Treatment of anaplastic lymphoma kinase-positive lung adenocarcinoma with pemetrexed and carboplatin after crizotinib failure and significant liver dysfunction; a case report.

Tyrosine kinase inhibitors are the first-line treatment for Anaplastic Lymphoma Kinase-positive lung adenocarcinomas. However, chemotherapy is still an option in patients who are unresponsive or intolerant of tyrosine kinase inhibitors. There is a high likelihood of brain metastasis in patient with lung adenocarcinomas with Anaplastic Lymphoma Kinase rearrangement. Surveillance brain imaging may have a role in clinical follow up. Brigatinib and lorlatinib are two tyrosine kinase inhibitors with excellent intracranial penetrance.

Unexpected case of critical left main coronary artery dissection in a young woman.

A 36-year-old woman presented with a 3-month history of recurrent substernal chest pain, which acutely worsened 2 days prior to presentation. Her initial troponin I was mildly elevated and ECG showed subtle changes initially concerning for ischaemia; however, these were present on her prior ECG and were not considered an acute change. Because of her age and lack of significant risk factors, she was considered low risk for cardiac disease and initially treated conservatively for a non-ST elevation myocardial infarction. Due to persistent symptoms and dynamic changes on ECG concerning for ischaemia, she was immediately taken for a cardiac catheterisation and was found to have critical left main coronary artery dissection with a focal stenotic lesion. She had an extensive workup to identify the underlying cause of her coronary artery dissection which was unrevealing. She underwent an uncomplicated coronary artery bypass graft surgery and was discharged home in stable condition.

Mepolizumab as Possible Treatment for Allergic Bronchopulmonary Aspergillosis: A Review of Eight Cases.

Allergic bronchopulmonary aspergillosis (ABPA) is an eosinophilic pulmonary disorder caused by a hypersensitivity reaction to Aspergillus fumigatus that manifests with uncontrolled asthma, peripheral blood eosinophilia, and radiological findings, such as mucus plugging. Early diagnosis and proper treatment of ABPA are essential to prevent irreversible lung damage such as pulmonary fibrosis and bronchiectasis and improve the quality of life of patients. Beside inhaled medication for asthma, anti-inflammatory agents (i.e., systemic glucocorticoids) and antifungal agents are the mainstay treatment of ABPA. The goal of therapy using glucocorticoids and antifungal agents is to suppress the immune hyperreactivity to A. fumigatus and attenuate the fungal burden. Since the systemic glucocorticoid therapy may lead to serious adverse effects including osteoporosis, avascular necrosis, myopathy, cushingoid appearance, hypertension, insomnia, and increased risk of infection, a glucocorticoid-sparing agent could be considered. Mepolizumab is a humanized monoclonal antibody that binds to interleukin-5, which is the key mediator for eosinophil differentiation, activation, migration, and survival. We review eight cases of ABPA treated successfully with mepolizumab. Treatment with mepolizumab was not restricted to the total immunoglobulin E level, the limiting factor for omalizumab in ABPA. In addition, mepolizumab therapy improved forced expiratory volume in one second, radiological findings, and patient quality of life.

Inappropriate Shock from Subcutaneous Implantable Cardioverter-Defibrillator Caused by Electrical Remodeling After Wolff-Parkinson-White Ablation.

Hypertrophic cardiomyopathy (HCM) and Wolff-Parkinson-White syndrome have been associated with sudden cardiac death. A subcutaneous implantable cardioverter-defibrillator (S-ICD) is an effective device used to reduce the risk of sudden cardiac death in these patients. The most common cause of inappropriate shocks with S-ICD is T-wave oversensing. We present the case of a 19-year-old man with repeated shocks from his S-ICD. This case highlights some of the sensing issues related to the S-ICD that can result in inappropriate shocks. A vector change may have occurred after T-wave remodeling, post accessory pathway ablation, and loss of R-waves due to HCM scar progression, leading to this consequence.

Rosai-Dorfman Disease and Unusual Local Invasive Presentation.

Rosai-Dorfman disease (RDD) is a rare medical condition with bilateral painless lymphadenopathy. We present the case of a young man diagnosed with a very unique presentation of Rosai-Dorfman disease. A 40-year-old African-American man presented with a firm, non-tender, progressive chest and neck mass appeared three months ago. Imaging of the neck demonstrated an 8.6-cm anterior neck subcutaneous soft tissue mass extending into the anterior mediastinum through the sternum with erosive changes in the sternum and the lesion is abutting the right common carotid artery and innominate vein and surrounds the medial aspect of the clavicles bilaterally. Ultrasound (US)-guided biopsy showed marked polytypic-appearing plasma cell proliferation associated with relatively prominent histiocytes with hemophagocytosis/emperipolesis and focal neutrophils. There were S100+ histiocytes; however, findings were not typical for RDD. As that biopsy was not diagnostic, incisional biopsy with adequate sampling was performed. Surgical pathology demonstrated a very abnormal infiltrate with prominent histiocytes including areas with the features of extranodal RDD. BRAF V600E immunohistochemistry (IHC) was negative. Modified radical neck dissection, proximal sternal resection and superior mediastinal nodal dissection surgery was recommended. However, the patient refused the procedure. Typical manifestations are lymphadenopathy with fever that our patient did not experience. Bone involvement happens in 5-10% of cases. There is not enough data about blood vessel invasion which make our case unique. Treatment plan is still controversial. Clinical monitoring is recommended if the symptoms are tolerable as regression has been reported in many cases (20-50%). Surgery is reserved for patients with vital organ involvement or extra-nodal disease.

A Case of Delayed Diagnosis of Moyamoya Disease After Recurrent Strokes.

We present a case of a 58-year-old man with delayed diagnosed moyamoya disease who underwent encephaloduroarteriosynangiosis (EDAS) procedure. This patient with a history of three strokes presented to our facility with new left facial droop. Neurological examination revealed left facial droop and hemiparesis. Brain magnetic resonance imaging (MRI) described right frontal lobe acute ischemia. Head computed tomography (CT) angiography revealed bilateral supraclinoid internal carotid artery (ICA) occlusions. Cerebral angiography demonstrated diffuse intracranial vascular irregularity with stenosis, more above the bilateral supraclinoid ICAs and the right middle cerebral artery (MCA) suggestive of moyamoya disease. Due to the lack of MCA patency, he underwent EDAS. Superficial temporal artery (STA) was dissected inferiorly and the posterior branch was bipolared, then STA was movable. A bur hole made at the superior and inferior portion along the STA. Dura was opened, and STA was brought on top of the pia. His facial droop gradually improved after that. Nine months later, no new strokes reported. Moyamoya disease is a rare neurovascular disorder characterized by narrowing and occlusion of the ICA branches. Its symptoms include recurrent ischemic/hemorrhagic strokes. Incidence in Hispanics has not been studied. The gap between the first manifestations and disease progression is one to eight years. Its diagnosis is often delayed. Our patient had recurrent strokes for five years. Despite therapy with antiplatelets, new ischemic stroke brought him to our institution. Rate of recurrent strokes despite antiplatelets was reported 10.3% per year. Brain CTs and MRIs had failed to detect strokes' etiology. Catheter-directed angiography is the gold standard test for diagnosis of moyamoya disease. Antiplatelet alone is ineffective and surgery is the effective method to prevent further strokes, although there are no studies in adults regarding the efficacy of indirect revascularization. In direct revascularization, usually STA anastomoses to MCA. Indirect method works through the development of leptomeningeal collaterals. Postoperative complications are infarction and hyperperfusion syndrome. Seong-eun et al. proposed that modified EDAS is simpler with less complications in comparison with direct revascularization. Some other studies showed higher chance of stroke in indirect method versus direct technique. In conclusion, it is important to consider moyamoya disease as a differential diagnosis in patients with recurrent strokes.

Prediction equations for pulmonary function values in healthy children in Mashhad city, North East Iran.

BACKGROUND: Pulmonary function tests (PFTs) are used in assessing physiological to clinical status of the respiratory system, which is expressed as a percentage of predicted values. Predicted PFTs values are varies in different ethnics. Predicted PFTs values were studied in a sample of Iranian children. MATERIALS AND METHODS: Prediction equations for PFTs were derived from urban children in the city of Mashhad (northeast Iran). Regression analysis using height and age as independent variables was applied to provide predicted values for both sexes. PFT values were measured in 414 healthy children (192 boy and 222 female, aged 4-10 years). Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), maximal mid-expiratory flow (MMEF), peak expiratory flow (PEF), MEF at 75%, 50% and 25% of the FVC (MEF75, MEF50 and MEF25 respectively) were measured. RESULTS: There were positive correlations between each pulmonary function variable with height and age. The largest positive correlations were found for FVC (r = 0.712, P < 0.0001) and FEV1 (r = 0.642, P < 0.0001) in boys and girls respectively with height and for PEF (0.698, P < 0.0001) and MEF (r = 0.624, P < 0.0001) with age. Comparison of PFTs derived from the equations of the present study showed significant differences with those of several previous studies (P < 0.001 for most cases). CONCLUSION: A set of PFT reference values and prediction equations for both sexes has been derived using relatively large, healthy, Iranian children for the first time, which the generated results were differ from several prediction equations.