Hyaline fibromatosis syndrome (HFS) is a rare autosomal recessive genetic disorder characterized by accumulation of hyalinized fibrous tissue with cutaneous, mucosal, osteoarticular, and systemic ...involvement. The condition is caused by a mutation of ANTXR2 gene that results in a faulty synthesis of a transmembrane protein which leads up to excessive deposition of hyaline material in extracellular space. The first signs may be present at birth or appear during infancy, and joint stiffness is the first, most common, symptom. Other manifestations include joint contractures, hyperpigmented macules over bony prominences of the joints, and gingival hypertrophy. The symptom that raises suspicion of HFS is present later, along with subcutaneous growths. The progression of the disease includes enteropathy with extensive protein loss, chronic diarrhea and frequent infections. We present a case of a five-year-old girl with severe gingival hypertrophy that caused difficulties in eating and speaking. To the best of our knowledge, this is also the first patient in Croatia with a confirmed ANTXR2 gene mutation described in the literature. Key words Systemic Hyalinosis; Gingival Hypertrophy; Gingivectomy; Extracellular Matrix; ANTXR2 gene Hijalini fibromatozni sindrom (HFS) rijedak je autosomno recesivni genetski poremecaj koji karakterizira nakupljanje hijaline tvari u tkivima s koznim, sluznickim, kostanim, zglobnim i sistemskim manifestacijama. Bolest je uzrokovana mutacijom gena ANTXR2 koja rezultira sintezom neispravnog transmembranskog proteina, pa se hijalini depoziti prekomjerno taloze u medustanicnom pro-storu. Prvi znakovi mogu biti prisutni pri rodenju ili tijekom dojenacke dobi, a prvi simptom najcesce je ukocenostzgloba. Ostale manifestacije ukljucuju zglobne kontrakture, hiperpigmentirane makule koze iznad kostanih prominecija zglobova i hipertrofiju gingive, sto je uz potkozne nodule (kvrzice), vrlo cesto simptom koji potice sumnju na HFS. Napredovanje bolesti ukljucuje enteropatiju s velikim gubitkom proteina, kronicnu dijareju i ceste infekcije. U radu predstavljamo petogodisnju djevojcicu s teskim oblikom gingivne hipertrofije koja je uzrokovala poteskoce s hranjenjem i govorom. Pre-ma nasim spoznajama to je prvi pacijent opisan u Hrvatskoj s dokazanom mutacijom gena ANTXR2. Kljucne rijeci sistemska hijalinoza; hipertrofija gingive; gingivektomija; izvanstanicni matriks; gen ANTXR2
The osteocutaneous radial free flap, even after 30 years, is still considered to be the "workhorse" for head and neck reconstruction. A high incidence of donor site fractures has remained a major ...problem, however. The technique described here is a method developed for the prophylaxis of fractures of the donor site of the harvested radial bone and is based on a modification of the intramedullary Rush nail fixation. The data were collected from 18 patients in whom the radial forearm free flap had been used during reconstruction. None of the prophylactically-nailed radii fractured. The complications experienced with this technique are general complications, such as scarring of the forearm and dehiscence of the wound. This technique is simple, and has given excellent results. Aesthetic and functional results were comparable to those of other flaps used for reconstruction. We recommend this technique because of its simplicity, vascular safety, and cost effectiveness.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
The authors present their experience of 27 cases with repairs of defects following radical maxillectomies with free flaps. A total of 28 flaps were used (five latissimus dorsi, six scapula, 16 ...combination flaps of scapula and latissimus dorsi and one combination of scapula, latissimus dorsi and serratus anterior flap). Only one scapula flap was completely lost and in three cases where a combination of scapula and latissimus dorsi flap was used, partial necrosis of one component occurred. The authors first choice for reconstruction is a scapula bone flap raised on the angular artery combined with the latissimus dorsi flap. The combination of flaps with a long pedicle and of the bony and muscular components provides the surgeon with the option of customizing the flap to meet individual patient needs. For intraoral closure the authors prefer the latissimus dorsi muscle which rarely requires secondary procedures for prosthesis placement following epithelialization and atrophy. The main disadvantage of the flap is the difficulty of two teams working simultaneously, thus increasing the average operating time. All postoperative corrections and prosthetic rehabilitation should be postponed for at least 2 months following surgery because of postoperative swelling.
Summary A total of 21 patients with latissimus dorsi-scapula free flap reconstruction immediately following radical maxillectomy together with orbital exenteration are presented. Orbital exenteration ...was performed in all patients due to tumour invasion at the time of diagnosis. There was no total flap failure. Two tissue components subdivided into separate flap units with individual vascular pedicles linked by a single vascular source provide an ideal reconstructive solution for massive defects of the mid-face and orbit. Separate arcs of rotation of each flap unit permit greater mobility necessary for complex three-dimensional reconstruction. A vertically positioned angle of the scapula enables simultaneous reconstruction of the malar eminence and alveolar ridge whereas spontaneous intraoral epithelialisation of the latissimus dorsi muscle requires no additional procedure. For these reasons, in our opinion, combined latissimus dorsi-scapula free flap should be considered the first choice in reconstruction of defects following total maxillectomy with orbital exenteration.
Summary
Peer-to-peer (P2P) computing is currently receiving a great deal of attention. Much of the current attention is focused on the use of P2P computing for exchanging media files. But P2P systems ...have actually been around for a long time and many application scenarios exist. In this paper we discuss on one hand the evolution of P2P middleware from simple file-sharing to decentralized data storage; on the other hand the impact on the future development of decentralized workflows. Building up a P2P based data storage, with similar properties as a standard data store, opens many research issues. While P2P databases enable distributed data storage, distributed control flows are considered in decentralized workflows. The paper will present several research issues addressing the collaboration and coordination problem in distributed environments without a centralized coordination instance by means of a B2B application scenario.
Madelung syndrome is a rare disease found predominantly in the Mediterranean area. It has a distinctive clinical appearance. Staged surgery is the treatment of choice, which produces substantial ...improvement in both functional and aesthetic appearance. Recurrences usually occur when it is impossible to resect the disease completely.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
38.
Enabling High Data Availability in a DHT Knezevic, P.; Wombacher, A.; Risse, T.
16th International Workshop on Database and Expert Systems Applications (DEXA'05),
2005
Conference Proceeding
Odprti dostop
Many decentralized and peer-to-peer applications require some sort of data management. Besides P2P file-sharing, there are already scenarios (e.g. BRICKS project) that need management of ...finer-grained objects including updates and, keeping them highly available in very dynamic communities of peers. In order to achieve project goals and fulfill the requirements, a decentralized/P2P XML storage on top of a DHT (distributed hash table) overlay has been proposed. Unfortunately, DHTs do not provide any guarantees that data will be highly available all the time. A self-managed approach is proposed where availability is stochastically guaranteed by using a replication protocol. The protocol recreates periodically missing replicas dependent on the availability of peers. We are able to minimize generated costs for requested data availability. The protocol is fully decentralized and adapts itself on changes in community maintaining the requested availability. Finally, the approach is evaluated and compared with replication mechanisms embedded in other decentralized storages
Hijalini fibromatozni sindrom (HFS) rijedak je autosomno recesivni genetski poremećaj koji karakterizira nakupljanje hijaline tvari u tkivima s kožnim, sluzničkim, koštanim, zglobnim i sistemskim ...manifestacijama. Bolest je uzrokovana mutacijom gena ANTXR2 koja rezultira sintezom neispravnog transmembranskog proteina, pa se hijalini depoziti prekomjerno talože u međustaničnom prostoru. Prvi znakovi mogu biti prisutni pri rođenju ili tijekom dojenačke dobi, a prvi simptom najčešće
je ukočenost zgloba. Ostale manifestacije uključuju zglobne kontrakture, hiperpigmentirane makule kože iznad koštanih prominecija zglobova i hipertrofiju gingive, što je uz potkožne nodule (kvržice), vrlo često simptom koji potiče sumnju na HFS. Napredovanje bolesti uključuje enteropatiju s velikim gubitkom proteina, kroničnu dijareju i česte infekcije. U radu predstavljamo petogodišnju djevojčicu s teškim oblikom gingivne hipertrofije koja je uzrokovala poteškoće s hranjenjem i govorom. Prema našim spoznajama to je prvi pacijent opisan u Hrvatskoj s dokazanom mutacijom gena ANTXR2.
Cleft lip and palate is the most common congenital deformity affecting craniofacial structures. Orofacial clefts have great impact on the quality of life which includes aesthetics, function, ...psychological impact, dental development and facial growth. Incomplete fusion of facial prominences during the fourth to tenth week of gestation is the main cause. Cleft gaps are closed with alveolar bone grafts in surgical procedure called osteoplasty. Autogenic bone is taken from the iliac crest as the gold standard. The time of grafting can be divided into two stages: primary and secondary. The alveolar defect is usually reconstructured between 7 and 11 years and is often related to the development of the maxillary canine root. After successful osteoplasty, cleft defect is closed but there is still a lack of tooth. The space closure with orthodontic treatment has 50-75 % success. If the orthodontic treatment is not possible, in order to replace the missing tooth there are three possibilities: adhesive bridgework, tooth transplantation and implants. Dental implant has the role of holding dental prosthesis, prevents pronounced bone atrophy and loads the augmentation material in the cleft area. Despite the fact that autologous bone from iliac crest is the gold standard, it is not a perfect source for reconstruction of the alveolar cleft. Bone morphogenic protein (BMP) is appropriate as an alternative graft material. The purpose of this review is to explain morphology of cleft defects, historical perspective, surgical techniques and possibilities of implant and prosthodontic rehabilitation. Key words Cleft Lip; Cleft Palate; Alveolar Bone Grafting; Bone Morphogenetic Proteins; Dental implants; Dental prosthesis; Orthodontic Space Closure Rascjep usne i nepca najcesci je prirodeni deformitet kraniofacijalne strukture. Orofacijalni rascjep znatno utjece na kvalitetu zivota--na estetiku, funkciju te na dentalni razvoj i rast lica, a ima i psiholoski ucinak. Glavni razlog za njegov nastanak jest nekompletno spajanje facijalnih nastavaka od cetvrtog do desetog tjedna gestacije. Zatvara se alveolarnim kostanim presatkom kirurskim postupkom nazvanim osteoplastika, a zlatnim standardom smatra se autogena kost s kriste ilijake. Presadivanje moze biti podijeljeno u dvije faze: primarnu i sekundarnu. Defekt alveolarnog grebena obicno se rekonstruira izmedu sedme i jedanaeste godine i cesto je povezan s razvojem korijena maksilarnog ocnjaka. Nakon uspjesnog zahvata defekt rascjepa je zatvoren, ali nedostaje zub. Zatvaranje toga prostora ortodontskom terapijom uspjesno je u 50 do 75 posto slucajeva. Ako to nije moguce, zub koji nedostaje moze se nadomjestiti na tri nacina: protetskim mostom, transplantacijom zuba i dentalnim implantatom. Dentalnom je implantatu zadatak drzati protetski nadomjestak, prevenirati atrofiju kosti i zadrzavati augmentacijski materijal u podrucju rascjepa. Unatoc tomu sto je autologna kost s kriste ilijake zlatni standard, nije idealni izbor za rekonstrukciju alveolarnog defekta. Kao alternativni kostani materijal moze se upotrijebiti morfogenetski protein (BMP). U ovom clanku zeli se objasniti morfologija rascjepa, povijesna saznanja, kirurske tehnike i mogucnosti implantoprotetske rehabilitacije. Kljucne rijeci rascjep usne; rascjep nepca; alveolarna kost, usadivanje nadomjestka, kostani morfogenetski proteini; zubni implantati, zubne proteze; ortodontsko zatvaranje prostora
PDF
