Cryptogenic Organizing Pneumonia with a Rare Radiographic Presentation of a Diffuse Micronodular Pattern Mimicking Miliary Lung Infiltration: A Case Report and Review of the Literature.

We reported a case of cryptogenic organizing pneumonia (COP) presenting with an unusual diffuse micronodular pattern (DMP) mimicking miliary lung infiltration. The patient is a 66-year-old man with a past medical history of diabetes mellitus type 2 and hyperlipidemia who presented with progressive dyspnea associated with significant weight loss and night sweats for 2 weeks. Upon admission, the patient's clinical condition rapidly progressed to respiratory failure requiring mechanical ventilation. Initial Chest X-ray (CXR) showed diffuse reticulonodular infiltration mimicking miliary pattern. Chest computed tomography (CT) showed diffuse centrilobular micronodular infiltrations with features of a tree-in-bud pattern consistent with the CXR findings. He was then started on empiric antibiotics for community-acquired pneumonia and underwent a diagnostic bronchoscopy with alveolar lavage and transbronchial biopsies, which yielded negative cultures and unrevealing pathology. Tissue from CT-guided lung biopsy performed later on was also inconclusive. Due to the lack of clinical improvement, he eventually underwent surgical lung biopsy. The pathology result showed organizing pneumonia (OP) pattern with heavy lymphoplasmacytic infiltrates and numerous multinucleated giant cells. His final culture results, microbiological data and serology workup for autoimmune disease were all unremarkable. The patient was diagnosed with COP and was started on systemic corticosteroids. He displayed dramatic clinical improvement and was successfully liberated from the ventilator. Subsequent chest imaging showed resolution of the reticulonodular infiltrations. Early diagnosis for OP and ability to distinguish OP from infectious pneumonitides are critical as the majority of patients with OP respond promptly to corticosteroids. Common findings of radiographic pattern for OP are patchy air space consolidation or ground-glass opacity, yet DMP is another rare radiographic pattern that must be recognized, especially in COP. In summary, this case illustrates a rare radiographic presentation of COP. With early recognition and prompt diagnosis, proper treatment can significantly prevent morbidity and reduce mortality.

Simultaneous Bilateral Primary Spontaneous Pneumothorax: A Case Report and a Review of the Literature.

BACKGROUND: Simultaneous bilateral primary spontaneous pneumothorax (SBPSP) is an extremely rare and potentially fatal condition. Patients usually have no relevant medical conditions. Some cases, however, may have certain risk factors such as smoking, being young, and male gender. We reported a case of a healthy young male who presented with BPSP. CASE PRESENTATION: A 21-year-old man with a past medical history of well-controlled intermittent asthma presented with acute worsening shortness of breath overnight. Chest X-ray performed showed bilateral large pneumothorax with significantly compressed mediastinum. Chest tubes were placed bilaterally with immediate clinical improvement. However, the chest tubes continued to have an air leak without full lungs expansion. Computed tomography scan without contrast of the chest revealed subpleural blebs in both upper lobes. The patient underwent bilateral video-assisted thoracoscopic surgery (VATS) with apical bleb resection, bilateral pleurectomy, and bilateral doxycycline pleurodesis. Biopsy of the apical blebs and parietal pleura of both lungs were negative for any atypical cells suspicious for malignancy or Langerhans cell histiocytosis. The patient had been doing well six months following surgery with no recurrence of pneumothorax. CONCLUSION: SBPSP is a rare and urgent condition that requires prompt intervention. In a young patient without any underlying disease, surgical intervention, such as VATS, is relatively safe and can be considered early.

Chronic eosinophilic pneumonia presenting with ipsilateral pleural effusion: a case report.

BACKGROUND: Chronic eosinophilic pneumonia is a rare idiopathic interstitial lung disease. The nearly pathognomonic radiographic finding is the peripheral distribution of alveolar opacities. Pleural effusions are rarely seen. We report a case of chronic eosinophilic pneumonia with transudative eosinophilic pleural effusion. CASE PRESENTATION: A 57-year-old Hispanic woman, a nonsmoker with a history of controlled asthma, presented to the hospital with unresolving pneumonia despite three rounds of antibiotics over a 2-month period. She was later diagnosed with chronic eosinophilic pneumonia based on the presence of peripheral blood eosinophilia, the peripheral distribution of alveolar infiltrates on chest radiograph, and a lung parenchymal biopsy with infiltrates of eosinophils. Upon presentation, our patient had a right-sided moderate-sized pleural effusion. The pleural fluid profile was consistent with a transudative effusion with eosinophil predominance. Our patient responded promptly to oral corticosteroid treatment in a few days. The pulmonary infiltrates and pleural effusion subsided on a 1-month follow-up chest radiograph after starting corticosteroid treatment. CONCLUSIONS: We report the first case of chronic eosinophilic pneumonia presenting with pneumonia with ipsilateral transudative eosinophilic pleural effusion. Like other cases of chronic eosinophilic pneumonia, early recognition and diagnosis is essential and prompt treatment with corticosteroids is the mainstay of therapy. Pleural effusion resolved without the further need for therapeutic thoracentesis.

Acute Fibrinous and Organizing Pneumonia Associated With Allogenic Hematopoietic Stem Cell Transplant Successfully Treated With Corticosteroids: A Two-Patient Case Series.

Acute fibrinous and organizing pneumonia (AFOP) is an extremely rare, relatively new, and distinct histological pattern of acute lung injury characterized predominately by the presence of intra-alveolar fibrin and associated organizing pneumonia. AFOP may be idiopathic or associated with a wide spectrum of clinical conditions. It has a variable clinical presentation from mild respiratory symptoms to that similar to the acute respiratory distress syndrome. Currently there is no consensus on treatment, and corticosteroids previously were of unclear benefit. To date, there are less than 40 cases of AFOP reported in the literature and only one has been linked to hematopoietic stem cell transplantation. Here we report the first case series of 2 patients who developed AFOP following allogenic stem cell transplant that were successfully treated with high-dose corticosteroids.

LINX®, a novel treatment for patients with refractory asthma complicated by gastroesophageal reflux disease: a case report.

BACKGROUND: Gastroesophageal reflux disease is one of the most common comorbidities in patients with asthma. Gastroesophageal reflux disease can be linked to difficult-to-control asthma. Current management includes gastric acid suppression therapy and surgical antireflux procedures. The LINX® procedure is a novel surgical treatment for patients with gastroesophageal reflux disease refractory to medical therapy. To the best of our knowledge, we report the first case of successful treatment of refractory asthma secondary to gastroesophageal reflux disease using the LINX® procedure. CASE PRESENTATION: Our patient was a 22-year-old white woman who met the American Thoracic Society criteria for refractory asthma that had remained poorly controlled for 5 years despite progressive escalation to step 6 treatment as recommended by National Institutes of Health-National Asthma Education and Prevention Program guidelines, including high-dose oral corticosteroids, high-dose inhaled corticosteroid plus long-acting ß2-agonist, leukotriene receptor antagonist, and monthly omalizumab. Separate trials with azithromycin therapy and roflumilast did not improve her asthma control, nor did bronchial thermoplasty help. Additional consultations with two other university health systems left the patient with few treatment options for asthma, which included cyclophosphamide. Instead, the patient underwent a LINX® procedure after failure of maximal medical therapy for gastroesophageal reflux disease with the additional aim of improving asthma control. After she underwent LINX® treatment, her asthma improved dramatically and was no longer refractory. She had normal exhaled nitric oxide levels and loss of peripheral eosinophilia after LINX® treatment. Prednisone was discontinued without loss of asthma control. The only immediate adverse effects due to the LINX® procedure were bloating, nausea, and vomiting. CONCLUSIONS: LINX® is a viable alternative to the Nissen fundoplication procedure for the treatment of patients with gastroesophageal reflux disease and poorly controlled concomitant refractory asthma.

A Rare but Reversible Cause of Hematemesis: "Downhill" Esophageal Varices.

"Downhill" varices are a rare cause of acute upper gastrointestinal bleeding and are generally due to obstruction of the superior vena cava (SVC). Often these cases of "downhill" varices are missed diagnoses as portal hypertension but fail to improve with medical treatment to reduce portal pressure. We report a similar case where recurrent variceal bleeding was initially diagnosed as portal hypertension but later found to have SVC thrombosis presenting with recurrent hematemesis. A 39-year-old female with history of end-stage renal disease presented with recurrent hematemesis. Esophagogastroduodenoscopy (EGD) revealed multiple varices. Banding and sclerotherapy were performed. Extensive evaluation did not show overt portal hypertension or cirrhosis. Due to ongoing bleeding requiring resuscitation, she underwent internal jugular (IJ) and SVC venogram in preparation for transjugular intrahepatic portosystemic shunt (TIPS), which demonstrated complete IJ and SVC occlusion. She underwent balloon angioplasty with stent placement across SVC occlusion with complete resolution of her varices and resolved hematemesis. "Downhill" varices are extremely rare, though previously well described. Frequently, patients are misdiagnosed with underlying liver disease. High index of suspicion and investigation of alternative causes of varices is prudent in those without underlying liver diseases. Prompt diagnosis and appropriate intervention can significantly improve morbidity and mortality.

Temporary Endobronchial Stent as a Bridge to Corrective Surgery For Severe Kyphoscoliosis-associated Central-Airway Extrinsic Compression.

Kyphoscoliosis is known to compromise lung function, with the primary mechanism being reduced chest wall compliance with a resultant restrictive pulmonary physiology. Severe scoliosis can also cause extrinsic compression of the central airways, leading to recurrent respiratory infections, lobar atelectasis, and potentially acute respiratory failure. Definitive therapy is corrective surgery of the spine. However, patients with severe scoliosis are at a potentially high risk of perioperative pulmonary complications. To our knowledge, we report the first successful use of retrievable endobronchial stents as a bridge to corrective surgery for kyphoscoliosis-associated complete central-airway extrinsic compression in a patient who was considered as too high risk for surgical correction due to her respiratory status. After surgery, the stents were removed and our patient experienced sustained improvement in pulmonary function and the clinical respiratory status.

Safety and Clinical Utility of Flexible Bronchoscopic Cryoextraction in Patients With Non-neoplasm Tracheobronchial Obstruction: A Retrospective Chart Review.

BACKGROUND: Airway obstruction from blood clots, airway secretions, and foreign bodies is a potentially life-threatening condition. Optimal management of this problem, whether by rigid or flexible bronchoscopy, has not been well studied. We report our single-center experience on the safety and clinical utility of cryoprobe extraction for this indication. METHODS: We performed a retrospective chart review from January 2006 to November 2014 of all subjects aged 18 and older who underwent flexible bronchoscopic cryoprobe extraction. Subjects with obstruction due to benign or malignant neoplasm or airway stenosis were excluded. RESULTS: A total of 38 cryotherapy sessions performed on 30 subjects were identified for inclusion. Cryoprobe extraction was successful in reestablishing airway patency in 32/38 (84%) sessions overall and in 24/26 (92%) for blood clots, 4/6 (67%) for mucous plugging, 2/4 (50%) for foreign bodies, and 2/2 (100%) for plastic bronchitis. Twenty-one of 31 (68%) sessions resulted in improvement in oxygenation or ventilation. There was 1 complication related to sedation. CONCLUSIONS: We conclude that flexible bronchoscopic cryoprobe extraction of blood clots, mucous secretions, plastic bronchitis, and foreign bodies is a safe and effective option. It can be safely performed at the bedside and in many cases eliminates the need for rigid bronchoscopy.

Drug reaction with eosinophilia and systemic symptoms syndrome (DRESS) syndrome associated with azithromycin presenting like septic shock: a case report.

INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms syndrome is a potentially life-threatening cutaneous hypersensitivity reaction characterized by extensive mucocutaneous eruption, fever, hematologic abnormalities including eosinophilia and/or atypical lymphocytosis, and extensive organ involvement. The drugs most often responsible for causing drug reaction with eosinophilia and systemic symptoms syndrome are anticonvulsants, antimicrobial agents and antipyretic or anti-inflammatory analgesics. Although azithromycin is widely prescribed in clinical practice, serious cutaneous reactions from this agent have been rarely described. We report the first adult case of drug reaction with eosinophilia and systemic symptoms syndrome associated with azithromycin. CASE PRESENTATION: A 44-year-old previously healthy Caucasian man with history of tobacco use presented to his primary care physician with fever and productive cough. He was prescribed azithromycin, promethazine hydrochloride and dextromethorphan hydrobromide syrup. One week later, he developed a blistering erythematous rash over both hands, which over the next two weeks spread to involve nearly his entire body surface, sparing only his face. He was admitted to an outside hospital with signs of systemic inflammatory response syndrome and severe sepsis, presumably from a skin infection. Despite aggressive therapy he deteriorated, with worsening diffuse erythema, and was transferred to our institution. He developed multiple organ failure requiring ventilatory and hemodynamic support. Pertinent laboratory studies included a leukocytosis with a white blood cell count of 17.6 × 10(9)/L and 47% eosinophils. A skin biopsy showed evidence of spongiotic lichenoid dermatitis with eosinophils and neutrophils, compatible with a systemic drug-induced hypersensitivity reaction. Our patient was started on high-dose steroids and showed dramatic improvement within 48 hours. CONCLUSIONS: We report the first adult case of drug reaction with eosinophilia and systemic symptoms syndrome associated with azithromycin exposure. Clinicians should be aware of this potentially devastating complication from this commonly prescribed medication.

Cardiovagal autonomic function in HIV-infected patients with unsuppressed HIV viremia.

PURPOSE: HIV infection has been implicated in dysregulation of the autonomic nervous system. METHOD: Cross-sectional study examining the relationship between the presence of persistent detectable HIV viral load with autonomic function, measured by heart rate variability (HRV). Non-virologic suppression (NVS) was defined as having a detectable viral load for at least 3 months prior to autonomic function testing. HRV was measured during the following 4 maneuvers: resting and paced respirations and sustained handgrip and tilt. Inferences on parasympathetic and sympathetic modulations were determined by analyzing time and frequency domains of HRV. RESULTS: 57 participants were enrolled in 3 groups: 22 were HIV-infected participants with HIV virologic suppression (VS; undetectable HIV viral load), 9 were HIV-infected participants who had NVS, and 26 were HIV seronegative controls. There were lower time domain parameters in the HIV-infected group as a whole compared to controls. There were no significant differences in time domain parameters among HIV-infected participants. There were no differences in frequency domain parameters during any of the maneuvers between controls and all HIV-infected participants, nor between the NVS and VS groups. CONCLUSION: There were differences in autonomic function between HIV-infected individuals and HIV seronegative controls, but not between the NVS and VS groups.

Two-step approach of percutaneous closure of a paravalvular leak and atrial septal defect after mitral valve reoperation.

Severe paravalvular leakage following mitral valve replacement, a rare but potentially serious complication, may result in heart failure and significant hemolysis. Reoperation is considered standard of care. However, in selected patients, re-do sternotomy carries excessively high surgical risk. Percutaneous closure of paravalvular leaks has become a viable option for these patients. We present a case of a highly symptomatic 42-year-old male who underwent successful percutaneous closure of two paravalvular leaks and a post-operative atrial septal defect after re-do mitral valve replacement surgery. As access to the left atrium was expected to be difficult following percutaneous atrial septal defect closure, a two-step approach of paravalvular leak closure followed by atrial septal defect closure was chosen. Difficulties of atrial septal defect closure following closure of a paravalvular leak next to the inter-atrial septum will be discussed.

Prevalences and association of ECG findings and cardiovascular risk factor in Shinawatra employees.

OBJECTIVES: There is significant incline trend in cardiovascular disease (CVD) mortality in developing countries such as Thailand and it is also the major contributor to the burden of premature mortality and morbidity throughout the world. In order to have well-stratified primary prevention plan, this study reports the prevalence of Electrocardiogram (ECG) abnormalities, as categorized by ECG Minnesota coding, and the association with major cardiovascular risk factors in Thailand. MATERIAL AND METHOD: In this study, we use the same data from a previous survey at Shinawatra Employee but only subjects with available ECG's were recruited in our study. Standard supine 12-lead ECG data were collected; all amplitude and intervals were measured and entered into a computer manually. Then the ECG was coded according to Minnesota Coding system. The study characteristics, the prevalence of major cardiovascular risk factors and ECG abnormalities were calculated. RESULTS: A total of 1,485 subjects were recruited in this study, 638 (43.0%) were male and 847 (57.0%) were female. The overall mean aged was 34.4 (5.4). The level of major cardiovascular risk factors among men and women respectively were: total Cholesterol 215.6 (41.0) mg/dl (5.6 (1.1) mmol/l), 202.8 (35.3) mg/dl (5.3 (0.9) mmol/l); LDL-cholesterol 139.1 (37.0) (3.6 (1.0) mmol/l), 123.6 (31.9) (3.2 (0.8) mmol/l). Hypercholesterolemia was 65.3%, 49.8%. The mean systolic and diastolic blood pressures were 121.5 (13.9) mmHg and 81.4 (10.5) mmHg, 111.7 (12.2) mmHg and 74.5 (8.6) mmHg; hypertension 21.0%, 4.2%; fasting blood sugar 95.5 (15.8) mg/dl (5.3 (0.9) mmol/l), 88.0 (8.6) mg/dl (5.1 (0.5) mmol/l); diabetes mellitus 3.3%, 0.5%; body mass index 23.5 (3.5) (kg/m2), 21.3 (3.1) (kg/m2); obesity 30.7%, 11.0%; smoking 12.3%, 14.0%. The prevalence of ECG abnormalities, as categorized based on the Minnesota coding criteria, among men and women respectively were: Q/QS wave abnormalities (Code 1) 2.2%, 0.8%; S-T-J segment depression (Code 4) 0.5%, 1.4%; T-wave inversion (Code 5) 1.4%, 9.6%; atrioventricular conduction abnormalities (Code 6) 2.5%, 0.8%; and ventricular conduction abnormalities (Code 7) 0.2%, 0.2%. CONCLUSIONS: This study reports higher prevalence of having major cardiovascular risk factors as compared to previous epidemiological studies in Thailand which should heighten the Ministry of Public Health concern to launch a better stratified preventive plan to combat the rising of coronary artery disease in the future. Moreover, this study is also the first study to report the prevalence of ECG abnormalities, as determined on the basis of the Minnesota coding criteria, and the association between major cardiovascular risk factors and the prevalences of several electrocardiographic findings in adult men and women in Thailand.

Short-term effects of extended-release niacin on endothelial function in HIV-infected patients on stable antiretroviral therapy.

OBJECTIVE: To assess the short-term effects of extended-release niacin (ERN) on endothelial function in HIV-infected patients with low high-density lipoprotein-cholesterol (HDL-c) levels. METHODS: Randomized controlled study to determine the short-term effects of ERN on endothelial function, measured by flow-mediated vasodilation (FMD) of the brachial artery, in HIV-infected adults with low HDL-c. Participants on stable HAART with fasting HDL-c less than 40 mg/dl and low-density lipoprotein-cholesterol less than 130 mg/dl were randomized to ERN or control arms. ERN treatment started at 500 mg/night and titrated to 1500 mg/night for 12 weeks. Controls received the same follow-up but were not given ERN (no placebo). Participants were excluded if they had a history of cardiac disease, uncontrolled hypertension, diabetes mellitus, or were on lipid-lowering medications such as statins and fibrates. Change in FMD was compared between arms with respect to baseline HDL-c. RESULTS: Nineteen participants were enrolled: 89% men, median age 50 years, 53% white/non-Hispanic, median CD4 cell count 493 cells/microl, and 95% of them had HIV RNA below 50 copies/ml. Participants receiving ERN had a median HDL-c (interquartile range) increase of 3.0 mg/dl (0.75 to 5.0) compared with -1.0 mg/dl in controls (-6.0 to 2.5), a P value is equal to 0.04. The median change in FMD was 0.91% (-2.95 to 2.21) for ERN and -0.48% (-2.65 to 0.98) for controls (P = 0.67). However, end of study FMD for ERN was significantly different from controls after adjusting for baseline differences in FMD and HDL-c, 6.36% (95% confidence interval 4.85-7.87) and 2.73% (95% confidence interval 0.95-4.51) respectively, a P value is equal to 0.048. CONCLUSION: This pilot study demonstrated that short-term niacin therapy could improve endothelial function in HIV-infected patients with low HDL-c.