Pyloric stenosis pathophysiology: Difference between revisions
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{{Pyloric stenosis}} | {{Pyloric stenosis}} | ||
{{CMG}} {{AE}} {{MMJ}} | {{CMG}}; {{AE}} {{MMJ}} | ||
==Overview== | ==Overview== | ||
The [[pathogenesis]] of infantile hypertrophic pyloric stenosis is not completely understood<ref name="pmid9553181">{{cite journal| author=Ohshiro K, Puri P| title=Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress. | journal=Pediatr Surg Int | year= 1998 | volume= 13 | issue= 4 | pages= 243-52 | pmid=9553181 | doi=10.1007/s003830050308 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9553181 }} </ref>. However, infantile hypertrophic pyloric stenosis may result from abnormal innervation of the pyloric [[smooth muscle]].<ref name="pmid9553181">{{cite journal| author=Ohshiro K, Puri P| title=Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress. | journal=Pediatr Surg Int | year= 1998 | volume= 13 | issue= 4 | pages= 243-52 | pmid=9553181 | doi=10.1007/s003830050308 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9553181 }} </ref> The [[chloride]] loss due persistent vomiting results in [[hypochloremia]] which impairs the [[kidney]]'s ability to excrete [[bicarbonate]]. This factor significantly prevents correction of the [[alkalosis]]. The secondary [[hyperaldosteronism]] develops due to the [[hypovolaemia]]. The body's compensatory response to the [[metabolic alkalosis]] is [[hypoventilation]] resulting in an elevated arterial p[[Carbon dioxide|CO<sub>2</sub>]]. | |||
In relatives of probands of infantile hypertrophic pyloric stenosis patients and [[monozygotic]] co-twins the recurrence pattern did not depict a single major inheritance. | |||
== Pathophysiology == | == Pathophysiology == | ||
The pathogenesis of infantile hypertrophic pyloric stenosis is not | 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]].<ref name="pmid9553181">{{cite journal| author=Ohshiro K, Puri P| title=Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress. | journal=Pediatr Surg Int | year= 1998 | volume= 13 | issue= 4 | pages= 243-52 | pmid=9553181 | doi=10.1007/s003830050308 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9553181 }} </ref> | |||
*Evidence of increased [[collagen]] production and abnormal amounts of [[extracellular matrix protein]]s has also been observed in patients with hypertrophic pyloric [[muscle]].<ref name="pmid9553181">{{cite journal| author=Ohshiro K, Puri P| title=Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress. | journal=Pediatr Surg Int | year= 1998 | volume= 13 | issue= 4 | pages= 243-52 | pmid=9553181 | doi=10.1007/s003830050308 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9553181 }} </ref> | |||
*The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric contents into the [[duodenum]]. As a consequence, all ingested [[food]] and gastric secretions can only exit via [[Nausea and vomiting|vomiting]], which is projectile in nature. The vomited material does not contain [[bile]] because the pyloric obstruction prevents entry of duodenal contents (containing bile) into the [[stomach]]. This results in loss of gastric acid ([[hydrochloric acid]]). The [[chloride]] loss results in [[hypochloremia]] which impairs the [[kidney]]'s ability to excrete [[bicarbonate]]. This factor significantly prevents correction of the [[alkalosis]].<ref name="AhmadThomson2011">{{cite journal|last1=Ahmad|first1=J.|last2=Thomson|first2=S.|last3=Taylor|first3=M.|last4=Scoffield|first4=J.|title=A reminder of the classical biochemical sequelae of adult gastric outlet obstruction|journal=Case Reports|volume=2011|issue=jan29 1|year=2011|pages=bcr0520102978–bcr0520102978|issn=1757-790X|doi=10.1136/bcr.05.2010.2978}}</ref> | |||
*A secondary [[hyperaldosteronism]] develops due to the [[hypovolaemia]]. The high [[aldosterone]] levels causes the [[Kidney|kidneys]] to:<ref name="pmid11850323">{{cite journal| author=Booth RE, Johnson JP, Stockand JD| title=Aldosterone. | journal=Adv Physiol Educ | year= 2002 | volume= 26 | issue= 1-4 | pages= 8-20 | pmid=11850323 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11850323 }} </ref> | |||
**Avidly retain Na<sup>+</sup> (to correct the intravascular [[volume depletion]]). | |||
**Excrete increased amounts of K<sup>+</sup> into the [[urine]] (resulting in [[hypokalaemia]]). The body's compensatory response to the [[metabolic alkalosis]] is [[hypoventilation]] resulting in an elevated arterial p[[Carbon dioxide|CO<sub>2</sub>]].<ref name="pmid6799256">{{cite journal| author=Javaheri S, Shore NS, Rose B, Kazemi H| title=Compensatory hypoventilation in metabolic alkalosis. | journal=Chest | year= 1982 | volume= 81 | issue= 3 | pages= 296-301 | pmid=6799256 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6799256 }} </ref> | |||
{{Family tree/start}} | |||
{{Family tree | | | | A01 | | | |A01= Hypertrophic pylorus}} | |||
{{Family tree | | | | |!| | | | | }} | |||
{{Family tree | | | | B01 | | | |B01= Gastric outlet obstruction}} | |||
{{Family tree | | | | |!| | | | | }} | |||
{{Family tree | | | | C01 | | | |C01= Impaired emptying of gastric content into duodenum}} | |||
{{Family tree | | | | |!| | | | | }} | |||
{{Family tree | | | | D01 | | | |D01= All ingested food and gastric content exit through vomiting}} | |||
{{Family tree | | | | |!| | | | | }} | |||
{{Family tree | | | | E01 | | | |E01= Hypochloremia and alkalosis}} | |||
{{Family tree/end}} | |||
== Genetics == | == Genetics == | ||
In | In relatives of probands of infantile hypertrophic pyloric stenosis patients and [[monozygotic]] co-twins the recurrence pattern did not depict a single major inheritance.<ref name="pmid8237916">{{cite journal| author=Mitchell LE, Risch N| title=The genetics of infantile hypertrophic pyloric stenosis. A reanalysis. | journal=Am J Dis Child | year= 1993 | volume= 147 | issue= 11 | pages= 1203-11 | pmid=8237916 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8237916 }} </ref> | ||
== Associated Conditions of Infantile Hypertrophic Pyloric Stenosis == | |||
Various conditions associated with infantile pyloric stenosis include:<ref name="pmid22777173">{{cite journal| author=Peeters B, Benninga MA, Hennekam RC| title=Infantile hypertrophic pyloric stenosis--genetics and syndromes. | journal=Nat Rev Gastroenterol Hepatol | year= 2012 | volume= 9 | issue= 11 | pages= 646-60 | pmid=22777173 | doi=10.1038/nrgastro.2012.133 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22777173 }} </ref> | |||
*[[Neuromuscular disorder|Neuromuscular disorders]] | |||
*[[Connective tissue disorders]] | |||
*[[Metabolic disorder|Metabolic disorders]] | |||
*[[Intracellular]] signalling pathway disturbances | |||
*[[Cell signaling|Intercellular]] communication disturbances | |||
*Ciliopathies | |||
*[[DNA]]-repair abnormalities | |||
*[[Transcription (genetics)|Transcription]] regulation disorders | |||
*[[MAPK]]-pathway abnormalities | |||
*[[Lymphatic system|Lymphatic]] abnormalities | |||
*[[Environmental factor|Environmental factors]] | |||
==Gross Pathology== | |||
On gross pathology, characteristic findings of pyloric stenosis include: | |||
* Hypertrophic muscularis mucosa | |||
* Protrusion of gastric mucosa into the smooth muscle layer<ref name="pmid7039756">{{cite journal| author=Spicer RD| title=Infantile hypertrophic pyloric stenosis: a review. | journal=Br J Surg | year= 1982 | volume= 69 | issue= 3 | pages= 128-35 | pmid=7039756 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7039756 }} </ref> | |||
==Microscopic Pathology== | |||
The following observations may be seen in pyloric stenosis:<ref name="pmid8583319">{{cite journal| author=Langer JC, Berezin I, Daniel EE| title=Hypertrophic pyloric stenosis: ultrastructural abnormalities of enteric nerves and the interstitial cells of Cajal. | journal=J Pediatr Surg | year= 1995 | volume= 30 | issue= 11 | pages= 1535-43 | pmid=8583319 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8583319 }} </ref> | |||
* [[Smooth muscle|Smooth muscle cells]] are in a proliferative phase and there is very few [[Gap junction|gap junctions]] between [[Smooth muscle|smooth muscle cells]] in pyloric stenosis. | |||
* The circular muscle layer is characterized by very few large granular [[vesicle]]-containing [[nerve fibers]]. | |||
* The number of nerve cell bodies in the [[myenteric plexus]], and the total number of [[Ganglion|ganglia]] is lower than normal. | |||
* Interstitial cells of [[Cajal-Retzius cell|Cajal]] are almost completely absent. | |||
== | ==References== | ||
{{Reflist|2}} | |||
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[[Category:Surgery]] | |||
[[Category:Gastroenterology]] | |||
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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[1]. However, infantile hypertrophic pyloric stenosis may result from abnormal innervation of the pyloric smooth muscle.[1] The chloride loss due persistent vomiting results in hypochloremia which impairs the kidney's ability to excrete bicarbonate. This factor significantly prevents correction of the alkalosis. The secondary hyperaldosteronism develops due to the hypovolaemia. The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2. In relatives of probands of infantile hypertrophic pyloric stenosis patients and monozygotic co-twins the recurrence pattern did not depict a single major inheritance.
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.[1]
- Evidence of increased collagen production and abnormal amounts of extracellular matrix proteins has also been observed in patients with hypertrophic pyloric muscle.[1]
- The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric contents into the duodenum. As a consequence, all ingested food and gastric secretions can only exit via vomiting, which is projectile in nature. The vomited material does not contain bile because the pyloric obstruction prevents entry of duodenal contents (containing bile) into the stomach. This results in loss of gastric acid (hydrochloric acid). The chloride loss results in hypochloremia which impairs the kidney's ability to excrete bicarbonate. This factor significantly prevents correction of the alkalosis.[2]
- A secondary hyperaldosteronism develops due to the hypovolaemia. The high aldosterone levels causes the kidneys to:[3]
- Avidly retain Na+ (to correct the intravascular volume depletion).
- Excrete increased amounts of K+ into the urine (resulting in hypokalaemia). The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2.[4]
| Hypertrophic pylorus | |||||||||||||||||||
| Gastric outlet obstruction | |||||||||||||||||||
| Impaired emptying of gastric content into duodenum | |||||||||||||||||||
| All ingested food and gastric content exit through vomiting | |||||||||||||||||||
| Hypochloremia and alkalosis | |||||||||||||||||||
Genetics
In relatives of probands of infantile hypertrophic pyloric stenosis patients and monozygotic co-twins the recurrence pattern did not depict a single major inheritance.[5]
Associated Conditions of Infantile Hypertrophic Pyloric Stenosis
Various conditions associated with infantile pyloric stenosis include:[6]
- 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, characteristic findings of pyloric stenosis include:
- Hypertrophic muscularis mucosa
- Protrusion of gastric mucosa into the smooth muscle layer[7]
Microscopic Pathology
The following observations may be seen in pyloric stenosis:[8]
- 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.
- The number of nerve cell bodies in the myenteric plexus, and the total number of ganglia is lower than normal.
- Interstitial cells of Cajal are almost completely absent.
References
- ↑ 1.0 1.1 1.2 1.3 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.
- ↑ Ahmad, J.; Thomson, S.; Taylor, M.; Scoffield, J. (2011). "A reminder of the classical biochemical sequelae of adult gastric outlet obstruction". Case Reports. 2011 (jan29 1): bcr0520102978–bcr0520102978. doi:10.1136/bcr.05.2010.2978. ISSN 1757-790X.
- ↑ Booth RE, Johnson JP, Stockand JD (2002). "Aldosterone". Adv Physiol Educ. 26 (1–4): 8–20. PMID 11850323.
- ↑ Javaheri S, Shore NS, Rose B, Kazemi H (1982). "Compensatory hypoventilation in metabolic alkalosis". Chest. 81 (3): 296–301. PMID 6799256.
- ↑ 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.
- ↑ Spicer RD (1982). "Infantile hypertrophic pyloric stenosis: a review". Br J Surg. 69 (3): 128–35. PMID 7039756.
- ↑ Langer JC, Berezin I, Daniel EE (1995). "Hypertrophic pyloric stenosis: ultrastructural abnormalities of enteric nerves and the interstitial cells of Cajal". J Pediatr Surg. 30 (11): 1535–43. PMID 8583319.