RESUMO
INTRODUÇÃO A endomiocardiofibrose (EMF) é uma cardiomiopatia restritiva, caracterizada por envolvimento fibrótico do endocárdio e miocárdio normalmente na região apical, levando a disfunção diastólica, porém, preservando função sistólica. É uma doença de etiologia desconhecida e ainda muito relacionada a países subdesenvolvidos com forte relação às síndromes hipereosinofilicas. Seu diagnóstico pode ser aventado através do ecodopplercardiograma e ressonância magnética cardíaca, podendo muitas vezes ser confundida com trombo em região apical ventricular. DESCRIÇÃO DO CASO Paciente de 38 anos, feminina, proveniente de São Paulo capital. Relata internação anterior há 4 meses em serviço externo por quadro de dor torácica com alteração de enzimas cardíacas, porém, sem evidência de coronariopatia isquêmica na Cinecoronariografia. Evidenciado em ecocardiograma transtorácico (ECOTT) uma hipertrofia excêntrica do ventrículo esquerdo (VE) com imagem hiperecogênica em região apical de 19mm podendo corresponder a trombo. Prosseguindo a investigação, realizada uma ressonância magnética cardíaca descrevendo trombo aderido à parede do ventrículo esquerdo, medindo 2,3 cm, além de hipocinesia dos segmentos apicais e realce miocárdico tardio subendocárdico não transmural nos segmentos apicais. A mesma comparece ao serviço com novo episódio de dispneia e dor precordial, agora com sinais de hipervolemia e hipereosinofilia (>1500). Em novo ECOTT realizado evidenciou-se aumento biatrial, disfunção sistólica do ventrículo direito, hipertensão pulmonar, refluxo de grau importante em valva mitral e tricúspide associado a uma obliteração do ápice em parede endocárdica até base do VE, poupando demais paredes, sugestivo de EMF. Com base nos exames iniciais, configurou-se o diagnóstico de EMF pela ressonância magnética e sua correlação com hipereosinofilia. Dessa forma, após o diagnóstico a paciente evolui com melhora clínica e seguirá tratamento em ambulatório do miocárdio. CONCLUSÕES Relatamos um caso de EMF associada a hipereosinofilia ainda em investigação etiológica. O caso supracitado traz uma paciente jovem proveniente de um país em desenvolvimento com diagnóstico de uma doença ainda pouco compreendida e de difícil diagnóstico, podendo muitas vezes passar-se como um trombo em região apical cardíaca, gerando subdiagnósticos e manejo incorreto.
Assuntos
Humanos , Feminino , Adulto , Fibrose Endomiocárdica/diagnóstico , Eosinofilia , Dor no Peito , Cardiomiopatia Restritiva , Espectroscopia de Ressonância Magnética , DispneiaRESUMO
El síndrome de Wells o celulitis eosinofílica es una enfermedad inflamatoria de origen desconocido, de aparición infrecuente en la edad pediátrica. Suele manifestarse clínicamente como placas eritematoedematosas, nódulos, pápulas, ampollas, entre otros. Se presenta una paciente en edad pediátrica con nódulos subcutáneos asintomáticos generalizados asociados a eosinofilia grave. El estudio histopatológico de las lesiones fue compatible con celulitis de Wells. Se realizó una evaluación interdisciplinaria en busca de la causa y trastornos eosinofílicos asociados, sin resultados positivos. Se indicó tratamiento sistémico con corticoides y presentó buena respuesta, pero, ante la recidiva de las lesiones tras su suspensión, se indicó dapsona como tratamiento de segunda línea, con mejoría posterior de las lesiones y de la eosinofilia. El objetivo del reporte es presentar una paciente con una manifestación atípica de síndrome de Wells y su desafío terapéutico.
Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge.
Assuntos
Humanos , Feminino , Pré-Escolar , Celulite (Flegmão)/diagnóstico , Eosinofilia/diagnósticoRESUMO
OBJECTIVE: An ethmoid-dominant shadow on computed tomography is an indicator of type 2 inflammation, and is one of the main items used to diagnose and classify the severity of eosinophilic chronic rhinosinusitis in the Japanese diagnostic criteria. Ethmoid sinus dominance is examined using the Lund-Mackay scoring system and may be overestimated due to scoring characteristics. We aim to investigate the accuracy of evaluations of ethmoid dominance using the conventional scoring system and the possibility of conducting an objective evaluation using a more detailed other scoring system. METHODS: Patients diagnosed with eosinophilic chronic rhinosinusitis and who underwent bilateral endoscopic sinus surgery were enrolled in the present study. Computed tomography was performed preoperatively on all subjects. The bilateral anterior and posterior ethmoid sinuses and bilateral maxillary sinus were scored, and the ethmoid-to-maxillary ratio was calculated using 3 different scoring systems: Lund-Mackay (each sinus score ranges between 0 and 2), simplified Zinreich (score ranging between 0 and 3), and Zinreich (score ranging between 0 and 5). RESULTS: A total of 149 patients were eligible for the present study. Significant differences were observed in ethmoid-to-maxillary ratio evaluated by the 3 different scoring systems (2.4⯱â¯0.7, 3.0⯱â¯1.1, and 3.7⯱â¯2.2). Only 2 patients were negative for ethmoid dominance by the Lund-Mackay scoring system, while 14 were negative by the simplified-Zinreich and Zinreich scoring systems. Severity changed from the initial grade in 12 patients. CONCLUSIONS: The present results confirmed a potential overestimation when only the Lund-Mackay scoring system was used to assess ethmoid dominance. Ethmoid dominance has been identified as one of the main predictive factors for the long-term postoperative outcomes of eosinophilic chronic rhinosinusitis and is included in the Japanese diagnostic criteria. A detailed evaluation of ethmoid dominance is desirable for more accurate evaluations of the severity and prognosis of eosinophilic chronic rhinosinusitis.
Assuntos
Seio Etmoidal , Rinite , Sinusite , Tomografia Computadorizada por Raios X , Humanos , Seio Etmoidal/diagnóstico por imagem , Doença Crônica , Feminino , Masculino , Sinusite/diagnóstico por imagem , Sinusite/cirurgia , Pessoa de Meia-Idade , Rinite/diagnóstico por imagem , Rinite/cirurgia , Adulto , Índice de Gravidade de Doença , Idoso , Eosinofilia/diagnóstico por imagem , Adulto Jovem , Endoscopia , Reprodutibilidade dos Testes , Adolescente , RinossinusiteAssuntos
Esofagite Eosinofílica , Hipersensibilidade a Leite , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Eosinofilia/imunologia , Esofagite Eosinofílica/imunologia , Esofagite Eosinofílica/diagnóstico , Esôfago/patologia , Esôfago/imunologia , Seguimentos , Hipersensibilidade a Leite/imunologia , Hipersensibilidade a Leite/diagnóstico , Hipersensibilidade a Leite/complicaçõesRESUMO
In solid tumors, hypereosinophilia is a rare phenomenon and is mainly associated with mucin-secreting carcinomas. Thyroid tumors associated with neutrophilia and/or eosinophilia have been described exclusively in patients with anaplastic thyroid cancer. Eosinophilia associated with papillary thyroid cancer is extremely rare and there are very few cases currently described. It has been suggested that three cytokines, namely interleukin-3 (IL-3), interleukin-5 (IL-5), and granulocyte-macrophage colony-stimulating factor (GM-CSF), may act as a peptide potential eosinophilic. To date, only three patients with differentiated thyroid cancer associated with eosinophilia have been reported, two of the papillary type and one of the medullary type. A 48-year-old patient consulted in 2022 due to bilateral cervical lymphadenopathy of 3 years' duration associated with wasting syndrome and hypereosinophilia. PET CT was requested, which showed hypermetabolic focus in the right thyroid lobe and lymph node, lung, bone, and liver metastases; Thyroid ultrasound showing a nodule of high suspicion of malignancy and a conglomerate of lymphadenopathy in the right lobe with positive needle wash for thyroglobulin. Hypereosinophilia was evaluated with initial leukocytosis values of GB 30,310/mm3 (10,608/mm3 of eosinophils) to maximum values of GB 77,090/mm3 (eosinophils 20,814/mm3). It was interpreted as paraneoplastic syndrome and corticosteroid therapy was started at immunosuppressive doses without response. Our observations presented in this article are in line with most studies reflecting that paraneoplastic hypereosinophilia is characterized by more advanced disease and poor prognosis.
En los tumores sólidos la hipereosinofilia es un fenómeno raro y se asocia principalmente con carcinomas secretores de mucina. Los tumores tiroideos asociados a neutrofilia y/o eosinofilia se han descrito exclusivamente en pacientes con cáncer anaplásico de tiroides. La eosinofilia asociada con cáncer papilar de tiroides es extremadamente rara y se encuentran muy pocos casos descriptos actualmente. Se ha sugerido que tres citocinas, a saber, la interleucina-3 (IL-3), la interleucina-5 (IL-5) y el factor estimulante de colonias de granulocitos y macrófagos (GM-CSF), pueden actuar como un péptido eosinofílico potencial. Hasta el momento solo se han reportado tres pacientes con cáncer diferenciado de tiroides asociados a eosinofilia, dos de tipo papilar y uno de tipo medular. Paciente de 48 años consultó en el año 2022 por adenopatías cervicales bilaterales de 3 años de evolución asociado a síndrome consuntivo e hipereosinofilia. Se solicitó PET CT que evidenció foco hipermetabólico en lóbulo tiroideo derecho y metástasis ganglionares, pulmonares, óseas y hepáticas; ecografía tiroidea que evidencia en lóbulo derecho nódulo de alta sospecha de malignidad y conglomerado de adenopatías con lavado de aguja positivo para tiroglobulina. Evaluada la hipereosinofilia con valores iniciales de leucocitosis de GB 30310/mm3 (10608/mm3 de eosinófilos) hasta valores máximos de GB 77090/mm3 (eosinófilos 20814/mm3) se interpretó como síndrome paraneoplásico y se inició corticoterapia en dosis inmunosupresoras sin respuesta. Nuestras observaciones presentadas en este artículo están en línea con la mayoría de los estudios que reflejan que la hipereosinofilia paraneoplásica se caracteriza por una enfermedad más avanzada y un mal pronóstico.
Assuntos
Síndromes Paraneoplásicas , Neoplasias da Glândula Tireoide , Humanos , Pessoa de Meia-Idade , Neoplasias da Glândula Tireoide/complicações , Neoplasias da Glândula Tireoide/patologia , Síndromes Paraneoplásicas/etiologia , Síndrome Hipereosinofílica/complicações , Masculino , Feminino , Carcinoma Papilar/complicações , Eosinofilia/complicaçõesRESUMO
BACKGROUND: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a severe adverse drug reaction marked by delayed hypersensitivity reactions causing skin and systemic complications. DRESS diagnosis is challenging due to the variety of clinical presentations and symptom overlap with other conditions. The perioperative period in these patients requires precise pharmacological strategies to prevent complications associated with this syndrome. The treatment of DRESS induced by unfractionated heparin during cardiopulmonary bypass (CPB) surgery presents some challenges that must be considered when selecting an anticoagulant to avoid side effects. In this case, bivalirudin, a direct thrombin inhibitor, is indicated as an alternative to heparin in patients undergoing CPB. However, in contrast to heparin/protamine, there is no direct reversal agent for bivalirudin. CASE PRESENTATION: We report the case of an 11-year-old male diagnosed with native aortic valve endocarditis and thrombosis in his left lower extremity. During valvular replacement surgery, systemic unfractionated heparin was administered. Postoperatively, the patient developed fever, eosinophilia and pruritic rash. Warm shock and elevated alanine transaminase (ALT) and aspartate transaminase (AST) levels followed, leading to the diagnosis of DRESS syndrome. Treatment with methylprednisolone resulted in complete resolution of symptoms. Seven years later, the patient was readmitted due to insufficient anticoagulation and a thrombus in the prosthetic aortic valve, presenting a recurrent DRESS episode due to the administration of unfractionated heparin, which was later replaced with low-molecular-weight heparin during hospitalization. Treatment with corticosteroids and antihistamines was initiated, resulting in the resolution of this episode. Ultimately, the patient required the Ross procedure. During this intervention the anticoagulation strategy was modified, unfractionated heparin was replaced with bivalirudin during the procedure and fondaparinux was administered during the postoperative period. This resulted in stable transaminases levels and no eosinophilia. CONCLUSION: The severity of DRESS Syndrome underscores the importance of early recognition, heightened monitoring, and a comprehensive approach tailored to each patient's needs. This particular case highlights the significance of this approach and may have a substantial clinical impact since it provides alternatives to heparin, such as bivalirudin and fondaparinux, in the anticoagulation strategy of CPB for patients who have a hypersensibility reaction to this medication; thus, enhancing clinical outcomes by minimizing risks linked to adverse drug reactions.
Assuntos
Anestésicos , Síndrome de Hipersensibilidade a Medicamentos , Eosinofilia , Masculino , Humanos , Criança , Heparina/uso terapêutico , Fondaparinux , Síndrome de Hipersensibilidade a Medicamentos/tratamento farmacológico , Anticoagulantes/uso terapêutico , Hirudinas/efeitos adversos , Eosinofilia/induzido quimicamente , Eosinofilia/tratamento farmacológico , Fragmentos de Peptídeos , Proteínas RecombinantesRESUMO
PURPOSE: Neurodevelopmental disorders, such as autism spectrum disorder (ASD), have been increasingly associated with eosinophilic gastrointestinal disorders (EGID). However, the relationship between these diseases remains unclear. We performed a systematic review with meta-analysis to address this issue. METHODS: The search was performed according to the PRISMA guidelines using descriptors for ASD and EGIDs from the MEDLINE, Embase, PsycInfo, LILACS, and Web of Science databases. Observational studies with the prevalence of ASD in any EGID were included. The study protocol was registered on the PROSPERO platform under the number CRD42023455177. RESULTS: The total dataset comprised 766,082 participants. The result of the single-arm meta-analysis showed an overall prevalence of ASD in the population with EGID of 21.59% (95% CI: 10.73-38.67). There was an association between EGID and ASD (OR: 3.44; 95% CI: 1.25-2.21), also significant when restricted only to EoE (OR: 3.70; 95% CI: 2.71-5.70). DISCUSSION: Recent studies have implicated the influence of an inadequate epithelial barrier integrity in the pathogenesis of several diseases. The role of this mechanism can be extended to situations beyond allergic reactions, including other conditions with underlying immunological mechanisms. Several diseases are potentially related to the systemic effect of bacterial translocation in tissues with defective epithelial barriers. CONCLUSION: Our meta-analysis provides evidence that supports the consideration of EGID in patients with ASD and ASD in patients with EGID. Despite its limitations, the results should also be validated by future studies, preferably using multicenter prospective designs in populations with low referral bias.