Selçuk Tıp Dergisi Arşiv

Familial Mediterranean Fever (FMF) is characterized by attacks and inflammation of serous membranes. After discovery of MEVF mutations, several studies have been carried out about the effect of genotype to phenotype, a number of them showed relationship between genotype and phenotype, but some of them not. The aim of our study was to investigate the relationship between genotype and clinical features in children with FMF. This study was performed with 131 patients who have FMF diagnosis. The demographic data, clinical findings, mutations were investigated retrospectively. 52.7% of our patients were boys, 47.3% were girls. Distribution of the age of diagnosed was different from age of onset of symptoms (p=0.00). The most frequent symptom was abdominal pain (94.7%) and it is followed by fever (91.6%), arthralgia (65.6%) and chest pain (27.5%). Infection, stress, menstruation and physical training triggered the attack in some patients. Respectively, the most common mutations were M694V (50.4%), M680I (26.7%) and 148Q (11.5%). No mutation was detected in 14.5% of our patients. Nausea and vomiting were less in M694V homozygous patients, and arthralgia was higher in E148Q heterozygous patients (p<0.05). We had 3 patients with colchicine-resistant who were siblings. In our study, a significant relationship between phenotype and genotype was not observed. It is difficult to recognize the disease in infancy period, FMF should be considered in patients with frequently admitted to hospital in order to avoid complications.

The aim of this study is to evaluate our experience of patients with their clinical presentations and treatment approaches followed and underwent atrial septal defect surgery in Cardiovascular Surgery Department. Between 2010-2013, 28 patients (20 females, 8 males) mean age 58 months (range 5 month to 14 year-old) who underwent atrial septal defect surgery were included in the study retrospectively. In two patients, there were sinus venosus atrial septal defects with partially abnormal pulmonary venous drainage. In one patient, there was primum atrial septal defect. 25 patients had secundum atrial septal defects. Mean pulmonary artery pressure was 18.6 mmHg(8- 39 mmHg) and mean Qp/Qs ratio was 2.2 (1.4-4.5) in patients who underwent cardiac catheterization. Atrial septal defects were closed with synthetical patch in all of the patients. The mean of intensive care unit hospitalization was 3.2 (1-12) days. The mean of cardiovascular surgery clinical hospitalization was 4.1 (1-17) days. The mean discharge time was 7.4 (4-29) days. No mortality was observed and no hemodinamically significant residual was observed. The average postoperative follow-up period of patients was 24 months (1 month-3.5 years). Surgically closure of atrial septal defect, one of the most common forms of congenital heart disease, is performed safely in many medical center with low mortality and complication rates. If atrial septal defect remains untreated, significant complications such as cardiac failure, pulmonary vascular obstructive disease and atrial arrhythmias may occur.

Sildenafil, a phosphodiesterase inhibitor, decreases pulmonary arterial pressure by providing vasodilation in the pulmonary vascular bed. In this study, the effect of sildenafil use was investigated for prevention of pulmonary hypertensive crisis attacks in the postoperative period in patients with congenital heart defects. In our clinic, 9 patients with pulmonary hypertension and congenital heart defects were operated in between November 2005 and May 2006. Mean age of the patients were 9.6±8.1 (3-12) months and the mean body weights were 5.5±0.9 (4-7) kg. All patients were operated following median sternotomy by using extracorporeal circulation after aortic and bicaval cannulation. Medium hypothermia with cold blood cardioplegia was used. Sildenafil treatment was started in the early postoperative period by 0.5 mg/kg/day through the nasogastric tube. The dose was gradually increased up to 2 mg/kg/day and the treatment was maintained for one week. The results of the patients were evaluated retrospectively. Mean pulmonary arterial pressure in the preoperative period was 52.7±7.9 mmHg. Mean intubation times of the patients were 58.4±34.0 (20-100) hours, whereas intensive care unit stay and hospitalization periods were 2-7 (mean 4.8±2.0) and 6-12 (mean 10.11±2.8) days, respectively. During the study, neither mortality, nor important hypotension caused by sildenafil, was detected. Only a pulmonary hypertensive crisis attack was detected in 1 patient. One patient had consistent heart block and permanent pacemaker was implanted. Sildenafil is an effective choice of treatment for prevention of postoperative pulmonary hypertensive crisis in patients with congenital heart defects accompanied by pulmonary hypertension.

Thrombotic thrombocytopenic purpura (TTP) is a rare and fatal disorder characterized with microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, fever, and kidney failure. The reason of TTP and optimal treatment method of TTP is still unknown. However, today, therapeutic plasma exchange (plasmapheresis) and / or plasma infusion is considered as the standard treatment method for these patients. In this study, the clinical characteristics and outcome of 18 patients with TTP treated with plasma exchange at the Department of Hematology, Meram Faculty of Medicine, Necmettin Erbakan University, between 2008 and 2011 years were evaluated retrospectively. Among the patients, 6 were male, 12 were females, median age was 39 (21-66), 8 were idiopathic, 7 were postpartum, 1 was post operative periods of prosthetic operation, 1 was pesticide exposure and 1 was NSAII induced TTP. The first admission of patients were evaluated for symptoms and complete blood counts, coagulation tests, BUN (blood urea nitrogen), creatinine, LDH (lactic dehydrogenase) levels were performed. All patients were initially treated with a daily plasma exchange plus steroids, which was continued until the normalization of platelet count and the level of LDH. In this study, a total of 165 plasmapheresis procedures were performed to total 18 cases. Fresh frozen plasma (FFP) was used as fluid of replacement. Of the 18 patients, 15 were discharged with complete remission (n:12) and partial remission (n:3), and 3 had no response and died of progressive neurological fingings and acute renal failure. Untreated TTP has a high mortality that may be as great as 90%. However, the rate of this mortality is dramitacally changed with plasma exchange treatment. As a result, today, plasma exchange is the most effective treatment available for treatment of TTP.

Obesity surgery is a highly effective method on ensuring a maintained weight loss. Following the obesity surgeries, while there are complications specific to type of the surgery, early or late complications also can be seen. In our study, we aim to present the determined complications during or post surgery on patients who were conducted bariatric surgery due to morbid obesity.Bariatric surgery was conducted on a total of 361 patients. Of these patients, 207 (57.3%) laparoscopic sleeve gastrectomy (LSG), 38 (10.5%) laparoscopic Roux-n-Y gastric bypass (LRYGB), 32 (8.8%) Rouxn-Y gastric bypass (RYGB), 32 (8.8%) mini gastric bypass (MGB), 28 (7.7%) laparoscopic mini gastric bypass (LMGB), 21 (5.8%) sleeve gastrectomy (SG), 2 (0.5%) vertical banded gastroplasty (VBG) and 1 (0.2%) intragastric balloon placement procedures were applied. Anastomotic leak was determined on 2 patients (0.5%) with Roux-n-Y gastric bypass. On 1 patient with SG conducted, a leak through stapler route has occurred. On 1 patient evisceration, on 1 patient a spleen injury during the surgery, on 44 patient (12.1%) surgical site infection were detected. 91% of these patients with surgical site infection had diabetes mellitus. While on patients with laparoscopic bariatric surgery the ratio of surgical site infection was 6.8%, in open surgery this ratio was increased up to 29.4%. Ileus, pulmonary embolism and DVT were developed in 9 (2.4%), 3, (0.8%) pulmonary and 8 (2.2%) patients, respectively. On super obese 2 patients mortality was occurred due to postoperative lung insufficiency. With bariatric surgery, regardless of the surgery technic used there is a possibility of morbidity. Consequently, it must be careful for selecting a morbid obese patient and during the treatment in terms of complications.

Amyand Hernia is described as the presence of normal or inflamed appendix vermiformis in inguinal hernia sac. It was first described by C. Amyand in 1735 and occurs in approximately 1% in inguinal hernia. In this article, we presented two patients with amyand hernia that operated incarcerated inguinal hernia with the acute appendicitis detected.

Caesarean delivery is preferred to vaginal delivery in the breech presentation because it is safer. In this case report, we aimed to present femur fractures associated with cesarean section as a rare complication of breech presentation. A 2670g male infant was delivered at 36 weeks by emergency cesarean section for breech presentation. On the third day after the birth, he was admitted to our hospital with a preliminary diagnosis of cellulitis because of swelling in the right leg. The examination revealed a fracture of the right femur. A simple immobilization of the limb in extension led to a complete healing of the fracture without sequelae. Caesarean delivery reduces the risk of traumatic injury of the newborn compared to vaginal delivery, but does not eliminate this possible accidental complication.

Occult breast cancer is a type of cancer with no symptoms found upon physical examination on the breasts or which can not be radiologically shown. It generally manifests itself with axillary metastasis with no known primary tumor. Breast cancer in males is rarely seen. Occult breast cancer, on the other hand, is even rarer and only case reports were found in literature. A 46-year-old male patient had excisional biopsy at another medical facility because of a palpable mass on his right armpit. The pathological diagnosis had stated that the patient had adenocarcinoma metastasis with symptoms implying an infiltrative ductal carcinoma metastasis of the breast. Abdominal and thoracic tomography, upper and lower gastrointestinal system endoscopy procedures were performed on the patient in order to determine the primary focus. Breast ultrasonography, breast magnetic resonance imaging and positron emission tomography were also performed. Upon not being able to detect any positive findings, the patient was considered to have occult breast carcinoma and modified radical mastectomy was performed. In conclusion, in the presence of metastatic lymph node with no known primary tumor in the axillary, the possibility of occult breast cancer should be taken into consideration even if the patient is male.

Mantle cell lymphoma represents 3-10% of all non-Hodgkin lymphomas. It is mostly a disease of the elderly and shows a male predominance. Patients with mantle cell lymphoma usually present with advanced stage. Extranodal involvements are common finding. In this paper, we presented a case of an 62 year old male admitting to hospital only right knee pain and swelling who had aggressive mantle cell lymphoma with unusual extranodal involvements.

Inguinal hernia repair surgery is one of the most common procedures. Strangulated inguinal hernia is one of the most widely performed surgical emergencies dealt with by surgeons worldwide. Uncommonly, strangulation of the contents can occur due to other causes like omental band adhesion. We reported a rare case of strangulated inguinal hernia, brought about by an omental band adhesion, causing closed loop small bowel obstruction and perforation within the hernial sac in the inguinal canal.