Ufuk Universitesi Akademik Acikerisim Sistemi
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Türkiye’de üçüncül bir merkezden duchenne ve becker müsküler distrofili hastaların klinik özellikleri ve yaşam kalitesi
Introduction: Duchenne Muscular Dystrophy (DMD) and Becker Muscular
dystrophy (BMD), are chronic and progressive and rare genetic disorders that
cause systemic involvement such as progressive muscle deterioration, motor
disability, cardiomyopathy, and respiratory problems, with an increased risk of
cognitive decline and psychological problems They are a group of neuromuscular
diseases in which psychological problems affect negatively on quality of life
(Qol) not only patients but also caregivers. This study demonstrates the clinical
features of patients with DMD/BMD and their caregivers and compares controls
with respects to psychological and social aspects.
Materials and Methods: A total of 20 patients (3 with BMD and 17 with DMD),
aged between 8 and 18 years, and 20 age-matched healthy children were included
in this descriptive and cross-sectional study. The patients were evaluated by the
pediatric neurology, cardiology, and psychiatry departments at the study time. Their
demographic and clinical features were recorded. The Wechsler Intelligence Scale
for Children-Revised (WISC-R), Pediatric Quality of Life Inventory (PedsQL)
and its parent form, and the Strengths and Difficulties Questionnaire (SDQ) were
applied to all of the participants.
Results: The QoL scores were lower in patients with moderate and severe DMD/
BMD and their caregivers. In patients with DMD and in both groups, emotional
symptoms, peer problems and prosocial behavior scores were higher in the subsets
of the SDQ. The PedsQL child-parent scores were lower in all of the subsets with
statistical significance.
Conclusions: DMD and BMD comprise a group of chronic diseases with multiple
complications that are difficult to manage. A QoL equal or close to that of the
healthy children should be targeted. Today, proposed or experimental treatments
for this disease group are assessed based on their ability to enhance QoL. Inquiring
into the QoL and counseling should become routine.Giriş: Duchenne ve Becker musküler distrofi (DMD,BMD); progresif kas güçsüzlüğü, motor beceriksizlik, kardiyomyopati ve r
solunumsal problemler, artmış bilişsel kayıp riski ve psikolojik problemler gibi sistemik bulgularla seyreden kronik, progresif seyirli,
nadir genetik bozukluklardır. Bu nöromusküler hastalık grubunda yalnızca hastaların değil bakımverenin de yaşam kalitesinin
negatif yönde etkilendili psikolojik problemlerin eşlik ettiği durumlardır. Bu çalışmada; DMD/BMD hastalarınnın klinik özellikleri
yanısıra hastalar ve bakımverenlrin psikolojik ve sosyal etkileninmleri ortaya konulmaya çalışılmıştır.
Gereç ve Yöntem: 3’ü BMD, 17’si DMD tanılı, 8-18 yaş aralığındaki 20 hasta ile age-matched 20 sağlıklı control çalışmaya
dahil edilerek tanımlayıcı ve kesitsel bir çalışma planlanmıştır. Hastalar; çocuk nörolojisi, çocuk kardiyolojisi ve çocuk psikiyatrisi
tarafından eş zamanlı değerlendirilmiştir. Demografik ve klinik özellikleri kaydedilmiştir. Çocuklar için revize edilmiş Wechsler
zeka ölçeği,Çocuklar ve ebeveynler için yaşam kalitesi ölçeğ ve Güç ve Güçlükler Anketi hasta ve kontrol grubuna uygulanmıştır.
Bulgular: Klinik ciddiyeti orta ve ciddi olan DMD/BMD hastalarında sağlıklı kontrollerle karşılaştırıldığında hem hasta hem
bakımverenin QOL skorları oldukça düşük saptandı. SDQ’ya göre ise alt içerikler açısından hem tüm grupta hem de DMD tanılı
hasta grubunda emosyonel symptoms, peer problems ve social behavior açısından sağlıklı kontrollere göre yüksek skorlar elde
edildiği başka bir deyişle bu alanlarda negative yönde belirgin etkilenme olduğu görüldü. PedsQL child-parent skorları tüm alt
başlıklarda sağlıklı kontrollere göre istatistiksel önemlilikle birlikte düşük saptanmıştı.
Sonuç: DMD/BMD yönetmekte güçlük yaşanan çok sayıda komplikasyonu olan bir grup kronik hastalıktır. Yaşam kalitesinin
sağlıklı çocuklar kadar veya onlara yakın bir düzeyde tutulması hastalık yönetiminin ana hedefi olmalıdır. Günümüzde bu hastalık
grubundaki önerilen veya henüz deneme aşamasındaki tedaviler de yaşam kalitesinin arttırılması ön koşulu ile değerlendirilmektedir.
Bu nedenle; klinik pratikte sağlık hizmetleri yanısıra yaşam kalitesinin sorgulanması ve arttırıcı önerilerin verilmesi bir rutin
olmalıdır
Experience with oral tofacitinib in severe alopecia areata with different clinical responses
Background: Alopecia areata (AA) and generalized form, universalis (AU) are common
causes of noncicatricial alopecia, targeting anagen hair follicles. A dominant
interferon-gamma
transcriptional signaling and cytotoxic T lymphocytes were accused
as the main drivers of disease pathogenesis. Tofacitinib is a Janus kinase inhibitor
that has been proven to interfere with the positive feedback loop between the
follicular cell and the cytotoxic T lymphocytes in AA. There is an increasing number
of studies reporting success with tofacitinib in AA.
Aims: We aimed to assess oral tofacitinib's safety and efficacy in 13 recalcitrant
AA and AU patients.
Methods: This is a retrospective pilot study performed between 2017 and 2020. The
demographic features and the treatment responses were evaluated with Severity of
Alopecia Tool score changes.
Results: Thirteen recalcitrant alopecia areata patients (3 AA, 10 AU), aged between
17 and 49, were included in the study. The treatment duration was 3-15
months. All
three AA patients responded well; however, the therapy was unsuccessful in five of
ten AU patients. Relapse was observed in one of the AA and three of the AU responders.
Acneiform lesions and elevation of transaminases were the major side effects.
Conclusion: Tofacitinib seems to be more promising and thriving in the treatment
of AA than AU. Starting the therapy earlier can bring more successful results.
Unfortunately, even in the cases that fully respond to treatment, relapse can be observed
after discontinuation of the treatment. It is essential to inform patients about
this situation in reducing the frustrations that may occur later
Concomitant occurrence of hepatopulmonary hydatid cysts in Turkey
Background: We aimed to report the demographic characteristics with diagnosis
and treatment methods in patients with concomitant hepatopulmonary hydatid
cysts.
Methods: Over a ten-year period (from 2002–2020) in Konya, Turkey, surgery
was performed on 52 patients with hepatopulmonary hydatid cyst. Main outcome
measure(s) were 52 hydatid cysts patients, which had cysts both in the liver and
lungs, were investigated regarding their age, gender, cyst localization, suppuration,
symptoms, and treatment methods.
Results: Seventeen of the patients were males. Their mean age was 39.7±18.8
years. The most common occupation was housewifery. The most common symp tom was coughing and none of the patients with concomitant hepatopulmonary
hydatid cysts was asymptomatic. The pulmonary hydatid cysts were mostly en countered in the right lung and the majority of the hepatic hydatid cysts were ob served in the right lobe. The mean hospitalization time of the operated patients
was 17.12±6.7 days.
Conclusion: In patients with hydatid cysts localized concomitantly in the right
lung and subdiaphragmatic area, right thoracotomy for the pulmonary cyst and a
transdiaphragmatic approach for the hepatic cyst is a safe, effective, and comforta ble method
Improvement of life after PVCR in complete paraplegic patients with posttraumatic severe kyphosis
AIM: To determine the effect of posterior vertebral column resection (PVCR) in patients with paraplegia by using the American Spinal Injury Association (ASIA) score and Scoliosis Research Society (SRS)-22 questionnaire. MATERIAL and METHODS: Twelve patients with posttraumatic paraplegia and severe angular kyphosis (>60°) had undergone PVCR between 6-24 months after the trauma for severe pain, persistent vertebral instability and difficulty in adherence to rehabilitation. ASIA scores and SRS-22 questionnaire results obtained in the preoperative and postoperative periods, and the last control were statistically compared to assess the presence of any change.RESULTS: The average age of twelve patients included in this study was 35.6 ± 10.2 (21-51) years. Female/male ratio was 2/10 (20.0%). The mean follow-up duration was 50.3 ± 17.6 (24-86) months. None of the patients had additional changes in neuromonitoring records during surgery. The mean preoperative kyphotic angle of the patients was 66.58° ± 7.1° (60°-82°) which decreased to 7.0° ± 5.4° in the postoperative period (p0.05).CONCLUSION: In the light of these data, it can be stated that PVCR is a safe and reliable procedure in paraplegic patients with rigid posttraumatic kyphosis and increases patient satisfaction
Outcomes with Additional Manual Lymphatic Drainage to Rehabilitation Protocol in Primary Total Knee Arthroplasty Patients: Preliminary Clinical Results
Objective: This study was carried out to evaluate the clinical outcomes such as pain, kinesiophobia and quality of life of additional manual lymph drainage (MLD) technique in theafter total knee arthroplasty (TKA) surgery.Methods: Twenty-one TKA patients were randomly allocated to a control group (n:10) and MLD group (n:11). Both groups received routine postoperative rehabilitation. MLD group also received MLD in the first three days after surgery. Clinical assessment was undertaken on postoperative 3rd day and at 6th week. This included knee pain using Visual Analog Scale (VAS), kinesiophobia using Tampa Kinesiophobia Scale (TKS) and quality of life using Nottingham Health Profile (NHP).Results: VAS and TKS values of the MLD group on the 3rd day and at the 6th week were found to be significantly lower than the control group (p<0.05). The 6th week NHP values of the MLD group were found to be significantly lower than the control group (p<0.05). For all three values, within-group, and between-group according to processes the effect size of the MLD group is greater than the control group.Conclusion: The results of the present study demonstrated that addition of MLD application to the standard rehabilitation protocol of TKA positively affected the healing process and it also improved the quality of life by reducing postoperative kinesiophobia and pain level. Adding this special technique to the rehabilitation program in TKA surgeries will provide patient satisfaction and contribute positively to the improvement in clinical outcomes
The Effect of The Subjective Well-Being Increasing Program on Subjective and Psychological Well-Being of Adolescents Having Harmful Habits
This study aims to investigate the effectiveness of the subjective well-being increasing program on the subjective and psychological well-being of adolescents having harmful habits. This study which is used an experimental design including pre-test and post-test measurements with experimental and control groups, Personal Information Form, Adolescent Subjective Well-Being Scale, and EPOCH Scale were used to collect data. The experimental group received a subjective well-being program while the control group received no intervention. The results of Wilcoxon Signed Ranks showed that the posttest scores of the experimental group were significantly higher than the pre-test scores. In addition, the results of Mann Whitney U showed that the post-test scores of the experimental group were significantly higher than the post-test scores of the control group. Finally, the results obtained were discussed and interpreted according to the background of the research. Besides, suggestions were offered to further studies
İntern Programı Kapsamında Hemşirelik Öğrencilerinin Mesleki Uygulama Yeterliliği
GİRİŞ ve AMAÇ: Bu çalışma intern programı kapsamında hemşirelik öğrencilerinin mesleki uygulama yeterliliğini belirlemek amacıyla yapılmıştır. YÖNTEM ve GEREÇLER: Tanımlayıcı ve kesitsel tipte yapılan araştırmanın evrenini, bir üniversitenin hemşirelik bölümünde okuyan dördüncü sınıf öğrencileri oluşturmuştur (N=301). Araştırmaya katılmayı kabul eden öğrenciler örnekleme alınmıştır (n=273). Veriler kişisel bilgi formu ve Nightingale İntern Programı Değerlendirme Ölçeği (N-İPDÖ) kullanılarak toplanmıştır. BULGULAR: Öğrencilerin yaş ortalaması 22.16±0.97, %88.3’ü kadındı. Öğrencilerin %59.0’unun hemşirelik bölümünü isteyerek tercih ettiği, %92.3’ünün mezuniyet sonrası hemşirelik yapmayı istediği, %43.6’sının klinik hemşiresi olarak çalışmak istediği ve %89.0’unun hemşirelik mesleğinde ilerlemeyi planladıkları saptanmıştır. N-İPDÖ ortalama puanının 3.80±0.54 olduğu belirlenmiştir. Ölçek alt boyut ortalama puanları; programın genel özellikleri 3.57±0.64, kişisel gelişim 3.92±0.63, mesleki gelişim 3.98±0.58, rehberlik/danışmanlık 3.69±0.76, uygulama alanının özellikleri 3.34±0.80, hasta ve yakınları ile iletişim 4.08±0.69 olarak bulunmuştur. Bölümü isteyerek tercih eden, mezuniyet sonrası hemşirelik yapmak isteyen öğrencilerin N-İPDÖ ortalama puanının daha yüksek olduğu (p.05). TARTIŞMA ve SONUÇ: Hemşirelik öğrencilerinin intern programına ilişkin değerlendirmelerinin olumlu yönde olduğu belirlenmiştir. Öğrencilerin N-İPDÖ “hasta ve yakınları ile iletişim” alt boyutundan en yüksek, “uygulama alanının özellikleri” alt boyutundan en düşük puanı aldıkları saptanmıştır. İntern programının hemşirelik müfredatına eklenmesi ve uygulama alanının özelliklerinin iyileştirilmesine yönelik düzenlemeler yapılması önerilmektedir
ULUSAL KÜLTÜR BOYUTLARININ İŞLETMELERİN KURUMSAL SOSYAL SORUMLULUK ÇALIŞMALARINA ETKİSİ
Bu çalışmanın temel amacı ulusal kültür boyutlarının, işletmelerin kurumsal sosyal sorumluluk çalışmaları üzerindeki muhtemel etkisini incelemektir. Araştırmanın amacına ulaşmak için GLOBE Projesi ile Ulusal Kurumsal Sosyal Sorumluluk Endeksinde yer alan altmış iki ülkeye ait veriler kullanılmıştır. Verilerin analizinde IBM SPSS 22.0 programından faydalanılmıştır. Yapılan korelasyon ve regresyon analizleri sonucunda ulusal kültür boyutları ile ulusal kurumsal sosyal sorumluluk çalışmaları arasında bir ilişki olduğu ve ulusal kültürün KSS çalışmalarını etkilediği belirlenmiştir. Ulusal kültürün alt boyutlarından belirsizlikten kaçınma, girişkenlik, gelecek odaklılık ve kolektivizm II değişkenlerinin Ulusal Kurumsal Sosyal Sorumluluk üzerinde önemli (anlamlı) bir yordayıcı etkisi olduğu ortaya çıkmıştır.Diğer değişkenler istatistiksel olarak önemli bir etkiye sahip değildir
The incidence of hypophosphatemia in the early posttransplant period in renal transplant recipients and its association with graft function
Objectives: To investigate the prevalence of posttransplant hypophosphatemia in the early posttransplant period among renal transplant recipients in relation to its impact on renal graft function.Methods: A total of 78 renal transplant recipients who were transplanted between January 2016 and March 2020 were included in this retrospective single center study. Data on laboratory findings (phosphate, creatinine, estimated glomerular filtration rate [eGFR], albumin, serum corrected calcium and parathyroid hormone [PTH] levels) at pre- and posttransplant 3 month follow up period were recorded.Results: Hypophosphatemia was detected in 16 (20.8%), 13 (16.7%) and 7 (9.1%) patients at the posttransplant day 10, month 1 and month 3, respectively. Posttransplant day 10 and day 30 measurements revealed significantly lower serum creatinine values (p < 0.001 and p < 0.07, respectively) and significantly higher eGFR values (p = 0.009 and p = 0.036, respectively) in the hypophosphatemic group compared to the normophosphatemic group. Serum phosphate displayed linear relationship with creatinine at day 10 (r=0.687, p < 0.001), day 30 (r=0.301, p = 0.007), while not correlated with PTH levels at posttransplant day 10, day 30 and day 90.Conclusions: Our findings suggest that hypophosphatemia is common in the early posttransplant period, particularly first month after kidney transplantation, being associated with better renal graft function