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Jana Kazandjieva, Dimitrina Guleva, Sonya Márina, Assya Nikolova, Gergana Mladenova and Alexander Kurtev

Abbreviations CGL - Congenital generalized lipodystrophy BSS - Berardinelli-Seip syndrome References 1. Garg A, Agarwal AK. Lipodystrophies: disorders of adipose tissue biology. Biochim Biophys Acta. 2009;1791(6):507–3. 2. Nolis T. Exploring the pathophysiology behind the more common genetic and acquired lipodystrophies. J Hum Genet. 2014;59(1):16-23. 3. Abate N. Adipocyte maturation arrest: a determinant of systemic insulin resistance to glucose disposal. J Clin Endocrinol Metab. 2012;97(3):760–3. 4. Bertrand AT, Chikhaoui K

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Mirjana Paravina, Dragan Jovanović, Milenko Stanojević and Ljiljana Nikolić

Abstract

Langerhans cell histiocytosis is a disease which results from accumulation or proliferation of a clonal population of cells with the phenotype of Langerhans cells arrested at an early stage of activation that are functionally deficient. The etiology and pathogenesis of the disorder are still unknown. There are ongoing investigations to determine whether it is a reactive or a neoplastic disease. The fact is that neoplastic and reactive processes may have many clinical and pathological similarities. Some emphasize the role of “cytokine storm” in Langerhans cells. Further studies are necessary in all areas, from the etiology and pathogenesis to diagnosis and therapy. Langerhans cell histiocytosis primarily affects bones, but less commonly it may involve other organ systems, or present as a multisystem disease. The clinical course is variable, from benign forms with spontaneous resolution, to chronic disseminated forms with fatal outcome. This is a report of a 29-year-old man with Langerhans cell histiocytosis with an onset at the age of 8, which later progressed to a multisystem disease. Apart from lesions on the skin and exposed mucous membranes, the patient also presented with: diabetes insipidus, granuloma of the right femur and slight bulbar protrusion of the right eye. The patient experienced spontaneous pneumothorax on two occasions. The diagnosis of Langerhans cell histiocytosis was histologically confirmed using electron microscopy by presence of Birbeck granules in the histiocytes. A favorable therapeutic response was obtained after systemic corticosteroid therapy.

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Svetlana Popadić, Zorica Ramić, Ljiljana Medenica, Marija Mostarica-Stojkovic and Dušan Popadić

human endothelial cells. J Cell Physiol 2005;204:881-8. 10. Popadic S, Popadic D, Ramic Z, Mostarica Stojkovic M, Trajkovic V, Milinkovic M, et al. Chloramphenicol induces in vitro growth arrest and apoptosis of human keratinocytes. Cell Biol Toxicol 2006;22:371-379. 11. Decker T, Lohmann-Matthes ML. A quick and simple method for the quantitation of lactate dehydrogenase release in measurements of cellular cytotoxicity and tumor necrosis factor (TNF) activity. J Immunol Methods 1998;115:61-9. 12 Trajkovic V, Vuckovic O