Plummer-Vinson syndrome associated with celiac disease and complicated by postcricoid carcinoma and carcinoma of the tongue [7]

ArticleinThe American Journal of Gastroenterology 98(5):1208-9 · June 2003with 32 Reads
Cite this publication
Abstract
The American Journal of Gastroenterology is published by Nature Publishing Group (NPG) on behalf of the American College of Gastroenterology (ACG). Ranked the #1 clinical journal covering gastroenterology and hepatology*, The American Journal of Gastroenterology (AJG) provides practical and professional support for clinicians dealing with the gastroenterological disorders seen most often in patients. Published with practicing clinicians in mind, the journal aims to be easily accessible, organizing its content by topic, both online and in print. www.amjgastro.com, *2007 Journal Citation Report (Thomson Reuters, 2008)
Ad

Do you want to read the rest of this article?

Request full-text
Request Full-text Paper PDF
  • Article
    Full-text available
    Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the syndrome are not available; the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia) may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers. Etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. 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. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.
  • Article
    Full-text available
    Plummer-Vinson syndrome (PVS) is characterized by the presence of postcricoid dysphagia, iron deficiency anemia, and upper esophageal web. This syndrome is now a rare condition because of the improvement in nutritional status and increased awareness regarding iron deficiency anemia and the early diagnosis and easy treatment of this anemia or underlying causes. In this presentation, we report two middle-aged female patients with PVS and briefly review the literature.
  • Article
    Plummer-Vinson (Paterson-Brown-Kelly) syndrome, characterized by iron-deficiency anemia, upper esophageal webs, and dysphagia, is extremely uncommon nowadays. Although iron repletion improves its symptoms, endoscopic dilatation of associated esophageal webs is sometimes required. We report nine cases of Plummer-Vinson syndrome that were successfully treated by endoscopic dilatation. All patients had a history of anemia and slowly progressive dysphagia of solid food. Hematological tests showed iron deficiency anemia. Upper gastrointestinal endoscopy showed the presence of a web in the subcricoid region. All patients were treated with esophageal bougienage, and iron supplementation. Endoscopic bougienage was performed with the use of Celestin dilators of serially increasing diameters. The webs were easily disrupted without complications. The patient’s dysphagia resolved shortly after the treatment. However, three patients had a recurrence of dysphagia associated with anemia within an average of 8.3 months. These patients were successfully treated by a second endoscopic dilatation. All patients were examined periodically after the initial treatment and found to be in good general condition. This experience indicates that endoscopic dilatation is safe, effective, and relatively easy to perform in patients with Plummer-Vinson syndrome, although several sessions are sometimes necessary.
  • Article
    Full-text available
    Plummer-Vinson syndrome (PVS) also known as Paterson-Brown-Kelly syndrome is a rare syndrome which comprises iron deficiency anemia, dysphagia, and esophageal webs. The pathogenesis of PVS is not clear. Iron deficiency anemia is essential for diagnosis of PVS. If left untreated, there is an increased risk of developing pharyngeal or esophageal cancer in about 10% of patients. There are no strict guidelines for endoscopic surveillance in patients with PVS. Iron replacement can improve dysphagia and potentially lead to regression of esophageal webs. In this case report, we present a patient who had long-standing dysphagia for years which progressed to squamous cell cancer of esophagus by the time she sought medical treatment.
  • Article
    Plummer-Vinson syndrome (PVS) also named as Patterson-Brown-Kelly syndrome is a combined presentation of three things- dysphagia, iron deficiency anemia, and esophageal webs, seen more often in middle aged females. A 30-year-old female presented to us with shortness of breath on exertion and long standing dysphagia and weight loss. After investigations she was found to be severely anemic. Upper GI endoscopy revealed esophageal web. Dilatation of esophageal web was done, anemia was corrected. Patient is on regular follow-up with marked improvement in terms of weight gain and increased functional capacity.
  • Article
    Full-text available
    Plummer Vinson syndrome (PVS) is a triad of iron deficiency anemia, esophageal web and dysphagia. The exact etiology of PVS remains controversial but it has been associated with nutritional deficiency, autoimmune disorders, hereditary factors and remarkable high female predominance. This paper reports an atypical presentation of PVS in a 38 year old Indian male with special emphasis given on chromosomal analysis. Chromosomal assessment is done as it is a good predictor of the possibility of development of post-cricoid carcinoma (PCC) in patients with PVS. Chromosomal aberrations like translocation, gain, loss, breakpoints and duplications are studied and they revealed normal male chromosomal pairing.
  • Article
    Full-text available
    Abstract The data on esophageal and gastric cancers in sub-Saharan Africa are fragmented and not numerous because of the rarity and of the inaccessibility of the diagnostic means. Esophageal cancer has very variable incidences according to the geographical zones there and nutritional and infectious factors are incriminated in its arisen. It reaches young subjects with a preferential siege the average esophagus with ascendancy of squamous cell carcinomas. For gastric cancer, its incidence there is low despite the strong prevalence of Helicobacter pylori infection. As in Western countries, Helicobacter pylori’s gene Cag A is correlated in its arisen there but the low prevalence of strains with multiple EPIYA-C segments might contribute to its low incidence. It affects young subjects more often male with a big ascendancy of adenocarcinoma. The improvement of the diagnostic conditions and the financing of researches are necessary for a better knowledge of the risk and the protective factors of esophageal and gastric cancers in sub-Saharan Africa.
  • Chapter
    There is a growing awareness of the important relationships between systemic inflammatory diseases, infections, genetic disorders, medical therapies and cancer risk. A number of systemic disorders have been associated with an increased risk of head and neck squamous cell carcinoma. These include autoimmune conditions, genetic syndromes, infections, iatrogenic causes such as haematopoietic stem cell transplantation and graft-versus-host disease and rare associations including novel drugs. This chapter will discuss conditions which confer an increased risk of head and neck squamous cell carcinoma, as well as review the evidence for diseases which have been historically associated with oral cancer.
  • Chapter
    • Hepcidin is the principal iron-regulatory hormone that controls the absorption of dietary iron and its distribution between stores and extracellular fluid. • Hepcidin acts by binding to the sole known cellular iron efflux channel, ferroportin, and inducing its internalization and degradation, and thereby inhibits the efflux of iron from cells. • Hepcidin synthesis and release are induced by iron loading or inflammation and inhibited by increased erythropoietic activity or hypoxia. • Hereditary hemochromatosis is due to the deficiency of hepcidin or rarely due to resistance to the effects of hepcidin. • Increased hepcidin concentrations from inflammation, genetic diseases, or tumor production lead to iron-restricted anemia refractory to oral iron.
  • Chapter
    • Stress was considered, in general, to be immunosuppressive. • This chapter indicates that stress hormones may influence the immune response in a different way. • There is a close connection between stress, cytokines, and the function of T helper. • Glucocortecoids and catecholamines, the major stress hormones, inhibit IL-12 and increase IL-10, thus causing a switch from Th1 to Th2. • Glucocortecoids and catecholamines induced by stress inhibit IL-6 and IL-1, thus inhibiting Th17 and causing an upregulation of Treg cells. • In conclusion, we suggest that stress may in fact be anti-inflammatory and may prevent manifestation of autoimmune diseases.
  • Article
    A 40-year-old man presented with insidious onset dysphagia for both solids and liquids for 4 years with recurrent oral ulcerations. On examination he was anemic, and barium swallow demonstrated a web in the postcricoid region. As part of the workup for unexplained iron deficiency anemia, a duodenal biopsy was taken that revealed moderate flattening of villi with increased intraepithelial lymphocytes consistent with the diagnosis of celiac disease. However, the serological tests for celiac disease (IgA antiendomysial antibody, IgA antitissue transglutaminase antibody, and IgA antigliadin antibody) were all negative. Serum level of IgA was markedly low. A diagnosis of atypical celiac disease with severe selective IgA deficiency was made. After the institution of a gluten-free diet (GFD), his general condition as well as anemia improved. Histological recovery was documented on repeat duodenal biopsy 6 months after GFD.
  • Chapter
    Full-text available
    • Brain iron deficiency induced structural and functional changes in the brain. • Brain iron deficiency reduced cognitive and learning capacities in animal and human. • Iron deficiency is the most prevalent in the world. • Early iron deficiency might induce longlasting behavioral consequences despite rehabilitation of the hematological values. Key WordsBrain iron deficiency-dopamine-Shoham behavior
  • Chapter
    • Many bacteria utilize low molecular weight molecules known as siderophores to obtain scarce iron nutrient from their extracellular environment. • Siderophores and the molecular mechanisms required to synthesize, secrete, and ultimately import them are known virulence factors in many pathogenic bacteria. • Extraintestinal pathogenic Escherichia coli as well as Salmonella possess a five-gene apparatus to modify siderophores in order to overcome the host’s innate immune system. • Siderophore modification systems present an ideal target for the development of novel antimicrobial agents that could potentially result in “virulence disarmament”. • Once the iron is inside the cell, it is directed to the most critical functions, especially during iron starvation. • The small regulatory RNA RyhB reduces the cell’s requirements in iron, which allows adaptation during iron starvation.
  • Article
    Dysphagia is a common complaint of patients with Sjogren's syndrome, but its mechanism remains a subject of controversy. The association of Sjogren's syndrome with Plummer-Vinson syndrome remains uncommon. We report a 56-year-old women who presented both disorders. The diagnosis of the Plummer-Vinson syndrome was based on the classic triad of dysphagia, iron-deficiency anaemia and oesophageal webs. The diagnosis of Sjogren's syndrome was based on the presence of three Fox criteria. This association should incite us to search for common immuno-genetic pathogenic factors between these two syndromes. Copyright © 2010 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.
  • Article
    Plummer Vinson Syndrome, a triad of dysphagia, esophageal web, and iron-deficiency anemia, is a rarely reported diagnosis in current literature. The exact etiology of the syndrome remains controversial, but it has been linked to complicated nutritional deficiencies, autoimmune disorders, and hereditary factors, and has a remarkably high female to male ratio. This paper describes an atypical case presentation in a 53-year-old male with a remote history of peptic ulcer disease surgery.
  • Chapter
    • Clinical manifestations of iron deficiency were described since 1500 bc and were well known in 16th and 17th century Europe as part of the disease “chlorosis”. • The major cause of iron deficiency is blood loss, commonly from the gastro-intestinal tract in adults. • The hematologic changes characteristic of iron deficiency include hypochromic and microcytic anemia. • The tongue and mouth, hypopharynx and esophagus, stomach, nails, and hair are common epithelial tissues affected by iron deficiency. • Glossitis, cheilitis, koilonychia, dysphagia, and pica are the signs and symptoms commonly associated with iron deficiency and iron deficiency anemia.
  • Article
    Full-text available
    There is definitive evidence that iron overload induces oxidative stress and DNA damage, which can enhance carcinogenic risk. However, other evidence suggests that iron deficiency and anemia also increase oxidative stress and DNA damage, which might increase carcinogenesis risk, especially in the gastrointestinal (GI) tract. The aim of this review is to provide essential background information for the accurate interpretation of future research on iron deficiency and increased GI cancer risk. Based on clinical, epidemiological, and experimental evidence, we discuss how iron deficiency might contribute to increased cancer risk through the impairment of several iron-dependent metabolic functions that are related to genome protection and maintenance (e.g., immune responses against cancer-initiated cells, metabolism of toxic compounds, and redox regulation of DNA biosynthesis and repair). Some epidemiological studies have indicated increased risk of GI tumors among individuals with low iron intake or low somatic iron stores, and in vivo data from rodent cancer models indicates the early progression of GI tumors during iron deficiency. Given the preliminary but consistent evidence relating iron deficiency to cancer risk and the fact that iron deficiency affects about one third of the world's population, further studies are needed to define the extent to which iron deficiency might increase GI cancer risk.
  • Article
    The dramatic improvement in knowledge concerning celiac disease (CD) has disclosed the pattern of the associated clinical manifestations and the often atypical or silent presentation of this disease, which makes clinical diagnosis difficult. Also oral manifestations, mostly recurrent apthous stomatitis (RAS) and dental enamel hypoplasia, are atypical signs of CD. Our opinion about the possibility of performing mass-screening to reveal atypical or silent CD is in agreement whit who is asserting that a sistematical case-finding is, at present, the most suitable epidemiological approach. So, we think that patients affected by RAS, or dental enamel hypoplasia, should be considered, even in the absence of any gastrointestinal symptom, at-risk subjects, and should therefore undergo diagnostic procedure for CD.
  • Article
    Non-diarrheal presentation of celiac disease (CD) is being increasingly recognized. Data on this form of CD from India are limited. Consecutive patients with CD presenting to a tertiary referral center in northern India over a 3-year period were studied, with special emphasis on non-diarrheal celiac disease (NDCD). Diagnosis was based on the presence of autoantibodies typical of CD (IgA anti-tissue transglutaminase antibodies and/ or IgA endomysial antibodies), abnormal duodenal biopsy and response to gluten-free diet (GFD). Clinical, hematological and histological responses were assessed over a one-year period after instituting GFD. Of 86 patients with CD, 31 (16 children, 15 adult) had NDCD. Mean (SD) age of these children (12 boys) and adults (4 male) was 10.2 (4.2) y and 35.3 (12.0) y, respectively. Failure to thrive was the most common (11/16) presentation in children, as was refractory anemia in adults (10/15). Malabsorption was found in 8 adults (54%) and 10 children (64%) with NCCD. The duration from onset of symptoms to diagnosis was 2.9 (1.5) y in children and 3.3 (0.3) y in adults. There was significant improvement in body weight (children--baseline 18.9 [5.8] Kg, follow up 27.4 [12.4] Kg; adults--baseline 47.6 [18.2] Kg, follow up 54.9 [5.1] Kg) and hemoglobin (children--8.1 [2.0] g/dL to 11.2 [1.4] g/dL; adults--7.3 [2.3] g/dL to 9.7 [1.7] g/dL) in both groups after one year of GFD; duodenal biopsy also improved, with a majority of patients attaining normal to IIIa Marsh grading. Five adults and all children had evidence of metabolic bone disease at presentation, which did not revert completely with GFD. Eight adults and nine children showed dietary transgression 6 weeks after starting GFD. NDCD ac-counted for nearly one-third of all cases with CD at our center, with 'failure to thrive' and refractory anemia being the most common presentations in children and adults, respectively.
  • Article
    Contrary to early beliefs, celiac disease (CD) is relatively common; however, it still remains underdiagnosed since most cases are atypical, with few or no gastrointestinal symptoms and predominance of extraintestinal manifestations. As a consequence, the diagnosis of the disorder often requires a multidisciplinary approach. Also some oral ailments have been described in celiac patients. In this study, we review the papers that have reported oral manifestations in subjects with CD. A comprehensive literature search was conducted in Medline and Embase databases using appropriate key words. Additional papers were selected by cross-referencing from the retrieved articles. Dental enamel defects are the oral lesions most closely related to CD. There are conflicting data on the association between CD and recurrent aphthous stomatitis. A correlation of CD with atrophic glossitis has been reported, although robust evidence in support of it is lacking. Patients with CD have caries indexes seemingly lower than healthy individuals, but they may experience delay in tooth eruption. Occurrence of other oral mucosal lesions in CD subjects is likely occasional. Patients with systematic dental enamel defects should be screened for CD even in the absence of gastrointestinal symptoms. CD screening tests for patients with oral aphthae or idiopathic atrophic glossitis should be selectively considered during a medical evaluation that focuses on all aspects of the patient's status.
  • Chapter
    Full-text available
    • A massive expression of the transferrin receptor ensures uptake of iron by neurons in the brain. • Oligodendrocytes do not contain transferrin receptors, which brings into question whether they are less capable of extracting iron under conditions where there is a shortage of iron as compared to that of neurons. • The impaired synthesis of myelin by oligodendrocytes is the key responsible factor for impaired motor function in iron deficiency. • In conditions with iron overload in the brain, ferritin expressed by microglia, and possibly also neurons, is responsible for scavenging excess iron which causes oxidative stress and damage to brain cells. • New objectives for understanding iron homeostasis in the brain should be directed toward iron uptake and intracellular transport in oligodendrocytes to learn more about why these cells are so affected by iron deficiency. In conditions with iron overload, relevant studies should include looking at possible scavenging processes that prevent iron toxicity, e.g., do neurons raise their levels of ferritin and ferroportin to increase storage and export of iron, respectively?
  • Chapter
    • Magnetic resonance imaging (MRI) brain imaging at high field strengths can demonstrate regions of high iron deposition in the brain. • Iron deposits are present in all normal adult brains, but increased or decreased iron deposition may serve as a biomarker of brain diseases. • Several rare genetic disorders are now known which have marked increases in brain iron. The specific mutations associated with these are known, and MRI can demonstrate the abnormal iron deposition with remarkable clarity. • Brain iron may play a role in many common neurodegenerative diseases, including Alzheimer’s and Parkinson’s diseases. This may permit the use of MRI of brain iron as a biomarker for the presence and progression of these diseases. • New MRI scanners operating at very high magnetic field strengths are becoming available and permit greatly improved clarity in brain iron images.
  • Chapter
    Full-text available
    • Iron is a two-way sword. Either its brain iron deficiency (ID) or excess profoundly affects brain function. • ID can result in reduction of brain iron by roughly 35% as contrast to a 90% depletion in the liver. Thus it is tightly controlled. It is associated with impairment of cognition and learning processes which may result from alteration in dopaminergic, at the level of its receptor subsensitivity, and increased opiate neurotransmission. Other aminergic systems are not profoundly affected. • The effect of ID on brain function is age dependent, and it is more severe in newborn rats than adults and is irreversible in newborn even after long-term supplementation with iron. • The exact mechanisms by which dopamine receptors are affected by ID and their effects cognition are not well understood, but may involve dopamine interaction with the endogenous opiates, enkephalin, and dynorphins, involving the hippocampus and striatum. • One of the major findings on brain iron metabolism is its accumulation at neuronal sites which degenerate and give rise to neurodegenerative disorders such as Parkinson’s disease, Alzheimer’s disease, Huntington’s diseases. Some are familial disorders, with mutation of genes involved in iron metabolism, such as Freidreich’s ataxia, PANK2, aceruloplasminemia. • The role of iron and its accumulation in substantia nigra pars compacta of parkinsonian brains, where melanized dopamine neuron selectively degenerates, has indicated that iron participates in the Fenton reaction to induce oxidative stress-dependent damage to the neurons. • Confirmation for participation of iron in Parkinson’s disease has come from its 6-hydroxydopmaine, MPTP (N-methy-4-phenyl-1,2,3,6-tetrahydropyridine), and lactacystin neurotoxin models, where similar iron accumulation occurs in substantia nigra pars compacta and pretreatment with iron chelators are neuroprotective. Several iron chelators have been developed as neuroprotective agents for Parkinson’s disease and other neurodegenerative disorders. • It is apparent that iron accumulation may have a pivotal role in the degeneration of dopamine neurons in Parkinson’s disease. Future studies must illuminate why the process of neurodegeneration results in iron deposition and from where it is transported when it has limited access across the blood–brain barrier.
  • Chapter
    Full-text available
    • Iron is indispensable for oxidation–reduction catalysis and bioenergetics but, unless appropriately handled, may generate highly toxic reactive oxygen species. • Organisms are endowed with a panoply of proteins that mediate acquisition, export, transport and storage of the transition metal, and mechanisms that maintain the intracellular labile iron pool at physiological levels. • In humans and other organisms, inherited or acquired disruption of these highly coordinated pathways may promote illness as a consequence of tissue iron deficiency or overload. • A number of hereditary conditions perturb iron homeostasis and promote pathological deposition of the metal predominantly or exclusively within the central nervous system. • Hereditary disorders of brain iron overload include aceruloplasminemia, neuroferritinopathy, Friedreich’s ataxia, pantothenate kinase-2-associated neurodegeneration, and possibly X-linked sideroblastic anemia with ataxia in humans, and iron-regulatory protein-2 (IRP2) deficiency in mice. • Gene mutations responsible for hemochromatosis, a genetic disorder of systemic iron homeostasis, may be risk factors or modulators of common degenerative (Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis) and vascular (ischemic stroke) CNS disorders.
  • Chapter
    Understanding the anatomy and embryology of the esophagus and stomach is necessary for dealing with clinically important congenital malformations. The esophagus acts as a conduit for transport of food from the oral cavity to the stomach which, as a J-shaped dilation of the alimentary canal, connects with the duodenum distally. Sphincters at the upper esophagus, distal esophagus/proximal stomach and distal stomach have strategic functions. Formation of the esophagus (primitive foregut) begins at six weeks and the stomach is recognizable in the fourth week of gestation as a dilation of the distal foregut. Congenital abnormalities of esophagus are common and of the stomach are rare.
  • Article
    To determine if epidemiologic differences exist between patients with oral tongue carcinoma compared to tumors arising from other oral cavity subsites, and the relationship between primary site and in-hospital mortality, postoperative complications, length of stay, and costs in patients undergoing surgery for oral cavity cancer. Retrospective cross-sectional study. The Nationwide Inpatient Sample was analyzed for patients who underwent an ablative procedure for a malignant oral cavity neoplasm in 2001 to 2008 using cross-tabulations and multivariate regression modeling. Overall, there were 45,071 patients treated surgically for oral cavity cancer, with oral tongue cancer comprising 35% of all oral cavity tumors. Patients with oral tongue cancer were significantly more likely to be female (odds ratio [OR] = 1.4) and undergo neck dissection (OR = 1.4), and significantly less likely to be black (OR = 0.4), over 40 years of age (OR = 0.4), have Medicaid payer status (OR = 0.7), advanced comorbidity (OR = 0.7), receive care at a teaching hospital (OR = 0.5), and undergo pedicled or free flap reconstruction (OR = 0.6, P < .001). Oral tongue primary site was not associated with in-hospital mortality or surgical complications, but was significantly associated with a reduced incidence of medical complications (OR = 0.8, P = .005). After controlling for all other variables, oral tongue primary site disease was associated with a significantly reduced length of hospitalization and hospital-related costs. Oral tongue cancer is associated with a distinct epidemiologic profile compared to other oral cavity cancer subsites, and is associated with lower postoperative morbidity, length of hospitalization, and hospital-related costs. Further investigation is warranted to determine if biologic factors underlie these observations. 2c. Laryngoscope, 2013
  • Article
    Plummer-Vinson syndrome (PVS) comprises a triad of iron deficiency anemia, dysphagia and esophageal webs. Its occurrence in association with celiac disease which presents as iron deficiency anemia, has rarely been reported [1, 2]. We describe a 59-year-old female with PVS whose iron-deficiency anemia was due to celiac disease.
  • Article
    Full-text available
    Plummer Vinson syndrome, (also known as Patterson Kelly brown syndrome, sideropenic dysphagia, hysterical dysphagia) is a premalignant condition, in which the patient has iron deficiency anemia, dysphagia and possibly a post-cricoid web that can predispose to malignant change. The oral mucosal changes such as atrophy, dryness, stomatitis and soreness may extend to the pharynx and upper part of the esophagus, causing spasm in the throat while swallowing. Diagnosis of the esophageal webs may be made by barium swallow, and then taking the radiographs. Usually, the dysphagic condition reverts back once the iron supplementation is instituted to the patient. However in some resistant cases, dysphagia donot respond to iron replenishment and require endoscopic dilatation. Hereby, presenting an overview of literature of plummer Vinson syndrome with a mention on the carcinomatous potential of this disorder.
  • Article
    Full-text available
    Plummer-Vinson syndrome (PVS) is a triad of microcytic hypochromic anemia, atrophic glossitis, and esophageal webs or strictures. It is one of the syndromes associated with iron deficiency anemia. Symptoms resulting from anemia predominates the clinical picture, apart from the additional features such as glossitis, angular cheilitis, and dysphagia. Dysphagia is main clinical feature of PVS. PVS carries an increased risk of development of squamous cell carcinoma of esophagus and pharynx. A classic case report of PVS with clinical features, oral manifestations, malignant potential, differential diagnosis, investigation, dental implication, and treatment is discussed here with the literature review from the dentist's point of view. The article carries a message that dental surgeons have to be familiar with the oral manifestations of anemia and be able to suspect PVS to aid in early diagnosis and prompt treatment.
  • Article
    The Plummer-Vinson syndrome (PVS), also named Kelly-Paterson syndrome, is a rare cause of dysphagia in children. This syndrome associates single or multiple web(s) in the upper esophagus with a frequent iron deficiency. we reported 3 pediatric cases of PVS before analyzing all cases of PVS in children reported in PUBMED and EMBASE databases. Among 17 reported PVS cases in children, all patients suffered from iron-deficiency anemia and no immunological disease was reported. The male/female ratio was 1/1.9 and most cases were observed in adolescents. Conversely to adults, endoscopic dilation was often necessary because dysphagia resisted iron supplementation. A single dilation was usually sufficient. One case of pediatric PVS suffered from esophageal cancer in adulthood. in the case of dysphagia in children, a swallow barium exploration should look for the esophageal web characterizing PVS. Conversely to adults, an endoscopic dilation is frequently necessary to control dysphagia in children.
  • Article
    The Plummer-Vinson syndrome, called sideropenic dysphagia, is characterized by dysphagia, iron deficiency, anemia and the presence of esophageal webs, and it has been identified as a risk factor for developing squamous cell carcinoma of the upper gastrointestinal tract. The cases of patients suffering Plummer-Vinson syndrome that develops intraoral carcinomas are very rare. We present one case of 45 years-female with diagnosis of sideropenic dysphagia who develops a squamous cell carcinoma of the tongue. The patient present at Oral Medicine and Oral pathology clinic at Dental School of the Autonomous University of Ciudad Juárez, México by glossodynia, glossopyrosis and dysphagia of 8 months of evolution. The medical file revealed antecedents of non-specified chronic anemia, with blood transfusions at medical institutions. The intraoral examination showed depapilation of two anterior thirds of the tongue. An ulcerated swallow with indurate borders site in the left lateral border of the tongue of unknown evolution was observes. An incisional biopsy was done and a histopathological diagnosis of squamous cell carcinoma of the tongue was emitted. In our knowledge this is the third case reported in the scientific literature of a lingual squamous cell carcinoma develops in a patient suffering Plummer-Vinson syndrome. The 3 reported cases are coincident in age, gender and oral features. The pertinence of to continue including to sideropenic dysphagia like a risk factor to develop intra-oral carcinomas is discuses.
  • Article
    Full-text available
    The frequency of oral squamous cell carcinoma is increasing worldwide. The major risk factors are smoking and alcohol. The other etiologic factors are genetic factors, viral pathogens, nutritional habits. Oral premalignant lesions especially oral leukoplakia can transform into malignant lesions. Despite the advances in therapy, prognosis of oral cancers remains poor. Nowadays early detection of premalignant lesions and primary and secondary chemoprevention strategies of oral cancers are investigated. There were many studies about oral cancer prevention with the use of vitamins (retinoids, beta caroten, alpha tokopherol), and COX inhibitors, and its chemopreventive effects.
  • There is paucity of prospective data on association between cervical esophageal webs and celiac disease. It is not clear whether all patients with cervical esophageal web need screening for celiac disease. Hence, the present study was carried out to determine the association of cervical esophageal web with celiac disease. This prospective study included consecutive patients with symptomatic cervical esophageal web diagnosed over a period of 4.5 years. Tissue transglutaminase antibody was measured in serum of each patient. Patients with elevated tissue transglutaminase antibody titer were subjected to esophagogastroduodenoscopy and biopsies were obtained from the descending duodenum to look for histological changes of celiac disease. Esophageal web was treated with bougie dilatation. Celiac disease was diagnosed on the basis of elevated tissue transglutaminase antibody and suggestive duodenal histology. Twenty one patients were diagnosed to have cervical esophageal web. Eighteen (85.7%) had evidence of iron deficiency. Five (23.8%) patients with cervical esophageal web fulfilled criteria for diagnosis of celiac disease. All five had evidence of iron deficiency. None of these patients gave a history of chronic diarrhea. All patients were treated with bougie dilatation. Patients with celiac disease were advised of a gluten-free diet. All five celiac disease patients are free of dysphagia without recurrence after a mean follow up of 10 months (range: 3 to 16 months). There is association between cervical esophageal web and celiac disease. All adult patients with cervical esophageal web and iron deficiency need screening for celiac disease even in the absence of chronic diarrhea.
  • The immunosuppressants cyclosporin A (CsA), FK506, and rapamycin suppress the immune response by inhibiting evolutionary conserved signal transduction pathways. CsA, FK506, and rapamycin bind to their intracellular receptors, immunophilins, creating composite surfaces that block the activity of specific targets. For CsA/cyclophilin and FK506/FKBP the target is calcineurin. Because of the large surface area of interaction of the drug-immunophilin complex with calcineurin, FK506 and CsA have a specificity for their biologic targets that is equivalent to growth factor-receptor interactions. To date, all the therapeutic as well as toxic effects of these drugs have been shown to be due to inhibition of calcineurin. Inhibition of the action of calcineurin results in a complete block in the translocation of the cytosolic component of the nuclear factor of activated T cells (NF-AT), resulting in a failure to activate the genes regulated by the NF-AT transcription factor. These genes include those required for B-cell help such as interleukin (IL-4) and CD40 ligand as well as those necessary for T-cell proliferation such as IL-2. The purpose of this article is to illustrate the means by which these drugs produce immunosuppression.
  • Article
    In this paper, we review the histological features of coeliac disease and propose a standardized report scheme based on the Marsh classification. Furthermore, terms used by pathologists are defined. The most important histological differential diagnoses are given, as well as a definition of the different clinical forms of coeliac disease such as symptomatic, silent, latent, potential, treated and refractory coeliac disease.
  • Article
    Three cases of autoimmune hemolytic anemia in association with idiopathic ulcerative colitis are reported; the literature is reviewed; and therapeutic modalities are considered. Successful treatment is shown to have resulted with steroids alone, steroids and immunosuppressives, splenectomy, and colectomy. It is concluded that moderate or severe hemolysis should be treated first with high-dose corticosteriods; if unsuccessful, immunosuppressive therapy may be added or a splenectomy performed. Finally, total colectomy should be reserved for fulminant colitis and its complications and is not indicated solely for hemolysis.
  • Article
    From a review of 202 patients with adult coeliac disease (ACD) or idiopathic steatorrhoea (IS) evidence is given that both of these disorders can be complicated by malignancy, either lymphoma or carcinoma of the gastrointestinal tract (especially of the oesophagus). The incidence is highly significant in men, and significant in women. The mean duration of symptoms of coeliac disease prior to the diagnosis of lymphoma was 21.2 years, and of carcinoma of the gastrointestinal tract 38.5 years. A gluten-free diet appeared to decrease the risk of malignant complication.
  • Article
    A 45-year-old female with a long history of HLA-B27-positive ankylosing spondylitis and ulcerative colitis developed cyclic neutropenia. She was hospitalized for high fever during each of three consecutive episodes of absolute neutropenia. On the third hospitalization, granulocyte-colony-stimulating factor (G-CSF), 5 micrograms/kg/day, was given by subcutaneous injection and resulted in an increase of absolute neutrophil count from 0 to 2.2 x 10(9)/liter and an associated decrease of platelet count and hemoglobin as well as severe bone and joint pain predominantly in the middle and lower back and purulent diarrhea. The back pain necessitated discontinuation of the drug. Oral cyclosporine therapy was begun, and although the neutrophil count continued to oscillate, both the peaks and the nadirs were higher than previously, and symptoms of neutropenia subsided. We conclude that cyclosporine can be an effective treatment for cyclic neutropenia associated with autoimmunity since G-CSF may cause exacerbations of autoimmune disorders.
  • Article
    Out of the 215 regularly controlled patients Coombs positive anaemia developed in two during the course of ulcerative colitis. Hemolysis was manifested clinically only in one of the two cases. Steroid therapy was effective in the first and combined with azathioprine in the second case. Their experiences with the simultaneous appearance of these diseases are being discussed on the bases of previous data.
  • Article
    To estimate the frequency of autoimmune hemolytic anemia and Coombs positivity without overt hemolysis in ulcerative colitis, to determine possible subsets of patients with ulcerative colitis susceptible to this complication, and to assess the efficacy of the applied therapeutic modalities. Three hundred and two patients with ulcerative colitis treated at the University Hospital of Heraklion, Crete, over a 6-yr period were included. Within this group, a subgroup of 152 patients were studied prospectively for the presence of a positive direct Coombs test. Autoimmune hemolytic anemia was diagnosed in five of 302 patients with ulcerative colitis (1.7%). One more patient developed Coombs-positive hemolytic anemia, attributed to sulfasalazine. A positive Coombs test without evidence of hemolysis was found in three of 152 patients (2%). The mean age of all Coombs-positive patients was 50.5 yr, and there was a definitive male preponderance (male: female, 2:1). Autoimmune hemolytic anemia occurred during active colitis in all cases. The mean time between the onset of colitis and the diagnosis of autoimmune hemolytic anemia was 17 months. Three of five patients with autoimmune hemolytic anemia (60%) and seven of nine of all Coombs-positive patients (77.7%) had total colitis. All patients with autoimmune hemolytic anemia were treated initially with large doses of corticosteroids. Three of five (60%) had good hematological responses. One patient responded to the addition of azathioprine, and one underwent splenectomy and proctocolectomy. In this study, the frequency with which autoimmune hemolytic anemia was associated with ulcerative colitis was higher than in previous reports. The complication occurred early in the course of colitis and was related to activity and extent of the disease. In contrast to others studies, we found a preponderance of males. Although corticosteroids and/or immunosuppressive therapy was successful in most of our cases, one patient required surgery.
  • Article
    We describe two patients with Paterson-Brown Kelly (Plummer-Vinson) syndrome whose iron deficiency anemia was due to celiac disease. They presented with dysphagia 13 and 9 yr, respectively, before celiac disease was diagnosed. Neither had gastrointestinal symptoms suggestive of malabsorption. Celiac disease is a recognized cause of chronic iron deficiency and should be considered as an etiological factor for sideropenic dysphagia.
  • Article
    Cyclosporin (CSA) is a promising alternative for patients with severe steroid-refractory ulcerative colitis (UC) previously facing only surgical options. Concerns over the long term efficacy and side effects resulted in this investigation of the University of Chicago's 5-yr CSA experience in these patients. All steroid-refractory severe ulcerative colitis (UC) patients treated with IV CSA from 1991 to 1995 were identified by using the university's IBD database, with additional information from patient charts and physician files. A total of 42 patients with severe UC unresponsive to IV steroids were treated with IV CSA (4 mg/kg/day). Of 42 patients, 36 (86%) responded; 31 were continued on oral CSA (8 mg/kg/day) for an overall mean of 20 wk. Ten initial CSA responders had colectomies after a mean of 6 months. Of the 36 initial responders, 25 (69%) also received 6-mercaptopurine (6-MP) or azathioprine (aza), and CSA and steroids were tapered. A total of 20% required colectomy, vs 45% of those not receiving 6MP/aza. In all, 62% of all patients, 72% of initial CSA responders, and 80% of initial CSA responders receiving 6MP/aza have avoided colectomy, with a life table analysis of "noncolectomy survival" of 58%, 70%, and 71%, respectively, at 5.5 yr. All colectomies occurred within 18 months of CSA initiation. Complications, resulting in CSA discontinuation in six patients, were all reversible, with complete recovery. CSA successfully allows most severe steroid resistant UC patients to retain their colons, and provides time for "elective" colectomy in others, especially if 6MP/aza are also given. Careful monitoring for side effects, including PCP prophylaxis, should be part of the treatment protocol.
  • Article
    The American Journal of Gastroenterology is published by Nature Publishing Group (NPG) on behalf of the American College of Gastroenterology (ACG). Ranked the #1 clinical journal covering gastroenterology and hepatology*, The American Journal of Gastroenterology (AJG) provides practical and professional support for clinicians dealing with the gastroenterological disorders seen most often in patients. Published with practicing clinicians in mind, the journal aims to be easily accessible, organizing its content by topic, both online and in print. www.amjgastro.com, *2007 Journal Citation Report (Thomson Reuters, 2008)
  • Article
    In this paper, we review the histological features of coeliac disease and propose a standardized report scheme based on the Marsh classification. Furthermore, terms used by pathologists are defined. The most important histological differential diagnoses are given, as well as a definition of the different clinical forms of coeliac disease such as symptomatic, silent, latent, potential, treated and refractory coeliac disease, Eur J Gastroenterol Hepatol 11:1185-1194 (C) 1999 Lippincott Williams & Wilkins.
  • Article
    Cyclosporine has been effective in patients with steroid-refractory attacks of ulcerative colitis (UC). We investigated the effects of intravenous (IV) cyclosporine as single IV therapy (without glucocorticosteroids) for severe UC and compared these with the response to glucocorticosteroids. Patients with a severe attack of UC were randomized to treatment with IV cyclosporine, 4 mg x kg(-1) x day(-1), or with methylprednisolone, 40 mg/day, in a randomized, double-blind, controlled trial. After 8 days, patients who had a response received the same medication orally in combination with azathioprine. Patients were followed up clinically, endoscopically, and by scintigraphy. Renal function was assessed using urinary inulin clearances. Endpoints were clinical improvement, discharge from the hospital, and remission up to 12 months after intravenous therapy. Thirty patients were included. After 8 days, 8 of 15 patients (53%) who received methylprednisolone had a response to therapy vs. 9 of 14 (64%) receiving cyclosporine. In nonresponders, 3 of 7 methylprednisolone patients and 1 of 3 cyclosporine patients improved when both treatments were combined. No serious drug-related toxicity was observed with either treatment. At 12 months, 7 of 9 patients (78%) initially controlled with cyclosporine maintained their remission vs. 3 of 8 (37%) initially treated with methylprednisolone. No clinically significant decrease of renal function was observed. Cyclosporine monotherapy is an effective and safe alternative to glucocorticosteroids in patients with severe attacks of UC.
  • Article
    To assess the efficacy of oral microemulsion cyclosporine (CyA Neoral) in the treatment of steroid-refractory attacks of ulcerative an indeterminate colitis. In this non-randomized study on the use of oral microemulsion cyclosporine in steroid refractory ulcerative colitis, we used CyA Neoral in 10 patients suffering from ulcerative colitis and in 1 patient suffering from indeterminate colitis with a steroid-refractory attack. The initial dose was 7-7.5 mg kg-1 day-1 adjusted to maintain whole blood cyclosporine levels between 250 and 350 ng/mL, as measured by RIA using monoclonal antibodies. Nine patients presented a favourable response in a mean time of 3.6 days, that is, 81.8% of cases. One initial responder developed megacolon on the 11th day and another did not respond; surgical treatment was performed in both cases. The remaining nine patients, followed for a mean period of 14.6 months (2-36 months). Nine patients presented side effects, the most frequent being slight hand tremor with hypomagnesaemia, followed by hypertension, slight increase in creatinine and hirsutism. No one needed to withdraw from treatment, but the dose was lowered in three cases. Oral microemulsion cyclosporine is an effective drug in the initial management of patients suffering from a steroid-refractory attack of ulcerative and indeterminate colitis. Additional controlled studies with the new oral formulation are required to confirm these results.
  • Article
    Although previous studies have shown increased mortality in patients with coeliac disease and their relatives, no data are available in relation to different patterns of clinical presentation. We assessed mortality in patients with coeliac disease and their first-degree relatives. We enrolled, in a prospective cohort study, 1072 adult patients with coeliac disease consecutively diagnosed in 11 gastroenterology units between 1962 and 1994, and their 3384 first-degree relatives. We compared the number of deaths up to 1998 with expected deaths and expressed the comparison as standardised mortality ratio (SMR) and relative survival ratio. 53 coeliac patients died compared with 25.9 expected deaths (SMR 2.0 [95% CI 1.5-2.7]). A significant excess of mortality was evident during the first 3 years after diagnosis of coeliac disease and in patients who presented with malabsorption symptoms (2.5 [1.8-3.4]), but not in those diagnosed because of minor symptoms (1.1 [0.5-2.2]) or because of antibody screening (1.2 [0.1-7.0]). SMR increased with increasing delay in diagnosis and for patients with poor compliance with gluten-free diet. Non-Hodgkin lymphoma was the main cause of death. No excess of deaths was recorded in relatives with coeliac disease. Prompt and strict dietary treatment decreases mortality in coeliac patients. Prospective studies are needed to clarify the progression of mild or symptomless coeliac disease and its relation to intestinal lymphoma.