272. An unusual cause of respiratory failure in a 25-year-old heart and lung transplant recipient.
A 25-year-old woman, a never smoker with a history of heart-lung transplantation for World Health Organization group 1 pulmonary arterial hypertension performed 20 months prior to presentation, was evaluated for shortness of breath. Following transplantation, she was initiated on standard therapy of prednisone, tacrolimus, and azathioprine, along with routine antimicrobial prophylaxis. Her posttransplant course was complicated by persistent acute cellular rejection, as determined from a transbronchial biopsy specimen, without evidence of rejection in an endomyocardial biopsy specimen. The immunosuppressive medications were supplemented with pulse-dosed steroids, and the patient was transitioned from azathioprine to mycophenolate mofetil. Sirolimus was added 9 months prior to presentation. Three months prior to presentation, she was admitted for increasing oxygen requirements, shortness of breath, and bilateral infiltrates on the CT scans of the chest.
273. A 44-year-old man with abdominal pain, lung nodules, and hemoperitoneum.
A 44-year-old man presented with a 1-day history of sudden-onset abdominal pain. The pain was characterized as severe, diffuse, sharp, and nonradiating. Associated symptoms included nausea, vomiting, diarrhea, and subjective fevers. He was originally from El Salvador, but had not traveled in > 10 years. Review of systems was positive for 2 weeks of dry cough with associated mild, bilateral, pleuritic chest pain and subjective weight loss. His medical history was notable for gout and end-stage renal disease secondary to chronic nonsteroidal antiinflammatory drug use, for which he attended hemodialysis sessions three times weekly. Surgical history consisted of a currently nonfunctioning left upper extremity fistula, a longstanding right internal jugular PermCath IV access for chronic hemodialysis that had been removed 2 weeks prior to presentation, and a left brachiocephalic fistula. He did not smoke, consume alcohol, or have a history of illicit drug use.
274. A rare cause of postoperative hypotension.
A 62-year-old woman presented with a 3-month history of abdominal distension and decreased exercise tolerance. A chest radiograph showed a probable left pleural effusion (Fig 1). A CT scan of the abdomen revealed a solid ovarian mass with omental caking and a large volume of ascites; there was also confirmation of a left pleural effusion. Three days before surgery a CT pulmonary angiogram (CTPA) showed no evidence of pulmonary thromboembolism (PTE). The patient had some improvement in her symptoms after paracentesis and thoracentesis with drainage of 2,000 mL and 250 mL of fluid, respectively. She underwent total abdominal hysterectomy, bilateral oophorectomy, and partial sigmoid resection with an estimated blood loss of 850 mL. During the operation, she received 5 L of crystalloid and required phenylephrine at 40 to 80 μg/min to maintain a mean arterial pressure > 65 mm Hg. She was extubated after surgery, but immediately after extubation, she became markedly hypotensive and hypoxemic with a BP of 50/20 mm Hg and an oxygen saturation of 70%. An ECG showed T-wave inversions from V1 to V5 and an S1Q3T3 pattern (Fig 2). A bedside echocardiogram showed an enlarged right ventricle (RV), septal dyskinesia, and obliteration of the left ventricle, all consistent with systolic and diastolic RV overload (Fig 3).
276. A 19-year-old man with relapsing bilateral pneumothorax, hemoptysis, and intrapulmonary cavitary lesions diagnosed with vascular Ehlers-Danlos syndrome and a novel missense mutation in COL3A1.
A 19-year-old sportsman experienced a right-sided pneumothorax and hemoptysis after having had an intermittent cough and blood-tinged sputum for 2 months. A chest CT scan revealed small cavitary lesions in both lungs. The relapsing pneumothorax was treated with a chest tube twice, as well as surgically after the second relapse. Two months after surgery, the patient developed a cough, fever, and high C-reactive protein levels. At that time, large consolidations had developed in the right lung, while the left lung subsequently collapsed due to pneumothorax. The patient's physical appearance and anamnestic information led us to suspect a genetic connective tissue disease. A sequencing analysis of the COL3A1 gene identified a novel, de novo missense mutation that confirmed the diagnosis of vascular Ehlers-Danlos syndrome (EDS). This atypical presentation of vascular EDS with intrathoracic complications shows that enhanced awareness is required and demonstrates the usefulness of the genetic analyses that are clinically available for several hereditary connective tissue disorders.
279. Starting a lung transplant program: a roadmap for long-term excellence.
作者: Julia Klesney-Tait.;Michael Eberlein.;Lois Geist.;John Keech.;Joseph Zabner.;Peter J Gruber.;Mark D Iannettoni.;Kalpaj Parekh.
来源: Chest. 2015年147卷5期1435-1443页
Lung transplantation is an effective therapy for many patients with end-stage lung disease. Few centers across the United States offer this therapy, as a successful lung transplant program requires significant institutional resources and specialized personnel. Analysis of the United Network of Organ Sharing database reveals that the failure rate of new programs exceeds 40%. These data suggest that an accurate assessment of program viability as well as a strategy to continuously assess defined quality measures is needed. As part of strategic planning, regional availability of recipient and donors should be assessed. Additionally, analysis of institutional expertise at the physician, support staff, financial, and administrative levels is necessary. In May of 2007, we started a new lung transplant program at the University of Iowa Hospitals and Clinics and have performed 101 transplants with an average recipient 1-year survival of 91%, placing our program among the top in the country for the past 5 years. Herein, we review internal and external factors that impact the viability of a new lung transplant program. We discuss the use of four prospectively identified quality measures: volume, recipient outcomes, financial solvency, and academic contribution as one approach to achieve programmatic excellence.
280. Mobile health: assessing the barriers.
Mobile health (mHealth) combines the decentralization of health care with patient centeredness. Mature mHealth applications (apps) and services could provide actionable information, coaching, or alerts at a fraction of the cost of conventional health care. Different categories of apps attract diverse safety and privacy regulation. It is too early to tell whether these apps can overcome questions about their use cases, business models, and regulation.
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