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Hypogammaglobulinemias, based on inborn errors of immunity, are primary immunodeficiencies (PIDs) that can also be diagnosed for the first time in adulthood. Common variable immunodeficiency (CVID) is a multifactorial disease often symptomatic due to antibody deficiency. In addition, some PIDs are classified into the category of immunodeficiencies with syndromic features due to their accompanying clinical findings unrelated to immunity. In this article, a patient with CVID who was diagnosed in adulthood and who was revealed to have a mutation specific to Rubinstein–Taybi syndrome and clinical features reminiscent of this syndrome only after molecular tests was presented.

Objective. Even for very successful peripheral revascularization therapy, treatment is not complete until the ulcerative, gangrenous, and infected wound is closed. This study was performed and compared the outcomes of vacuum-assisted closure (VAC) and maggot debridement therapy (MDT) following peripheral revascularization to accelerate the wound healing process. Methods. We did a prospective randomized clinical trial between January 1, 2014, and June 21, 2019. This study included 72 patients (63 males and nine females). Balloon angioplasty was performed in 21 patients (29.2%), peripheral bypass in 39 (54.2%), and both balloon angioplasty and revascularization (hybrid) surgery in 12 (16.7%). Thirty-three patients (45.8%) received 15 VAC therapy sessions for a month. Therapy progress was monitored at 48 h intervals, and wound debridement was performed. Thirty-nine patients (54.2%) received an average of six larval therapy sessions for a month. Groups were compared with the X2 test, and a statistically significant difference was found ( P < 0.001 ). Results. In the VAC therapy group (n = 33), 14 patients (42.4%) had their feet amputated, 5 (15.1%) had a toe amputated, and 4 (12.1%) had all of their toes amputated. A skin graft was performed on four patients (12.1%) who developed granulation tissue. The wounds of six patients (18.2%) undergoing VAC therapy healed. In the larval therapy group (n = 39), the wounds healed in 36 patients (92.3%), and 3 (7.7%) had a toe amputated. Conclusion. Larval therapy was shown to be more effective than VAC therapy for the treatment of postrevascularization ischemic wounds. Thus, larval therapy can be used as an effective biological treatment method when major amputation is not required.

Objectives: we endeavored to retrospectively assess the prognostic merit of pretreatment systemic immune response index (SIRI) in glioblastoma multiforme (GBM) patients who underwent postoperative partial brain radiotherapy (RT) and concurrent plus adjuvant temozolomide (TMZ), namely, the Stupp protocol. Methods: the records of 181 newly diagnosed GBM patients who received the postoperative Stupp protocol were retrospectively analyzed. The SIRI value for each eligible patient was calculated by utilizing the platelet, neutrophil, and lymphocyte measures obtained on the first day of treatment: SIRI=NeutrophilsxMonocytes/Lymphocytes. The ideal cutoff values for SIRI connected with the progression-free- (PFS) and overall survival (OS) results were methodically searched through using the receiver operating characteristic (ROC) curve analysis. Primary and secondary end-points constituted the potential OS and PFS distinctions among the SIRI groups, respectively. Results: the ROC curve analysis labeled the ideal SIRI cutoffs at 1.74 (Area under the curve (AUC): 74.9%; sensitivity: 74.2%; specificity: 71.4%) and 1.78 (AUC: 73.6%; sensitivity: 73.1%; specificity: 70.8%) for PFS and OS status, individually. The SIRI cutoff of 1.78 of the OS status was chosen as the common cutoff for the stratification of the study population (Group 1: SIRI 1.78 (N=85)) and further comparative PFS and OS analyses. Comparisons between the two SIRI cohorts manifested that the SIRI 1.78 counterparts. The results of multivariate Cox regression analyses ratified the independent and significant alliance between a low SIRI and longer PFS (P, NA

Allogeneic hematopoietic stem cell transplantation (AHSCT) is a promising strategy for treatment of heavily pretreated mycosis fungoides/Sezary syndrome (MF/SS). Herein, we aimed to evaluate the outcomes of AHSCT for heavily pretreated patients with MF/SS retrospectively. This analysis included consecutive 19 patients with MF/SS who received 20 AHSCT between 2012-2021 in our transplant center. Eight patients have been previously reported. Fifteen patients had diagnosis of MF and referred to SS in five patients. In our cohort, all cases had advanced disease (stages IIB: n = 1, IIIA: n = 7; IIIB: n = 4, IVA: n = 4, and IVB: n = 3). Nine patients (47.4%) had developed large cell transformation. Only two patients received AHSCT in complete response, one very good partial response and two partial response while the others had progressive disease (n = 15) before transplant. Seven (35%) patients were alive at the time of analysis, with a median follow up of 10.5 months (range, 0.3-113 months) after AHSCT. Nine patients (47.4%) died without disease relapse or progression. Non-relapse mortality was 35.9% at 1 year and 26.9% at 3 years and thereafter. For all patients the probability of overall survival was 48.5% and 32.3% at 1- and 5-year post-transplant, respectively. AHSCT for MF/SS resulted in an estimated progression free survival of 45.4% at 1 year. Given the poor prognosis of patients not receiving transplants and in the absence of curative non-transplantation therapies, our results support that AHSCT is able to effectively rescue 32.3% of the population of transplant eligible, heavily pretreated patients in 5 years.

Margin status is one of the significant prognostic factors for recurrence in breast-conserving surgery (BCS). The issue that merits consideration for oncologic safety and cost-effectiveness about the modalities to assure clear margins at initial surgical intervention remains controversial after neo-adjuvant chemotherapy (NAC). The presented study aimed to assess the impact of intraoperative ultrasound (IOUS)-guided surgery on accurate localization of tumor site, adequacy of excision with clear margins, and healthy tissue sacrifice in BCS after NAC. Patients who had IOUS-guided BCS ater NAC were reviewed. No patient had preoperative localization with wire or radiotracer. Intraoperative real-time sonographic localization, sonographic margin assessment during resection, macroscopic and sonographic examination of specimen, and cavity shavings (CS) were done as the standard procedure. No frozen assessment was performed. One hundred ninety-four patients were included, in which 42.5% had pCR. IOUS-guided surgery accomplished successful localization of the targeted lesions in all patients. Per protocol, all inked margins on CS specimens were reported to be tumor-free in permanent histopathology. No re-excision or mastectomy was required. For a setting without CS, the negative predictive value (NPV) of IOUS rate was 96%. IOUS was found to over and underestimate tumor response to NAC both in 2% of patients. IOUS-guided surgery seems to be an efficient modality to perform adequate BCS after NAC with no additional localization method. Especially, when CS is integrated as a standard to BCS, IOUS seems to provide safe surgery for patients with no false negativity and a high rate of NPV.

BACKGROUND Neuromyelitis optica spectrum disorders (NMOSD) are a group of antibody-mediated chronic inflammatory diseases of the central nervous system. Rituximab is a monoclonal antibody that leads to a reduction in disease activity. OBJECTIVE To evaluate the efficacy of rituximab as monotherapy in NMOSD and to determine whether the efficacy varies depending on the presence of antibodies in this cohort. METHOD This multicentre national retrospective study included patients with NMOSD treated with rituximab at least for 12 months from Turkey. The primary outcomes were the change in the annualised relapse rate, the Expanded Disability Status Scale (EDSS), the number of relapse and radiological activity-free patients. RESULTS A total of 85 patients with NMOSD were included in the study. Of 85 patients, 58 (68.2%) were seropositive for anti-Aquaporin4-IgG (antI-AQP4-IgG). All patients were Anti-Myelin Oligodendrocyte Glycoprotein IgG (anti-MOG-IgG) negative. The median follow-up for rituximab treatment was 21 months (Q1 16-Q3 34.5). During rituximab treatment, the mean annualised relapse rate (ARR) significantly decreased from 1.45 ± 1.53 to 0.15 ± 0.34 (P

Background. The aim was to investigate the amounts of saliva and serum asprosin in order to determine whether it is related to obesity and whether salivary glands synthesize asprosin or not.Methods. A total of 116 underweight, normal weight, overweight, and obese (class I, class II, and class III) volunteers participated in the study. Saliva and blood samples were collected simultaneously from the participants. The amounts of asprosin in saliva, salivary gland tissue supernatants, and bloods were determined by ELISA, whereas asprosin synthesis sites of salivary gland tissues were determined immunohistochemically.Results. The amount of asprosin from the lowest to the highest was in the order as follows: underweight, normal weight (control), overweight, and obese classes I and III. The lowest level of asprosin was detected in underweight individuals. It was also found that the interlobular striated ducts and the interlobular ducts of the submandibular and parotid salivary glands produce asprosin. According to these data, the asprosin level is related with obesity as the amount increases in accordance with increasing body mass index (BMI). On the other hand, there is also a relationship between the underweight and asprosin because the amount decreases with BMI decrease.Conclusions. Asprosin, a new adipokine, may be a novel indicator of adipose tissue mass. Therefore, we anticipate that antiasprosin preparations may be an alternative in the treatment of obesity in the future.

In this paper, we introduce degenerate multi-poly-Bernoulli polynomials and derive some identities of these polynomials. We give some relationship between degenerate multi-poly-Bernoulli polynomials degenerate Whitney numbers and Stirling numbers of the first kind. Moreover, we define degenerate multi-poly-Bernoulli polynomials of complex variables, and then, we derive several properties and relations.

Purpose: we explored the prognostic influence of the systemic inflammation response index (SIRI) on the survival outcomes of stage IIIB/C non-small-cell lung cancer (NSCLC) patients who underwent concurrent chemoradiotherapy. Methods: present propensity score-matching (PSM) analysis comprised 876 stage IIIB/C NSCLC patients who received 1-3 cycles of platinum-based doublets concurrent with thoracic radiotherapy from 2007 to 2017. The primary and secondary objectives were the relationships between the SIRI values and overall (OS) and progression-free survival, respectively. Propensity scores were calculated for SIRI groups to adjust for confounders and to facilitate well-balanced comparability between the SIRI groups by creating 1 : 1 matched study groups. Results: the receiver operating characteristic curve analysis identified an optimal SIRI cutoff at 1.9 for OS (AUC: 78.8%; sensitivity: 73.7%; specificity: 70.7%) and PFS (AUC: 80.5%; sensitivity: 75.8%; specificity: 72.9%) and we grouped the patients into two PSM cohorts: SIRI < 1.9 (N = 304) and SIRI >= 1.9 (N = 304), respectively. The SIRI >= 1.9 cohort had significantly worse median OS (P= 1.9) cohorts had the best and worst outcomes, respectively, with SIRI-2 cohort (IIIB and SIRI >= 1.9 or IIIC and SIRI < 1.9) being remained in between (P= 1.9 independently prognosticated significantly worse OS and PFS results and plated the stage IIIB/C patients into three fundamentally distinct prognostic groups., NA

AIMS JAK2V617F (JAK2), calreticulin (CALR) and MPL515L/K (MPL) mutations are important in essential thrombocythemia (ET) and may be associated with various clinical consequences of the disease. This study aimed to compare the clinical and haematological parameters of ET patients regarding the mentioned mutations and the role of plateletcrit (PCT). METHODS Seventy patients who were diagnosed with ET between 2005 and 2017 in a single centre were included in this descriptive study. The initial symptoms and clinical findings were retrieved from the electronic database. JAK2 gene V617F mutations, MPL gene exon 10 mutations and CALR gene exon 9 DNA sequence analyses were performed. Forty-one healthy volunteers were included to perform ROC curve analysis for interpreting PCT value. RESULTS The distributions of patients according to the mutations were as follows: Thirty-seven (52.9%) patients were JAK2-positive, 15 (21.4%) were CALR-positive, 2 (2.8%) patients were positive for both CALR and JAK2, and 1 (1.4%) was only MPL-positive. Fifteen (21.4%) patients were triple-negative. The ET patients with JAK2 mutation showed a higher level of haemoglobin at the time of diagnosis. The ET patients with CALR mutation presented with higher platelet and LDH levels (P = .002 and P = .001, respectively). The PCT level was higher in the CALR-positive group when compared to the others (P = .026). A sensitivity value of 97.6% and specificity value of 98.6% were determined regarding PCT% at a cut-off value of 0.37 in ET patients. In CALR-positive patients, the sensitivity and specificity values were 100% for the PCT at a cut-off value of 0.42%. CONCLUSION We determined that the platelet count and blood LDH level was high in the ET patient group with CALR mutation. Besides, we found that the blood haemoglobin level was higher in the ET patient group with JAK2 mutation. Additionally, the PCT level was higher in the CALR group when compared to the other patient groups.

Background: we investigated the prognostic significance of pretreatment systemic inflammation response index (SIRI) in locally advanced pancreatic carcinoma (LAPC) patients treated with concurrent chemoradiotherapy (CRT). Methods: present retrospective cohort analysis investigated consecutive 154 LAPC patients who received radical CRT. The SIRI was defined as:SIRI=neutrophilxmonocyte/lymphocyte counts. Ideal SIRI cutoff(s) influencing overall survival (OS) and progression-free survival (PFS) results were sought by using receiver operating characteristic (ROC) curve analysis. The primary endpoint was the interaction between the SIRI and OS results. Results: the median follow-up, PFS, and OS durations were 14.3 (range: 2.9-74.6), 7.9 [%95 confidence interval (CI): 5.7-10.1), and 14.7 months (%95 CI: 11.4-18.0) for the entire cohort, respectively. ROC curve analyses determined the ideal SIRI cutoff that exhibiting a significant link with OS and PFS outcomes at the rounded 1.6 point (AUC: 74.3%; sensitivity: 73.8%; specificity: 70.1%).The SIRI = 1.6 patients (N=96), respectively. Although the N0 (versus N1; P90 U/mL) appeared as the other significant associates of better OS and PFS in univariate analyses, yet the results of multivariate analyses confirmed the SIRI, NA

The aim of this study is to evaluate the association between seven important H. pylori virulence factors and antibiotic resistance in patients with gastritis. H. pylori strains isolated from 33 patients with gastritis were examined. Antimicrobial susceptibilities were tested by GenoType® HelicoDR (Hain Life Science, Germany) test kit and RT-PCR. The virulence-factors were determined using conventional PCR. 39% of patients were resistant for clarithromycin and 27% of patients were resistant for fluoroquinolone. 15% of patients were resistant to both clarithromycin and fluoroquinolone. The H. pylori vacA m1/s2 genotype was the most frequent allelic combination. Patients were possessed the vacA s1, m1 (6.1%); s1, m2 (6.1%); s2, m1 (15.1%); and s2, m2 (3.0%) genotypes. 94% of patients with gastritis were positive for H. pylori napA gene. Also, there were no dupA gene-positive gastritis patients. There was no significant correlation between the vacA, cagA, oipA, hpaA, babA, napA, dupA, ureA, ureB virulence genes, clarithromycin, and fluoroquinolone resistance. Herein, we report that the relationship between the H. pylori napA gene and gastritis. Although we found a correlation between H. pylori virulence factor and clinical outcome, there is a need for further studies to enlighten the relation between H. pylori virulence genes and antibiotic resistance.

Purpose: we aimed to retrospectively investigate the prognostic worth of pretreatment advanced lung cancer inflammation index (ALI) in locally advanced nasopharyngeal carcinoma (LA-NPC) patients treated with concurrent chemoradiotherapy (C-CRT). Patients and methods: A total of 164 LA-NPC patients treated with cisplatinum-based definitive C-CRT were included in this retrospective cohort analysis. The convenience of ideal pre-C-CRT ALI cut-offs affecting survival results was searched by employing the receiver operating characteristic (ROC) curve analyses. The primary endpoint was the link between the ALI groups and overall survival (OS), while cancer-specific survival (CSS), locoregional progression-free survival [LR(PFS)], distant metastasis-free survival (DMFS), and PFS comprised the secondary endpoints. Results: the ROC curve analyses distinguished a rounded ALI cut-off score of 24.2 that arranged the patients into two cohorts [ALI ≥ 24.2 (N = 94) versus, NA

Introduction. The most important result of head trauma, which can develop with a blunt or penetrating mechanism, is traumatic brain injury. Tranexamic acid (TXA) can be used safely in multiple trauma. Recent studies showed that TXA can be useful in management of intracerebral hemorrhage, especially in reducing the amount of bleeding. The TXA given in the first 3 hours has been shown to reduce mortality. The aim of our study was to evaluate the effectiveness of tranexamic acid used in patients with traumatic brain injury. Method. Patients with trauma in the emergency room between January 2012 and January 2020 were screened in this retrospective study. The inclusion criteria were being over the age of 18 years, tranexamic acid administration in the emergency department, and traumatic brain injury on brain computerized tomography (CT) and control CT imaging after 6 hours. Results. The number of study patients was 51. The median score of GCS was 12.00 (8.00–15.00). Subdural hemorrhage and subarachnoid hemorrhage were the most common findings on brain CT scans. In the group TXA treatment for less than 1 hour, the arrival MAP was low and the pulse was high (p=0.022 and p=0.030, respectively). All the patients were admitted with multiple trauma. None of the 51 patients had thrombotic complications and died due to head injury. Conclusion. TXA appears to be a safe drug with few side effects in the short term in head injuries. According to our experience, it comes to mind earlier in multiple trauma, especially in head trauma with pelvic trauma.

Aim. The aim of this study is to detect the prevalence of substance use among tenth-grade students; their thoughts, attitudes, behaviors, and tendencies towards substance use; and risk factors of substance use in tenth-grade students in general. Methods. This study is descriptive and cross-sectional conducted between April and May 2016. Research population consists of tenth-grade students in 2015-2016 school year in the city of Ordu. Since the study involved all tenth-grade students, no sampling was done. Questions on substance use were prepared by Ordu Public Health Directorate and the authors by making use of European School Survey Project on Alcohol and Other Drugs (ESPAD) study questions, AMATEM’s “Drugs and Addiction Youth Survey” study conducted on May 1996, and scientific studies conducted previously on similar subjects. Results. 9825 tenth-grade students in 88 schools from 19 counties in the city of Ordu were included in the study. 8714 of the students participated in the survey. Being male, being over the age of 15, mother and father being separated, living with relatives, being in low income, negative feelings about school, perception of being unsuccessful in school, failing a year, absenteeism, and not being content with life are the risk factors for substance use. Conclusions. The tendency of illegal substance use becoming more and more prevalent especially among youth requires the development of new treatment strategies.