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Hughes-Stovin Syndrome


Presentation

  • Case presentation We report the case of an 18 years old, greek male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis.[ncbi.nlm.nih.gov]
  • We report the case of an 18 years old, greek male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis.[ncbi.nlm.nih.gov]
  • The first patient was a 26-year-old male who initially presented with deep vein thrombosis (DVT) in the right lower limb followed shortly by hemoptysis.[ncbi.nlm.nih.gov]
  • The lack of formal diagnostic criteria and the rarity of the disease make the diagnosis very challenging, especially when respiratory complaints are not present at onset, as in the presented case.[ncbi.nlm.nih.gov]
Recurrent Pulmonary Embolism
  • We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy.[read.qxmd.com]
Dry Cough
  • We report the case of a young Afro-Caribbean patient who complained of dry cough and low-grade fever.[pubfacts.com]
  • After 10 days on heparin therapy, dry cough begun, followed by an episode of moderate haemoptysis (100–120 ml). At review his vital signs were normal and his platelet count and coagulation tests were within normal limits.[doi.org]
Loss of Appetite
  • She presented with complaints of fever of 8 months duration with cough, loss of weight, and loss of appetite. She had recurrent hemoptysis which was of moderate quantity for the past 6 months.[indjvascsurg.org]

Workup

  • Her coagulation profile was normal and workup for systemic lupus erythematosus and antiphospholipid antibody syndrome was negative. Her chest X-ray showed homogenous radiopaque lesion in the right lower lobe [Figure 1] a.[indjvascsurg.org]
  • Further laboratory workups ruled out other connective tissue diseases except for elevated lupus anticoagulant level (LA1 54.9, normal 30 to 44; LA2 37.2, normal 26 to 32).[jmedicalcasereports.biomedcentral.com]
Normal Chest X-Ray
  • Urinalysis and protein: creatinine ratio were normal. Chest x-ray demonstrated a right lower zone circumscribed lesion. He was treated with antibiotics for community-acquired pneumonia.[academic.oup.com]
Fibrinoid Necrosis
  • In comparison, the histology of vascular lesions in BD is characterized by a triad of "neutrophilic infiltration", "endothelial cell swelling" and "fibrinoid necrosis" [ 5 ].[doi.org]
Foam Cell
  • The elastic and muscular fibers become annihilated whereas tunica media is completely filled with lymphocytes, plasma cells and foam cells in the affected vessels.[g3m.me]

Treatment

  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • Accordingly, HSS associated with hyperhomocysteinemia was diagnosed, and the clinical and radiological improvement was achieved after treatment with prednisolone, warfarin, and folic acid.[ncbi.nlm.nih.gov]
  • Pathogenesis, imaging investigation and treatment of this syndrome are also briefly discussed.[ncbi.nlm.nih.gov]
  • The treatment aims to reduce inflammation, although there is debate about anticoagulation therapy because of the risk of pulmonary haemorrhage.[ncbi.nlm.nih.gov]
  • These case studies cast certain doubts as to the effectiveness of the corticosteroid treatment usually prescribed. Finally, we suggest that Hughes-Stovin syndrome might be, in fact, a manifestation of Behcet's disease.[ncbi.nlm.nih.gov]

Prognosis

  • Overall, patients with HSS have a poor prognosis and aneurysmal rupture is the leading cause of death. However, early diagnosis and timely intervention is crucial in improving the prognosis.[ncbi.nlm.nih.gov]
  • Prognosis As most patients with HSS are diagnosed late in the disease course, the syndrome is associated with significant mortality due to massive hemoptysis resulting from PAA rupture or systemic bronchial artery hypertrophy secondary to ischemia related[orpha.net]
  • Epidemiology, genetics, pathogenesis, organ system involvement, differential diagnosis, novel treatments, surgical management, and prognosis are just some of the topics addressed.[books.google.de]
  • In particular, pulmonary artery aneurysms have a poor prognosis. The documents contained stovib this web site are yughes for information purposes only. Hughes-Stovin syndrome.[olox.pro]

Etiology

  • Etiology The etiology of HSS is unknown; however, it is assumed that HSS is a form of vasculitis following a similar mechanism of pathogenesis to that thought to be involved in BD.[orpha.net]
  • Hughes-Stovin syndrome is an exceedingly rare disorder of unknown etiology, and it is characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis.[ncbi.nlm.nih.gov]
  • There is a need to clearly elucidate the genetic, etiologic and pathologic basis for HSS in the future.[ncbi.nlm.nih.gov]

Epidemiology

  • Summary Epidemiology Prevalence is unknown but fewer than 30 cases have been reported in the literature since its first description in 1959 by Hughes and Stovin.[orpha.net]
  • Epidemiology, genetics, pathogenesis, organ system involvement, differential diagnosis, novel treatments, surgical management, and prognosis are just some of the topics addressed.[books.google.de]
  • Behcet’s: Epidemiology Disease prevalence and expression vary geographically Mediterranean Basin Turkiye has the highest prevalence: 80 to 370 cases per 100,000 population. 5.[slideshare.net]
Sex distribution
Age distribution

Pathophysiology

  • A similar pathophysiology seems to underlay the two syndromes and BS may represent a more full phenotype of this pathophysiology with multisystem involvement. Conclusion: HSS may be a forme fruste of BS.[cmsc.confex.com]
  • Indeed, pulmonary involvement can be indistinguishable between these two conditions of unknown pathophysiology.[ncbi.nlm.nih.gov]
  • Behcet’s: Pathophysiology The underlying cause of Behcet's disease is unknown.[slideshare.net]
  • […] of Asthma •The New Perspective of Pathophysiology and Pathogenesis Cow Milk Allergy •Patogenesis dan Patofisiologi Terkini Alergi Rinitis •The New Perspective Antiphospohlipid syndrome •The New Insight of Neonatal Autoimmune Diseases •The New Insight[pingpdf.com]
  • However, whether the two conditions are identical is open to both debate and speculation as the exact pathophysiology of both syndromes remains unclear.[doi.org]

Prevention

  • Conventional pulmonary angiography could not be performed, because thrombus in both the inferior and superior vena cavae prevented catheter passage.[ncbi.nlm.nih.gov]
  • Richard Webb MD Key HRCT Findings of Lung Disease interlobular septal thickening honeycombing irregular reticular opacities traction bronchiectasis More information Preventing Venous Thromboembolism Preventing Venous Thromboembolism (VTE) Patient Information[docplayer.net]
  • It is also associated with low platelet or blood elements that prevent bleeding.[books.google.de]
  • The development of better therapeutic agents is also needed to address and prevent the serious consequences arising from pulmonary arterial aneurysms seen in BD and HSS.[ncbi.nlm.nih.gov]
  • Conventional pulmonary M- giography could not be per- formed, because thrombus in both the inferior and superior vena cavae prevented cathe- ter passage.[documents.tips]

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