A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler
Hereditary thrombotic thrombocytopenic purpura (hTTP) is a thrombotic microangiopathy caused by pathogenic variants in the ADAMTS13 gene resulting in highly reduced activity of von Willebrand factor-cleaving metalloprotease. hTTP is characterized by systemic for-mation of platelet-rich thrombi in mi...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Hrvatski liječnički zbor
2023-01-01
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| Series: | Liječnički vjesnik |
| Subjects: | |
| Online Access: | https://hrcak.srce.hr/file/453789 |
| _version_ | 1797206184553349120 |
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| author | Ozana Jakšić Jana Jelčić Maša Davidović |
| author_facet | Ozana Jakšić Jana Jelčić Maša Davidović |
| author_sort | Ozana Jakšić |
| collection | DOAJ |
| description | Hereditary thrombotic thrombocytopenic purpura (hTTP) is a thrombotic microangiopathy caused by pathogenic variants in the ADAMTS13 gene resulting in highly reduced activity of von Willebrand factor-cleaving metalloprotease. hTTP is characterized by systemic for-mation of platelet-rich thrombi in microvasculature resulting in organ ischemia, throm-bocytopenia, and microangiopathic hemolytic anemia. |
| first_indexed | 2024-04-24T09:02:59Z |
| format | Article |
| id | doaj.art-78159ac67a7c40eab9b3d48746749d15 |
| institution | Directory Open Access Journal |
| issn | 0024-3477 1849-2177 |
| language | English |
| last_indexed | 2024-04-24T09:02:59Z |
| publishDate | 2023-01-01 |
| publisher | Hrvatski liječnički zbor |
| record_format | Article |
| series | Liječnički vjesnik |
| spelling | doaj.art-78159ac67a7c40eab9b3d48746749d152024-04-15T19:21:36ZengHrvatski liječnički zborLiječnički vjesnik0024-34771849-21772023-01-01145Supp 8242410.26800/LV-145-supl8-15A rare case of hereditary thrombotic thrombocytopenic purpura in a toddlerOzana JakšićJana JelčićMaša DavidovićHereditary thrombotic thrombocytopenic purpura (hTTP) is a thrombotic microangiopathy caused by pathogenic variants in the ADAMTS13 gene resulting in highly reduced activity of von Willebrand factor-cleaving metalloprotease. hTTP is characterized by systemic for-mation of platelet-rich thrombi in microvasculature resulting in organ ischemia, throm-bocytopenia, and microangiopathic hemolytic anemia.https://hrcak.srce.hr/file/453789ADAMTS13 protease, pediatrics, thrombotic thrombocytopenic purpura |
| spellingShingle | Ozana Jakšić Jana Jelčić Maša Davidović A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler Liječnički vjesnik ADAMTS13 protease, pediatrics, thrombotic thrombocytopenic purpura |
| title | A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler |
| title_full | A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler |
| title_fullStr | A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler |
| title_full_unstemmed | A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler |
| title_short | A rare case of hereditary thrombotic thrombocytopenic purpura in a toddler |
| title_sort | rare case of hereditary thrombotic thrombocytopenic purpura in a toddler |
| topic | ADAMTS13 protease, pediatrics, thrombotic thrombocytopenic purpura |
| url | https://hrcak.srce.hr/file/453789 |
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