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    February 11

    Hemoptysis &. Ehlers Danlos syndrome

     

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    Medical Insight

    Self-made Audio Programme 41

    download Roy audio material at

    http://www.zshare.net/audio/7422195c868cbd/

    Welcome to Medical Insight February 11, 2008. I’m Dr. Alex Roy.A 26-year-old man presented with a 1-month history of persistent cough productive of white sputum, which was occasionally tinged with blood. He reported mild pleuritic chest pain, his cough had been treated with azithromycin阿奇霉素 with no resolution of his symptoms. Five years before this presentation, the patient had been treated for a right-sided spontaneous pneumothorax. Three years before presentation, he was found to have a spontaneous left renal-artery dissection with renal infarction. On present examination, the patient appeared comfortable and was in no acute distress. He had generalized joint hypermobility involving both small and large joints. A chest X-ray revealed a left lower-lobe cavity with an air–liquid level, small left-sided apical pneumothorax, and left hydrothorax. Chest CT revealed  subcentimeter nodules亚厘米节结 with surrounding haziness. The patient received 14 days of intravenous ampicillin–sulbactam to treat a presumed lung abscess. 17 weeks later, the patient presented with massive hemoptysis咯血. In this case, both the discussant and the clinical team did what experienced diagnosticians often do when confronted with a patient whose illness does not lend itself to a simple diagnosis: try to find the connection between the present illness and past abnormalities.

    Comments (6)

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    Picture of Anonymous
    かわいいの兔 wrote:
    Arterial tears are the most serious complication of the Ehlers–Danlos syndrome, vascular type. Approximately half of all arterial complications (including tears, dissections, and aneurysms) affect arteries within the abdomen and thorax; the renal artery is commonly affected.
    Feb. 13
    alex Luowrote:
    You are right, Rose! Ehlers–Danlos syndromes are a heritable disease. Although the patient’s mother reportedly died of pulmonary embolism, it is possible that she died from a pulmonary manifestation of Ehlers–Danlos syndromes.
    Moreover, The patient’s hemoptysis, pulmonary cavitary nodules, and well organized hematoma on lung biopsy are consistent with pulmonary parenchymal tears, also reported in Ehlers–Danlos syndrome, vascular type.
    Feb. 12
    Picture of Anonymous
    Roma Rose wrote:
    Arterial tears are the most serious complication of the Ehlers–Danlos syndrome, vascular type.
    Feb. 12
    Picture of Anonymous
    Roma Rose wrote:
    The Ehlers–Danlos syndromes are a heterogeneous group of heritable disorders of connective tissue.
    Feb. 12
    Picture of Anonymous
    小千子 wrote:
    The main causes of cavities include neoplasm, infection,and collagen vascular disease.
    (1) Infection    Bacteria and fungi can cause cavities, but generally are associated with fever, leukocytosis, and elevation of the C-reactive protein level. Hydatid disease is another infectious possibility, except that patients are generally quite ill when a cavity ruptures into the pleural space. A primary infection, therefore, seems unlikely.
    (2) Neoplasm  The patient’s age and absence of smoking history make bronchogenic carcinoma unlikely. Lymphoma and metastases may cavitate, but in the absence of other systemic symptoms or examination findings, these possible diagnoses are unlikely.
    (3) Collagen vascular disease   Cavitary nodules can be seen with Wegener’s granulomatosis and rheumatoid arthritis, occasionally as the initial manifestation of disease.
    (4) Others      Septic emboli and thromboemboli may also occasionally cavitate, but the cavities are generally smaller than
    those here and multiple.
    Feb. 11
    alex Luowrote:
    爱-唐综合征(Ehlers-Danlos syndrome)亦称皮肤弹性过度症。1901年Ehler首次报告本病皮肤过度伸展;1904年Danlos报告本病患者血管脆性增强,是一具有遗传倾向的疾病。本病主要临床特点:皮肤弹性过度,拉起皮肤后松开迅速恢复,可有毛绒感,皮肤和血管脆弱,轻微外伤即出现瘀斑,关节松驰致过伸;常伴有先天畸形,如髋关节脱位,先天性心脏病等,亦可有贫血,出、凝血时间延长,束臂试验阳性,但血小板计数正常。
    Feb. 11

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