Evaluation of immune system in patients with transfusion-dependent beta-thalassemia in Rasoul-e-Akram Hospital in 2021: A descriptive cross-sectional study.

Background and aims: Thalassemia syndromes are the most common hemoglobinopathy globally related to blood transfusion and iron overload in the body. Splenectomy, excessive iron overload, and repeated exposure to antigens in blood transfusions can cause severe damage to the patient's immune system making the patient prone to frequent infection. This study evaluates the immune system status and infection rate in beta-thalassemia major patients receiving iron chelators. Methods: This descriptive cross-sectional study was performed in Rasoul-e-Akram Hospital on patients with a beta-thalassemia major who had iron overload due to frequent blood transfusions. The percentage of lymphocyte markers was determined by flow cytometry. Serum levels of immunoglobin were measured by nephelometric assay. Also, Nitro blue tetrazolium and dihydrorhodamine assays were used to evaluate the phagocytic function. Results: Of the 106 patients participating in this study, 59 (55.7%) and 47 (44.3%) are male and female, respectively. The mean age ± SD of participants was 24.7 ± 12.1 years with 4 to 55 years. There was no significant correlation between sex, the C3 and C4 complements, the lymphocyte markers, and the immunoglobulin levels. Furthermore, all of these variables increased significantly over 30 (p < 0.05). Moreover, there was a strong positive correlation between splenectomy and IgG immunoglobulin (p < 0.001) and CD16 (p = 0.005) lymphocyte marker. Conclusion: Iron chelator agents effectively improve patients' immune system with thalassemia major. The increase in IgG and IgM immunoglobulins levels is due to frequent blood transfusions, which stimulate the immune system.

First Report of an Association Between Crohn's Disease and Isolated Splenic Tuberculosis in Pediatric Population.

Isolated splenic tuberculosis (TB) in children is extremely rare, and congenital or acquired immunodeficiency is usually a predisposing factor for this disease. Herein, we report a case of isolated splenic TB in a 5.5-year-old child associated with Crohn's disease. As far as we are aware, this association is reported for the first time in children. Clinicians should be aware and consider extra pulmonary TB, especially in endemic regions. In addition, evaluation of an underlying disorder in unusual presentations of TB is advisable.

Vascular reconstruction with the superficial femoral vein following major oncologic resection.

INTRODUCTION: Involvement of critical vascular structures has historically been considered a contraindication to tumor resection. This study describes outcomes following radical oncologic resection with concomitant resection of critical vascular structures and reconstruction with the superficial femoral vein (SFV). METHODS: All patients undergoing radical oncologic resection requiring resection of major vascular structures and concomitant reconstruction using the SFV as conduit were retrospectively reviewed. Primary outcomes were surgical morbidity and mortality; secondary measures included long-term patency and oncologic outcomes. RESULTS: Seven patients were included. There were three retroperitoneal and two groin sarcomas, and two squamous cell carcinomas metastatic to groin lymph nodes. No perioperative mortality occurred. Five patients experienced minor morbidity. One vein graft in a patient with pre-existing chronic deep venous thrombosis (DVT) occluded post-operatively. No subsequent long-term venous or arterial graft occlusions occurred (median 20.2 months, range 9.0-49.7). Two patients died of tumor recurrence during follow-up. CONCLUSIONS: Resection of tumors involving critical vascular structures is feasible. The SFV conduit is a versatile option for major vascular reconstruction, providing good long-term patency rates with acceptable morbidity and mortality. Vascular resection and reconstruction with the SFV offers another technique to provide limb-sparing surgery in patients traditionally offered only amputation, while providing favorable oncologic outcomes.

Improved survival after introduction of an emergency endovascular therapy protocol for ruptured abdominal aortic aneurysms.

BACKGROUND: The study was conducted to demonstrate improved survival (30-day mortality) after the introduction of an emergency endovascular therapy protocol for ruptured abdominal aortic aneurysms (rAAA). Numerous authors have successfully demonstrated reduced mortality in patients with rAAA using endovascular techniques. Comparison of endovascular aneurysm repair (EVAR) with open repair for rAAA may be misleading, however, because EVAR cannot be performed on all patients, and selection bias may explain the superior performance of any given surgical or endovascular strategy. We developed a model to predict mortality in patients before the introduction of EVAR (preprotocol population), applied this model to predict 30-day mortality among prospective patients (postprotocol population), and compared observed vs expected results. METHODS: We assessed 126 patients with rAAA. Primary outcome was 30-day mortality. Potential confounding variables were age, sex, presurgical lowest recorded systolic blood pressure (SBP), and glomerular filtration rate (GFR). A logistic regression model incorporating significant confounders was used to evaluate changes in 30-day mortality for all patients with rAAA after introduction of the EVAR protocol. Separate logistic regressions were done to compare 30-day mortality for preprotocol vs patients receiving EVAR and preprotocol vs patients receiving postprotocol open repair. Cumulative sum (CUSUM) analysis was used to assess shifts in the performance of the rAAA program over time. RESULTS: Significant confounders were SBP, absence of SBP, and GFR. Logistic regression found evidence of lower mortality after the protocol was introduced, 17.9% vs 30.0% (odds ratio [OR], 0.385; 95% confidence interval [CI], 0.141 to 0.981; P = .046). Comparison of all open repairs (preprotocol and postprotocol) and EVAR demonstrated decreased risk for EVAR of 5.0% vs 28.3% (OR, 0.109; 95% CI, 0.013 to 0.906; P = .0084). Unstable patients (SBP

Endovascular treatment for aortic disease: is a surgical environment necessary?

OBJECTIVE: Interventional radiologists, cardiologists, and vascular surgeons are capable of performing endovascular procedures successfully in their respective environments. Suboptimal anatomy or intraoperative technical problems can be encountered, and endovascular management alone is not always suitable. The objectives of this study were to define the incidence of adjunctive surgical techniques, to discuss the rationale for endovascular reconstruction in a well-developed surgical environment, and to assess the effect of experience on the incidence of adjunctive repair. METHODS: All primary aortic and aortoiliac elective, urgent, and emergent endovascular procedures performed at the Peter Lougheed Center and entered into a prospective database from May 25, 1999 to June 01, 2005, were reviewed. All adjunctive surgical techniques to enable stent deployment, enhance attachment site, or solve intraoperative difficulties were captured. The study period was divided into two time periods based on learning curve data to assess the effect of experience on the rate of adjunctive repairs. RESULTS: Four hundred thirty-eight patients underwent elective (80%), urgent (15%), or emergent (5%) endovascular procedures during the study period. These consisted of 101 thoracic and 337 abdominal operations, including the use of 13 fenestrated stents. One hundred thirty-nine patients (31.7%) required 180 open surgical procedures. Complete data were available for the entire patient cohort. The mean follow-up was 793.2 days (SD, 519.1 days). Procedures were necessary for vascular access, arterial dissection/rupture, limb ischemia, and enhancement/elongation of the stent attachment site. The persistent endoleak rate was 5.3%, the late rupture rate was 0.7%, the conversion rate was 1.6%, the 30-day surgical mortality rate was 3.2%, all-cause mortality to date is 7.3%, and the reintervention rate was 4.6%. There was no statistically significant effect of the learning curve on the incidence of surgical adjunctive procedures in either the thoracic group (11/26 [42.3%] for phase 1 vs 17/75 [22.6%] for phase 2) or the abdominal group (14/50 [28.0%] for phase 1 vs 97/287 [33.8%] for phase 2). Overall, 31.5% of patients required adjunctive surgical repair. CONCLUSIONS: Successful endografting requires endovascular expertise in addition to a well-developed surgical environment to increase applicability and decrease patient risk. Despite advances in endovascular technology, hybrid techniques will continue to be required to achieve good overall success rates.