Congenital myasthenic syndrome associated with paucity of synaptic vesicles and reduced quantal release.
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Acetylcholine↗
Biomedical subjects
V F Trastek
Publications and source records attributed to V F Trastek.
Congenital myasthenic syndrome attributed to an abnormal interaction of acetylcholine with its receptor.
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Acetylcholine↗
Epiphrenic diverticulum: results of surgical treatment.
From 1975 to 1991, 112 patients (64 men and 48 women) were found to have an epiphrenic diverticulum. Symptoms were absent or minimal in 71 patients and incapacitating in 41. All patients with minimal symptoms were managed conservatively; 35 were available for follow-up, which ranged from 1 to 25 years (median, 9 years). None of these 35 patients had clinically significant progression of symptoms. Surgical repair was done in 33 patients with incapacitating symptoms. Achalasia was present in 8 of the surgical patients (24.2%), diffuse esophageal spasm in 3 (9.1%), hypertensive lower esophageal sphincter alone in 1 (3.0%), and nonspecific motor abnormalities of the esophageal body in 7 (21.2%). Diverticulectomy and esophagomyotomy were performed in 22 patients, diverticulectomy alone in 7, esophageal resection in 3, and esophagomyotomy alone in 1. Concomitant hiatal hernia repair was done in 6 patients. Complications occurred in 11 patients; 6 had esophageal leaks. There were three operative deaths (9.1%), all occurring in patients with abnormal manometry. Follow-up was complete in 29 patients and ranged from 4 months to 15 years (median, 6.9 years). Long-term results were excellent in 14 patients (48.2%), good in 8 (27.6%), fair in 5 (17.2%), and poor in 2 (6.9%). We conclude that operation has significant risks and is not warranted in patients with minimal symptoms because progression is unlikely. Surgical treatment, however, is advisable in patients with incapacitating symptoms because most operative survivors will have long-term symptomatic palliation.
Adult↗
Transhiatal esophagectomy for carcinoma of the esophagus.
One hundred thirty-one patients (107 men and 24 women) underwent transhiatal esophagectomy for carcinoma of the esophagus. Median age was 65.3 years (range, 30 to 89 years). Signs and symptoms were present in 130 patients, which included dysphagia in 96 (73.3%) and weight loss (median, 7.7 kg) in 52 (39.7%). The cancer involved the gastroesophageal junction in 94 patients, the lower half of the intrathoracic esophagus in 25, the upper half in 10, and multiple sites in 2. An adenocarcinoma was present in 101 patients (77.1%), squamous cell carcinoma in 29 (22.1%), and adenosquamous cell in 1 (0.8%). The cancer was classified as stage 0 in 4 patients, stage I in 16, stage IIA in 26, stage IIB in 18, stage III in 65, and stage IV in 1. The stomach was used to replace the esophagus in all patients. Operative mortality was 2.3%. Anastomotic leak developed in 32 patients; 6 leaks were not clinically significant, 12 healed with drainage alone, and 14 required further surgical intervention. Follow-up ranged from 1 month to 6.7 years (median, 1.4 years). Currently, 42 patients are alive, 34 without evidence of recurrence. Overall 5-year survival was 20.8% and varied according to stage. Five-year survival was 47.5% for patients with stage I disease compared with 37.7% for patients in stage II and only 5.8% 4-year survival for patients in stage III. Cell type also influenced survival. Five-year survival for patients with adenocarcinoma was 27.1% compared with zero for patients with squamous cell carcinoma (p < 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)
Adenocarcinoma↗
Equipment for thoracoscopy.
The combining of miniaturized video technology with thoracoscopy now allows surgeons to perform a variety of thoracic procedures percutaneously. Both rigid and flexible video thoracoscopes are available. The rigid endoscope has a camera located proximally at the eye-piece and is capable of excellent resolution. However, visualization of the entire pleural cavity is difficult because of the rigid chest wall. Placing the video camera at the distal end of a flexible thoracoscope, as in the electronic video thoracoscope (EVE-L; Fujinon, Wayne, NJ), yields better visualization of these relatively inaccessible areas. However, disadvantages of the flexible thoracoscope include increased expense and complexity, reduced resolution as compared to rigid systems, and the need for a strobed light source, thus making video-assisted surgery more difficult. Thoracoscopic wedge excisions of the lung are now possible because of the adaptation of gastrointestinal staplers for percutaneous use. The initial design consisted of a reloadable 30-mm disposable stapler. Newer models, however, have a longer staple line and some are reusable. Future refinements may allow the head of the instrument to articulate, thus permitting it to be applied to the lung at various angles. Thoracoscopic ports that provide an air-tight seal are available but are not essential; therefore, standard thoracotomy instruments can be utilized through small open incisions. Specialized disposable thoracoscopic instruments are also available, including scissors, dissectors, and fan retractors. It is hoped that the future will bring improved optics, better staplers, and refined percutaneous instrumentation.
Humans↗
Influence of pancreatic and biliary reflux on the development of esophageal carcinoma.
We previously presented an experimental model of Barrett's adenocarcinoma of the esophagus by demonstrating that esophagojejunostomy combined with subcutaneous injection of 2,6-dimethylnitrosomorpholine in Sprague-Dawley rats resulted in development of adenocarcinoma in the distal esophagus. The present study was devised to investigate the influence of pancreatic and biliary duodenal-content reflux on the induction of esophageal carcinoma. Three groups of 8-week-old Sprague-Dawley rats were controls: the first was exposed to pancreatic reflux, the second to biliary reflux, and the third to both. The other three experimental groups were similar except that a 1/100 LD50 dose of 2,6-dimethylnitrosomorpholine was injected subcutaneously weekly, starting on day 15. Carcinoma of the esophagus was induced only in animals receiving the carcinogen after exposure to either pancreatic reflux (3/22, 13%) or pancreatic and biliary reflux (9/27, 33%). Half of the carcinomas were adenocarcinoma and half were squamous cell carcinoma. These findings suggest that under these experimental conditions, in which the carcinogen is used in a low dose, esophageal carcinoma is induced only when pancreatic secretions are present in the duodenal-content reflux. Biliary reflux, however, appears to exert a cocarcinogenic effect when combined with pancreatic secretions. The clinical relevance of these findings needs further evaluation. Conceivably, the elimination of pancreatic and biliary duodenal-content reflux in patients with documented Barrett's mucosa may inhibit the progression from metaplasia to adenocarcinoma.
Adenocarcinoma↗
Barrett's disease: pathophysiology of metaplasia and adenocarcinoma.
Peptic ulceration arising in the lower esophagus lined by columnar epithelium was described in detail by Tileston in 1906. Although this concept was challenged in 1950 by Barrett, experimental and clinical evidence has now conclusively demonstrated that Barrett's metaplasia is an acquired condition and is a consequence of chronic reflux of gastric or duodenal contents or both. Current concepts suggest that unknown trophic factors present in these secretions stimulate proliferation of multipotential reserve cells located in the esophageal submucosal glands resulting in columnar metaplasia of the normal squamous epithelium with subsequent potential for malignant degeneration. Today, numerous patients are affected by reflux esophagitis, a lesser number by Barrett's metaplasia, and a smaller but ever-enlarging group by adenocarcinoma. Although high-grade dysplasia is considered a precursor to invasive adenocarcinoma, detection of this abnormal mucosa remains controversial and currently requires esophagoscopy with biopsy. Epithelial markers, such as increased activity of mucosal ornithine decarboxylase, sulfomucin production, nuclear DNA aneuploidy, and recently molecular analysis, have also been proposed to identify those paitents at increased risk for malignant degeneration. As more is learned about the pathogenesis of Barrett's disease, perhaps these cancers can ultimately be prevented.
Adenocarcinoma↗
Increasing incidence of adenocarcinoma of the esophagus and esophagogastric junction.
BACKGROUND: The aim of this study was to determine whether the incidence of adenocarcinoma of the esophagus and esophagogastric junction in a well-defined population was higher than previously recognized. METHODS: Clinical records and original histological slides from patients residing in Olmsted County, Minnesota, were reviewed and compared with a previous study in the same population. RESULTS: The incidence of esophageal adenocarcinoma rose from 0.13 for 1935-1971 to 0.74 for 1974-1989, and the incidence of adenocarcinoma of the esophagogastric junction rose from 0.25 to 1.34 per 100,000 person-years. Histological review of preserved surgical specimens showed associated intestinal metaplasia (Barrett's esophagus) in 2 of 2 esophageal and in 5 of 9 esophagogastric adenocarcinomas. CONCLUSIONS: The incidence of adenocarcinoma in each location increased five to sixfold compared with the earlier study. This increase could not be explained by improved diagnostic methods or classification changes. The association with Barrett's esophagus and the parallel increased incidence of cancer in each location is evidence that adenocarcinoma of the esophagus and of the esophagogastric junction are related disorders.
Adenocarcinoma↗
Staging systems of lung cancer.
The staging of lung cancer involves assessment of the anatomic extent of disease based on the best available data. Such a definition of neoplastic burden facilitates the systematic analysis and meaningful communication of diagnostic, therapeutic, and prognostic information. Clinical staging involves the best estimate of extent of disease before performance of surgical resection or biopsy procedures (or both). Surgical-pathologic staging is based on the histopathologic analysis of resected specimens, including determining the extent of local and regional disease. During the past 50 years, two major classification schemes for staging of lung cancer have evolved--one for non-small-cell lung cancers (the TNM system, indicating the status of primary tumor [T], regional lymph node [N], and metastatic [M] involvement) and the other for small-cell carcinoma of the lung (based on limited versus extensive disease). In this report, we review the evolution of the current staging systems used for primary lung cancer and their prognostic implications.
Carcinoma, Non-Small-Cell Lung↗
Achalasia: prospective evaluation of relationship between lower esophageal sphincter pressure, esophageal transit, and esophageal diameter and symptoms in response to pneumatic dilation.
The aims of this study were to investigate a group of patients with achalasia prospectively to determine (1) the relationship between changes in symptoms and esophageal motor function in response to pneumatic dilation and (2) the effects of the balloon size as well as the frequency and duration of inflation on the outcome of treatment. Fourteen patients with achalasia who were symptomatic for a median duration of 27 months participated in the study. The patients were randomized to one combination of the following pneumatic dilation conditions: a 30- or 35-mm balloon dilator, one or two balloon inflations, and 20, 40, or 60 seconds per balloon inflation. A comprehensive assessment of their symptoms and esophageal motility, transit, and diameter were performed before and 3 months after pneumatic dilation. Pneumatic dilation provided significant relief of dysphagia (P < 0.01), but other symptoms (heartburn, regurgitation, and chest pain) remained unchanged. Pneumatic dilation also caused a significant decrease in lower esophageal sphincter pressure and esophageal diameter and improved esophageal emptying of a solid bolus. Nevertheless, no significant association was detected between changes in the symptom score for dysphagia and changes in objective response measures as a result of pneumatic dilation. Changes in the symptom score for dysphagia or objective responses were similar regardless of the size of the dilator used or the frequency and duration of the balloon inflations.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Video-assisted thoracoscopic stapled wedge excision for indeterminate pulmonary nodules.
Between June 1991 and July 1992, 118 patients (57 men and 61 women) underwent video-assisted thoracoscopy for indeterminate pulmonary nodules. Median age was 64 years (range 30 to 85 years). Thoracotomy was performed in 33 patients (28.0%) after thoracoscopy only because the nodule could not be located in 17 patients, was too large to safely resect in 5, appeared malignant in 4, and for technical reasons in 7. Eighty-five patients underwent thoracoscopic wedge excision. Twenty-one (24.7%) of these 85 patients also had thoracotomy--15 to perform formal lung resection for bronchogenic carcinoma, 3 for nondiagnostic abnormalities, 2 to locate a second nodule, and 1 for stapler malfunction. The remaining 64 patients (54.2%) had only video-assisted thoracoscopic wedge excision. A single wedge excision was performed in 56 patients, two in 6, and three in 2. Pathologic examination of these 74 nodules revealed a granuloma in 30, metastatic cancer in 25, hamartoma in 7, lymphoma in 1, and other benign lesions in 11. There were no deaths and only 4 (6.3%) complications in these 64 patients. The 64 patients treated by thoracoscopy only were compared with a similar group of 64 patients who had wedge excision via thoracotomy without prior thoracoscopy. Postoperative analgesic requirements were less in the patients treated by thoracoscopy. Median hospitalization in the thoracoscopy group was 3 days compared with 6 days in the thoracotomy group (p < 0.05). Median total charge for the thoracoscopy-only group was $12,898 as compared with $12,502 for patients undergoing wedge excision via thoracotomy. We conclude that thoracoscopic wedge excision is a safe and effective procedure in selected patients with an indeterminate pulmonary nodule. A significant number of patients (45.8%), however, required a thoracotomy to accomplish a safe operation or to ensure adequate staging and resection for malignancy. Although thoracoscopy reduces postoperative analgesia requirements and shortens hospital stay, total hospital charges were similar to charges for a wedge excision via thoracotomy.
Adult↗
Intrathoracic stomach. Presentation and results of operation.
Between January 1, 1980, and December 31, 1990, 147 patients (93 female and 54 male) were found to have an intrathoracic stomach. Median age was 69 years (range 34 to 89). Signs and symptoms occurred in 140 patients (95.2%) and were primarily obstructive. They included postprandial pain in 87 (59.2%), vomiting in 46 (31.3%), and dysphagia in 44 (29.9%); only 23 patients (15.7%) had symptoms of gastroesophageal reflux. Anemia was present in 31 patients (21.1%) and melena in 3. Elective repair was done in 119 patients and included an uncut Collis-Nissen repair in 81 patients (68.1%), a Belsey Mark IV repair in 19 (16.0%), a Nissen repair in 17 (14.3%), and a Harrington (anatomic) repair in 2 (1.7%). Thirty-two patients had complications (26.9%). There were no operative deaths. Median follow-up was 42 months. Results were excellent in 69 patients (60.0%), good in 38 (33.0%), fair in 6 (5.2%), and poor in 2 (1.7%). Five patients had emergency operations for suspected strangulation; three had gastric necrosis, and one died. Two of the four operative survivors had excellent results. Twenty-three other patients were followed up with medical management for a median of 78 months (range 12 to 268 months). In four patients progressive symptoms developed, and one patient died from aspiration. We conclude that patients with an intrathoracic upside-down stomach who have obstructive symptoms at initial presentation should undergo repair and that elective operation is safe and effective. Gastric strangulation, however, is rare.
Adult↗
Esophageal perforation. A reassessment of the criteria for choosing medical or surgical therapy.
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Clinical Protocols↗
Videothoracoscopic wedge excision of the lung.
Recent advances in video technology and endoscopic instrumentation have expanded the use of thoracoscopy from diagnosis to treatment of pulmonary parenchymal disease. We recently performed 14 pulmonary wedge excisions using videothoracoscopic techniques in 10 patients (7 women and 3 men). Median age was 60 years (range, 21 to 82 years). Indications were small peripheral solitary pulmonary nodules in 4 patients, diffuse pulmonary infiltrates in 4, and recurrent pneumothoraces in 2. Thoracoscopic wedge excisions were accomplished using double-lumen endotracheal anesthesia and a percutaneous stapling device. Tissue diagnosis was obtained in all patients; 6 had benign disease, 3 had metastatic cancer, and 1 had diffuse bronchoalveolar cell carcinoma. Median operating time was 90 minutes (range, 40 to 140 minutes). There were no operative deaths. The single complication was a prolonged air leak. Median hospitalization was 5 days (range, 3 to 16 days). All patients returned to full activity within 10 days of discharge. Median follow-up was 6 months (range, 5 to 8 months). We conclude that videothoracoscopic wedge excision is a safe and effective procedure for selected small peripheral indeterminate pulmonary nodules, diffuse interstitial lung diseases, and recurrent spontaneous pneumothoraces. Further evaluation and prospective studies are indicated.
Adult↗
Barrett's esophagus with high-grade dysplasia: an indication for esophagectomy?
Between 1982 and 1991, 19 patients (17 men and 2 women) with Barrett's esophagus, 10 of whom were in a surveillance program, were found to have high-grade dysplasia without evidence of invasive carcinoma. Median age was 66 years (range, 30 to 79 years). Heartburn was the most common presenting symptom. Esophagoscopy at the time of high-grade dysplasia diagnosis demonstrated normal Barrett's mucosa in 10 patients (53%), shallow ulcers in 3, slight mucosal irregularities in 2, small mucosal nodules in 2, stricture in 1, and shallow ulcer with stricture in 1. Eighteen patients underwent esophagectomy. There were no operative deaths. Nine patients (50%) had invasive carcinoma. Postsurgical stage was stage 0 in 9 patients, stage I in 6, stage IIA in 2, and stage IIB in 1. Median follow-up was 34 months (range, 2 to 116 months). Recurrent cancer developed in 2 patients. Overall 5-year survival was 66.7%; 5-year survival for patients with stage 0 disease was 100% and for stage I and II disease, 35.7%. We conclude that high-grade dysplasia in an indication for esophageal resection because of the high rate of associated early invasive carcinoma and that resection can be done safely with the expectation of excellent long-term survival. Because of these findings, we continue to recommend endoscopic surveillance in all patients with Barrett's esophagus.
Adenocarcinoma↗
Colorectal lung metastases: results of surgical excision.
Between 1960 and 1988, 139 consecutive patients underwent pulmonary resection for metastatic colorectal carcinoma. Median interval between colon resection and lung resection was 34 months. Ninety-eight patients (70.5%) had a solitary metastasis. Wedge excision was performed in 68 patients, lobectomy in 53, lobectomy plus wedge excision in 9, bilobectomy in 4, and pneumonectomy in 5. Operative mortality was 1.4%. Localized extrapulmonary colorectal cancer was also resected in 20 patients. Median follow-up was 7 years (range 1 to 20.4 years). Overall 5- and 20-year survival was 30.5% and 16.2%, respectively. Five-year survival for patients with solitary metastasis was 36.9%, as compared with 19.3% for those with two metastases (p less than 0.05) and 7.7% for those with more than two (p less than 0.01). Patients with normal carcino-embryonic antigen had a 5-year survival of 46.8% versus 16.0% for patients with increased levels (p less than 0.01). Five-year survival for patients with resected extrapulmonary disease was 30.0% versus 30.7% for those without extrapulmonary cancer (p = not significant). Repeat thoracotomy for recurrent metastases was done in 19 patients. Five-year survival after the second lung resection was 30.2%. We conclude that resection of colorectal lung metastases is safe and effective, that resectable extrapulmonary disease does not necessarily contraindicate pulmonary resection, and that repeat thoracotomy is warranted in selected patients with recurrent colorectal lung metastases.
Adult↗
Video-assisted thoracic surgical procedure: management of a solitary pulmonary nodule.
Solitary pulmonary nodules continue to challenge all diagnostic skills. Herein we describe video-assisted thoracic surgical intervention, a new, minimally invasive technique that aids physicians in obtaining a definitive histologic diagnosis in a select group of patients with an indeterminate solitary pulmonary nodule.
Granuloma↗
Dirofilaria immitis: a rare, increasing cause of pulmonary nodules.
Dirofilariasis is an unusual but increasing cause of solitary pulmonary nodules. In this study, we reviewed the entire experience with dirofilariasis at our institution. Five such patients were identified. In all patients, the Dirofilaria immitis infection manifested as a solitary pulmonary nodule, and all patients underwent thoracotomy for diagnosis. None required systemic treatment. D. immitis is found in dog, cat, wolf, coyote, and fox populations throughout the United States, but the highest concentrations have been noted in the eastern, southeastern, and southern coastal states. The distribution of human cases of D. immitis infection has a similar pattern. Pulmonary dirofilariasis should be included in the differential diagnosis of peripheral noncalcified pulmonary nodules, especially in endemic areas.
Aged↗