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OBJECTIVE: This study investigated the correlation between dental calculi grading and renal stone burden grading. PATIENTS AND METHODS: This study was designed as an observational single-center study and included consecutive patients with radiologically confirmed renal stones at our center between January 2022 and July 2022. These patients were referred to the dentistry clinic for a dental examination to assess dental calculi and oral hygiene. Investigated parameters included demographic characteristics, renal stone location and diameter, urine pH, and dental evaluation findings (teeth brushing habits, oral hygiene, and dental calculi). Renal stone burden grade and dental calculi grade were calculated, and Spearman’s rank-order correlation analysis was used for correlation analyses. RESULTS: Overall, 204 patients were included. The mean patient age was 36.3±15.2. Approximately half of the patients (49.2%) had multiple stones. About 36% of the participants had high-grade dental calculi, while 29.4% had intermediate low-grade dental calculi. Oral hygiene was significantly associated with dental calculi grade (p<0.001). The dental calculi grade was positively and moderately correlated with the renal stone diameter (Spearman’s rho=0.493, p<0.001). Among patients with a renal stone diameter greater than 20 mm, intermediate to high-grade dental calculi were found in 88.4%. This proportion was 49.1% for those with a renal stone diameter smaller than 20 mm. CONCLUSIONS: Dentists should consider the presence of undiagnosed kidney stones in patients with especially intermediate or highgrade dental calculi. Urologists should know that patients with large and multiple kidney stones may have dental calculi. [ABSTRACT FROM AUTHOR]

Background and Purpose: Workplace ostracism is the situation in which the employee is rejected and ignored by the employees in the same workplace. Since this is a situation that prevents the success of the employees, it affects the performance of the employee. In this respect we assume that it is an important mediator in terms of experiencing psychological resilience in this relationship. In our research model, we also suggested that cyberloafing will affect the severity of the relationship between workplace ostracism and resilience. Therefore, we suggested that psychological resilience would have a mediator role and cyberloafing would have a moderating role in the relationship between workplace ostracism and employee performance, based on the opinions of employees.

Abstract. Objective:. To investigate the effects of spontaneous labor before elective repeat cesarean delivery (ERCD) on short-term maternal and neonatal outcomes. Methods:. This was a prospective cohort study. All consecutive ERCDs, occurring at ≥37 weeks of gestation between July 1, 2017 and December 31, 2019 in Makassed General Hospital, were evaluated. The maternal and neonatal outcomes of 183 laboring women undergoing unscheduled repeat cesarean delivery (URCD) group were compared with those of 204 women undergoing cesarean delivery (CD) without spontaneous labor (ERCD) group. Primary outcomes were “composite adverse maternal outcome” and “composite adverse neonatal outcome.” Fisher's exact and Student's t tests were used to assess the significance of differences in dichotomous and continuous variables, respectively. Two logistic regression models were constructed to identify risk factors with most significant influence on the rate of composite adverse maternal and neonatal outcomes. Results:. “Composite adverse maternal outcome” was significantly more common in women who underwent spontaneous labor ((40/183) 21.9% vs. (19/204) 9.3%, P = 0.001, relative risk (RR): 2.7, 95% confidence interval (CI): 1.50–4.90). Similarly, “composite adverse neonatal outcome” was significantly increased in the URCD group ((24/183) 13.1% vs. (12/204) 5.9%, P = 0.014, RR: 2.4, 95% CI: 1.18–4.98). These adverse effects persisted after adjustment for confounders. Multivariate regression models revealed that, besides labor, CD-order impacted maternal outcome (RR: 1.5, 95%CI: 1.02–2.30, P = 0.036), while CD-order and teenage pregnancy influenced neonatal outcome (RR: 2.1, 95%CI: 1.29–3.38, P = 0.003, and RR: 16.5, 95%CI: 2.09–129.80, P = 0.008, respectively). Conclusion:. In our study, spontaneous labor before ERCD, including deliveries at term, was associated with adverse maternal and neonatal outcomes, indicating that it is preferable to conduct ERCD before the onset of labor. Screening women with MRCD may identify those at increased risk for spontaneous labor for whom CD could be scheduled 1–2 weeks earlier. Further large prospective studies to assess the effects of such an approach on maternal and neonatal outcomes are strongly warranted.

Background: The aim of this study was to determine the effect of colistin resistance and other predictors on fatality among patients with Klebsiella pneumoniae bloodstream infections (Kp-BSI) and to describe the effect of amikacin and tigecycline on the outcome in an OXA-48 dominant country. Method: This was a retrospective study performed among patients >16 years of age in a tertiary hospital with 465 beds. All cases had ≥1 positive blood culture for K. pneumoniae 48 h after admission. Results: Among 210 patients with Kp-BSI, the 30-day mortality rate after isolation of the microorganism was 58%. The rate of carbapenem resistance was higher (64% vs. 38%, p

ABSTRACTHere, we presented 11 cases with colistin-resistant Pseudomonas aeruginosa infection and co-existence of OXA-48 and NDM-1 in the ST235 high-risk clone. The molecular analyses were performed by Sanger sequencing and RT–PCR. The eight patients (72.7%) had an invasive infection and three (27.3%) had colonization. The 30-day mortality rate was 87.5% (7/8). Three patients (37.5%, 3/8) received colistin therapy before isolation of P. aeruginosa. In the Multilocus sequence typing (MLST) analysis of 11 isolates, eight (72.7%) isolates belonged to P. aeruginosa ST235 clone. All isolates were NDM-1 positive, and nine isolates (81.8%) were found to be positive for both OXA-48 and NDM-1. Sequences of pmrAB and phoPQ revealed numerous insertions and deletions in all isolates. In 10 isolates pmrAB and phoPQ were found to be upregulated. In conclusion, the co-existence of OXA-48 and NDM-1 genes in colistin-resistant P. aeruginosa ST235 high-risk clone indicates the spread of carbapenemases in clinical isolates and highlights need of continuous surveillance for high-risk clones of P. aeruginosa.

Background: Crohn’s disease (CD) and ulcerative colitis (UC) are the two major relapsing conditions of inflammatory bowel diseases. Case Report: A case of Crohn’s disease with orofacial manifestations in a 10 year old girl is described. She had suffered from fever, dysphagia, arthralgia, painful recurrent ulcers of the oral mucosa and swelling of the lower lip lasting over 6 weeks. Clinical examination and the punch biopsy from the buccal mucosa revealed major recurrent aphthous ulcerations. A partial regression and significant relief of lesions were achieved two weeks after the treatment, but the patient suffered from abdominal pain, irregular bowel movements, arthritis, multiple hyperplastic and swollen mucosal folds, after 3 months. The patient was referred to a pediatric gastroenterologist. Esophagogastroduodenoscopy showed pyloric ulcer formation. Abdominal ultrasound showed increased thickening of the ileal wall with multiple enlarged lympadenopathies in the periileal region. Colonoscopy images showed deep ulcers with surrounding erythema. The histopathological examination of biopsies from the terminal ileum and the colon showed basal plasmacytosis, minimal crypt distortions and aphthous ulcerations. The diagnosis of Orofacial Crohn’s disease was made. Exclusive enteral nutrition for 8 weeks, followed by azathiopurine treatment was started with an excellent clinical response on abdominal and oral symptoms. Conclusion: Diagnosis of the disease by dentists and other clinicians through the evaluation of oral clinical findings is very rare. Mucocutaneous and granulomatous lesions of the oral cavity should alert the clinician to pursue an underlying systemic cause. Early communication with a gastroenterologist can help early diagnosis of Crohn’s disease for better patient management and prognosis.

Tyrosinemia type III is the rarest type of tyrosinemia, because of a mutation in 4-OH-phenylpyruvate dioxygenase (HPD). This causes two different types of diseases with different modes of inheritance: tyrosinemia type III and hawkinsinuria. Hawkinsinuria is an autosomal dominant disease, which presents a failure to thrive and metabolic acidosis; however, the liver is not affected. P.A33T heterozygous mutation was reported by Tomoeda et al. to cause hawkinsinuria. This case report will present the first case of an Egyptian-Lebanese male who developed direct hyperbilirubinemia and was found to have tyrosinemia type III, due to elevated tyrosine levels in the blood and tyrosine derivatives in the urine, but genetic testing revealed a P.A33T heterozygous mutation, a cause of hawkinsinuria.

Mesenteric lipoma is a rare benign tumor of mature fat cells. Although generally asymptomatic, it occasionally causes abdominal pain, ileus, and small bowel volvulus, depending on its location and size. A definitive diagnosis can be made by pathological examination. Ultrasonography and abdominal computed tomography show this lesion as a well-defined, homogenous mass with fat density surrounded by a thin capsule. Because of its rare etiologic origin, we report the case of a 2-year-old male presented with progressive abdominal distension and failure to thrive, found to be caused by a mesenteric lipoma.

Introduction: In this multicenter study, we analysed the magnitude of healthcare worker (HCW) [infection control practitioner (ICP), nurses and others] workforce in hospitals participated in the study. Materials and Methods: This study was performed in 41 hospitals (with intensive care units-ICU) located in 22 cities from seven regions of Turkey. We analysed the ICP workforce, nursing and auxiliary HCW (AHCW) workforce in ICUs, number of ICU beds and occupied beds in four different days [two of which were in summer during the vacation time (August 27 and 31, 2016) and two others in autumn (October 12 and 15, 2016)]. The Turkish Ministry of Health (TMOH) requires two patients per nurse in level 3 ICUs, three patients per nurse in level 2 ICUs and five patients per nurse in level 1 ICUs. There is no standardization for the number of AHCW in ICUs. Finally, one ICP per 150 hospital beds is required by TMOH. Results: The total number of ICUs, ICU beds and ICPs were 214, 2377 and 111, respectively in he 41 participated centers. The number ICPs was adequate only in 12 hospitals. The percentage of nurses whose working experience was 2. The number of patients per other HCW was minimum 3.75 and maximum 4.89 on weekdays and on day shift while it was minimum 5.02 and maximum 7.7 on weekends or on night shift. When we compared the number of level 1, 2 and 3 ICUs with adequate nursing workforce vs inadequate nursing workforce, the p value was

Ranula is a mucus extravasation cyst originating from the sublingual gland on the floor of the mouth. Congenital ranula is very rare. We report a case of a 4-month-old girl with a congenital ranula in the floor of mouth. The ranula was treated first by marsupialization, but the cyst recurred after 1 week. Excision of the ranula was done and was successful.

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by the triad of vascular malformations, venous varicosities, and bone and soft-tissue hypertrophy. We present a case of Klippel-Trenaunay syndrome with limb hypertrophy, port-wine stains, lymphangiomas, and venous varicosities in the limbs.

Through an ex vivo experimental study, we aimed to compare the effects of the Ho: YAG laser Virtual Basket (VB™) modulation and a Thulium fiber laser (TFL) on kidney tissue in different environments and using laser configurations. The 100 W Ho: YAG (Cyber Ho, Quanta System, Italy) and 60 W TFL (Fiber Dust, Quanta System, Italy) laser devices were used. The following laser settings were selected: power in the range of 10-60 W, frequency of 20-40 Hz, and energy of 0.5-1-1.5 J. A medium pulse duration of 600 µsec was used for VB™, while short (spdTFL; 50 µsec) and long (lpdTFL; 15,000 µsec) were used for TFL. The tissue's incision depth (ID), vaporization area (VA), coagulation area (CA), total laser area (TLA = VA + CA), surface section (SS), and lateral effect (LE) were measured. In total, 108 experiments were conducted. No statistically significant difference in mean VA, TLA, ID, LE, or SS was observed between VB™, spdTFL, and lpdTFL in the low-power output group in saline (p > 0.05). However, the mean CA was statistically significantly higher for VB™ (p = 0.005). In saline and high-power output group, the mean VA, CA, TLA, LE, and ID were higher when using lpdTFL than other pulse durations (p = 0.001, p = 0.001, p = 0.001, p = 0.006, and p = 0.001, respectively). Similar to lpdTFL, VB™ may provide controlled dissection and incision as well as haemostasis. At different laser settings, the individual effects of laser properties (such as pulse length, energy and frequency) on tissue may be more significant., (© 2024. The Author(s), under exclusive licence to Springer-Verlag London Ltd., part of Springer Nature.)

For patients with inborn errors of immunity (IEI) and other inborn diseases, mixed donor chimerism is a well-accepted outcome of hematopoietic stem cell transplantation (HSCT). Cytoreductive chemotherapy for a secondary malignancy is a potential challenge for the stability of the graft function after HSCT. We report on a boy with X-SCID who developed Ewing sarcoma ten years after HSCT which was successfully treated with cytoreductive chemotherapy, surgery and local radiation. Surprisingly, this treatment had a positive impact on mixed chimerism with an increase of donor-cell proportions from 40% for neutrophils and 75% for non-T-mononuclear cells (MNCs) to >90% for both. T-cell counts remained stable with 100% of donor origin. This is -to our knowledge- the first report on the impact of cytoreductive chemotherapy on post-HSCT mixed chimerism and provides an important first impression for future patients., Competing Interests: Declaration of competing interest None., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Graft failure requires urgent salvage HSCT, but there is no universally accepted approach for this situation. We investigated T-cell replete haploidentical HSCT with post-transplantation cyclophosphamide following serotherapy-based, radiation-free, reduced intensity conditioning in children with non-malignant disorders who had rejected their primary graft. Twelve patients with primary or secondary graft failure received T-cell replete bone marrow grafts from haploidentical donors and post-transplantation cyclophosphamide. The recommended conditioning regimen comprised rituximab 375 mg/m 2 , alemtuzumab 0.4 mg/kg, fludarabine 150 mg/m 2 , treosulfan 20-24 g/m 2 and cyclophosphamide 29 mg/kg. After a median follow-up of 26 months (7-95), eleven of twelve patients (92%) are alive and well with complete donor chimerism in ten. Neutrophil and platelet engraftment were observed in all patients after a median of 18 days (15-61) and 39 days (15-191), respectively. Acute GVHD grade I was observed in 1/12 patients (8%) and mild chronic GVHD in 1/12 patients (8%). Viral reactivations and disease were frequent complications at 75% and 42%, respectively, but no death from infectious causes occurred. In summary, this retrospective analysis demonstrates that a post-transplantation cyclophosphamide-based HLA-haploidentical salvage HSCT after irradiation-free conditioning results in excellent engraftment and overall survival in children with non-malignant diseases., (© 2021. The Author(s).)

Complete remission from recurrent EBV-positive lymphoma is not mandatory before HSCT to achieve long-term cure in a patient suffering from a recently described immunodeficiency affecting the T-cell coactivation molecule 4-1BB., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The main purpose of this study was to identify the concentrations of naturally occurring radionuclides ( 226 Ra, 232 Th and 40 K) and some metals (Fe, Ni, Cu, Zn, Pb and Cd) in tooth samples collected from humans living in the Rize province of Turkey. It was found that the activity concentrations ranged from 8.1 to 114.51 Bq kg -1 for 226 Ra, from 10.44 to 97.3 Bq kg -1 for 232 Th and from 14.53 to 489.27 Bq kg -1 for 40 K. The mean activity concentration values of 226 Ra and 232 Th in tooth samples were higher than the average world values for bones. Furthermore, when the metal concentrations were examined, there was no regular increase or decrease according to age or sex. The results of the metal analysis on the tooth samples were compared with the results of similar studies and were generally found to be consistent.

Combined immune deficiency due to athymia in patients with complete DiGeorge syndrome can be corrected by allogeneic thymus transplantation. Hypoparathyroidism is a frequent concomitant clinical problem in these patients, which persists after thymus transplantation. Cotransplantation of allogeneic thymus and parental parathyroid tissue has been attempted but does not achieve durable correction of the patients' hypoparathyroidism due to parathyroid graft rejection. Surprisingly, we observed correction of hypoparathyroidism in one patient after thymus transplantation. Immunohistochemical analysis and fluorescence in situ hybridization confirmed the presence of allogeneic parathyroid tissue in the patient's thymus transplant biopsy. Despite a lack of HLA-matching between thymus donor and recipient, the reconstituted immune system displays tolerance toward the thymus donor. Therefore we expect this patient's hypoparathyroidism to be permanently cured. It is recognised that ectopic parathyroid tissue is not infrequently found in the thymus. If such thymuses could be identified, we propose that their use would offer a compelling approach to achieving lasting correction of both immunodeficiency and hypoparathyroidism., (© 2019 The American Society of Transplantation and the American Society of Transplant Surgeons.)

In the present study, the concentration levels of heavy metals such as Cr, Fe, Ni, Cu, Zn, As and Pb in soil samples collected from 88 sampling locations around Sinop Province, Turkey were measured using energy dispersive X-ray fluorescence spectroscopy (EDXRF). To interpret and to evaluate the pollution status and distribution of heavy metals in soil, metal pollution parameters such as enrichment factor (EF), geo-accumulation index (I geo ), pollution factor (CF) and pollution load index (PLI) and geo-spatial distribution patterns were used. The mean concentrations of Cr, Fe, Ni, Cu, Zn, As, and Pb were found to be 194.73, 39,848.57, 85.02, 43.19, 65.10, 5.66, and 17.01 mg/kg, respectively. Results indicated that the mean concentrations of Cr, Ni, As, and Pb exceeded the world crustal average, with the exception of Fe, Cu, and As. Multivariate analysis results showed that Cr, Ni, Zn, As, and Pb levels in the investigated region were highly influenced by anthropogenic inputs such as agricultural practices. According to the health risk assessment model introduced by USEPA to evaluate the human health risks, the non-carcinogenic risk for children was above the threshold level, but low for adults. Total potential carcinogenic health risks for both children and adults in the study area were in acceptable range. Overall, when health risks are evaluated, it shows that children are more susceptible to non-carcinogenic and carcinogenic health effects of trace metals compared to adults., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

This study makes a first attempt at a detailed estimation of the background radioactivity level and its distribution at the Sinop nuclear power plant site. The activity concentration levels of 226 Ra, 232 Th, 40 K and 137 Cs radionuclides in soil samples collected from 88 locations around Sinop Province, Turkey, in November 2016, were measured using gamma spectrometry. The distributions of radionuclide levels obtained from the results were evaluated using a geostatistical method, and the estimated radiation levels were determined using the ordinary kriging (OK) method, which is the best linear unbiased estimator (BLUE) for unmeasured points. Estimates of distribution results were evaluated using cross-validation diagrams, and it was shown that the OK method could predict radiological distributions for appropriate criteria. Finally, using the kriging parameters, distributions of radiation levels for the entire work area were mapped at a spatial resolution of 100 × 100 m 2 . These maps show that the natural radionuclides ( 226 Ra, 232 Th and 40 K) are distributed at higher levels to the southeast of Sinop than in the other regions, and the activity of an artificial radionuclide ( 137 Cs) is high in the interior and northern sections.

Major histocompatibility complex class II (MHC II) is essential for adaptive immune response. We recently reported on disturbed adaptive mucosal immunity due to MHC II deficiency and prolonged enteropathy. Here, we share medical history, flow cytometric analysis of blood lymphocytes, immunohistopathology, and fecal analysis of seven genetically confirmed patients with MHC II deficiency suffering from enteropathy. Data on flow cytometric analysis of HLA-DR expression on monocytes and B cells before hematopoietic stem cell transplantation (HSCT) and after in-vitro stimulation is shown. The course of immune reconstitution after HSCT of MHC II deficient patients in comparison to severe combined immunodeficiency (SCID) patients is described. In addition, immunohistopathology illustrating CD4 and CD8 T cell infiltration, absence of B lymphocytes and plasma cells, and disturbed immunoglobulin expression in the gut as well as absent HLA-DR expression in the liver is shown. Furthermore, data from fecal analysis such as stool fat, nitrogen, and water fraction as well as faecal markers such as alpha-1-antitrypsin, pancreas specific elastase 1, eosinophilic protein X (EPX), and beta defensin 2 are presented. Altogether this data demonstrates the complex phenotype of MHC II deficiency. The data can be valuable for researchers interested in mucosal immunity. For further interpretation of the data presented in this article, please see the research article "Persisting enteropathy and disturbed adaptive mucosal immunity due to MHC class II deficiency" (Posovszky et al., 2019).

Purpose: The purpose of our study was to conduct a chemical analysis of extracranial foreign bodies (FBs) causing artifacts in cranial magnetic resonance imaging (MRI) and to investigate the association between chemical composition, magnetic susceptibility, and artifact size., Materials and Methods: A total of 12 patients were included in the study. The FBs responsible for the artifacts were visualized using cranial computed tomography (CT). Artifact-causing FBs were removed from the scalps of 10 patients and analyzed using scanning electron microscope with energy dispersive spectroscopy (SEM-EDS), X-ray diffraction spectroscopy (X-RD), and Fourier-transform infrared spectroscopy (FT-IR). The magnetic susceptibility of the samples was determined using the reference standard material MnCl 2 .6H 2 O. The volume of the MRI artifacts was measured in cubic centimeters (cm 3 )., Results: EDS results demonstrated that the mean Fe ratio was 5.82% in the stone samples and 0.08% in the glass samples. Although no phase peaks were detected in the X-RD spectra of the glass samples, peaks of Fe 2 O 3 , Al 2 Ca (SiO 4 ) were detected in the X-RD spectra of the stone samples. The FT-IR spectra revealed metal oxide peaks corresponding to Fe, Al, in the stone samples and peaks confirming Al 2 SiO 5 and Na 2 SiO 3 structures in the glass samples. The mean volumes of the MRI artifacts produced by the stone and glass samples were 5.9 cm 3 and 2.5 cm 3 , respectively., Conclusions: Artifacts caused by extracranial FBs containing metal/metal oxide components are directly associated with their chemical composition and the artifact size are also related to element composition and magnetic susceptibility., Competing Interests: There are no conflicts of interest., (Copyright: © 2019 Indian Journal of Radiology and Imaging.)

Intestinal epithelial cells (IECs) form a fundamental mucosal barrier and actively participate in tolerance and immunity against intestinal contents. Major histocompatibility complex class II (MHC II) and invariant chain (Ii) molecules are essential for adaptive immune response. MHC II deficiency often presents with gastrointestinal disorders. Intestinal biopsy samples revealed an absence of HLA-DR, Ii, and local immunoglobulins in both hematopoietic immune cells and IECs accompanied by a lack of faecal sIgA. After successful hematopoietic stem cell transplantation (HSCT) absent HLA-DR and Ii expression persisted in IECs and faecal stool analysis indicated inflammation and high microbial activity. We describe multifaceted disturbance of adaptive mucosal immunity in MHC II deficient patients suffering from enteropathy. HLA-DR and Ii expression on enterocytes is not restored by HSCT. This may account for increased susceptibility to enteric infections and intestinal inflammation leading to prolonged enteropathy reported in MHC II deficient patients., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Osteopetrosis (OP) is a rare disease caused by defective osteoclast differentiation or function. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment available in the infantile "malignant" form of OP. Improved clinical and genetic diagnosis of OP has seen the emergence of a cohort of patients with less severe and heterogeneous clinical presentations. This intermediate form of OP does not call for urgent intervention, but patients accumulate debilitating skeletal complications over years and decades, which are severe enough to require curative treatment and may also require intermittent transfusion of blood products. Here we present data from 7 patients with intermediate OP caused by mutations in TCIRG1 (n = 2), CLCN7 (n = 2), RANK (n = 1), SNX10 (n = 1), and CA2 (n = 1), who were transplanted between the ages of 5 to 30 years (mean, 15; median, 12). Donors were matched siblings or family (n = 4), matched unrelated (n = 2), or HLA haploidentical family donors (n = 1). Conditioning was fludarabine and treosulfan based. All 6 patients transplanted from matched donors are currently alive with a follow-up period between 1 and 8 years at time of publication (median, 4 years) and have demonstrated a significant improvement in symptoms and quality of life. Patients with intermediate OP should be considered for HSCT., (© 2019 by The American Society of Hematology.)

The aim of this study was to determine the activity concentrations and radiological hazard parameters of 137 Cs and 40 K in soft tissue samples of sea snails (Rapana venosa) collected from 12 different stations in the East Black Sea coastal area of Turkey. The values in soft tissues of sea snail samples with three different sizes were between 0.31 and 1.79 Bq kg -1 for 137 Cs and 163.9 and 269.4 Bq kg -1 for 40 K. The mean activity concentrations of 137 Cs and 40 K in tissues of small-sized sea snails were determined to be higher than those of 137 Cs and 40 K in soft tissues of the other sizes. The determined activity concentration values were lower than the concentrations reported in similar studies. Radiological hazard parameters such as the daily intake (D int ) of 137 Cs and 40 K, annual committed effective dose (E eff ), and lifetime cancer risk (LCR) were calculated and compared with the international recommended values., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Infantile malignant osteopetrosis (IMO) is a rare, lethal, autosomal recessive disorder characterized by nonfunctional osteoclasts. More than 50% of the patients have mutations in the TCIRG1 gene, encoding for a subunit of the osteoclast proton pump. The aim of this study was to develop a clinically applicable lentiviral vector expressing TCIRG1 to correct osteoclast function in IMO. Two mammalian promoters were compared: elongation factor 1α short (EFS) promoter and chimeric myeloid promoter (ChimP). EFS promoter was chosen for continued experiments, as it performed better. IMO osteoclasts corrected in vitro by a TCIRG1-expressing lentiviral vector driven by EFS (EFS-T) restored Ca 2+ release to 92% and the levels of the bone degradation product CTX-I to 95% in the media compared to control osteoclasts. IMO CD34 + cells from five patients transduced with EFS-T were transplanted into NSG mice. Bone marrow was harvested 9-19 weeks after transplantation, and human CD34 + cells were selected, expanded, and seeded on bone slices. Vector-corrected IMO osteoclasts had completely restored Ca 2+ release. CTX-I levels in the media were 33% compared to normal osteoclasts. Thus, in summary, evidence is provided that transduction of IMO CD34+ cells with the clinically applicable EFS-T vector leads to full rescue of osteoclasts in vitro and partial rescue of osteoclasts generated from NSG mice engrafting hematopoietic cells. This supports the continued clinical development of gene therapy for IMO.

This study investigated the contents of Cu, Zn and Pb in seawater, sediment, different shell sizes of mussel (Mytilus galloprovincialis) and sea snail (Rapana venosa) samples collected from four different provinces of the Eastern Black Sea Region. With the exception of Zn, all the metal concentration values measured in the sea snail were observed to be higher than those of mussels in all stations. While the correlation between mussels and sea snail according to metal concentrations was found to be positive (p˂0.05), this relation was not observed between the other parameters, such as the shell sizes, salinity and pH (p˃0.05). Although the mean concentration values of Cu, Zn, and Pb for mussel and sea snail are significantly above the tolerable levels, the estimated daily intake values for mussel were below the daily intake recommended., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

We report 5 individuals in 3 unrelated families with severe thrombocytopenia progressing to trilineage bone marrow failure (BMF). Four of the children received hematopoietic stem cell transplants and all showed poor graft function with persistent severe cytopenias even after repeated transplants with different donors. Exome and targeted sequencing identified mutations in the gene encoding thrombopoietin ( THPO ): THPO R99W, homozygous in affected children in 2 families, and THPO R157X, homozygous in the affected child in the third family. Both mutations result in a lack of THPO in the patients' serum. For the 2 surviving patients, improvement in trilineage hematopoiesis was achieved following treatment with a THPO receptor agonist. These studies demonstrate that biallelic loss-of-function mutations in THPO cause BMF, which is unresponsive to transplant due to a hematopoietic cell-extrinsic mechanism. These studies provide further support for the critical role of the MPL-THPO pathway in hematopoiesis and highlight the importance of accurate genetic diagnosis to inform treatment decisions for BMF., (© 2017 by The American Society of Hematology.)

Seawater, sediment and fish (anchovy) samples consumed in the Rize province of the Eastern Black Sea region of Turkey were collected from five different stations. The radioactivity levels ( 226 Ra, 232 Th, 40 K and 137 Cs) were determined in all the samples using a high-purity germanium detector. While 226 Ra, 232 Th and 40 K radionuclides were detected in all samples, the radionuclide concentration of 137 Cs, except for the sediment samples (mean activity is 9±1.4Bqkg -1 ), was not detected for the seawater and fish samples. The total annual effective dose rates from the ingestion of these radionuclides for fish were calculated using the measured activity concentrations in radionuclides and their ingested dose conversion factor. Also, the concentrations of some heavy metals in all the samples were determined. The activity and heavy metal concentration values that were determined for the seawater, sediment and fish samples were compared among the locations themselves and with literature values., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Innate immunity recognizes microbial nucleic acids by endosomal TLR and cytosolic recognition systems. Despite increasing knowledge on the properties of nucleic acid recognition for viral RNA and bacterial DNA, little is known about the immunogenicity of prokaryotic RNA. Here we show that bacterial RNA is a potent trigger for type-I IFN secretion in human PBMC. Activation of human plasmacytoid dendritic cells was dependent on endosomal maturation and could be blocked by a TLR7-specific inhibitor. Murine plasmacytoid dendritic cells from TLR7-deficient mice were unresponsive to bacterial RNA. Surprisingly, in myeloid DC, TLR were dispensable for TNF-alpha and IL-12 induction by bacterial RNA. Even non-immune stroma cells were able to mount a NF-kappaB response upon triggering with bacterial RNA. Retinoic-acid inducible gene I and melanoma-differentiation-associated gene 5 could be ruled out to be responsible for this reactivity. Although the inflammasome adaptor protein, apoptosis-associated speck-like protein, and a functional type-I IFN receptor were necessary for IL-1beta secretion in myeloid DC, these proteins were dispensable for TNF-alpha and IL-12 induction by cytosolic bacterial RNA. Our results show that besides of activation of TLR7 and inflammasomes, bacterial RNA activates additional cytosolic receptors similarly as has been reported for recognition of bacterial DNA.