiratory function test immediately before and after the DIOS event, number of hospitalizations, sputum culture results, and CF-related conditions diagnosed according to the CF clinical practice guidelines. In the study group, data on the DIOS treatment and tendency for DIOS recurrence were also analyzed.
  
  
  RESULTS
  The medical charts for a total of 350 CF patients were reviewed. Of the 350 CF patients, 26 (7.4%) were diagnosed with DIOS. The control group included 31 CF patients with no DIOS diagnosis. The mean follow-up period was 21.6 ± 8.2 years. The total of DIOS episodes in the follow-up period was 60. The distribution of DIOS episodes was as follows: 6/26 (23.1%) study patients had one episode of DIOS in their lifetime, 7/26 (26.9%) had two episodes, 7/26 (26.9%) had three episodes, and 6/26 (23.1%) had four or more episodes. Compared to the control group, DIOS patients had a significantly higher incidence of meconium ileus in the past (65.4% vs 0%, respectively, P < 0.02), more Aspergillus spp. colonization (34.6% vs 3.2%, respectively, P < 0.02), and a higher number of hospitalizations due to respiratory exacerbations (8.6 vs 6.2 mean total hospitalizations per follow-up period, respectively, P < 0.02). No other significant differences were found between the control and study groups. The conservative treatment of DIOS, which mainly includes hydration and stool softeners, was successful in 82% of the episodes. The survival rate was similar for both groups.
  
  
  CONCLUSIONS
  CF patients with DIOS suffer from recurrent hospitalizations and airway pathogen acquisition. Although recurrence of DIOS is common, conservative treatment is successful in most patients.
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Moran
authors.initials: M
authors.lastname: Lavie
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Tzipora
authors.initials: T
authors.lastname: Manovitz
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Daphna
authors.initials: D
authors.lastname: Vilozni
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Sarina
authors.initials: S
authors.lastname: Levy-Mendelovich
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Ifat
authors.initials: I
authors.lastname: Sarouk
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Ilana
authors.initials: I
authors.lastname: Weintraubv
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: David
authors.initials: D
authors.lastname: Shoseyov
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Malena
authors.initials: M
authors.lastname: Cohen-Cymberknoh
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Joseph
authors.initials: J
authors.lastname: Rivlin
authors.affiliation: Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel.
authors.forename: Ori
authors.initials: O
authors.lastname: Efrati
mesh_terms: D000293:Adolescent; D000328:Adult; D001228:Aspergillosis / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D018450:Disease Progression; D005260:Female; D006760:Hospitalization; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D007557:Israel / Q000453:epidemiology; D008172:Lung Diseases, Fungal / Q000382:microbiology; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Cystic fibrosis; Distal intestinal obstruction syndrome; Meconium ileus; Recurrence; Treatment