- Hypersensitivity Pneumonitis and Acute Respiratory Distress Syndrome From E-Cigarette Use. [Journal Article]
- PedPediatrics 2018 May 17
- Electronic cigarette (e-cigarette) use, or "vaping," is gaining widespread popularity as an alternative to conventional cigarettes among adolescents. Little is known of the health risks of e-cigarett...
Electronic cigarette (e-cigarette) use, or "vaping," is gaining widespread popularity as an alternative to conventional cigarettes among adolescents. Little is known of the health risks of e-cigarette use, especially in children and adolescents. We present a Case Report of a previously healthy 18-year-old woman who presented with dyspnea, cough, and pleuritic chest pain after e-cigarette use. She developed respiratory failure with hypoxia and was intubated, and ultimately met diagnostic criteria for acute respiratory distress syndrome. Chest tubes were placed to drain worsening pleural effusions. Computed tomography of the chest revealed dependent opacities in both lung bases, superimposed smooth interlobular septal thickening, and pleural effusions. Bronchoalveolar lavage revealed cellular debris and reactive mononuclear cells, and cell counts were remarkable for elevated mononuclear cells and eosinophilia. After the results of a workup for an infectious etiology came back negative, the patient was diagnosed with hypersensitivity pneumonitis and intravenous methylprednisolone therapy was initiated. After this the patient rapidly improved, was weaned off vasopressor support, and was extubated. This is the first reported case of hypersensitivity pneumonitis and acute respiratory distress syndrome as a risk of e-cigarette use in an adolescent, and it should prompt pediatricians to discuss the potential harms of vaping with their patients. Hypersensitivity pneumonitis, lipid pneumonia, and eosinophilic pneumonia should be included in the differential diagnosis of patients who exhibit respiratory symptoms after the use of an e-cigarette.
- Unravelling the NERDS syndrome. [Journal Article]
- BCBMJ Case Rep 2018 May 12; 2018
- A 22-year-old man presented with symmetric polyarthritis, pruritus and deviation of angle of mouth to the right side since the last 7 years. His symptoms were persistent despite receiving ayurvedic m...
A 22-year-old man presented with symmetric polyarthritis, pruritus and deviation of angle of mouth to the right side since the last 7 years. His symptoms were persistent despite receiving ayurvedic medications and symptomatic therapy. Examination revealed dry skin, cutaneous nodules, xanthelasma, periarticular non-tender swellings, pitting oedema of hands and feet and lower motor neuron type right facial palsy. Haematological investigations revealed eosinophilia and skin biopsy had cutaneous eosinophilic infiltration. The constellation of above findings comprises the nodules, eosinophilia, rheumatism, dermatitis and swelling syndrome. It a rare syndrome with few reported cases in literature. The patient was started on oral corticosteroids which was subsequently tapered and methotrexate therapy. His polyarthritis and skin rashes resolved with therapy. He has been followed-up for 2 years and is presently asymptomatic for the last 1 year.
- Adult-Type Rhabdomyoma of the Larynx in Birt-Hogg-Dubé Syndrome: Evidence for a Real Association. [Journal Article]
- HNHead Neck Pathol 2018 May 09
- The autosomal dominant Birt-Hogg-Dubé syndrome is known to be associated with skin, lung and kidney lesions. It is caused by heterozygous germline mutations in the folliculin gene and has a high pene...
The autosomal dominant Birt-Hogg-Dubé syndrome is known to be associated with skin, lung and kidney lesions. It is caused by heterozygous germline mutations in the folliculin gene and has a high penetrance. We report the case of a 51 year old woman with Birt-Hogg-Dubé syndrome who presented with a laryngeal mass. Imaging confirmed a mass centered on the piriform sinus and following excision histological examination confirmed the lesion was composed of polygonal cells with abundant eosinophilic cytoplasm consistent with a rhabdomyoma. Laryngeal rhabdomyoma is rare condition and has not been previously described in association with Birt-Hogg-Dubé. In patients with Birt-Hogg-Dubé syndrome who develop upper aerodigestive tract symptoms secondary to mass lesion an adult-type rhabdomyoma might be considered as a differential, with endoscopic excision being the treatment of choice.
- [Clinical Analysis of 40 Patients with Eosinophilic Lung Diseases in Peking Union Medical College Hospital]. [Journal Article]
- ZYZhongguo Yi Xue Ke Xue Yuan Xue Bao 2018 Apr 28; 40(2):170-177
- Objective To summarize the clinical features of eosinophilic lung diseases(ELD). Methods We retrospectively analyzed the clinical manifestations,laboratory findings,accessory examination results,and ...
Objective To summarize the clinical features of eosinophilic lung diseases(ELD). Methods We retrospectively analyzed the clinical manifestations,laboratory findings,accessory examination results,and pathology of 40 patients who were diagnosed with ELD and hospitalized in Peking Union Medical College Hospital from January 2013 to December 2016.Results There were 19 males and 21 females,and the average age was(48.58±18.25) years.The diagnoses included allergic bronchopulmonary aspergillosis(n=20),eosinophilic granulomatosis with polyangiitis(also known as churg-strauss syndrome)(n=10),chronic eosinophilic pneumonia(n=8),parasitic infection(n=1),and drug-induced eosinophilic pneumonia(n=1).Eosinophils counts in peripheral blood were increased in 35 patients(87.5%),and eosinophils counts in bronchoalveolar lavage fluids increased in 17 of 18 patients(94.4%).Arterial blood gas analysis showed varying degrees of hypoxemia in 23 patients(57.5%),and pulmonary function test showed ventilatory dysfunction in 27 patients(67.5%) and defect in diffusion capacity in 12 patients(30.0%).Chest CT revealed bilateral flaky,streaky,or diffuse ground-glass infiltrates and consolidations;in addition,central cylindrical bronchiectasis and mucous plugging with "finger-in-glove" pattern were seen in patients with allergic bronchopulmonary aspergillosis.Diffuse eosinophil infiltration was revealed in lung or other tissue biopsy.Glucocorticoids alone or combined with other therapies were effective in most patients.Conclusions ELD has a wide range of clinical presentations and can easily be misdiagnosed.Increased eosinophils count in peripheral blood and bronchoalveolar lavage fluids combined with infiltration manifestations in chest imaging are helpful for the diagnosis of ELD.Oral administration of glucocorticoids is the primary therapy for ELD.
- A case of infection-related glomerulonephritis with massive eosinophilic infiltration . [Journal Article]
- CNClin Nephrol 2018 Apr 27
- Infection-related glomerulonephritis (IRGN) is rarely complicated with eosinophil infiltration into the glomerulus. Here we report a case of eosinophilic proliferative glomerulonephritis related with...
Infection-related glomerulonephritis (IRGN) is rarely complicated with eosinophil infiltration into the glomerulus. Here we report a case of eosinophilic proliferative glomerulonephritis related with infection. A 70-year-old man with respiratory symptoms displayed hypereosinophilia, hypocomplementemia, impaired renal function, and nephrotic syndrome. Renal biopsy revealed endocapillary proliferative glomerulonephritis with immunostaining for immunoglobulin G and complement 3, and subepithelial hump-like electron-dense deposits, thus fulfilling the criteria for IRGN. Immunostaining for the nephritis-associated plasmin receptor (NAPlr) in the glomerulus confirmed the diagnosis of IRGN. Of note, eosinophils infiltrated into the glomerular subendothelial spaces, renal tubules, peritubular capillaries, and the interstitium in the kidney as well as in the alveolar walls and pulmonary arteries in the lung. Corticosteroid therapy rapidly improved hypereosinophilia as well as respiratory symptoms and renal function. Urinary protein exertion was decreased, and serum level of complement and albumin was increased. These findings suggest that eosinophil infiltration might play a prominent role in respiratory and renal disorders. Severe endothelial damage of glomeruli and tubulointerstitial nephritis, caused by eosinophil-rich inflammation, might significantly contribute to exacerbation of renal insufficiency. .
- Revisiting the NIH Taskforce on the Research needs of Eosinophil-Associated Diseases (RE-TREAD). [Review]
- JLJ Leukoc Biol 2018 Apr 19
- CONCLUSIONS: Although progress has been made since 2012, unmet needs in eosinophil research remain a priority.
- Hypereosinophilic Syndrome Complicated by Eosinophilic Myocarditis With Dramatic Response to Steroid. [Journal Article]
- JIJ Investig Med High Impact Case Rep 2018 Jan-Dec; 6:2324709618764512
- Introduction. Eosinophilic myocarditis is an infiltrative disease that affects the myocardium leading to various presentations. It can be precipitated by medications, helminthiasi...
Introduction. Eosinophilic myocarditis is an infiltrative disease that affects the myocardium leading to various presentations. It can be precipitated by medications, helminthiasis, or hypereosinophilic syndrome.Case.We present the case of a young, male patient who presented with palpitations and dyspnea and was found to have heart failure with reduced ejection fracture of 12%. His past medical history was significant for recent lung problem treated with steroids. Based on his history and laboratory findings, he was started on intravenous steroids for treatment of eosinophilic myocarditis. Within 3 days, his ejection fracture improved to 35%.Conclusion.Given the nonspecific clinical presentations, mimicking other diseases, high index of suspicion is warranted to diagnose eosinophilic myocarditis. This is crucial as early detection and treatment with steroids can lead to a dramatic response.
- Anti-PL-7 Antisynthetase Syndrome with Eosinophilic Pleural Effusion. [Journal Article]
- IMIntern Med 2018 Mar 09
- A 68-year-old woman was admitted to our hospital with fever and pleural effusion. Her thoracentesis showed eosinophilic pleural effusion (EPE) without any evidence of malignancy, infection, or trauma...
A 68-year-old woman was admitted to our hospital with fever and pleural effusion. Her thoracentesis showed eosinophilic pleural effusion (EPE) without any evidence of malignancy, infection, or trauma. Pleural biopsy revealed pleuritis and intercostal myositis. Characteristic skin manifestations, including Gottron's sign, interstitial lung disease, and pericardial effusion, appeared later in the clinical course. She was finally diagnosed with anti-PL-7 antisynthetase syndrome (ASS) based on the presence of anti-PL-7 antibody, and she fulfilled the diagnostic criteria for dermatomyositis. These clinical manifestations improved with immunosuppressive therapy. EPE might therefore be one of the characteristic features of anti-PL-7 ASS.
- GeneReviews® [BOOK]
- BOOKUniversity of Washington, Seattle: Seattle (WA)
- Loeys-Dietz syndrome (LDS) is characterized by vascular findings (cerebral, thoracic, and abdominal arterial aneurysms and/or dissections), skeletal manifestations (pectus excavatum or pectus carinat...
Loeys-Dietz syndrome (LDS) is characterized by vascular findings (cerebral, thoracic, and abdominal arterial aneurysms and/or dissections), skeletal manifestations (pectus excavatum or pectus carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus, cervical spine malformation and/or instability), craniofacial features (widely spaced eyes, strabismus, bifid uvula / cleft palate, and craniosynostosis that can involve any sutures), and cutaneous findings (velvety and translucent skin, easy bruising, and dystrophic scars). Individuals with LDS are predisposed to widespread and aggressive arterial aneurysms and pregnancy-related complications including uterine rupture and death. Individuals with LDS can show a strong predisposition for allergic/inflammatory disease including asthma, eczema, and reactions to food or environmental allergens. There is also an increased incidence of gastrointestinal inflammation including eosinophilic esophagitis and gastritis or inflammatory bowel disease. Wide variation in the distribution and severity of clinical features can be seen in individuals with LDS, even among affected individuals within a family who have the same pathogenic variant.
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- Correlation between upper and lower airway inflammations in patients with combined allergic rhinitis and asthma syndrome: A comparison of patients initially presenting with allergic rhinitis and those initially presenting with asthma. [Journal Article]
- ETExp Ther Med 2018; 15(2):1761-1767
- Allergic rhinitis (AR) and asthma often coexist. The terminology combined allergic rhinitis and asthma syndrome (CARAS) was introduced to describe patients with combined AR and asthma. The aim of the...
Allergic rhinitis (AR) and asthma often coexist. The terminology combined allergic rhinitis and asthma syndrome (CARAS) was introduced to describe patients with combined AR and asthma. The aim of the present study was to evaluate the correlation between eosinophilic inflammation in the upper and lower airways of patients with CARAS. Stable patients with CARAS initially presenting with AR or asthma were recruited. Healthy subjects and patients with AR alone were recruited as controls. Clinical characteristics, including disease history, lung function, nasal airway inspiratory resistance and upper and lower airway eosinophilic inflammation were evaluated and compared. A total of 73 subjects (22 patients with CARAS initially presenting with AR, 15 patients with CARAS initially presenting with asthma, 25 patients with AR alone and 11 healthy subjects) were studied. The nasal symptoms visual analogue scale scores at the week prior to enrollment and nasal airway inspiratory resistances were comparable among the groups. The percentage of predicted forced expiratory volume in 1 sec and percentage of predicted maximal middle expiratory flow in patients with CARAS initially presenting with asthma were significantly lower compared with the other three groups (P<0.05). No significant different in the percentage of eosinophils in the nasal lavage was observed between patients with CARAS and those with AR only; however, it was significantly increased compared with healthy subjects (P<0.05). The fractional concentration of exhaled nitric oxide and percentage of eosinophils in the sputum were significantly increased in patients with CARAS compared with those in the AR only and healthy subject groups (P<0.05). The difference in the percentage of eosinophils in the nasal lavage and sputum between patients with CARAS initially presenting with AR and initially presenting with asthma was not significant; however, a positive correlation between the percentage of eosinophils in the upper and lower airways was present in patients with CARAS initially presenting with AR only (r=0.526, P=0.030).