Hirschsprung Disease and Intestinal Neuronal Dysplasia Type B: Is There a Difference in the Clinical Presentation of These Two Diseases?

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Autor(es): dc.contributorUniversidade Estadual Paulista (UNESP)-
Autor(es): dc.contributorOuro Verde Hosp-
Autor(es): dc.creatorGoncalves, Anderson Cesar-
Autor(es): dc.creatorOliveira, Bruna Camargo de-
Autor(es): dc.creatorCoelho, Mariana Patti Sanches-
Autor(es): dc.creatorCaproni, Eloa Scalfi-
Autor(es): dc.creatorComes, Giovana Tuccille-
Autor(es): dc.creatorRodrigues, Maria Aparecida Marchesan-
Autor(es): dc.creatorOrtolan, Erika Veruska Paiva-
Autor(es): dc.creatorLourencao, Pedro Luiz Toledo de Arruda-
Data de aceite: dc.date.accessioned2025-08-21T19:26:41Z-
Data de disponibilização: dc.date.available2025-08-21T19:26:41Z-
Data de envio: dc.date.issued2025-04-29-
Data de envio: dc.date.issued2023-12-15-
Fonte completa do material: dc.identifierhttp://dx.doi.org/10.7759/cureus.50618-
Fonte completa do material: dc.identifierhttps://hdl.handle.net/11449/299422-
Fonte: dc.identifier.urihttp://educapes.capes.gov.br/handle/11449/299422-
Descrição: dc.descriptionBackground: Although the signs and symptoms that comprise the clinical presentation of Hirschsprung disease (HD) and intestinal neuronal dysplasia type B (IND-B) are well established, no studies have specifically compared the clinical characteristics presented by patients with these diseases. We compared the clinical pictures of patients with HD and IND-B at the time of histopathological diagnosis. Methods: This was a single-center, retrospective, analytical, and comparative study. We included 119 patients aged 0-15 years diagnosed with HD or IND-B. Information from the medical records of these patients was retrieved to obtain demographic and clinical information at the time of diagnosis. The data were compared statistically according to the characteristics of the variables. Results: Sixty-nine patients (58.0%) were diagnosed with HD, and 50 (42.0%) had IND-B. The HD group had significantly more individuals with symptom onset in the neonatal period (p = 0.001), delayed meconium clearance (p < 0.001), failure to thrive (p = 0.02), and acute complications, such as enterocolitis (p = 0.049) or acute abdominal obstruction (p = 0.031), more commonly requiring emergency surgery (p < 0.001). Patients with IND-B were diagnosed at a significantly older age (p = 0.002). They more commonly had chronic constipation as their main symptom (p = 0.004), with local complications, such as evacuation bleeding (p = 0.007). Conclusion: There were significant differences between the clinical pictures of patients with HD and IND-B. Knowledge of each disease's most common signs and symptoms can help direct diagnostic susception and initial management.-
Descrição: dc.descriptionFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)-
Descrição: dc.descriptionSwedish Research Council-
Descrição: dc.descriptionSao Paulo State Univ UNESP, Botucatu Med Sch, Dept Surg, Div Pediat Surg, Botucatu, Brazil-
Descrição: dc.descriptionOuro Verde Hosp, Surg, Campinas, Brazil-
Descrição: dc.descriptionSao Paulo State Univ UNESP, Botucatu Med Sch, Pathol Dept, Botucatu, Brazil-
Descrição: dc.descriptionSao Paulo State Univ UNESP, Botucatu Med Sch, Dept Surg, Div Pediat Surg, Botucatu, Brazil-
Descrição: dc.descriptionSao Paulo State Univ UNESP, Botucatu Med Sch, Pathol Dept, Botucatu, Brazil-
Descrição: dc.descriptionFAPESP: 2019/03441-4-
Descrição: dc.descriptionSwedish Research Council: 2019-03441-
Formato: dc.format8-
Idioma: dc.languageen-
Publicador: dc.publisherSpringer-
Relação: dc.relationCureus Journal Of Medical Science-
???dc.source???: dc.sourceWeb of Science-
Palavras-chave: dc.subjectenteric nervous system disorders-
Palavras-chave: dc.subjectchildren-
Palavras-chave: dc.subjectconstipation-
Palavras-chave: dc.subjectintestinal neuronal dysplasia type b-
Palavras-chave: dc.subjecthirschsprung disease-
Título: dc.titleHirschsprung Disease and Intestinal Neuronal Dysplasia Type B: Is There a Difference in the Clinical Presentation of These Two Diseases?-
Tipo de arquivo: dc.typelivro digital-
Aparece nas coleções:Repositório Institucional - Unesp

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