Plummer-Vinson syndrome pathophysiology: Difference between revisions
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** The dysphagia in Plummer-Vinson syndrome results from esophageal (postcricoid) web or stricture. However, patients who do not exhibit obstructive lesions may have dysphagia resulting from muscular in-coordination. | ** The dysphagia in Plummer-Vinson syndrome results from esophageal (postcricoid) web or stricture. However, patients who do not exhibit obstructive lesions may have dysphagia resulting from muscular in-coordination. | ||
*Other factors involved in the pathogenesis of Plummer-Vinson syndrome include malnutrition, genetic predisposition and autoimmune disorders. | *Other factors involved in the pathogenesis of Plummer-Vinson syndrome include malnutrition, genetic predisposition and autoimmune disorders.<ref name="pmid23372354">{{cite journal |vauthors=Chong VH |title=Clinical significance of heterotopic gastric mucosal patch of the proximal esophagus |journal=World J. Gastroenterol. |volume=19 |issue=3 |pages=331–8 |year=2013 |pmid=23372354 |pmc=3554816 |doi=10.3748/wjg.v19.i3.331 |url=}}</ref><ref name="pmid8508079">{{cite journal |vauthors=Buse PE, Zuckerman GR, Balfe DM |title=Cervical esophageal web associated with a patch of heterotopic gastric mucosa |journal=Abdom Imaging |volume=18 |issue=3 |pages=227–8 |year=1993 |pmid=8508079 |doi= |url=}}</ref><ref name="pmid19473208">{{cite journal |vauthors=Basseri B, Conklin JL, Mertens RB, Lo SK, Bellack GS, Shaye OA |title=Heterotopic gastric mucosa (inlet patch) in a patient with laryngopharyngeal reflux (LPR) and laryngeal carcinoma: a case report and review of literature |journal=Dis. Esophagus |volume=22 |issue=4 |pages=E1–5 |year=2009 |pmid=19473208 |doi=10.1111/j.1442-2050.2008.00915.x |url=}}</ref><ref name="pmid8122657">{{cite journal |vauthors=Jerome-Zapadka KM, Clarke MR, Sekas G |title=Recurrent upper esophageal webs in association with heterotopic gastric mucosa: case report and literature review |journal=Am. J. Gastroenterol. |volume=89 |issue=3 |pages=421–4 |year=1994 |pmid=8122657 |doi= |url=}}</ref><ref name="pmid4007426">{{cite journal |vauthors=Jabbari M, Goresky CA, Lough J, Yaffe C, Daly D, Côté C |title=The inlet patch: heterotopic gastric mucosa in the upper esophagus |journal=Gastroenterology |volume=89 |issue=2 |pages=352–6 |year=1985 |pmid=4007426 |doi= |url=}}</ref><ref name="pmid15056100">{{cite journal |vauthors=von Rahden BH, Stein HJ, Becker K, Liebermann-Meffert D, Siewert JR |title=Heterotopic gastric mucosa of the esophagus: literature-review and proposal of a clinicopathologic classification |journal=Am. J. Gastroenterol. |volume=99 |issue=3 |pages=543–51 |year=2004 |pmid=15056100 |doi=10.1111/j.1572-0241.2004.04082.x |url=}}</ref> | ||
** Plummer-Vinson syndrome is seen in certain genetically predisposed individuals (such as malformation of esophagus) and those with malnutrition. | ** Plummer-Vinson syndrome is seen in certain genetically predisposed individuals (such as malformation of esophagus) and those with malnutrition. | ||
** These patients have been associated with the presence of heterotopic gastric mucosa of the proximal esophagus (HGMPE). | ** These patients have been associated with the presence of heterotopic gastric mucosa of the proximal esophagus (HGMPE). | ||
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Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Pathogenesis
- Plummer-Vinson syndrome is a rare condition characterized by iron-deficiency anemia, glossitis and dysphagia.
- The exact pathogenesis of Plummer-Vinson syndrome is not fully understood. It is postulated that Plummer-Vinson syndrome results from iron deficiency. Other possible factors include malnutrition, genetic predisposition and autoimmune disorders.[1][2][3][4]
- Iron plays a major role in the expression of citric acid cycle enzymes such as citrate synthase, isocitric dehydrogenase, and succinate dehydrogenase.
- Iron replete cells with normal citric acid cycle have an increased formation of reducing equivalents (NADH) thus leading to increased ATP formation via oxidative phosphorylation.
- In patients with iron deficiency, all these metabolic pathways (oxidative phosphorylation) are reduced. This promotes anaerobic metabolism with increased consumption of glucose and increased production of lactic acid.
- In patients with iron deficiency, the iron-dependent oxidative enzymes are unable to function at optimum level and may lead to myasthenic changes in muscles.
- These myasthenic changes are often seen in muscles involved in swallowing and may lead to atrophy of the esophageal mucosa and formation of esophageal webs.
- The dysphagia in Plummer-Vinson syndrome results from esophageal (postcricoid) web or stricture. However, patients who do not exhibit obstructive lesions may have dysphagia resulting from muscular in-coordination.
- Other factors involved in the pathogenesis of Plummer-Vinson syndrome include malnutrition, genetic predisposition and autoimmune disorders.[5][6][7][8][9][10]
- Plummer-Vinson syndrome is seen in certain genetically predisposed individuals (such as malformation of esophagus) and those with malnutrition.
- These patients have been associated with the presence of heterotopic gastric mucosa of the proximal esophagus (HGMPE).
- The HGMPE is also known as inlet patch or cervical inlet patch. HGMPE is a salmon colored patch and is located just distal to the upper esophageal sphincter.
- The HGMPE has been associated with increased laryngopharyngeal acid reflux and neoplastic transformation.
- Thus patients with HGMPE have been associated with an increased frequency of bleeding (iron deficiency anemia) and ulceration leading to formation of web like structures in the esophagus
- Some researchers postulate that Plummer-Vinson syndrome may be due to an autoimmune condition. This can be attributed to the fact that Plummer-Vinson syndrome has been associated with other autoimmune conditions such as pernicious anemia, rheumatoid arthritis, autoimmune thyroiditis and celiac disease.[11][12][13]
OR
- It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
- [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
- Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
- [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
- The progression to [disease name] usually involves the [molecular pathway].
- The pathophysiology of [disease/malignancy] depends on the histological subtype.
Genetics
- [Disease name] is transmitted in [mode of genetic transmission] pattern.
- Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
- The development of [disease name] is the result of multiple genetic mutations.
Associated Conditions
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, Plummer-Vinson syndrome presents with the following findings:
- Epithelial atrophy
- Chronic submucosal inflammation
- Epithelial atypia or dysplasia (in advanced cases)
Pathophysiology
References
- ↑ Chisholm M (1974). "The association between webs, iron and post-cricoid carcinoma". Postgrad Med J. 50 (582): 215–9. PMC 2495558. PMID 4449772.
- ↑ Dantas RO, Villanova MG (1993). "Esophageal motility impairment in Plummer-Vinson syndrome. Correction by iron treatment". Dig. Dis. Sci. 38 (5): 968–71. PMID 8482199.
- ↑ Novacek G (2006). "Plummer-Vinson syndrome". Orphanet J Rare Dis. 1: 36. doi:10.1186/1750-1172-1-36. PMC 1586011. PMID 16978405.
- ↑ Ekberg O, Malmquist J, Lindgren S (1986). "Pharyngo-oesophageal webs in dysphageal patients. A radiologic and clinical investigation in 1134 patients". Rofo. 145 (1): 75–80. doi:10.1055/s-2008-1048889. PMID 3016824.
- ↑ Chong VH (2013). "Clinical significance of heterotopic gastric mucosal patch of the proximal esophagus". World J. Gastroenterol. 19 (3): 331–8. doi:10.3748/wjg.v19.i3.331. PMC 3554816. PMID 23372354.
- ↑ Buse PE, Zuckerman GR, Balfe DM (1993). "Cervical esophageal web associated with a patch of heterotopic gastric mucosa". Abdom Imaging. 18 (3): 227–8. PMID 8508079.
- ↑ Basseri B, Conklin JL, Mertens RB, Lo SK, Bellack GS, Shaye OA (2009). "Heterotopic gastric mucosa (inlet patch) in a patient with laryngopharyngeal reflux (LPR) and laryngeal carcinoma: a case report and review of literature". Dis. Esophagus. 22 (4): E1–5. doi:10.1111/j.1442-2050.2008.00915.x. PMID 19473208.
- ↑ Jerome-Zapadka KM, Clarke MR, Sekas G (1994). "Recurrent upper esophageal webs in association with heterotopic gastric mucosa: case report and literature review". Am. J. Gastroenterol. 89 (3): 421–4. PMID 8122657.
- ↑ Jabbari M, Goresky CA, Lough J, Yaffe C, Daly D, Côté C (1985). "The inlet patch: heterotopic gastric mucosa in the upper esophagus". Gastroenterology. 89 (2): 352–6. PMID 4007426.
- ↑ von Rahden BH, Stein HJ, Becker K, Liebermann-Meffert D, Siewert JR (2004). "Heterotopic gastric mucosa of the esophagus: literature-review and proposal of a clinicopathologic classification". Am. J. Gastroenterol. 99 (3): 543–51. doi:10.1111/j.1572-0241.2004.04082.x. PMID 15056100.
- ↑ Dickey W, McConnell B (1999). "Celiac disease presenting as the Paterson-Brown Kelly (Plummer-Vinson) syndrome". Am. J. Gastroenterol. 94 (2): 527–9. doi:10.1111/j.1572-0241.1999.889_r.x. PMID 10022662.
- ↑ Malhotra P, Kochhar R, Varma N, Kumari S, Jain S, Varma S (2000). "Paterson-Kelly syndrome and celiac disease--a rare combination". Indian J Gastroenterol. 19 (4): 191–2. PMID 11059192.
- ↑ ELWOOD PC, JACOBS A, PITMAN RG, ENTWISTLE CC (1964). "EPIDEMIOLOGY OF THE PATERSON-KELLY SYNDROME". Lancet. 2 (7362): 716–20. PMID 14193944.