The cardiomyopathy of cystic fibrosis: a modern form of Keshan disease.

Introduction: We conducted a study to determine the prevalence of structural heart disease in patients with CF, the characteristics of a cardiomyopathy not previously described in this population, and its possible relationship with nutritional deficiencies in CF.

Methods: We studied 3 CMP CF patients referred for heart-lung transplantation and a prospective series of 120 adult CF patients. All patients underwent a clinical examination, blood tests including levels of vitamins and trace elements, and echocardiography with evaluation of myocardial strain.

Danon Disease Presenting as Eosinophilic Myocarditis: Key Role of Endomyocardial Biopsy.

A 21-year-old woman with a history of atopy, peripheral eosinophilia, Wolf-Parkinson-White syndrome, and 5 episodes of myocarditis was diagnosed with eosinophilic myocarditis. Despite adequate immunosuppressive treatment and resolution of the myocarditis episode, the patient developed dilated cardiomyopathy and presented with worsening of her functional class. Finally, genetic testing unveiled an additional diagnosis: Danon disease.

Individualizing mechanical ventilation: titration of driving pressure to pulmonary elastance through Young's modulus in an acute respiratory distress syndrome animal model.

Background: Mechanical ventilation increases the risk of lung injury (VILI). Some authors propose that the way to reduce VILI is to find the threshold of driving pressure below which VILI is minimized. In this study, we propose a method to titrate the driving pressure to pulmonary elastance in an acute respiratory distress syndrome model using Young's modulus and its consequences on ventilatory-induced lung injury.

OPHTHALMOLOGIC INVOLVEMENT IN PATIENTS WITH HEREDITARY TRANSTHYRETIN AMYLOIDOSIS.

Purpose: The aim of this study was to determine the ophthalmologic involvement in patients with hereditary transthyretin amyloidosis and its correlation with the mutations described in the literature.

Methods: Cross-sectional, noninterventional study. Fifty-two eyes of 26 consecutive patients diagnosed with hereditary transthyretin amyloidosis who visited the Puerta de Hierro-Majadahonda University Hospital from September 2019 to March 2022.

Endomyocardial biopsy-confirmed myocarditis and inflammatory cardiomyopathy: clinical profile and prognosis.

Introduction And Objectives: Endomyocardial biopsy (EMB) is the only technique able to establish an etiological diagnosis of myocarditis or inflammatory cardiomyopathy (ICM). The aim of this study was to analyze the clinical profile, outcomes, and prognostic factors of patients with suspected myocarditis/ICM undergoing EMB.

Methods: We retrospectively analyzed the clinical characteristics, histological findings, and follow-up data of all patients with suspected myocarditis or ICM who underwent EMB between 1997 and 2019 in a Spanish tertiary hospital.

Peripheral blood eosinophilia as a marker of acute cellular rejection in lung transplant recipients.

Unlabelled: Previous studies in solid organ transplantation have shown a relationship between circulating eosinophil (EOS) counts and the presence of acute cellular rejection (ACR). However, the relationship between this potential biomarker and ACR in lung transplant (LTx) patients remains unclear.

Objective: To assess the association between EOS and the presence of acute cellular rejection in lung transplant recipients.

Checkpoint inhibitor-induced fulminant myocarditis, complete atrioventricular block and myasthenia gravis-a case report.

Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy over the last decade. Pembrolizumab, a humanized monoclonal IgG4 antibody, binds to the programmed death 1 (PD-1) receptor, blocking its interaction with programmed death-ligand 1 (PD-L1) and thereby increasing the anti-tumor activity of the host immune system. These drugs are associated with immune-mediated side effects that can be life threatening, and myocarditis is among the most serious events.

[Primary pulmonary myxoid sarcoma associated with translocation EWSR1-CREB1 and locorregional micrometastases].

Primary myxoid pulmonary sarcoma is a rare entity with an endobronchial growth. Although it should be included in the differential diagnosis of other sarcomas, its prognosis is usually favorable. We present the case of a 51-year-old patient with a mesenchymal tumor in the right lung, diagnosed as a primary pulmonary myxoid sarcoma, positive for EMA, vimentine and with a Ki-67 less than 5%; FISH revealed a EWSR1-CREB1 translocation.

Neoadjuvant chemotherapy and nivolumab in resectable non-small-cell lung cancer (NADIM): an open-label, multicentre, single-arm, phase 2 trial.

Background: Non-small-cell lung cancer (NSCLC) is terminal in most patients with locally advanced stage disease. We aimed to assess the antitumour activity and safety of neoadjuvant chemoimmunotherapy for resectable stage IIIA NSCLC.

Methods: This was an open-label, multicentre, single-arm phase 2 trial done at 18 hospitals in Spain.

Appendicular bleeding: an excepcional cause of lower hemorrhage.

Chronic complications of acute appendicitis managed in a conservative manner are not frequent. We present a case of acute lower gastrointestinal hemorrhage in a young patient with a previous acute appendicitis without surgical intervention. The colonoscopy detected an appendicular bleeding which was surgically treated.

Role of cardiac scintigraphy with ⁹⁹mTc-DPD in the differentiation of cardiac amyloidosis subtype.

Introduction And Objectives: We investigated the diagnostic accuracy of (99m)Tc-3,3-diphosphono-1,2 propanodicarboxylic acid ((99m)Tc-DPD) scintigraphy in differentiating between monoclonal immunoglobulin light chain and transthyretin-related cardiac amyloidosis.

Methods: Nineteen patients with documented cardiac amyloidosis were included: 8 with transthyretin-related amyloidosis (group A) and 11 with light chain amyloidosis (group B). All the patients underwent scintigraphy with (99m)Tc-DPD and (99m)Tc-methylene diphosphonate ((99m)Tc-MDP).