An Aggressive Form of Medullary Thyroid Carcinoma-Melanocytic Subtype: A Case Report.

Medullary thyroid cancer (MTC) represents a small proportion of thyroid cancers. In MTC, melanin production is extremely uncommon. Few case reports have documented this rare variant, and follow-up on these cases has been very limited. Our case studies a 51-year-old female who initially presented with goiter. This tumor recurred multiple times despite surgery with rapid growth and poor response to radiotherapy. Microscopic examination showed high-grade malignant neoplasm with lymphocytic differentiation. Immunohistochemical studies were diffusely positive for S100, SOX10, and Melan-A. Histology confirmed melanocytic medullary carcinoma that had undergone a high-grade transformation with loss of epithelial and neuroendocrine expression. Due to the scarcity and rarity of this subtype, further evaluation and case studies are needed for further categorization and prognostication.

Continuous positive airway pressure usage in hospitalized patients with known obstructive sleep apnea: discrepancy between admission pressure settings and laboratory-determined settings.

PURPOSE: The aim of this study was to determine the frequency and predictors of correctly initiated continuous positive airway pressure (CPAP) settings on the initial night of hospitalization in patients with known obstructive sleep apnea-hypopnea syndrome (OSAHS). METHODS: Hospital records of all patients who underwent an outpatient therapeutic polysomnogram (PSG) at our institution between January 2005 and December 2010 were retrospectively reviewed. Data collected included initial CPAP settings on hospital admission, latency to hospitalization (from sleep study), hospital length of stay, demographic variables, and PSG variables. RESULTS: One hundred seventy subjects were included in the analysis: 51 % were male, average age (±SD) was 55.3 ± 13.7 years, and body mass index was 43.7 ± 10.4 kg/m2. OSAHS was generally severe (apnea-hypopnea index (AHI) 52.8 ± 37.3 event/h). Mean CPAP setting during in-laboratory titration was 11.1 ± 3.1 cm H2O and during the first night of hospitalization was 9.5 ± 2.8 cm H2O (p < 0.0001). Of 170 subjects, only 71 (42 %) received the correct laboratory-derived CPAP setting on the first night of hospitalization. In a multivariable logistic regression analysis, higher body mass index (BMI), lower CPAP level determined during PSG, and shorter latency (months) between PSG and hospitalization were associated with receiving the correct CPAP setting during the first night of hospitalization: Each 1 kg/m2 increase in BMI was associated with a 7 % increase odds of receiving the correct CPAP setting during the first night of hospitalization (OR 1.07, 95 % CI 1.02-1.12), while each 1 cm H2O increase in CPAP during PSG and each 1 month longer latency between PSG and hospitalization was associated with a 15 and 7 %, respectively, decrease in the odds of receiving the correct CPAP setting during the first night of hospitalization (CPAP OR 0.85, 95 % CI 0.74-0.97 and latency OR 0.93, 95 % CI 0.90-0.97). There was no in-hospital mortality, and correct CPAP settings did not affect hospital length of stay. CONCLUSIONS: Among patients admitted to the hospital, a correct, laboratory-derived CPAP setting is infrequently prescribed during the first night of hospitalization. Predictors for correctly ordering CPAP include latency from the time of in-laboratory CPAP titration, BMI, and laboratory-derived CPAP level.

Polysomnographic Measurement of Sleep Duration and Bodily Pain Perception in the Sleep Heart Health Study.

STUDY OBJECTIVES: To determine whether total sleep time (TST) and specific sleep stage duration are associated with bodily pain perception and whether sex, age, or subjective sleepiness modifies this relationship. METHODS: Data from adults ages 39-90 y (n = 5,199) who took part in the Sleep Heart Health Study Exam 1 were analyzed. TST, rapid eye movement (REM) sleep time, and slow wave sleep (SWS) time were measured by unattended, in-home nocturnal polysomnography. Bodily pain perception was measured via the Short Form-36 questionnaire bodily pain component. We used logistic regression to examine associations between total and individual sleep stage durations and bodily pain perception controlling for age, sex, race, body mass index, apnea-hypopnea index, antidepressant use, and important cardiovascular conditions (smoking [pack-years], history of diabetes, and history of percutaneous coronary intervention and/or coronary artery bypass graft). RESULTS: In the fully adjusted model, REM sleep time and SWS time were not associated with "moderate to severe pain," whereas TST was: Each 1-h decrement in TST was associated with a 7% increased odds of "moderate to severe pain" (odds ratio 1.07, 95% confidence interval 1.002, 1.14). Due to modification of the association between SWS time and "moderate to severe pain" by sex (P for interaction = 0.01), we performed analyses stratified by sex: Each 1-h decrement in SWS time was associated with a 20% higher odds of "moderate to severe pain" among men (odds ratio 1.20, 95% confidence interval 1.03-1.42) whereas an association was not observed among women. CONCLUSIONS: Shorter TST among all subjects and shorter SWS time in men was associated with "moderate to severe pain." REM sleep time was not associated with bodily pain perception in this cohort.

Severe hypoxemic respiratory failure: part 1--ventilatory strategies.

Approximately 16% of deaths in patients with ARDS results from refractory hypoxemia, which is the inability to achieve adequate arterial oxygenation despite high levels of inspired oxygen or the development of barotrauma. A number of ventilator-focused rescue therapies that can be used when conventional mechanical ventilation does not achieve a specific target level of oxygenation are discussed. A literature search was conducted and narrative review written to summarize the use of high levels of positive end-expiratory pressure, recruitment maneuvers, airway pressure-release ventilation, and high-frequency ventilation. Each therapy reviewed has been reported to improve oxygenation in patients with ARDS. However, none of them have been shown to improve survival when studied in heterogeneous populations of patients with ARDS. Moreover, none of the therapies has been reported to be superior to another for the goal of improving oxygenation. The goal of improving oxygenation must always be balanced against the risk of further lung injury. The optimal time to initiate rescue therapies, if needed, is within 96 h of the onset of ARDS, a time when alveolar recruitment potential is the greatest. A variety of ventilatory approaches are available to improve oxygenation in the setting of refractory hypoxemia and ARDS. Which, if any, of these approaches should be used is often determined by the availability of equipment and clinician bias.

Analysis of hospitalizations for COPD exacerbation: opportunities for improving care.

BACKGROUND: Little is known about the actual treatment of patients with chronic obstructive pulmonary disease (COPD), either in the inpatient or outpatient settings. We hypothesized that there are substantial opportunities for improvement in adherence with current guidelines and recommendations. METHODS: We reviewed the medical records of all patients hospitalized with acute exacerbation of COPD between January 2005 and December 2006 at 5 New York City hospitals. RESULTS: There were 1285 unique patients with 1653 hospitalizations. Of these 1653, 83% were for patients with a prior history of COPD and 368 (22%) represented repeat admissions during our study period. The majority were treated during their hospitalization with a combination of systemic steroids (85%), bronchodilators (94%) and antibiotics (80%). There were 59 deaths (3.6%). Smoking cessation counseling was offered to 48% of active smokers. Influenza and pneumococcal vaccines were administered to half of eligible patients. On discharge, only 46.0% were prescribed maintenance bronchodilators and 24% were not prescribed any inhaled therapy. Even in the 226 unique patients (17.6%) readmitted at least once during course of the study, on discharge only 44.7% were prescribed maintenance bronchodilators and 23% were not prescribed any regular inhaled therapy. CONCLUSIONS: Patients hospitalized with acute exacerbation of COPD generally receive adequate hospital care, but there may be opportunities to improve care pharmacologically and with smoking cessation counseling and vaccination during and after hospitalization.

Tracheobronchial manifestations of aspergillosis.

OBJECTIVE: The purpose of this article is to describe 3 patients each of whom developed a different form of tracheobronchial aspergillosis. METHODS: We describe our clinical experience with 3 patients who developed significant respiratory symptoms secondary to Aspergillus infection. All patients were followed closely until there was resolution of pulmonary problems or they succumbed to respiratory insufficiency. RESULTS: The first patient had asthma and her clinical and bronchoscopic findings were compatible with the diagnosis of mucoid impaction syndrome caused by Aspergillus. Response to therapy was excellent with complete recovery. The second and third patients had what we believe was tracheobronchial pseudomembranous aspergillosis. The precise reason for this complication in the second patient is unknown. The third patient was immunosuppressed and developed tracheobronchial aspergillosis. Despite aggressive therapy, both of these patients died. Diagnostic bronchoscopy was helpful in detecting the airway abnormalities and for obtaining respiratory specimens for culture. CONCLUSIONS: These cases show the diverse tracheobronchial manifestations of Aspergillus species. Diagnostic bronchoscopy was helpful in the diagnosis of airway involvement by aspergillus.

Clavicular tuberculosis following trivial trauma.

Tuberculosis of the clavicular bone is a very rare clinical entity, with limited cases reported in the United States. Furthermore, sparing of the sternoclavicular joint is exceedingly unusual. A literature review of the prevalence of clavicular tuberculosis identified fewer than 80 cases reported since the discovery of the tubercular bacillus, over a century ago. To our knowledge, there have been no cases reported over the last decade in the United States. A rare case of tuberculous osteomyelitis of the clavicle in an immunocompetent patient who presented with swelling of the upper chest is reported.

Transthyretin as a marker to predict outcome in critically ill patients.

BACKGROUND: A determination of serum Transthyretin (TTR, Prealbumin) level is an objective method of assessing protein catabolic loss of severely ill patients and numerous studies have shown that TTR levels correlate with patient outcomes of non-critically ill patients. We evaluated whether TTR level correlates with the prevalence of PEM in the ICU and evaluated serum TTR level as an indicator of the effectiveness of nutrition support and the prognosis in critically ill patients. METHODS: We studied PEM prevalence in 118 patients admitted to a community hospital's medical intensive care unit and the association between TTR, low albumin (ALB) concentration and high-risk disease (HRD), i.e., sepsis, inability to take in oral nutrients, etc. Serum TTR was measured on the day of admission, day 3 and day 7 of their ICU stay. APACHE II and SOFA score was assessed on the day of admission and the nutritional status and nutritional requirement was assessed for their entire ICU stay. Patients were divided into three groups based on initial TTR level and the outcome analysis was performed for APACHE II score, SOFA score, ICU length of stay, hospital length of stay, and mortality. RESULTS: TTR showed excellent concordance with patients classified with PEM or at high malnutrition risk, and followed for 7 days, it is a measure of the metabolic burden. TTR levels decline from day 1 to day 7 in spite of providing nutritional support. Patients were classified in 3 categories with respect to the level of TTR: more than 170 mg/L, twenty-five patients (group 3); 100-170 mg/L, forty-eight patients (group 2); less than 100 mg/L, forty-five patients (group 1). TTR level correlated with ICU length of stay, hospital length of stay, and APACHE II score, and predicts mortality. CONCLUSIONS: TTR identified patients at highest risk for metabolic losses associated with stress hypermetabolism as serum TTR levels did not respond early to nutrition support because of the delayed return to anabolic status. It is particularly helpful in removing interpretation bias, and it is an excellent measure of the systemic inflammatory response concurrent with a preexisting state of chronic inanition.

Dyspnea with slow-growing mass of the left hemithorax.

We report a case of a 65-year-old male patient who presented with gradually worsening dyspnea over 2 years. History was significant for smoking and the absence of any hazardous occupational exposure. The clinical findings at presentation included absent breath sounds and stony dullness on the left side, with tracheal deviation contralaterally and clubbing. A chest roentgenogram showed a left-sided opacity occupying almost the entire left hemithorax. A subsequent CT scan of the chest revealed an intrathoracic, extrapulmonary lesion producing a mediastinal shift. Surgical resection of the mass was performed, and pathology, along with immunohistochemical studies positive for CD34 and negative for epithelial markers, confirmed the diagnosis of solitary fibrous tumor of the pleura (SFTP). SFTP is a rare neoplasm, and diagnosis is often difficult. Suspicion of SFTP should arise in the setting of a patient presenting with a paucity of symptoms (except in the case of an accompanying paraneoplastic syndrome), the absence of exposure to asbestos, and a large mass with sharp margins and encapsulation seen on a chest radiograph.

Update on pleural diseases--2007.

BACKGROUND: New information is available on pleural diseases. The authors selected articles to make recommendations on diagnostic and treatment aspects of pleural diseases. MATERIALS AND METHODS: Eleven articles published in the English language between 2004 and 2007 were chosen. The basis of selection of the articles was the impact on daily practice, change in prior thinking of a disease process or specific treatment modality, as well as proper design and execution of the study. 5-Amino-laevulinic acid with fluorescent light combined with white light may allow further diagnostic yield in undiagnosed pleural disease. FDG-PET may allow prognostication of patients with pleural tumors. Utilizing ultrasound by trained Emergency Department physicians is a rapid and effective technique to evaluate non-traumatic pleural effusions in symptomatic patients. Serum osteopontin levels may distinguish patients exposed to asbestos with benign disease from those with pleural mesothelioma. Administration of streptokinase in patients with empyema does not need for surgical drainage, length of hospital stay, or mortality as compared to conventional treatment with chest tube drainage and intravenous antibiotics. Silver nitrate may be an alternative agent to talc for producing pleurodesis. Routine use of graded talc (50% particles greater than 25 microns) is recommended to reduce the morbidity associated with talc pleurodesis. Study design does not permit us to conclude that aspiration of spontaneous pneumothorax is as effective as chest tube drainage. Pleural catheter may prove to be an important palliative modality in treating debilitated patients or patients with trapped lung who show symptomatic improvement with drainage; however, at the present time, these catheters cannot be considered a first line treatment option for patients with malignant pleural effusion. One of the studies reviewed showed no significant difference in tract metastasis in patients with malignant mesothelioma undergoing an invasive pleural procedure with or without irradiation to the procedure site. However, the design of the trial does not allow us to make this conclusion at the present time.

Amniotic fluid embolism.

Amniotic fluid embolism is a rare syndrome that can have debilitating and lethal consequences. It is a difficult and somewhat intangible diagnosis that warrants a high index of suspicion by physicians. Prompt and aggressive supportive treatment is required to lessen an otherwise dismal outcome, which may include death and permanent disability. This article provides an account of the protean clinical features, pathogenesis, and principles involved in treatment.

New therapies: plasmapheresis, intravenous immunoglobulin, and monoclonal antibodies.

Rheumatologic emergencies may pose a serious threat to life, and the treatment of patients with these illnesses continues to be challenging. In the last decade extensive animal and human research has led to development of new therapies. Considerable progress has been made in the therapy for RA. Newly developed biologic therapies have shown promising results in clinical studies, and two agents have already been approved by the FDA. These drugs are currently available for therapy and are under close postmarketing scrutiny to assess long-term efficacy and safety. Similar therapies are under investigation for SLE. Plasmapheresis, once used for many diseases, is now restricted mostly to conditions for which its use has been shown to be beneficial in randomized, controlled studies. Immunoadsorption is used to target specific disease-producing pathogens for removal during extracorporeal therapy. Evidence is accumulating for the use of IVIGs in several immune-mediated conditions. The outlook for some emergencies continues be grim, however, and various therapies are used based on evidence from anecdotal case reports and case series. The new therapies are relatively safe, but careful monitoring is needed, because there is potential for serious adverse events.