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 completely understood. However, infantile hypertrophic pyloric stenosis may result from abnormal innervation of the pyloric smooth muscle.
Pathophysiology
The pathogenesis of infantile hypertrophic pyloric stenosis is not completely understood.
- However, infantile hypertrophic pyloric stenosis (IHPS) may result from abnormal innervation of the pyloric smooth muscle.
- Evidence of increased collagen production and abnormal amounts of extracellular matrix proteins has also been seen in patients with hypertrophic pyloric muscle.[1]
- The pathophysiology for adult-onset hypertrophic pyloric stenosis is not comprehensively understood.
Genetics
In relatives of probands of infantile hypertrophic pyloric stenosis patients and monozygotic cotwins the recurrence pattern did not depict a single major inheritance.[2]
Associated Conditions of Infantile Hypertrophic Pyloric Stenosis
Various conditions associated with infantile pyloric stenosis include:[3]
- Neuromuscular disorders
- Connective tissue disorders
- Metabolic disorders
- Intracellular signalling pathway disturbances
- Intercellular communication disturbances
- Ciliopathies
- DNA-repair abnormalities
- Transcription regulation disorders
- MAPK-pathway abnormalities
- Lymphatic abnormalities
- Environmental factors
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
The following observations may be seen in pyloric stenosis:
- Smooth muscle cells are in a proliferative phase and there is very few gap junctions between smooth muscle cells in pyloric stenosis.
- The circular muscle layer is characterized by very few large granular vesicle-containing nerve fibers.
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.