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Secondary pneumothorax is a rare but serious complication of allergic bronchopulmonary aspergillosis (ABPA) and bronchiectasis [1,2]. Persistent air leak (PAL) after secondary pneumothorax is an ongoing abnormal communication between bronchi or alveoli and the pleural space, despite drainage. Ongoing PAL for 5 days after initial chest tube insertion necessitates prolonged ambulatory drainage or aggressive management with video-assisted thoracoscopic surgery (VATS) or pleurodesis [3,4]. There are no randomized trials examining the efficacy of endobronchial valves (EBVs) for PAL with underlying inflammatory pulmonary disease. We describe the successful use of an EBV for PAL in a man with ABPA on high dose steroids, with a large bronchopleural fistula (BPF).

Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with poor quality of life. Debilitating symptoms and the reality of shortened survival impact patients’ physical and emotional well-being and constrain the lives of patients’ caregivers. This study assessed the informational needs of medical providers who care for patients with IPF, IPF patients themselves, and their caregivers. Tailored surveys were sent electronically to providers, patients with IPF, and caregivers of patients with IPF collected on a rolling basis in March of 2017. Providers answered questions regarding their own informational needs and what information they believed patients needed. Patients and caregivers identified their own informational needs and the perceived needs for each other. About 2636 surveys were sent to providers, including 2041 to physicians, of whom 156 completed it. One hundred sixty patients and 29 caregivers responded to the survey via a link on a website. Eighty-six percent of providers described themselves as physicians who diagnose and treat IPF patients themselves. Providers ranked information on “making the diagnosis of IPF” as their top informational need. Patients and caregivers chose “disease progression/what to expect” as the most important informational need for themselves and for each other. Providers want to make a correct diagnosis when IPF is in the differential diagnosis. Patients and caregivers desire clarity around how IPF will behave over time and what their futures with IPF will look like. Resources for patients and their caregivers should include information on disease natural history in empathically worded, clear, and easily accessible formats.

Patient: Female, 56 Final Diagnosis: Dry Beri Beri Symptoms: Anasarca • ascending paralysis • hypotension • unresponsiveness Medication: — Clinical Procedure: — Specialty: Neurology Objective: Challenging differential diagnosis Background: Beriberi due to thiamine (vitamin B1) deficiency has two clinical presentations. Patients with dry beriberi present with neuropathy, and patients with wet beriberi present with heart failure, with or without neuropathy. Dry beriberi can mimic the most common form of Guillain-Barré syndrome (GBS), an acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Severe thiamine deficiency results in Wernicke’s encephalopathy. This report of a case of dry beriberi and Wernicke’s encephalopathy due to thiamine deficiency includes a review of the literature. Case Report: A 56-year old woman with a history of gallstone pancreatitis and protein-calorie malnutrition was treated six months previously with total parenteral nutrition (TPN). She initially presented at another hospital with paresthesia of the lower limbs, arms, and neck, and symptoms of encephalopathy. Initial diagnosis of GBS was made, based on magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) findings. Despite five days of intravenous immunoglobulin (IVIG) treatment, her encephalopathy worsened, requiring transfer to our hospital, where she required intubation and treatment with vasopressors. A repeat MRI of her brain showed changes consistent with Wernicke’s encephalopathy. Following treatment with high-dose intravenous thiamine, her mental status improved within 48 hours, and by the third hospital day, she no longer required intubation. Conclusions: Symptoms and signs of dry beriberi due to thiamine deficiency can mimic those of acute or chronic GBS. However, thiamine repletion leads to rapid clinical improvement and can prevent irreversible neurologic sequelae, including Korsakoff syndrome. Clinicians should consider thiamine deficiency in malnourished patients presenting with symptoms and signs of GBS.

Background Cardioprotective medication adherence can mitigate the risk of recurrent cardiovascular events and mortality after acute myocardial infarction ( AMI ). We examined the associations of diabetes mellitus status and glycemic control with cardioprotective medication adherence after AMI . Methods and Results We performed a retrospective observational cohort study of 14 517 US veterans who were hospitalized for their first AMI between 2011 and 2014 and prescribed a beta‐blocker, 3‐hydroxy‐3‐methyl‐glutaryl‐CoA‐reductase inhibitor, and angiotensin‐converting enzyme inhibitor or angiotensin receptor blocker. The primary exposure was a diagnosis of type 2 diabetes mellitus; in diabetes mellitus patients, hemoglobin A1c (HbA1c) was a secondary exposure. The primary outcome was 1‐year adherence to all 3 medication classes, defined as proportion of days covered ≥0.8, assessed using adjusted risk differences and multivariable Poisson regression. Of 14 517 patients (mean age, 66.3 years; 98% male), 52% had diabetes mellitus; 9%, 31%, 24%, 15%, and 21% had HbA1c Conclusions Although diabetes mellitus status had a minor positive impact on cardioprotective medication adherence after AMI , glycemic control at the time of AMI may help identify diabetes mellitus patients at risk of medication nonadherence who may benefit from adherence interventions after AMI .

Although idiopathic pulmonary fibrosis (IPF) is a progressive, fibrosing lung disease without a cure, there are a number of pharmacologic and nonpharmacologic treatments to consider. Patients with IPF should be encouraged to stay physically and socially active, and therapeutic maneuvers should be reassessed at follow-up. Comorbid conditions are common among patients with IPF; practitioners should be on the lookout for their occurrence and discuss treatment options if they arise.

Background May patients with interstitial lung disease (ILD) require supplementary oxygen (O2) therapy to maintain normoxia. However, ambulatory O2 delivery devices are constraining and cumbersome. The physiologic and symptomatic impact of these devices on ILD patients is unknown. Methods We conducted a prospective study of 30 clinically stable ILD patients (with varying disease severity), half of whom used O2 at baseline. Each subject completed two six-minute walk tests (6MWTs); for O2 users, one walk was completed while wearing a backpack (weight 7.2 pounds) containing a tank with compressed O2, and for non-users, one walk was completed with a similarly-weighted backpack. For each subject, during the second walk, no backpack was worn; for the second walk, O2 users received oxygen via a stationary delivery system. For both walks, O2 non-users wore a portable metabolic system, which measured variables related to respiratory physiology and gas exchange. Borg dyspnea and exertion ratings were recorded after each walk. Results Wearing the O2-containing backpack resulted in decreased distance covered during the 6MWT, and increased dyspnea and perceived exertion among O2 users. While wearing the weighted backpack, O2 non-users had a significantly lower peripheral O2 saturation and distance-saturation product. Compared with carrying O2 in the backpack, receiving O2 via the stationary concentrator resulted in the largest improvement in walk distance for the three subjects with greatest impairment at baseline (6MWT ≤ 300 m). Conclusion Among ILD patients, carrying portable O2 versus receiving O2 via a stationary concentrator results in significantly greater dyspnea and shorter distances covered in timed testing. Patients with the greatest impairment may be affected most. When prescribing O2, practitioners should alert patients to this effect and help patients decide on the best O2 delivery mode to meet their needs.

BackgroundIdiopathic pulmonary fibrosis (IPF) is a progressive, incurable lung disease whose intrusive symptoms rob patients of their quality of life. Patients with IPF rely on their caregivers for support and assistance in amounts that vary according to patients’ individual circumstances and disease severity. Knowledgeable and well-informed patients and caregivers are best suited to deal with life-altering conditions like IPF.MethodsWe conducted two hour-long focus groups with 13 patients with IPF and 4 caregivers of patients with IPF to better understand their informational needs and in what format such information should be delivered.ResultsPatients discussed the challenges IPF creates in their daily lives. They wanted information on how to live well despite having IPF, practical information on how they could remain active and travel and how they could preserve their quality of life despite living with a life-threatening disease like IPF. Caregivers wanted information on the general aspects of IPF, because it would help them understand what patients were going through. They also wanted specific information on how to give care to a patient with IPF, even when physical care may not be needed (as in earlier phases of the disease). Patients and caregivers both needed efficient information delivery from trustworthy sources, including the healthcare team involved in their care. They considered both spoken and written information valuable, and ease of access was critical.ConclusionThis study provides valuable insight regarding the informational needs of IPF patients and their caregivers. It is hoped that identifying or creating sources of this information, and insuring that patients and caregivers have access to it, will improve well-being for patients with IPF and their caregivers.

The cryopreservation and autotransplantation of ovarian tissue is emerging as a powerful approach for preserving fertility. However, for cancer patients, it may not be possible to transplant ovarian tissue due to the risk of re-seeding disease. We investigated strategies for transplantation of individually isolated follicles to minimize the risk of re-introducing cancer cells present within the vasculature of ovarian stroma. Procedures for large-scale isolation of early-stage follicles and their encapsulation into fibrin hydrogels were developed. For in vivo validation studies, mice were ovariectomized and transplanted with encapsulated follicles into the ovarian bursa. A substantial increase in the number of secondary follicles was observed in the graft at 9 days after transplantation, and antral follicles by day 21, demonstrating primordial follicle recruitment into the growing pool. Initially, elevated follicle-stimulating hormone levels declined substantially by day 21, indicating feedback from the graft; presence of corpora lutea showed the graft's capability of restoring hormone cyclicity. Taken together, the transplanted follicles were able to engraft, mature, and restore ovarian function in an infertile mouse. This biomaterial may, thus, provide a platform for follicle transplantation with a low risk of cancer contamination and for developing strategies that preserve fertility for women facing a cancer diagnosis.