Miocarditis aguda como manifestación de granulomatosis eosinofílica

We present the case of a young male traveller in Southeast Asia who consulted for chest pain, and was initially managed as an acute coronary syndrome. Once coronary artery disease was ruled out and hypereosinophilia was observed, we carried out a broad differential diagnosis of its possible causes. Complementary tests confirmed eosinophilic myocarditis with ventricular dysfunction. Further clinical history and tests led to a diagnosis of eosinophilic granulomatosis with polyangiitis. Following the introduction of drugs for heart failure, as well as corticosteroids and mepolizumab, the patient improved clinically, and the ejection fraction was completely recovered.Se presenta el caso de un varón joven, viajero por el sudeste asiático, que consulta por dolor torácico y es tratado inicialmente como síndrome coronario agudo. Se descarta enfermedad coronaria y se objetiva hipereosinofilia, por lo que comienza un amplio diagnóstico diferencial de sus posibles causas. Las pruebas complementarias confirman una miocarditis eosinofílica con disfunción ventricular. Profundizando en la historia clínica y ampliando las pruebas, se alcanza el diagnóstico de granulomatosis eosinofílica con poliangeítis. Tras la instauración de fármacos para insuficiencia cardíaca, así como corticoides y mepolizumab, el paciente mejora clínicamente y la fracción de eyección se restablece completamente

Clinical Heterogeneity of Pulmonary Arterial Hypertension Associated With Variants in TBX4

Background: The knowledge of hereditary predisposition has changed our understanding of Pulmonary Arterial Hypertension. Genetic testing has been widely extended and the application of Pulmonary Arterial Hypertension specific gene panels has allowed its inclusion in the diagnostic workup and increase the diagnostic ratio compared to the traditional sequencing techniques. This is particularly important in the differential diagnosis between Pulmonary Arterial Hypertension and Pulmonary Venoocclusive Disease. Methods: Since November 2011, genetic testing is offered to all patients with idiopathic, hereditable and associated forms of Pulmonary Arterial Hypertension or Pulmonary Venoocclusive Disease included in the Spanish Registry of Pulmonary Arterial Hypertension. Herein, we present the clinical phenotype and prognosis of all Pulmonary Arterial Hypertension patients with disease-associated variants in TBX4. Results: Out of 579 adults and 45 children, we found in eight patients from seven families, disease-causing associated variants in TBX4. All adult patients had a moderate-severe reduction in diffusion capacity. However, we observed a wide spectrum of clinical presentations, including Pulmonary Venoocclusive Disease suspicion, interstitial lung disease, pulmonary vascular abnormalities and congenital heart disease. Conclusions: Genetic testing is now essential for a correct diagnosis work-up in Pulmonary Arterial Hypertension. TBX4-associated Pulmonary Arterial Hypertension has marked clinical heterogeneity. In this regard, a genetic study is extremely useful to obtain an accurate diagnosis and provide appropriate management.This project was founded by Project "Bases Gene´tico Moleculares de la Medicina de Precisio´n en la Hipertensio´n Arterial Pulmonar". Funder: Instituto Carlos III. Ministerio de Economı´a y Competitividad. https://www.isciii.es/Paginas/Inicio.aspx Award number: PI 18/01233 Grant Recipient: P E-

Influence of platelet-rich plasma on the histologic charcteristics of the autologous fat graft to the upper lip of rabbits

Background: This study evaluated the influence of platelet-rich plasma (PRP) on the histologic characteristics of autologous fat grafts to the upper lip of rabbits. Methods: Nine New Zealand white rabbits were used. eight of the rabbits underwent fat harvest from the groin fat pads using a modified Coleman technique. One side of the upper lip was infiltrated with autologous fat and the other side with fat and PRP. Four of the inflitrated rabbits were killed 8 weeks after the lip augmentation, and the remaining four infiltrated rabbits plus one control case were killed 12 weeeks after the procedure. Coronal sections of both upper lips were analyzed microscopically to evaluate the quality of the fat graft, the inflammtory reaction, the presence of oil cysts, the degree of fibrosis, and the neovascularization. Results: The infiltration of adipose tissue plus PRP presented less inflammtory reaction (p<0.05) and fewer oil cysts (p<0.05) than the infiltration of isolated fat. Platelet-rich plasma increases the maintenance of the transplanted fat cells

Giant Cell Reparative Granuloma of the Orbit: Clinicopathological Characteristics and Treatment

Giant cell reparative granuloma (GCRG) is a rare fibroosseous lesion uncommonly seen in the orbital area. Although benign, it is known to be recurrent and locally destructive. We report two cases of GCRG of the orbit. In both cases, computed tomography revealed a heterogeneously growing well-defined mass, arising from the roof of the orbit, affecting the cortex, and invading the orbit. In the first case, the mass extended into the anterior cranial fossa. Magnetic resonance imaging with gadolinium showed, in both cases, a cystic character of the lesion with fluid levels. The surgical treatment was performed via an upper crease incision. An ultrasonic aspirator system was used to remove the tumor tissue and its extension into cranial fossa. Careful histopathologic analysis established the diagnosis of GCRG. Symptoms resolved completely with no evidence of recurrence after a follow-up of 18 and 14 months, respectively. We present the clinicopathological and radiological findings, and we describe the surgical approach. As a rare entity, GCRG of the orbit should be considered in differential diagnosis of fibroosseous orbital masses. Complete surgical excision carries a low risk of recurrence

Clinical heterogeneity of pulmonary arterial hypertension associated with variants in TBX4

Background The knowledge of hereditary predisposition has changed our understanding of Pulmonary Arterial Hypertension. Genetic testing has been widely extended and the application of Pulmonary Arterial Hypertension specific gene panels has allowed its inclusion in the diagnostic workup and increase the diagnostic ratio compared to the traditional sequencing techniques. This is particularly important in the differential diagnosis between Pulmonary Arterial Hypertension and Pulmonary Venoocclusive Disease. Methods Since November 2011, genetic testing is offered to all patients with idiopathic, hereditable and associated forms of Pulmonary Arterial Hypertension or Pulmonary Venoocclusive Disease included in the Spanish Registry of Pulmonary Arterial Hypertension. Herein, we present the clinical phenotype and prognosis of all Pulmonary Arterial Hypertension patients with disease-associated variants in TBX4. Results Out of 579 adults and 45 children, we found in eight patients from seven families, disease-causing associated variants in TBX4. All adult patients had a moderate-severe reduction in diffusion capacity. However, we observed a wide spectrum of clinical presentations, including Pulmonary Venoocclusive Disease suspicion, interstitial lung disease, pulmonary vascular abnormalities and congenital heart disease. Conclusions Genetic testing is now essential for a correct diagnosis work-up in Pulmonary Arterial Hypertension. TBX4-associated Pulmonary Arterial Hypertension has marked clinical heterogeneity. In this regard, a genetic study is extremely useful to obtain an accurate diagnosis and provide appropriate managementInstituto de Salud Carlos III | Ref. PI 18/0123