bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Long-Term Clinical and Endoscopic Outcomes of Cronkhite–Canada Syndrome
Authors: Liu Shuang , You Yan , Ruan Gechong , Zhou Liangrui , Chen Dan , Wu Dong , Yan Xuemin , Zhang Shengyu , Zhou Weixun , Li Ji , Qian Jiaming 
Published: April 2020

INTRODUCTION:
The prognosis of Cronkhite–Canada syndrome (CCS) is considered poor. Despite the recent therapeutic improvements, the survival outcomes and prognostic factors have been less studied. This study aimed to investigate the long-term clinical and endoscopic outcomes of CCS.

METHODS:
Thirty-one patients diagnosed since 1999 and followed up for over 6 months were included. Data regarding survival outcomes, clinical symptoms, endoscopic findings, and treatment were collected and analyzed. R (version 3.6.1) was used to perform the survival analyses.

RESULTS:
The median (interquartile range) follow-up time was 42.5 (19.5–85.8) months. The 5-year overall survival (OS) was 87.4%. The maximum gastric polyp size over 2 cm was associated with worse OS (Hazard ratio [HR]: 18, 95% confidence interval [CI]: 1.6–210, P = 0.021). The 3-year relapse-free survival (RFS) after corticosteroid treatment was 66.8%. Age older than 60 years (HR: 7.0, 95% CI: 1.5–33.0, P = 0.015) and maximum gastric polyp size over 2 cm (HR: 6.0, 95% CI: 1.6–23.0, P = 0.009) were associated with worse RFS. Twenty-three patients received follow-up endoscopic examinations, with a median (interquartile range) follow-up time of 29.0 (14.0–53.5) months. Eight (34.8%) and 12 (52.2%) patients achieved complete remission under gastroscopy and colonoscopy, respectively. Colonic lesions showed a tendency of earlier responses compared with gastric lesions (25.0 [11.3–39.8] months vs 31.0 [21.0–39.8] months).

DISCUSSION:
Patients with CCS usually responded well to glucocorticoids with a fairly good 5-year survival rate. Large gastric polyp was associated with worse OS and RFS, whereas age older than 60 years was another predictor for worse RFS. Diffuse gastrointestinal lesions partly or completely resolved after treatment, and colonic lesions showed a better response than gastric lesions.
Acute Psychological Effects of Resistance Exercise in Men With Symptoms of Muscle Dysmorphia: A Pilot Study
Authors: SantaBarbara Nicholas J. , Nosrat Sanaz , Whitworth James W. , Ciccolo Joseph T. 
Published: April 2020

Abstract
SantaBarbara, NJ, Nosrat, S, Whitworth, JW, Ciccolo, JT. acute psychological effects of resistance exercise in men with symptoms of muscle dysmorphia: A pilot study. J Strength Cond Res 37(2): 277–283, 2023—This study tested the acute psychological effects of resistance exercise in men with symptoms of muscle dysmorphia. Twenty-one men completed 4 on-site sessions including a single session of moderate- (70% of 10 repetition maximum [RM]) and high- (100% of 10RM) intensity resistance exercise in a counter balanced order separated by at least 48 hours. State body image, perceived muscle size, exercise enjoyment, and ratings of perceived exertion (RPE) were assessed before, during, and after each session, and statistical significance was set a priori at p < 0.05. State body image significantly improve from pre to post during both sessions (p < 0.01), with greater effect sizes seen for the high- (d = 0.57) compared with the moderate- (d = 0.39) intensity session. Perceived muscle size improved from pre to post during the high-intensity only (p < 0.01, d = 0.66), and subjects enjoyed the high-intensity session significantly more than the moderate-intensity session (p = 0.01), despite significantly higher RPE at each timepoint (p < 0.01). Results contend with previous findings that show more positive psychological effects of moderate- compared with high-intensity resistance exercise. Education and client observation are essential to be aware of the potential for muscle dysmorphia. In instances where muscle dysmorphia is suspected, referral to qualified mental health professionals is recommended.
Idebenone Has Distinct Effects on Mitochondrial Respiration in Cortical Astrocytes Compared to Cortical Neurons Due to Differential NQO1 Activity
Authors: Jaber Sausan M. , Ge Shealinna X. , Milstein Joshua L. , VanRyzin Jonathan W. , Waddell Jaylyn , Polster Brian M. 
Published: April 2020

Idebenone is a synthetic quinone that on reduction in cells can bypass mitochondrial Complex I defects by donating electrons to Complex III. The drug is used clinically to treat the Complex I disease Leber's hereditary optic neuropathy (LHON), but has been less successful in clinical trials for other neurodegenerative diseases. NAD(P)H:quinone oxidoreductase 1 (NQO1) appears to be the main intracellular enzyme catalyzing idebenone reduction. However, NQO1 is not universally expressed by cells of the brain. Using primary rat cortical cells pooled from both sexes, we tested the hypotheses that the level of endogenous NQO1 activity limits the ability of neurons, but not astrocytes, to use idebenone as an electron donor to support mitochondrial respiration. We then tested the prediction that NQO1 induction by pharmacological activation of the transcription factor nuclear erythroid 2-related factor 2 (Nrf2) enables idebenone to bypass Complex I in cells with poor NQO1 expression. We found that idebenone stimulated respiration by astrocytes but reduced the respiratory capacity of neurons. Importantly, idebenone supported mitochondrial oxygen consumption in the presence of a Complex I inhibitor in astrocytes but not neurons, and this ability was reversed by inhibiting NQO1. Conversely, recombinant NQO1 delivery to neurons prevented respiratory impairment and conferred Complex I bypass activity. Nrf2 activators failed to increase NQO1 in neurons, but carnosic acid induced NQO1 in COS-7 cells that expressed little endogenous enzyme. Carnosic acid–idebenone combination treatment promoted NQO1-dependent Complex I bypass activity in these cells. Thus, combination drug strategies targeting NQO1 may promote the repurposing of idebenone for additional disorders.SIGNIFICANCE STATEMENTIdebenone is used clinically to treat loss of visual acuity in Leber's hereditary optic neuropathy. Clinical trials for several additional diseases have failed. This study demonstrates a fundamental difference in the way idebenone affects mitochondrial respiration in cortical neurons compared with cortical astrocytes. Cortical neurons are unable to use idebenone as a direct mitochondrial electron donor due to NQO1 deficiency. Our results suggest that idebenone behaves as an NQO1-dependent prodrug, raising the possibility that lack of neuronal NQO1 activity has contributed to the limited efficacy of idebenone in neurodegenerative disease treatment. Combination therapy with drugs able to safely induce NQO1 in neurons, as well as other brain cell types, may be able to unlock the neuroprotective therapeutic potential of idebenone or related quinones.
Visual Size Processing in Early Visual Cortex Follows Lateral Occipital Cortex Involvement
Authors: Zeng Hang , Fink Gereon R. , Weidner Ralph 
Published: April 2020

Neural activation in the early visual cortex (EVC) reflects the perceived rather than retinal size of stimuli, suggesting that feedback possibly from extrastriate regions modulates retinal size information in EVC. Meanwhile, the lateral occipital cortex (LOC) has been suggested to be critically involved in object size processing. To test for the potential contributions of feedback modulations on size representations in EVC, we investigated the dynamics of relevant processes using transcranial magnetic stimulation (TMS). Specifically, we briefly disrupted the neural activity of EVC and LOC at early, intermediate, and late time windows while participants performed size judgment tasks in either an illusory or neutral context. TMS over EVC and LOC allowed determining whether these two brain regions are relevant for generating phenomenological size impressions. Furthermore, the temporal order of TMS effects allowed inferences on the dynamics of information exchange between the two areas. Particularly, if feedback signals from LOC to EVC are crucial for generating altered size representations in EVC, then TMS effects over EVC should be observed simultaneously or later than the effects following LOC stimulation. The data from 20 humans (13 females) revealed that TMS over both EVC and LOC impaired illusory size perception. However, the strongest effects of TMS applied over EVC occurred later than those of LOC, supporting a functionally relevant feedback modulation from LOC to EVC for scaling size information. Our results suggest that context integration and the concomitant change of perceived size require LOC and result in modulating representations in EVC via recurrent processing.SIGNIFICANCE STATEMENTHow we perceive an object's size is not entirely determined by its physical size or the size of its retinal representation but also the spatial context. Using transcranial magnetic stimulation, we investigated the role of the early visual cortex (EVC) and the higher-level visual area, lateral occipital cortex (LOC), known to be critically involved in object processing, in transforming an initial retinal representation into one that reflects perceived size. Transcranial magnetic stimulation altered size perception earlier over LOC compared with EVC, suggesting that context integration and the concomitant change in perceived size representations in EVC rely on feedback from LOC.
MULTİPLE SKLEROZDA OPTİK KOHERENS TOMOGRAFİ İLE RETİNA SİNİR LİFİ TABAKASININ DEĞERLENDİRİLMESİ
Authors: SONGUR Murat Serkan , HAMAMCI Mehmet , ASLAN BAYHAN Seray , BAYHAN Hasan Ali , İNAN Levent Ertuğrul 
Published: April 2020

Amaç:
Multiple skleroz (MS) hastalarında spektral optik koherens tomografi (OKT) ile
retina sinir lifi tabakası (RSLT)
kalınlığını ölçmek ve bu
değerlerle hastaların genişletilmiş özürlülük durum ölçeğinin (EDSS) ilişkisini
değerlendirmektir.

Gereç ve Yöntem:
Bu ileri dönük çalışmada iki grup oluşturuldu.
Çalışmaya nöroloji kliniğinde Mc Donald kriterlerine göre multiple skleroz
tanısı konmuş ve takip edilen 30 hastanın 30 gözü ve 33 sağlıklı kontrol grubunun 33 gözü dahil
edildi. Nöroloji kliniğinde multiple skleroz hastalarına genişletilmiş
özürlülük durum ölçeğinin testi yapıldı. Tüm hastalara tam detaylı oftalmolojik muayene
yapıldıktan sonra spektral optik koherens tomografi ile temporal,nasal.inferior
ve superior kadranlarda retina sinir lifi tabakası kalınlıkları incelendi. Her
iki grupta da oküler patoloji saptanan ve oküler hastalık hikayesi olan hastalar
çalışma dışında bırakıldı.

Sonuçlar: Hasta
ve kontrol grubunun yaş ve cinsiyet dağılımı açısından birbirinden farkı
yoktu(p&lt;0,005). İstatiksel olarak iki grup arasında dört kadranda da anlamlı
fark bulundu. Sırasıyla; hastaların temporal retina sinir lifi tabakası
kalınlığı ortalaması 77,93 ±7,71 mm, kontrol
grubunun 83,66 ±5,16 mm
(p=0.001); hastaların nasal retina sinir lifi tabakası kalınlığı ortalaması
76,76 ±6,43 mm,
kontrol grubunun 80,18 ±4,17mm
(p=0.014); hastaların superior retina sinir lifi tabakası kalınlığı ortalaması
120,26 ±11,84mm,
kontrol grubunun 126,90±10,33mm (p=0.021); hastaların inferior retina sinir lifi
tabakası kalınlığı ortalaması 124,83 ±12,00 mm, kontrol
grubunun ise 133,12 ±12,77 mm (p=0.010) idi. multiple skleroz
hastalarına yapılan genişletilmiş özürlülük durum ölçeğinin testinin ortalaması
2,46±1.83 olarak bulundu. Genişletilmiş özürlülük durum ölçeğinin sonuçlarıyla
temporal (p&lt;0.001,r= -0.724) ve nasal (p&lt;0.001,r= -0.777) retina sinir
lifi tabakası kadranları arasında anlamlı ve yüksek düzeyde negatif korelasyon;
superior(p=0.001,r= -0.562) ve inferior(p=0.004,r= -0,515) retina sinir lifi
tabakası kadranları arasında anlamlı ve orta düzeyde negatif korelasyon olduğunu tespit ettik.

Tartışma:
Multiple skleroz hastalarında retina
sinir lifi tabakası kalınlığında azalma olmaktadır. Bu azalmada genişletilmiş
özürlülük durum ölçeğinin skoruyla korele şekilde gerçekleşmektedir. Optik koherens tomografi
ile retina sinir lifi tabakası ölçümü, klinik olarak ortaya çıkmamış nörodejenerasyonun tespitinde, tedavinin
izleminde ve takipte yardımcı bir yöntem
olabilir.
AİLEVİ AKDENİZ ATEŞİ TANISINA EŞLİK EDEN HASTALIKLAR: TEK MERKEZ DENEYİMİ
Authors: PAÇ KISAARSLAN Ayşenur , ÖZDEMİR ÇİÇEK Sümeyra , ŞAHİN Nihal , TAŞKIN Sema Nur , POYRAZOĞLU Hakan , GÜNDÜZ Zübeyde , DÜŞÜNSEL Ruhan 
Published: April 2020

Giriş:
Ailevi
Akdeniz Ateşi(AAA) tekrarlayan ateş, serozit, artrit atakları ile
seyreden, otoinflamatuar hastalıkların
en sık görülenidir. Klasik bulguların yanında giderek artan oranda ve
çeşitlilikte eşlik eden hastalıkla beraber seyretmektedir.

Amaç: Çalışmamızın amacı çocuk
romatoloji merkezimize başvuran AAA tanılı çocuk hastalardaki eşlik eden
hastalıkları incelemektir.

Gereç ve Yöntemler: Çalışmaya takipteki 586 AAA tanılı hastadan
eşlik eden hastalığı olan 107 hasta alındı. Hasta dosyalarından yaş, AAA tanı
yaşı, klinik özellikleri, eşlik eden hastalık tanı yaşları, başvuru klinik ve
laboratuvar özellikleri, tedavileri kaydedildi.

Bulgular:
Hastaların yaş median değeri 16(IQR: 11,5-18) yıl, AAA tanı yaşı 7(IQR: 4-10)
yıl olarak tespit edildi. Eşlik eden 1. hastalık tanı yaşı median 10(IQR:7-14),
2.sinin 11(IQR:7-15), 3.sünün 8(IQR:8-9) yıl idi. Hastalardan 96'sının (%89.71)
ekson 10 mutasyonu mevcuttu. Hastalarda IgA vasküliti 9(%1.53), Behçet
hastalığı 2(%0.34), poliarteritis nodosa(PAN) 1(%0.17), kutanöz PAN 2(%0.34),
ürtikeryal vaskülit 1( %0.17), oligoartiküler jüvenil idiyopatik artrit(JİA)
6(%1.02), poliartiküler JİA 3(% 0.51), EİA 37(%6.31), kronik nonenfeksiyöz
osteomyelit 7(%1.19), inflamatuar bağırsak hastalığı 3(%0.51), dirençli AAA 33(%5.63),
amiloidoz 4(%0.68), akut romatizmal ateş 3(%0.51), PFAPA 4(%0.86), çölyak
hastalığı 2(%0.34), psöriyasis 1(%0.17), morfea 1(%0.17), mukopolisakkaridoz
tip 6 1(%0.17) hastada tespit edildi.

Sonuç: AAA tanısı ile takip edilen hastalar sistemik sorgu ve
muayeneleri dikkatli şekilde yapılarak atak durumları, ilaç uyumları ve eşlik
eden hastalıklar açısından değerlendirilmelidir.
The pharmacoeconomic efficacy of chemotherapy for malignant neoplasms of the bronchus and lung
Authors: Yarovoy S. K. , Shikina I. B. 
Published: April 2020

Aim: to conduct a pharmacoeconomic analysis of chemotherapy taking into account the types of malignant neoplasms of the bronchi and lung, and to calculate pharmacoeconomic efficiency.Materials and methods. The materials for the study were the data of the Moscow Cancer Hospital n.a. D.D. Pletnev for 2019. The cost of drugs was calculated according to the data of the website aptekamos.ru (for June 2019).Results and discussion. For 300 patients with squamous non-small cell lung cancer, it is required 3205.5 courses of chemotherapy of I (1747.5) and II (1458) lines. For 200 patients with non-small cell non-squamous EGFR positive lung cancer, it is required 1,413.1 courses of chemotherapy and targeted therapy. For 70 patients with non-squamous cell non-small cell lung cancer with ALK mutation, it is required 450.2 courses of chemotherapy and targeted therapy. For 280 patients with non-squamous cell non-small cell lung cancer lung cancer without mutations, it is required 7515 chemotherapy courses. For 150 patients with small cell lung cancer, it is required 1,656.8 courses of chemotherapy. Thus, a total sample of 1000 patients, excluding the type of malignant neoplasm of broncus and lung are required 14,239.8 chemotherapy courses. The determined cost of 67,000 rubles for the average chemotherapy course per patient takes into account the necessary colony stimulating factors, antiemetic, infusion solutions, etc.; in the case of treatment with erlotinib (the length of the course is 8 months), the cost is 663,408.0 rubles; in the case of treatment with crizotinib (the length of the course is 11 months course), the cost is 2044110.0 rubles. The average cost of drug therapy for one patient with malignant neoplasm of bronchus and lung is 1136155.9 rubles.Conclusion. We determined the pharmacoeconomic dependence of a chemotherapy on the type of malignant neoplasms of bronchus and lung and the main anticancer drug used. The median of the overall survival rate of patients with malignant neoplasms of bronchus and lung was 42 months. The average cost of drug therapy was 27051.3 rubles per month. The median survival rate without therapy is 3 months. Thus, therapy can extend the patient’s life by an average of 39 months. The cost of a month of a saved life is 29132.2 rubles.
Pharmacoeconomic efficacy of atesolizumab compared with other PD-1 inhibitors in patients with advanced non-small cell lung cancer after chemotherapy
Authors: Zyryanov S. K. , Diyakov I. N. 
Published: April 2020

Aim: to evaluate the pharmacoeconomic efficacy of the application of the atesolizumab (PD-L1 inhibitor) preparation compared with other control point inhibitors (PD-1 inhibitors) in patients with advanced non-small cell lung cancer (NSCLC) after chemothe rapy.Materials and methods. Study design included a retrospective analysis of literature data and modeling. Based on the calculations conducted in a Microsoft Excel model, we analyzed the effect of minimizing costs on using comparable drugs with comparable efficacy; we evaluated how the provision of all patients with NSCLC will impact the health system budget taking into consideration the fact that all these patients are currently provided with PD-1 inhibitors in the second and third lines and with the atezolizumab preparation. For calculations, we used the prices stated in the state register of maximum selling prices; the weighted average maximum wholesale premium was calculated according to the Federal Antimonopoly Service (FAS). Results. In the analysis of cost minimization, atesolizumab proved itself to be highly clinically and economically effective. It allowed reducing the costs by 28.6% over 3 years compared with the use of nivolumab, and by 31.3% compared with the use of pembrolizumab in the second- and third-line NSCLC treatment regimen. Analysis of the impact on the budget showed that if all 848 patients currently receiving PD-1/PD-L1 inhibitors in the second- and third-line NSCLC treatment regimens had been initially provided with atesolizumab, this would have reduced the pressure on budget by 21.90% or 664.25 million rubles for 3 years.Conclusion. The use of the atesolizumab preparation is pharmacoeconomically reasonable and appropriate in comparison with the use of nivolumab and pembrolizumab. It will allow reducing the cost of PD-1/PD-L1 inhibitors in the second- and third-line NSCLC treatment regimens.
Real-time RT-PCR diagnostics of virus causing COVID-19
Authors: Goncharova E. V. , Donnikov A. E. , Kadochnikova V. V. , Morozova S. A. , Boldyreva M. N. , Galkina I. S. , Blinov D. V. 
Published: April 2020

Aim: the study was aimed to develop a reagent kit for the real-time RT-PCR diagnostics of virus causing COVID-19.Materials and Methods.Three target sites were chosen in the genome SARS-CoV-2. The testing included 220 samples, 48 artificially created positive samples (made from patients’ biomaterial) and 172 clinical samples (scrapes from nasal and pharyngeal cavities, bronchoalveolar lavage, expectoration, endotracheal/nasopharyngeal aspirate, feces, post-mortem material), obtained from two medical centers. Preliminary, the obtained biomaterial was analyzed with a reagent kit of comparison. The evaluation was performed with a confidential interval CI 95%. The calculation of CI for the sensitivity and specificity was made based on the distribution of χ2.Results.The authors developed a technology of novel coronavirus infection (COVID-19) real-time RT-PCR diagnostics for the application in practical healthcare and proposed the variants of testing at all the stages (preanalytical, analytical, and post-analytical, including automated results processing). The proposed reagent kit meets the requirements of the World Health Organization and the Ministry of Healthcare of the Russian Federation. The study results demonstrated high sensitivity and specificity. The sensitivity was 100% (95% CI) 95.6–100%; the specificity was 100% (95% CI) 96.7–100%.Conclusion.The proposed reagent kit was registered in the RF as a medical product; the registration certificate No. RZN 2020/9948 dated 01.04.2020. The application of the reagent kit in network laboratories will provide patients with access to testing for the virus causing COVID-19 and contribute to quick differential diagnostics, improvement of pandemic control, and accurate statistics on the spread of the virus.
Levels of vitamin D in tuberculosis and comparison of vitamin D level in severe variant of tuberculosis like tubercular meningitis, miliary tuberculosis, disseminated tuberculosis with non severe variant of tuberculosis like pulmonary tuberculosis, tubercular lymphadenitis etc in paediatric age group
Authors: Gupta Pankaj , Nainiwal Lalit 
Published: April 2020

Background: Tuberculosis is deadliest disease killing nearly 2 million people every year. Before the etiologic cause of TB was determined by Koch, cod liver oil and sunlight, both sources of vitamin D, were used in treatment of tuberculosis. After discovery of antibiotics, anti-infectious value of vitamin D was ignored until increasing cost of antibiotics and rise in resistance led to the need to search for alternative and antibiotic-independent therapeutic strategies. This study shed light on vitamin D, which is very safe and inexpensive by adding vitamin D to antibiotic treatment, immune system can be boosted to help body to clear TB, rather than relying on antibiotics.Methods: A prospective, observational, comparative study in which 62 TB patients were taken and vitamin D level were estimated.Results: The results of study show that out of total 62 patients, 31 (50.00%) had deficient(<20ng/ml) vitaminD,23(37.10%) had insufficient(20-30ng/ml) vitamin D, 8(12.90%) had sufficient (>30ng/ml) vitamin D level. Out of 62 patients, 10(62.13%) had severe variety of TB and 52(83.87%) had nonsevere TB. Among the 52 patients, 24(46.2%) had deficient vitamin D, 20(38.5%) had insufficient vitamin D and 8(15.4%) had sufficient vitamin D. Among the 10 patients with severe TB, 7(70.00%) had deficient vitamin D, 3(30.00%) had insufficient vitamin D and none had sufficient vitamin D level.Conclusions: Majority of children with tuberculosis demonstrated low serum levels of vitamin D (deficient and insufficient levels) suggest that vitamin D deficiency is risk factor of tuberculosis and very low levels of vitamin D were noted in severe variant of TB then non severe suggest that in severe form of tuberculosis vitamin D levels were less compared to nonsevere variant.
Bladder exstrophy with inguinal hernia: a case report
Authors: Ansari Tazeem Fatima , Gandhi Prachi , Wade Poonam , Singh Vinaya Lichade , Khedkar Kiran , Malik Sushma 
Published: April 2020

Exstrophy of urinary bladder with epispadias involves protrusion of the urinary bladder through a defect in the lower abdominal wall accompanied by separation of pubic symphysis. It is a rare but challenging condition that causes significant physical, functional, social, sexual and psychological problems later in life. Bladder exstrophy commonly involves males and most cases are sporadic. Inguinal hernia is a complication associated with bladder exstrophy and it occurs due to lack of obliquity of the inguinal canal secondary to pubic diastasis. Authors report here, a case of antenatally diagnosed case of classic bladder exstrophy associated with left sided inguinal hernia which was incidentally diagnosed on tenth day of life. Our neonate underwent primary bladder closure with herniotomy. Staged reconstruction of epispadias and bladder neck has been planned at a later date. Recurrence of inguinal hernia after repair is common and bilateral inguinal exploration while performing herniotomy is advised to prevent its recurrence. Prognosis of such cases depends on the degree of continence achieved. With timely reconstructive surgery, continence rates can be as high as 60-70 percent.