A very rare cause of intestinal pseudoobstruction: familial visceral myopathy type IV

Kansu, Aydan; Ensarı, Arzu; Kalaycı, A. G.; Girgin, Nurten

doi:10.1080/080352500750044115

Cited by 4 publications

(1 citation statement)

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“…Such different properties could potentially play a role in the differential response of these two muscles in disease. For example, familial type IV visceral myopathy presents with hypertophy of the inner circular and atrophy of the outer longitudinal muscle of the small bowel and is a rare cause of chronic intestinal pseudoobstruction40, 41. Given the ability of Hh signaling to modulate villus core smooth muscle as shown above and in our earlier studies17, it will be important to examine whether increased Hedgehog signaling plays any role in the etiology of this rare but usually fatal pathological condition.…”

Section: Discussionmentioning

confidence: 91%

Paracrine Hedgehog Signaling in Stomach and Intestine: New Roles for Hedgehog in Gastrointestinal Patterning

et al. 2009

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Background & Aims-Hedgehog signaling is critical in gastrointestinal patterning. Mice deficient in Hedgehog signaling exhibit abnormalities that mirror deformities seen in the human VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) association. However, the direction of Hedgehog signal flow is controversial and the cellular targets of Hedgehog signaling change with time during development. We profiled cellular Hedgehog response patterns from embryonic day 10.5 (E10.5) to adult in murine antrum, pyloric region, small intestine and colon.

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Section: Discussionmentioning

confidence: 91%

Paracrine Hedgehog Signaling in Stomach and Intestine: New Roles for Hedgehog in Gastrointestinal Patterning

et al. 2009

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From the bench to the ‘crib’‐side: implications of scientific advances to paediatric neurogastroenterology and motility

Lorenzo

2006

Neurogastroenterology Motil

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Paediatric gastrointestinal motility disorders may present in the neonatal period as the result of a congenital insult that occurred during embryonic development or as a manifestation of an abnormal genetic background. Functional gastrointestinal and motility disorders may also be acquired and present during childhood as the first presentation of a condition that can persist or re-occur throughout adolescence and adulthood. These disorders can have a significant psychological and financial impact on the lives of the affected children and their families. Recently, enteric neuroscience research has advanced the understanding of the pathogenesis and treatment of uncommon congenital or developmental gastrointestinal motility disorders such as Hirschsprung disease and chronic intestinal pseudo-obstruction. In addition, research has contributed to improvements in the understanding of more prevalent functional gastrointestinal disorders in children, such as chronic constipation and functional abdominal pain syndromes. The purpose of this review is to highlight these advances with particular regard to the clinical impact they have in the understanding and management of disorders in the field of paediatric neurogastroenterology and motility.

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Familial visceral myopathy with pseudo-obstruction, megaduodenum, Barrett's esophagus, and cardiac abnormalities

Mungan¹,

Akyüz²,

Buğra³

et al. 2003

Am J Gastroenterology

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This report describes a new subgroup of familial visceral myopathy. Three patients from within this family were admitted to the hospital with pseudo-obstruction. Barium x-ray, abdominal plain film, esophageal manometry, colonoscopy, gastroscopy, and echocardiography were performed in all siblings for diagnostic evaluation. Two of our patients had surgery because of suspicion of acute abdomen. In one of them, full-thickness biopsy, which was performed during laparotomy, revealed findings that were compatible with familial visceral myopathy. Three siblings from this family with visceral myopathy, in which the parents were consanguineous, had megaduodenum, long-segment Barrett's esophagus, and different cardiac abnormalities.

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A very rare cause of intestinal pseudoobstruction: familial visceral myopathy type IV

Cited by 4 publications

References 0 publications

Paracrine Hedgehog Signaling in Stomach and Intestine: New Roles for Hedgehog in Gastrointestinal Patterning

Paracrine Hedgehog Signaling in Stomach and Intestine: New Roles for Hedgehog in Gastrointestinal Patterning

From the bench to the ‘crib’‐side: implications of scientific advances to paediatric neurogastroenterology and motility

Familial visceral myopathy with pseudo-obstruction, megaduodenum, Barrett's esophagus, and cardiac abnormalities

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