| dbo:abstract |
متلازمة باكاك تشوانغ هي أعراض مرض موصوفة حديثا في الإناث وتشمل ورم جنيب العقدة العصبيةوكثرة خلايا الدم الحمراء مع مستويات مرتفعة لهرمون إريثروبويتن. أورام جنيب العقدة العصبية في هؤلاء المرضى تكون مُركزة بشكل أساسي في البطن كما يبدو من التصوير الرنيني والكيمياء الحيوية. (ar) Le syndrome de Pacak-Zhuang est une maladie génétique rare, due à une mutation sur le gène EPAS1 et associant paragangliomes et polycythémie. (fr) The Pacak-Zhuang syndrome is a recently described disease manifestation in females that includes multiple paragangliomas or pheochromocytomas and somatostatinomas (in some), both neuroendocrine tumors, and secondary polycythemia associated with high erythropoietin levels. Paragangliomas in these patients are mainly localized to the abdomen whereas somatostatinomas are found in the second portion of the duodenum, as shown by imaging or biochemistry. This syndrome is of special interest as finding more than one type of neuroendocrine tumor in one individual is unusual. Such co-occurrences are usually seen in patients carrying hereditary syndromes like multiple endocrine neoplasia (MEN), neurofibromatosis 1 (NF1), or von Hippel-Lindau (VHL) disease. (en) |
| dbo:wikiPageID |
43506671 (xsd:integer) |
| dbo:wikiPageLength |
5866 (xsd:nonNegativeInteger) |
| dbo:wikiPageRevisionID |
1077164264 (xsd:integer) |
| dbo:wikiPageWikiLink |
dbr:Multiple_endocrine_neoplasia dbr:Belzutifan dbr:Antihypertensive_drug dbr:Hypertension dbr:Hypoxia-inducible_factor dbr:Medical_imaging dbr:Optic_disc dbr:Anxiety dbr:Cholecystitis dbr:Functional_imaging dbr:Point_mutation dbr:Syndrome dbr:Duodenum dbr:Erythropoietin dbr:Biochemical dbr:Vasodilation dbc:Syndromes dbr:Diabetes dbr:Pheochromocytoma dbr:Phlebotomy dbr:Somatostatinoma dbr:Fibrosis dbr:Polycythemia dbr:Paragangliomas dbr:Palpitations dbr:Neuroendocrine dbr:Von_Hippel-Lindau dbr:Neurofibromatosis_1 |
| dbp:wikiPageUsesTemplate |
dbt:Cite_journal |
| dcterms:subject |
dbc:Syndromes |
| rdfs:comment |
متلازمة باكاك تشوانغ هي أعراض مرض موصوفة حديثا في الإناث وتشمل ورم جنيب العقدة العصبيةوكثرة خلايا الدم الحمراء مع مستويات مرتفعة لهرمون إريثروبويتن. أورام جنيب العقدة العصبية في هؤلاء المرضى تكون مُركزة بشكل أساسي في البطن كما يبدو من التصوير الرنيني والكيمياء الحيوية. (ar) Le syndrome de Pacak-Zhuang est une maladie génétique rare, due à une mutation sur le gène EPAS1 et associant paragangliomes et polycythémie. (fr) The Pacak-Zhuang syndrome is a recently described disease manifestation in females that includes multiple paragangliomas or pheochromocytomas and somatostatinomas (in some), both neuroendocrine tumors, and secondary polycythemia associated with high erythropoietin levels. Paragangliomas in these patients are mainly localized to the abdomen whereas somatostatinomas are found in the second portion of the duodenum, as shown by imaging or biochemistry. This syndrome is of special interest as finding more than one type of neuroendocrine tumor in one individual is unusual. Such co-occurrences are usually seen in patients carrying hereditary syndromes like multiple endocrine neoplasia (MEN), neurofibromatosis 1 (NF1), or von Hippel-Lindau (VHL) disease. (en) |
| rdfs:label |
متلازمة باكاك تشوانغ (ar) Syndrome de Pacak-Zhuang (fr) Pacak–Zhuang syndrome (en) |
| owl:sameAs |
freebase:Pacak–Zhuang syndrome wikidata:Pacak–Zhuang syndrome dbpedia-ar:Pacak–Zhuang syndrome dbpedia-fr:Pacak–Zhuang syndrome https://global.dbpedia.org/id/mx7g |
| prov:wasDerivedFrom |
wikipedia-en:Pacak–Zhuang_syndrome?oldid=1077164264&ns=0 |
| foaf:isPrimaryTopicOf |
wikipedia-en:Pacak–Zhuang_syndrome |
| is dbo:wikiPageRedirects of |
dbr:Pacak-Zhuang_syndrome |
| is dbo:wikiPageWikiLink of |
dbr:Belzutifan dbr:Pheochromocytoma dbr:List_of_syndromes dbr:Pacak-Zhuang_syndrome |
| is foaf:primaryTopic of |
wikipedia-en:Pacak–Zhuang_syndrome |