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BACKGROUND: Achalasia is a primary esophageal motor disorder characterized by degenerative changes of the myenteric plexus. The pathophysiologic abnormalities may be the final result of several intermeshing mechanisms, and more than one single factor may cause the motor abnormalities. AIMS: To report our experience in investigating the myenteric plexus of achalasia patients undergoing esophagomyotomy. PATIENTS AND METHODS: Tissue samples from 12 patients undergoing Heller myotomy for achalasia were evaluated and compared with esophageal tissue specimens from 7 controls. Enteric neurons and interstitial cells of Cajal (ICC) were assessed by immunohistochemical methods, and the presence of vasoactive intestinal polypeptide ergic fibers and of CD3 lymphocytes. The possible presence of herpesvirus was also assessed by immunohistochemistry, whereas that of papillomavirus was assessed by in-situ hybridization. RESULTS: Compared with controls, achalasia patients displayed a significant decrease of both enteric neurons and ICC. Immunoreactivity for vasoactive intestinal polypeptide was completely absent in each patient. CD3 staining disclosed myenteric plexitis in 5 (42%) patients; no control patient had plexitis. All patients were completely negative for the presence of both herpes simplex virus and human papillomavirus. CONCLUSIONS: The enteric nervous system of the lower esophageal sphincter area is impaired in patients with "idiopathic achalasia," and the abnormalities involve ICC and neurons in many patients. The triggering factors for these abnormalities are, however, still unknown.
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Acalasia Esofágica , Imuno-Histoquímica/métodos , Plexo Mientérico , Adulto , Idoso , Complexo CD3/metabolismo , Acalasia Esofágica/imunologia , Acalasia Esofágica/fisiopatologia , Esôfago/imunologia , Esôfago/fisiopatologia , Feminino , Humanos , Células Intersticiais de Cajal/metabolismo , Células Intersticiais de Cajal/patologia , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Músculo Liso/citologia , Plexo Mientérico/imunologia , Plexo Mientérico/fisiopatologia , Neurônios/metabolismo , Neurônios/patologia , Peptídeo Intestinal Vasoativo/metabolismoRESUMO
Exogenous lipoid pneumonia (ELP) is caused by the inhalation of vaporized oily products. Long-term exposure can result in chronic disease, whereas acute form usually results from massive aspiration of fatty substances. It has an incidence of 1.0%-2.5%. In case of symptomatic patients, the clinical presentation mainly includes acute or chronic respiratory symptoms such as dyspnea, fever, cough and less frequently chest pain, hemoptysis, or weight loss. Radiological findings are often aspecific or misinterpreted, and ELP is sometimes misdiagnosed as a malignancy of the lungs. Patient history and radiological findings can lead to a suspicion of ELP, but histological microscopic findings of intra-alveolar lipid and lipid-laden macrophages are required to confirm the diagnosis The mainstay of treatment consists of avoiding ongoing exposure and providing supportive care as repeated whole-lung lavage, corticosteroids, and/or immunoglobulins. Surgery is reserved for cases of high suspicion of cancer or serious clinical impact (as recurrent infections). Prognosis is benign, even if it has been reported cases of progression to severe respiratory failure, cor pulmonale, superinfection, and association with lung cancer. Here, we describe a case of ELP due to chronic inhalation of oily product (Vaseline) used as a lubricant of tracheotomy cannula.
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OBJECTIVE: The aim of the present study is to show the predictive value of the preoperative resting pressure of the lower esophageal sphincter (LES) correlated with the type of fundoplication (Nissen or Dor) after Heller myotomy in our series. MATERIALS AND METHODS: From January 1998 to June 2010, 88 patients affected by esophageal achalasia underwent surgery at our unit. However, our study focused on a sample of 36 patients, because many data were lost or was never recorded. Among these, 14 patients underwent laparoscopic Heller myotomy plus Nissen fundoplication (group N), whereas 22 patients underwent laparoscopic Heller myotomy plus Dor fundoplication (group D). Clinical evaluation was performed using a modified DeMeester symptom scoring system consisting of the assessment of three symptoms: dysphagia, regurgitation, and heartburn. To each symptom was assigned a score from 0 to 3, depending on its severity, and the reduction in the severity of each symptom after surgery was assessed. RESULTS: The surgical treatment is considered to be effective (p<0.0001). The preoperative resting pressures of LES were compared by Student's t-test, and it was found that patients who reported a greater improvement in the dysphagia symptom had a preoperative average pressure of LES that was significantly higher than that in other patients in both group N (p=0.03) and group D (p=0.01; p=0.003; p=0.001). The Dor treatment was shown to be more effective than the Nissen treatment (p<0.0001). CONCLUSION: The preoperative resting pressure of LES is a predictive factor of surgical success both before Dor fundoplication and before Nissen fundoplication, but its predictive power is influenced by the chosen type of fundoplication.
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Here, we report a retrospective evaluation of long-term behaviour of lung carcinoids after surgery. A total of 23 patients (17 with typical pulmonary carcinoids and 6 with atypical pulmonary carcinoids) were enrolled in our hospital from April 1994 to July 2009. All patients underwent intervention at the Unit of Surgery and then were followed at the Unit of Oncology. The standard protocol for patient monitoring consisted of follow-up at 3 months after surgery, 6 months after first control and annually for 5 years. The follow-up evaluations consisted in blood tests, imaging of chest and abdomen, bone scintigraphy, and brain computed tomography. In case of disease recurrence, patients underwent chemotherapy (etoposide, carboplatin) and radiotherapy. All patients were followed for a mean of follow-up period of 100 months, ranging between 20 and 203 months. In the group of typical carcinoid, the observed recurrence rate at 5 years was zero, at 10 years was 5.8 %, whereas the observed mortality rate at 5 and 10 years was zero. In the group of atypical carcinoid, both the recurrence rate and the mortality rate at 5 and 10 years were 16.6 %. A statistical significant difference (p = 0.002) in the recurrence rate between stage I and stage II was observed. The overall prognosis of pulmonary carcinoids was favourable, and the typical carcinoids presented a better prognosis than the atypical ones. The stage at time of diagnosis could be considered as a predictive prognostic factor.
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Keloids are defined as excessive scar tissue formation extending beyond the area of the original skin injury and occurring in predisposed individuals. While no single treatment has proven widely effective, several series report excellent outcomes for keloids with post-surgery radiation therapy as described in the literature. We present a patient with recurrent giant keloid of left buttock after several surgical removals, that at physical examination shows the size of 40×22×10 cm in the largest dimension. Patient underwent a surgical excision of gluteal lesion and postoperative radiotherapy using photons at 8 MV of linear accelerator: the total dose delivered was 22 Gy in 11 days, with a daily fraction of 2 Gy. No relapse was showed at 36 months post-therapy. Several methods seem unsatisfactory for preventing keloid recurrence. The combination of surgery and adjuvant radiotherapy seems an excellent strategy to prevent recurrent disease.
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Nádegas/patologia , Queloide/radioterapia , Humanos , Queloide/cirurgia , Masculino , Radioterapia Adjuvante , Prevenção Secundária , Adulto JovemRESUMO
Irradiation-induced sarcomas are a late sequelae of irradiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gys and above, with a dose ranging from 16 Gys to 112 Gys. These tumours are very aggressive and an early detection is needed for a timely intervention. Surgery is only effective treatment for local control instead chemotherapy is a valuable tool for systemic control of disease. Irradiation therapy use is controversial because of its side effects on a site previously irradiated. Irradiation therapy combined with hyperthermia is a new treatment that overcomes these problems without limiting the effect of radiation therapy. It may become a tool for local control of the unresectable tumours or an adjuvant treatment of the surgery. In this report we present a rare case of irradiation-induced recurrent osteosarcoma involving the chest wall that was treated with surgical resection followed by radiation therapy combined with hyperthermia as an adjuvant treatment of the surgery.
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OBJECTIVE: A retrospective comparison between Nissen and Dor fundoplication after laparoscopic Heller myotomy for achalasia. MATERIALS AND METHODS: From 1998 to 2004 a first group of 48 patients underwent Heller myotomy and Nissen fundoplication for idiopathic achalasia (H+N group). From 2004 to 2010 a second group of 40 patients underwent Heller myotomy followed by Dor fundoplication (H+D group). Some patients received a previous endoscopic treatment with pneumatic dilatation or endoscopic injection of botulinum toxin that provided them only a temporary clinical benefit. Changes in clinical and instrumental examinations from before to after surgery were evaluated in all patients. Clinical evaluation was carried out using a modified DeMeester symptom score system. RESULTS: Dor fundoplication treatment reduced both dysphagia and regurgitation severity scores significantly more than Nissen fundoplication (p<0.0001). Indeed, the incidence of dysphagia was significantly higher in patients treated with floppy-Nissen than in those treated with Dor fundoplication: by defining dysphagia as a DeMeester score equal to 3 (arbitrary cut-off), at the end of follow-up dysphagia occurred in 17.65% and 0% (p=0.037) of patients belonging to the H+N and H+D groups, respectively. CONCLUSION: Heller myotomy followed by Dor fundoplication is a safe and valuable treatment. The procedure showed a lower incidence of postoperative dysphagia versus Nissen fundoplication and a negligible incidence of postoperative GERD in a long-term postoperative follow-up.
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The KEAP1/Nrf2 pathway is a master regulator of several redox-sensitive genes implicated in resistance of tumor cells against chemotherapeutic drugs. Recent data suggest that epigenetic mechanisms may play a pivotal role in the regulation of KEAP1 expression. We performed a comprehensive genetic and epigenetic analysis of the KEAP1 gene in 47 non-small cell lung cancer tissues and normal specimens. Promoter methylation analysis was performed using a quantitative methylation specific PCR assay in real time. Methylation at the KEAP1 promoter region was detected in 22 out of the 47 NSCLCs (47%) and in none of the normal tissues analyzed. Somatic mutations were detected in 7 out of the 47 tumors (15%) and loss of heterozygosity (LOH) in 10 out of the 47 (21%) of the cases. Overall, we found at least one molecular alteration in 57% of the cases. Approximately one third of the tumors had two alterations and this feature was associated with higher risk of disease progression in univariate COX regression analysis (HR = 3.62; 95% CI 1.24-10.65, p = 0.02). This result was confirmed by Kaplan-Meier analysis, which demonstrated an association between worst outcome and KEAP1 double alterations (p = 0.01, Log rank test). Our results further suggest that deregulation of the NRF2/KEAP1 system could play a pivotal role in the cancerogenesis of NSCLC. In addition identifying patients with KEAP1 genetic and epigenetic abnormalities may contribute to disease progression prediction and response to therapy in lung cancer patients.
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Carcinoma Pulmonar de Células não Pequenas/genética , Epigênese Genética , Peptídeos e Proteínas de Sinalização Intracelular/genética , Neoplasias Pulmonares/genética , Idoso , Sequência de Aminoácidos , Animais , Sequência de Bases , Carcinoma Pulmonar de Células não Pequenas/química , Carcinoma Pulmonar de Células não Pequenas/patologia , Metilação de DNA , Progressão da Doença , Feminino , Humanos , Peptídeos e Proteínas de Sinalização Intracelular/química , Proteína 1 Associada a ECH Semelhante a Kelch , Neoplasias Pulmonares/química , Neoplasias Pulmonares/patologia , Masculino , Pessoa de Meia-Idade , Dados de Sequência Molecular , Mutação , Alinhamento de SequênciaRESUMO
n-ELAV (neuronal-Embryonic Lethal, Abnormal Vision)-like genes belong to a family codifying for onconeural RNA-binding proteins, also called Hu antigens. Anti-Hu-antibodies (anti-Hu-Ab) are typically associated with paraneoplastic encephalomyelitis/sensory neuropathy (PEM/PSN), and low titres of anti-Hu-Ab were found in neural/neuroendocrine neoplasms, especially small cell lung cancer (SCLC). To date, few studies have been published focused on the genetic causes of their involvement in the pathogenesis of neuroendocrine tumors (NE). Here we analyzed 20 primary human neuroendocrine lung tumor tissues for somatic mutations in the HuD gene. Two inactivating mutations (a frameshift and a stop codon mutation) and 11 nucleotide changes were detected in the coding sequence of HuD gene in 7 different lung tumors. Our results on SCLC and carcinoid tissues support the hypothesis that alterations of nELAV genes could be involved in the onset and/or progression of a subset of neuroendocrine lung tumors.