Chest
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Lymphangioleiomyomas occur in 38% of patients with sporadic lymphangioleiomyomatosis (LAM) and may cause pain and increased abdominal girth, mimicking the presence of a malignancy. Lymphatic involvement in LAM is closely associated with elevated serum levels of vascular endothelium growth factor-D (VEGF-D). Because lymphangioleiomyomas undergo diurnal variation in volume, we hypothesized that daytime ingestion of food, by increasing chyle formation and lymphatic flow, is the cause of an increase in lymphangioleiomyoma volume. ⋯ Lymphangioleiomyoma volume decreased during the fasting state. Conversely, a combination of food intake and decreased chyle flow through lymphatics partially obstructed by LAM cells may account for increases in lymphangioleiomyoma size. Imaging studies performed under fasting conditions may help in determining whether an abdominal tumor is a result of LAM or malignancy.
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Interstitial lung disease (ILD) is a heterogeneous group of rare diseases that primarily affect the pulmonary interstitium. Studies have implicated a role for telomere length (TL) maintenance in ILD, particularly in idiopathic interstitial pneumonia (IIP). Here, we measure TL in a wide spectrum of sporadic and familial cohorts of ILD and compare TL between patient cohorts and control subjects. ⋯ The results show telomere shortening across all ILD diagnoses. The difference in TL between the FIP-TERT and FIP-SFTP groups indicates the distinction between acquired and innate telomere shortening. Short TL in the IPF and FIP-no mutation groups is indicative of an innate telomere-biology defect, while a stress-induced, acquired telomere shortening might be the underlying process for the other ILD diagnoses.
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Case Reports
A 60-Year-Old Woman With Cough, Dyspnea, and Atelectasis 19 Years After Liver Transplant.
A 60-year-old black woman presented with nonproductive cough of 1-month duration. She had also experienced rapidly progressive dyspnea for 1 week and one bout of vomiting a day before presentation. ⋯ Her medical history was significant for diabetes mellitus and liver transplant 19 years earlier for hepatitis C cirrhosis, for which she was receiving tacrolimus and mycophenolate. She was a current smoker with 40 pack-years of smoking history.
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Case Reports
Three-dimensional Modeled T-tube Design and Insertion in a Patient With Tracheal Dehiscence.
A 68-year-old man with recurrent medullary thyroid cancer underwent cervical tracheal resection and reconstruction. His course was complicated by tracheal anastomotic dehiscence, right carotid blowout, and ultimately cervical tracheoplasty with AlloDerm. Given the complex vascular interventions and upper-airway anatomy, a custom-designed Montgomery T-tube was designed for him. ⋯ The patient was discharged to home with the ability to phonate. To our knowledge, this is the first demonstration of three-dimensional modeling of an upper-airway defect with subsequent T-tube design using engineering software. The success of this case demonstrates a possible avenue for personalized airway prosthesis design and manufacturing in the future.
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A 33-year-old man, never smoker, presented with acute-onset dyspnea secondary to bilateral pulmonary emboli. Echocardiography at the time revealed a right atrial myxoma, for which he underwent resection, followed by anticipated lifelong therapeutic anticoagulation therapy.