The Abbé island flap for the reconstruction of severe secondary cleft lip deformities

Primary repair of the cleft lip is often associated with secondary deformities, which require revision and secondary reconstruction. Patients with one or all of the following, a tight lip restricting use of orthodontic appliances, absence of a Cupid's bow, or absence of vermilion tubercle, were treated with the Abbé island flap. A triangular muco-musculo-cutaneous island flap was designed in the central segment of the lower lip. A full-thickness incision of skin, muscle, and mucosa was made in the midline of the upper lip, dividing the lip into two segments, and the island Abbé flap was inserted. One week after surgery, the pedicle of the island flap was divided and the inset of the flap completed. Sixty patients with severe secondary cleft lip deformities (36 males and 24 females) were treated. Good aesthetic and functional results were achieved during 1 to 17 years of follow-up. Insertion of the Abbé flap resulted in release of the tight upper lip and a new, inconspicuous scar. Use of orthodontic appliances was facilitated, and tightness of the tissue, which restricted the expansion of the alveolar process, was eliminated. Adequate tissue was transferred to the upper lip, which improved the bulk of the lip and vermilion tubercle. The retrusion of the midface and the projection of the upper lip were also improved, and the upper and lower lips became better balanced. Donor site morbidities were insignificant. Use of the Abbé flap in selected patients resulted in successful reconstruction of secondary cleft lip deformities.     

Multiscale analysis of SRY-positive 46,XX testicular disorder of sex development: Presentation of nine cases

46,XX testicular disorder of sex development (46,XX TDSD) is a relatively rare condition characterised by the presence of testicular tissue with 46,XX karyotype. The present study aims to reveal the phenotype to genotype correlation in a series of sex-determining region Y (SRY)-positive 46,XX TDSD cases. We present the clinical findings, hormone profiles and genetic test results of six patients with SRY-positive 46,XX TDSD and give the details and follow-up findings of our three of previously published patients. All patients presented common characteristics such as azoospermia, hypergonadotropic hypogonadism and an SRY gene translocated on the terminal part of the short arm of one of the X chromosomes. Mean ± standard deviation (SD) height of the patients was 164.78 ± 8.0 cm. Five patients had decreased secondary sexual characteristics, and three patients had gynaecomastia with varying degrees. Five of the seven patients revealed a translocation between protein kinase X (PRKX) and inverted protein kinase Y (PRKY) genes, and the remaining two patients showed a translocation between the pseudoautosomal region 1 (PAR1) of X chromosome and the differential region of Y chromosome. X chromosome inactivation (XCI) analysis results demonstrated random and skewed XCI in 5 cases and 1 case, respectively. In brief, we delineate the phenotypic spectrum of patients with SRY-positive 46,XX TDSD and the underlying mechanisms of Xp;Yp translocations.     

Effects of quercetin and fish n‐3 fatty acids on testicular injury induced by ethanol in rats

The aim of this study was to investigate the effects of quercetin and fish n‐3 fatty acids on the changes in testis induced by ethanol. Forty‐five rats divided into five groups, control, ethanol, ethanol+quercetin, ethanol+fish n‐3 fatty acids and ethanol+quercetin+fish n‐3 fatty acids. At the end of 8 weeks, all the rats were sacrificed. Degenerative changes in histopathological analyses, the decreased body weight gain and seminiferous tubule diameters in ethanol group have been observed. TUNEL assay also showed an increase in apoptotic cell number. The activities of superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GSH‐Px), xanthine oxidase (XO) and testosterone levels were decreased as well as the levels of malondialdehyde (MDA) and nitric oxide (NO) were increased in ethanol group. Histopathological changes caused by ethanol have been improved by quercetin and fish n‐3 fatty acids. It was also found that protection was provided by increasing SOD, CAT and GSH‐Px activities in groups administered quercetin, fish n‐3 fatty acids and quercetin+fish n‐3 fatty acids, and by decreasing the levels of MDA and NO in groups administered both quercetin and fish n‐3 fatty acids together. These results suggest that quercetin and fish n‐3 fatty acids are beneficial agents to reduce testicular injury induced by ethanol except for testosterone levels.     

Joint instability and cartilage compression in a mouse model of posttraumatic osteoarthritis

Joint instability and cartilage trauma have been previously studied and identified as key mediators in the development of posttraumatic osteoarthritis (PTOA). The purpose of this study was to use an in vivo model to compare the effect of joint instability, caused by the rupture of the anterior cruciate ligament (ACL), versus cartilage compression. In this study, mice were subjected to cyclical axial loads of twelve Newtons (N) for 240 cycles or until the ACL ruptured. One and eight weeks after this procedure, knees were sectioned coronally and evaluated for osteoarthritis by histology. Using a scoring scale established by [Pritzker K, Gay S, Jimenez S, et al. (2006): Osteoarthritis Cartilage 14:13–29], the articular cartilage across each surface was scored and combined to produce a total degeneration score. The ACL‐ruptured group had a significantly greater total degeneration score than either control or compression treated joints at 1 and 8 weeks. Additionally, only sections from ACL‐ruptured knees consistently showed synovitis after 1 week and osteophyte formation after 8 weeks. Thus, it appears using that ACL rupture consistently creates a severe osteoarthritis phenotype, while axial cartilage compression alone does not appear to be an appropriate method of inducing PTOA in vivo. © 2013 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 32:318–323, 2014.     

New use of teflon to reduce bleeding in modified bentall operation

We analyzed the postoperative short- and mid-term outcomes of a series of patients with annuloaortic ectasia who underwent a modified Bentall operation in our clinic from September 2000 through March 2006. The study included 44 patients. Their average age was 53.4 +/- 14.1 years. The underlying disease was degenerative aortic aneurysm in 42 patients (95.5%) and acute aortic dissection in 2 patients (4.5%). Six patients (13.6%) had Marfan phenotype. Aortic insufficiency was moderate in 30 patients (68.2%) and severe in 14 patients (31.8%). In our modification of the Bentall technique, we completed the resection of the aortic root while leaving 5 to 10 mm of native aortic wall tissue to support the anastomosis. A long piece of Teflon felt (width, 0.5-1 cm) was laid on the annulus, and nonpledgeted 2-0 polyester sutures were passed in turn through the Teflon felt, the preserved aortic tissue, and the aortic annulus. A thin piece of Teflon felt was also used in the coronary artery reimplantation sites. Fibrin glue was routinely applied to all anastomoses. There were no intraoperative deaths. One patient died in the hospital after surgery for acute type I aortic dissection. Another patient died 1 year after the operation from prosthetic-valve endocarditis. No patient required surgical correction of excessive postoperative bleeding. Kaplan-Meier curves showed overall survival of 0.94 (95% confidence intervals, 0.9-0.99). We consider our approach an easy, effective way to minimize bleeding from the anastomoses and at the aortic root--a common challenge in aortic surgery.     

Hypovitaminosis D in obese and overweight schoolchildren

Aim: To determine the prevalence of vitamin D hypovitaminosis among obese and overweight schoolchildren.Design: A cross−sectional population based sample.Methods: In a cross−sectional study, 301 students (177 girls and 124 boys) aged 11−19 years were selected by multistage stratified sampling design. Subjects were classified according to their body mass index as obese, overweight and normal. Serum 25−hydroxyvitamin D (25−OHD), intact parathyroid hormone (iPTH) and alkaline phosphatase (ALP) were measured in late winter months. Vitamin D deficiency was defined as a 25−OHD 20 ng/ml.Results: The prevalence of hypovitaminosis D was found as 65% in all students. Vitamin D deficiency was found in 12% and insufficiency in 53% of all students. Vitamin D deficiency in female students was about two times more common than in males. In obese and overweight schoolchildren with hypovitaminosis D, serum 25−OHD levels decreased as BMI increased. There were no correlations between serum 25−OHD and ALP and iPTH levels.Conclusion: Vitamin D deficiency and insufficiency are common in obese and overweight schoolchildren, especially in girls. Obesity could be a risk factor in terms of hypovitaminosis D in adolescents. Vitamin D supplementation should be administered particularly to adolescent girls.Conflict of interest:None declared.     

Imaging Frontotemporal Lobar Degeneration

The term frontotemporal lobar degeneration (FTLD) refers to a group of neurodegenerative disorders that target the frontal and temporal lobes. It accounts for approximately 10 % of pathologically confirmed dementias but has been demonstrated to be as prevalent as Alzheimer’s disease in patients below the age of 65. The 3 major clinical syndromes associated with FTLD include behavioral variant frontotemporal dementia, semantic and nonfluent variants of primary progressive aphasia. The more recently introduced term logopenic variant appears to represent an atypical form of Alzheimer’s disease in the majority of cases. The neuropathology underlying these clinical syndromes is very heterogeneous and does not correlate well with the clinical phenotype. This causes great difficulties in early and reliable diagnosis and treatment of FTLD. However, significant advances have been made in recent years via the application of magnetic resonance imaging and positron emission tomography imaging methods as biomarkers. The current review aims to provide a synopsis on the value of magnetic resonance imaging-based and molecular imaging procedures in FTLD.