Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy.

OBJECTIVES: The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS). METHODS: Demographic, clinical, and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen pediatric rheumatology centers across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment. RESULTS: One hundred and sixty-two patients were included in the study. 45 of the MAS events were detected under the effect of anti-IL-1/-6 biologics, while the patients experiencing the remaining 155 events have not received biological treatment in the last three months. Platelet count [128 (72-232) vs 199 (130-371) 109/l], ferritin level on admission [1107 (676-2050) vs 2863 (1193-9562) ng/ml], C-reactive protein level [15.4 (2.9-56) vs 90 (32-160) mg/l], erythrocyte sedimentation rate [13 (3-36) vs 43.5 (13-77) mm/h] and fever duration [5 (4-7.5) vs 10 (7-14.3) days] were found lower in the group under the impact of anti-IL-1/-6 biologics. Among patients treated with biologics, 26.6% did not meet the published 2016 MAS classification criteria at presentation. The rates of hepatomegaly and splenomegaly were relatively lower in the canakinumab-treated group when compared with those receiving other biologicals or to patients, not on biologicals. CONCLUSION: Anti-IL-1/-6 therapies can mask the clinical and laboratory features of MAS, and proposed guidelines for MAS classification criteria may not be met.

Rhupus syndrome in children: A multi-center retrospective cohort study and literature review.

OBJECTIVE: In this study, we aimed to evaluate the characteristics of pediatric rhupus patients including all the related series in the literature. METHODS: Thirty pediatric patients with rhupus syndrome from 12 different centers in Turkey were included in this study. The literature was also reviewed for pediatric patients with rhupus syndrome. RESULTS: The most prominent phenotype of these 30 patients was juvenile idiopathic arthritis (JIA) (60%) at the disease onset and SLE (73.3%) at the last visit. Major SLE-related organ involvements were skin (80%), hematological system (53.3%), and kidney (23.3%). Arthritis was polyarticular (73.3%), asymmetric (66.7%), and erosive (53.3%) in most patients. Hydroxychloroquine (100%), glucocorticoids (86.7%), and mycophenolate mofetil (46.7%) were mostly used for SLE, while glucocorticoids (76.6%), methotrexate (73.3%), and nonsteroidal anti-inflammatory drugs (NSAIDs) (57.6%) were mainly preferred for JIA. Our literature search revealed 20 pediatric patients with rhupus syndrome (75% were RF positive). The most prominent phenotype was JIA (91.7%) at the disease onset and SLE (63.6%) at the last visit. Major SLE-related organ involvements were skin (66.7%), hematological system (58.3%), and kidney (58.3%). Arthritis was polyarticular (77.8%), asymmetric (63.6%), and erosive (83.3%) in most patients. Glucocorticoid (100%), hydroxychloroquine (76.9%), and azathioprine (46.2%) were mostly used for SLE, while methotrexate (76.9%) and NSAIDs (46.2%) were mainly preferred for the JIA phenotype. CONCLUSION: Our study is the largest cohort in the literature evaluating pediatric rhupus cases. Most of the pediatric patients had polyarticular, asymmetric, and erosive arthritis, as well as organ involvements associated with SLE, including the skin, hematological system, and kidney.

Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study.

Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration. CONCLUSIONS: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests. WHAT IS KNOWN: • During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring. WHAT IS NEW: • There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment. • This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests.

Turkish nurses' psychological resilience and burnout levels during the COVID-19 pandemic: A correlational study.

AIMS: To investigate the relationship between the psychological resilience and burnout of nurses caring for COVID-19 patients and to determine the factors that affect their psychological resilience and burnout. BACKGROUND: In pandemic diseases such as COVID-19, nurses experience burnout due to long working hours, decreased quality of life and anxiety/fear about their own/families' health. Psychological resilience helps to control burnout in nurses and prevent the development of a global nurse shortage. DESIGN: This was a descriptive, correlational study. METHODS: The sample of this study included 201 nurses in a Training and Research Hospital. The study used the Brief Resilience Scale and the Burnout Measure Short Version. Data were collected between 4 May and 1 June 2020. Statistical analysis was made with Pearson/Spearman, independent sample t test, one-way analysis of variance (ANOVA) test. RESULTS: Nurses reported moderate burnout and psychological resilience, with a negative and highly significant correlation between psychological resilience and burnout levels. CONCLUSIONS: In order to increase the quality of patient care/treatment, nurse managers need to reduce nurses' burnout and increase their psychological resilience. Nurses are recommended to adopt a healthy lifestyle, organize training programmes and implement psychological resilience interventions to prevent sleep disorders. Giving nurses the tools to understand what they need to manage within their locus of control will allow them to find a new sense of resilience, preventing potential burnout.

Chronic Nonbacterial Osteomyelitis Associated With Familial Mediterranean Fever in Children.

OBJECTIVE: Chronic nonbacterial osteomyelitis (CNO) is a noninfectious autoinflammatory bone condition that frequently occurs alongside other inflammatory diseases, such as familial Mediterranean fever (FMF). We aimed to determine the demographic, clinical, laboratory, and radiological characteristics of patients diagnosed with both FMF and CNO. METHODS: We reviewed the medical records of pediatric patients with both CNO and FMF at 3 pediatric rheumatology centers in Turkey from December 2008 to 2022. Patients' demographics, laboratory features, imaging findings, and treatment were recorded. RESULTS: Twelve patients with FMF and CNO were included in the study. Half of them were female. The mean ages at onset for FMF and CNO symptoms were 80 and 116 months, whereas the ages at diagnosis for FMF and CNO were 100 and 125 months, respectively. Ten patients (83.3%) had M694V mutation on at least 1 allele of the Mediterranean fever (MEFV) gene. The most common sites of osteitis were the long bones (58.3%), pelvis (50%), and clavicles (25%). Ten patients (83%) received nonsteroidal anti-inflammatory drugs; 8 (66%) received disease-modifying antirheumatic drugs; biological therapy was administered to 5 patients (41%), who did not respond to these treatments; and all patients received colchicine. CONCLUSION: The increased frequency of FMF in patients with CNO is of interest. Because most patients with CNO and FMF carried a homozygous or combined heterozygous M694V mutation, we speculated that the M694V mutation may play a role in the development of osteitis. Further studies are needed to elucidate the link between FMF and CNO.

A clinical overview of paediatric sarcoidosis: Multicentre experience from Turkey.

OBJECTIVES: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS). METHODS: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis. RESULTS: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7). CONCLUSIONS: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications.

Efficacy and safety of anti-interleukin-1 in children with colchicine-resistant familial Mediterranean fever.

BACKGROUND: The aim of this study was to evaluate the efficacy and safety of anti-interleukin-1 (IL-1) therapies in colchicine-resistant pediatric patients with familial Mediterranean fever (FMF). METHODS: In this study, we retrospectively evaluated 656 children with FMF and 27 patients who had been treated with anti-IL-1 therapies (anakinra/canakinumab) . Clinical and laboratory features, MEFV gene mutations, treatment responses were investigated. RESULTS: Twenty of the patients were treated with anakinra (the treatment of 6 patients who initially used anakinra was switched to canakinumab in the follow-up period), and 13 patients were treated with canakinumab. Clinical symptom and severity scores decreased in all patients A decrease in acute phase reactants was also observed in patients. A total of 18 (66%) patients had a M694V homozygous mutation, while 24 (89%) patients had a M694V mutation, at least in one allele. CONCLUSIONS: FMF patients with colchicine resistance may progress to amyloidosis. IL-1 antagonist treatment could be used safely with a favorable outcome in pediatric patients with FMF resistance to colchicine therapy and/or who have renal amyloidosis.

Effective immobilization of lactate dehydrogenase onto mesoporous silica.

This study presents that covalent immobilization technique has been utilized for the immobilization of l-lactate dehydrogenase (l-LDH) from porcine on mesoporous silica. To develop mesoporous silica as support material for use in l-LDH immobilization, the particle surfaces were functionalized with 3-aminopropyltrimethoxysilane and further conjugated with glutaraldehyde. The effect of some parameters such as glutaraldehyde concentration, immobilization pH, initial enzyme concentration, and immobilization time was investigated and the optimum conditions for these parameters were determined as 1% (w/v), pH 8.0, 1 mg/ml, and 120 min, respectively. The maximum working pH and temperature for the oxidation of lactate to pyruvate reaction were determined as 10.0 and 35°C for free and 9.0 and 40°C for immobilized l-LDH, respectively. The kinetic parameters (Km and Vmax ) of l-LDH for the oxidation of lactate to pyruvate reaction were examined as 1.02 mM and 7.58 U/mg protein for free and 0.635 mM and 1.7 U/mg protein for immobilized l-LDH, respectively. Moreover, the immobilized l-LDH was 1.3-fold more stable than free l-LDH at 25°C according to calculated t1/2 values. The immobilized l-LDH retained 80% of its initial activity in a batch reactor after 14 reuses.

Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey.

BACKGROUND: Although not validated fully, recommendations are present for diagnosis, screening and treatment modalities of patients with familial Mediterranean fever (FMF). OBJECTIVE: To review the current practices of clinicians regarding FMF and reveal their adherence to consensus guidelines. METHODS: Fifteen key points selected regarding the diagnosis and management of FMF were assessed by 14 paediatric rheumatologists with a three-round modified Delphi panel. RESULTS: Consensus was reached on the following aspects: genetic analysis should be ordered to all patients when clinical findings support FMF, but its result is not decisive alone. In the absence of clinical features, colchicine should be commenced when two pathogenic alleles and family history of amyloidosis are present. Serum amyloid A testing at each visit is recommended in patients resistant to colchicine, with subclinical inflammation and family history of amyloidosis. Consensus was reached on both the definition of colchicine resistance and starting biologic in resistant cases. Cost, efficiency, ease of use, treatment adherence, accessibility and emergence of adverse events are the factors affecting the choice of biologic agents. In patients without any attack and evidence of subclinical inflammation within the last 6 months following initiation of biologics, treatment dose intervals can be prolonged. CONCLUSION: A consensus was achieved regarding the routine diagnosis and screening and treatment of FMF patients. The definition of colchicine resistance was made and a protocol was created for prolongation of treatment intervals of biologic agents. We anticipate that the results of the study reveal real-life data on the approach to patients in clinical practice.

Differences and similarities of multisystem inflammatory syndrome in children, Kawasaki disease and macrophage activating syndrome due to systemic juvenile idiopathic arthritis: a comparative study.

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.

The clinical course of SARS-CoV-2 infection among children with rheumatic disease under biologic therapy: a retrospective and multicenter study.

The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course of COVID-19 on children with underlying rheumatologic diseases have not been fully demonstrated. To evaluate the course of COVID-19 infection in patients with rheumatic disease receiving bDMARD treatment. This was a retrospective, multicenter study conducted in pediatric patients infected by SARS-CoV-2 and under bDMARDs therapy. The study population consisted of 113 patients (72 female/41 male). The mean age of the patients was 12.87 ± 4.69 years. The primary diagnosis of the cohort was as follows: 63 juvenile idiopathic arthritis, 35 systemic autoinflammatory diseases, 10 vasculitides, and five cases of connective tissue diseases. The mean duration of the primary disease was 4.62 ± 3.65 years. A total of 19 patients had additional comorbid diseases. Thirty-five patients were treated with canakinumab, 25 with adalimumab, 18 with etanercept, 10 with infliximab, nine with tocilizumab, six with rituximab, four with anakinra, three with tofacitinib, and one with abatacept. The median exposure time of the biological drug was 13.5 months. Seventy-one patients had symptomatic COVID-19, while 42 were asymptomatic. Twenty-four patients required hospitalization. Five patients presented with MIS-C. The hospitalized patients were younger and had a shorter duration of rheumatic disease compared to ambulatory patients, although the difference was not statistically significant. Steroid usage, presence of fever, and dyspnea were more common among the hospitalized patients. A worsening in the course of both COVID-19 and current disease was not noticed under bDMARDs, however, to end with a strong conclusion multicentric international studies are required.

Does Age at Onset Affect the Clinical Presentation of Familial Mediterranean Fever in Children?

BACKGROUND/OBJECTIVE: The aim of this study was to compare the clinical and demographic features and evaluate the phenotypic and genotypic differences of pediatric familial Mediterranean fever (FMF) patients according to their age at disease onset. METHODS: Records of 854 patients who were diagnosed with FMF between 2006 and 2017 were evaluated. Patients were divided into 2 subgroups according to their age at disease onset. Group 1 comprised FMF patients who had experienced their first attack at 2 years or younger (younger onset), and group 2 comprised FMF patients who had experienced their first attack at older than 2 years. RESULTS: There were 155 patients in group 1 and 699 patients in group 2. Delay in diagnosis, attack frequency, duration of attacks, fever, chest pain, erysipelas-like erythema, incidence of family history, anti-interleukin 1 therapy use, and M694V homozygous and M680I homozygous mutations were significantly higher in group 1, whereas arthralgia and abdominal pain were significantly higher in group 2. There were no significant differences in arthritis, amyloidosis, and protracted febrile myalgia between the groups. The colchicine dose at last visit and Pras activity score were higher in group 1. CONCLUSIONS: It seems that FMF patients with a younger onset has a more severe disease course. They needed higher doses of colchicine to control the attacks. M694V and M680I homozygous mutations presented more frequently in younger-onset FMF patients. Increased awareness of physicians of the early presentation of FMF may prevent delays in FMF diagnosis.

Predictors of persistent inflammation in children with familial Mediterranean fever.

OBJECTIVES: Persistent inflammation is an insidious feature of familial Mediterranean fever (FMF) that may cause chronic complications. This study aimed to investigate the predictors of persistent inflammation in children with FMF. METHODS: The medical charts of 1077 paediatric FMF patients were retrospectively collected. The patients were divided into two groups: with and without subclinical inflammation. RESULTS: A total of 133 (12%) patients had persistent inflammation. M694V homozygosity, colchicine resistance, positive family history for FMF, erysipelas-like erythema, leg pain, arthritis, chest pain, inflammatory comorbidities, early disease onset, high PRAS score, and long attack duration were established as independent predictors of persistent inflammation (P < .001, P < .001, P < .001, P < .001, P = 0.006, P < .001, P < .001, P = .014, P < .001, P < .001, and P < .001, respectively). However, gender, abdominal pain, fever, and attack frequency were not found to be independent risk factors for predicting persistent inflammation (P = .412, P = .531, P = .451, and P = .693, respectively). CONCLUSIONS: M694V homozygosity, colchicine resistance, positive family history, erysipelas-like erythema, leg pain, arthritis, chest pain, inflammatory comorbidities, early disease onset, high activity score, and long attack duration may be predictors of persistent inflammation in FMF. These predictors may help clinicians suspect the occurrence of subclinical inflammation and should aid in better disease management in FMF.

COVID-19 in pediatric nephrology centers in Turkey.

BACKGROUND: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. METHODS: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. RESULTS: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. DISCUSSION: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage.

Strong light emission from a defective hexagonal boron nitride monolayer coupled to near-touching random plasmonic nanounits.

In this Letter, we demonstrate strong light emission from defective hexagonal boron nitride (hBN) defect centers upon their coupling with disorder near-touching plasmonic units. Based on numerical simulations and characterization results, the plasmonic design at thin layer thicknesses of 20 nm can provide above 2 orders of magnitude enhancement in photoluminescence (PL) spectra. Moreover, this plasmonic platform shortens the luminescence lifetime of the emitters. The proposed design can be easily extended to other plasmonic-emitter combinations where strong light-matter interaction can be achieved using large-scale compatible routes.

Evaluation of caregiver burden and coping strategies in parents of paediatric familial Mediterranean fever patients in relation to illness severity, therapy and health-related quality of life.

PURPOSE: The study aimed to determine the caregiver burden and coping strategies in caregivers of familial Mediterranean fever (FMF) patients in relation to illness severity, therapy and health-related quality of life (HRQoL). METHODS: The study included 171 paediatric FMF patients and their caregivers (parents). The caregivers were asked to complete a socio-demographic form, the Zarit caregiver burden interview (ZCBI) and the Brief COPE. The patients and their caregivers were asked to complete the KINDer Lebensqualitätsfragebogen questionnaire (self-report and proxy report, respectively) for assessing HRQoL. The patients were categorised according to their disease activity (mild, moderate or severe) and the presence or absence of anti-IL-1 therapy. RESULTS: The mean ZCBI score of the caregivers was 44.7 ± 13.5. ZCBI and COPE scores did not differ significantly between the caregivers of FMF patients receiving and not receiving anti-IL-1 therapy. However, dysfunctional COPE (p = 0.039) and ZCBI (p = 0.021) scores showed a significant difference between the caregivers in relation to patient's disease severity. ZCBI scores were positively correlated with dysfunctional coping (p = 0.01). Self-reported HRQoL disease module scores were lower for the patients who received anti-IL-1 therapy than for those did not (p = 0.009). Proxy-reported (p < 0.001) and self-reported (p = 0.043) HRQoL disease module scores were lower for the patients with severe disease activity. CONCLUSIONS: As the caregiver burden increases, parents tend to use a dysfunctional coping strategy. Good control of disease activity with administration of medical therapy can reduce the disease severity, thereby decrease the caregiver burden, and secondly help to reduce the usage of dysfunctional coping in caregivers.

Numerical analysis of a thermally tunable spectrally selective absorber enabled by an all-dielectric metamirror.

In this Letter, we numerically propose a temperature-tunable, ultra-narrowband one-way perfect near-infrared radiation absorber with high transmission in the longer wavelength neighboring spectral range. We obtained this functionality by using a guided-mode resonance-based grating-waveguide metamirror that is comprised of silicon, a spacer dielectric, an absorbing semiconductor, and germanium. Within the ultra-narrow bandwidth of the guided-mode resonance excited at 1.16 µm with a full width at half-maximum of 3.3 nm, we confirmed perfect absorption when light is incident from one of the two opposite directions. Excitation from the opposite direction resulted in perfect reflection. The thickness of the entire structure is limited to about one third the operating wavelength. Furthermore, due to the temperature tunability of silicon and germanium the thermo-optical sensitivity was found to be approximately 0.068 nm/K. In addition to this spectral tunability, our proposed device supports transparency windows with 80% transmission in the higher wavelength ranges. Our device is highly promising in the applications of thermo-tunable modulators and obtaining single frequency near-infrared signals from broadband sources.

One-way and near-absolute polarization insensitive near-perfect absorption by using an all-dielectric metasurface.

In this Letter, we numerically propose the one-way perfect absorption of near-infrared radiation in a tunable spectral range with high transmission in the neighboring spectral ranges. This functionality is obtained by using a two-dimensional, guided-mode resonance-based grating-waveguide metasurface that acts as a frequency-selective reflector, a spacer dielectric, and an absorbing oxide layer. Within the bandwidth of the excited guided-mode resonance excited at 1.82 µm (with a full-width at half-maximum of 19 nm), we confirmed perfect absorption when light was incident from one of the two opposite directions, whereas in the other direction, perfect reflection was observed. The forward-to-backward absorption ratio reached as high as 60, while the thickness of the entire structure was on the order of the operating wavelength. In addition to the spectral tunability of the excited resonances and their bandwidths, our proposed device supports transparency windows with 65% transmission in the adjacent frequency bands. Our 2D grating is also verified to enable near-absolute insensitivity to the polarization state of incident light. Geometrical parameter modification also gives our design great tunability, as we also designed a device with a 300 nm absorption/reflection linewidth.

Disease activity and outcomes in juvenile Behçet's disease: 10 years' experience of a single centre.

OBJECTIVES: The aims of this study were to investigate the development of new events (new clinical signs related to Behçet's disease) and to evaluate outcomes in juvenile Behçet's disease (jBD) patients over a 10-year follow-up. METHODS: We included 57 patients diagnosed with jBD according to International Behçet's Study Group (ISG) criteria and/or the International Criteria for BD (ICBD) and/or Paediatric BD (PEDBD) group criteria, followed-up between 2008 and 2018. Any new organ system involvement during follow-up was defined as an event in event-free survival analysis. RESULTS: The patients' female/male ratio was 33/24. The most prevalent clinical feature was recurrent oral aphthosis (100%), followed by musculoskeletal symptoms (63%), genital ulcers (56%), ocular manifestations (47%) and cutaneous manifestations (46%). Vascular, neurological, gastrointestinal and genitourinary manifestations were observed in 4-17% of the patients. Fifty-four (95%) cases fulfilled the ICBD, while 31 (54%) and 34 (60%) fulfilled ISG and PEDBD criteria, respectively. The median Iranian Behçet's disease dynamic activity measure (IBDDAM) score at diagnosis was 5 (range: 3-14) and decreased to 1 (range: 0-6) at the last visit. One to three events occurred in 21 (37%) cases. One fifth (19%) of these events were severe. The event-free survival rate was 95% at one year, 70% at three years and 50% at eight years. CONCLUSIONS: This study shows that with effective treatment, jBD has favourable outcome and a remarkable event-free survival. Underdiagnosed cases according to ISG and PEDBD criteria could be diagnosed using the ICBD.

Erysipelas-like erythema in children with familial Mediterranean fever.

OBJECTIVES: Erysipelas-like erythema (ELE) is a well-known pathognomonic skin lesion associated with familial Mediterranean fever (FMF). The aim of this study was to describe the clinical and demographic features and phenotypic differences between paediatric FMF patients with and without ELE. METHODS: We retrospectively collected the medical charts of paediatric patients who had been diagnosed with FMF and followed by the Paediatric Rheumatology Department of Gazi University, Turkey, from 2006 to 2016. RESULTS: Among 782 FMF patients, 59 (33 males and 26 females; median age, 11.1±5.1) were found to have ELE. More patients had arthritis in the ELE group than in the other group (p=0.011). Arthritis occurred in the ankle (77.4%), knee (19.3%) and hip (3.2%) joints. The coexistence of arthritis and ELE was seen in 12 (20.3%) patients. All ELE plaques were located on the lower legs and dorsum of the feet. Eleven patients (18.6%) presented with ELE as the initial symptom and were diagnosed with FMF, and 48 (81.4%) patients experienced ELE attacks while receiving colchicine therapy. The median dose of colchicine at last visit, PRAS activity score and M694V homozygous mutation status were significantly higher in the ELE group than in the other group (p=0.041, p=0.001 and p=0.023, respectively). CONCLUSIONS: ELE is an uncommon but important feature of FMF. In patients with ELE, arthritis is more frequently encountered, and M694V homozygous mutation is more frequently found. FMF patients with ELE have more severe disease activity, and they use higher doses of colchicine in relation to this severe disease course.