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Objective: Takayasu arteritis is a systemic vasculitis. There have been some cases reported in the literature that suggest distortion of hearing in this disease. Patients may experience hearing loss as the first symptom or during the course of the disease. This study aims to investigate disturbances in hearing function in patients with Takayasu arteritis by comparing them to healthy subjects. Materials and Methods: Patients and randomly selected age-gender compatible healthy subjects were enrolled in the study. Subjects who previously had congenital hearing loss, otologic surgery, severe head trauma, ototoxicity, were exposed to noise, or worked in noisy environments beforehand were excluded. Pure Tone Audiometry (PTA), tympanometry, and Otoacoustic Emissions (OAE) were performed on all subjects. The Indian Takayasu Arteritis Activity Score (ITAS 2010) was also estimated for the Takayasu group. Statistical comparisons of air and bone-conduction thresholds and OAE values between the Takayasu and control groups were performed. Results: A total of 31 subjects were included in each group, comprising 27 women and 4 men. The mean ages were 45.3±2 for the Takayasu group and 44.8±1.8 for the control group, respectively. Both groups were similar in age and gender composition (p>0.05). No significant differences were found between the Takayasu and healthy control groups in terms of PTA averages and OAE values (p>0.05). However, significant differences were observed at the right and left 8000 Hz frequencies (p<0.05), with higher values in the Takayasu group. Additionally, two patients in the Takayasu group experienced idiopathic sudden sensorineural hearing loss (ISSHL). Conclusion: Hearing function was similar between the Takayasu group and age-gendermatched healthy controls, except at the 8000 Hz frequency in PTA. Takayasu arteritis may manifest with ISSHL. [ABSTRACT FROM AUTHOR]

To evaluate the clinical results of insulin degludec/aspart (IDEgAsp) therapy and its effect on the fear of hypoglycemia. A prospective observational study has been conducted through surveys of 36 patients using insulin because of type 2 diabetes mellitus who initiated treatment with IDegAsp switching from other insulins. Patients, 18–75 years old, were recruited to the study, consecutively. Participants' age, gender, height, weight, body mass index (BMI), daily insulin dose, glycated hemoglobin (HbA1c), hypoglycemia rate, hypoglycemia fear survey (HFS) were recorded at the beginning of the study. By the end of 12th month, data was re-measured and compared with each other. HbA1c was declined by mean of −1.59% (95% CI −1.06 to −2.12, p<0.001). There was also a significant decrease in mean, daily insulin dose, weight and BMI values of patients via IDegAsp. While there was an increase in the amount of dipeptidyl peptidase 4-inhibitors (DPP4-i) and sodium-glucose co-transporter 2-inhibitors (SGLT2-i), there was a decrease in daily injection frequency. There was also a significant decrease in the median values of monthly hypoglycemia rate (from 2.0 to 1.0, p<0.001) and the entire HFS scores (HFS-T: from 1.09 to 0.73, p<0.001; HFS-B: from 0.83 to 0.60, p<0.001; HFS-W: from 1.33 to 0.88, p<0.001). There was a strong positive correlation between ΔHFS-B and daily injection frequency (Rho: 0.398; P: 0.016). IDegAsp co-formulation, combined with DPP4-i and/or SGLT2-i, can provide usefulness in terms of rates of hypoglycemia, reduced HbA1c, less injection administration, and decreased the fear of hypoglycemia in diabetics. [ABSTRACT FROM AUTHOR]

Objective: Allogeneic hematopoietic stem cell transplantation is a treatment with a considerable rate of complete cure in hematological disorders. Hepatic complications are common causes of morbidity and mortality affecting the survival after stem cell transplantation. This study was conducted to identify risk factors for hepatic dysfunction and related potential factors affecting the survival in patients undergoing allogeneic hematopoietic stem cell transplantation. Materials and Methods: We retrospectively evaluated the data from 300 allogeneic hematopoietic stem cell transplantation recipients between March 2004 and May 2014 in the Erciyes University Hematology and Bone Marrow Transplantation Center, Turkey. The study included 300 patients and their serial monitorization of the liver function tests, examined before and after transplantation. Results: The transplantation in 30 patients was performed from haploidentical donors and in 13 patients from unrelated donors. We identified the liver function abnormalities in 71.7% of patients in the post-transplantation period. The most common causes were graft-versus-host disease (43.6%), drug toxicity (24.7%), and sepsis (13.9%). Post-transplantation liver abnormalities were more common in patients with acute leukemia (p=0.02), iron overload (p<0.001), and in those who also had transaminitis in the pretransplantation period (p<0.001). Relapsed underlying disease (p<0.001), iron overload, and a bilirubin level >2 mg/dL in association with hepatic dysfunction during the post-transplantation period were identified as major factors influencing mortality following transplantation (p<0.001). Conclusion: We concluded that liver function abnormalities are frequent in the hematopoietic stem cell transplantation process. For a successful management, it is important to monitor the liver function and to identify additional risk factors before and after transplantation. [ABSTRACT FROM AUTHOR]

Giriş: Akut generalize ekzantematöz püstüloz (AGEP), ani başlangıçlı vücutta yaygın döküntüler ile giden bir hastalıktır. Ödemli eritem zemininde gelişen, steril püstüllerle karakterizedir. Genellikle ilaç ilişkili olup, sık kullandığımız hidroksiklorokin (HCQ) AGEP'nin nadir nedenlerindendir. Genellikle ilaç kesiminden sonra tablonun spontan gerilemesi beklenir. Bizde HCQ sonrası AGEP gelişen, ilacın kesilmesi ve steroid tedavisine rağmen direnç göstererek olağan iyileşme sürecini takip etmeyen olgumuzu sunarak farkındalığı artırmayı amaçladık. Olgu: Altı aydır eklem şikayetleri olan altmış yaşındaki kadın hastanın romatolojik sorgulamasında ağız kuruluğu, fizik muayenesinde eklemlerde hassasiyet mevcuttu. Anti-nükleer antikor 1/160 titrede, anti-SSA (++) olarak görüldü. Hastaya ön tanı olarak bağ dokusu hastalığı düşünülerek HCQ ve deltacortil başlanıldı. Üç hafta sonra gövdede yoğun olmak üzere yaygın döküntülüyle başvurdu. Muayenesinde vücutta yer yer deskuamasyonun eşlik ettiği püstüler lezyonlar ve eritemli plaklar (Resim 1) görüldü. Ateşi subfebril, diğer sistem muayeneleri doğaldı. Öz geçmişinde ve soy geçmişinde psoriasis yoktu. Labarotuvarda hemoglobin 10,4 g/dL, lökosit 20,000/mm3 (%81 nötrofil), eritrosit sedimentasyon hızı 5 mm/s, C-reaktif protein 62,4 mg/L, romatoid faktör, anti-siklik sitrüline peptit ve diğer parametreler normaldi. Kültürlerinde üreme olmadı. Lezyonlardan biyopsi yapıldı; "subkorneal püstüler dermatoz, bulgular AGEP ile uyumludur" olarak rapor edildi. Dermatoloji ile hastada klinik ve histopatolojik olarak AGEP düşünüldü, HCQ kesildi. Hastaya 64 miligram (mg) metilprednizolon, antihistaminik ve topikal nemlendirici başlandı. Yüksek doz steroide iyi yanıt verse de doz azaltılırken lezyonların direnç göstermesi üzerine tedaviye 15 mg metotreksat eklendi. Lezyonların tedavi ile geriledi, steroid dozu kademeli olarak azaltılarak kesilebildi. Sonuç: Romatoloji pratiğinde sıkça kullanılan ve genellikle masum olarak kabul gören HCQ'ya bağlı, latent periyodu uzun, AGEP gelişebilmekte, steroid tedavisine direnç gösterebilmektedir. Literatürde bu tip olgular ve metotreksatla tedavisi azdır. Biz de hastamızı metotreksat ile başarılı bir şekilde tedavi ettik. [ABSTRACT FROM AUTHOR]

Giriş: İmmünoglobulin G4 ile ilişkili hastalık (IgG4-RD) birçok malign, enfeksiyöz ve enflamatuvar bozukluğu taklit eden immün aracılı ve fibroenflamatuvar multiorgan tutulumlu bir hastalıktır. IgG4-RD tanısı klinik, serolojik, radyolojik ve karakteristik histopatolojik bulgulara dayanır. Radyolojik görüntülemelerde; kitle veya şişlik şeklinde olan yaygın-fokal organ lezyonları görülür. Histopatolojik değerlendirmede; IgG4 için yüksek oranda pozitif boyanan plazma hücreleri, storiform fibrozis, obliteratif flebit ve yoğun lenfoplazmasitik infiltrasyonlar karakteristiktir. Olgu: Bilinen kronik hastalığı olmayan 42 yaşında kadın hasta mide ve sırt ağrısı nedeni ile tetkik edilirken çekilen bilgisayarlı tomografi ve manyetik rezonans görüntülemede pankreas uncinat ve kuyruk kesiminde solid nodüler kitle lezyonu saptanmış, bu kitleler pozitron emisyon tomografisinde malignite lehine yorumlanmış (Resim 1). Hemogram ve biyokimya değerleri normal, serum tümör belirteçleri negatif bulunmuş. Hastaya 01/09/2021'de pankreas adenokarsinomu ön tanısı ile whipple operasyonu, total gastrektomi ve total splenektomi uygulanmış. Histopatolojik incelemede pankreas duktus yapılarını çevreleyen yoğun lenfoplazmositik enflamasyon, bazı alanlarda storiform özellikte fibrosis, perivenüler enflamasyon, bir büyük büyütme alanında IgG4 ile boyanan 30 plazma hücresi saptanmış, neoplazi saptanmamış. Tarafımızca değerlendirilerek gönderilen IgG4 düzeyi 1,970 g/L idi. Hasta mevcut görüntüleme ve patoloji bulguları ile IgG4-RD olarak kabul edildi. Hastaya metilprednizolon 0,5 mg/kg ve metotreksat haftalık 15 mg olarak başlandı. Operasyon sonrası 30 kilogram kaybı olan ve brittle diyabet gelişen hastanın takibi stabil olarak devam etmektedir. Sonuç: IgG4 ilişkili otoimmün pankreatit kortikosteroid tedavisine oldukça iyi yanıt veren bening bir hastalıktır. Pankreas karsinomlarını dışlamak için operasyon kararı öncesi hastalardan serum IgG4, CA19-9 düzeyi bakılması ve endoskopik ultrason kılavuzluğunda ince iğne aspirasyonu ile histopatolojik tanı konulması hastaları gereksiz majör cerrahi girişimlerden ve buna bağlı gelişebilecek komorbidetelerden koruyacaktır. [ABSTRACT FROM AUTHOR]

Aim: This study (1) compared skeletal muscle mass (SMM) and muscle strength in familial Mediterranean fever (FMF) patients with those of healthy controls (HCs) and (2) investigated the association of SMM and muscle strength with disease severity and quality of life (QOL). Materials and Methods: This study included 31 FMF patients and 30 matched HCs. Disease severity was evaluated using the International Severity Scoring System for FMF (ISSF). Body composition parameters were measured using a bioelectrical impedance analysis. Grip and pinch strengths were calculated for muscle strength. Health status was assessed with Short Form 36 (SF-36). Results: The all-body composition parameters of the FMF patients, including SMM, were similar to those of the HCs, with significantly lower grip and pinch strengths. All SF-36 scores of the FMF patients were significantly lower than those of the HCs. Positive correlations were observed between muscle-related indices and pinch strength for some SF-36 domains. Conclusion: Muscle- or fat-related indices were similar among adult patients with FMF and the HCs. In contrast, patients with FMF had a poorer health-related QOL and lower grip and pinch strengths than the HCs. Further studies are needed to determine the clinical significance of these correlations in patients with FMF. [ABSTRACT FROM AUTHOR]

Interstitial lung disease (ILD) is a common complication that can develop during the course of systemic sclerosis (SSc). Nindetanib is an antifibrotic drug approved for the treatment of systemic sclerosis-associated interstitial lung disease. Although there is an insufficient data on the development of pneumothorax, the safety of Nintedanib treatment is also uncertain. We observed recurrent resistant pneumothorax under nintedanib treatment in a patient with systemic sclerosis-associated interstitial lung disease. Nintedanib use may increase the risk of developing refractory pneumothorax. Ssc patients who are started on nintedanib should be followed carefully for pneumothorax.