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The article describes the case of a female American Cocker dog trained as narcotic detector brought to the Animal Hospital of the Faculty of the Veterinary Medicine of Afyon Kocatepe University, Turkey for routine medical check-up. At cardiologic inspection, the heart was found to be hypertrophic and an anamnesis revealed that the dog had no complaints that reflect a cardiac disease.

Objective: In this study, we aimed to evaluate the relationship between the level of improvement in blood gas parameters in the first hours of age and normal and diffusion-restriction brain magnetic resonance imaging (MRI)., Materials and Methods: The study is a retrospective cohort study. Cases of the diagnosis of hypoxic-ischemic encephalopathy (HIE) who received therapeutic hypothermia in our unit between January 2022 and January 2024 were included in the study. Clinical findings, blood gas values (first, cord; second, first hours of age; third, 24th hour of age), and MRI results were recorded from the case files and compared between normal and diffusion-restricted brain MRI groups., Results: Diffusion-restricted brain MRI was detected in 10 out of a total of 19 cases. The 5-minute Apgar score was lower (p=0.038) and mechanical ventilator support was higher (P=.003) in the diffusion-restricted MRI group than in the normal MRI group. The relationship was shown between high base excess (P=.022) in cord blood gas, low HCO₃ (p=0.025) in the 24th hour blood gas, and convulsion (P=.033) in the diffusion-restricted MRI group. Additionally, it was found that only the improvement level of the pH value in the first hour of age was significant (P=.025) in the diffusion-restricted brain MRI group than in the normal MRI group., Conclusion: We showed that there was a relationship between diffusion-restricted brain MRI and the improvement level in the pH value in the first hours of age of patients diagnosed with HIE who received treatment for therapeutic hypothermia.

Purpose: Immunodeficiency with centromeric instability and facial anomalies (ICF) syndrome is a rare autosomal recessive combined immunodeficiency. The detailed immune responses are not explored widely. We investigated known and novel immune alterations in lymphocyte subpopulations and their association with clinical symptoms in a well-defined ICF cohort., Methods: We recruited the clinical findings from twelve ICF1 and ICF2 patients. We performed detailed immunological evaluation, including lymphocyte subset analyses, upregulation, and proliferation of T cells. We also determined the frequency of circulating T follicular helper (cT FH ) and regulatory T (Treg) cells and their subtypes by flow cytometry., Results: There were ten ICF1 and two ICF2 patients. We identified two novel homozygous missense mutations in the ZBTB24 gene. Respiratory tract infections were the most common recurrent infections among the patients. Gastrointestinal system (GIS) involvements were observed in seven patients. All patients received intravenous immunoglobulin replacement therapy and antibacterial prophylaxis; two died during the follow-up period. Immunologically, CD4 + T-cell counts, percentages of recent thymic emigrant T cells, and naive CD4 + T decreased in two, five, and four patients, respectively. Impaired T-cell proliferation and reduced CD25 upregulation were detected in all patients. These changes were more prominent in CD8 + T cells. GIS involvements negatively correlated with CD3 + T-, CD3 + CD4 + T-, CD16 + CD56 + NK-cell counts, and CD4 + /CD8 + T-cell ratios. Further, we observed expanded cT FH cells and reduced Treg and follicular regulatory T cells with a skewing to a T H 2-like phenotype in all tested subpopulations., Conclusion: The ICF syndrome encompasses various manifestations affecting multiple end organs. Perturbed T-cell responses with increased cT FH and decreased Treg cells may provide further insight into the immune aberrations observed in ICF syndrome., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Lipopolysaccharide-responsive beige-like anchor protein (LRBA) deficiency and cytotoxic T-lymphocyte protein-4 (CTLA-4) insufficiency are recently described disorders that present with susceptibility to infections, autoimmunity, and lymphoproliferation. Clinical and immunological comparisons of the diseases with long-term follow-up have not been previously reported. We sought to compare the clinical and laboratory manifestations of both diseases and investigate the role of flow cytometry in predicting the genetic defect in patients with LRBA deficiency and CTLA-4 insufficiency., Methods: Patients were evaluated clinically with laboratory assessments for lymphocyte subsets, T follicular helper cells (T FH ), LRBA expression, and expression of CD25, FOXP3, and CTLA4 in regulatory T cells (Tregs) at baseline and 16 h post-stimulation., Results: LRBA-deficient patients (n = 29) showed significantly early age of symptom onset, higher rates of pneumonia, autoimmunity, chronic diarrhea, and failure to thrive compared to CTLA-4 insufficiency (n = 12). In total, 29 patients received abatacept with favorable responses and the overall survival probability was not different between transplanted versus non-transplanted patients in LRBA deficiency. Meanwhile, higher probability of survival was observed in CTLA-4-insufficient patients (p = 0.04). The T-cell subsets showed more deviation to memory cells in CTLA-4-insufficiency, accompanied by low percentages of Treg and dysregulated cT FH cells response in both diseases. Cumulative numbers of autoimmunities positively correlated with cT FH frequencies. Baseline CTLA-4 expression was significantly diminished in LRBA deficiency and CTLA-4 insufficiency, but significant induction in CTLA-4 was observed after short-term T-cell stimulation in LRBA deficiency and controls, while this elevation was less in CTLA-4 insufficiency, allowing to differentiate this disease from LRBA deficiency with high sensitivity (87.5%) and specificity (90%)., Conclusion: This cohort provided detailed clinical and laboratory comparisons for LRBA deficiency and CTLA-4 insufficiency. The flow cytometric approach is useful in predicting the defective gene; thus, targeted sequencing can be conducted to provide rapid diagnosis and treatment for these diseases impacting the CTLA-4 pathway., (© 2022 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)

Purpose: Severe combined immunodeficiency (SCID) is one of the most severe forms of inborn errors of immunity characterized by absence or loss of function in T cells. The long-term outcomes of all forms of SCID have been evaluated in a limited number of studies. We aimed to evaluate the pre- and post-transplant manifestations of SCID patients and determine the factors affecting the survival of patients., Methods: We included 54 SCID patients (classical SCID, Omenn syndrome, atypical SCID (AS)) in this study. We evaluated the clinical presentation, infections, and outcome of hematopoietic stem cell transplantation (HSCT). Lymphocyte subsets and T-cell receptor (TCR) repertoire were analyzed by flow cytometry., Results: The median age at diagnosis was 5 (range: 3-24) months and follow-up time was 25 (range: 5-61) months. Symptom onset and diagnostic ages were significantly higher in AS compared to others (p = 0.001; p < 0.001). The most common SCID phenotype was T-B-NK + , and mutations in recombination-activating genes (RAG1/2) were the prominent genetic defect among patients. The overall survival (OS) rate was 83.3% after HSCT, higher than in non-transplanted patients (p = 0.001). Peripheral blood stem cell sources and genotypes other than RAG had a significant favorable impact on CD4 + T cells immune reconstitution after transplantation (p = 0.044, p = 0.035; respectively). Gender matching transplantations from human leukocyte antigen (HLA)-identical and non-identical donors and using peripheral blood stem cell source yielded higher B-cell reconstitution (p = 0.002, p = 0.028; respectively). Furthermore, receiving a conditioning regimen provided better B-cell reconstitution and chimerism (p = 0.003, p = 0.001). Post-transplant TCR diversity was sufficient in the patients and showed an equal distribution pattern as healthy controls. The OS rate was lower in patients who underwent transplant with active infection or received stem cells from mismatched donors (p = 0.030, p = 0.015; respectively)., Conclusion: This study identifies diagnostic and therapeutic approaches predictive of favorable outcomes for patients with SCID., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Biallelic loss-of-function mutations in CARMIL2 cause combined immunodeficiency associated with dermatitis, inflammatory bowel disease (IBD), and EBV-related smooth muscle tumors. Clinical and immunological characterizations of the disease with long-term follow-up and treatment options have not been previously reported in large cohorts. We sought to determine the clinical and immunological features of CARMIL2 deficiency and long-term efficacy of treatment in controlling different disease manifestations., Methods: The presenting phenotypes, long-term outcomes, and treatment responses were evaluated prospectively in 15 CARMIL2-deficient patients, including 13 novel cases. Lymphocyte subpopulations, protein expression, regulatory T (Treg), and circulating T follicular helper (cT FH ) cells were analyzed. Three-dimensional (3D) migration assay was performed to determine T-cell shape., Results: Mean age at disease onset was 38 ± 23 months. Main clinical features were skin manifestations (n = 14, 93%), failure to thrive (n = 10, 67%), recurrent infections (n = 10, 67%), allergic symptoms (n = 8, 53%), chronic diarrhea (n = 4, 27%), and EBV-related leiomyoma (n = 2, 13%). Skin manifestations ranged from atopic and seborrheic dermatitis to psoriasiform rash. Patients had reduced proportions of memory CD4 + T cells, Treg, and cT FH cells. Memory B and NK cells were also decreased. CARMIL2-deficient T cells exhibited reduced T-cell proliferation and cytokine production following CD28 co-stimulation and normal morphology when migrating in a high-density 3D collagen gel matrix. IBD was the most severe clinical manifestation, leading to growth retardation, requiring multiple interventional treatments. All patients were alive with a median follow-up of 10.8 years (range: 3-17 years)., Conclusion: This cohort provides clinical and immunological features and long-term follow-up of different manifestations of CARMIL2 deficiency., (© 2021 European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd. This article has been contributed to by US Government employees and their work is in the public domain in the USA.)

Background: To determine the frequency of breastfeeding of mothers working in primary care, the differences between different employment groups, and the effective factors., Methods: This descriptive research study was conducted with a self-report online survey design. The snowball sampling method was used for the sample selection, and 151 family physicians and 126 family health professionals were included in the study during the research period (June 2019-December 2019). A 35-item survey was used to collect data. The response rate was 44.9% (49.5% family physicians/40.3% family health professionals)., Results: The mean duration of exclusive breastfeeding was 3.9 ± 2.0 months, and the mean duration of total breastfeeding was 16.7 ± 8.5 months. There was no significant difference between the family physicians and family health professionals in terms of exclusive breastfeeding (P = .580) and total breastfeeding (P = .325) durations. The most common reasons for weaning were reduced milk supply (25.6%) and not being able to use breastfeeding leave (23.1%) due to problems at work. Of the sample, 41.3% had problems with their coworkers and 41.9% had problems related to patient care when taking breastfeeding leave. Working in a baby-friendly centre (P = .01), prolonged exclusive breastfeeding (P < .001), and increased hours of breastfeeding leave taken (P = .001) had a positive effect on breastfeeding for ≥24 months while experiencing problems with coworkers in taking breastfeeding leave (P = .023) had a negative effect on this variable., Conclusions: All of the factors that were determined to affect the continuation of breastfeeding for ≥24 months are modifiable. It is very important for relevant authorities to undertake necessary action to improve the conditions of working mothers based on these results. Health professional that can maintain the balance between family and work will work more efficiently., (© 2021 John Wiley & Sons Ltd.)

Objectives: The profession of medicine is very depressing both mentally and physically. There were studies conducted to investigate the reasons of depression and burnout. Hopelessness leads to depression and may be it is the first sign. The aim of our study is to determine the despair levels of primary care physicians., Methods: This research is a descriptive study. The study conducted with family physicians and Beck Hopelessness Scale was applied. Statistical analysis of the data was performed using IBM SPSS 23., Results: 40.9% of those without children, 48.9% of those with chronic diseases, 47% of those who had been subjected to violence and 53.5% of those who are not satisfied with working in the primary care received severe hopelessness scores, and these score were significant (P < .05)., Conclusion: The dissatisfied aspects of Family Medicine System should be examined in detail, and necessary measures should be taken against violence, which is a common problem in the whole healthcare sector. In this way, employee satisfaction and therefore patient satisfaction can be achieved., (© 2021 John Wiley & Sons Ltd.)

Aim: The aim of this study was to describe the attitudes, views and solution proposals of family physicians (FPs) about primary healthcare problems of Syrian refugee patients. This study would be the very first study for Turkey that evaluates the attitudes, views and solution proposals of FPs about primary healthcare problems of Syrian refugee patients., Background: Following the anti-regime demonstrations that started in March 2011, the developments in Syria created one of the biggest humanitarian crises in the world and the largest number of asylum seekers continue to be hosted in Turkey. There are some studies evaluating asylum seekers' access to healthcare services in Europe, and the common result is that refugees have free access to primary healthcare services in most countries; however, they face many obstacles when accessing primary healthcare services. While there are studies in the literature evaluating the situation of access to primary healthcare services from the perspective of asylum seekers; there are few studies evaluating the opinions/views of FPs., Methods: A qualitative methodology informed by the grounded theory was used to guide the research. A total of 20 FPs were interviewed face to face through semi-structured interviews, using 12 questions about their lived experience and views caring of refugee population. Interviews were analysed thematically., Finding: The following themes were revealed: Benefiting from Primary Health Care Services, Benefiting from Rights, Differences Between the Approach/Attitudes of Turkish Citizens and Refugees, Barriers to Healthcare Delivery, Training Needs of Physicians, Solution proposals. FPs reported that there is a need for support in primary care and a need for training them and refugees in this regard and they specified refugee healthcare centres are the best healthcare centres for refugees; however, the number of these and provided services should be increased.

Objective: In this study, we aim to investigate the thoughts and attitudes of individuals towards the future COVID-19 vaccine., Methods: This descriptive study was carried out on the web between 10/06/2020 and 10/07/2020. The sample constitutes all individuals above 18 years of age using social media and smartphone. The e-survey form was shared by the researchers via the web for a month, and those who completed the survey were included in the study and formed the sample of the research., Results: Seven-hundred and fifty-nine people participated. 49.7% of the participants stated to be vaccinated; 38.4% of them stated to be vaccinated their children against COVID-19; if the vaccine for COVID-19 is developed. The request for the COVID-19 vaccine had relationship with gender, occupation, health insurance, anxiety level, having children and willing to get vaccinated for their children. "Afraid of the side effects of vaccine", "don't think it can be reliable as it will be a new vaccine" and "COVID-19 infection is a biological weapon and the vaccine will serve those who produce this virus" were the most common reasons for rejection of vaccine., Conclusion: In our study, afraid of the side effects of vaccine and not thinking it can be reliable as it will be a new vaccine are the most reasons of indecision and rejection about COVID 19 vaccine. In order for the future COVID 19 vaccination campaign to not fail, media, politicians and healthcare professionals should closely follow the vaccination development processes, inform the public transparently and consider public's concerns., (© 2020 John Wiley & Sons Ltd.)

Background: Heterozygous relatives of ataxia-telangiectasia (AT) patients are at an increased risk for certain AT-related manifestations. We also show that there is an increase of infection frequency in parents of AT patients. Thus, we hypothesized that the parents might exhibit immune alterations similar to their affected children., Methods: Lymphocyte phenotyping to enumerate T- and B-cell subsets was performed. Functional analyses included in vitro quantified γ-H2AX, poly (ADP-ribose) polymerase (PARP) and caspase-9 proteins. Chromosomal instability was determined by comet assay., Results: We analyzed 20 AT patients (14F/6M), 31 parents (16F/15M), and 35 age-matched healthy controls. The AT patients' parents exhibited low frequency of naive CD4 + T- (n = 14, 45%) and recent thymic emigrants (n = 11, 35%) in comparison with the age-matched healthy donors. Interestingly, parents with low naive T cells also demonstrated high rate of recurrent infections (9/14, 64%). In comparison with age-matched controls, parents who had recurrent infections and low naive T cells showed significantly higher baseline γ-H2AX levels and H 2 O 2 -induced DNA damage as well as increased cleaved caspase-9 and PARP proteins., Conclusion: Parents of AT patients could present with recurrent infections and display cellular defects that mimic AT patients. The observed immunological changes could be associated with increased DNA double-strand breaks., (© 2020 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)

Hyper-IgE syndrome (HIES) patients may share many features observed in severe atopic dermatitis (SAD), making a diagnostic dilemma for physicians. Determining clinical and laboratory markers that distinguish both disorders could provide early diagnosis and treatment. We analyzed patients (DOCK8 deficiency:14, STAT3-HIES:10, SAD:10) with early-onset SAD. Recurrent upper respiratory tract infection and pneumonia were significantly frequent in HIES than SAD patients. Characteristic facial appearance, retained primary teeth, skin abscess, newborn rash, and pneumatocele were more predictable for STAT3-HIES, while mucocutaneous candidiasis and Herpes infection were common in DOCK8 deficiency, which were unusual in SAD group. DOCK8-deficient patients had lower CD3 + and CD4 + T cells with a senescent phenotype that unique for this form of HIES. Both DOCK8 deficiency and STAT3-HIES patients exhibited reduced switched memory B cells compared to the SAD patients. These clinical and laboratory markers are helpful to differentiate HIES from SAD patients., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: In our study, we aimed to evaluate the sleep quality, insomnia presence and severity, obstructive sleep apnoea syndrome (OSAS) risk of the patients who applied to family health centres and to determine the situations that might be related with these features., Methods: This study is a descriptive research and conducted in Ankara Güdül, Antalya Değirmenözü, Bursa Sırameşeler, Gaziantep Family Health Centre policlinics. The study population consisted of all patients over 18 years of age who were admitted to the family health centres for any reason. A 10-question questionnaire, Berlin questionnaire, Pittsburgh sleep quality questionare and insomnia severity questionare were applied by the researchers from October to December 2017 by using face-to-face interview method., Results: Two hundred nineteen nine people participated in study and 54.5% of them were women. According to the results of Pittsburgh Sleep Quality Questionare, it was found that 27.1% of the participants' sleep quality was good; according to the Berlin sleep questionnaire, 27.4% of the participants had high OSAS risk. According to Insomnia Severity Questionare, 27.1% of them had insomnia lower threshold, 15.4% had moderate insomnia and 3.7% severe insomnia., Conclusions: In this context, it will be very effective in terms of the quality of life of patients in order to determine the conditions that disrupt sleep hygiene and to perform the necessary interventions which can be intervened in the primary healthcare institutions and the other patients to be delivered to the related upper levels., (© 2020 John Wiley & Sons Ltd.)

Objective: The tools used for critically appraising the quality of clinical practice guidelines are complex and not suitable for the busy end users. So rapid, effective and simple instruments are more preferred. The aim of this study is to compare two critical appraisal tools: iCAHE as a rapid instrument and AGREE II as a complex instrument on guideline quality assessment., Material and Methods: The diabetes mellitus guidelines of the Scottish Intercollegiate Guidelines Network (SIGN), the National Institute for Health and Clinical Excellence (NICE), the International Diabetes Federation (IDF) and the Society of Endocrinology and Metabolism of Turkey (SEMT) were assessed separately by four appraisers using the iCAHE and AGREE II instruments. The mean iCAHE criteria scores and the total and domain AGREE II scores given by the four appraisers are presented for each guideline., Results: No statistically significant difference was detected between the iCAHE scale scores of the guidelines evaluated (P = 0.063). The rank of the guidelines according to their average total iCAHE and AGREE II instrument scores was similar. The iCAHE mean scores of the guidelines were as follows: NICE, 92.85%; SIGN, 92.85%; IDF, 66.07% and SEMT, 73.21%. The AGREE II mean scores of the guidelines were as follows: NICE, 87.13%; SIGN, 78.25%; IDF, 53.44% and SEMT, 53.22%., Conclusions: In addition to being a quality scale, the iCAHE checklist is easy, practical and short to implement. It also helps the users to understand the quality of the guideline in a shorter time. To increase the use of guidelines, it is important that users with little experience and time use the iCAHE scale as a rapid appraisal tool, but more studies are needed to decide the best appraisal tool., (© The Author(s) 2020. Published by Oxford University Press on behalf of International Society for Quality in Health Care. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Chronic granulomatous disease (CGD) is characterized by defective microbial killing due to mutations affecting subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Definitive genetic identification of disease subtypes may be delayed or not readily available., Objective: Sought to investigate the role of intracellular staining of NADPH oxidase enzyme subunits in predicting the respective genetic defects in patients with CGD and carriers., Methods: Thirty-four patients with genetically inherited CGD, including 12 patients with X-linked CGD (gp91 phagocyte oxidase (phox) deficiency due to cytochrome b-245, beta polypeptide [CYBB] mutations) and 22 patients with autosomal-recessive CGD (p22 phox , p47 phox , and p67 phox deficiency due to cytochrome b-245, alpha polypeptide [CYBA], neutrophil cytosolic factor 1 [NCF1] and NCF2 mutations, respectively) were recruited from different immunology centers and followed up prospectively. Dihydrorhodamine testing and NADPH oxidase subunit expression in white blood cells were determined by flow cytometry., Results: gp91 phox and p22 phox defects, which result in simultaneous loss of both proteins due to their complex formation, were differentiated only by comparative analysis of patients' and mothers' intracellular staining. p47 phox and p67 phox protein expression was almost undetectable in patients compared with carrier mothers and healthy controls. The expression values of the respective subunits were found to be significantly higher in all controls as compared with carrier mothers, which in turn were higher than those of patients., Conclusions: Analysis of NADPH oxidase enzyme subunits by flow cytometry in patients and carriers is useful in the rapid prediction of the genetic defect of patients with CGD, thus guiding targeted sequencing and aiding in their early diagnosis., (Copyright © 2020 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Introduction: Common variable immunodeficiency (CVID) is characterized by recurrent infections, autoimmunity, lymphoproliferation, hypogammaglobulinemia, and defective antibody production. In CVID, B-cell abnormalities were described to predict end organ involvement and prognosis. Pediatric-onset CVID is much rarer than adult CVID, and lymphocyte subset abnormalities have not been thoroughly evaluated., Objective: We sought to determine lymphocyte subset abnormalities and their association with end organ involvement in pediatric-onset CVID patients., Methods: The clinical manifestations and laboratory findings including absolute numbers and percentages of B-, T-, and NK cell populations were assessed in pediatric-onset CVID patients and compared to age-matched healthy controls. The patients were divided into 2 groups according to age at assessment (pediatric CVID patients: 10-16 years, n = 9; and adult CVID patients: >16 years, n = 13). The comparisons between lymphocyte subsets and organ involvement were also evaluated., Results: Mean age at symptom onset was 18 (3-204) months. All CVID patients with pediatric onset had decreased levels of total and memory B cells, CD4+ T cells, CD4+CD45RA+ naive T cells, and recent thymic emigrant (RTE) cells. On the other hand, they had increases in CD8+CD45RO+ memory T cells. Interestingly, adult CVID patients demonstrated high frequencies of activated and double-negative T cells, which were unique only for this group of patients. Specific cellular abnormalities associated with the reduction in B and NK cells and increase in CD8+ T cells were found in patients with bronchiectasis. Moreover, in pediatric CVID patients, low serum IgA levels and decreased numbers of naive T and RTE cells were determined as risk factors for chronic diarrhea., Conclusions: Specific abnormalities in B- and T-lymphocyte compartments were identified in pediatric-onset CVID patients and appear to be associated with end organ manifestations., (© 2020 S. Karger AG, Basel.)

Background: LPS-responsive beige-like anchor (LRBA) deficiency presents with susceptibility to infections, autoimmunity, and lymphoproliferation. The long-term efficacy of cytotoxic T-lymphocyte-associated antigen 4-immunoglobulin (abatacept) as targeted therapy for its immune dysregulatory features remains to be established., Objective: To determine the clinical and immunologic features of LRBA deficiency and long-term efficacy of abatacept treatment in controlling the different disease manifestations., Methods: Twenty-two LRBA-deficient patients were recruited from different immunology centers and followed prospectively. Eighteen patients on abatacept were evaluated every 3 months for long-term clinical and immunologic responses. LRBA expression, lymphocyte subpopulations, and circulating T follicular helper cells were determined by flow cytometry., Results: The mean age of the patients was 13.4 ± 7.9 years, and the follow-up period was 3.4 ± 2.3 years. Recurrent infections (n = 19 [86.4%]), immune dysregulation (n = 18 [81.8%]), and lymphoproliferation (n = 16 [72.7%]) were common clinical features. The long-term benefits of abatacept in 16 patients were demonstrated by complete control of lymphoproliferation and chronic diarrhea followed by immune dysregulation, most notably autoimmune cytopenias. Weekly or every other week administration of abatacept gave better disease control compared with every 4 weeks. There were no serious side effects related to the abatacept therapy. Circulating T follicular helper cell frequencies were found to be a reliable biomarker of disease activity, which decreased on abatacept therapy in most subjects. However, high circulating T follicular helper cell frequencies persisted in 2 patients who had a more severe disease phenotype that was relatively resistant to abatacept therapy., Conclusions: Long-term abatacept therapy is effective in most patients with LRBA deficiency., (Copyright © 2019 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Rationale, Aims, Objectives: Clinical Practice Guidelines are mostly developed by 3 methods; namely, de novo, adoption, and adaptation. Nonpublished studies and authors experience shows that most guidelines in Turkey are either by adoption or by adaptation. There is no available local tool for adaptation, so the process is not standardized and most of the time not explicitly defined. The objective of this study is to search for international guideline adaptation tools and test their feasibility in Turkish context, to serve a final goal of developing a unique local strategic tool for guideline adaptation., Methods: The methodological design of this study includes selection of an international tool for Clinical Practice Guideline adaptation, piloting this tool with selected Turkish guidelines, identifying the feasibility of this tool and exploring the needs for adaptation of the tool, drawing recommendations for adaptation of the strategies, and validation of the process by local experts., Results: The study from planning phase to finalizing the guidance, including pilot studies and panel but excluding translation of ADAPTE, lasted 18 months. Nine researchers were involved in the adaptation process and 15 more experts were involved in the validation panel. Following the suggestions of the research team on modifications and validation through the expert panel; 2 steps of the ADAPTE toolkit were rejected, 2 steps were accepted by modification, 7 steps were accepted by additional recommendations. In addition, 2 tools were suggested to be added to the toolkit., Conclusion: This is the first study on adaptation of guidelines in Turkey. Pilot adaptation of 2 guidelines with ADAPTE revealed that ADAPTE is a useful and feasible tool in Turkish setting, but might require certain changes in recommendations and revision of tools., (© 2017 John Wiley & Sons, Ltd.)