Aneurismas de las arterias pulmonares en relación al síndrome de Hughes Stovin / Aenurysms of the pulmonary arteries in relation to Hughes Stovin syndrome

El síndrome de Hughes Stovin es una vasculitis de pequeños y grandes vasos, más frecuente en hombres jóvenes. Se manifiesta con trombosis y aneurismas. Inicia con tromboflebitis que evoluciona a aneurismas con eventual desenlace fatal por ruptura de los mismos. Algunos autores consideran el síndrome de Hughes Stovin como una variante de la enfermedad de Behçet. El diagnóstico es clínico y el tratamiento de primera línea son los corticoides y la ciclofosfamida. Frente a un caso de trombosis, la anticoagulación es controversial por el riesgo de sangrado. Presentamos el caso de un varón joven con cuadro clínico compatible en el cual se descartaron otras patologías con requerimiento de tratamiento quirúrgico por mala evolución clínica.

Hughes Stovin syndrome is characterized by small and big vessels vasculitis, more commonly presented in young men. Initially it manifests with thrombophlebitis and aneurysms which can cause fatal bleed due to rupture. Some authors consider Hughes Stovin syndrome as a variant of Behcet's disease. Diagnosis is made by clinical findings and the first line treatment are corticosteroids and cyclophosphamide. Anticoagulation is controversial due to the increased risk of hemorrhage.

Effect of Tibetan medicine E-se on insulin resistance in KK-Ay mice with spontaneous type 2 diabetes / 中国药理学通报

Aim To evaluate the effect of E-se extract on insulin resistance in KK-Ay mice with spontaneous type 2 diabetes anrl explore its mechanism.Methods Ten C57/6J mice were assigned to a normal control group.Fifty KK-Ay model mice were randomly divided into model group, positive control group ( rosiglita- zone, 2.67 mg • kg 1 ), and low- ( 0.75 g • kg 1 ) , medium- ( 1.50 g • kg 1 ) , and high-dose ( 3.00 g • kg ') E-se groups, with 10 mice in each group.All mice were measured for body weight and fasting blood glucose weekly, insulin tolerance on the 32nd day, and insulin after the last administration on the 35th day, and the insulin resistance/sensitivity indexes were calculated.The pancreas was stained by hematoxylin- eosin ( HE ).Islet cell apoptosis was detected by TUNEL staining.Glucagon-like peptide-1 ( GLP-1 ) was detected by immunohistochemistry.Results j j Compared with the model group, the E-se groups showed reduced body weight, fasting blood glucose, serum insulin concentration, and insulin resistance in¬dex, elevated insulin sensitivity index, decreased le¬sion grading score of pancreatic tissues and apoptosis percentage of islet cells, and increased content of GLP- 1 protein in pancreatic tissues.Conclusions E-se ex¬tract can improve insulin resistance by reducing serum insulin level, inhibiting islet cell apoptosis, and in¬creasing the sensitivity of the body to insulin.

Actualización diagnóstico-terapéutica del síndrome antifosfolípido o de Hughes / Therapeutic diagnostic update of Hughes antiphospholipid syndrome

El síndrome antifosfolipídico es una enfermedad del sistema inmune (trombofilia), que se caracteriza por la asociación de los anticuerpos antifosfolipídicos con trombosis de repetición, abortos o pérdidas fetales recurrentes y trombocitopenia. Descrito inicialmente en el lupus eritematoso sistémico, aparece también en personas que no reúnen criterios de alguna enfermedad conocida, por lo que su identificación y tratamiento adquieren gran importancia en personas con fenómenos trombóticos aparentemente inexplicables y en mujeres con abortos y muertes fetales recurrentes sin otra causa reconocible. A pesar de que los fenómenos clínicos que caracterizan a esta enfermedad ocurren frecuentemente, la incidencia es baja. Por lo que toma importancia, la identificación de los anticuerpos antifosfolípidos mediante ensayos para su detección. El síndrome antifosfolípido no tiene cura, pero pueden prevenirse los eventos trombóticos corrigiendo los factores de riesgo para trombosis y usando una terapia con anticoagulante oral para el resto de la vida.

Antiphospholipid Syndrome is a disease of the immune system (thrombophilia), which is characterized by the association of antiphospholipid antibodies with recurrent thrombosis, abortions or recurrent fetal losses and thrombocytopenia. Initially described in systemic lupus erythematosus, it also appears in people who do not meet the criteria of a known disease, so its identification and treatment acquire great importance in people with apparently inexplicable thrombotic phenomena and in women with abortions and recurrent fetal deaths without other cause recognizable. Although the clinical phenomena that characterize this disease occur frequently, the incidence is low. So it is important, the identification of antiphospholipid antibodies through tests for their detection. Antiphospholipid Syndrome has no cure, but thrombotic events can be prevented by correcting the risk factors for thrombosis and using an oral anticoagulant therapy for the rest of your life.

O estudo de práticas médicas: o cenário da sociologia das profissões / L'étude des pratiques médicales: l'écran de la sociologie des professions / Study of medical practices: the framework of the sociology of professions

Résumé La sociologie de la médecine s'est constituée à partir du modèle de la sociologie américaine des professions qui a émergé au cours des années 1930 (modèle qui a aussi marqué les historiens de la médecine). Cet article retrace le contexte dans lequel a émergé puis s'est institutionnalisé la sociologie des professions aux États-Unis à partir notamment des travaux des Talcott Parsons, Everett Hughes et Eliot Freidson. Aujourd'hui encore les questionnements portés sur les pratiques médicales en subissent fortement l'empreinte alors même que la sociologie des professions comporte des postulats discutés et discutables et des points aveugles importants, tel le lien entre l'État et la médecine.

Abstract The sociology of medicine is constituted from the model of the American sociology of professions, which has emerged in the 1930s (a model that has also marked the historians of medicine). This article traces the context in which the sociology of professions emerged and later became institutionalized in the United States, notably from the works by Talcott Parsons, Everett Hughes and Eliot Freidson. Even now, questionings about medical practices are strongly influenced by these authors, while the sociology of professions include debated and debatable postulates and important blind spots, such as the link between State and medicine.

Resumo A sociologia da medicina é feita a partir do modelo da sociologia americana das profissões que surgiu no decorrer da década de 1930 (modelo que também marcou os historiadores da medicina). O artigo traz de volta o contexto em que a sociologia das profissões surgiu e, em seguida, quando foi institucionalizada nos Estados Unidos, sobretudo a partir dos trabalhos de Talcott Parsons, Everett Hughes e Eliot Freidson. Ainda hoje os questionamentos realizados em práticas médicas são fortemente influenciados por essa empreitada, enquanto a sociologia das profissões comporta suposições discutidas e discutíveis e pontos cegos importantes, tais como a ligação entre o Estado e a medicina.

A brief history of antiphospholipid antibodies and antiphospholipid syndrome / Uma breve história dos anticorpos antifosfolípides e da síndrome antifosfolípide

AIMS: To review the historical reports on antiphospholipid antibodies (aPL) from the early years of the 20th century; to outline the cardinal features of the antiphospholipid syndrome (APS) from 1983 on, including clinical criteria, etiopathogenesis and current therapy. METHODS: Literature review using PubMed. Articles on the history of aPL and APS were selected. RESULTS: The original aPL were described in patients with syphilis yet in 1906 by Wassermann. A first definition of lupus anticoagulant was proposed in 1963,while the anticardiolipin antibody (aCL) test was depicted twenty years later. The APS, initially reported by Hughes in 1985as the "aCL syndrome", is one of the most prevalent acquired thrombophilia. Venous and arterial thrombosis, associated or not to pregnancy morbidity, comprise the main features. It is a novel disorder firstly associated to systemic lupus erythematosus. A primary form of APS was put forward in 1989, and many APS variants are currently known. Lifelong, full-dose anticoagulation is the mainstream for treatment of thrombotic APS. In obstetric APS, the combination of acetil-salicilic acid and enoxoparin has been a mostly effective therapy. CONCLUSIONS: The sequential characterization of aPL since Wassermann in 1906, and later of the APS in the 1980-thies, is a rather interesting example of how a new entity is sketched step by step. APS is an intriguing novel cause of autoimmune thrombophilia, with a complex pathogenesis and a plethora of clinical and laboratory abnormalities. Treatment is based on life-long anticoagulation.

OBJETIVOS: Revisar os relatos históricos sobre anticorpos antifosfolípides (aAF) dos primeiros anos do século XX; delinear as características cardinais da síndrome antifosfolípide (SAF) a partir de 1983, incluindo critérios clínicos, etiopatogênese e terapia atual. MÉTODOS: Revisão de literatura utilizando o PubMed. Foram selecionados artigos com foco na história dos aAF e da SAF. RESULTADOS: Os aAF foram originalmente descritos em pacientes com sífilis ainda em 1906 por Wassermann. Uma primeira definição do anticoagulante lúpico foi proposta em 1963, enquanto o anticorpo anticardiolipina (aCL) foi descrito 20 anos mais tarde. A SAF, inicialmente reportada por Hughes em 1985 como "síndrome do aCL" é uma das mais prevalentes trombofilias adquiridas. Tromboses arteriais e venosas, associadas ou não à morbidade gestacional, compreendem os achados principais. É uma nova entidade, tendo sido primeiramente associada ao lupus eritematoso sistêmico. Uma forma primária de SAF foi reconhecida em1989, e muitas variantes de SAF são modernamente conhecidas. A terapia-padrão para a SAF trombótica é a anticoagulação plena e ininterrupta. Na SAF obstétrica, a combinação de ácido acetil-salicílico com enoxaparina tem-se mostrado altamente efetiva. CONCLUSÕES: A caracterização sequencial dos aAF desde Wasserman em 1906, e mais tarde da SAF nos anos 1980, é um interessante exemplo de como uma nova entidade é concebida passo a passo. A SAF é uma nova e intrigante causa de trombofilia autoimune, com uma complexa patogênese e uma pletora de manifestações clínicas e laboratoriais. O tratamento é baseado em anticoagulação contínua.

Hughes-Stovin Syndrome as an Outcome of Behçet Disease or as a Different Entity / 대한흉부외과학회지

Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym “Hughes-Stovin syndrome” was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease.

Síndrome de Hughes-Stovin / Hughes-Stovin Syndrome

El síndrome de Hughes-Stovin (SHS) es una enfermedad rara, caracterizada por la presencia de tromboflebitis y aneurismas de las arterias pulmonares y/o bronquiales. Si bien la etiología y patogénesis del SHS se desconoce, se han identificado asociaciones con ciertas infecciones y angiodisplasia. Pertenece al grupo de las vasculitis generalizadas, con un mecanismo patológico muy parecido al de la enfermedad de Behçet (EB), incluso considerándose como una variante de esta última. Las manifestaciones clínicas incluyen tos, disnea, fiebre, toracodinia y hemoptisis. Es fundamental realizar un diagnóstico y tratamiento de manera temprana debido a que estos pacientes presentan un alto riesgo de ruptura de los aneurismas de arteria pulmonar. El tratamiento se basa en el uso de agentes inmunosupresores.

The Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. HSS has also been considered as a variant of Behcet's disease (BD). Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. Early diagnosis and treatment are important, because patients are at a high risk for devastating pulmonary artery aneurysm rupture. Treatment usually involves immunosuppressive agents.

Massive Hemoptysis Caused by Atypical Behcet's Disease

Pulmonary and/or bronchial aneurysm with systemic thrombophlebitis can be seen in the case of both Behcet's disease and Hughes-Stovin syndrome. These two diseases may be indistinguishable since the clinical, radiological, and histopathological findings are similar in both cases. In particular, from the perspective of pulmonary involvement, hemoptysis is the leading cause of death in both conditions and is followed by aneurysmal rupture. Here, we report a case in which surgical resection was performed for a patient presenting recurrent hemoptysis as an atypical presentation of Behcet's disease.

Síndrome Hughes-Stovin: Caso clínico / Hughes-Stovin syndrome: Report of one case

We report a 25yearold mole admitted to a critical care unit for fever, lung opacities and acute respiratory failure. A chest angio-CAT sean showed multiple pulmonary artery aneurysms. A deep venous thrombosis of both lower limbs was also documented. A Hughes-Stovin syndrome was postulated. An inferior vena cava filter was placed. The patient received antimicrobial therapy subsiding fever and respiratory failure. Subsequently, he was treated with intravenous and oral steroids and one dose of cyclophosphamide. The patient was discharged in good conditions fifteen days after admission.

Cloning, bioinformatics analysis, and expression of the dust mite allergen Der f 5 of Dermatophagoides farinae

Crude extracts of house dust mites are used clinically for diagnosis and immunotherapy of allergic diseases, including bronchial asthma, perennial rhinitis, and atopic dermatitis. However, crude extracts are complexes with non-allergenic antigens and lack effective concentrations of important allergens, resulting in several side effects. Dermatophagoides farinae (Hughes; Acari: Pyroglyphidae) is one of the predominant sources of dust mite allergens, which has more than 30 groups of allergen. The cDNA coding for the group 5 allergen of D. farinae from China was cloned, sequenced and expressed. According to alignment using the VECTOR NTI 9.0 software, there were eight mismatched nucleotides in five cDNA clones resulting in seven incompatible amino acid residues, suggesting that the Der f 5 allergen might have sequence polymorphism. Bioinformatics analysis revealed that the matured Der f 5 allergen has a molecular mass of 13604.03 Da, a theoretical pI of 5.43 and is probably hydrophobic and cytoplasmic. Similarities in amino acid sequences between Der f 5 and allergens of other domestic mite species, viz. Der p 5, Blo t 5, Sui m 5, and Lep d 5, were 79, 48, 53, and 37%, respectively. Phylogenetic analysis indicated that Der f 5 and Der p 5 clustered together. Blo t 5 and Ale o 5 also clustered together, although Blomia tropicalis and Aleuroglyphus ovatus belong to different mite families, viz. Echimyopodidae and Acaridae, respectively.

Síndrome de Hughes-Stovin / Hughes-Stovin syndrome

A síndrome de Hughes-Stovin é uma condição rara, de causa desconhecida, caracterizada pela associação de múltiplos aneurismas de artéria pulmonar e trombose venosa profunda. Alguns autores consideram tal entidade como uma forma incompleta de apresentação da doença de Behçet, devido à semelhança entre os achados radiológicos e anatomopatológicos do comprometimento pulmonar. Os autores relatam um caso de síndrome de Hughes-Stovin cujo primeiro evento trombótico venoso antecedeu em cinco anos o aparecimento dos aneurismas pulmonares.

Hughes-Stovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep venous thrombosis. Some authors consider this entity an incomplete form of Behcet's disease due to the similarities between the radiologic and anatomopathological findings of pulmonary involvement. The authors report a case of Hughes-Stovin syndrome whose first venous thrombotic event preceded the development of pulmonary aneurysms by five years.

Reverse Modified Hughes Procedure for Reconstruction of Upper Eyelid

PURPOSE: The purpose of this article is to describe reverse modified Hughes procedure to reconstruct extensive full-thickness defect developed on over 75% of the upper eyelid. METHODS: We performed upper eyelid reconstruction on 4 patiets with extensive full-thickness upper eyelid defect more than 80% due to tumor resection. A tarsoconjunctival flap from a donor lower eyelid reconstituted the posterior lamella, orbicularis oculi muscle was mobilized over the tarsoconjunctival flap, and an advanced redundant cutaneous flap adjacent to the eyelid defect were used to reconstruct the anterior lamella. Tarsoconjunctival flap splitting was performed 6 weeks later. Follow up ranges from 6 months to 5 years. RESULTS: We obtained satisfactory results on all 4 patients both clinically and cosmetically. There have been no complications such as ectropion, lower-lid necrosis. CONCLUSIONS: Reverse modified Hughes procedure is an ideal option for reconstruction of extensive fullthickness upper eyelid defect.

A Case of Hughes - Stovin Syndrome / 결핵

Hughes-Stovin Syndrome is an exceedingly rare combination of distal pulmonary arterial aneurysm and deep vein thrombosis, mostly found in young patients. There are striking similarities between the vascular manifestation of Behcet's disease and Hughes-Stovin Syndrome. It has been suggested that they may have a similar pathogenesis. Most patients died of massive hemoptysis due to rupture of aneurysm. Recently we have experienced the first case of Hughes-Stovin Syndrome in Korea. A 37 year old male patient was admitted because of recurrent hemoptysis and intermittent fever. He had a history of recurrent aphthous ulcers and erythema nodosum-like skin rash, But no other findings of Behcet's disease was found. Angiography showed multiple pulmonary arterial aneurysm and deep vein thrombosis in Right lower extremity. Histologic examination of specimens of open lung biopsy revealed leukocyto- clastic angiitis. Pulmonary arterial aneurysms were successfully treated by coil embolization and he is in good condition with corticosteroid and cyclophosphamide therapy.

Relationship between Clinical Neuro-Electrophysiology and Prognosis in Children with Guillain-Barr? Syndrome / 实用儿科临床杂志

ObjectiveTo explore the relationship between the changes of neuro-electrophysiology and prognosis in children with Guillain-Barr? syndrome(GBS).MethodsThirty-eight children with GBS were divided into group A(rapid recovery,n=16) and group B(slow recovery,n=22) according to the time required for podosoma motor function recovery,at the same time,they were divided into the better prognosis group(n=22) and the worse prognosis group(n=16),for analyzing the difference between group A and B in terms of age,preceding infections,maximal Hughes grades and neuro-electrophysiology including motor conduction velocity(MCV),distal complex muscle action potential(dCMAP) and F wave,and investigating the related factors with the prognosis of GBS.Results1.MCV of tibial nerve was(40.2?2.53) m/s and(33.4?2.46) m/s in group A and group B,respectively;MCV of peroneal nerve was(45.2?3.23) m/s and(38.3?2.16) m/s in group A and group B,respectively,and the difference between group A and group B was significant(Pa0.05);abnormal rate of F wave(68.42%) was higher than abnormal rate of MCV(42.11%) and dCMAP(42.11%)(Pa