Pyloric stenosis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]
Overview
The pathogenesis of infantile hypertrophic pyloric stenosis is not fully understood but it may be due abnormal innervation of the pyloric smooth muscle.
Pathophysiology
The pathogenesis of infantile hypertrophic pyloric stenosis is not fully understood but it may be due abnormal innervation of the pyloric smooth muscle. There are also some locus evidences of increased collagen production and abnormal amounts of extracellular matrix proteins in hypertrophic pyloric muscle.[1] There is no known pathophysiology for adult-onset hypertrophic pyloric stenosis (HPS).
Genetics
In one study, the familial recurrence pattern among monozygotic cotwins and more remote relatives of IHPS probands was found to be inconsistent with generalized single major inheritance.[2]
Associated Conditions of Infantile Hypertrophic Pyloric Stenosis
- Neuromuscular disorders
- Connective tissue disorders
- Metabolic disorders
- Intracellular signalling pathway disturbances
- Intercellular communication disturbances
- Ciliopathies
- DNA-repair disturbances
- Transcription regulation disorders
- MAPK-pathway disturbances
- Lymphatic abnormalities
- Environmental factors[3]
References
- ↑ Ohshiro K, Puri P (1998). "Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress". Pediatr Surg Int. 13 (4): 243–52. doi:10.1007/s003830050308. PMID 9553181.
- ↑ Mitchell LE, Risch N (1993). "The genetics of infantile hypertrophic pyloric stenosis. A reanalysis". Am J Dis Child. 147 (11): 1203–11. PMID 8237916.
- ↑ Peeters B, Benninga MA, Hennekam RC (2012). "Infantile hypertrophic pyloric stenosis--genetics and syndromes". Nat Rev Gastroenterol Hepatol. 9 (11): 646–60. doi:10.1038/nrgastro.2012.133. PMID 22777173.