BACKGROUND/AIMS: Post-extubation respiratory failure (PERF) is associated with poor clinica l outcomes. High-f low nasa l cannula (HF NC) ox ygen therapy has been used in patients with respiratory failure, but the clinical benefit in patients with PERF remains unclear. The aim of this study was to evaluate the clinical efficacy of HFNC compared to noninvasive ventilation (NIV) in patients with PERF. METHODS: A historic retrospective cohort analysis was performed in 28 beds in the medical Intensive Care Unit (ICU) at a single medical center in South Korea. In total, 73 patients with PERF were enrolled: 39 patients who underwent NIV from April 2007 to March 2009 and 34 patients who received HFNC from April 2009 to May 2011. RESULTS: The rate of avoidance of reintubation was not different between the HFNC group (79.4%) and NIV group (66.7%, p = 0.22). All patients with HFNC tolerated the device, whereas five of those with NIV did not tolerate treatment (p = 0.057). The mean duration of ICU stay was significantly shorter in the HFNC group than in the NIV group (13.4 days vs. 20.6 days, p = 0.015). There was no difference in ICU or in-hospital mortality rate. CONCLUSIONS: HFNC is likely to be as effective as, and better tolerated than, NIV for treatment of PERF.
Administration, Inhalation ; Aged ; Airway Extubation/*adverse effects/mortality ; *Cannula ; Female ; Hospital Mortality ; Humans ; Intensive Care Units ; Male ; Middle Aged ; *Noninvasive Ventilation/adverse effects ; Oxygen/*administration & dosage ; Oxygen Inhalation Therapy/adverse effects/*instrumentation/mortality ; Republic of Korea ; Respiratory Insufficiency/diagnosis/etiology/mortality/*therapy ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome

OBJECTIVETo study the pathology, imaging and clinical features of a child with trisomy 21 syndrome associated interstitial lung disease.

METHODData of a case with trisomy 21 syndrome associated interstitial lung disease confirmed by lung imaging and pathology were collected, analyzed and the related reports in literature were reviewed.

RESULTThe patient was a one year and 7 months old boy who suffered from severe pneumonia and recurrent infection during his hospital stay. When his disease was stable, he did not have shortness of breath and cyanosis, but a chest computed tomography (CT) showed ground-glass opacity, regional emphysema, band-like change in lung parenchyma, which indicated interstitial lung diseases. Unequal air inflation in bilateral lungs and diffuse over-distension of peripheral air spaces in lung surface were seen through thoracoscope. Pathological examination indicated that alveolar, alveolar ducts and alveolar sac were enlarged, alveolar septa was expanded. There were two reports in lung pathology of trisomy 21 syndrome, alveolar growth abnormalities was seen in 86%-88% cases. The multiple subpleural cysts in chest CT was characteristic. Clinically, trisomy 21 syndrome had high morbidity of respiratory tract infection and progress to respiratory failure frequently. Prolonged postoperative desaturation was constant which required long duration of respiratory support.

CONCLUSIONTrisomy 21 syndrome associated alveolar growth abnormalities were confirmed, which manifest as alveolar simplification in pathology and interstitial lung diseases in imaging. The risk of respiratory failure in these cases caused by infection and surgery should be considered.

Cysts ; pathology ; Down Syndrome ; complications ; Humans ; Infant ; Lung ; pathology ; Lung Diseases, Interstitial ; diagnosis ; etiology ; Male ; Postoperative Period ; Pulmonary Alveoli ; pathology ; Respiratory Insufficiency ; Respiratory Tract Infections ; Tomography, X-Ray Computed

Knowledge of clinical demographics and outcomes of mechanically ventilated patients is important but there are few prospectively collected data in Korea. The objective of the present study was to describe the current status of mechanically ventilated patients in Korea as of 2010. We analyzed the data of Korean patients (275 patients in 12 Korean intensive care units [ICU]) participating in a multinational prospective cohort study on mechanical ventilation. The most common indication for mechanical ventilation was pneumonia (23%). Pressure-limited ventilation modes were preferred over volume-cycled ventilation modes. Non-invasive positive pressure ventilation was used in only seven (2%) patients as the initial ventilatory support. Median duration of mechanical ventilation was 7 days and ICU mortality was 36%. The multiple logistic regression model revealed that the Simplified Acute Physiology Score II (SAPS II) score at ICU admission (odds ratio [OR], 1.034; 95% confidence interval [CI], 1.001-1.036; P=0.033), peak pressure (OR, 1.054; 95% CI, 1.016-1.095; P=0.006), and the number of failed organs (OR, 2.132; 95% CI, 1.634-2.781; P<0.001) were independently associated with ICU mortality. This study provides a snapshot of current practice of mechanical ventilation in Korea.
Acute Disease ; Aged ; Cohort Studies ; Demography ; Female ; Hospital Mortality ; Humans ; Intensive Care Units/*statistics & numerical data ; Length of Stay ; Male ; Middle Aged ; Odds Ratio ; Prognosis ; Prospective Studies ; Republic of Korea ; *Respiration, Artificial ; Respiratory Insufficiency/*diagnosis/epidemiology/mortality ; Sepsis/etiology ; Severity of Illness Index

BACKGROUNDAlthough noninvasive positive pressure ventilation (NPPV) has been successfully used for various kinds of acute respiratory failure, the data are limited regarding its application in postoperative respiratory failure after cardiac surgery. Therefore, we conducted a prospective randomized control study in a university surgical intensive care unit to evaluate the efficacy and safety of NPPV in the treatment of acute respiratory failure after cardiac surgery, and explore the predicting factors of NPPV failure.

METHODSFrom September 2011 to November 2012 patients with acute respiratory failure after cardiac surgery who had indication for the use of NPPV were randomly divided into a NPPV treatment group (NPPV group) and the conventional treatment group (control group). The between-group differences in the patients' baseline characteristics, re-intubation rate, tracheotomy rate, ventilator associated pneumonia (VAP) incidence, in-hospital mortality, mechanical ventilation time after enrollment (MV time), intensive care unit (ICU) and postoperative hospital stays were compared. The factors that predict NPPV failure were analyzed.

RESULTSDuring the study period, a total of 139 patients who had acute respiratory failure after cardiac surgery were recorded, and 95 of them met the inclusion criteria, which included 59 males and 36 females with a mean age of (61.5 ± 11.2) years. Forty-three patients underwent coronary artery bypass grafting (CABG), 23 underwent valve surgery, 13 underwent CABG+valve surgery, 13 underwent major vascular surgery, and three underwent other surgeries. The NPPV group had 48 patients and the control group had 47 patients. In the NPPV group, the re-intubation rate was 18.8%, tracheotomy rate was 12.5%, VAP incidence was 0, and the in-hospital mortality was 18.8%, significantly lower than in the control group 80.9%, 29.8%, 17.0% and 38.3% respectively, P < 0.05 or P < 0.01. The MV time and ICU stay (expressed as the median (P25, P75)) were 18.0 (9.2, 35.0) hours and 4.0 (2.0, 5.0) days, which were significantly shorter than in the control group, 96.0 (26.0, 240.0) hours and 6.0 (4.0, 9.0) days respectively, P < 0.05 or P < 0.01. The postoperative hospital stays of the two groups were similar. The univariate analysis showed that the NPPV success subgroup had more patients with acute lung injury (ALI) (17 vs. 0, P = 0.038), fewer patients with pneumonia (2 vs. 7, P < 0.001) and lower acute physiology and chronic health evaluation II (APACHE II) scores (16.1 ± 2.8 vs. 21.8 ± 3.2, P < 0.001). Multivariate analysis showed that pneumonia (P = 0.027) and a high APACHE II score >20 (P = 0.002) were the independent risk factors of NPPV failure.

CONCLUSIONSWe conclude that NPPV can be applied in selected patients with acute respiratory failure after cardiac surgery to reduce the need of re-intubation and improve clinical outcome as compared with conventional treatment. Pneumonia and a high APACHE II score >20 might be the independent risk factors of NPPV failure in this group of patients.

Aged ; Cardiac Surgical Procedures ; adverse effects ; Female ; Humans ; Male ; Middle Aged ; Postoperative Complications ; therapy ; Prospective Studies ; Respiration, Artificial ; adverse effects ; Respiratory Distress Syndrome, Adult ; etiology ; therapy ; Respiratory Insufficiency ; diagnosis ; therapy ; Treatment Outcome

OBJECTIVETo summarize the clinical characteristics, diagnosis, treatments and outcomes of perinatal autosomal recessive polycystic kidney disease.

METHODSThe clinical data of one case with infantile polycystic kidney disease diagnosed in perinatal stage and the reports of 11 cases seen in the past 15 years searched in Pubmed, OVID and Elsevier and CNKI, Wanfang database by using the polycystic kidney disease, infant, perinatal, autosomal recessive and case report as keyword were reviewed and analyzed.

RESULTSThe infant was characterized by huge kidneys, severe respiratory and renal compromise. The kidneys were symmetrically enlarged and highly echogenic by ultrasonographic examination and showed high-signal intensity on T2-weighted images by MRI. Histologic analysis showed pulmonary hypoplasia, numerous dilated and elongated tubular structures in the kidney and dilated intrahepatic biliary ducts. Among the 12 cases, 8 cases' presumptive diagnosis was made by prenatal ultrasound revealed enlarged kidneys and oligohydramnios. All cases suffered respiratory distress after birth, and 5 cases complicated pneumothorax. 6 cases died in neonatal stage because of respiratory failure.1 case died 2 m after birth because of renal failure. Five cases are alive and underwent dialysis, nephrectomy or renal transplant.

CONCLUSIONNewborn infants with perinatal autosomal recessive polycystic kidney disease often have poor outcome and died from respiratory and renal failure. Aggressive respiratory support and renal replacement therapy (including nephrectomy, dialysis and transplantation) may give these infants a favorable outcome.

Bronchopulmonary Dysplasia ; etiology ; pathology ; Fatal Outcome ; Humans ; Infant, Newborn ; Infant, Newborn, Diseases ; diagnosis ; genetics ; pathology ; Kidney ; pathology ; Male ; Perinatology ; Polycystic Kidney, Autosomal Recessive ; complications ; diagnosis ; genetics ; pathology ; Renal Dialysis ; Renal Insufficiency ; etiology ; pathology ; Respiratory Insufficiency ; etiology ; pathology ; Retrospective Studies ; Ultrasonography

OBJECTIVETo analyze and summarize the characteristics of glycogen storage disease type II (Pompe disease) patients according to the clinical description and prognosis.

METHODSeventeen Chinese patients diagnosed by acid alpha-glucosidase (GAA) enzyme activity test were reviewed. Clinical data tables were designed. Interviews were made via phone calls. Information was collected to reach the objective.

RESULTFour of 17 patients diagnosed by acid alpha-glucosidase are infantile-onset, symptoms started between 2 to 6 months after birth with increased serum creatine kinase and cardiac problems, with or without respiratory concerns. Other 13 patients were later-onset cases, and their symptoms started between 2 to 22 years of age with increased serum creatine kinase. Eleven later-onset patients started with muscle weakness, 2 patients developed respiratory insufficiency, 2 patients showed scoliosis, and 1 patient expressed increased serum creatine kinase with abnormal liver function. Just 3 of the later-onset patients were treated with mechanical ventilator and adjuvant therapy, others were not. All patients' acid alpha-glucosidase (GAA) enzyme activity analysis showed lower than 10% of normal. Fourteen patients were tested by muscle biopsy pathology, and 9 of them progressed to glycogen storage disease type II; 10 patients received genetic analysis, and 6 of them had two mutations which cause the disorder. Twelve of the 17 patients were interviewed successfully. In 3 of the infant-onset patients the disease resulted in death from respiratory failure, and 1 is still alive at the age of 1 year and 7 months. In 4 of 8 later-onset patients the disease resulted in death from respiratory failure between 3 to 5 years after onset of symptoms. Three of 4 survivors had increased muscle weakness, and 1 patient kept alive with ventilator without any changes. Seven of 12 interviewed patients died, the mortality rate was 58.3%.

CONCLUSIONGlycogen storage disease type II (Pompe disease) present differently in the clinic. Infant-onset Pompe disease is mainly characterized by generalized muscle weakness and obvious cardiac involvement. It's a dangerous disease, with high mortality rate. Later-onset Pompe disease is characterized by chronic proximal muscle weakness and respiratory insufficiency. GAA enzyme activity analysis, muscle biopsy and genetic analysis used to support the diagnosis of Pompe disease. Prognosis of the disease depends on age of onset and respiratory muscle involvement.

Adolescent ; Biopsy ; Cardiomyopathies ; epidemiology ; etiology ; Child ; Child, Preschool ; Clinical Enzyme Tests ; Creatine Kinase ; blood ; Female ; Follow-Up Studies ; Glucan 1,4-alpha-Glucosidase ; genetics ; metabolism ; Glycogen Storage Disease Type II ; diagnosis ; genetics ; pathology ; Humans ; Infant ; Male ; Muscle Weakness ; epidemiology ; etiology ; Prognosis ; Respiratory Insufficiency ; epidemiology ; Retrospective Studies ; Young Adult

BACKGROUND/AIMS: Home oxygen therapy (HOT) costs a great deal every year and demand for the service is growing. In Korea, health insurance has covered HOT since November 1, 2006. The objective of this study was to evaluate clinical features of patients who used long-term HOT due to chronic respiratory failure and to determine the appropriateness of oxygen prescriptions. METHODS: Between November 2006 and April 2010, patients prescribed long-term HOT were enrolled in the study at a tertiary university referral hospital and their medical records and telephone survey information were evaluated. In total, 340 patients were evaluated retrospectively. RESULTS: Regarding the initial indications for HOT, their mean PaO2 was 49.8 mmHg and mean SpO2 was 82.2%. Underlying diseases included chronic obstructive pulmonary disease (COPD, 19.8%), lung cancer (12.6%), and interstitial lung disease (11.2%). The admission rate within 1 year was 53.4% and the average number of admissions was 1.64/patient. Other underlying diseases for which oxygen was prescribed, despite not meeting the insurance coverage criteria, were lung cancer (36.6%) and interstitial pneumonia (16.6%). CONCLUSIONS: Home oxygen prescriptions have increased since health insurance coverage was extended. However, cases of oxygen prescriptions frequently do not meet the coverage criteria. It is important to discuss extending the coverage criteria to other disease groups, such as interstitial lung disease and lung cancer, in terms of cost-effectiveness. Further, physicians prescribing oxygen therapy should be educated regarding the criteria.
Adult ; Aged ; Aged, 80 and over ; Cause of Death ; Chronic Disease ; Eligibility Determination ; Emergency Service, Hospital ; Female ; *Home Care Services, Hospital-Based ; Hospitalization ; *Hospitals, University ; Humans ; Insurance Coverage ; Insurance, Health ; Kaplan-Meier Estimate ; Male ; Middle Aged ; *Oxygen Inhalation Therapy ; Patient Compliance ; Program Evaluation ; Republic of Korea ; Respiratory Insufficiency/diagnosis/etiology/mortality/*therapy ; Retrospective Studies ; Time Factors ; Treatment Outcome ; Young Adult

Pseudomembranous necrotizing bronchial aspergillosis (PNBA) is a rare form of invasive aspergillosis with a very poor prognosis. The symptoms are non-specific, and the necrotizing plugs cause airway obstruction. Atelectasis and respiratory failure can be the initial manifestations. Recently, we treated an immunocompromised patient with PNBA, who presented with a sudden onset of atelectasis and acute respiratory failure. There were no preceding signs except for a mild cough and one febrile episode. Bronchoscopy revealed PNBA, and Aspergillus nidulans was cultured from the bronchial wash.
Adult ; Female ; Humans ; Immunocompromised Host ; Invasive Pulmonary Aspergillosis/*complications/*diagnosis ; Leukemia, Myeloid, Acute/complications ; Neutropenia/complications ; Pulmonary Atelectasis/*etiology ; Respiratory Insufficiency/*etiology

Myasthenia gravis (MG) is often complicated by respiratory failure, known as a myasthenic crisis. However, most of the patients who develop respiratory symptoms do so during the late course of disease and have other neurological signs and symptoms. However, in some patients respiratory failure is the initial presenting symptom. We report the case of a 68-year-old woman with MG who presented with isolated respiratory failure as her first presenting symptom. As illustrated by this case, it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.
Acute Disease ; Aged ; Electromyography ; Female ; Humans ; Myasthenia Gravis/*complications/*diagnosis ; Pulmonary Atelectasis/etiology/radiography ; Respiratory Insufficiency/*etiology/*radiography ; Tomography, Spiral Computed

Smoking is associated with poor symptom control and impaired therapeutic responses in asthma. A total of 843 patients with asthma were recruited. The patients received treatment for 1 yr according to the severity of their asthma. We compared the forced expiratory volume in 1 sec (FEV1), the ratio of FEV1 to forced vital capaity (FVC), atopy, total IgE, emphysema on high-resolution computed tomography (HRCT), the number of near-fatal asthma attacks, and physiological fixed airway obstruction between the smoking and nonsmoking groups. The study population consisted of 159 (18.8%) current smokers, 157 (18.7%) ex-smokers, and 525 (62.5%) nonsmokers. Although the prevalence of atopy was not different between the smoking and nonsmoking groups, the total IgE was higher among the smokers than the nonsmokers. Compared with the nonsmoking group, the smokers had a lower FEV1 % predicted and forced expiratory flow between 25 and 75% of FVC. A greater prevalence of emphysema and a significantly higher number of asthmatic patients with fixed airway obstruction were detected in the smoking versus nonsmoking group. The 37.5% of asthmatic patients who were former or current smokers showed decreased pulmonary function and increased IgE, emphysema on HRCT, and fixed airway obstruction, indicating that smoking can modulate the clinical and therapeutic responses in asthma.
Airway Obstruction/etiology ; Asthma/complications/*diagnosis/*drug therapy ; Female ; Forced Expiratory Volume/physiology ; Humans ; Immunoglobulin E/analysis ; Male ; Middle Aged ; Pulmonary Emphysema/etiology/radiography ; Respiratory Function Tests ; Respiratory Insufficiency/etiology ; Smoking/*adverse effects ; Tomography, X-Ray Computed