Diagnosis and Treatment of Chronic Intestinal Pseudo-Obstruction in Children: Report of Consensus Workshop

Rudolph, Cd.; Hyman, P E; Sm, Altschuler; Christensen, James; Rb, Colletti; Cucchiara, Salvatore; Lorenzo, Cherubino Di; Flores, AF; Ac, Hillemeier; Rw, McCallum; Ja, Vanderhoof

doi:10.1097/00005176-199701000-00021

Cited by 169 publications

(132 citation statements)

References 140 publications

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“…No levantamento dos dados, das crianças atendidas com quadro de POIC no Hospital das Clínicas da Universidade Federal de Minas Gerais, foi observado que as manifestações clínicas são semelhantes às relatadas pela literatura 6,12,13 . As alterações extradigestivas encontradas nos pacientes foram também descritas por outros autores 6,12-16 .…”

Section: Discussionunclassified

“…As alterações radiológicas foram semelhantes às referidas por Rudolph & Granata 6,12,14 . Alguns achados na radiografia contrastada são tidos como padrão de POIC como dilatação sem estenose, atividade peristáltica anormal, retardo no esvaziamento gástrico e diminuição das haustrações do cólon 12 .…”

Section: Discussionunclassified

“…O termo "idiopático" é aplicado ao grupo em que a etiologia secundária não foi determinada ou quando o tecido afetado é indisponível para análise ou parece normal ao estudo histopatológico por técnicas convencionais de coloração 6 .…”

Section: Chronic Intestinal Pseudo-obstruction In Children -Report Ofunclassified

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Chronic intestinal pseudo-obstruction in children - report of seven cases

Almeida

Penna²

2000

J Pediatr (Rio J)

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ResumoA pseudo-obstrução intestinal crônica (POIC) é uma rara e grave enfermidade clínica, caracterizada por disfunção intestinal, na ausência de obstrução mecânica. A fisiopatologia da POIC permanece pouco conhecida. O diagnóstico é difícil e o tratamento, na maioria das vezes, paliativo.Objetivo: A proposta deste estudo é descrever as manifestações clínicas e alterações histológicas na biopsia intestinal de 7 crianças atendidas no serviço de gastroenterologia (GI).Métodos: Estudo retrospectivo de todos os pacientes atendidos no serviço de GI no período de 10 anos. Os prontuários de sete pacientes foram revisados e todos os dados clínicos relevantes registrados. O diagnóstico foi baseado nos achados clínicos, laboratoriais e histológicos.Conclusão: Embora seja uma síndrome rara, o diagnóstico diferencial deverá ser realizado nos pacientes que apresentem sinais e sintomas de obstrução intestinal do trato digestivo, sem causa mecânica associada. Estudos devem ser incentivados com relação ao diagnóstico precoce, utilizando técnicas menos invasivas, assim como, uma terapêutica específica (clínica e cirúrgica), a fim de melhorar a qualidade de vida e obter maior sobrevida para os pacientes afetados. J. pediatr. (Rio J.). 2000; 76(6):453-457:pseudo-obstrução intestinal crônica, infância, diagnóstico, tratamento. AbstractChronic intestinal pseudo-obstruction (CIP) is a rare and severe clinical entity characterized by impaired intestinal function in the absence of mechanical occlusion demonstrable by X-ray or surgery. The pathophysiology of CIP remains poorly known. The diagnostic approach in CIP is difficult and in most of the times the treatment is merely palliative.Objective: The purpose of this report is to describe the clinical characteristics and histological abnormalities on full-thickness intestinal biopsy in seven children from the Gastroenterology (GI) outpatient clinic.Methods: A retrospective study of all patients who attended the GI outpatient clinic in the last ten years. The medical records of seven patients were reviewed, and all the relevant clinical data recorded. Diagnosis was based on clinical, laboratorial, and histological findings.Conclusion: Although this is a rare syndrome, the differential diagnosis shall be performed whenever the patient presents signs and symptoms of obstruction along the digestive tract with no association of any mechanical cause. Studies shall be encouraged regarding the precocious and accurate diagnosis, using less invasive techniques and specific therapeutics (both clinical and surgical), in order to improve life quality and extended survival of affected patients. Descrita pela primeira vez em 1958 por Dudley 1 , a pseudo-obstrução intestinal crônica (POIC) é uma entidade clínica rara e heterogênea. Caracteriza-se por sinais e sintomas intermitentes de obstrução intestinal sem causa mecânica, decorrente de alteração neuromuscular do tubo digestivo, reversível ou irreversível, na qual o segmento acometido pode ser único ou múltiplo. A apresentação clínica é variável e dep...

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Section: Discussionunclassified

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Chronic intestinal pseudo-obstruction in children - report of seven cases

Almeida

Penna²

2000

J Pediatr (Rio J)

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“…CIPO is characterized by more than six-months of severe symptoms of intestinal obstruction such as abdominal distention and pain, nausea and vomiting with clinical/radiographic evidence of dilated bowel in the absence of mechanical obstruction. 29 CIPO may be caused by neurologic or myopathic defects or secondary to medications. 30,31 A recent study has shown a potential higher burden of strokes following acute worsening of CIPO in MELAS patients.…”

Section: Gastroenterologymentioning

confidence: 99%

Patient care standards for primary mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society

Parikh

Goldstein

Karaa

et al. 2017

Genetics in Medicine

211

208

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Purpose The purpose of this statement is to provide consensus-based recommendations for optimal management and care for patients with primary mitochondrial disease. This statement is intended for physicians who are engaged in the diagnosis and management of these patients.Methods Working Group members were appointed by the Mitochondrial Medicine Society. The panel included members with several different areas of expertise. The panel members utilized surveys and the Delphi method to reach consensus. We anticipate that this statement will need to be updated as the field continues to evolve.

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“…Evaluation for gastrointestinal complications should be pursued in the presence of unexplained anemia or weight loss, abdominal bloating, sudden or persistent abdominal pain, chronic diarrhea, signs of malabsorption, gastrointestinal bleeding, or recurrent emesis. 29,30 Seizures occur in 6% to 7% of cases. 12 Although electroencephalographic abnormalities are commonly reported, electroencephalography is not routinely recommended.…”

Section: Complicationsmentioning

confidence: 99%

Health Supervision for Children With Neurofibromatosis

Hersh¹

2008

Pediatrics

161

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Neurofibromatosis 1 is a multisystem disorder that primarily involves the skin and nervous system. Its population prevalence is 1 in 3500. The condition usually is recognized in early childhood, when cutaneous manifestations are apparent. Although neurofibromatosis 1 is associated with marked clinical variability, most affected children do well from the standpoint of their growth and development. Some features of neurofibromatosis 1 are present at birth, and others are age-related abnormalities of tissue proliferation, which necessitate periodic monitoring to address ongoing health and developmental needs and to minimize the risk of serious medical complications. This clinical report provides a review of the clinical criteria needed to establish a diagnosis, the inheritance pattern of neurofibromatosis 1, its major clinical and developmental manifestations, and guidelines for monitoring and providing intervention to maximize the growth, development, and health of an affected child.

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Diagnosis and Treatment of Chronic Intestinal Pseudo-Obstruction in Children: Report of Consensus Workshop

Cited by 169 publications

References 140 publications

Chronic intestinal pseudo-obstruction in children - report of seven cases

Chronic intestinal pseudo-obstruction in children - report of seven cases

Patient care standards for primary mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society

Health Supervision for Children With Neurofibromatosis

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