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Background Proteus syndrome is a very rare condition with less than 100 confirmed cases reported worldwide. We report a case of Proteus syndrome in a two-year-old male who has hemophilia A comorbidity. Case Presentation A two-year-old male patient was admitted with the chief complaint of severe bleeding in mouth cavity after trauma for two weeks. At admission he was found to have petechiae on buccal mucosa and fecal discoloration due to GI bleeding. We noted multiple abnormalities in his musculoskeletal system and skin. He had lymph edema in left leg, hemihypertrophy, macrodactyly in both foots and macrocephaly. With the history of severe bleeding and recurrent blood product transfusion, we suspected a hemorrhagic disorder. The reduced level of Factor VIII activity confirmed the diagnosis of hemophilia A. Considering patient's various musculoskeletal abnormalities according to the diagnostic criteria and after ruling out similar disorders the diagnosis of Proteus syndrome was established. Conclusion Because of the variability of clinical features, Proteus syndrome can be confused with other disorders of multiple tissue overgrowth. Our case of Proteus syndrome, who had hemophilia A comorbidity outlines the challenges in diagnosis of such rare combination of diseases.

Cancer patient receive various cytotoxic drugs in association with antiemetic drugs such as 5HT3 receptor antagonists as their chemotherapy regimen. 5HT3 receptor antagonists have been reported to produce changes in ECG parameter. There are only a few studies about cardiovascular events of these drugs in patient receiving potentially cardiotoxic chemotherapies. The subject of this study is to evaluate ECG changes after administration of chemotherapeutic agents and granisetron (the most commonly used 5HT3 antagonist in Iran) in adults with cancer. For this clinical trial study, all cancer patients referred to department of radiation oncology of Imam Hossein Hospital since August 2005 to March 2006 were evaluated if they had inclusion criteria. Granisetron (3 mg) was infused intravenously over 30 seconds just a few minutes before chemotherapeutic agent administration. The 12-lead ECG recording was obtained before and 90 minutes after infusion of granisetron. One cardiologist determined PR, QRS, QTc intervals and heart rate of all ECGs. During the study period 54 patients fulfilled our criteria. With paired t-test, the PR and QTc intervals, but not QRS interval showed statistically significant prolongation after drug infusion (P < 0.0001), and heart rate showed statistically significant decrease (P < 0.0001). The ECG findings of chemotherapeutic agents and granisetron administration were prolongation of PR and QTc intervals and decrease of heart rate (P < 0.0001). Although these changes did not cause clinical signs, with keeping in mind that there may be possible drug-drug interactions and preexisting cardiac comorbidities in cancer patient, it seems reasonable and necessary to consider physical condition specifically cardiac condition and drug usage of each patient, while designing chemotherapy regimen and supportive drugs.

Background: Stress is not always a direct result of stressful conditions, but rather the way they are perceived. Thus individual variables that may be associated with perceived stress should be examined in stress studies. This study investigates the intermediary role of self-efficacy in relation with stress, glycosylated haemoglobin and health-related quality of life in patients with type2 diabetes.Methods: All women with diabetes in Yazd Diabetes Research Centre, in 2012, were considered and 80 women were selected by random sampling. They completed Shirer’s self-efficacy scale questionnaire, depression, anxiety and stress scale (DASS), and ADDQ0L19 questionnaire. Then they were introduced to the lab for blood test. Data were analysed by SPSS software and stepwise regression method.Results: Pearson correlation test results showed that the hemoglobin A1c (r = 0.35) and Quality of Life (r = -0.22) are associated with stress (P

Epidermal growth factor (EGF) is known to be present in the alpha granules of human platelets; however the source of this EGF, ie, whether it is taken up by the platelets from the circulation, or whether it is packaged into the platelets from the megakaryocyte during thrombopoiesis, is unknown. To determine whether EGF is taken up by platelets, platelets for EGF receptors were assayed and it was attempted to detect uptake of EGF by the platelets from culture medium. Platelets were found to lack EGF receptors, and no uptake of EGF from the culture medium was detected. To assess whether EGF is packaged into platelets from the megakaryocyte, megakaryocytes in frozen section bone marrow cores were stained for EGF protein by immunohistochemistry, and it was demonstrated that EGF is present in megakaryocytes. In addition, staining of megakaryocytes by in situ hybridization for EGF mRNA demonstrated its presence in these cells. Therefore it is concluded that the source of EGF in human platelets is the megakaryocyte and that this EGF is synthesized in the megakaryocyte rather than being taken up from its environment.

Anti-Leu M1 generally does not stain the lymphocytic and histiocytic (L&H) variants of Reed-Sternberg cells in the lymphocyte predominant type of Hodgkin's disease. However, the authors found that after neuraminidase treatment for removal of sialic acid, the L&H cells in more than half of the cases studied could be stained by anti-Leu M1. This result strongly suggests that L&H cells differ from the Reed-Sternberg cells in other types of Hodgkin's disease in their unique capacity to sialylate the 150-kd Leu M1 antigen.

Monocytoid B lymphocytes (MBLs), originally described as part of the histologic picture of toxoplasmic lymphadenitis, have been recognized as a reactive component in a variety of lymph node disorders. The authors now report 3 cases of non-Hodgkin's lymphoma in which a multidisciplinary approach allowed them to confirm the existence of a malignant lymphoma composed of the neoplastic counterpart of the MBLs found in nonneoplastic disorders. In all 3 cases, the lymphoma was composed of a relatively monomorphous infiltrate of atypical MBLs that had rather uniform-appearing nuclei and had well-defined, moderately abundant pale cytoplasm. The pattern of lymph node involvement in all 3 cases was predominantly sinusoidal and interfollicular. The neoplastic lymphoid cells were strongly positive for B-cell-restricted antigens; the light- and heavy-chain phenotypes were kappa-IgM (2 cases) and kappa-IgG (1 case). In all 3 cases, rearrangement of heavy- and/or light-chain genes was clearly identified by Southern blot hybridization. The name "monocytoid B-cell lymphoma" is proposed for this newly described malignant B-cell neoplasm.

Background: Gigantomastia is a breast disorder marked by exaggerated rapid growth of the breasts, generally bilaterally. Since this disorder is very rare and has been reported only in sparse case reports its etiology has yet to be fully established. Treatment is aimed at improving the clinical and psychological symptoms and reducing the treatment side effects; however, the best therapeutic option varies from case to case. Case Presentation: The present report described a case of gestational gigantomastia in a 30-year-old woman, gravida 2, parity 1, 17 week pregnant admitted to Pars Hospital, Tehran, Iran, on May 2014. The patient was admitted to hospital at week 17 of pregnancy, although her breasts initially had begun to enlarge from the first trimester. The patient developed hypercalcemia in her 32nd week of pregnancy. The present report followed this patient from diagnosis until the completion of treatment. Conclusion: Although gestational gigantomastia is a rare condition, its timely prognosis and careful examination of some conditions like hyperprolactinemia and hypercalcemia is essential in successful management of this condition.

There is still contradictory evidence on disclosure preferences regarding cancer diagnosis. The aim of this study was to evaluate the preference of cancer patients for knowing the truth about their disease, as well as the factors that might have an impact on these preferences.This study was conducted in 11 cancer centers in Iran. A questionnaire was used to collect data, and all patients above 15 years of age who were willing to participate were included in the study. The patients were asked if they were aware of the malignant nature of their disease, and if they came to know about their disease at the time of initial diagnosis, or later. The patients were then asked about the way they looked upon their disease. In the final part of the questionnaire, the participants were asked the level of involvement they prefer to have in making treatment decisions.In total, 1226 patients were enrolled in this study, only 565 (46.7%) of whom were aware of their disease at the time of diagnosis, and 878 (72.7%) at the time of interview, while 980 (85.2%) were willing to receive information about their disease. Patients' awareness was significantly associated with age under 50 years, female gender, having breast, skin or head and neck cancer, and having medical care in Shiraz or Hamadan while it was not associated with the stage or accompanying illness.While the majority of Iranian cancer patients prefer to be aware of the nature of their disease and have an active role in treatment decision making, they do not receive this information.

Background:In recent years, the success in management of thalassemic patients, has allowed for some previously unrecognized complications including renal abnormalities to emerge. This prospective study aimed to investigate kidney iron overload by means of MRI T2* and also renal function based on laboratory tests for early markers of glomerular and tubular dysfunction among adult Iranian transfusion-dependent thalassemia major patients. Subjects and Methods: Two-hundred and two patients with transfusion-dependent β-thalassemia major were included in this study in Zafar Adult Thalassemia Center, Tehran, Iran. For all patients, kidney MRI T2* as well as evaluation of BUN, creatinine, uric acid, calcium, phosphorus, sodium (Na), potassium (K), total protein, albumin, cystatin C, serum ferritin β2-microglobulin, NAG (N-acetyl-beta-D-Glucosaminidase), and urine protein were performed. Results: One-hundred and fourteen female and 88 male transfusion-dependent β-thalassemia major patients with mean age of 30.1 ± 9.4 participated in the present study. We found that 77.7% of our patients had kidney hemosiderosis based on MRI T2*. Also, 67 patients (33.2%) had elevation of serum cystatin C, and 104 patients (51.5%) had reduced estimated glomerular filtration rate (e-GFR). Increased urinary excretion of NAG and hypercalciuria were found in 50% and 79.2% of participants, respectively. Conclusion: Renal hemosiderosis and asymptomatic renal dysfunction are prevalent among transfusion- dependent β-thalassemia major patients which necessitate regular screening with early markers of glomerular and tubular dysfunction. Further studies in order to investigate the correlation between renal hemosiderosis and early markers of kidney dysfunction among these patients are recommended.

Background: The purpose of this study was to introduce a technique to extract the remaining peritoneal gas in order to improve the post-laparoscopic shoulder pain. Methods: This study included 12 patients undergoing laparoscopic gynecologic procedures between February and March 2016 in Minimally Invasive Techniques Research Center, Pars Hospital, Tehran, Iran. For complete suction of the air from abdominal cavity, the air was first vacuumed from the pelvic cavity in Trendelenburg position and then the patients were put in anti-Trendelenburg position. In this position, as the remaining gas was shifting toward subdiaphragmatic area, the suction tube was shifted to a position next to the camera canal and the remaining air was suctioned. A 10 point visual analogue scale was used to measure the severity of patients’ post-operative shoulder pain. Results: The mean VAS for shoulder pain was 0.8±1.7 4 hr post-surgery. At 12 hr post-surgery, the mean VAS was 0.8±1.5. At 24 hr post-surgery, the mean VAS for shoulder pain was 0.3±0.8. Finally, 48 hr post-surgery, the VAS score for all patients was zero. Conclusion: Our approach for emptying the abdominal cavity from residual gas after laparoscopic procedures seems to be useful in preventing post-operative shoulder pain among patients undergoing gynecological laparoscopic surgeries. Further studies are suggested to compare the effect of our proposed method with other methods.