Reliability of Measurements on Plaster and Digital Models of Patients with a Cleft Lip and Palate

Objective:The purpose of this study was to determine (1) the more and less reliable measurements/methods and (2) the influence of knowledge and skill on the inter- examiner, intra-examiner, and inter-method reliability of nasolabial measurements on plaster casts and three dimensional (3D) stereophotogrammetric images of casts in infants with an unrepaired unilateral cleft lip and palate (UUCLP).Methods:Preoperative extraoral plaster casts from 42 patients with UUCLP were measured with a digital caliper, and the image acquisition of casts was performed with the 3dMDface stereophotogrammetry system (3dMD, Atlanta, GA). Two examiners (one postgraduate student, one lecturer) evaluated 19 nasolabial measurements in two separate sessions.Results:Intra-rater, inter-rater, and inter-method reliability was lower in measurements of nasal, philtral, and nasal floor width. Almost all of the interclass correlation coefficients (ICC) for measurements performed by the lecturer were above 0.75, whereas the intra-examiner reliability of some measurements performed by the postgraduate student showed low ICC (<0.75).Conclusion:Measurements of curving slopes, such as nasal width, of small dimensions, such as nostril floor width, and deformity-affected anatomic parts, such as philtrum width, presented a low reliability. Measurements on 3D images showed a higher reliability compared to plaster model measurements performed by the postgraduate student. Therefore, it may be recommended to use 3D digital images of infants with CLP for nasolabial measurements especially if performed in postgraduate settings

Türkiye Romatizma Araştırma ve Savaş Derneği ankilozan spondilit ulusal tedavi önerileri

Objectives: To develop Turkish League Against Rheumatism (TLAR) National Recommendations for the management of ankylosing spondylitis (AS). Materials and methods: A scientific committee of 25 experts consisting of six rheumatologists and 19 physical medicine and rehabilitation specialists was formed by TLAR. Recommendations were based on the 2006 ASsessment in Ankylosing Spondylitis International Working Group(ASAS)/European League Against Rheumatism (EULAR) recommendations and a systematic review of associated publications between January 2005 and September 2010. A Delphi process was used to develop the recommendations. Twelve major recommendations were constructed for the management of AS. Voting using a numerical rating scale assessed the strength of each recommendation. Results: The 12 recommendations include patient assessment, patient follow-up along with pharmacological and non-pharmacological methods. Some minor additions and changes have been made to the ASAS/EULAR recommendations. All of the recommendations had sufficient strength. Conclusion: National recommendations for the management of AS were developed based on scientific evidence and consensus expert opinion. These recommendations will be updated regularly in accordance with recent developments.Türkiye Romatizma Araştırma ve Savaş Derneği᾽nin (TRASD) Ankilozan Spondilit (AS) için ulusal tedavi önerilerinin oluşturulmasıdır. Gereç ve yöntemler: TRASD tarafından altı Romatoloji ve 19 Fiziksel Tıp ve Rehabilitasyon uzmanı olmak üzere toplam 25 kişiden oluşan bir bilimsel kurul oluşturuldu. Önerilerde 2006 yılında yayınlanan Ankilozan Spondilit Değerlendirme Uluslararası Çalışma Grubu (ASAS)/Romatizmaya karşı Avrupa Ligi (EULAR) önerileri ve Ocak 2005 - Eylül 2010 arasında yayınlanmış olan ilişkili yayınlar konusundaki sistematik bir inceleme temel alındı. Öneriler oluşturulurken Delphi süreci kullanıldı. Ankilozan spondilit tedavisi ile ilgili 12 ana öneri oluşturuldu. Oylama yapılarak önerilerin güçlülük düzeyi bir nümerik derecelendirme skalası ile belirlendi. Bulgular: On iki öneri hasta değerlendirilmesini, hasta takibini ve farmakolojik ve non-farmakolojik yöntemleri içermektedir. ASAS/EULAR önerilerine bazı ilaveler ve önerilerde bazı küçük değişiklikler yapılmıştır. Tüm öneriler yeterli kuvvete sahipti. Sonuç: Bilimsel kanıtlar ve uzmanların görüş birliği ile AS tedavisine yönelik ulusal öneriler oluşturulmuştur. Bu öneriler, yeni gelişmeler doğrultusunda düzenli olarak güncellenmelidi

The frequency of Duchenne muscular dystrophy/Becker muscular dystrophy and Pompe disease in children with isolated transaminase elevation: results from the observational VICTORIA study

IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia.MethodsThis multi-center, prospective study enrolled patients aged 3–216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels &gt;2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients were analyzed.ResultsOverall, 589 patients [66.8% male, mean age of 63.4 months (standard deviation: 60.5)] were included. In total, 251 patients (188 male and 63 female) had CPK levels above the ULN. Of the patients assessed, 47% (85/182) of male patients were diagnosed with DMD/BMD and 1% (3/228) of male and female patients were diagnosed with PD. The median ALT, AST, and CPK levels were statistically significantly higher, and the questioned neurological symptoms and previously unnoticed examination findings were more common in DMD/BMD patients than those without DMD/BMD or PD (p &lt; 0.001).DiscussionQuestioning neurological symptoms, conducting a complete physical examination, and testing for CPK levels in patients with isolated hypertransaminasemia will prevent costly and time-consuming investigations for liver diseases and will lead to the diagnosis of occult neuromuscular diseases. Trial RegistrationClinicaltrials.gov NCT04120168

Liver Involvement in Children during the Diagnosis of Celiac Disease: A Single-Center Experience from Turkey

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