A novel screening test for esophageal squamous cell carcinoma: sirtuin-3.

OBJECTIVE: Human sirtuin-3, a protein involved in the mediation of tumors, has been shown to be present in malignancies. The goal of this study was to measure serum sirtuin-3 levels in esophageal squamous cancer cells and to determine whether sirtuin-3 may possess predictive value in advanced cases of esophageal squamous cell carcinoma (ESCC). PATIENTS AND METHODS: A total of 130 ESCC patients and 50 healthy control subjects participated to the study. Serum sirtuin-3 levels for all 180 subjects were measured using enzyme-linked immunosorbent assay (ELISA). RESULTS: Median sirtuin-3 levels were significantly higher in patients with ESCC than in the control subjects. CONCLUSIONS: The presence of considerably elevated levels of sirtuin-3, could be a powerful mediator of advanced ESCC in ESCC patients, suggests that sirtuin-3 may be a useful indicator of the disease.

A pilot study of Helicobacter pylori genotypes and cytokine gene polymorphisms in reflux oesophagitis and peptic ulcer disease.

Helicobacter pylori causes various diseases such as chronic gastritis, peptic ulcer and gastric cancer. While majority of the people infected with H. pylori is asymptomatic, 15-20 % of them develop such diseases. The main factors, which determine the development of H. pylori related diseases might be bacterial virulence, host genetic and environmental factors.The aim of this study was to reveal the factors that play a role in the disease development in patients with reflux esophagitis and peptic ulcer, infected with Helicobacter pylori. Environmental factors such as medical agents, smoking and body mass index were evaluated. The factors specific to bacteria such as vacA, CagA, babA and iceA virulence genotypes and the host factors such as IL-1, IL-2, IL-4, IL-6, IL-10, IL-12, interferon-γ, TNF-α, ve TGF-ß1 gene polymorphisms were compared between the two groups.H. pylori infected twenty five patients with reflux esophagitis and peptic ulcer were enrolled in the study. There was no statistical difference between the two groups regarding environmental factors. IL-2 -330T +166T (p=0.037) and IL10 -1082A; -819C (p=0.049) gene polymorphisms were significantly more common in the group of patients with peptic ulcer compared to the group with reflux esophagitis. In both groups of patients, either with reflux esophagitis or peptic ulcer, multiple H. pylori virulence genotypes (cagA, vacA, babA) (mean values 74 %, 78 %, 54 % respectively) were observed.In this study, we revealed that cytokine gene polymorphisms may play a role in the development peptic ulcer while H. pylori virulence genotypes seem to be crucial for the development of associated diseases (Tab. 4, Ref. 51).

Oxidative stress and antioxidant enzyme activities in newborns with oesophageal atresia and their mothers.

OBJECTIVE: To measure the oxidant/antioxidant status of newborn babies with oesophageal atresia and their mothers, compared with healthy control subjects. METHODS: This case-control study included 40 participants: 10 newborns with oesophageal atresia and their mothers, and 10 healthy newborns and their mothers. Whole blood malondialdehyde (MDA) levels and the activities of antioxidant enzymes (catalase, carbonic anhydrase [CA], glucose-6-phosphate dehydrogenase [G-6-PD], and superoxide dismutase [SOD]) were measured. RESULTS: MDA levels and CA activity were significantly higher, and catalase, SOD and G-6-PD activities were significantly lower, in newborns with oesophageal atresia and their mothers than in healthy newborns and their mothers. Although CA activity was similar between the newborns and mothers in the patient group, it was significantly lower in newborns than in mothers in the healthy group. CONCLUSIONS: Increased lipid peroxidation might play an important role in the pathogenesis of oesophageal atresia. Impairment of the free radical/antioxidant balance may lead to increased free radical and decreased antioxidant levels in oesophageal atresia.

Surgical treatment of right middle lobe syndrome in children.

OBJECTIVE: Right middle lobe syndrome is a rare entity in children, causing high morbidity. Our experience of these patients including their clinical and laboratory characteristics, indications forsurgical management, postoperative courses, and follow-up results was evaluated. METHODS: A retrospective analysis was performed involving 20 children with right middle lobe syndrome who were hospitalized and treated with surgical resection of the right middle lobe in Van Training and Research Hospital and Yüzüncüyil university hospital, Turkey, between January 2002 and January 2011. RESULTS: The mean age of the patients was 10.5 years (range, 5 to 15 years). Twelve patients were boys and eight were girls. The most frequent symptom was chronic cough (75%). Hemoptysis was present in two (10%) patients. One patient was being treated for asthma. 25% positive cultures were identified among the patients. Streptococcus pneumoniae was the most frequently identified agent in sputum. All patients underwent chest computed tomography. There were bronchiectasis in 11 (55%) patients, atelectasis and bronchiectasis in five (25%) patients, and destroyed lung in four (20%) patients. A narrowed middle lobe bronchus was shown in 15 (75%) patients. Bronchoscopy was performed in 18 (90%) patients. Stenosis due to external compression was seen in 12 (60%) patients, hyperemia and bronchitis in two (10%) patients, granulation tissue in two (10%) patients, and dense secretions in two (10%) patients. A history of doctor-diagnosed tuberculosis was present in two (10%) patients. These patients had completed antituberculous treatment. The patients had been symptomatic for the last 1 to 10 years (mean, 4 years) and had received several medical treatments. All patients (totally 20 patients) underwent right middle lobe resection. In one patient, a bronchial abnormality was found intraoperatively. One patient died on postoperative day 10 due to a brain abscess. Three other patients had postoperative complications (15%). Mean duration of follow-up of the patients was 4.5 years (range, 2 months to 12 years). Seventeen patients were asymptomatic, and two patients had improved. CONCLUSIONS: Children with right middle lobe syndrome unresponsive to medical treatment should undergo early lobe resection to avoid serious complications and the progression of the disease to other segments or lobes.

The effects of E-cadherin and bcl-2 on prognosis in patients with breast cancer.

BACKGROUND: Breast cancer is the most common malignancy in women. Axillary lymph node involvement and tumor size are the most significant prognostic factors in breast cancer. However, more factors are needed for evaluating the prognosis and individualizing the treatment in these patients. Intracellular adhesion molecule E-cadherin, antiapopitotic protein bcl-2, and p53 might have predictive and prognostic properties in breast cancer. OBJECTIVE: We have investigated the effects of E-cadherin, bcl-2, and p53 on disease free survival and overall survival in patients with breast cancer. METHODS: Positivity of aforementioned genes was detected with immunohistochemistry staining. Seventy-six women patients with invasive ductal and lobular breast cancer who had received adjuvant therapy were included in the study. RESULTS: Bcl-2, E-cadherin, and p53 expression in tumor tissue specimens were found in 26.31%, 35.52%, and 9.21%, respectively. Mean duration of follow-up was 93.58 +/- 3.40 months. In multivariate analyses, axillary lymph node presence and C-erb-B2 overexpression were a strong negative prognostic factor of disease free survival and overall survival. E-cadherin and bcl-2 failed to have an effect on disease free survival and overall survival in our study. In addition, p53 overexpression was determined in a limited number of patients. CONCLUSIONS: The results of this study showed that E-cadherin, bcl-2, and p53 did not have any significant prognostic value for our patients. In order to arrive at a decision, we need studies, which include more patients and long follow-up periods (Tab. 2, Fig. 2, Ref. 24).

Nodular fasciitis of the external auditory canal.

PROBLEM: Nodular fasciitis (NF) is a reactive myofibroblastic proliferation that may be misdiagnosed as a sarcoma because of its rapid growth, rich cellularity, and mitotic activity. NF is uncommon in the auricular region. We describe a case of nodular fasciitis of the external auditory canal in an 8-year-old male. METHODOLOGY: An excisional biopsy was performed, and the pathologic examination was consistent with nodular fasciitis; however, the lesion recurred within a month requiring that a total excision be done. RESULTS: The patient is without recurrence 7 months after the total excision. CONCLUSIONS: Because its histologic features closely mimick a malignant lesion, NF must be considered in the differential diagnosis of a mass originating from the external auditory canal to avoid overly aggressive and functionally debilitating treatment.

Liver cirrhosis in an infant with Chanarin-Dorfman syndrome caused by a novel splice-site mutation in ABHD5.

UNLABELLED: We described a Turkish girl with Chanarin-Dorfman syndrome who developed liver cirrhosis in the early infancy. She had all the clinical features of Chanarin-Dorfman syndrome such as ichthyosis, Jordan's anomaly, fatty liver disease and mild ectropion. The diagnosis was confirmed with a novel ABHD5 mutation. Liver steatosis or steatohepatitis with or without hepatomegaly is the predominant finding of Chanarin-Dorfman syndrome. Cirrhosis has been reported in patients with long-duration disease. CONCLUSION: Local factors or dysfunction of local proteins such as mutations or polymorphisms in hepatic microsomal lipase and arylacetamide deacetylase may contribute the severity of liver involvement, and steatosis may progress to cirrhosis in the early infancy in Chanarin-Dorfman syndrome.

Giant mass due to Castleman's disease causing superior vena cava syndrome.

Castleman's disease is an uncommon disorder occurring mostly in patients presenting with localized mediastinal lymphadenopathy.It is usually asymptomatic. With localized disease, surgical excision is curative. Castleman's disease can very rarely present as superior vena cava syndrome. We describe a case of mediastinal Castleman's disease which presented as vena cava superior syndrome; it is the largest mediastinal mass from Castleman's disease reported in the literature.

Neurofibromatosis type 1 associated with pheochromocytoma: a case report and a review of the literature.

Pheochromocytoma (PHEO) occurs in 0.1-5.7% of patients with neurofibromatosis type 1 (NF1). We report a case of adrenal PHEO in a patient with NF1. A 30-yr-old Turkish man was admitted to our hospital for further examinations of a right adrenal mass, that was incidentally discovered by abdominal ultrasonography during examinations for acute hepatitis B infection in another hospital. In his past medical history, the patient had only had one palpitation, sweating and headache episode 4 yr before. On admission, his blood pressure was 110/70 mmHg. Physical examination revealed signs of NF1. He had multiple neurofibromas over the entire skin, café-au-lait spots on the trunk and extremities and skinfold freckling. Bilateral opthalmic examination revealed multiple Lisch nodules. The 24-h ambulatory blood pressure monitoring revealed paroximal hypertension attacks (190/148 mmHg). Urinary catecholamines were markedly increased. Magnetic resonance imaging (MRI) revealed a solid round tumor approximately 5 cm in diameter, located in right adrenal gland. A 131Iodine-metaiodobenzylguanidine (131I-MIBG) scan showed uptake in the right adrenal gland. The pre-operative treatment with an alpha-blocker (phenoxybenzamine) was performed. Right adrenalectomy was performed; the surgical specimen revealed PHEO. Urine catecholamines and their metabolites returned to normal ranges on post-operative day 7. In conclusion, an adrenal mass can be incidentally discovered in any patient. After diagnosis of NF1, patients who have episodes of hypertension, sweating, headache and palpitation should be evaluated for PHEO.

Primary adenocarcinoma of the fallopian tube: a case report.

We report a case of a 75-year-old postmenopausal woman with primary fallopian tube carcinoma confined to the left fallopian tube in Stage IA-2, who is alive without evidence of disease three years after total abdominal hysterectomy, bilateral salpingo-oophorectomy, total omentectomy, pelvic and paraaortic lymph node dissection were performed. Histopathological examination revealed a poorly differentiated (grade 3) papillary serous adenocarcinoma of the left fallopian tube. Adjuvant irradiation was given. Primary fallopian cancer should be suspected by clinicians even if the presenting symptoms are atypical and the primary treatment remains surgical resection followed by adjuvant chemotherapy or radiation. Appropriate therapy for each stage of the disease should be defined and new studies are needed to better depict the clinical course and prognostic factors.

Aneurysmal bone cyst of the sternum: a case report of successful treatment with radiotherapy.

An unusual case of a lytic, expanding lesion of the manubrium with histological diagnosis of aneurysmal bone cyst in a 13-year-old girl is presented. After a recurrence following primary surgery, the patient was treated successfully by external beam radiotherapy. A total dose of 25.2 Gy was delivered using conventional fractionation (1.8 Gy day(-1)) to the whole sternum. She remains recurrence- and symptom-free 46 months after the end of the treatment. This is the sixth patient with primary aneurysmal bone cyst in the sternal region, the first paediatric patient for this location, and the first case of its kind treated exclusively by radiotherapy ever reported in the literature. The histopathological, radiological and clinical findings of the patient are presented, relevant literature is reviewed, and radiotherapeutic management of such lesions is discussed.

A treatment for biliary hypoplasia: tube cholecystostomy and irrigation.

PURPOSE: Biliary Hypoplasia (BH), characterised by a small ductal system and reduction in the number of interlobular bile ducts, has a bad prognosis. It has been claimed that other treatment methods apart from liver transplantation are not effective. Seven patients with BH underwent tube cholecystostomy, decompression and saline irrigation of the biliary tree via tube cholecystostomy. We present our treatment method, together with the early and late biochemical and histopathological results of these patients. PATIENTS AND METHODS: The records of seven patients with BH were reviewed retrospectively. BH was proved by operative cholangiography. Irrigation was performed intermittently with warm saline to the biliary tract via tube cholecystostomy over two to three months. SGOT, SGPT, alkaline phosphatase (AP) and bilirubin levels were evaluated preoperatively and postoperatively. Histopathological findings were also evaluated. RESULTS: Median age at operation was 46 days (range 20 - 90 days). There were six males and one female. Five patients recovered completely. There was a statistical difference between preoperative and postoperative SGOT, SGPT, AP, and bilirubin levels of patients who recovered (p < 0.05). CONCLUSION: Similar to biliary atresia, many factors such as the patient's age, postoperative bilirubin level, histopathology of liver, treatment method, and cholangitis as a complication of the surgical procedure affect the prognosis of BH. Operation before the age of 70 days, normal bilirubin levels after operation, no cholangitis attack, intracellular and intracanalicular cholestasis, and mild mixed cellular infiltration are favourable factors. We believe that decompression and irrigation of the biliary tract is an effective treatment method for suitable cases of BH.

Mesenteric involvement in neurofibromatosis type 1: CT and MRI findings in two cases.

We report computerized tomographic and magnetic resonance imaging findings of neurofibromatosis type 1 with mesenteric involvement in two patients. The first patient was a 13-year-old female with a panmesenteric plexiform neurofibroma with segmental involvement of the bowel wall; she had a 3-year history of abdominal pain. The second patient was a 60-year-old female who presented with malignant transformation of multiple mesenteric neurofibromas 4 months after primary operation.

[Chest trauma: analysis of 126 cases]. / Gögüs travmasi: 126 olgunun analizi.

To evaluate our experience in chest trauma we review the records of the patients admitted for chest trauma to our service between December 1994 and April 2000. There were 126 patients (113 males, 89.7%), with an age range of 7 to 96 years (mean 35.3). The most frequent cause of chest injury were traffic accidents 48 cases (38%) and 73 cases (57.9%) were victims of blunt trauma. In 36 cases there was a politrauma. Hemo and/or pneumothorax represented the most common thoracic injury (85.7%). Rib fractures were present in 47 cases (36.2% with more than 3 rib fractures). Four patients sustained flail chest and 5 defect of thoracic wall. Six patients were pulmonary contusion and 8 intraparenchymal hematoma. Pleural drainage was performed in 106 cases (8 cases bilateral) and thoracotomy was indicated in 11 (6 cases emergency). Mortality was 6.9% (n:8), of which 4 belonged to politrauma group. Mortality is directly related with politrauma. In the patients of the chest trauma, aggressive diagnostic and therapeutic approaches in association with intensive care follow-up remains one of the most important points to decrease mortality and morbidity rate.