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Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years.

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Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years.

Pediatr Blood Cancer. 2013 Feb;60(2):175-84

Authors: Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, Egeler RM, Janka G, Micic D, Rodriguez-Galindo C, Van Gool S, Visser J, Weitzman S, Donadieu J, Euro Histio Network

Abstract
These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented.

PMID: 23109216 [PubMed - indexed for MEDLINE]

Why do patients with interstitial lung diseases fail in the ICU? A 2-center cohort study.

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Why do patients with interstitial lung diseases fail in the ICU? A 2-center cohort study.

Respir Care. 2013 Mar;58(3):525-31

Authors: Güngör G, Tatar D, Saltürk C, Cimen P, Karakurt Z, Kirakli C, Adigüzel N, Ediboglu O, Yilmaz H, Moçin OY, Balci M, Yilmaz A

Abstract
BACKGROUND: Admitting patients with interstitial lung disease (ILD) to the ICU is controversial, due to their associated high mortality when they require invasive mechanical ventilation. We aimed to determine the risk factors for mortality in ILD patients requiring ICU support due to acute respiratory failure.
METHODS: An observational cohort study was performed in 2 chest diseases teaching hospitals. We included all ILD patients with acute respiratory failure admitted between 2008 and 2010. Subject demographics, noninvasive ventilation (NIV) and invasive ventilation use, and mortality were obtained from medical records. Subjects receiving NIV were divided based on their continuous or non-continuous demand for NIV. NIV failure was defined as intubation for invasive ventilation, or death during NIV. Cox regression analysis was used to determine the hazard ratio for NIV failure.
RESULTS: We enrolled 120 subjects: 71 male, median age 66 years. The types of ILD were idiopathic pulmonary fibrosis (n = 96), collagen vascular disease (n = 10), silicosis (n = 9), drug induced (n = 3), and eosinophilic pneumonia (n = 2). The median (IQR) Acute Physiology and Chronic Health Evaluation (APACHE II) score was 24 (19-31), and 75 (62.5%) subjects received NIV on ICU admission, 47 (62.7%) of whom needed continuous NIV. The NIV failure rate was 49.3% (n = 37). The mortality rates of continuous NIV, non-continuous NIV, invasive ventilation, and total ICU were 61.7% (29/47), 10.7% (3/28), 89.7% (61/68), 60% (72/120), respectively. APACHE II > 20 and continuous NIV demand indicated significant risk for NIV failure: hazard ratio 2.77 (95% CI 1.19-6.45), P < .02, and 5.12, (1.44-18.19), P < .01, respectively.
CONCLUSIONS: Because of higher mortality, physicians should consider invasive ventilation cautiously in the ICU management of ILD patients with acute respiratory failure. NIV may be an option in less severely ill patients with APACHE II score < 20.

PMID: 23443285 [PubMed - in process]

Diagnosis and treatment of connective tissue disease-associated interstitial lung disease.

Diagnosis and treatment of connective tissue disease-associated interstitial lung disease.

Chest. 2013 Mar 1;143(3):814-24

Authors: Vij R, Strek ME

Abstract
Interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Although the various CTDs associated with ILD often are considered together because of their shared autoimmune nature, there are substantial differences in the clinical presentations and management of ILD in each specific CTD. This heterogeneity and the cross-disciplinary nature of care have complicated the conduct of prospective multicenter treatment trials and hindered our understanding of the development of ILD in patients with CTD. In this update, we present new information regarding the diagnosis and treatment of patients with ILD secondary to systemic sclerosis, rheumatoid arthritis, dermatomyositis and polymyositis, and Sjögren syndrome. We review information on risk factors for the development of ILD in the setting of CTD. Diagnostic criteria for CTD are presented as well as elements of the clinical evaluation that increase suspicion for CTD-ILD. We review the use of medications in the treatment of CTD-ILD. Although a large, randomized study has examined the impact of immunosuppressive therapy for ILD secondary to systemic sclerosis, additional studies are needed to determine optimal treatment strategies for each distinct form of CTD-ILD. Finally, we review new information regarding the subgroup of patients with ILD who meet some, but not all, diagnostic criteria for a CTD. A careful and systematic approach to diagnosis in patients with ILD may reveal an unrecognized CTD or evidence of autoimmunity in those previously believed to have idiopathic ILD.

PMID: 23460159 [PubMed - in process]

Sleep in hypercapnic critical care patients under noninvasive ventilation: conventional versus dedicated ventilators.

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Sleep in hypercapnic critical care patients under noninvasive ventilation: conventional versus dedicated ventilators.

Crit Care Med. 2013 Jan;41(1):60-8

Authors: Córdoba-Izquierdo A, Drouot X, Thille AW, Galia F, Roche-Campo F, Schortgen F, Prats-Soro E, Brochard L

Abstract
OBJECTIVE: To compare sleep quality between two types of ventilators commonly used for noninvasive ventilation: conventional ICU ventilators and dedicated noninvasive ventilators; and to evaluate sleep during and between noninvasive ventilation sessions in critically ill patients.
DESIGN: Physiological sleep study with a randomized assessment of the ventilator type.
SETTING: Medical ICU in a university hospital.
PATIENTS: Twenty-four patients admitted for acute hypercapnic respiratory failure requiring noninvasive ventilation.
INTERVENTIONS: Patients were randomly assigned to receive noninvasive ventilation with either an ICU ventilators (n = 12) or a dedicated noninvasive ventilators (n = 12), and their sleep and respiratory parameters were recorded by polysomnography from 4 PM to 9 AM on the second, third, or fourth day after noninvasive ventilation initiation.
MEASUREMENTS AND MAIN RESULTS: Sleep architecture was similar between ventilator groups, including sleep fragmentation (number of arousals and awakenings/hr), but the dedicated noninvasive ventilators group showed a higher patient-ventilator asynchrony-related fragmentation (28% [17-44] vs. 14% [7.0-22]; p = 0.02), whereas the ICU ventilators group exhibited a higher noise-related fragmentation. Ineffective efforts were more frequent in the dedicated noninvasive ventilators group than in the ICU ventilators group (34 ineffective efforts/hr of sleep [15-125] vs. two [0-13]; p < 0.01), possibly as a result of a higher tidal volume (7.2 mL/kg [6.7-8.8] vs. 5.8 [5.1-6.8]; p = 0.04). More sleep time occurred and sleep quality was better during noninvasive ventilation sessions than during spontaneous breathing periods (p < 0.05) as a result of greater slow wave and rapid eye movement sleep and lower fragmentation.
CONCLUSIONS: There were no observed differences in sleep quality corresponding to the type of ventilator used despite slight differences in patient-ventilator asynchrony. Noninvasive ventilation sessions did not prevent patients from sleeping; on the contrary, they seem to aid sleep when compared with unassisted breathing.

PMID: 23222258 [PubMed - indexed for MEDLINE]

High-frequency ventilation versus conventional ventilation for treatment of acute lung injury and acute respiratory distress syndrome.

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High-frequency ventilation versus conventional ventilation for treatment of acute lung injury and acute respiratory distress syndrome.

Cochrane Database Syst Rev. 2013;2:CD004085

Authors: Sud S, Sud M, Friedrich JO, Wunsch H, Meade MO, Ferguson ND, Adhikari NK

Abstract
BACKGROUND: High frequency oscillation is an alternative to conventional mechanical ventilation that is sometimes used to treat patients with acute respiratory distress syndrome, but effects on oxygenation, mortality and adverse clinical outcomes are uncertain. This review was originally published in 2004 and was updated in 2011.
OBJECTIVES: To determine clinical and physiological effects of high frequency oscillation (HFO) in patients with acute lung injury (ALI) or acute respiratory distress syndrome (ARDS) compared to conventional ventilation.
SEARCH METHODS: We electronically searched CENTRAL (Ovid), MEDLINE (Ovid), EMBASE (Ovid), and ISI (from inception to March 2011). The original search was performed in 2002. We manually searched reference lists from included studies and review articles; searched conference proceedings of the American Thoracic Society (1994 to 2010), Society of Critical Care Medicine (1994 to 2010), European Society of Intensive Care Medicine (1994 to 2010), and American College of Chest Physicians (1994 to 2010); contacted clinical experts in the field; and searched for unpublished and ongoing trials in clinicaltrials.gov and controlled-trials.com.
SELECTION CRITERIA: Randomized controlled clinical trials comparing treatment using HFO with conventional mechanical ventilation for children and adults diagnosed with ALI or ARDS.
DATA COLLECTION AND ANALYSIS: Three authors independently extracted data on clinical, physiological, and safety outcomes according to a predefined protocol. We contacted investigators of all included studies to clarify methods and obtain additional data. We used random-effects models in the analyses.
MAIN RESULTS: Eight RCTs (n = 419) were included; almost all patients had ARDS. The risk of bias was low in six studies and unclear in two studies. The quality of evidence for hospital and six-month mortality was moderate and low, respectively. The ratio of partial pressure of oxygen to inspired fraction of oxygen at 24, 48, and 72 hours was 16% to 24% higher in patients receiving HFO. There were no significant differences in oxygenation index because mean airway pressure rose by 22% to 33% in patients receiving HFO (P < 0.01).  In patients randomized to HFO, mortality was significantly reduced (RR 0.77, 95% CI 0.61 to 0.98; P = 0.03; 6 trials, 365 patients, 160 deaths) and treatment failure (refractory hypoxaemia, hypercapnoea, hypotension, or barotrauma) was less likely (RR 0.67, 95% CI 0.46 to 0.99; P = 0.04; 5 trials, 337 patients, 73 events). Other risks, including adverse events, were similar. We found substantial between-trial statistical heterogeneity for physiological (I = 21% to 95%) but not clinical (I = 0%) outcomes.  Pooled results were based on few events for most clinical outcomes.
AUTHORS' CONCLUSIONS: The findings of this systematic review suggest that HFO was a promising treatment for ALI and ARDS prior to the uptake of current lung protective ventilation strategies. These findings may not be applicable with current conventional care, pending the results of large multi-centre trials currently underway.

PMID: 23450549 [PubMed - in process]

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