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1.
Wien Klin Wochenschr ; 2024 Apr 18.
Artigo em Inglês | MEDLINE | ID: mdl-38634909

RESUMO

The purpose of this case report is to present misdiagnosed sebaceous gland carcinoma of the lower eyelid with rapid growth in a young woman during pregnancy. Eyelid sebaceous gland carcinoma is a relatively rare tumor and a disease primarily of older patients. It occurs more commonly in oriental populations and with a predilection for the upper eyelid. Early diagnosis and appropriate treatment may help improve disease control and patient survival.

2.
Wien Klin Wochenschr ; 136(3-4): 118-121, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37966526

RESUMO

Hughes-Stovin syndrome (HSS) is a rare vasculitis of unknown etiology. The disease is characterized by pronounced inflammation and damage to the vessel walls, with subsequent widespread vascular thrombosis and the formation of pulmonary artery aneurysms that can lead to fatal hemoptysis. This disorder can be mistaken for other conditions, such as chronic thromboembolic pulmonary disease (CTEPD) without or with pulmonary hypertension at rest (CTEPH).We report the case of a 20-year-old female with HSS, which was misdiagnosed as CTEPH and subsequently treated with anticoagulants, which led to severe hemoptysis and eventually death of the patient. This case highlights the challenges of diagnosing HSS at early stages of the disease.HSS should be considered in young patients with signs of large vessel vasculitis in combination with thrombotic occlusions of pulmonary arteries, with or without aneurysms of the pulmonary arteries, and particularly, if there are no risk factors for thromboembolic disease.


Assuntos
Aneurisma , Hipertensão Pulmonar , Tromboembolia , Vasculite , Feminino , Humanos , Adulto Jovem , Adulto , Síndrome , Hemoptise/diagnóstico , Hemoptise/etiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/complicações , Diagnóstico Diferencial , Vasculite/complicações , Vasculite/diagnóstico , Artéria Pulmonar/diagnóstico por imagem , Aneurisma/complicações , Aneurisma/diagnóstico
3.
Revista argentina de cirugia plastica ; 29(2): 122-130, 20230000.
Artigo em Espanhol | BINACIS | ID: biblio-1523060

RESUMO

Introducción. Los párpados son estructuras delicadas, compuestas por piel fina y cartílago (tarso), las cuales, al presentar una lesión, son de difícil reconstrucción, debido a su importante papel estructural, funcional y estético, como así también la delicadeza de las estructuras que la componen1. En cuanto a las pérdidas de sustancia que asientan en el párpado inferior, existen diversas técnicas para intentar reestablecer su anatomía. Entre ellas se encuentra la ampliamente conocida y utilizada técnica de Hughes, descripta en 1937, que consiste en crear, desde el párpado superior, un colgajo de lamela posterior, compuesto de un segmento de tarso unido a la conjuntiva, las cuales se suturan al párpado inferior, al que hay que asociar otro tipo de procedimiento para formar la lamela anterior, ya sea un injerto cutáneo libre o un colgajo de piel adyacente al defecto2. Fue modificada en 1982 por McCord y Nunery, quienes plantean dejar un margen de 4 mm del borde libre palpebral, para no debilitar el párpado superior5. Su indicación principal son los defectos mayores al 50-60% de la longitud palpebral horizontal, con cantos intactos3, aunque se puede combinar con otro tipo de técnicas que reparen los defectos cantales1. Objetivo. Demostrar la versatilidad y efectividad del colgajo tarsoconjuntival de Hughes para la reconstrucción de defectos de cobertura asentados en el párpado inferior. Descripción de la técnica "paso a paso", resaltando tips quirúrgicos para mejorar resultados. Materiales y métodos. Estudio descriptivo retrospectivo, en el cual se intervinieron 5 pacientes con afección de párpado inferior, en el Parque de la Salud, Posadas, Misiones, Argentina, entre julio del 2019 y febrero del 2022, de los cuales 3 fueron con anestesia local (casos clínicos 1, 2 y 4) en quirófano ambulatorio, y dados de alta luego del procedimiento, y el resto con anestesia general y dados de alta al día siguiente (casos clínicos 3 y 5). Resultados. Se analizaron 5 pacientes, 1 mujer (20%) y 4 hombres (80%), con un rango etario que fue desde los 39 hasta los 70 años (media de 56 años). Con respecto al diagnóstico, 3 fueron reconstrucciones inmediatas posresección de carcinomas basocelulares con cirugía micrográfica de Mohs (2 variante lobulado, y 1 macronodular) y a los restantes 2 pacientes se los realizó como método reconstructivo pos ectropión cicatrizal (de los cuales uno tenía antecedentes de resección de carcinoma basocelular palpebral y el otro un dermatofibrosarcoma en mejilla). Considerando el segundo paso quirúrgico, es decir la autonomización, se realizaron en 2 casos a los 21 días, y los 3 restantes próximos a cumplir el mes (media 25 días). El seguimiento posoperatorio varía desde el año hasta los 36 meses posoperatorios, con una media de 22,6 meses. Discusión. El colgajo tarsoconjuntival de Hughes es un colgajo versátil, que requiere una curva de aprendizaje corta, el cual está indicado para defectos del párpado inferior que no se extienden mucho más allá del borde inferior de la placa tarsal. Para el mismo se utiliza una tira ancha de placa tarsal superior sobre un pedículo de conjuntiva y posteriormente, para reconstruir la lamela posterior del párpado inferior, se puede utilizar un nuevo colgajo de tejidos adyacentes del párpado inferior o un autoinjerto de piel, para el cual la mejor opción por la similitud de los tejidos es la piel del párpado superior contralateral. Conclusiones. El colgajo de Hughes es una técnica que proporciona resultados estéticos y funcionales muy satisfactorios. Sus principales limitaciones son que requiere procedimientos asociados para reestablecer la lamela anterior (injertos o colgajos), y otra intervención posterior para autonomizar el colgajo a las dos semanas y reformar el nuevo borde libre palpebral, considerando además la incomodidad que representa la oclusión ocular que requiere hasta su autonomización. En esta serie de casos la lesión cutánea más frecuente fue el carcinoma basocelular, y a 4 de ellas se les realizó cirugía micrográfica de Mohs, de los cuales no hubo recidiva hasta la actualidad, lo cual demuestra además la efectividad de este método para tratar esta clase de tumores en zonas faciales especiales


Introduction. The eyelids are delicate structures, composed of thin skin and cartilage (tarsus), which, when presenting an injury, are difficult to reconstruct, due to their important structural, functional and aesthetic role, as well as the delicacy of their structures that compose1. Regarding the loss of substance that settles in the lower eyelid, there are various techniques to try to reestablish its anatomy, among them is the widely known and used Hughes Technique, described in 1937, which consists of creating, from the upper eyelid , a flap of the posterior lamella, composed of a tarsal segment attached to the conjunctiva, which are sutured to the lower eyelid, to which another type of procedure must be associated to form the anterior lamella, either a free skin graft or a flap of skin adjacent to the defect2. It was later modified in 1982, by McCord and Nunery, who propose leaving a 4 mm margin from the eyelid free edge, so as not to weaken the upper eyelid5. Its main indication is defects greater than 50-60% of the horizontal palpebral length, with intact edges3, although it can be combined with other types of techniques that repair canthal defects1 Objective. To demonstrate the versatility and effectiveness of the Hughes tarsoconjunctival flap for the reconstruction of coverage defects located in the lower eyelid. Description of the "step by step" technique, highlighting surgical tips to improve results. Materials and methods. This is a retrospective descriptive study, in which a total of 5 patients with lower eyelid disease were operated on, in the Parque de la Salud, Posadas, Misiones, Argentine, between July 2019 and February 2022, of which 3 patients were with local anesthesia (Clinical Cases 1, 2 and 4) in the ambulatory operating room, and discharged after the procedure, and the rest underwent general anesthesia and discharged the next day (Clinical Cases 3 and 5). Results. A total of 5 patients were analyzed, 1 woman (20%) and 4 men (80%), with an age range from 39 to 70 years (mean 56 years). Regarding the diagnosis, 3 were immediate reconstructions after resection of Basal Cell Carcinomas with Mohs Micrographic Surgery (2 lobulated and 1 macronodular variants) and the remaining 2 patients underwent post-scarring ectropion reconstructive method (of which one had a history of resection). Basal Cell Carcinoma of the eyelid and the other a Dermatofibrosarcoma on the cheek). Considering the second surgical step, that is, autonomization, 2 cases were performed at 21 days, and the remaining 3 were close to a month old (mean 25 days). Postoperative follow-up varies from one year to 36 postoperative months, with a mean of 22.6 months. Discussion. The Hughes tarsoconjunctival flap is a versatile flap, requiring a short learning curve, which is indicated for lower lid defects that do not extend much beyond the lower border of the tarsal plate. For this, a wide strip of the upper tarsal plate is used on a conjunctival pedicle, and later, to reconstruct the posterior lamella of the lower eyelid, a new flap of adjacent tissues from the lower eyelid or a skin autograft can be used, for which the best option due to the similarity of the tissues is the skin of the contralateral upper eyelid. Conclusions. The Hughes flap is a technique that provides very satisfactory aesthetic and functional results. Its main limitations are that it requires associated procedures to reestablish the anterior lamella (grafts or flaps), and another subsequent intervention to autonomize the flap after two weeks and reform the new eyelid free edge, also considering the discomfort represented by ocular occlusion that requires even its autonomy. In this series of cases, the most frequent skin lesion was Basal Cell Carcinoma, and in 4 of them Mohs micrographic surgery was performed, of which there has been no recurrence to date, which also demonstrates the effectiveness of this method to treat this class of tumors in special facial areas.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Cuidados Pós-Operatórios , Procedimentos Cirúrgicos Operatórios/métodos , Retalhos Cirúrgicos/transplante , Pálpebras/cirurgia
4.
Cureus ; 15(4): e37121, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37153244

RESUMO

Hughes-Stovin Syndrome (HSS) is a rare clinical condition characterized by thrombophlebitis as well as multiple pulmonary and bronchial aneurysms. It commonly presents with coughing, dyspnea, fever, chest pain, and hemoptysis, and its management usually consists of surgical and medical approaches. In this report, we discuss a case of a patient with HSS. A 30-year-old male patient was admitted to the pulmonary medicine ward for hemoptysis. After evaluation with chest CT, bilateral pulmonary embolism and pulmonary aneurysms were observed. Due to a history of aphthous lesions, Behçet's disease (BD) was considered the initial diagnosis; however, the patient did not fit the criteria and was later diagnosed with HSS. Intravenous methylprednisolone was initiated, along with a maintenance treatment with cyclophosphamide. Treatment response was observed in the fourth month; however, due to the persistence of hemoptysis, additional cycles of cyclophosphamide were later required, under which the patient's condition has been stable. HSS currently lacks clear diagnostic criteria, and further studies are needed to investigate genetic backgrounds, familial transmissions, and treatment alternatives.

6.
J Hist Neurosci ; 32(4): 470-490, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37199685

RESUMO

Efforts to treat epileptic seizures likely date back to primitive, manmade skull openings or trephinations at the site of previous scalp or skull injuries. The purpose may have been the release of "evil spirits," removal of "cerebral excitement," and "restoral of bodily and intellectual functions." With progressive discoveries in brain function over the past 100 to 300 years, the cerebral cortical locations enabling voluntary movements, sensation, and speech have been well delineated. The locations of these functions have become surgical targets for the amelioration of disease processes. Disease entities in particular cerebral-cortical areas may predispose to the onset of focal and or generalized seizures, which secondarily interfere with normal cortical functioning. Modern neuroimaging and electroencephalography usually delineate the location of seizures and often the type of structural pathology. If noneloquent brain regions are involved, open surgical biopsy or removal of only abnormal tissue may be undertaken successfully. A number of the early neurosurgical pioneers in the development of epilepsy surgery are credited and discussed in this article.


Assuntos
Epilepsia , Neurocirurgia , Humanos , Epilepsia/cirurgia , Procedimentos Neurocirúrgicos , Encéfalo/cirurgia , Convulsões , Eletroencefalografia
7.
Artigo em Inglês | MEDLINE | ID: mdl-37200981

RESUMO

Three young males with Hugh-Stovin's syndrome presented with cough, haemoptysis, fever, raised inflammatory markers, and pulmonary artery aneurysm. Only one had recurrent oral ulcers suggestive of Behcet's disease, and none were HLA B51 positive. All responded well to immunosuppression but eventually needed either an endovascular procedure or surgery.

8.
J Investig Med High Impact Case Rep ; 11: 23247096231166672, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37032536

RESUMO

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.


Assuntos
Falso Aneurisma , Aneurisma , Trombose , Vasculite , Masculino , Humanos , Adolescente , Falso Aneurisma/complicações , Falso Aneurisma/terapia , Síndrome , Artéria Pulmonar , Aneurisma/complicações , Aneurisma/diagnóstico , Vasculite/complicações , Trombose/tratamento farmacológico , Trombose/etiologia
9.
Int J Mol Sci ; 24(4)2023 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-36834577

RESUMO

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The etiology and pathogenesis of HSS are incompletely known. The current consensus is that vasculitis underlies the pathogenic process, and pulmonary thrombosis follows arterial wall inflammation. As such, Hughes-Stovin syndrome may belong to the vascular cluster with lung involvement of Behçet syndrome, although oral aphtae, arthritis, and uveitis are rarely found. Behçet syndrome is a multifactorial polygenic disease with genetic, epigenetic, environmental, and mostly immunological contributors. The different Behçet syndrome phenotypes are presumably based upon different genetic determinants involving more than one pathogenic pathway. Hughes-Stovin syndrome may have common pathways with fibromuscular dysplasias and other diseases evolving with vascular aneurysms. We describe a Hughes-Stovin syndrome case fulfilling the Behçet syndrome criteria. A MYLK variant of unknown significance was detected, along with other heterozygous mutations in genes that may impact angiogenesis pathways. We discuss the possible involvement of these genetic findings, as well as other potential common determinants of Behçet/Hughes-Stovin syndrome and aneurysms in vascular Behçet syndrome. Recent advances in diagnostic techniques, including genetic testing, could help diagnose a specific Behçet syndrome subtype and other associated conditions to personalize the disease management.


Assuntos
Aneurisma , Síndrome de Behçet , Vasculite , Humanos , Aneurisma/complicações , Aneurisma/diagnóstico , Aneurisma/patologia , Síndrome de Behçet/diagnóstico , Artéria Pulmonar/patologia , Vasculite/patologia
10.
Lupus ; 32(3): 319-324, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36708185

RESUMO

BACKGROUND: Headache, often migrainous, is common in patients with antiphospholipid antibodies, whether or not they meet Sydney criteria for a definite diagnosis of Hughes syndrome. Migraine may be a harbinger of stroke in this patient population and even refractory migraine may be highly responsive to antithrombotic therapy in this clinical context. PURPOSE: To summarize what is known to date about managing this important manifestation of the immune-mediated hypercoagulable Hughes syndrome. RESULTS: We provide a suggested management algorithm for refractory headache in this unique patient population. CONCLUSION: Most neurologists don't see or recognize many aPL-positive patients in their practice, so hematologists and rheumatologists who see these patients should recognize that refractory headache may be a manifestation of their immune-mediated hypercoagulable disorder and understand that the potential risks of not addressing this issue may be high.


Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Transtornos de Enxaqueca , Humanos , Síndrome Antifosfolipídica/diagnóstico , Anticorpos Antifosfolipídeos/uso terapêutico , Cefaleia
11.
Psychol Med ; 53(9): 4294-4295, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-35012695

RESUMO

After reading an article in the journal, regarding affective disorders in patients with rare illnesses, the authors would like to discuss a case of non-affective psychosis, presenting with olfactory reference and Truman symptoms, in a patient with three unusual conditions: Gilbert disease, Hughes syndrome and Lyme neuroborreliosis.


Assuntos
Doença de Gilbert , Neuroborreliose de Lyme , Humanos , Neuroborreliose de Lyme/complicações , Neuroborreliose de Lyme/diagnóstico , Doença de Gilbert/complicações , Transtornos do Humor/complicações , Anticorpos Antifosfolipídeos
12.
Environ Sci Pollut Res Int ; 30(3): 5580-5602, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36434463

RESUMO

Hyperspectral image (HSI) contains hundreds of adjacent spectral bands, which can effectively differentiate the region of interest. Nevertheless, many irrelevant and highly correlated spectral bands lead to the Hughes phenomenon. Consequently, hyperspectral image dimensionality reduction is necessary to select the most informative and significant spectral band and eliminate the redundant spectral band. To this end, this paper represents an extensive and systematic survey of hyperspectral dimensionality reduction approaches for land use land cover (LULC) classification. Moreover, this paper reviewed the following important points: (1) hyperspectral imaging data acquisition methods, (2) the difference between hyperspectral and multispectral images, (3) hyperspectral image dimensionality reduction based on machine learning (ML) and deep learning (DL) techniques, (4) the popular benchmark hyperspectral datasets with the performance metrics for LULC classification, and (5) the significant challenges with the future trends for hyperspectral dimensionality reduction.


Assuntos
Benchmarking , Aprendizado de Máquina
13.
Sensors (Basel) ; 22(24)2022 Dec 10.
Artigo em Inglês | MEDLINE | ID: mdl-36560046

RESUMO

With the development of deep learning, the use of convolutional neural networks (CNN) to improve the land cover classification accuracy of hyperspectral remote sensing images (HSRSI) has become a research hotspot. In HSRSI semantics segmentation, the traditional dataset partition method may cause information leakage, which poses challenges for a fair comparison between models. The performance of the model based on "convolutional-pooling-fully connected" structure is limited by small sample sizes and high dimensions of HSRSI. Moreover, most current studies did not involve how to choose the number of principal components with the application of the principal component analysis (PCA) to reduce dimensionality. To overcome the above challenges, firstly, the non-overlapping sliding window strategy combined with the judgment mechanism is introduced, used to split the hyperspectral dataset. Then, a PSE-UNet model for HSRSI semantic segmentation is designed by combining PCA, the attention mechanism, and UNet, and the factors affecting the performance of PSE-UNet are analyzed. Finally, the cumulative variance contribution rate (CVCR) is introduced as a dimensionality reduction metric of PCA to study the Hughes phenomenon. The experimental results with the Salinas dataset show that the PSE-UNet is superior to other semantic segmentation algorithms and the results can provide a reference for HSRSI semantic segmentation.


Assuntos
Artrópodes , Imageamento Hiperespectral , Animais , Semântica , Algoritmos , Julgamento , Processamento de Imagem Assistida por Computador
14.
Front Glob Womens Health ; 3: 1031190, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36505012

RESUMO

Historically, women with an autoimmune disease (AD) could not get pregnant due to infertility frequently linked to the medical condition or because the pregnancy was contraindicated, as it could harm the mother and the future child. Sometimes, pregnancy was contraindicated because the medication needed to control the AD could not be given during pregnancy. All these items are no longer true nowadays. Fertility treatments have advanced, obstetric care is better, and the medical treatments of autoimmune diseases have progressed, so women with any kind of AD are encouraged to get pregnant, and their presence in obstetric clinics is arising. This is challenging for the obstetricians, as to be sure that these pregnancies are safe for the mother and the future child, the obstetricians need to know the natural evolution of these conditions, the impact of pregnancy and postpartum on the illness, and the impact of the AD in the pregnancy. In this narrative review, we aim to make a brief resume of the obstetric management of the most common diseases (Systemic lupus erythematosus, antiphospholipid syndrome, the Anti-Ro/SSA and Anti-La/SSB antigen-antibody systems, rheumatoid arthritis, Sjögren's syndrome and Undifferentiated systemic rheumatic disease and overlap syndromes).

15.
Cureus ; 14(10): e29919, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36348871

RESUMO

Guillain-Barre syndrome (GBS) is a rare inflammatory demyelinating polyradiculoneuropathy characterized by motor impairment, progressive, ascending, symmetrical flaccid limb paralysis, areflexia or hyporeflexia, and with or without cranial nerve involvement, which are the hallmark clinical indications of GBS, which can last over weeks to months. Miller-Fisher syndrome (MFS) is a post-infectious localized variant of GBS that includes ophthalmoplegia, ataxia, and areflexia, and is often associated with lower cranial and facial nerve involvement. In this case, a 22-year-old young man was taken to a hospital after 10 days with complaints of bilateral symmetrical upper extremity and lower extremity paralysis, with the legs being more afflicted than the arms. For the past six days, he had an episode of fever, slurred speech, bilateral eye drops, and swallowing difficulty. On examination, the patient was identified with MFS, a variant of GBS. On the first and last day of treatment, the patient's outcome measures were recorded on Manual Muscle Testing, Hughes (GBS disability score), and the Functional Independence Measure Scale. Treatment options have been shown to reduce challenges and improve patient outcomes and quality of life, all of which are important at this stage. This case study concluded with a rehabilitation program that helped the patient to enhance his strength, range of motion, functional mobility, postural control, balancing abilities, weight-bearing, and prevent secondary impairments.

16.
J Med Food ; 25(12): 1112-1125, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36445749

RESUMO

Diabetic patients are more prone to developing nonalcoholic fatty liver disease (NAFLD) compared with healthy people. As a plant homologous to both medicine and food, Malus toringoides (Rehd.) Hughes has been used as an intervention for both NAFLD and diabetes. However, the effect and mechanism of M. toringoides on NAFLD on type 2 diabetes mellitus (T2DM) is unclear. The current investigation was designed to evaluate the ameliorative effects and mechanism of M. toringoides ethanol extract (CBTM-E375) on T2DM, and to identify the compounds in these extracts. The effects of CBTM-E375 on T2DM were verified using a high-fat diet-/streptozotocin-induced diabetic rat and free fatty acid (0.5 mM)-induced human hepatocellular carcinoma cell (HepG2) models. The components of CBTM-E375 were identified by high performance liquid chromatography-mass spectrometry/mass spectrometry. Our results demonstrate that CBTM-E375 ameliorated lipid accumulation (total cholesterol, triglyceride), oxidative stress (superoxide dismutase, catalase, malondialdehyde, glutathione peroxidase), and inflammation (tumor necrosis factor-α [TNF-α], interleukin [IL]-1ß, IL-6, C-reactive protein [CRP]) in vivo and in vitro, these effects were associated with a CBTM-E375-mediated downregulation of SREBP-1c (sterol regulatory element binding protein 1c) and the NF-κB (nuclear factor κB) signaling pathway. A total of 20 chemical compounds were identified in CBTM-E375, including phlorizin, isoquercitrin, chlorogenic acid, quercetin, naringenin, and trigonelline, which have been reported to have positive effects on diabetes or on NAFLD.


Assuntos
Diabetes Mellitus Tipo 2 , Malus , Hepatopatia Gordurosa não Alcoólica , Humanos , Ratos , Animais , Hepatopatia Gordurosa não Alcoólica/tratamento farmacológico , Hepatopatia Gordurosa não Alcoólica/genética , Hepatopatia Gordurosa não Alcoólica/metabolismo , Proteína de Ligação a Elemento Regulador de Esterol 1/genética , Proteína de Ligação a Elemento Regulador de Esterol 1/metabolismo , NF-kappa B/genética , NF-kappa B/metabolismo , Malus/metabolismo , Regulação para Baixo , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/tratamento farmacológico , Diabetes Mellitus Tipo 2/genética , Fígado/metabolismo , Metabolismo dos Lipídeos
17.
Rev. argent. reumatolg. (En línea) ; 33(4): 232-234, oct. 2022. graf
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1449429

RESUMO

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.


Assuntos
Masculino
18.
Front Neurol ; 13: 972367, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36071910

RESUMO

Introduction: To study cerebrospinal fluid neurofilament light chain (CSF-NfL) levels as a prognostic biomarker in pediatric Guillain-Barré syndrome (GBS). Methods: Prospective study enrolling 26 pediatric GBS patients and 48 healthy controls (HCs) from neurology units between 2017 to 2021. The CSF-NfL levels were measured by enzyme-linked immunosorbent assay. The children's disability levels were evaluated using Hughes Functional Score (HFS) at nadir, 1 month, and 6 months after onset. The receiver operating characteristic (ROC) curve derived from logistic regression (with age as a covariate) was used to assess the prognostic value of CSF-NfL on the possibility of walking aided at 1 month after symptom onset. Results: The mean CSF-NfL levels were significantly increased in GBS patients (111.76 pg/mL) as compared to that in HCs (76.82 pg/mL) (t = 6.754, p < 0.001). At follow- up, the mean CSF-NfL levels after treatment (65.69 pg/mL) declined significantly (t = 6.693, p < 0.001). CSF-NfL levels upon admission were significantly associated with the HFS at nadir (r s = 0.461, p = 0.018). Moreover, the mean CSF-NfL levels in GBS patients with poor prognosis (130.47pg/mL) were significantly higher than that in patients with good prognosis (104.87pg/mL) (t = 2.399, p = 0.025). ROC curve analysis of the predictive value of CSF-NfL levels with respect to the inability to walk unaided within 1 month showed a significant difference (area under the curve: 0.857,95% confidence interval 0.702-1.000; p = 0.006). Conclusion: CSF-NfL levels were increased in pediatric GBS patients. High CSF-NfL level predicted worse motor function, and was strongly associated with poor short-term prognosis of pediatric GBS. We propose a biomarker for early prediction of outcome in pediatric GBS, which would be applicable for clinical practice and efficacy of treatment in the future.

19.
Health Technol Assess ; 26(34): 1-100, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35938554

RESUMO

BACKGROUND: Incisional hernias can cause chronic pain and complications and affect quality of life. Surgical repair requires health-care resources and has a significant associated failure rate. A prospective, multicentre, single-blinded randomised controlled trial was conducted to investigate the clinical effectiveness and cost-effectiveness of the Hughes abdominal closure method compared with standard mass closure following surgery for colorectal cancer. The study randomised, in a 1 : 1 ratio, 802 adult patients (aged ≥ 18 years) undergoing surgical resection for colorectal cancer from 28 surgical departments in UK centres. INTERVENTION: Hughes abdominal closure or standard mass closure. MAIN OUTCOME MEASURES: The primary outcome was the incidence of incisional hernias at 1 year, as assessed by clinical examination. Within-trial cost-effectiveness and cost-utility analyses over 1 year were conducted from an NHS and a social care perspective. A key secondary outcome was quality of life, and other outcomes included the incidence of incisional hernias as detected by computed tomography scanning. RESULTS: The incidence of incisional hernia at 1-year clinical examination was 50 (14.8%) in the Hughes abdominal closure arm compared with 57 (17.1%) in the standard mass closure arm (odds ratio 0.84, 95% confidence interval 0.55 to 1.27; p = 0.4). In year 2, the incidence of incisional hernia was 78 (28.7%) in the Hughes abdominal closure arm compared with 84 (31.8%) in the standard mass closure arm (odds ratio 0.86, 95% confidence interval 0.59 to 1.25; p = 0.43). Computed tomography scanning identified a total of 301 incisional hernias across both arms, compared with 100 identified by clinical examination at the 1-year follow-up. Computed tomography scanning missed 16 incisional hernias that were picked up by clinical examination. Hughes abdominal closure was found to be less cost-effective than standard mass closure. The mean incremental cost for patients undergoing Hughes abdominal closure was £616.45 (95% confidence interval -£699.56 to £1932.47; p = 0.3580). Quality of life did not differ significantly between the study arms at any time point. LIMITATIONS: As this was a pragmatic trial, the control arm allowed surgeon discretion in the approach to standard mass closure, introducing variability in the techniques and equipment used. Intraoperative randomisation may result in a loss of equipoise for some surgeons. Follow-up was limited to 2 years, which may not have been enough time to see a difference in the primary outcome. CONCLUSIONS: Hughes abdominal closure did not significantly reduce the incidence of incisional hernias detected by clinical examination and was less cost-effective at 1 year than standard mass closure in colorectal cancer patients. Computed tomography scanning may be more effective at identifying incisional hernias than clinical examination, but the clinical benefit of this needs further research. FUTURE WORK: An extended follow-up using routinely collected NHS data sets aims to report on incisional hernia rates at 2-5 years post surgery to investigate any potential mortality benefit of the closure methods. Furthermore, the proportion of incisional hernias identified by a computed tomography scan (at 1 and 2 years post surgery), but not during clinical examination (occult hernias), proceeding to surgical repair within 3-5 years after the initial operation will be explored. TRIAL REGISTRATION: This trial is registered as ISRCTN25616490. FUNDING: This project was funded by the National Institute for Health and Care Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 26, No. 34. See the NIHR Journals Library website for further project information.


Bowel cancer treatment involves surgery for the majority of patients. A complication of this surgery is the formation of a hernia at the site of the incision in the abdominal wall, known as an incisional hernia. The lining of the abdomen, fat or the intestine can squeeze through the gap and form a lump under the skin. An incisional hernia can form any time after surgery and can cause serious complications and pain, and can also affect the patient's quality of life. Surgery to correct incisional hernias is not always successful, so finding a way of preventing them is important. This research compares the traditional way of sewing up the abdomen, where the two sides are brought together in one layer with a continuous thread, with an alternative method called the Hughes abdominal closure method/Hughes repair. In the Hughes repair, a series of horizontal and vertical stitches are arranged to spread the load and ease the tension across the wound. A total of 802 patients from 28 sites in the UK were recruited to the trial. Half of the patients were randomly allocated to have traditional abdominal closure and half were randomised to have Hughes abdominal closure. All were followed up for 1 year after surgery to assess whether or not an incisional hernia had occurred. We also assessed quality of life during follow-up, and we compared the costs and benefits of each procedure to see which option was the better value for money. By comparing the results from the two methods, it was hoped that the best method of abdominal closure to reduce the risk of an incisional hernia occurring would be found. The analysis of the data suggested that the risk of an incisional hernia was no different with either closure method. Furthermore, Hughes abdominal closure was more expensive and provided less value for money than standard abdominal closure.


Assuntos
Neoplasias Colorretais , Hérnia Incisional , Adulto , Neoplasias Colorretais/cirurgia , Análise Custo-Benefício , Humanos , Hérnia Incisional/epidemiologia , Hérnia Incisional/prevenção & controle , Estudos Prospectivos , Qualidade de Vida
20.
Am J Ophthalmol Case Rep ; 27: 101667, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35959476

RESUMO

Purpose: The reconstruction of near-total upper eyelid defects is challenging and complicated. There are multiple possible techniques, including multi-stage lower eyelid flaps (such as Mustarde's lid-switch flap and the Cutler-Beard techniques) as well as single-stage techniques with free grafts. Here we present two patients requiring near-total upper eyelid repairs that were accomplished by a single-eyelid, single-stage technique using a tarsoconjunctival flap. Observations: Two cases of near-total upper eyelid defects are described, one secondary to resection of a basal cell carcinoma and the other secondary to resection of a Merkel cell carcinoma. Both cases had sufficient residual tarsus to supply a single-eyelid tarsoconjunctival flap. Results were excellent. Conclusions: When there is sufficient tarsus remaining, the illustrated technique provides an excellent repair of near-total upper-eyelid defects. It is a simpler procedure than its alternatives, spares other tissue sites, eliminates free grafts, and does not require multiple stages.

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