Mukopolisakkaridoz hastalarındaki otolarengolojik bulgular

Bu derleme makalede MPS hastalarındaki otolarengolojik sorunları raporladık. Mukopolisakkaridozlar (MPS'ler) her biri mukopolisakkaritlerin parçalanmasında rol alan ve şimdilerde glikozaminoglikanlar denilen (GAG'ler) kalıtsal bir enzim eksikliği nedeniyle oluşan bir lizozom depo hastalıkları grubudur. Mukopolisakkaridozlar, mukopolisakkaridoz tip 1-VII diye bilinen 7 metabolizma hastalığı grubunu oluştururlar. Grupların tümünde klinik ve otolaringolojik belirtiler görülür. Mukopolisakkaridoz hastalarında sık görülen otolarengolojik bulgular olarak üst hava yolu obstrüksiyonu, obstrüktif uyku apnesi, ağız açmanın kısıtlanması, orta kulak efüzyonu, işitme ve soluma sorunları vb. bildirilmektedir. KBB uzmanları arasında MPS'ler konusunda farkındalığın artırılması çocuk doktorundan ziyade bir KBB uzmanına giden MPS'den kuşkulanılan hastalar için yaşam kurtarıcı bir çaba olacaktır. Mukopolisakkaridoz hastalarında kısa boyun nedeniyle trakeotomi yapmak zorlaşabilir. Ağız açmanın kısıtlanması nedeniyle tonsillektomi ve adsenoidektomi ameliyatlarından önce hastalar dikkatle değerlendirilmelidir. Anesteziden önce hava yolu sorunları değerlendirilmelidir. Tüm KBB uzmanlarının bu sorunların farkınnda olmaları konusunda dikkati çekilmelidir.In this review paper, we reported otolaryngological problems in patients with mucopolysaccharidoses (MPSs). Mucopolysaccharidoses are a group of lysosomal storage diseases, each of which is produced by an inherited deficiency of an enzyme involved in the degradation of acid mucopolysaccharides, now called glycosaminoglycans (GAGs). The mucopolysaccharidoses consist of a group of 7 metabolic disorders, known as mucopolysaccharidoses types I-VII. In all groups, there are clinical and otolaryngological manifestations. In MPS patients, upper airway obstruction, obstructive sleep apnea, restriction of mouth opening, middle ear effusion, hearing and breathing problems, etc. are reported as common otolaryngological findings. Increasing awareness of MPS's among ENT doctors will be a life saving attempt for MPS suspected patients who admit an ENT doctor rather than a pediatrician. In MPS patients, tracheotomy may be difficult due to short neck. Due to mouth opening restriction, patients should be evaluated carefully before tonsillectomy and adenoidectomy operations. Airway problems must be evaluated before anesthesia. All ENT doctors should be noticed to be aware of these problems

Does Maxillary Protraction with Alt-RAMEC Protocol Affect Inferior Sclera Exposure? A Controlled 3dMD Study

Objective: The purpose of this controlled retrospective study was to measure and evaluate the inferior sclera exposure changes using 3dMD stereophotogrammetric images in a prepubertal Class III patient sample that underwent maxillary protraction with alternate rapid maxillary expansions and constrictions protocol followed by facemask.Methods: The study group included 15 prepubertal patients (mean age: 9.85 ± 1.44 years) with Class III malocclusion due to maxillary retrognathism. Nine weeks of alternate rapid maxillary expansions and constrictions protocol was followed prior to 7 months of face mask treatment and 3 months of retention with Bionator. Pretreatment (T0) and post-retention (T1) lateral cephalometric radiographs and 3dMD images were retrieved from clinical archive. The same records were used for a control group of 15 well-matched, untreated patients (mean age: 9.4 ± 0.79 years). The distance between the upper eyelid margin and the lower eyelid margin was recorded as the overall eye height (E), and the distance between inferior limbus and the lower eyelid margin was recorded as inferior sclera exposure (S). The S : E ratio in percentage was calculated. Sella-nasion-A point angle (SNA) was used as the skeletal variable.Results: SNA angle, right S : E, and left S : E changed significantly in both groups at T1-T0. The intergroup comparison was highly significant for SNA angle but was not significant for right and left S : E variables.Conclusion: The S : E ratio decreased significantly in both alternate rapid maxillary expansions and constrictions/facemask and the control groups. However, the change in S : E ratio between groups was not significant

The Effect of Phospholipids (Surfactant) on Adhesion and Biomechanical Properties of Tendon: A Rat Achilles Tendon Repair Model

Adhesion of the tendon is a major challenge for the orthopedic surgeon during tendon repair. Manipulation of biological environment is one of the concepts to prevent adhesion. Lots of biochemicals have been studied for this purpose. We aimed to determine the effect of phospholipids on adhesion and biomechanical properties of tendon in an animal tendon repair model. Seventy-two Wistar rats were divided into 4 groups. Achilles tendons of rats were cut and repaired. Phospholipids were applied at two different dosages. Tendon adhesion was determined histopathologically and biomechanical test was performed. At macroscopic evaluation of adhesion, there are statistically significant differences between multiple-dose phospholipid injection group and Control group and also hyaluronic acid group and Control group ( < 0.008). At microscopic evaluation of adhesion, there was no statistically significant difference ( > 0.008). Ultimate strength was highest at hyaluronic acid injection group and lowest at multiple-dose phospholipid injection group. Single-dose phospholipids (surfactant) application may have a beneficial effect on the tendon adhesion. Although multiple applications of phospholipids seem the most effective regime to reduce the tendon adhesion among groups, it deteriorated the biomechanical properties of tendon

Zihin Engelliler Öğretmen Adaylarının Öğretmenlik Uygulaması Dersine ve Uygulama Okullarına İlişkin Beklentilerinin Belirlenmesi

Bu çalışmanın amacı, özel eğitim öğretmen adaylarının Öğretmenlik Uygulaması dersine ve dersin yürütüldüğü uygulama okullarına ilişkin beklentilerinin belirlenmesidir. Çalışmada durum çalışması deseni kullanılmıştır. Çalışmaya, zihin engeliler öğretmenliği programına devam eden ve Öğretmenlik Uygulaması I dersini alan 75 özel eğitim öğretmen adayı katılmıştır. Veriler, açık-uçlu sorulardan oluşmuş, yarı-yapılandırılmış bir görüşme formu kullanılarak toplanmıştır. Verilerin analizinde içerik analiz kullanılmıştır. Değerlendiriciler arası güvenirlik ortalama %97 bulunmuştur. Bulgular, öğretmen adaylarının Öğretmenlik Uygulaması dersiyle ilgili beklentilerinin, dersin amacı, içeriği, işlenişi ve değerlendirmesiyle tutarlı olduğunu ortaya koymaktadır. Öğretmen adaylarının dersin öğretim elemanından hem mesleki hem de kişisel beklentileri olduğu görülmektedir. Öğretmen adayları ayrıca, dersin yürütüldüğü uygulama okullarına ilişkin hem fiziksel hem de sosyal beklentilere; uygulama okulundaki personele ilişkin ise hem mesleki hem de kişisel beklentilere sahiptir. Çalışmanın bulguları, uygulamalara ve ileriki araştırmalara ilişkin öneriler açısından tartışılmıştır

Behçet disease: evaluation of clinical manifestations in Turkish children

PubMe

Habits of using social media and the internet in psoriasis patients

Introduction: Psoriasis significantly affects the patients quality of life, which often leads patients to seek online information about this disease. Objectives: To explore the habits of patients with psoriasis related to their use of social media (SM) and the internet to obtain information about their disease. Methods: 1,520 patients completed the survey and the Dermatology Life Quality Index (DLQI) questionnaire. The Psoriasis Area Severity Index scores (PASI) and clinical data of the patients were recorded by their physicians. Results: Of the 1,114 patients that reported using SM and internet, 48.38% regularly and 31.14% sometimes resorted to obtain information about psoriasis. The use of SM and internet for psoriasis was statistically significantly higher among young people (P = 0.000), those with university or higher education (P = 0.009), higher DLQI (P = 0.000) and PASI (P = 0.011) scores, facial (P = 0.050), scalp (P = 0.032), hand (P = 0.048), genital (P = 0.001) and inverse (P = 0.000) involvement, and arthralgia/arthritis (P = 0.006). The participants mostly used the Google (86%) and Facebook (41%). More than half of the participants (62.8%) expected dermatologists to inform society that psoriasis is not contagious. Conclusions: Internet and SM being widely available and offering substantial information to be easily accessed make it very attractive for patients to use these platforms to investigate diseases, including psoriasis. If what is presented on SM conflicts with what the physician says, patients mostly trust the latter, but at the same time, they tend not to share the results of their online inquiries with their physicians

Shear Strength Between Adhesive Cement and Yttria-Stabilized-Zirconia and Cobalt-Chrome Alloy With and Without Retentive Holes - an in vitro study

ABSTRACT De-bonding is the most common failure of resin-bonded fixed partial dentures. The aim was to determine if the shear bond strength (SBS) differed between a dental adhesive and a Co-Cr alloy and an yttria-stabilized zirconia (Y-TZP). Furthermore, to determine whether retention holes in the two materials and storage for 21 days in water affected the results. The hypothesis was that there are no significant differences between the compared groups. 10 embedded discs of Co-Cr alloy without and 10 with retentive holes, and 10 discs of Y-TZP without and 17 with retentive holes were sand- and steam blasted. A pillar of adhesive cement was bonded to the surface of the discs and stored in water at 37 °C for 24 hr and / or 21 days. An UltraTest machine with a crosshead speed of 1 mm/ min was used for SBS. The mean SBS after 24 hr / 21 days in water were recorded. The SBS of Co-Cr alloy without retentive hole was 16.3 MPa after 24 hr and 11.4 MPa after 21 days, the SBS of Y-TZP without retentive hole was 18.5 MPa after 24 hr and 12.6 MPa after 21 days. The SBS of Co-Cr alloy with retentive hole 13.9 MPa after 21 days and Y-TZP with retentive hole 16.9 MPa after 21 days. No statistically significant differences were found between the groups with or without retentive holes and after 24 hr or 21 days in water, p> 0.05

Cell cycle inhibitor p57 expression in normal and diabetic rat placentas during some stages of pregnancy

Placentomegaly, an abnormal increase in the size of the placenta, is commonly seen in human diabetic pregnancies and diabetic animal experimental models. Proper placental development depends on the proliferation and differentiation of trophoblasts. However, our knowledge about the mitotic regulators that play key roles in synchronizing these events is limited. p57 is a cyclin-dependent kinase (CDK) inhibitor acting in the G1/S transition of the cell cycle. There is no data regarding p57 expression in either rat or human diabetic placentas. The purpose of this study was to investigate p57 expression in control and diabetic rat placentas at different stages of pregnancy. Diabetes was induced by streptozotocin on the first day of pregnancy, and placentas were taken on days 11, 13, 17, and 21 of pregnancy. Our results showed that on day 11, p57 immunostaining intensity was stronger in control group placentas compared to the diabetic group. On day 13, p57 immunostaining intensity increased in both groups, but increased more in the diabetic group. On day 17, p57 immunostaining intensity decreased in both the control and diabetic groups compared to day 13, yet the intensity remained higher in control placentas compared to diabetic placentas. On day 21 of pregnancy, p57 immunostaining intensity increased in the control group and it decreased from the day 17 level in the diabetic group. Western blot results showed consistency with immunohistochemistry results. Our study shows different expression patterns of p57 between control and diabetic rat placentas, which indicate p57 may play a role in abnormal placental formation resulting in placentomegaly arising from diabete