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In the Qur'an, it is reported that the mountains, which are described as piles that nailed the Earth for the earth to stand still without shaking, were created as balance elements. In addition, to that, mountains have added material value to human life and have an important role because they function as natural water tanks and contain many natural riches in them. Mountains have an important function in the history of religions with the spiritual value they carry. The mountains, which are considered as sacred centers, also stand out as the places where revelation descends. They are also seen as places endowed with divine power manifested by God. The mountains, which are the symbol of power and Glory, are manifested as the power that emphasizes the greatness of Allah with their height and glory and are shown as examples at the point of breaking man's arrogance. Except for this, the mountains, which are mentioned in the Qur'an in other features, were offered trust, but they refused to undertake it being afraid thereof. They felt awe and feared from the revelation of the Qur'an. Moses was honored with revelation on the mountain, and the mountain was chosen for him as a place of the covenant with Allah. Likewise, the mountain hosted the first revelation to the Prophet. The mountains, which are the symbol of Allah's greatness, have been used in the Qur'an to form nuance with various words in different contexts. These mentioned features of the mountains brought about the formation of metaphorical interpretations in the understanding of the verses about them. In this article, the conceptual area of the mountain in the Qur'an and the metaphorical interpretations that make themselves clear in the Sufi explanation will be given. [ABSTRACT FROM AUTHOR]

Objective: Acne-like skin lesions and more severe disease course in males suggest a role for sex hormones in the pathogenesis of Behçet disease (BD). HLA-B51 is the main genetic susceptibility factor for BD, and CYP21A2 gene responsible for most of congenital adrenal hyperplasia (CAH) is located within the MHC locus on chromosome 6p21.3. We aimed to investigate the possible role of 21-hydroxylase deficiency in linkage disequilibrium with HLA-B51 and causing androgen excess. Materials and Methods: We studied 18 healthy controls, 29 BD patients and 15 patients with ankylosing spondylitis (AS). All subjects underwent ACTH stimulation test. Basal and stimulated serum cortisol and 17-OH-progesterone (17-OH-P) levels and basal dehydroepiandrosterone sulfate (DHEA-S), total testosterone and sex hormone binding globulin (SHBG) levels were measured. Results: According to current guidelines, we accepted 10 ng/mL as the cut-off point for 17-OH-P to define non-classic CAH (NCAH). After ACTH stimulation 3 of BD patients (10.3%) and 5 AS patients (33.3%) had high 17-OH-P concentrations. Those individuals were considered as NCAH or possible carriers for CAH mutations. Three out of 8 BD patients with prominent acne were identified as NCAH biochemically. Mean total testosterone levels of BD patients were significantly lower than those of healthy controls, however these levels were normal in BD patients with high 17-OH-P. Conclusion: This preliminary work documented high 17-OH-P levels following ACTH stimulation in a subset of BD and AS patients, and genetic studies are necessary to investigate the role of 21-hydroxylase deficiency in association with HLA-B alleles in their pathogenesis. [ABSTRACT FROM AUTHOR]

15N labeled soluble metallo- and metal-free phthalocyanines are described for the first time. The complexes were synthesized starting from phthalic anhydride derivatives using 98% 15N enriched urea. The effects of the substitution pattern, aggregation, and coordinated metal on 15N chemical shifts in liquid state NMR were studied. [ABSTRACT FROM AUTHOR]

The recent advances in the synthesis of carborane and boronic ester-substituted tetrapyrroles (porphyrins, porphyrazines, and phthalocyanines) are reviewed together with their possible utilization as agents for photodynamic therapy (PDT) and boron neutron capture therapy of cancer (BNCT) as well as dual PDT/BNCT sensitizers. [ABSTRACT FROM AUTHOR]

Abstract: 4-(4-Formyl-3-hydroxyphenoxy)phthalonitrile (3) and its condensation product with 2-aminophenol have been synthesized to reach 4-(3-hydroxy-4-(((2-hydroxyphenyl)imino)-methyl)phenoxy)phthalonitrile (5), a tridentate ligand possessing ONO binding sites. Subsequent condensation of phenylboronic acid with 5 afforded a novel boronic ester of a Schiff base with a phthalonitrile group (7). Boronate 7 displays high stability and can be handled in air due to the presence of coordinative B–N and covalent B–O bonds in its structure. The novel compounds were characterized by elemental analyses, IR, UV–vis, mass, 1H-, 13C- and 11B NMR spectra. [Copyright &y& Elsevier]

Objective: To assess the indications and distribution of cases chosen for termination of pregnancy and reasons delaying until third trimester termination. Methods: Retrospective study of cases between 2002 and 2006 in the hospital council. Cases were divided in two groups , as early termination (<23 weeks of gestation) and late termination (≥23 weeks of gestation). All pregnant women who underwent termination were classified according to related systemic pathology and chorionicity. Reasons for delaying until third trimester termination were evaluated in four groups. Results: During this five year period 1.449 complicated pregnancies were counseled and in 713 cases termination was offered. Of 677 cases (94.95%) with termination, 412 cases (60.09%) had early and 265 cases (39.91%) late termination. The most frequent indications were central nervous system abnormalities (51.7%), chromosomal abnormalities (11.7%), and urogenital abnormalities (8.4%). The main reason for delaying termination was failure of screening by ultrasound (65.6%). Conclusion: Systematic screening for fetal anomalies is the main step for prevention of affected pregnancies. Information given to parents for TOP is important, but the decision for TOP is influenced by many factors. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the sonographic characteristics of fetuses with trisomy 18 and trisomy 13. Materials and Methods: From March 2002 to December 2006, we reviewed the database and medical records of 25 cases with trisomy 18 and trisomy 13. The subjects were recruited from pregnant women undergoing prenatal sonographic examinations at 13-28 weeks of gestation and subsequently proven trisomy 18 or 13. The results of ultrasound findings were reviewed in these cases with chromosomes confirmed as trisomy 18 and/or 13. Results: All cases had at least one abnormal sonographic finding. The common sonographic findings included choroid plexus cysts, abnormal head shape, cardiac anomalies, holoprosencephaly with associated facial anomalies, abnormal feet and/or hands, especially polydactyly, clenched hand, omphalocele. Non-structural abnormal findings such as polyhydroamnios or fetal growth restriction were seen in less than one third of the fetuses. Discussion: Nearly all fetuses with trisomy 18 or 13 had characteristic sonographic patterns of abnormalities demonstrated at midpregnancy. Detailed ultrasound at midpregnancy could effectively screen fetuses with trisomy 18 or 13 for further genetic testing. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the sonographic characteristics of fetuses with trisomy 18 and trisomy 13. Materials and Methods: From March 2002 to December 2006, we reviewed the database and medical records of 25 cases with trisomy 18 and trisomy 13. The subjects were recruited from pregnant women undergoing prenatal sonographic examinations at 13-28 weeks of gestation and subsequently proven trisomy 18 or 13. The results of ultrasound findings were reviewed in these cases with chromosomes confirmed as trisomy 18 and/or 13. Results: All cases had at least one abnormal sonographic finding. The common sonographic findings included choroid plexus cysts, abnormal head shape, cardiac anomalies, holoprosencephaly with associated facial anomalies, abnormal feet and/or hands, especially polydactyly, clenched hand, omphalocele. Non-structural abnormal findings such as polyhydroamnios or fetal growth restriction were seen in less than one third of the fetuses. Discussion: Nearly all fetuses with trisomy 18 or 13 had characteristic sonographic patterns of abnormalities demonstrated at midpregnancy. Detailed ultrasound at midpregnancy could effectively screen fetuses with trisomy 18 or 13 for further genetic testing. [ABSTRACT FROM AUTHOR]

Objective: We have aimed to evaluate perinatal outcome of selective fetocide in monochorionic diamniotic twins discordant for major fetal anomalies. Materials and Methods: The study group included nine cases of monochorionic diamniotic twin (triplet) pregnancies discordant for major fetal anomalies including anencephaly (n=2), inencephaly (n=1), lumbar meningomyolocele (n=1), hydrops fetalis (n=1), acardiac twin (n=2), twin-to-twin transfusion syndrome (stage III, n=1) and monochorionic triamniotic triplet discordant for anencephaly (n=1). For bipolar coagulation of the umbilical cord, we used 3.5 mm laparoscopic trocar and 3.0 mm bipolar forcep. The procedures were performed under the guidance of transabdominal ultrasonography. Results: The mean gestational age of the cases at intervention and at delivery were 20 weeks (R=16-24) and 34 weeks (R=22-38), respectively. The procedure of bipolar coagulation was carried out in mean 20 min (R=15-40 min) without maternal complication. One case was aborted at 20 weeks of gestation because of preterm premature rupture of membrane after the procedure. Another case was delivered before 32 weeks, at 28+4 week of gestation, and died six months postnatally which was unrelated to the intervention. The other seven cases were delivered after 32 gestational weeks. In eight cases, the procedures were performed without fetal complication. Perinatal outcome was as 88.8% (n=8/9) live births. Postnatal ages of surviving seven babies were between 8-40 months, and they have been growing up as healthy. Discussion: Ultrasonographic guided bipolar cord coagulation as a selective fetocide can be succesfully performed to increase the survival rate or prevent morbidity of healthy co-twin in the cases of monochorionic diamniotic (triplets), discordant major fetal anomalies candidate for selective termination, and should be offered as a choice of prenatal therapy after the parents were informed of the intervention or follow up. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the prenatal findings, associated anomalies, and prognosis of right aortic arch (RAA) anomalies. Material and Methods: All cases referred for detailed ultrasonography and fetal echocardiography between October 2006 and July 2009 were systematically examined for aortic arch anomalies and associated cardiac and extracardiac anomalies. Prenatal findings of all cases with aortic arch anomalies and intracardiac and extracardiac findings were prospectively registered in an electronic database that included fetal echocardiography. Outcomes of the cases were collected postnatally from the patients' obstetricians, neonatal unit archieves, and pediatric cardiologists. Results: We detected 12 cases of RAA (0.37%; n=12/3200). Mean gestational age at diagnosis was 24 weeks (range, 21-33 weeks). Of the 12 cases of RAA, five (41.7%) had a major cardiac defect, including tetralogy of Fallot (n= 3), atrioventricular septal defect (n=1), and ventricular septal defect (n=1). An extracardiac anomaly was observed in three cases (25%). The fetal karyotype was trisomy 21 in one case with increased nuchal translucency (6.6 mm). Microdeletion 22q11 analyses performed in three cases were normal. The postnatal courses of the cases with isolated RAA were uneventful. Two cases associated with major cardiac and extracardiac anomalies were lost in the early neonatal period. The case of trisomy 21 was terminated. The other four cases of RAA with an associated cardiac anomaly are currently in follow up. Conclusion: Aortic arch anomalies, particularly RAA, can be diagnosed by fetal echocardiography. The prognosis for isolated RAA is relatively good compared with that for RAA with associated anomalies. [ABSTRACT FROM AUTHOR]

Background/aim: In this prospective study, we aimed to investigate the association of serum (s) and urine (u) IP-10, galectin-9, and SIGLEC-1 with disease activity in patients with systemic lupus erythematosus (SLE). Materials and methods: Sixty-three patients with active SLE (31 renal, 32 extrarenal) were included. Thirty patients with inactive SLE (15 renal, 15 extrarenal), 17 with renal active AAV, and 32 healthy volunteers were selected as control groups. Serum and urine IP-10, galectin-9, and SIGLEC-1 were tested using ELISA. Results: Levels of sIP-10 (p = 0.046), uIP-10 (p < 0.001), sGalectin-9 (p = 0.03), and uSIGLEC-1 (p = 0.006) were significantly higher in active SLE group compared to the inactive SLE; however, no differences were detected in the comparison of uGalectin-9 (p = 0.18) and sSIGLEC-1 (p = 0.69) between two groups. None of the biomarkers discriminated patients with active renal SLE from active extrarenal SLE. ROC analyses revealed an AUC of 0.63 (0.52--0.73) for sIP-10, 0.78 (0.68--0.86) for uIP-10, 0.64 (0.53--0.74) for sGalectin-9, and 0.68 (0.57--0.77) for uSIGLEC-1 in discriminating disease activity in SLE, which did not outperform C3 (0.75, 0.64--0.84) and C4 (0.72, 0.61--0.82). sIP-10 (p = 0.001), uIP-10 (p = 0.042), and uGalectin-9 (p = 0.009) were significantly increased in patients with active renal SLE compared to active renal AAV. sGalectin-9 (p < 0.001) and sIP-10 levels (p = 0.06) were decreased after 8 (5--22.5) months of treatment. Conclusion: sIP-10, uIP-10, sGalectin-9, and uSIGLEC-1 reflect global disease activity in SLE but do not outperform C3 and C4. sIP-10 and uIP-10 may be specific for active SLE compared to active AAV. sIP-10 and sGalectin-9 might be valuable in monitoring response after treatment. [ABSTRACT FROM AUTHOR]

Background/aim: The aim of this study is to evaluate the baseline F18-FDG PET/CT findings of individuals diagnosed with giant cell arteritis (GCA) and to explore its association with clinical findings and classification criteria. Materials and methods: We analysed data from patients who underwent F18-FDG PET/CT scans to investigate large vessel (LV) involvement between 2010 and 2019. Only patients with a clinical diagnosis of GCA and at least 6 months of follow-up were included. We compared initial clinical features and laboratory findings based on the presence of LV vasculitis on PET/CT and the maximum standard uptake value (SUVmax) of vascular territories. Results: Twenty-nine patients (median age at diagnosis: 70, F/M: 24/5) were included in the study. Among them, 21 patients (72.4%) presented with cranial symptoms, while 8 patients (27.5%) had isolated LV-GCA. Twenty-two patients (75.9%) met the ACR/EULAR 2022 GCA classification criteria. LV vasculitis was detected on PET/CT in 23 patients (79.3%). A positive correlation was observed between SUVmax in the thoracic aorta and both CRP and ESR levels (r = 0.50, p = 0.026 and r = 0.63, p = 0.002, respectively). PET/CT positive patients were found to be younger (p = 0.016) and more frequently female (p = 0.017). They also exhibited fewer headaches (56.5% vs. 100%, p = 0.04), experienced fewer flares during follow-up (p = 0.03), and had a lower cumulative glucocorticoid dose at the 6th month (p = 0.036). Comparison of PET/CT-positive patients (n = 23) based on the fulfilment of the ACR/EULAR 2022 classification criteria revealed that patients who met these criteria were older (p = 0.02) and had significantly lower CRP levels at diagnosis (p = 0.02). Conclusion: The performance of F18-FDG PET/CT in diagnosing LV involvement in GCA is favourable, and the severity of FDG uptake in the vessel wall correlates with the acute phase response. Patients with extracranial involvement on PET/CT exhibit distinct features, including a younger age and female predominance. Additionally, these patients appear to experience fewer relapses and require lower doses of glucocorticoids. However, the clinical significance of PET/CT in patients who met ACR/EULAR classification criteria, predominantly consisting of patients with ischemic cranial symptoms, could not be determined in our study. [ABSTRACT FROM AUTHOR]

Background/aim: B-cell depletion with rituximab (RTX) is widely used as a rescue therapy in patients with systemic sclerosis (SSc). The aim herein was to analyze the progress of disease-related outcomes after RTX therapy in severe SSc patients. Materials and methods: Included in this study were 27 SSc patients who were followed-up between 2012 and 2020 and received at least 1 cycle of RTX for active disease, despite receiving standard immunosuppressives (ISs). In addition to the European Scleroderma Study Group and European Scleroderma Trials and Research Group activity scores, Medsger's severity, and the recently developed Scleroderma Clinical Trials Consortium Damage Index values were evaluated initially and at 1 year after the first infusion. The progress of individual organ damage was also assessed at the end of the follow-up period (at least 6 months after the last infusion) using the data extracted from the medical records. Results: Disease activity and severity-improved and disease-related overall damage worsened after the first year of RTX therapy (p < 0.001, p = 0.008, and p = 0.005). Some of the disease-related organ damage had improved at the end of the follow-up period, indicating its reversibility. Overall damage scores =11 after the first year of RTX therapy were found to be associated with mortality (p = 0.035). Conclusion: RTX contributed to reducing the activity and severity in SSc patients with severe disease, nonetheless the efficacy related to the damage was limited. High damage scores in the first year were found to be associated with mortality. Spontaneous progress of manifestations requiring a longer period to improve and irregular consecutive RTX courses might lead to difficulties in differentiation between activity and damage. [ABSTRACT FROM AUTHOR]

Methylphenidate (MPH) is the most preferred drug for treatment of the attention deficit hyperactivity disorder (ADHD). Here, we aimed to discuss the possible effects and mechanisms of MPH on precocious puberty (PP) via a case series with seven children who had normal body mass index. In this case series we evaluated seven children with ADHD, who had received MPH for at least 6 months (0.5 mg/kg/dose three times a day, maximum 60 mg) and admitted to Department of Pediatric Endocrinology with PP symptoms. The mean age was 8.16 years. Basal hormonal levels (luteinizing hormone [LH], follicle stimulating hormone, and estrogen/testosterone) were within normal range. Results of LH-releasing hormone stimulation tests demonstrated central pubertal responses. Glutamine, dopamine and noradrenaline are most important excitatory neurotransmitters that have a role at the beginning of puberty. The effect of MPH, cumulating dopamine and noradrenaline in the synaptic gap could be associated with the acceleration of puberty with the excitatory effect of dopamine’s gonadotropin-releasing hormone (GnRH) release, excitatory effect of noradrenaline’s GnRH release and the disappearance of GnRH receptor expression suppressor effect on prolactin disinhibitory effect. [ABSTRACT FROM AUTHOR]

Background/aim: In this cross-sectional study, it was aimed to test the predictive value of noncriteria antiphospholipid antibodies (aPL) in addition to the global antiphospholipid syndrome score (GAPSS) in predicting vascular thrombosis (VT) in a cohort of patients with APS and aPL (+) systemic lupus erythematosus (SLE). Material and methods: This study included 50 patients with primary APS, 68 with SLE/APS, and 52 with aPL (+) SLE who were classified according to VT as VT ± pregnancy morbidity (PM), PM only or aPL (+) SLE. Antiphospholipid serology consisting of lupus anticoagulant (LA), anticardiolipin (aCL) immunoglobulin G (IgG)/IgM/IgA, antibeta2 glycoprotein I (aß2GPI) IgG/IgM/IgA, antiphosphatidylserine/prothrombin (aPS/PT) IgG/IgM and antidomain-I (aDI) IgG was determined for each patient. The GAPSS and adjusted GAPSS (aGAPSS) were calculated for each patient, as previously defined. Logistic regression analysis was carried out with thrombosis as the dependent variable and high GAPSS, aCL IgA, aß2GPI IgA, and aDI IgG as independent variables. Results: The mean GAPSS and aGAPSS of the study population were 11.6 ± 4.4 and 9.6 ± 3.8. Both the VT ± PM APS (n = 105) and PM only APS (n = 13) groups had significantly higher GAPSS and aGAPSS values compared to the aPL (+) SLE (n = 52) group. The patients with recurrent thrombosis had higher aGAPSS but not GAPSS than those with a single thrombotic event. The computed area under the receiver operating characteristic curve demonstrated that a GAPSS =13 and aGAPSS =10 had the best predictive values for thrombosis. Logistic regression analysis including a GAPSS =13, aCL IgA, aß2GPI IgA, and aDI IgG showed that none of the factors other than a GAPSS =13 could predict thrombosis. Conclusion: Both the GAPSS and aGAPSS successfully predict the thrombotic risk in aPL (+) patients and aCL IgA, aß2GPI IgA, and aDI IgG do not contribute to high a GAPSS or aGAPSS. [ABSTRACT FROM AUTHOR]

Background: The interleukin-1 receptor antagonist (IL-1Ra) is a crucial molecule that counteracts the effects of interleukin-1 (IL-1) by binding to its receptor. A high concentration of IL-1Ra is required for complete inhibition of IL-1 activity. However, the currently available Escherichia coli-expressed IL-1Ra (E. coli IL-1Ra, Anakinra) has a limited half-life. This study aims to produce a cost-effective, functional IL-1Ra on an industrial scale by expressing it in the pyrG auxotroph Aspergillus oryzae. Results: We purified A. oryzae-expressed IL-1Ra (Asp. IL-1Ra) using ion exchange and size exclusion chromatography (53 mg/L). Sodium dodecyl sulfate–polyacrylamide gel electrophoresis (SDS-PAGE) analysis revealed that Asp. IL-1Ra is N-glycosylated and approximately 17 kDa in size. We conducted a comparative study of the bioactivity, binding kinetics, and half-life between Asp. IL-1Ra and E. coli IL-1Ra. Asp. IL-1Ra showed good bioactivity even at a low concentration of 0.5 nM. The in vitro half-life of Asp. IL-1Ra was determined for different time points (0, 24, 48, 72, and 96 h) and showed higher stability than E. coli IL-1Ra, despite exhibiting a 100-fold lower binding affinity (2 nM). Conclusion: This study reports the production of a functional Asp. IL-1Ra with advantageous stability, without extensive downstream processing. To our knowledge, this is the first report of a recombinant functional and stable IL-1Ra expressed in A. oryzae. Our results suggest that Asp. IL-1Ra has potential for industrial-scale production as a cost-effective alternative to E. coli IL-1Ra. [ABSTRACT FROM AUTHOR]

Objective The skin pathergy test (SPT) is an important tool in the diagnosis of Behçet disease (BD), but its decreasing sensitivity over years has resulted in less frequent use in the clinical practice. This study aimed to improve the sensitivity of the SPT without compromising its specificity. Methods BD patients, patients with other inflammatory diseases, recurrent aphthous stomatitis, and healthy controls comprised the study group. The SPT was conducted using 20G needle and 21G lancet pricks, or with additional application of 23-valent polysaccharide pneumococcal vaccine (PS-23), Alum, or ATP to the prick site. Development of erythema and induration at 24 h/48 h were evaluated by the same observer. Induration (≥2 mm) with erythema at 48 h was accepted as a positive reaction. Proinflammatory cytokine production following stimulation with lipopolysaccharide or PS-23 was investigated by whole-blood assay (WBA) in a subgroup. Results Stimulation of the forearm skin by PS-23 and a 20G needle prick showed the highest sensitivity and specificity in BD (64.3% and 100%, respectively), especially in patients with active disease (80.3% and 100%, respectively), compared with a sensitivity of 4.8% in all and 6.1% in active patients using a single 20G prick. A positive result was associated with active disease and no use of immunosuppressives. In WBA, increased IL-1β and IL-1Ra production in response to PS-23 was observed in the group with active BD, while the cytokine response to lipopolysaccharide was similar in all groups. Conclusions The SPT conducted using a 20G needle prick and PS-23 antigens was shown to be a promising tool for the diagnosis of BD owing to its improved sensitivity compared with the standard approach. [ABSTRACT FROM AUTHOR]

A new phthalonitrile precursor with tyrosine substituent and the zinc phthalocyanine derived from it were synthesized and their interaction with diphenylborinic acid was studied. The new compounds were characterized by using elemental analysis, IR, 1 H and 11 B-NMR and UV-VIS spectral data. 11 B-NMR data demonstrated that boron atom attached to the phthalonitrile precursor is in a bulky tetra-coordinated state with the formation of a coordinative N-B bond while completely planar phthalocyanine moieties with high aggregation tendencies at the concentrations used in NMR measurements lead the borinic ester side of the molecule into a planar tri-coordinated form. Moreover, the consequences of the presence of diphenylborinic ester groups on the spectral, photophysical and photochemical properties of the new zinc phthalocyanine are investigated in detail. [ABSTRACT FROM AUTHOR]

Objective To provide outcome data concerning pregnancies exposed to the Interleukin-1 (IL-1) inhibitors prior to conception in both men and women, during pregnancy and breast feeding. Methods Retrospective data were collected from members of the International Society for Systemic Autoinflammatory diseases and collated in a single centre. A uniform data collection sheet was used to obtain standardized data including maternal age and diagnosis, type, duration of and response to IL-1 blockade, pregnancy duration, delivery, mode of feeding and neonatal development. Results There were 31 maternal-exposed pregnancies from seven countries and we report the first data on paternal exposure: six to anakinra and five to canakinumab, with no negative outcomes. We also report the first data on canakinumab-exposed pregnancies: eight pregnancies that resulted in the delivery of seven healthy infants of normal gestational age and birthweight. There were 23 anakinra-exposed pregnancies resulting in the birth of 21 healthy infants, and one baby with unilateral renal agenesis and ectopic neurohypophysis. There were two first trimester miscarriages affecting a mother with active disease. There were no serious neonatal infections. Fourteen infants were breast fed with no complications. There were no reports of developmental delay, with follow-up of up to 10 years (median 18 months). Conclusion This series substantially increases the published experience of IL-1 blockade and reproduction including the first data on canakinumab and on paternal exposure to these agents. Data are generally reassuring, although the case of renal agenesis is the second reported in an anakinra-exposed pregnancy. [ABSTRACT FROM AUTHOR]

Objective: Multisystem Inflammatory Syndrome (MIS) is a condition seen in the early post-COVID-19 period and thought to develop with an impaired immune response. It has been usually reported in children but rarely in adults. Here we report the first adult MIS (MIS-A) case series from Turkiye. Material and Methods: Six patients who met the Centers for Disease Control and Prevention’s MIS-A diagnostic criteria were included in the study. The demographic, clinical, laboratory, radiological characteristics and therapy regimes and outcomes of the patients were recorded. Results: All of our cases had a history of mild COVID-19. They presented with fever, severe fatigue and hypotension. Abnormal echocardiography findings were detected in five patients. Only one patient had multiple mucocutaneous findings. Common laboratory features were lymphopenia, markedly increased C-Reaktive Protein, procalcitonin, pro-brain natriuretic peptide (pro-BNP), D-dimer, and ferritin. All patients had positive SARSCoV-2 antibody result. Corticosteroids and/or anakinra were used in five, and intravenous immunoglobulin was used in two patients. Low-molecular-weight heparin (LMWH) was used for all cases. Empirically initiated antibiotic treatments were discontinued after cultures were negative. After anti-inflammatory treatment, the hypotension of the patients resolved, they did not need intensive care follow-up and no mortality was seen in our cases. Conclusions: MIS-A is a severe and mortal condition that causes various clinical pictures and can be confused with sepsis. Anakinra, a recombinant IL-1 receptor antagonist, is a significant agent that can be used in the treatment of MIS-A since it blocks the cytokine cascade at an early stage. The satisfactory responses will be obtained with early diagnosis and anti-inflammatory treatment. In this period when the pandemic is not over yet, it is necessary to increase the awareness of clinicians about MIS-A, which can be fatal. [ABSTRACT FROM AUTHOR]

In order to realize the photocatalytic reactions by visible light more efficiently, the photocatalytic activity of polycrystalline TiO 2 samples impregnated with carboxy functionalized Co(II) or Zn(II)-phthalocyanine as heteregoneous photocatalyst has been investigated. For this purpose, metallophthalocyanines(MPc) substituted with mercapto benzoic acid groups on each benzo moiety were incorporated into TiO 2 semiconductors to prepare TiO 2 –MPc composites as heterogeneous catalysts. TiO 2 and TiO 2 –MPc composites were characterized by making use of X-ray diffraction (XRD), infrared spectroscopy (FTIR) and UV–vis diffuse reflectance spectroscopy. The characterization results show that MPcs are anchored on the surface of TiO 2 through CO–O–TiO 2 bonds. The optimum loading of the dyes on TiO 2 were 0.49 μmol/g TiO 2 for ZnPc and 0.56 μmol/g TiO 2 for CoPc, nearly independent of TiO 2 used. These novel heterogeneous photocatalysts were used for the degradation of 4-chlorophenol (4-CP) in aqueous media in the presence of visible irradiation monitored by GC–MS spectroscopy. The catalysts revealed enhanced activity in visible region for the degradation of 4-CP when compared with that of pure TiO 2 , which is attributed to the cooperative function of the two components of the photocatalyst. The amount of remaining 4-chlorophenol residue was determined by using an internal standard (phenol) with gas chromatography–mass spectrometry and during the first 30 minutes 99% degradation of 4-chlorophenol was observed. [ABSTRACT FROM AUTHOR]

Background/aim: Remdesivir, which was first developed for the treatment of Ebola disease but failed to meet expectations, has become hope in the fight against the COVID-19 pandemic. This study aimed to evaluate risk factors for mortality and prognosis of adult moderate/severe COVID-19 patients treated with remdesivir, and safety and tolerability of 5 days of remdesivir treatment. Materials and methods: This multicenter prospective observational study was conducted in 14 centers in Turkey. Pregnancy or breastfeeding, multiorgan failure, or usage of vasopressors for septic shock, ALT > 5 x the upper limit of the normal range, or eGRF <30 mL/min or dialysis and receiving favipiravir were the exclusion criteria of the study. Results: Among 500 patients, 494 patients were included in the study. On admission, 392 (79.3%) patients had moderate and 102 (20.6%) patients had severe COVID-19. The 28-day mortality was 10.1%. The median of the scores of the seven-category ordinal scale assessed on days 0, 3, 5, 7 were 4 and 3 on day 14. When the survival status of the patients was evaluated according to the time between the remdesivir start date and the end date of the symptoms, no statistically significant difference was found between the medians of the groups (p = 0.404). In multivariable analysis, age (OR, 1.05; 95%CI, 1.02-1.08; p = 0.003), SpO2 level on admission (OR, 3.03; 95%CI, 1.35-6.81; p = 0.007), heart rate (OR, 2.48; 95%CI, 1.01-6.07; p = 0.047), follow-up site at the hospital (clinic/ICU) (OR, 26.4; 95%CI, 11.6-60.17; p < 0.001) were independently associated with increased mortality. Grade 3 adverse event (AE) was observed in 4 (0.8%) patients. None of the patients experienced grade 4 or 5 AEs. Conclusion: Remdesivir is a safe and well-tolerated drug and older age, low SpO2 level on admission, tachycardia, and ICU admission are independently associated with increased mortality among patients with moderate/severe COVID-19 receiving remdesivir treatment. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to examine the impact of smoking on axial spondyloarthritis (axSpA) patients taking tumor necrosis factor inhibitors (TNFi). Materials and Methods: Our study consisted of 211 patients who were diagnosed with axSpA and received TNFi treatment in the rheumatology outpatient clinic. The patients were evaluated retrospectively, cross-sectionally and grouped by intensity of smoking (pack-years). Those who smoked>20 pack-years were defined as heavy smokers. Groups were compared in terms of physical examination, laboratory values and disease evaluation indexes (Bath Ankylosing Spondylitis Metrology Index (BASMI)), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), Ankylosing Spondylitis Quality of Life (ASQoL) and Beck Depression Inventory (BDI) scores. Mann-Whitney U and Spearman correlation analysis tests were used for data analysis Results: Comparison of the smoker (n=121) and non-smoker groups (n=90) revealed BASMI was lower in the non-smoker group (p=0.04). Smoking intensity correlated with BDI (r=0.323, p<0.001), BASDAI (r=0.257, p=0.005), BASMI components (lomber lateral flexion (LLF) (r=-0.303, p=0.001), cervical rotation (CR) (r=-0.232, p=0.012), and tragus wall distance (TWD) (r=0.27, p=0.003)). Multivariate analysis revealed an association between the pack-years of smoking and the BASMI [regression coefficient (B)=0.067, standard error (SE)=0.22, 95%CI=0.02, 0.10; p=0.003], Accordbaseline (B=pretreatment)-last value (L.=posttreatment) difference of BASFI [B=-0.063, SE=0.02, 95%CI=-0.10, -0.20; p=0.003], of BASDAI [B=-0.047, SE=0.02, 95%CI=-0.08, -0.007; p=0.026], ASQoL [B=-0.125, SE=0.04, 95%CI=0.04, -0.20; p=0.003]. In heavy smokers, significant worsening was found in LLFL (p=0.01), CRL (p=0.04), TWDL (p=0.001), BASFIL (p=0.035) and BASMIL (p=0.001). Significant differences were found in the baseline (B) and last (L) BASDAI (p=0.042), BASFIL (p=0.002), BASFIB-L (p=0.07) and BASMI (p=0.03) values in the nonradiograpic-axSpA group in heavy smokers, compared to the AS group. Conclusion: Our study showed that smoking, especially heavy smoking, has a negative effect in every phase of axSpA. Smoking intensity may correlate with reduced response to TNFi. [ABSTRACT FROM AUTHOR]

Budd Chiari syndrome (BCS) is defined as obstruction of hepatic venous outflow that can be located anywhere from small hepatic venules up to the entrance of inferior vena cava (IVC) into right atrium. Etiologies of BCS include myeloproliferative disorders, congenital, and acquired hypercoagulable states. Anticoagulation is the mainstay of treatment for all cases of BCS with a demonstrable hypercoagulable state. Interventional radiology procedures such as transjugular intrahepatic portosystemic shunting (TIPS) can be utilized to reduce portal hypertension and to improve complications related to portal hypertension. We present a patient with systemic lupus erythematosus who first presented with fever, weight loss, malar rash, alopecia, livedo reticularis, symmetric polyarthritis, pancytopenia, and class IV lupus nephritis when she was 23 years old. After receiving an induction treatment of cyclophosphamide and glucocorticoids, she received a maintenance treatment of azathioprine. She presented with ascites and abdominal pain when she was 36 and the abdominal imaging revealed reduced calibration of hepatic venules and intrahepatic segment of inferior vena cava. Lupus anticoagulant was positive and anti cardiolipin IgM and IgG were positive. Work up for hereditary hypercoagulable states was negative. Thus, the diagnosis was secondary antiphospholipid syndrome where BCS was the first clinical manifestation of the antiphospholipid syndrome. Patient was anticoagulated with warfarin and received diuretics for ascites. After the ascites became refractory to diuretics and the patient had multiple vertebral compression fractures due to volume overload secondary to ascites, she was successfully treated with TIPS. When control imaging was performed, 50% of stenosis was observed in the stent. Balloon dilation of the stent was performed. Interventional radiology techniques like TIPS can be used in BCS patients secondary to APS, in cases when medical treatment is insufficient to control complications of portal hypertension. In BCS patients secondary to APS, TIPS enables clinical improvement but due to the presence of endothelial dysfunction in APS patients, there is a risk of shunt dysfunction secondary to thrombosis or stenosis secondary to intimal hyperplasia. Therefore, strict anticoagulation and regular follow up of patients after TIPS is recommended. In cases with stent stenosis, reintervention may be necessary. [ABSTRACT FROM AUTHOR]

Abstract: Metal-free and Co(II)-phthalocyanines carrying eight dodecaborate substituents (96 boron atoms) on the peripheral positions were synthesized in a multistep reaction sequence. The first step of the preparation of metal-free and Co(II) target phthalocyanines was cyclotetramerization of 4-(3,5-dimethoxyphenoxy)phthalonitrile in pentanol in the presence of lithium pentanolate and Co(II) salt, respectively. After cleavage of methyl groups the reaction of (Bu4N)[B12H11O(CH2CH2)2O] with hydroxyphenoxy substituted phthalocyanines gives the novel phthalocyanines 7, 8 with eight dodecaborate units. The new phthalocyanine compounds were characterized by NMR, FT-IR and UV–VIS spectral data. The electrochemical investigations of cobalt(II) phthalocyanine together with Zn(II) derivative having identical substituents were studied. [Copyright &y& Elsevier]

Abstract: The synthesis of novel, hexadeca-substituted metal-free and zinc(II)phthalocyanines bearing naphthoxy groups and chloro groups in peripheral positions and hexyloxy groups in non-peripheral positions is described. These phthalocyanines were characterized with elemental analyses, mass, proton nuclear magnetic resonance (1H NMR), fourier transform infrared spectroscopy (FT-IR) and ultraviolet–visible spectroscopy (UV–vis) techniques. Aggregation properties of these compounds were investigated in the different concentration range. The influences of the substituent on the hexadeca-substituted phthalocyanine framework and metal ion on the spectroscopic and photophysical properties have been investigated. The energy transfer to phthalocyanine core and radiative decays of the naphthol emission and phthalocyanine core were examined. [Copyright &y& Elsevier]

LINKED CONTENT This article is linked to Al‐Ani et al papers. To view these articles, visit https://doi.org/10.1111/apt.15779 and https://doi.org/10.1111/apt.15940 [ABSTRACT FROM AUTHOR]

Budd Chiari syndrome (BCS) is defined as obstruction of hepatic venous outflow that can be located anywhere from small hepatic venules up to the entrance of inferior vena cava (IVC) into right atrium. Etiologies of BCS include myeloproliferative disorders, congenital, and acquired hypercoagulable states. Anticoagulation is the mainstay of treatment for all cases of BCS with a demonstrable hypercoagulable state. Interventional radiology procedures such as transjugular intrahepatic portosystemic shunting (TIPS) can be utilized to reduce portal hypertension and to improve complications related to portal hypertension. We present a patient with systemic lupus erythematosus who first presented with fever, weight loss, malar rash, alopecia, livedo reticularis, symmetric polyarthritis, pancytopenia, and class IV lupus nephritis when she was 23 years old. After receiving an induction treatment of cyclophosphamide and glucocorticoids, she received a maintenance treatment of azathioprine. She presented with ascites and abdominal pain when she was 36 and the abdominal imaging revealed reduced calibration of hepatic venules and intrahepatic segment of inferior vena cava. Lupus anticoagulant was positive and anti cardiolipin IgM and IgG were positive. Work up for hereditary hypercoagulable states was negative. Thus, the diagnosis was secondary antiphospholipid syndrome where BCS was the first clinical manifestation of the antiphospholipid syndrome. Patient was anticoagulated with warfarin and received diuretics for ascites. After the ascites became refractory to diuretics and the patient had multiple vertebral compression fractures due to volume overload secondary to ascites, she was successfully treated with TIPS. When control imaging was performed, 50% of stenosis was observed in the stent. Balloon dilation of the stent was performed. Interventional radiology techniques like TIPS can be used in BCS patients secondary to APS, in cases when medical treatment is insufficient to control complications of portal hypertension. In BCS patients secondary to APS, TIPS enables clinical improvement but due to the presence of endothelial dysfunction in APS patients, there is a risk of shunt dysfunction secondary to thrombosis or stenosis secondary to intimal hyperplasia. Therefore, strict anticoagulation and regular follow up of patients after TIPS is recommended. In cases with stent stenosis, reintervention may be necessary. [ABSTRACT FROM AUTHOR]

Background/aim: Systemic lupus erythematosus (SLE) is an autoimmune disease with a variety of organ/system involvement. Respiratory system involvement is common in these patients and usually manifests itself by disorders of the lung parenchyma, pleura, pulmonary vasculature or diaphragm. In this study, we sought to determine the frequency of interstitial lung disease (ILD) in patients with SLE and associated risk factors. Materials and methods: Three hundred randomly chosen patients with SLE were included. Chest x-ray (CXR), lung spirometry and carbon monoxide diffusion test (DLCO) were performed. High-resolution thorax computed tomography (HRCT) was performed for a definite diagnosis of ILD. Results: Of 300 patients, 16% had ILD. At the start of the study, the prevalence obtained from the patients’ records showed that 4% had ILD. The median age, mean duration of disease, and follow-up time were significantly higher and longer in patients with ILD compared to patients without (p < 0.05). Forced expiratory volume (FEV1), forced vital capacity (FVC), DLCO and total lung capacity (TLC) were significantly lower in patients with ILD (p < 0.001). Patients with ILD had a significantly higher frequency of arthritis, serositis, Raynaud’s phenomenon, myositis, and anti-Scl70 positivity (p = 0.01, 0.001, 0.02, 0.004, and 0.001, respectively). A significantly higher number of patients had stopped using hydroxychloroquine (HCQ) in the ILD group (p = 0.04). Conclusion: ILD is common in patients with SLE. Spirometry, diffusion tests, and CXR are simple but valuable tools to diagnose ILD in patients with SLE. Considering the significant difference of prevalence between the start and the end of the study, one of the possibilities is the underrecognition of SLE-associated interstitial pulmonary disease. The higher administration of immunosuppressives in these patients may support a multisystemic active disease including the lungs. [ABSTRACT FROM AUTHOR]

Abstract: Phthalocyanines (M=Co, Zn or 2H) with four ({4-[(Z or E)-phenylazo]-1-naphthyl}oxy) substituents on the periphery have been synthesized to enlarge the absorbing range of the dyestuffs. Cyclopalladation of the azobenzene groups lead to network-type oligomeric products by formation of binuclear palladium (II) complexes and they have been further converted into monomeric species by treatment with acetylacetonate. The electronic spectra clearly indicate the absorptions resulting from phenylazo and naphthyl groups along with the Q and B bands of the phthalocyanines. The consequence of the palladation is a relatively intense broad absorption due to LMCT with maxima around 520nm. [Copyright &y& Elsevier]

Background/aim: COVID-19 pandemic created concerns among patients receiving immunosuppressive therapy. Frequency of COVID-19 and impact of lockdown on treatment compliance in patients with vasculitis are largely unknown. Materials and method: Patients with ANCA-associated and large vessel vasculitis that have been followed-up in our clinic were contacted by phone and a questionnaire containing home isolation status, treatment adherence and history of COVID-19 between March 1st and June 30th, 2020 was applied. Results: The survey was applied to 103 patients (F/M: 59/44, mean age: 53.2±12.5). Thirty-three (32%) patients didn’t attend at least one appointment; 98(95.1%) noted that they spent 3 months in home isolation. Five patients (4.8%) received immunosuppressives irregularly and 3(2.9%) developed symptoms due to undertreatment. Four (3.9%) patients admitted to hospital with a suspicion of COVID-19, but none of them had positive PCR or suggestive findings by imaging. COVID-19 was diagnosed in a patient with granulomatosis with polyangiitis during hospitalization for disease flare and she died despite treatment. Conclusion: Frequency of COVID-19 was low in patients with vasculitis in our single center cohort. Although outpatient appointments were postponed in one-third of our patients, high compliance with treatment and isolation rules ensured patients with vasculitis overcome this period with minimal morbidity and mortality. [ABSTRACT FROM AUTHOR]

Objectives Colchicine is the main treatment for FMF. Although a number of individuals with FMF are intolerant/resistant to colchicine, there is no standard definition of colchicine resistance/intolerance. We developed a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF that may serve as a guide for clinicians and health authorities. Methods A set of statements was identified using a modified-Delphi consensus-based approach. The process involved development of an initial colchicine resistance/intolerance-related questionnaire derived from a systematic literature review. The questionnaire, which was completed by an international panel of 11 adult and paediatric rheumatologists with expertise in FMF, was analysed anonymously. The results informed draft consensus statements that were discussed by a round-table expert panel, using a nominal group technique to agree on the selection and wording of the final statements. Results Consensus among the panel was achieved on eight core statements defining colchicine resistance/intolerance in patients with FMF. A definition of resistance was agreed upon that included recurrent clinical attacks (average one or more attacks per month over a 3-month period) or persistent laboratory inflammation in between attacks. Other core statements recognize the importance of assessing treatment adherence, and the impact of active disease and intolerance to colchicine on quality of life. Conclusion Based on expert opinion, a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF were identified to help guide clinicians and health authorities in the management of patients with FMF. [ABSTRACT FROM AUTHOR]

[Display omitted] • Tocilizumab, anakinra and prednisolone did not affect the antibody response to SARS-CoV-2. • The antibody response correlated with and could be predicted by involvement on thoracic CT. • The antibody level also correlated with the level of lung involvement on thoracic CT. Disease severity, previous medications and immunosuppressive agents could affect the antibody response against SARS-CoV-2. This study aimed to analyze variables affecting the humoral response to SARS-CoV-2. This prospective cohort study included adult patients who recovered from COVID-19 and were admitted to a COVID-19 follow-up unit. Eight patient groups were defined in accordance with the results of thoracic computed tomography (CT), SARS-CoV-2 PCR test, and tocilizumab or anakinra use during active disease. Anti-S IgG antibodies were determined by ELISA in serum samples. Anti-S positive and negative cases were compared. A total of 518 patients were included in the study. SARS-CoV-2 IgG antibodies were positive in 82.8% of patients. SARS-CoV-2 PCR positivity, extent of lung involvement on CT, and time to antibody testing were independently associated with antibody positivity. Tocilizumab, anakinra or prednisolone use was not a factor affecting the antibody response. The rate of antibody response and sample/CO values among antibody-positive patients showed a linear relationship with the extent of lung involvement on CT. The use of tocilizumab, anakinra and prednisolone for COVID-19 did not affect the antibody response against SARS-CoV-2. The main driver of antibody response among patients with COVID-19 was the extent of pulmonary involvement on CT. [ABSTRACT FROM AUTHOR]

[Display omitted] • Unsymmetrical A 3 B type Zn(II) and Ti(IV) Pcs are prepared for efficient DSSCs. • Novel dyes have been characterized by spectral, electrochemical and TD-DFT techniques. • In order to increase the solar absorption range, dye cocktails have been prepared with the Pcs and YD2 por dye. • The best conversion efficiencies were found to be 9.96% (YD2 + ZnFPNPc) and 10.72% (YD2 + TiOFPNPc) with cocktail dye (3:1). • The champion DSSC achieves 10.72% under sun and retains 98% of its initial value after 24 h. To increase the efficiency of dye-sensitized solar cells (DSSCs), there is still a need for alternative dyes that can absorb a broad spectrum of sunlight and maintain high conversion efficiencies. In order to meet these requirements, a cocktail dye made up of novel phthalocyanines together with a porphyrin have been designed. The novel unsymmetrical phthalocyanines are two ferrocenylphenoxy-naphthalenephthalocyanine (FPNPc) with Zn (ZnFPNPc) or TiO (TiOFPNPc) at the inner core and 4-ferrocenylphenoxy and 4-carboxybenzo substituents at peripheral positions. In order to increase their UV–Vis absorption capacity, cocktails have been prepared with the synthesized new dyes (ZnFPNPc or TiOFPNPc) and YD2 porphyrin dye. The full characterization of novel synthesized dyes has been performed by UV–Vis absorption, FTIR, H1-NMR, MALDI-TOF, electrochemical, TD-DFT techniques. In this work we have prepared DSSCs based on YD2 and phthalocyanine dye molecule with different cocktail ratio (YD2 + ZnFPNPc or YD2 + TiOFPNPc ratios: 1:1, 1:2, 2:1, 1:3, 3:1, 1:4 and 4:1). The best solar cell performance has been performed in the cases of YD2 + ZnFPNPc (3:1) (9.96%) and YD2 + TiOFPNPc (3:1) (10.72%) cocktail dyes. Due to the high efficiency values obtained, these new cocktail dyes are expected to be a good alternative to the ruthenium-based complex dyes used in DSSCs and pave the way for potential alternative dyes. [ABSTRACT FROM AUTHOR]

In this study, (ethylsulfanyl) porphyrazines have been modified with one peripheral ruthenium bipyridine groups. These compounds possess (bpy)2RuII moieties directly attached to the β position in the (ethylsulfanyl) porphyrazines through thioether chelation. The new compounds have been characterized by elemental analysis, FT-IR, ¹H-NMR spectroscopy and mass spectra. The spectral properties such as electronic spectra, aggregation and fluorescence of compounds have been studied in THF. Insertion of (bpy)2RuII moiety on the porphyrazines structure have been investigated photophysical properties of the complexes. [ABSTRACT FROM AUTHOR]

Aim: Familial Mediterranean Fever (FMF) is the most frequent and historically the oldest autosomal recessive autoinflammatory disorder. No pathogen or auto-antibody has been shown to be associated with FMF. The disorder manifests with bouts of fever and abdominal pain, which are called "attacks". In the present study, we aimed to find the most frequent mutations in the MEFV gene in the Turkish population. Methods: Thousand eight hundred and forty patients with an initial diagnosis of FMF were enrolled in the study. DNA was extracted from the peripheral blood according to the kit protocol. Twenty-two target sequences of the MEFV gene were amplified with polimeraze chain reaction and sequenced with the pyrosequencing method. Results: When the patients were divided into two groups according to age, the mean age of 866 patients was 8.5±7.5 years in <18 age group, and that of 974 patients was 35.4±12.5 years in ≥18 age group. No mutations associated with FMF were found in 547 of patients in <18 age and 479 of the patients in ≥18 age groups. Conclusion: In the present study, we evaluated the frequency of 22 mutations in MEFV in 1840 patients, who had been admitted to the medical genetics division between the years 2014 and 2016 with an initial diagnosis of FMF. Analysis of our findings showed that our data are in agreement with previous publications concerning Turkish population. [ABSTRACT FROM AUTHOR]

Familial mediterranean fever (FMF) and Cryopyrin associated periodic syndromes (CAPS) are two prototypical hereditary autoinflammatory diseases, characterized by recurrent episodes of fever and inflammation as a result of mutations in MEFV and NLRP3 genes encoding Pyrin and Cryopyrin proteins, respectively. Pyrin and Cryopyrin play key roles in the multiprotein inflammasome complex assembly, which regulates activity of an enzyme, Caspase 1, and its target cytokine, IL-1β. Overproduction of IL-1β by Caspase 1 is the main cause of episodic fever and inflammatory findings in FMF and CAPS. We present a unifying dynamical model for FMF and CAPS in the form of coupled nonlinear ordinary differential equations. The model is composed of two subsystems, which capture the interactions and dynamics of the key molecular players and the insults on the immune system. One of the subsystems, which contains a coupled positive-negative feedback motif, captures the dynamics of inflammation formation and regulation. We perform a comprehensive bifurcation analysis of the model and show that it exhibits three modes, capturing the Healthy, FMF, and CAPS cases. The mutations in Pyrin and Cryopyrin are reflected in the values of three parameters in the model. We present extensive simulation results for the model that match clinical observations. [ABSTRACT FROM AUTHOR]

A novel zinc(II) phthalocyanine substituted with four diphenylborinic ester units was synthesized by the reaction of 2,9,16,23-tetra (salicylaldimino)-phthtalocyaninatozinc(II) and triphenylborane in THF at reflux condition. The novel zinc phthalocyanine was characterized by using elemental analysis, IR, 1 H and 11 B-NMR and UV–Vis spectral data. 11 B-NMR data demonstrated that the boron atom is in tetracoordinated state and diphenylborinate substituents have a cyclic structure with a coordinative N–B bond enhancing the stability of this borinated phthalocyanine derivative. Moreover, the consequences of the presence of diphenylborinic ester groups on the spectral, photophysical and photochemical properties of the new zinc phthalocyanine are investigated in detail. [ABSTRACT FROM AUTHOR]

The HLA protein, HLA-B*51, encoded by HLA-B in MHC, is the strongest known genetic risk factor for Behçet disease (BD). Associations between BD and other factors within the MHC have been reported also, although strong regional linkage disequilibrium complicates their confident disentanglement from HLA-B*51. In the current study, we examined a combination of directly obtained arid imputed MHC-region SNPs, directly obtained HLA-B locus types, and imputed classical HLA types with their corresponding polymorphic amino acid residues for association with BD in 1,190 cases and 1,257 controls. SNP mapping with logistic regression of the MHC identified the HLA-B/MICA region and the region between HLA-F and HLA-A as independently associated with BD (P < 1.7 × 10-8). HLA-B*51, -A*03, -B*15, -B*27, -B*49, -B*57, and -A*26 each contributed independently to BD risk. We directly examined rsi 16799036, a noncoding SNP upstream of HLA-B that was recently suggested to underlie the association of HLA-B*51 with BD, but we were unable to replicate that finding in our collection. Instead, we mapped the BD association to seven MHC class I (MHC-I) amino acid residues, including anchor residues that critically define the selection and binding of peptides to MHC-I molecules, residues known to influence MHC-Ikiller immunoglobulin-like receptor interactions, and a residue located in the signal peptide of HLA-B. The locations of these variants collectively implicate MHC-I peptide binding in the pathophysiology of BD. Furthermore, several lines of evidence suggest a role for altered regulation of cellular cytotoxicity in BD pathogenesis. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the natural history of isolated muscular ventricular septal (m-VSD) defects during gestation and up to 1 year postnatally, as well as the association with chromosomal anomalies. Material and Methods: Between August 2007 and July 2012, 76 fetuses with isolated m-VSDs represented the study population. The following variables were evaluated: site and size of the m-VSDs, presence of chromosomal anomalies, pregnancy outcome, and spontaneous closure rate from diagnosis up to 1 year postnatally. Results: Of the 76 cases with m-VSD, 1 fetus died after birth and 31 cases were lost to follow-up after birth. Thus, a total of 44 fetuses reached their first year of postnatal life, and these cases were available for analysis. Three (6.8%) of 44 defects closed spontaneously in utero, 33 (75%) closed within 1 year, and 8 (18.2%) remained patent. Overall, spontaneous closure occurred more frequently in the apical defects, but no significant difference was found for spontaneous closure between the mid-muscular and apical defects (p > 0.05). Also, 83.8% (36 of 44) of defects ≤3 mm closed during gestation or the first year of life. Conclusion: We infer that m-VSDs have a high spontaneous closure rate during the first year of life. Also, small m-VSDs frequently close spontaneously. [ABSTRACT FROM AUTHOR]

The d.c. and a.c. electrical transport properties of Au/Pz/Au devices with various thickness of Pz(octakis[(4-tert-butylbenzylthio)-porphyrazinato]Cu(II)) layer have been investigated. Measurements revealed that, in contrast to previously investigated Au/Pc/Au structures, low voltage d.c. behaviour of the films can be described by the field-lowering mechanisms with a log( J) ∝ V current density-voltage characteristics under forward and reverse bias. For high reverse voltages, the observed ln( J/V) - 1/V characteristics indicated that the origin of conduction mechanism is Fowler-Nordheim tunnelling (FNT). On the other hand, the voltage dependence of current density at the higher forward-voltage region indicates that the mechanism of conduction in Au/Pz/Au devices is space charge limited conduction dominated by exponential trap distribution. A thickness independent barrier height was observed for tunnelling, while the total trap concentration show a general tendency to decrease with increasing film thickness. The a.c. conductivity showed two regions in the ln( σ) - ln( f ) plots having different slopes, leading to the conclusion that for low frequency region, the dominant conduction mechanism is a small polaron tunnelling at all temperatures, whereas for high frequency region, correlated barrier hopping model is the dominant mechanism in the investigated devices. [ABSTRACT FROM AUTHOR]

The effects of substituents and solvents on the photophysical and photochemical parameters of zinc(II) phthalocyanines containing four Schiff s base substituents attached directly and through phenyleneoxy-bridges on peripheral positions are reported. The group effects on peripheral position and the continual and intermittent conjugation of the phthalocyanine molecules on the photophysical and photochemical properties are also investigated. General trends are described for photodegradation, singlet oxygen, and fluorescence quantum yields of these compounds in dimethylsulfoxide (DMSO), dimethylformamide (DMF), and tetrahydrofurane (THF). Among the different substituents, phthalocyanines with cinnamaldimine moieties (1c and 2c) have the highest singlet oxygen quantum yields (ΦΔ) and those with nitro groups (1a and 2a) have the highest fluorescence quantum yields in all the solvents used. The fluorescence of the substituted zinc(II) phthalocyanine complexes is effectively quenched by 1,4-benzoquinone (BQ) in these solvents. [ABSTRACT FROM AUTHOR]

A novel Zn phthalocyanine bearing 4 salicylideniminophenyloxy substituents on peripheral positions was synthesized from the reaction of 4-aminophenoxy substituted phthalocyanine and salicylaldehyde. The condensation reaction of salicylidene Schiff base groups and benzeneboronic acid produced a new phthalocyanine carrying benzeneboronic acid ester derivatives as substituents. Ethoxy groups were bound to boronic acid as a second ligand. The presence of B-N bond interactions enhanced the stability of this boronated phthalocyanine derivative, which exhibited sufficient air stability during the purification and characterization processes. The novel compounds were characterized by using elemental analysis, IR, ¹H-NMR, 11 B-NMR, 13C-NMR, UV-Vis and MALDI-TOF MS spectral data. [ABSTRACT FROM AUTHOR]

Objective: To compare maternal and perinatal outcomes in pregnancies complicated by severe preeclampsia, eclampsia, and HELLP (hemolysis, elevated liver enzyme levels, and low platelets) syndrome. Materials and Methods: Maternal and neonatal charts of 1,222 consecutive pregnancies complicated by severe preeclampsia, eclampsia, or HELLP syndrome at our maternal-perinatal unit were reviewed. Patients were divided into three groups: 903 (73.9%) with severe preeclampsia, 123 (10.1%) with eclampsia, and 196 (16.0%) with HELLP syndrome. Results: The overall incidence of adverse maternal outcome was 5.9%. The rates of adverse maternal outcomes for women with HELLP syndrome and eclampsia were higher than for severe preeclampsia (13.8% vs. 11.4% vs. 3.4%, respectively) (p=0.000). Birth weight was lower in patients with HELLP syndrome than in patients with eclampsia and severe preeclampsia (p=0.005). No significant difference in neonatal morbidity was found among the three groups. Perinatal mortality tended to be higher in the severe preeclampsia group than in the HELLP syndrome and eclampsia groups (p=0.231). Conclusion: Pregnancies complicated by HELLP syndrome had significantly higher maternal morbidity than those with severe preeclampsia and eclampsia. Perinatal and neonatal outcomes in pregnancies complicated by severe preeclampsia, eclampsia, and HELLP syndrome were dependent on gestational age rather than being disease dependent. [ABSTRACT FROM AUTHOR]

Objective: To determine the clinical outcomes and decisions of families of fetuses with prenatally-diagnosed cardiac abnormalities. Methods: Prenatally diagnosed cases (n=155) with congenital heart disease were retrospectively categorized according to the Allan-Huggon grading system: Group A (cardiac disease associated with severe / lethal extracardiac disease); Group B1 (low risk with a postnatal prognosis); Group B2 (moderate risk, amenable to surgical repair with a low mortality); and Group B3 (high risk, associated with high mortality after surgery). Neonatal outcomes, including termination of pregnancy, were recorded for 18 months of follow-up after counseling the parents. Student's t-test, Mann-Whitney U, Pearson's Chi-square test and Fischer's exact Chi-square test were used for statistical analyses. Results: One hundred forty-five cases completed follow up. Thirty-nine cases (Group A) were associated with extracardiac lethal defects and the pregnancies were terminated; these cases were excluded from statistical evaluation. Twenty parents in Group B3 opted also for termination. The survival rates of ongoing pregnancies after 18 months of follow-up between the three cardiac abnormality Groups (Group B1, n=37; Group B2, n=12; and Group B3, n=37) were 89.2%, 66.7%, and 13.5%, respectively. Significance was present between the survival rates of the three Groups [Group B3 vs. Group B1: p=0.0001; OR: 52.8 (12.9-214.5); Group B3 vs. Group B2: p=0.0009; OR: 12.8 (2.8-58.9); Group B2 vs. Group B1: p=0.087; OR: 4.12 (0.84-20.2)]. Conclusion: Our practice and the findings reported herein support the efficacy of this staging system and counseling parents of fetuses for congenital heart diseases. [ABSTRACT FROM AUTHOR]

The article studies the association of common Mediterranean fever (MEFV) variations with ankylosing spondylitis (AS) in a group of Turkish patients. The study covered 193 patients with AS and 103 matched healthy control subjects genotyped with four MEFV variations and HLA-B27 using genomic DNA. Associations of the variations with the patients' clinical laboratory features are analyzed. The results showed familial Mediterranean fever (FMF)-related MEFV variations are associated with AS and may contribute to AS pathogenesis.

Objective: The aim of this study was to evaluate prognosis of types of ventricular septal defects and coexistence of associated cardiac and extracardiac defects. Methods: 120 prenatal diagnosed pregnancies associated with ventricular septal pathology were retrospectively evaluated and divided into four groups, as atrioventricular septal defects, perimembranous septal defects, muscular septal defects and univentricular formation. Each group was divided further into four groups, as isolated defect, co-existing extracardiac defect, septal defect with extracardiac defect and septal defect with co-existing cardiac and extracardiac defect. Postnatal follow-up was continued at least until 8 months of life. Results: Median gestational age at diagnosis was 26.3 weeks, 47 cases were diagnosed before 24 weeks. After dispersion of septal defects there was a statistical significance of p=0.0089 between groups. Of 31 cases with atrioventricular septal defects, only one case survived (3.2%) and there was a high association with extracardiac defects and abnormal karyotype (p=0.002). 69 cases with perimembranous ventricular septal defects were diagnosed, and 24 cases (34.8%) survived with significance for abnormal karyotype (p=0.039). Of 18 cases born with muscular septal defects 12 cases (66.7%) stay alive. We had two cases with univentricular structure; both cases decided for termination of pregnancy. Conclusion: The more complicated and severe the pathology, the worse the prognosis. Individualized counseling is the most important point in decision making together with families. [ABSTRACT FROM AUTHOR]

Objectives. There had been evidence that low-dose local IFN could be beneficial in the management of recurrent oral ulcers (OUs). We investigated the efficacy and collected initial data on the safety of low-dose natural human IFN-α administered by the oral mucosal route in Behçet's syndrome (BS) in a placebo controlled, double blind study. Methods. Eighty-four (59 males and 25 females) patients with BS with mainly skin mucosa disease and a history of recurrent OU for ≥ 1 year were studied. When they had at least two OUs with a total diameter of ≥ 4mm, they were randomly allocated to (i) 2000 IFN-α IU/day, (ii) 1000 IFN-α IU/day and (iii) placebo groups. Subjects were monitored weekly over an initial 4 weeks and bi-weekly for an additional 8 weeks of treatment. OU were counted and measured at each study visit. The primary efficacy end point was the difference in the total ulcer burden at Week 0 compared with that at Week 12. Results. Out of the 84 patients enrolled, 72 completed the trial. There were no statistically significant differences between the treatment arms in terms of the primary endpoint. Conclusions. Low-dose natural human IFN-α did not have beneficial effects on reducing the total ulcer burden among BS patients from Turkey. The study also showed that counting the number of ulcers rather than measuring the size would be adequate in future studies. Trial registration. ClinicalTrials.gov, NCT00483184, http://www.clinicaltrials.gov/ct2/results?term=NCT00483184 [ABSTRACT FROM AUTHOR]