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Gastrointestinal Kohlmeier–Degos disease: a narrative review
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Orphanet Journal of Rare Diseasesvolume 17, Article number: 172 (2022) Cite this article
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Abstract
Introduction
Kohlmeier-Degos (K-D) disease is a rare obliterative vasculopathy that can present as a benign cutaneous form or with potentially malignant systemic involvement. The gastrointestinal tract is most frequently involved in systemic disease and mortality is often related to bowel perforations. Herein, we provide information to providers and patients regarding gastrointestinal K-D symptomology, pathology, treatment, and diagnosis, with a focus on the importance of timely diagnostic laparoscopy. We present three new cases of gastrointestinal K-D to highlight varying disease presentations and outcomes.
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Based on reviewed reports, perforation is preceded by at least one gastrointestinal symptom: abdominal pain/cramping, anorexia/weight loss, vomiting, diarrhea, nausea, gastrointestinal bleeding, obstipation, constipation, and abdominal fullness. Perforation most commonly occurs in the small intestine and often results in sepsis and death. Although underutilized, laparoscopy is the most sensitive and specific diagnostic technique, demonstrating serosal porcelain plaques similar to those on the skin and characteristic for K–D. The combination of eculizumab and treprostinil is presently the most effective treatment option for gastrointestinal K–D. The pathology of gastrointestinal K-D is characterized by an obliterative intimal arteriopathy eventuating in occlusive acellular deposits of mucin and collagen along with an extravascular pauci-cellular sclerosing process resembling scleroderma confined to the subserosal fat. C5b-9 and interferon-alpha are both expressed in all caliber of vessels in the affected intestine. While C5b-9 blockade does not prevent the intimal expansion, enhanc
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Overview of Balkan musician Gazmend Rama
From Kosovo, Gazmend Rama is a musician who performs European and Balkan music. He has established a distinct sound for himself in the music industry by fusing modern European sounds with traditional Balkan folk music. Rama is known for creating bouncy rhythms, bright melodies, and haunting voices that take listeners to numerous eras and locations in his songs.
Rama incorporates regional folk music into his songs, drawing inspiration from his Balkan heritage. He has made it his goal to preserve and promote his native country's musical legacy, and his music is a celebration of Balkan culture. Rama's passion for his nation and its people is evident in his music.
Gazmend Rama stands out for his commitment to preserving the distinctive musical traditions of his native country in a world where music is becoming more and more homogenized. His work serves as a reminder of the rich musical legacy of the Balkans and a celebration of Balkan culture. Rama is a force to be reckoned with in the European and Balkan music scenes thanks to his contagious enthusiasm and unparalleled talent.
What are the most popular songs for Balkan musician Gazmend Rama?
Kosovo's Gazmend Rama is renowned for his distinctive fusion of European and Balkan music. He is best known for songs like "ME MERCEDES," "Frangat si sheqera," and "Virusi." His talent to pen catchy, lively tunes that are ideal for dancing is demonstrated in these tracks.
"Je katundare," another of Rama's well-known songs, makes use of conventional Balkan instruments and rhythms. The song is a fan favorite due to its upbeat tempo and catchy tune. Another well-known song that demonstrates Rama's capacity to make cheerful yet emotional music is "Nusja djalit Tem".
Rama's music is renowned for its infectious beats and positive attitude. With its upbeat tune and contagious rhythm, his song "
Lernu Esperanton: Why Writers Embrace Artificial Languages
When no well-known language can adequately express what you want to convey, what do you do? For a few bold writers in the Low Countries, the answer was clear: they chose a new language, such as Esperanto, Ido, or Volapük. Marc van Oostendorp, an Esperanto enthusiast himself, rediscovers three writers who found their unique voice in an artificial language.
Writing is making choices, and the first choice of every multilingual writer is: which language to write in? Do you choose the language or dialect of your daily life, and perhaps that of your characters? Or do you go for a more widely spoken language to reach a broader audience?
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Both options are valid, yet writers sometimes choose a language that is not their native tongue, not spoken by the people around them, and rarely spoken elsewhere, with no native speakers. A language that, unlike Dutch, Chinese, or Arabic, didn’t naturally develop over time, but was crafted deliberately, such as Esperanto, introduced in 1887 by Polish-Jewish ophthalmologist Lejzer Zamenhof for international communication, or Dothraki, created in the 21st century by American David Peterson for the TV series Game of Thrones. They are languages that have no native speakers, nation, or historical heritage. Why would anyone ever want to write stories or poems in such a language?
Some writers are driven by idealism, aiming to expand the language’s global reach through their literature. Other authors believe this language offers greater potential to express themselves. The language’s artificiality gives them greater freedom to find their own distinct voice.
Occasionally, writers with similar views emerge in the Low Countries. For obvious reasons, they didn’t find a large audience, but that doesn’t seem to bother them. Three of them