Case reports in pulmonology 

About: Case reports in pulmonology is an academic journal published by Hindawi Publishing Corporation. The journal publishes majorly in the area(s): Lung & Medicine. It has an ISSN identifier of 2090-6854. It is also open access. Over the lifetime, 340 publications have been published receiving 1720 citations.

Diffuse Alveolar Hemorrhage Induced by Vaping

Abstract: There has been a significant increase in electronic cigarette (e-cigarette) use since its introduction in 2007. Ironically, there remains very few published literature on the respiratory complications of e-cigarettes. The use of personalized vaporizers or commonly known as “vaping” has started to overtake standard e-cigarette. Its dynamic vaporizer customization makes it challenging to assess long-term health effects. Case reports on the pulmonary complications of e-cigarettes are limited to bronchiectasis, eosinophilic pneumonia, pleural effusion, and suspected hypersensitivity pneumonitis. Diffuse alveolar hemorrhage (DAH) is bleeding into the alveolar spaces of the lung secondary to disruption of the alveolar-capillary basement membrane. We report a case of young male presenting with subacute respiratory failure. He was later found to have diffuse alveolar hemorrhage syndrome that is likely induced by aggressive vaping. This adds up to the rising concern on the possible serious complications of this innovative technology designed as a safer alternative to traditional cigarettes.

Pediatric Plastic Bronchitis: Case Report and Retrospective Comparative Analysis of Epidemiology and Pathology

Abstract: Plastic bronchitis (PB) is a pathologic condition in which airway casts develop in the tracheobronchial tree causing airway obstruction. There is no standard treatment strategy for this uncommon condition. We report an index patient treated using an emerging multimodal strategy of directly instilled and inhaled tissue plasminogen activator (t-PA) as well as 13 other cases of PB at our institution between 2000 and 2012. The majority of cases (n = 8) occurred in patients with congenital heart disease. Clinical presentations, treatments used, histopathology of the casts, and patient outcomes are reviewed. Further discussion is focused on the epidemiology of plastic bronchitis and a systematic approach to the histologic classification of casts. Comorbid conditions identified in this study included congenital heart disease (8), pneumonia (3), and asthma (2). Our institutional prevalence rate was 6.8 per 100,000 patients, and our case fatality rate was 7%.

A Case of Pulmonary Tumor Thrombotic Microangiopathy Diagnosed by Transbronchial Lung Biopsy and Treated with Chemotherapy and Long-Term Oxygen and Anticoagulation Therapies

Abstract: A 41-year-old woman, who underwent breast resection for cancer of the right breast and adjuvant chemotherapy 2 years ago, was admitted to our hospital due to shortness of breath upon exertion. High-resolution computed tomography of the chest showed small nodular opacities in the peribronchiolar area in both lungs, as well as mediastinal and hilar lymphadenopathy. A transbronchial lung biopsy revealed breast cancer metastasis and pulmonary tumor thrombotic microangiopathy (PTTM). Treatment of PTTM is rarely reported due to the difficulty of antemortem diagnosis; however, the patient was effectively treated with chemotherapy and oxygen and anticoagulation therapies for 3 months.

Transition from Hepatopulmonary Syndrome to Portopulmonary Hypertension: A Case Series of 3 Patients

Abstract: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are the two major pulmonary vascular complications of liver disease. While HPS is characterized by low pulmonary vascular resistance, PPHTN is defined by the presence of elevated pulmonary vascular resistance. Given these seemingly opposing pathophysiologic mechanisms, these conditions were traditionally felt to be mutually exclusive. In this series, we present three patients with severe hepatopulmonary syndrome who had spontaneous resolution of their HPS with the subsequent development of PPHTN. To our knowledge, this is the largest case series presented of this phenomenon in nontransplanted patients. One proposed mechanism for the occurrence of this phenomenon involves dysregulation of the same vascular signaling pathway, which may lead to both pulmonary vascular dilatations and pulmonary arterial remodeling in the same patient. Another theory involves the possible differential binding of endothelin-1, a vasoactive signaling peptide that induces vasoconstriction when bound to receptor A and vasodilation when bound to receptor B. Although the mechanisms for this phenomenon remain unclear, it is important to be vigilant of this phenomenon as it may change the patient's overall treatment plan, especially in regard to appropriateness and timing of liver transplant.

TINF2 Gene Mutation in a Patient with Pulmonary Fibrosis

Abstract: Pulmonary fibrosis is a frequent manifestation of telomere syndromes. Telomere gene mutations are found in up to 25% and 3% of patients with familial disease and sporadic disease, respectively. The telomere gene TINF2 encodes an eponymous protein that is part of the shelterin complex, a complex involved in telomere protection and maintenance. A TINF2 gene mutation was recently reported in a family with pulmonary fibrosis. We identified a heterozygous Ser245Tyr mutation in the TINF2 gene of previously healthy female patient that presented with progressive cough due to pulmonary fibrosis as well as panhypogammaglobulinemia at age 52. Retrospective multidisciplinary evaluation classified her as a case of possible idiopathic pulmonary fibrosis. Telomere length-measurement indicated normal telomere length in the peripheral blood compartment. This is the first report of a TINF2 mutation in a patient with sporadic pulmonary fibrosis, which represents another association between TINF2 mutations and this disease. Furthermore, this case underlines the importance of telomere dysfunction and not telomere length alone in telomere syndromes and draws attention to hypogammaglobulinemia as a manifestation of telomere syndromes.