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intestinal neuronal dysplasia

 
Intestinal neuronal dysplasia is a complex alteration of the enteric nervous system that may involve rectum, colon, or the whole intestine. This disorder is a frequent cause of intestinal dysmotility and pseudo-obstruction in the first 3 years of life.

Associations (30.5%) (#11819202#)

-  anorectal malformations
-  intestinal malrotation
-  megacystis
-  congenital short small bowel
-  hypertrophic pyloric stenosis
-  necrotizing enterocolitis
-  mental retardation
-  short stature
-  facial dysmorphism
-  Down syndrome
-  intestinal atresia
-  diffuse intestinal angiomatosis
-  histiocytosis
-  microvillus agenesia
-  hearing loss

References

-  Kapur RP. Neuronal dysplasia: a controversial pathological correlate of intestinal pseudo-obstruction. Am J Med Genet. 2003 Nov 1;122A(4):287-93. PMID: #14518065#

-  Holland-Cunz S, Krammer HJ, Suss A, Tafazzoli K, Wedel T. Molecular genetics of colorectal motility disorders. Eur J Pediatr Surg. 2003 Jun;13(3):146-51. PMID: #12939697#

-  Schofield DE, Yunis EJ. What is intestinal neuronal dysplasia? Pathol Annu. 1992;27 Pt 1:249-62. PMID: #1736245#

-  Schofield DE, Yunis EJ. Intestinal neuronal dysplasia. J Pediatr Gastroenterol Nutr. 1991 Feb;12(2):182-9. PMID: #2051270#

-  Puri P, Wester T. Intestinal neuronal dysplasia. Semin Pediatr Surg. 1998 Aug;7(3):181-6. PMID: #9718657#



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