Critical Factors Affecting Quality of Life of Adult Patients With Anorectal Malformations or Hirschsprung's Disease

Hartman, Esther; Oort, Frans J.; Aronson, Daniël C.; Hanneman, Marianne J. G.; Zee, David C. van der; Rieu, P.N.M.A.; Madern, Gerard C.; Langen, Zacharias J. de; Heurn, L. W. Ernest van; Silfhout-Bezemer, Marina van; Looyaard, Nic; Sprangers, Mirjam A. G.

doi:10.1111/j.1572-0241.2004.04149.x

Cited by 79 publications

(81 citation statements)

References 27 publications

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“…Another recently published study showed that patients with HD encountered more "overall" physical health problems compared with healthy subjects, but HD patients did not show additional pain or limitations in functioning due to physical problems [66]. Psychosocial functioning had the most important effect on the quality of life of patients with HD, whereas fecal incontinence and constipation had almost no effect on their quality of life.…”

Section: Prognosis After Surgerymentioning

confidence: 92%

Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood

Lorijn

Boeckxstaens²,

Benninga

2007

Curr Gastroenterol Rep

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In the majority of infants and children with constipation, no obvious cause can be identified. A rare cause of constipation is Hirschsprung disease (HD). HD is characterized by the absence of ganglion cells from the anorectum for a variable length up to the duodenum. The extent of the aganglionic segment varies, but in most patients the lesion does not extend beyond the rectum and sigmoid colon. This review focuses on the passage of meconium, the recognition of HD, and new insights in its pathophysiology and genetics. The authors also provide a summary of the diagnostic evaluation and treatment of HD in infancy and childhood.

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Section: Prognosis After Surgerymentioning

confidence: 92%

Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood

Lorijn

Boeckxstaens²,

Benninga

2007

Curr Gastroenterol Rep

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“…The incidence is relatively high and can require extensive surgery to provide a tolerable quality of life. Even with surgical intervention the outlook for those with more serious defects is poor and for those who respond well to surgery there is often a long-term follow up required, in addition to psychological damage (Bai et al, 2000, Hartman et al, 2004. Our studies provide new insights into the molecular bases of ARMs, and suggest a robust model system for future analysis of the genetic and teratogenic contributors to ARMs.…”

Section: The Zebrafish As a Model For Armsmentioning

confidence: 99%

Hedgehog signalling is required for cloacal development in the zebrafish embryo

Parkin¹,

Allen²,

Ingham³

2009

Int. J. Dev. Biol.

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The Hedgehog (Hh) family of signalling molecules is essential for a wide range of developmental processes. Mammalian studies have implicated the Hedgehog pathway in the aetiology of anorectal malformations (ARMs), relatively common congenital anomalies caused by failures in the development of the cloaca. In this study we demonstrate that Hh signalling is absolutely required for the formation of the zebrafish cloaca and that the severity of the posterior gut abnormalities induced by a reduction in Hh activity is dependent on the levels of Hh signal transduction. The complete loss of all Hh activity results in the most severe defects and the critical period for Hh activity is between 34 and 74 hours post fertilisation. Using a range of mutant genotypes that cause notochord and floorplate abnormalities, we show that the source of the Hh signals required for posterior gut formation is the endoderm and not the notochord, as previously postulated in mammalian models of ARMs. We show that Adriamycin, a drug known to cause ARMs in rat, but not chick embryos, has no effect on the development of the zebrafish gastrointestinal tract. These studies establish the zebrafish as a model for ARMs, and for the elucidation of other pathways involved in hindgut developmental processes.

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“…[1][2][3][4][5][6] It has been shown in several studies that there is no statistically significant difference in the functional outcome with respect to bowel function between the various pull through surgeries. [7][8][9][10][11] As with many surgery, for HD also has some long and short term complications. Faecal incontinence and constipation remains major postoperative complication that impede social and psychological developmental in these patients.…”

Section: Discussionmentioning

confidence: 99%

“…Additional modification has involved the creation of the anastomosis with the use of mechanical stapling devices. 11 An additional modification has centred around eliminating the proximal rectal pouch "SPUR" in the aganglionic rectum, which can trap large accumulation of stool. Martin and Altemeir and Martin and Caudill described careful clamp placement to entirely eliminate the rectal pouch.…”

Section: Introductionmentioning

confidence: 99%

Assessment of functional outcome in patients of Hirschprung’s disease following two stages Duhamel pull through

2020

IJMPO

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Purpose: The aim of this study was to assess the functional outcome in patients of Hirschprung's disease (HD) following two stages Duhamel pull through operation, that is levelling colostomy followed by the pull through. Materials and Methods: The study was conducted in the Department of Pediatric Surgery of a busy tertiary care children's hospital. Patients who completed the two stage Duhamel pull through operation at least 6 months prior and aged more than three years were included in the study. The quality of life (QOL) was assessed by using a questionnaire to the parents and clinical examination of the child. Continence status was assessed by applying the Krickenbeck Score.

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Critical Factors Affecting Quality of Life of Adult Patients With Anorectal Malformations or Hirschsprung's Disease

Cited by 79 publications

References 27 publications

Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood

Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood

Hedgehog signalling is required for cloacal development in the zebrafish embryo

Assessment of functional outcome in patients of Hirschprung’s disease following two stages Duhamel pull through

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