Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis,
Autor(a) principal: | |
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Data de Publicação: | 2020 |
Outros Autores: | , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Jornal de Pediatria (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572020000600732 |
Resumo: | Abstract Objective: To evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. Methods: Case series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. Results: All patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. Conclusion In this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time. |
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Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis,Cystic fibrosisIntestinal obstructionChildDiagnosisTherapeuticsAbstract Objective: To evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. Methods: Case series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. Results: All patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. Conclusion In this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time.Sociedade Brasileira de Pediatria2020-12-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572020000600732Jornal de Pediatria v.96 n.6 2020reponame:Jornal de Pediatria (Online)instname:Sociedade Brasileira de Pediatria (SBP)instacron:SBPE10.1016/j.jped.2019.08.009info:eu-repo/semantics/openAccessSandy,Natascha S.Massabki,Lilian H.P.Gonçalves,Aline C.Ribeiro,Antonio F.Ribeiro,Jose D.Servidoni,Maria de Fátima C.P.Lomazi,Elizete A.eng2020-12-10T00:00:00Zoai:scielo:S0021-75572020000600732Revistahttp://www.jped.com.br/https://old.scielo.br/oai/scielo-oai.php||jped@jped.com.br1678-47820021-7557opendoar:2020-12-10T00:00Jornal de Pediatria (Online) - Sociedade Brasileira de Pediatria (SBP)false |
dc.title.none.fl_str_mv |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, |
title |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, |
spellingShingle |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, Sandy,Natascha S. Cystic fibrosis Intestinal obstruction Child Diagnosis Therapeutics |
title_short |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, |
title_full |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, |
title_fullStr |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, |
title_full_unstemmed |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, |
title_sort |
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis, |
author |
Sandy,Natascha S. |
author_facet |
Sandy,Natascha S. Massabki,Lilian H.P. Gonçalves,Aline C. Ribeiro,Antonio F. Ribeiro,Jose D. Servidoni,Maria de Fátima C.P. Lomazi,Elizete A. |
author_role |
author |
author2 |
Massabki,Lilian H.P. Gonçalves,Aline C. Ribeiro,Antonio F. Ribeiro,Jose D. Servidoni,Maria de Fátima C.P. Lomazi,Elizete A. |
author2_role |
author author author author author author |
dc.contributor.author.fl_str_mv |
Sandy,Natascha S. Massabki,Lilian H.P. Gonçalves,Aline C. Ribeiro,Antonio F. Ribeiro,Jose D. Servidoni,Maria de Fátima C.P. Lomazi,Elizete A. |
dc.subject.por.fl_str_mv |
Cystic fibrosis Intestinal obstruction Child Diagnosis Therapeutics |
topic |
Cystic fibrosis Intestinal obstruction Child Diagnosis Therapeutics |
description |
Abstract Objective: To evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. Methods: Case series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. Results: All patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. Conclusion In this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time. |
publishDate |
2020 |
dc.date.none.fl_str_mv |
2020-12-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572020000600732 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572020000600732 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1016/j.jped.2019.08.009 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Pediatria |
publisher.none.fl_str_mv |
Sociedade Brasileira de Pediatria |
dc.source.none.fl_str_mv |
Jornal de Pediatria v.96 n.6 2020 reponame:Jornal de Pediatria (Online) instname:Sociedade Brasileira de Pediatria (SBP) instacron:SBPE |
instname_str |
Sociedade Brasileira de Pediatria (SBP) |
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SBPE |
institution |
SBPE |
reponame_str |
Jornal de Pediatria (Online) |
collection |
Jornal de Pediatria (Online) |
repository.name.fl_str_mv |
Jornal de Pediatria (Online) - Sociedade Brasileira de Pediatria (SBP) |
repository.mail.fl_str_mv |
||jped@jped.com.br |
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1752122322384322560 |