Nonphysiologic lactation, resulting from endocrinologic causes or from a pituitary disorder. Adhesions within the endometrial cavity, causing amenorrhea and infertility. Sideropenic dysphagia is more commonly known as plummer vinson syndrome pvs in the united states. Prognosis of the plummer vinson syndrome is excellent. Henry plummer in 19 described a clinically distinct form of thyrotoxicosis due to solitary or multiple toxic nodules in the thyroid.
Rapidly progressive plummer michael skolka and vinson. Treatment of the iron deficiency frequently relieves dysphagia though some patients may need endoscopic web dilatation. Symptoms resulting from anemia predominates the clinical picture, apart from the additional features such as glossitis, angular cheilitis, and dysphagia. It has been known since the beginning of the 20th century. Plummer 18741937 and surgeon porter p vinson 18901959. May 24, 2005 the american thyroid association has found no scientific evidence supporting the existence of wilsons syndrome. Vinson syndrome, esophagography often reveals a web at the anterior wall of the cervical esophagus. Plummer vinson syndrome with simultaneous midesophageal growth mukesh nasa1, gaurav patil 2, zubin sharma, rajesh puri3 abstract patterson brown kelly or plummer vinson syndrome is characterized by dysphagia, iron deficiency anemia and postcricoidal esophageal web. Definition plummer vinson syndrome pvs is a classical triad of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia. Even though the syndrome is very rare nowadays, its recognition is important because it identifies a group of patients at increased risk of squamous cell carcinoma of the pharynx and the esophagus. With the forceps biopsy some membranes are removed. Plummer vinson syndrome in a male and his chromosomal study a case report article pdf available in egyptian journal of medical human genetics 583 may 2015 with 102 reads. Plummervinson syndrome orphanet journal of rare diseases. Aug 04, 2014 plummer vinson syndrome pvs is a triad of microcytic hypochromic anemia, atrophic glossitis, and esophageal webs or strictures.
Diagnostic criteria for wilsons syndrome are imprecise and could lead to misdiagnosis of many other conditions. History of painless dysphagia and the finding of an esophageal web. Plummervinson syndrome definition of plummervinson. To determine the role of esophagoscopy as a safe and costeffective procedure which can be used. Plummer vinson syndrome symptoms, causes, diagnosis, treatment, esophageal cancer. It is one of the syndromes associated with iron deficiency anemia. Elemental iron in the range of 150200 mg is required for correction of iron deficiency anemia which in most cases corrects. His high energy style and mnemonics make learning medicine fun. Sep 01, 2006 plummer vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. Plummer vinson syndrome symptoms, diagnosis, treatment, causes. Plummer vinson syndrome has been identified as a risk factor for developing squamous cell carcinoma of the upper gastrointestinal tract. Esophagoscopy in the early detection and treatment of plummer.
In adults, plummer vinson syndrome may be associated with iron deficiency or diverse inflammatory disorders. Fulfilling the classical triad of dysphagia, irondeficiency anaemia and upper oesophageal webs, plummer vinson syndrome or patersonbrown kelly syndrome, sideropenic dysphagia was diagnosed. Wilson disease is present at birth, but symptoms usually start between ages 5 and 35. The pathogenesis of the esophageal web and the cause of dysphagia in this syn. Also known as sideropenic dysphagia and, in the uk, as patersonkelly syndrome. This name comes from two americans named henry stanley plummer and porter paisley vinson.
It is a rare disorder consisting of the clinical triad of dysphagia, an esophageal web or membrane and iron deficiency anemia. At the first diagnostic evaluation final results of only 5 out 17 patients were designated as associated graves disease and plummers disease table 1. Vinson was a surgeon born in 1890 and also worked at the mayo. This means that plummer vinson syndrome, or a subtype of plummer vinson syndrome, affects less than 200,000 people in the us population. Wilsons temperature syndrome, also called wilsons thyroid syndrome or wts, is an alternative medicine concept which attributes various common and nonspecific symptoms to low body temperature and impaired conversion of thyroxine t4 to triiodothyronine t3, despite normal thyroid function tests. Dec 01, 20 read plummer vinson syndrome, clinical gastroenterology and hepatology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Patients must be screened regularly for any development of upper gastrointestinal malignancy.
Plummer vinson syndrome can be treated effectively with iron. Plummer vinson syndrome is characterized by cervical dysphagia, iron deficiency anemia, and upper esophageal web or webs. The reason additional investments in quality dont always lead to greater impact is the opportunity cost of time. Pdf plummer vinson syndrome in a male and his chromosomal. Revisiting plummer vinson syndrome gude d, bansal dp1, malu a internal medicine, 1pulmonology and critical care, medwin hospital, nampally, hyderabad, andhra pradesh, india abstract plummer vinson syndrome is a rare association of postcricoid dysphagia. For 17 patients of the last group by detailed followup of relevant diagnostic parameters, associated graves disease and plummers disease is documented. American thyroid association statement on wilsons syndrome. Ingestion of food produces nausea, pain, and duodenal distension. If we had an infinite supply of time, this may not be the case, but we know that. On upper gastrointestinal endoscopy, post cricoids oesophageal web was seen and the endoscope could not be passed across the cricoid level. Plummer vinson syndrome is a rare cause of dysphagia in children because of an upper esophageal web. Feb 14, 2019 the association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as plummer vinson syndrome pvs in the united states and patersonbrown kelly syndrome in the united kingdom. In 1912, plummer reported a series of twenty one patients with.
Over a period of two weeks, the patient first noticed difficulty swallowing large chunks of food followed shortly by trouble. Esophagoscopy in the early detection and treatment of plummer vinson syndrome muhammad aslam chaudhry, ashar alamgir. Jul 10, 2017 mowat wilson syndrome mws is a rare genetic disorder that affects many systems of the body. Plummervinson syndrome, diseases of the esophagus 10.
Plummer s disease is a condition where there is a single hyperfunctioning nodule producing excess thyroid hormone. Mowat wilson syndrome mws is a rare genetic disorder that may be apparent at birth or later in childhood. This is a syndrome occurring with chronic, long term iron deficiency anemia. Plummer vinson syndrome is a condition that can occur in people with longterm chronic iron deficiency anemia. Plummer vinson syndrome also known as paterson brownkelly syndrome is a syndrome associated with the triad of symptoms comprising microcytic hypochromic anemia, oesophageal strictures, and dysphagia. The most characteristic sign is a rusty brown ring around the cornea of the eye. Plummer vinson syndrome is defined by the classic triad of dysphagia, irondeficiency anemia and esophageal webs. Plummervinson syndrome chiachun liu, yaohshiang lin, 2019.
Angular chelitis and atrophic glossitis are other common. Plummer vinson syndrome pvs also named as pattersonbrownkelly syndrome is a combined presentation of three things dysphagia, iron deficiency anemia, and esophageal webs, seen more often in. Gastrojejunal loop obstruction, proximal to a gastrojejunostomy. Dysphagia koilonychia, gastric achlorhydria, glossitis, and hypochromic microcytic anemia caused by iron deficiency. Dec 23, 2016 case report case reports in surgery and invasive procedures 2017 case reports in surgery and invasive procedures special issue 12017 chronic iron deficiency anaemia after rouxeny gastric bypass leading to plummer vinson syndrome. The first step in the management of plummervinson syndrome is to clarify the cause of iron deficiency in order to. Pdf plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, irondeficiency anemia and esophageal webs. People with this condition have problems swallowing due to small, thin growths of tissue that partially block the upper food pipe esophagus. Plummer was a prominent internist and endocrinologist born in 1874 and was one of the founding members of the mayo clinic. Plummervinson syndrome symptoms, diagnosis, treatments. Mws is characterized by intellectual disability, distinctive facial features and seizures.
Plummervinson syndrome is a rare disease characterized by difficulty swallowing, irondeficiency anemia, glossitis, cheilosis and esophageal webs. Pvs is commonly found in women of middle age especially in the fourth and fifth decade of life and is rarely reported in males. Full text iron deficiency anemia and plummervinson syndrome. Other findings, probably related to the iron deficiency, can include glossitis, cheilosis and. It first attacks the liver, the central nervous system or both. Postcricoid web or plummer vinson syndrome has been identi.
We report a case of pvs in a 62yearold patient admitted to the department of gastroenterology an\ d internal medicine for high dysphagia. Waldenstorm introduced the term sideropenic dysphagia because of absence. In adults, iron supplementation is frequently sufficient to control dysphagia. Treatment is iron supplementation and, if need be, dilation of the web to permit normal swallowing and the passage of food. Cae e urg naie roe 21 oue 1 ue 1 2 population that has undergone rouxeny gastric bypass surgery 2,3. Syndromes are the group of symptoms that collectively indicate or characterize a disease. Plummervinson syndrome, clinical gastroenterology and. Plummer vinson syndrome classically presents as a triad of irondeficiency anaemia, postcricoid dysphagia and upper esophageal webs. Also known as patersonkelly syndrome explanation of plummer winson syndrome.
Fahmi yousef khan, a haleem elhiday and nader a morad. Wilson s disease is a rare inherited disorder that causes copper to accumulate in your liver, brain and other vital organs. Plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, irondeficiency anemia and esophageal. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Find out information about plummer winson syndrome. Revisiting plummer vinson syndrome a report of three cases. Data regarding this condition is limited to mostly case reports and a few small cohort studies. Although most cases have a benign and indolent course, the risk of malignancy warrants longterm surveillance. Some of the main features include intellectual disability, distinctive facial features, delayed development, and hirschsprung disease. Chronic iron deficiency anaemia after rouxeny gastric bypass leading to plummer vinson syndrome.
Case report a 47yearold african american female with a history of hypertension presented to the emergency room with dysphagia. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome. Plummer vinson syndrome is a rare condition associated with dysphagia, iron deficiency, and esophageal webs. A physical exam and laboratory tests can diagnose it. Plummer vinson syndrome is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Dr barone teaches usmle pathology, internal medicine, medical genetics, immunology, and physiology. Summary plummervinson syndrome is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal web or webs. The most usual presenting symptom the dysphagia can be intermittent or progressive through the years. Major signs of this disorder frequently include distinctive facial features, intellectual disability, delayed development, an intestinal disorder called hirschsprung disease, and other birth defects. Presence of glossitis, angular cheilitis, and koilonychia.
Esophageal motility impairment in plummervinson syndrome. Plummervinson syndrome radiology reference article. Fda approves new iv treatment for iron deficiency anemia fast five quiz. Esophagus patersonbrownkelly or plummer vinson syndrome. Mowatwilson syndrome genetic and rare diseases information. Department of ent, rawalpindi general hospital and rawalpindi medical college, rawalpindi. There are a lot of disease that are all to do with the thyroid gland and they are all very horrible disease that can cause a lot. In subsequent endoscopy, the webs were dilated with considerable resultant improvement in.
The term sideropenic dysphagia has also been used, since the syndrome can occur with iron deficiency sideropenia, but it is not as. Pdf plummervinson or patersonkelly syndrome presents as a classical triad of dysphagia, iron deficiency anemia and esophageal webs. Plummer vinson syndrome \pvs\ is a rare condition characterized by classical triad. Both patients presented with dysphagia, anemia, sideropenia, glossitis and cheilitis. Plummer vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. Plummer disease, thyroid condition characterized by marked enlargement of the thyroid gland goitre, firm thyroid nodules, and overproduction of thyroid hormone hyperthyroidism. Currently this syndrome is extremely rare but it is significant to be able to recognize it as it isolates a patient group that has an enlarged risk of squamous cell carcinoma of the esophagus and pharynx. Declaration of conflicting interests the authors declared no potential conflicts of interest with respect to the research, authorship, andor publication of this article. Mowat wilson syndrome is a genetic condition that affects many parts of the body. Sep 15, 2006 plummer vinson syndrome has been identified as a risk factor for developing squamous cell carcinoma of the upper gastrointestinal tract. Plummerwinson syndrome article about plummerwinson. Plummer vinson syndrome symptoms, diagnosis, treatment. As described above, dysphagia and anemia can be treated effectively.
Dysphagia, plummer vinson syndrome, iron deficiency anaemia, oesophageal webs, deglutition disorders introduction plummer vinson syndrome pvs is named after henry stanley plummer and porter paisley vinson, who were physicians on the staff of the mayo clinic. The theory proposed to explain this condition is at odds with established facts about thyroid hormone. The plummervinson syndrome is characterized by dysphagia. Department of medicine, university of california, san diego, california. Three to 15 per cent of the patients with plummer vinson syndrome, mostly women between 15 and 50 years of age, have been reported to develop esophageal or pharyngeal cancer 15, 16. Most people with wilson s disease are diagnosed between the ages of 5 and 35, but it can affect younger and older people, as well. Chronic iron deficiency anaemia after rouxeny gastric. Plummer vinson syndrome, also known as the patersonbrownkelly syndrome, predisposes to hypopharyngeal squamous cell carcinoma and consists of four features. While this syndrome is known as plummer vinson syndrome in the united states, it is known as patersonbrown kelly syndrome in the united kingdom. Revisiting plummer vinson syndrome annals of medical and. Kelly syndrome pvs is a rare entity comprising upper esophageal webs and iron deficiency anemia. Three to 15 per cent of the patients with plummer vinson syndrome, mostly women between 15 and 50 years of age, have been reported to. Iatrogenic or secondary to medical treatment afferent loop syndrome.
Plummer vinson syndrome, if left untreated, carries an increased risk of developing squamous cell carcinoma of the upper alimentary tract. The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as plummer vinson syndrome pvs in the united states and patersonbrown kelly syndrome in the united kingdom. Three to 15 per cent of the patients with plummer vinson syndrome, mostly women between 15 and 50 years of age, have been reported to develop esophageal or pharyngeal cancer 15,16. Whats where the does name plummer vinson syndrome come from. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Mowatwilson syndrome nord national organization for rare. The plummer vinson syndrome, also called the patersonbrownkelly syndrome, consists of a triad of dysphagia, iron deficiency anemia, and esophageal webs. In case of an associated squamous cell carcinoma of the hypopharynx or upper esophagus the prognosis worsens dramatically. Plummers disease is an illness that is caused by the thyroid gland which contains a small round mass and this then causes too much thyroid hormone and then that is why the patient can contract plummers disease. Treatment includes correction of iron deficiency and endoscopic dilation of the esophageal webs to relieve dysphagia. Congenital defect in ceruloplasmin, leading to buildup of copper mental retardation, cirrhosis, hepatolenticular degeneration.
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