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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Verrucous carcinoma (VC) of the esophagus is a rare variant of squamous cell carcinoma and only 20 cases have so far been reported in the international literature. The neoplasm is usually highly differentiated, presents a slow growth pattern with invasion of surrounding organs rather than blood-borne metastases. Recently, a causative role of human papillomavirus (HPV) has been hypothesized. The case of a patient affected with locally advanced VC of the esophagus and treated by means of local antiviral therapy is reported. A 41-year-old male patient was admitted to our institution for persistent atypical thoracic pain. The imaging techniques (thoracic and abdominal CT scans; upper GI endoscopy; hydrosoluble contrast swallow; endoscopic US) revealed a cauliflower-like protruding esophageal mass, active mucosal mycosis, multiple ulcerations of the distal esophagus, as well as 2 broncho-esophageal fistulas. The neoplasm extended beyond the esophageal wall, infiltrating surrounding cervical and mediastinal organs and the patient presented with secondary esophageal achalasia and right bilobar pneumonia. The histologic specimen was consistent with VC of the esophagus and the presence of HPV infection was detected by means of qualitative PCR assay. The patient was deemed not fit for surgery and a local antiviral treatment with hydroxy-phosphonyl-methoxypropyl-cytosine 5 mg/kg a week was started. After initial response to treatment, the patient presented with sudden progression leading to further broncho-esophageal fistula treated with endoscopic stent placement and ultimate death 6 months after referral to our center. In keeping with international data, our case confirms that esophageal VC has a highly unfavorable outcome, despite its high degree of differentiation and slow growth pattern. The long natural history, the lack of specific symptoms and the presence of coexisting esophageal diseases delay the diagnosis and account for the local advancement of this malignancy. Surgery is the option of choice for early stage lesions and advanced VC does not seem to benefit from current chemotherapeutic regimens. The causative role of HPV and the advancements of molecular pharmacology might allow for effective treatment in high-risk patients.
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PMID:[Verrucous carcinoma of the esophagus. A case report]. 1590 55

Ten clinical cases of neuromuscular dysplasia of the ureter (NMDU) are reported. Eight patients were young (24-38 years), two--of the middle age (41-58 years). NMDU was bilateral in two patients. Ureteral achalasia of the congenital solitary kidney occured in one case. One 28-year-old female with megaureter of the solitary kidney had interstitial cystitis. Clinical picture of the disease was characterized primarily with acute pyelonephritis, pain and secondary urolithiasis. Surgical treatment consisted in resection of the affected part of the ureter with modeling of the lumen of the latter on the drainage and Boari plastic repair. Bilateral Boari operation was made in 2 patients. In one case of ureteral achalasia and ureterocele direct ureterocystoanastomosis was created with good result. Sigmocystoplasty with transplantation of the solitary kidney ureter into the intestinal transplant was made in the patient with scar contracture of the detrusor and megaureter. Functional result of the operation was good. Complications were registered in 4 patients, 2 of which were reoperated. In nine patients of ten good and satisfactory functional results were obtained.
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PMID:[Surgical treatment of neuromuscular dysplasia of the ureter]. 1609 12

When patients with the typical reflux symptoms of heartburn, regurgitation, or both, undergo endoscopy, up to 75% will not have endoscopic oesophagitis or evidence of Barrett's oesophagus. These patients have been described as having endoscopic negative or, more commonly, non-erosive reflux disease (NERD). Patients without oesophagitis, but with a positive pH test, can be diagnosed with gastro-oesophageal reflux disease (GERD). Some experts also consider a response to proton pump inhibitor therapy as proof of GERD in a patient with the correct symptoms and a negative endoscopy. Patients with normal acid exposure, but who report symptoms with a majority of their reflux episodes documented during an ambulatory pH study, have also been considered to have NERD, although others have labelled them as having 'functional heartburn'. Finally, there are some patients who have reflux symptoms and respond to reflux therapy, but have no demonstrable reflux by either endoscopy or ambulatory reflux testing. Whether these patients are part of the GERD spectrum or have another diagnosis is not clear. It seems that the most widely used definition of functional disease (the Rome II criteria) would include these patients as having functional heartburn, as it was defined as 'greater than or equal to 12 weeks of either continuous or intermittent symptoms of burning retrosternal discomfort or pain without pathologic GERD, achalasia, or other motility disorders with a recognized pathologic basis'. This article reviews potential differences in pathophysiology between erosive oesophagitis and NERD; explores whether symptoms can help distinguish NERD patients from erosive oesophagitis patients; and explores the evaluation and therapy of these patients.
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PMID:Review article: the role of acid suppression in patients with non-erosive reflux disease or functional heartburn. 1648 68

The immense success of laparoscopic surgery as an effective treatment of gastroesophageal reflux disease (GERD) and achalasia has established minimal invasive surgery as the gold standard for these two conditions with lower morbidity and mortality, shorter hospital stay, faster convalescence, and less postoperative pain. One controversy in the treatment of GERD evolves around laparoscopic antireflux surgery (LARS) as the preferred treatment for Barrett's esophagus and the procedure's potential to reduce the risk of adenocarcinoma of the esophagus. GERD has also been associated with respiratory symptoms, asthma and laryngeal injury, and a second controversy prompts discussions about whether total or partial fundoplication is the more appropriate treatment for GERD. A new and promising alternative in the treatment of GERD is endoluminal therapy. Three types of this new treatment option will be discussed: radiofrequency energy delivered to the lower esophageal sphincter, the creation of a mechanical barrier at the gastroesophageal junction, and the direct endoscopic tightening of the lower esophageal sphincter. Laparoscopic surgery is discussed not only as a very effective treatment for GERD but also as permanent cure for achalasia. This review analyzes the three most important treatment options for achalasia: medications, pneumatic dilatation, and surgical therapy. Medications as the only true non-invasive option in the treatment of achalasia are not as effective as LARS because of their short half-life and variable absorption due to the poor esophageal emptying. The second treatment option, pneumatic dilatation, involves the stretching of the lower esophagus and is still considered the most effective non-surgical treatment for achalasia. Finally, surgical therapy for achalasia and the two major controversies concerning this laparoscopic treatment are discussed. The first involves the extent to which the myotomy is extended onto the stomach, and the second concerns the necessity and type of antireflux procedure to prevent GERD after myotomy. LARS and laparoscopic Heller myotomy are the agreed upon as the gold standards for surgical treatment of GERD and achalasia, respectively. In the hands of an experienced laparoscopic surgeon both are safe and effective treatments for patients with excellent subjective and objective long-term results with at least 90% patient satisfaction.
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PMID:Controversies in the treatment of gastroesophageal reflux and achalasia. 1671 33

Achalasia is a rare motor disorder of the oesophagus, characterised by the absence of peristalsis and impaired swallow-induced relaxation. These motor abnormalities result in stasis of ingested food in the oesophagus, leading to clinical symptoms, such as dysphagia, regurgitation of food, retrosternal pain and weight loss. Although it is well demonstrated that loss of myenteric oesophageal neurons is the underlying problem, it still remains unclear why these neurons are preferentially attacked and destroyed by the immune system. This limited insight into pathophysiology explains the fact that treatment is limited to interventions aimed at reducing the pressure of the lower oesophageal sphincter. The most successful therapies are clearly pneumatic dilatation and Heller myotomy with short-term success rates of 70-90%, declining to 50-65% after more than 15 years. The challenge for the coming years will undoubtedly be to get more insight into the underlying disease mechanisms and to develop a treatment to restore function.
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PMID:Achalasia. 1764 3

A wide spectrum of gastrointestinal motility disorders present in childhood. Some are unique to children, especially congenital disorders including certain pseudo-obstruction disorders or those associated with anatomic developmental defects, whereas others are common adult disorders, such as achalasia, that rarely manifest in children. This article reviews the pediatric presentations and sequelae of childhood gastrointestinal motility disorders and then discusses long-term management issues for these children as they progress into adulthood. The goal is to optimize medical care and ensure the adequate nutritional status essential for neurocognitive and psychosocial development of the child. Multidisciplinary care from specialists, including gastroenterologists, psychologists, and pain specialists, is often required to optimize the lives of these patients.
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PMID:Gastrointestinal motility disorders in adolescent patients: transitioning to adult care. 1795 Apr 47

Botulinum neurotoxin (BoNT), for more than a hundred years, has been a recognized poisonous principle in spoiled food. As its chemical structure became unraveled, and as more knowledge was gained over its mechanism of toxicity, it became clear that BoNT had the potential to act therapeutically as a targeted toxin that could inactivate specific nerve populations, and thus achieve a therapeutic goal. BoNT has evolved over the past 25 years into a viable therapeutic, now being a first line treatment for dystonia, overtly altering the course of progression of this disorder. BoNT is used for hyperhidrosis and gustatory sweating syndrome, alleviation of pain, as a treatment for overactive bladder, achalasia and anal fissure; and it has gained popularity as a cosmetic aid. Many other possible uses are being explored. The greatest potential for BoNT may lie in its being a molecular Trojan Horse - able to carry a specific enzyme or specific drug to the inside of a cancer or other type of cell while bypassing other cells and thereby having little or no ill effect. BoNT's pharmaceutical potential is boundless.
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PMID:Botulinum neurotoxin: evolution from poison, to research tool--onto medicinal therapeutic and future pharmaceutical panacea. 1820 55

Achalasia is a rare motor disorder of the esophagus, characterized by the absence of peristalsis and impaired swallow-induced relaxation. These motor abnormalities result in stasis of ingested food in the esophagus, leading to clinical symptoms, such as dysphagia, regurgitation of food, retrosternal pain and weight loss. Etiology is unknown. Some familial cases have been reported, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor. Association of achalasia with viral infections and auto-antibodies against myenteric plexus has been reported, but the causal relationship remains unclear. In terms of diagnosis, esophageal manometry is the gold standard to diagnose achalasia. Still, its role in post-treatment surveillance remains controversial. Radiological studies support the initial diagnosis of achalasia and have been proposed for detecting preclinical symptomatic recurrence. Although endoscopy is considered to have a poor sensitivity and specificity in the diagnosis of achalasia, it has an important role in ruling out secondary causes of achalasia. Treatment is strictly palliative. Current medical and surgical therapeutic options (pneumatic dilation, surgical myotomy, and pharmacologic agents) aimed at reducing the lower esophageal sphincter (LES) pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids.
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PMID:Achalasia. 1861 76

Megaesophagus is the end-stage of achalasia cardiae. It is the result of peristaltic disorders and slow decompensation of the muscular layer of the esophagus. The aim of this article is to detail the diagnostic criteria and surgical management of megaesophagus. Criteria were acute bending of esophagus axis; lack of esophagus peristalsis, and no response to stimulation in the manometric test; and Los Angeles C/D esophagitis in the endoscopic examination. Between 1991 and 2004 seven patients (5 females, 2 males; age, 51-67 years; average age, 59 +/- 8 years) were treated. A bypass made from the pedunculated part of the jejunum connecting the part of esophagus above the narrowing with the praepyloric part of the stomach was made. Access was by an abdominal approach. A jejunum bypass was made in six patients with megaesophagus. A transhiatal esophageal resection was carried out, and in the second stage a supplementary esophagus was made from the right half of the colon on the ileocolic vessels in one patient who had experienced two earlier unsuccessful operations. Symptoms of dysphagia, recurrent inflammation of the respiratory tract, and pain subsided in all patients. Complications were not reported in the postoperative period. All patients survived. Subsequent radiographic and endoscopic examination showed very good outcome. The jejunum bypass gave very good results in the surgical treatment of megaesophagus.
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PMID:Diagnostic criteria and surgical procedure for megaesophagus--a personal experience. 1920 50

Difficulty in swallowing, i.e. dysphagia should be distinguished from the sensation of a lump in the throat and the pain on swallowing. A careful anamnesis will help in determining the ailment as a problem of oral, pharyngeal or esophageal stage of swallowing. Videofluorography, FEES investigation and transnasal esophagoscopy as well as gastroscopy are helpful for the diagnosis. Cerebral infarction is the most significant cause of oropharyngeal dysphagia. Esophageal causes include reflux disease, tumors and achalasia. Diagnostics, treatment and rehabilitation of dysphagia patients require multidisciplinary collaboration. In addition, surgical therapy may be required.
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PMID:[Dysphagia--a multidisciplinary challenge]. 1971 76


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