Laurence Moon Bardet Biedel syndrome: A case report

Laurence Moon Bardet Biedl syndrome is characterizedby obesity, retinitis pigmentosa, hypogonadism, mentalretardation and polydactyly. Additionally renal, cardiacand neurological manifestations may be seen. We reporthere a case with the syndrome that also had chronic renaldisease

Double-orifice mitral valve and an associated malformation: secundum atrial septal defect

The authors report a case of double-orifice mitral valve (DOMV) which showed mitral stenosis and mild insufficiency. An associated anomaly was secundum atrial septal defect. DOMV is an unusual congenital heart defect. The occurrence of this anomaly with or without secundum atrial septal defect is very rare. More often it is associated with other congenital malformations arising from atrioventricular canal defects. There may be no haemodynamic consequences but mitral insufficiency and/or stenosis may complicate this malformation. Treatment can be summarised as abstention, surgical repair or valve replacement

The Role of Somatostatin Treatment in the Management of Chylous Fistula after Neck Dissection

Chylous fistula is a serious complication of neck dissection. The use of somatostatin after neck surgery, has been described in literature. Because of the absence of definitive guidelines, reporting of our experience in three cases may guide the surgeons to overcome this life threatening complication. We descibe three cases who underwent neck dissection. While two of them had high output (>1000 mL/day), one had a low output (250 mL/ day) chyle leakage, after neck dissection. After it was noticed in earlier days, conservative management was started immediately. While Somatostatin was started after the re-exploration in two, it was started due to the failure of conservative treatment for low output leakage in one. All of the chyleous fistulas were healed within less than two weeks. We think that somatostatin may be considered as an effective treatment in low output chyle fistulas which the leakage continues after conservative treatment; however in high output chyle fistulas, somatostatin should be used in patients due to lack of control of leakage after ligation of thoracic ductus in revision Somatostatin treatment was discussed in the light of the current literature

Aort Koarktasyonu Tanısıyla Cerrahi Düzeltme Uygulanan Hastalarda Rekoarktasyon Gelişimi Üzerine Etki Eden Faktörler

Amaç: Çalışmamızda, aort koarktasyonu (AK) tanısıyla opere edilmiş hastalarda rekoarktasyon gelişimi üzerine etki eden faktörler araştırılmış ve elde edilen sonuçlar ışığında, rekoarktasyon oranlarının azaltılmasına katkıda bulunulması hedeflenmiştir. Gereç ve Yöntem: Bu retrospektif çalşmaya, Hacettepe Üniversitesi Tıp Fakültesi Kalp ve Damar Cerrahisi Kliniği’nde 1977-2010 arasında AK tanısıyla cerrahi düzeltme yapılan 358 hastadan, verilerine tam olarak ulaşılabilen ve düzenli olarak takiplerine gelmiş olan 217 hasta dahil edildi. Rekoarktasyon gelişen olgularda cerrahi düzeltme esnasındaki yaş, vücut ağırlığı ve kullanılan cerrahi tekniklerin sonuçları karşılaştırıldı. Veriler istatistiksel olarak değerlendirildi. Kategorik değişkenler için tanımlayıcı istatistikler sayı (n) ve yüzde (%) olarak belirtildi. Sonuçlar ortalama±standart sapma ve/veya yüzde (frekans) cinsinden sunuldu. Alfa anlamlılık değeri <0,05 olarak kabul edildi. Bulgular: Hastaların 147’si (%67,7) erkek, 70’i (%32,3) kadındı. Doksan (%41,4) hasta basit koarktasyon, 127 (%58,6) hasta ise koarktasyon ve eşlik eden kompleks intrakardiyak anomaliler tanıları ile opere edildi. Otuz altı hastada (%16,5) rekoarktasyon tespit edildi. Rekoarktasyon tespit edilen 36 hastadan 21’i (%58,3) balon anjiyoplasti, 10’u (%27,7) cerrahi, 5’i stent implantasyonu (%13,8) ile tedavi edildi. Sonuç: Çalışmamızda vücut ağırlığı <3 kg ve yaşı <1 yaş olan hasta grubunda rekoarktasyon gelişimi istatistiksel olarak anlamlı bulunmuştur. Kullanılan cerrahi teknikler arasındaysa istatistiksel bir anlam tespit edilmemiştir. AK nedeni ile opere edilecek hasta gruplarında vücut ağırlığı <3 kg ve yaşı <1 yaş olan hasta gruplarının post operatif takiplerinde rekoarktasyon gelişimi açısından daha dikkatli olunması gerekmektedir

Assessment of electromechanically stimulated bone marrow stem cells seeded acellular cardiac patch in a rat myocardial infarct model

In this study, we evaluated cardiomyogenic differentiation of electromechanically stimulated rat bone marrow-derived stem cells (rt-BMSCs) on an acellular bovine pericardium (aBP) and we looked at the functioning of this engineered patch in a rat myocardial infarct (MI) model. aBP was prepared using a detergent-based decellularization procedure followed by rt-BMSCs seeding, and electrical, mechanical, or electromechanical stimulations (3 millisecond pulses of 5 V cm-1at 1 Hz, 5% stretching) to enhance cardiomyogenic differentiation. Furthermore, the electromechanically stimulated patch was applied to the MI region over 3 weeks. After this period, the retrieved patch and infarct region were evaluated for the presence of calcification, inflammatory reaction (CD68), patch to host tissue cell migration, and structural sarcomere protein expressions. In conjunction with any sign of calcification, a higher number of BrdU-labelled cells, and a low level of CD68 positive cells were observed in the infarct region under electromechanically stimulated conditions compared with static conditions. More importantly, MHC, SAC, Troponin T, and N-cad positive cells were observed in both infarct region, and retrieved engineered patch after 3 weeks. In a clear alignment with other results, our developed acellular patch promoted the expression of cardiomyogenic differentiation factors under electromechanical stimulation. Our engineered patch showed a successful integration with the host tissue followed by the cell migration to the infarct region

Robust video streaming over time-varying wireless networks

Multimedia services and applications became the driving force in the development and widespread deployment of wireless broadband access technologies and high speed local area networks. Mobile phone service providers are offering wide range of multimedia applications over high speed wireless data networks. People can watch live TV, stream on-demand video clips and place videotelephony calls using multimedia capable mobile devices. Mobile devices will soon support capturing and displaying high definition video. Similar evolution is also occurring in the local area domain. The video receiver or storage devices were conventionally connected to display devices using cables. By using wireless local area networking (WLAN) technologies, convenient and cable-free connectivity can be achieved. Media over wireless home networks prevents the cable mess and provides mobility to portable TVs. However, there still exit challenges for improving the quality-of-service (QoS) of multimedia applications. Conventional service architectures, network structures and protocols lack to provide a robust distribution medium since most of them are not designed considering the high data rate and real-time transmission requirements of digital video. In this thesis the challenges of wireless video streaming are addressed in two main categories. Streaming protocol level issues constitute the first category. We will refer to the collection of network protocols that enable transmitting digital compressed video from a source to a receiver as the streaming protocol. The objective of streaming protocol solutions is the high quality video transfer between two networked devices. Novel application-layer video bit-rate adaptation methods are designed for handling short- and long-term bandwidth variations of the wireless local area network (WLAN) links. Both transrating and scalable video coding techniques are used to generate video bit-rate flexibility. Another contribution of this thesis study is an error control method that dynamically adjusts the forward error correction (FEC) rate based on channel bit-error rate (BER) estimation and video coding structure. The second category is the streaming service level issues, which generally surface in large scale systems. Service system solutions target to achieve system scalability and provide low cost / high quality service to consumers. Peer-to-peer assisted video streaming technologies are developed to reduce the load of video servers. Novel video file segment caching strategies are proposed for more efficient peer-to-peer collaboration.Ph.D.Committee Chair: Yucel Altunbasak; Committee Member: Chuanyi Ji; Committee Member: Ghassan AlRegib; Committee Member: Ozlem Ergun; Committee Member: Russell M. Merserea

Neurocognitive Effects in Peritoneal Dialysis Patients

Many of the children with chronic kidney disease (CKD) are frequently affected with neurological and cognitive disorders that increase the morbidity and mortality of the disease and decrease the quality of life. İrrespective of the cause, injury can ocur at all levels of the nervous system including central nervous system and disorders like cerebrovascular disease, cognitive dysfunction, and uremic encephalopathy can develop together with the peripheral neuropathies and myopathies. Although some of these disorders improve after the initiation of renal replacement therapy (RRT), other neurological complications like dialysis disequilibrium syndrome can ocur due to RRT. These predialysis and post dialysis complications and the risk factors should be known well for early recognition and adequate therapy leading to decrease in the morbidity and mortality of the disease and increase the life quality of patients.Kronik böbrek hastalıklı (KBH) çocuklarda pek çok nörolojik ve bilişsel bozukluk gelişebilmektedir ve bu bozukluklar hastalığın mortalite ve morbiditesini artırmakta ve hastaların yaşam kalitesini düşürmektedir. Etiyolojik neden ne olursa olsun sinir sisteminin her seviyesinde hasar meydana gelebilir. Serebrovasküler hastalık, bilişsel bozukluk, üremik ensefalopati gibi santral sinir sistemi (SSS) bozukluklarının yanı sıra periferal nöropati ve miyopati gibi pek çok nörolojik bulgu gelişebilir. Renal replasman tedavisi ile bu bulguların ancak bir kısmı düzelirken tedaviye bağlı olarak diyaliz disekilibrium sendromu (DES) gibi başka nörolojik bulgular da ortaya çıkabilmektedir. Diyaliz öncesi ve sonrası gelişebilen bu komplikasyonların ve risk faktörlerinin iyi bilinmesi, erken tanı ve tedaviyi sağlamakta ve uzun süreli takipte mortalite ve morbiditeyi azaltarak yaşam kalitesini artırmaktadır

Familial Mediterranean Fever

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder characterized by recurrent attacks of fever and serosal inflammation. It occurs most frequently in the East Mediterranean region. It is generally an autosomal recessive disease and develops due to the mutations in the MEFV gene. It has self-limiting attacks with abrupt onset lasting for 12-72 hours. The main symptom is fever and usually accompanied by abdominal pain, joint manifestations, chest pain and erysipelas-like skin rash. The diagnosis relies on mainly clinical findings and molecular analysis of MEFV gene provides genetic confirmation. The most important complication of the disease is amyloidosis type AA that is the main cause of mortality and morbidity though its frequency decreased due to the therapeutical advances. The main drug of treatment in FMF is colchicine. Anti IL-1 treatment was shown to be effective in a few patients that are resistant to colchicine. The prognosis of FMF is very good in compliant patients providing a high quality of life.Ailesel Akdeniz ateşi (AAA) tekrarlayan ateş ve serözit ataklarıyla karakterize herediter, otoinflamatuar bir hastalıktır. Daha çok doğu Akdeniz kökenli toplumlarda görülen AAA genellikle otozomal resessif kalıtılmakta ve MEFV (MEditerranean FeVer) genindeki mutasyonlar sonucu gelişmektedir. Ataklar genellikle ani başlar, 12-72 saat sürer ve kendiliğinden geçer. Ateş hastalığın en önemli bulgusudur ve beraberinde sıklıkla karın ağrısı, eklem bulguları, göğüs ağrısı ve erizipel benzeri döküntüler görülebilir. Tanı esas olarak klinik bulgularla konulur ve MEFV geninin moleküler analizi ile genetik olarak doğrulanır. En ciddi komplikasyonu önemli bir mortalite ve morbidite nedeni olan tip AA amiloidoz olup tedavideki geişmeler nedeniyle görülme sıklığı çok azalmıştır. Tedavide ilk başlanacak en etkin ilaç kolşisindir. Kolşisine dirençli çok az sayıdaki hastada ise anti IL-1 ilaçların fayda sağladığı gösterilmiştir. Tedaviye uyumlu hastalarda prognoz çok iyidir ve genellikle yaşam kalitesi yüksek, normal bir yaşam sürerler