Pulmonary involvement in patients with thrombotic thrombocytopenic purpura.

OBJECTIVES: To determine the frequency of pulmonary involvement in patients with thrombotic thrombocytopenic purpura (TTP). METHODS: The experience of the Oklahoma TTP-HUS (hemolytic-uremic syndrome) Registry, a population-based cohort of consecutive patients without selection or referral bias, 1995-2012, was analyzed. Evidence for pulmonary involvement in patients with TTP was also documented with a systematic review of published reports. RESULTS: Only one of 74 Registry patients with acquired severe ADAMTS13 deficiency (activity <10%) had clinically important pulmonary involvement (transient PaO2 , 42 mm Hg; arterial O2 saturation, 78%; normal chest X-ray). No clinically important pulmonary involvement occurred in the remaining 73 patients. The systematic review identified 144 articles with search terms for TTP and pulmonary involvement; seven, published 1978-2002, had evaluable individual patient data. Five articles described single patients; only one patient was documented to have severe acquired ADAMTS13 deficiency; in none of the five patients was pulmonary involvement clearly related to TTP. Two articles were case series of seven patients each; no patients had ADAMTS13 activity measured. Each of the 14 patients had potential etiologies other than TTP for pulmonary involvement. CONCLUSIONS: Only one of 74 patients in the Oklahoma Registry had clinically important pulmonary involvement. A systematic review of published reports documented no clear evidence for pulmonary involvement resulting from TTP. Clinically important pulmonary involvement may be rare in patients with TTP because (i) pulmonary microvasculature may be inherently resistant to the formation of platelet thrombi and (ii) pulmonary function can be maintained in spite of multiple microvascular thrombi.

Reversible Blindness as Presenting Manifestation of Severe Diabetic Ketoacidosis.

The presenting symptoms of diabetic ketoacidosis (DKA) include abdominal pain, polyuria and nausea. Diabetes has well known chronic ocular complications like glaucoma, cataracts and retinopathy. We report a case of reversible blindness as a presenting manifestation of DKA that has been reported in literature only 3 times previously. Our objective is to highlight a rare manifestation of a common disease. A 59-year-old male presented with painless vision loss for 3 days and was found to have DKA. The blindness was completely reversed with insulin and bicarbonate treatment. The dramatic presentation and reversibility of blindness was found to be intimately tied with the pH of the patient's serum. Our report gives mechanistic insight for this interesting condition. Clinicians should be aware of reversible blindness as a complication of DKA. Timely correction of the severe acidosis and other metabolic disturbances of DKA may be instrumental in preventing permanent vision loss.

Bronchoscopic advances in the management of aerodigestive fistulas.

Malignant aerodigestive fistula (ADF) is an uncommon condition complicating thoracic malignancies. It results in increased morbidity and mortality and warrants therapeutic intervention. The management approach depends on symptoms, configuration, location, and extent of the fistula. This article will discuss the therapeutic considerations in the management of ADF.

Activated protein C improves survival in severe sepsis patients with elevated troponin.

OBJECTIVE: Multiple studies in sepsis have demonstrated that elevated troponin is associated with poor outcome. The elevated troponin in this situation is thought to be secondary to microthrombi. We hypothesized that recombinant human activated protein C (APC) treatment would improve outcomes in severe sepsis patients who have elevated troponin. METHODS: Patients with severe sepsis by consensus criteria in a university ICU were divided into a troponin elevated group (cTnI+) and a normal troponin (cTnI(-)) group. Outcome was compared using Fisher's exact test. APACHE[Symbol: see text]II and MODS were calculated by standard methods. PATIENTS: We identified 105 patients with severe sepsis and troponin measured, of which 48 (46%) were in the cTnI+ group. The two groups were similar in terms of age and other comorbid conditions. RESULTS: APACHE II (28+/-8 vs. 25+/-8) was slightly higher and MODS (11+/-4 vs. 9+/-3) was significantly higher in the cTnI+ group. Mortality was 52% (25/48) in cTnI+ group and 30% (17/57) in cTnI(-) group. Mortality was 30% in cTnI+ patients treated with APC and 72% in untreated cTnI+ patients. CONCLUSIONS: Patients with severe sepsis who have elevated troponin have increased mortality. In patients with severe sepsis who have elevated troponin, treatment with APC improves outcome. Further study is needed to determine whether troponin can serve as a simple, readily available marker to identify which patients with severe sepsis will benefit from APC.

False-negative PET scan with bronchioloalveolar carcinoma: an important diagnostic caveat.

Positron emission tomography (PET) is becoming widely accepted as a powerful diagnostic tool for the diagnosis of lung cancer, but it has very poor sensitivity for the detection of bronchioloalveolar carcinoma (BAC) and adenocarcinoma with BAC pattern, the less common form of pulmonary neoplasia. We present a case of a patient with a negative PET scan who might have been followed by observation but was found to have bronchioloalveolar carcinoma at thoracotomy. PET has a reported sensitivity of over 98% in most series but misses almost two-thirds of BAC lesions, which might delay invasive testing and early diagnosis of this potentially lethal cancer. Although this diagnostic limitation has been well reported in the radiology literature, the high reported sensitivity and sensitivity can give clinicians a false sense of security with negative PET scans of lung nodules. The usual risk factors for bronchogenic carcinoma are less reliable for these subtypes of non-small-cell lung cancer; thus, clinicians need to have a high index of suspicion for BAC and exercise caution when making decisions on the basis of PET.

Whole lung lavage-technical details, challenges and management of complications.

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with resulting hypoxemia and reduced lung function. Whole lung lavage (WLL) to physically remove the proteinaceous material from the affected lung is the standard treatment. Since its original description in 1964, there have been increasing numbers of WLL procedures done worldwide and the technique has been variously refined and modified. When done in experienced centers, WLL provides long lasting benefit in the majority of patients. It is considered safe and effective. There are no guidelines standardizing the procedure. Our preferred method is to lavage one lung at a time, with the patient supine, filling to functional residual capacity (FRC) and repeating cycles of drainage and instillation with chest percussion until the effluent is clear. The aim of this article is to provide a detailed description of the technique, equipment needed and logistic considerations as well as providing a physiologic rationale for each step of WLL. We will also review the available data concerning variations of the technique described in the literature.

Stenting of critical tracheal stenosis with adjuvant cardio-pulmonary bypass.

Severe and critical central airway obstruction causing impaired ventilation and/or oxygenation can impose tremendous challenges on the interventional pulmonologist. Near total airway obstruction can rapidly evolve into potentially fatal complete airway occlusion during bronchoscopic airway manipulation under moderate sedation; as well as during the induction of the general anesthesia. Although there are currently interventional pulmonary procedures available to tackle the critical airway obstruction in extreme situations, cardio-pulmonary bypass should be considered prior to the intervention to maintain the adequate gas exchange during the procedure. Orotracheal intubation with mechanical ventilation in this situation can be fatal itself if the obstructing airway lesion functions as a "one way valve" allowing air to follow distally during inspiration but impeding expiratory flow leading to gas trapping, high intrathoracic pressure, tension pneumothorax, and ultimately a cardiac arrest.

Management of oral antiplatelet agents and anticoagulation therapy before bronchoscopy.

Although, bronchoscopy is a relatively safe procedure, small amount of bleeding in the airway can have serious consequences. Careful consideration of the risks of diagnostic and therapeutic bronchoscopic intervention can help minimize potential complications. With increasing number of patients using antiplatelet and anticoagulation therapies, strategies for minimizing thromboembolic and operative bleeding events need to be included in the risk and benefit analyses. Growing evidence suggests that aspirin is safe and does not increase bleeding during bronchoscopy. In addition, despite small studies reporting that it may be safe to perform bronchoscopic procedures that have low risk for bleeding such as endobronchial ultrasound with transbronchial needle aspiration on clopidogrel, it is still recommended to hold it for 7 days prior to performing elective bronchoscopy. It is recommended to hold vitamin K antagonist, as well as new oral anticoagulation agents prior to bronchoscopy. The timing for pre-procedural discontinuation of anticoagulation therapy and the decision to bridge depend on the agent used, the renal function and the thromboembolic risk. In this review article, we will discuss available data regarding management of anticoagulation and antiplatelet therapy as it applies to bronchoscopic procedures.

Radial Probe Ultrasound-Guided Cryobiopsy.

Fluoroscopy-guided transbronchial forceps biopsy has a low diagnostic yield in patients with radiographic suspicion of interstitial lung disease. Cryobiopsy has a higher diagnostic yield likely due to preserved lung architecture and larger biopsies; however, there is an increased risk of major airway bleeding and pneumothorax. Simultaneous use of endobronchial balloon blocker allows for containment of bleeding after cryobiopsy to the affected lobe. In the current article we describe use of radial ultrasound in identification of a target lung parenchyma without a major blood vessel adjacent to distal bronchi. After fluoroscopic marking of the selected airway a 3 second cryobiopsy was performed after localization of cryoprobe. Simultaneous use of radial ultrasound and fluoroscopy can possibly decrease bleeding complication associated with cryobiopsy in patients with suspected interstitial lung disease.

Fibrous dysplasia protuberans in a patient with McCune-Albright syndrome.

Fibrous dysplasia protuberans refers to a benign fibro-osseous exophytic mass originating in the intra-medullary cavity of an adjacent bone. A case of fibrous dysplasia protuberans is hereby presented, manifesting as an exudative pleural effusion in a 49 years old male with history of McCune-Albright syndrome. This case represents the first reported case of fibrous dysplasia protuberans in a patient with McCune-Albright syndrome. Without a high index of suspicion, detailed radiographic imaging and meticulous histological examination, this benign bony excrescence is likely to be misdiagnosed, especially in a patient with no previous history of fibrous dysplasia.

Review and Outcome of Prolonged Cardiopulmonary Resuscitation.

The maximal duration of cardiopulmonary resuscitation (CPR) is unknown. We report a case of prolonged CPR. We have then reviewed all published cases with CPR duration equal to or more than 20 minutes. The objective was to determine the survival rate, the neurological outcome, and the characteristics of the survivors. Measurements and Main Results. The CPR data for 82 patients was reviewed. The median duration of CPR was 75 minutes. Patients mean age was 43 ± 21 years with no significant comorbidities. The main causes of the cardiac arrests were myocardial infarction (29%), hypothermia (21%), and pulmonary emboli (12%). 74% of the arrests were witnessed, with a mean latency to CPR of 2 ± 6 minutes and good quality chest compression provided in 96% of the cases. Adjunct therapy included extracorporeal membrane oxygenation (18%), thrombolysis (15.8%), and rewarming for hypothermia (19.5%). 83% were alive at 1 year, with full neurological recovery reported in 63 patients. Conclusion. Patients undergoing prolonged CPR can survive with good outcome. Young age, myocardial infarction, and potentially reversible causes of cardiac arrest such as hypothermia and pulmonary emboli predict a favorable result, especially when the arrest is witnessed and followed by prompt and good resuscitative efforts.

Pancreaticopericardial Fistula: A Case Report and Literature Review.

Purpose. Pancreaticopericardial fistula (PPF) is an extremely rare complication of acute or chronic pancreatitis. This paper presents a rare case of PPF and provides systematic review of existing cases from 1970 to 2014. Methods. A PubMed search using key words was performed for all the cases of PPF from January 1970 to December 2014. Fourteen cases were included in the study. The cases were reviewed for demographic characteristics, diagnostic modalities, and treatment. Descriptive analysis of these variables was performed. Results. Median age was 43 years. 78% were known alcoholics and 73.3% had chronic pancreatitis. Dyspnea was present in 78%. Cardiac tamponade was present in 53%; 75% of patients had known chronic pancreatitis (RR = 0.74). Surgery was associated with best treatment outcomes and 50% of patients who underwent endoscopic treatment survived. Conclusion. PPF is a rare disease. This paper indicates that acute cardiac tamponade in patients with history of alcoholism and chronic pancreatitis could be a sign of an existing pancreaticopericardial fistula and early surgical intervention could be life-saving.

Granulocytic sarcoma manifesting as multiple skeletal lesions.

Granulocytic sarcoma is an extramedullary collection of myeloblasts that is usually associated with acute or chronic myeloid leukemia. It can be found in any location throughout the body; however, multifocal skeletal involvement is extremely rare. This report describes a case of granulocytic sarcoma in a 33-year-old man, manifesting as multiple skeletal lesions along with signs of cord compression without any preceding history of myeloid leukemia. Cytogenetic studies revealed t(8,9) translocation, which has never been reported in association with granulocytic sarcoma. The prognostic significance of this finding is unknown. This case report underscores the importance of considering granulocytic sarcoma in the differential diagnosis of spinal tumors, since the tumor may occur before other manifestations of myeloid leukemia are evident.

Transudative chylothorax in a patient with liver cirrhosis: A rare association.

Chylothorax is an unusual type of pleural effusion which results from the accumulation of chyle in the pleural cavity. High triglyceride content and presence of chylomicrons in the chyle give this fluid a characteristic milky appearance. Chylothorax most commonly results from the obstruction of the thoracic duct by a malignant lesion or from its traumatic disruption. Liver cirrhosis is an uncommon and frequently underappreciated cause of chylothorax. Pleural effusion in chylothorax is typically described as a lymphocytic predominant, exudative type and it is exceedingly rare to encounter a transudative type of chylothorax. To date, very few cases of transudative chylothoraces have been described in the literature, most commonly in association with liver cirrhosis. Only a limited range of other clinical settings have been linked to transudative chylothorax and timely recognition of these associations can prevent unnecessary, expensive and sometimes invasive workup in this patient population.

STOP-BANG questionnaire as a screening tool for diagnosis of obstructive sleep apnea by unattended portable monitoring sleep study.

The Snoring, Tiredness, Observed apnea, high blood Pressure (STOP)-Body mass index (BMI), Age, Neck circumference, and Gender (BANG) questionnaire is a well validated screening tool for diagnosis of Obstructive sleep apnea (OSA) by an in- lab sleep study. However, performance of STOP-BANG as a screening tool for diagnosis of OSA in patients undergoing portable monitoring (PM) sleep study has not been well validated. We conducted a retrospective chart review of patients older than 18 years who had unattended portable monitoring sleep study done at a VA medical center between June 2012 and October 2014. STOP-BANG questionnaire and Epworth sleepiness scale (ESS) were routinely done prior to study. Sensitivity, specificity, and positive predictive value (PPV) various STOP-BANG score thresholds were calculated for diagnosis of OSA defined by Apnea Hypopnea Index (AHI) ≥5. Out of 502 unattended portable monitoring sleep studies, there were 465 males and 37 females. STOP-BANG thresholds of ≥2 and 3 have high sensitivity of 99.8 and 98.9 %, respectively, but very low specificity. Higher score thresholds of ≥7 and 8 have high specificity of 95 and 98.3 %, and PPV of 98.1 and 98.5 %, respectively, but very low sensitivity. A threshold of ≥7 in patients with BMI ≥30 was 100 % specific. The false negative rate for unattended portable monitoring sleep study compared to in-lab study was 80 %. STOP-BANG score thresholds of ≥7 and 8 are highly specific and have high PPV and therefore can potentially reduce need of diagnostic sleep studies in selected patients. Score thresholds of ≤2 or 3 are highly sensitive for AHI ≥5 by unattended portable monitoring sleep study but have high false negative rates. Therefore, in-lab sleep study should be performed to rule out OSA.

Sleep, immunity and inflammation in gastrointestinal disorders.

Sleep disorders have become a global issue, and discovering their causes and consequences are the focus of many research endeavors. An estimated 70 million Americans suffer from some form of sleep disorder. Certain sleep disorders have been shown to cause neurocognitive impairment such as decreased cognitive ability, slower response times and performance detriments. Recent research suggests that individuals with sleep abnormalities are also at greater risk of serious adverse health, economic consequences, and most importantly increased all-cause mortality. Several research studies support the associations among sleep, immune function and inflammation. Here, we review the current research linking sleep, immune function, and gastrointestinal diseases and discuss the interdependent relationship between sleep and these gastrointestinal disorders. Different physiologic processes including immune system and inflammatory cytokines help regulate the sleep. The inflammatory cytokines such as tumor necrosis factor, interleukin-1 (IL-1), and IL-6 have been shown to be a significant contributor of sleep disturbances. On the other hand, sleep disturbances such as sleep deprivation have been shown to up regulate these inflammatory cytokines. Alterations in these cytokine levels have been demonstrated in certain gastrointestinal diseases such as inflammatory bowel disease, gastro-esophageal reflux, liver disorders and colorectal cancer. In turn, abnormal sleep brought on by these diseases is shown to contribute to the severity of these same gastrointestinal diseases. Knowledge of these relationships will allow gastroenterologists a great opportunity to enhance the care of their patients.

Bronchial responsiveness in patients with restrictive spirometry.

BACKGROUND: Improvement in PFT after bronchodilators is characteristic of obstructive airway diseases such as COPD. However, improvement in patients with restrictive pattern is occasionally seen. We aim to determine the clinical significance of a bronchodilator responsive restrictive defect. METHODS: Patients with restrictive spirometry and a bronchodilator study were identified at the University of Oklahoma and Oklahoma City VAMC between September 2003 and December 2009. Restriction was defined as a decreased FVC and FEV1, with normal FEV1/FVC. Responsiveness to bronchodilators was defined as an improvement in FEV1 and/or FVC of at least 12% and 200 mL. Patients with lung volume measurements had their clinical and radiographic records reviewed. RESULTS: Twenty-one patients were included in the study. Most were current or ex-smokers, with most being on bronchodilators. The average FVC and FEV1 were 65 ± 11% and 62 ± 10% of the predicted, respectively. Most patients (66%) had a normal TLC, averaging 90 ± 16% of the predicted. RV, RV/TLC, and the TLC-VA values strongly suggested an obstructive defect. CONCLUSIONS: Reversible restrictive pattern on spirometry appears to be a variant of obstructive lung disease in which early airway closure results in air trapping and low FVC. In symptomatic patients, a therapeutic trial of bronchodilators may be beneficial.