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Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis.
World J Gastroenterol 2015; 21(1):318-25WJ

Abstract

AIM

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.

CONCLUSION

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+Show Affiliations

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.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.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.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.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.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.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.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.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.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.

Pub Type(s)

Journal Article
Multicenter Study
Observational Study

Language

eng

PubMed ID

25574107

Citation

Lavie, Moran, et al. "Long-term Follow-up of Distal Intestinal Obstruction Syndrome in Cystic Fibrosis." World Journal of Gastroenterology, vol. 21, no. 1, 2015, pp. 318-25.
Lavie M, Manovitz T, Vilozni D, et al. Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis. World J Gastroenterol. 2015;21(1):318-25.
Lavie, M., Manovitz, T., Vilozni, D., Levy-Mendelovich, S., Sarouk, I., Weintraubv, I., ... Efrati, O. (2015). Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis. World Journal of Gastroenterology, 21(1), pp. 318-25. doi:10.3748/wjg.v21.i1.318.
Lavie M, et al. Long-term Follow-up of Distal Intestinal Obstruction Syndrome in Cystic Fibrosis. World J Gastroenterol. 2015 Jan 7;21(1):318-25. PubMed PMID: 25574107.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis. AU - Lavie,Moran, AU - Manovitz,Tzipora, AU - Vilozni,Daphna, AU - Levy-Mendelovich,Sarina, AU - Sarouk,Ifat, AU - Weintraubv,Ilana, AU - Shoseyov,David, AU - Cohen-Cymberknoh,Malena, AU - Rivlin,Joseph, AU - Efrati,Ori, PY - 2014/06/16/received PY - 2014/08/27/revised PY - 2014/09/29/accepted PY - 2015/1/10/entrez PY - 2015/1/13/pubmed PY - 2015/9/15/medline KW - Cystic fibrosis KW - Distal intestinal obstruction syndrome KW - Meconium ileus KW - Recurrence KW - Treatment SP - 318 EP - 25 JF - World journal of gastroenterology JO - World J. Gastroenterol. VL - 21 IS - 1 N2 - AIM: 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. CONCLUSION: 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. SN - 2219-2840 UR - https://www.unboundmedicine.com/medline/citation/25574107/Long_term_follow_up_of_distal_intestinal_obstruction_syndrome_in_cystic_fibrosis_ L2 - http://www.wjgnet.com/1007-9327/full/v21/i1/318.htm DB - PRIME DP - Unbound Medicine ER -