Damir Matoković, Mihovil Plečko, Krešimir Crnogaća, Nika Šlaus, Boris Žulj, Marko Pećina
{"title":"Arthropathia ochronotica","authors":"Damir Matoković, Mihovil Plečko, Krešimir Crnogaća, Nika Šlaus, Boris Žulj, Marko Pećina","doi":"10.21857/yvjrdcl4ey","DOIUrl":"https://doi.org/10.21857/yvjrdcl4ey","url":null,"abstract":": Alkaptonuria (AKU) is a rare autosomal recessive metabolic disease caused by a disorder of phenylalanine and tyrosine metabolism, resulting in accumulation and deposition of homogentisic acid (HGA) in the body. This deposition further causes progressive functional disorders in different organ systems, with the locomotor system being the most affected one. A specific triad of symptoms occurs in patients suffering from AKU: 1. at birth, a change of urine color is present when urine is exposed to air, 2. occurrence of dark pigmentations in connective tissues becomes visible over time, 3. complications of the locomotor, urogenital and cardiovascular systems present gradually. Arthropathia ochronotica occurs in patients suffering from AKU due to precipitation and deposition of HGA in the joint tissue (cartilage, tendons, ligaments, menisci, etc.). The accumulation can be seen as small foci of blue-black pigmentations. HGA deposits in collagen fibers, causing tendons and ligaments thickening. This causes them to be less resistant to mechanical forces, resulting in frequent ruptures caused by minimal trauma. Also, the deposition facilitates cartilage degeneration, often requiring an operative treatment. The knees are the most commonly affected joints, while changes can be seen in the spine and other large joints. As there is no specific treatment, alleviation of symptoms is the only treatment option. It has the goal of increasing individual functionality and quality of life. As an option for end-stage treatment, joint replacement surgery proved to be effective. In the future, an enzyme replacement therapy or gene therapy may be developed to treat AKU successfully.","PeriodicalId":115186,"journal":{"name":"Knj. 52-53(2020)","volume":"105 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127525095","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Terrestrial rabies eliminated in Croatia – a historical overview","authors":"J. Madić, L. Barbić, I. Lojkić","doi":"10.21857/yrvgqtejr9","DOIUrl":"https://doi.org/10.21857/yrvgqtejr9","url":null,"abstract":": Rabies is an ancient disease, known worldwide for a thousand years. Nowadays, vast areas of Western and Central Europe have been freed from rabies. To mark the expectation that Croatia will be officially declared free of classical terrestrial rabies by 2020, this paper was written to present a historical overview of the epidemiological characteristics of rabies in Croatia, based on literature data. Rabies in humans and animals was described in the 19 th century on the Croatian territory as a significant public health problem. It caused considerable human and animal death. One of the oldest report of rabies in Croatia is dated in 1783, when two people from the city of Šibenik were bitten by a rabid cat and a rabid dog. Epidemics of rabies that were spread by packs of stray dogs prompted the state authorities in the second half of the 19 th century to control the movements of these dogs. The first announce-ment of taxes on dogs in Croatia was recorded in 1857. However, it was introduced as early as January 1 st , 1867. The first campaign of preventive dog vaccination by a phenol vaccine prepared from virus fixé, was performed in 1933. Dog vaccination and strict control measures resulted in the reduction of dog-mediated rabies cases to zero in the whole of Croatia in 1967. Ten years later, the first cases of sylvatic rabies in Croatia were detected in three foxes. By the end of 1986 rabies was found in the whole territory of Croatia apart from Dubrovnik and the islands. Introduction of oral vaccination of foxes throughout the whole Croatian territory in 2011, resulted in the rapid decrease of rabies cases in animals to zero in 2014. Recent studies have been focused on rabies in bats. Neutralizing antibodies against the European bat lyssavirus-1 were detected in bats which proves that the bat population in Croatia was in contact with the virus.","PeriodicalId":115186,"journal":{"name":"Knj. 52-53(2020)","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121093190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}