Acute Life-threatening Events Research Articles

The Role of Thrombolytic Therapy for Patients with a Submassive Pulmonary Embolism.

A pulmonary embolism (PE) is an acute life-threatening respiratory event that results in upwards of 200,000 deaths per year in the United States. While anticoagulation is currently the standard of treatment for PEs, there is increasing evidence to suggest that in certain cases anticoagulation in combination with thrombolytic therapy may improve patient outcomes and reduce mortality. This article aims to compare the effects of combined intervention with thrombolytic therapy and anticoagulation to the effects of anticoagulation alone in patients with submassive PEs in terms of various outcome measures, including but not limited to: mortality, hemodynamic status, length of hospital stay, and safety. The methodology consisted of the critical appraisal of the primary literature articles pertaining to intervention with thrombolytic agents in cases of a submassive or intermediate risk PE, including a discussion of each study’s strengths and limitations. Ultimately, this review found that the use of thrombolytic agents in conjunction with anticoagulants has been associated with decreased hemodynamic decompensation and decreased length of hospital stay, with no change in mortality outcomes, at a cost of increased rate of bleeding and stroke. The use of thrombolytic agents with anticoagulants may be warranted in a specific subset of patients, but clinicians should consider the potential benefits and harms of this intervention.

Cardiac-disease-induced PTSD and Fear of illness progression: Capturing the unique nature of disease-related PTSD.

According to Edmondson's Enduring Somatic Threat (EST) Model of PTSD Due to Acute Life-Threatening Medical Events, the nature of PTSD in the context of illness may differ from the nature of "traditional" PTSD in that it includes future-oriented alongside past-related intrusive thoughts. Yet almost no empirical studies to date have assessed the putative future-oriented quality of cardiac-disease-induced PTSD (CDI-PTSD). In the current study, we assessed the hypothesized associations between CDI-PTSD and fear of illness progression (FoP) - a novel theoretical conceptualization of patients' future-related anxieties. We hypothesized that FoP would be positively associated with CDI-PTSD, and especially with its specific items of intrusive thoughts. Patients (N = 112) were interviewed three months post-hospitalization for an acute coronary event via use of the PSS-SR-5 (to assess PTSD symptomatology), the FoP-Q-SF (to assess fear of illness progression), and the HADS (to assess anxiety and depression levels). Results indicated a strong positive association between CDI-PTSD and FoP, even when controlling for anxiety and depression. As hypothesized, the concepts of CDI-PTSD and fear of illness progression were strongly associated both at the level of the clusters as well as at the level of the specific items. The current study provides an initial empirical validation of the EST Model, especially regarding the future-oriented nature of PTSD resulting from acute cardiovascular disease.

An Interventionalist's Guide to Hemoptysis in Cystic Fibrosis.

Massive hemoptysis occurs in a minority of patients with cystic fibrosis, with an annual incidence of 1%. Although rare, massive hemoptysis can be a severe and potentially fatal complication of this disease. Beyond the acute life-threatening event, hemoptysis in patients with cystic fibrosis has been associated with faster decline in lung function, accelerated need for lung transplant, and increased mortality. The bronchial arteries are the culprit vessels in over 90% of cases of hemoptysis. This normally quiescent vascular system undergoes remarkable hypertrophy, collateralization, and angiogenesis before the onset of hemoptysis, introducing numerous pitfalls for the interventionalist. However, in experienced hands, bronchial artery embolization is a safe and potentially lifesaving therapy. Preprocedural noninvasive imaging, specifically computed tomographic angiography, has been repeatedly validated for helping to localize the likely site of bleeding, characterizing pertinent arterial anatomy, and promoting efficient and effective intervention; it has been recommended for all stable patients with hemoptysis. Success in the angiographic suite requires a thorough understanding of normal and variant bronchial arterial anatomy, appropriate patient selection, and a meticulous embolization technique. A meticulous approach to imaging and intervention, conscientious of both visualized and nonvisualized collateral pathways and nontarget vessels, can minimize potentially devastating complications. This review summarizes the current literature, modern procedural techniques, and emerging controversies, serving to guide an evolving approach to management of patients with cystic fibrosis and hemoptysis. ©RSNA, 2018.

Laryngotracheal anomalies associated with esophageal atresia: importance of early diagnosis.

Esophageal atresia (EA) is the most common congenital esophageal malformation. Airway pathology, in particular, tracheomalacia and laryngotracheal anomalies is a major cause of morbidity and mortalilty in patients with EA. The aim of this study was to report the incidence and type of laryngotracheal anomalies seen in a large series of patients with EA, and to evaluate their impact on the management of children with EA. Retrospective study. Retrospective cohort including all patients referred to the EA National Reference Center from January 2002 to December 2014. Airway assessment was based on endoscopy performed before, during and/or after esophageal surgery. One-hundred and fifty-eight patients were included in the study. Endoscopy revealed tracheomalacia in 141 cases (89.2%) and other laryngotracheal anomalies in 43 patients (27.2%). Ninety-six patients (60.7%) presented with persistent respiratory symptoms, including acute life-threatening events in 21 cases, leading to death in 6 cases. A correlation was observed between degree of tracheal collapse and presence of acute life-threatening events. Laryngotracheal surgery was required in 35 cases (22%). Laryngotracheal anomalies are frequently associated with EA and represent an important etiology of morbidity and mortality that can be prevented by early and systematic diagnosis and aggressive management. An early systematic endoscopic evaluation is recommended to coordinate the airway management with the EA surgery.

Infantil delirium induced by cycloplegic eye drops

Cyclopentolate 1% and tropicamide 1% eye drops are considered to be safe in the pediatric population for the fundus examination and treatment of ocular diseases. Although adverse reactions rarely occur, some of them can be life-threatening such as seizure, delirium, coma, arrhythmia, acute life-threatening event, and death. Here we report 15 months old boy who developed delirium after administration of ocular cyclopentolate 1% drops in both eyes during a routine examination for retinopathy of prematurity. Cyclopentolate is a parasympatholytic drug with actions similar to atropine. With ophthalmic administration of cyclopentolate, severe central nervous system effects and systemic reactions may occur. In order to minimize absorption following instill one or two drops of 1% solution, applying pressure to nasolacrimal sac for 2 to 3 min should be considered. In mild to moderate toxicity, benzodiazepines may be used to control central nervous system findings secondary to anticholinergic effects.

Outcomes of fundoplication in oesophageal atresia associated gastrooesophageal reflux disease

Aim of the studyConservative management of gastrooesophageal reflux (GORD) in oesophageal atresia (OA) is sometimes inefficient, and fundoplication is required. We assessed the outcomes of fundoplication among OA patients from 1980 to 2016. MethodsAfter ethical consent, hospital records of 290 patients, including 22 referred patients, were reviewed. Included were 262 patients with end–to–end repair. Excluded were patients who underwent oesophageal reconstruction (n=23) or no repair (n=5). Primary outcome measures included survival, retaining the native oesophagus, resolution of GGORD symptoms, failure of fundoplication, and long-term endoscopic results. Main resultsGross types of OA in 262 patients were A (n=12), B (n=2), C (n=217), D (n=10), E (n=19), and F (n=2). Eighty-six (33%) patients, type A (n=12, 100%), B (n=2, 100%), C (n=69, 31%), D (n=3, 30%), and F (n=1, 50%), underwent fundoplication at the median age of 5.4 (IQR 3.1–16) months. Main indications included recalcitrant anastomotic stenosis (RAS) in 41 (48%), respiratory symptoms in 16 (19%), and acute life threatening events (ALTE) in 15 (17%) of patients. Associated tracheomalacia in 25 (29%) patients were treated with aortopexy. Median follow–up was 7.5 (IQR 1.8–15) years. RAS resolved in 30 (73%) patients, whereas 11 (27%) with unresolved RAS underwent oesophageal resection (n=8) or replacement (n=3). Six (7%) patients died of heart failure (n=4), bolus impaction (n=1), and ALTE (n=1). Fundoplication failed in 27 (31%) patients, and 13 (15%) underwent redo fundoplication. Fundoplication failure was predicted by long-gap OA RR=3.8 (95%CI=1.1–13), P=0.04. In total GORD associated symptoms persisted in 7 (8%) patients, including one with permanent feeding jejunostomy. Latest endoscopy showed moderate or severe oesophagitis in 7% of fundoplicated and in 3% nonfundoplicated patients and intestinal metaplasia in 3% and 1% (p=0.20–0.29). ConclusionFundoplication provided a safe and relatively effective control of OA associated symptomatic GORD and oesophagitis. The failure rate of fundoplication was high in those with long-gap OA. Type of studyTreatment study. Level of evidenceIV

Bronchoscopic assessments and clinical outcomes in pediatric patients with tracheomalacia and bronchomalacia.

Tracheomalacia and bronchomalacia (TM/BM) are one of the serious causes of airway obstruction in infants and children. This study reviewed our bronchoscopic assessments and clinical outcomes in pediatric patients with TM/BM, and investigated risk factors of surgical intervention for TM/BM. Fifty-seven consecutive patients who were diagnosed as TM/BM by bronchoscopy between 2009 and 2013 were reviewed retrospectively. They were divided into two groups according to the presence (group E, n = 26) or absence (group N, n = 31) of acute life-threatening events and extubation failure (ALTE/EF). The severity of TM/BM was evaluated by Oblateness Index which was obtained from bronchoscopic images. Oblateness Index was significantly higher in Group E than in Group N. Patients in Group E underwent surgical intervention for TM/BM more frequently, and had significantly longer intubation period and hospital stay. Clinical symptoms of ALTE/EF, Oblateness Index ≥ 0.70, and multiple malacic lesions were significant risk factors indicating surgical events in patients with TM/BM. Patients with TM/BM who had ALTE/EF had more severe malacic lesions indicating surgical intervention, and worse clinical outcomes. Oblateness Index is a simple and semi-quantitative index for bronchoscopic assessment of TM/BM, and can be one of the prognostic tools to predict clinical severity of pediatric TM/BM.

Prognostic factors of maternal near miss events and maternal deaths in a tertiary healthcare facility in India

To study maternal near miss (MNM) and maternal mortality to identify rectifiable risk factors. The present cross-sectional retrospective study included pregnant women who experienced acute life-threatening pregnancy-related adverse events at Deen Dayal Upadhyay hospital, New Delhi, India, between September 1, 2009, and August 31, 2011. Patient data were analyzed to investigate factors associated with MNM events and maternal deaths. There were 369 patients included, and 302 MNM events and 67 maternal deaths were recorded. The recorded causes of MNM events included hemorrhage, hypertensive disorders, severe anemia with cardiac failure, organ failure, and infection in 192 (63.6%), 62 (20.5%), 13 (4.3%), 8 (2.6%), and 8 (2.6%) patients, respectively. Higher rates of anemia (P=0.007) and infection (P=0.007) were recorded among patients in the maternal death group than the MNMN group. Hemorrhage and hypertension were major causes of MNM events and are likely major barriers to reducing maternal mortality in low-income countries. Anemia and infection were significant prognostic factors of maternal death in the present study. MNM could be used as surrogate for maternal death in the provision of standard obstetric care.

Management of gastroesophageal reflux in children. Single centre experience in conventional and laparoscopic Nissen fundoplication in the last 15 years

Abstract Introduction Nissen fundoplication (NF) is the most used and effective technique for the treatment of gastroesophageal reflux in children. The laparoscopic approach (LNF) is safe, with low morbidity and high success rate, although some cases require a conventional approach (CNF). The aim of the study is to compare the results between LNF and CNF in our centre. Material and methods A retrospective review was performed on patients Results Of the total 75 NF performed, 49 (65.3%) were LNF, 23 (30.7%) CNF, and 3 (4.0%) reconversions. Concomitant laparoscopic gastrostomy was performed in 10.7%, and open gastrostomy in 5.3% of cases. Prior to NF, 10.7% had a gastrostomy. The mean age was 4 years and 68.7% were male. Of the diagnoses, 36% had encephalopathy, 14.7% hiatal hernia, 5.4% oesophageal atresia, and 5.4% an acute life-threatening event. No differences were found in operation time. More than two-thirds (36%) had complications, which were more frequent in the CNF (OR = 3.30, 95% CI: 1.1–9.6). The hospital-stay decreased by 9 days in the LNF (95% CI: 5.5–13.5). Mean follow-up was 26 months (95% CI: 20.9–31.6). Mortality during follow-up was of 5.3% (5 respiratory failure, 1 sudden cardiac death, and 2 due to complications of the encephalopathy), 4.2% required re-fundoplication, 15.8% had symptomatic improvement, and 64.0% had absence of symptoms. Conclusions The LNF is an effective technique for the treatment of gastroesophageal reflux, with lower morbidity and shorter hospital stay than CNF. It is recommended as the first surgical option.

Hypercalcemia in Patients with Williams-Beuren Syndrome

To evaluate the timing, trajectory, and implications of hypercalcemia in Williams-Beuren syndrome (WBS) through a multicenter retrospective study. Data on plasma calcium levels from 232 subjects with WBS aged 0-67.1 years were compared with that in controls and also with available normative data. Association testing was used to identify relevant comorbidities. On average, individuals with WBS had higher plasma calcium levels than controls, but 86.7% of values were normal. Nonpediatric laboratories overreport hypercalcemia in small children. When pediatric reference intervals were applied, the occurrence of hypercalcemia dropped by 51% in infants and by 38% in toddlers. Across all ages, 6.1% of the subjects had actionable hypercalcemia. In children, actionable hypercalcemia was seen in those aged 5-25 months. In older individuals, actionable hypercalcemia was often secondary to another disease process. Evidence of dehydration, hypercalciuria, and nephrocalcinosis were common in both groups. Future hypercalcemia could not be reliably predicted by screening calcium levels. A subgroup analysis of 91 subjects found no associations between hypercalcemia and cardiovascular disease, gastrointestinal complaints, or renal anomalies. Analyses of electrogradiography data showed an inverse correlation of calcium concentration with corrected QT interval, but no acute life-threatening events were reported. Actionable hypercalcemia in patients with WBS occurs infrequently. Although irritability and lethargy were commonly reported, no mortality or acute life-threatening events were associated with hypercalcemia and the only statistically associated morbidities were dehydration, hypercalciuria, and nephrocalcinosis.

Sudden unexplained early neonatal death or collapse: a national surveillance study.

The incidence of sudden unexpected early neonatal death (SUEND) or acute life-threatening events (ALTEs) is reported as 0.05/1,000 to 0.38/1,000 live births. There is currently no national system in Australia for reporting and investigating such cases. A 3-y prospective, national surveillance study, run in collaboration with the Australian Pediatric Surveillance Unit (APSU). Data were provided by pediatricians reporting to APSU; and independently ascertained by the Coroner in two states (NSW and QLD) and the Newborn Early Transport Network in NSW. A detailed deidentified questionnaire was created. In NSW and QLD, the incidence was 0.1 and 0.08/1,000 live births, respectively. Forty-eight definitive cases were identified. Common causes included accidental asphyxia, cardiac disease, persistent pulmonary hypertension of the newborn, and sudden infant death syndrome. Twenty-six babies collapsed on day 1 and 19 were found on the carer's chest. The incidence in NSW and QLD is higher than previously published. The first postnatal day is a vulnerable period for newborns, who require close observation particularly during skin-to-skin contact. Development and implementation of guidelines for safe sleeping in hospital are needed. Collaboration between obstetricians, midwives, and pediatricians is essential to ensure safety of the newborn.

Surveillance of bloodstream infections in pediatric cancer centers - what have we learned and how do we move on?

Pediatric patients receiving conventional chemotherapy for malignant disease face an increased risk of bloodstream infection (BSI). Since BSI may represent an acute life-threatening event in patients with profound immunosuppression, and show further negative impact on quality of life and anticancer treatment, the prevention of BSI is of paramount importance to improve and guarantee patients’ safety during intensive treatment. The great majority of all pediatric cancer patients (about 85%) have a long-term central venous access catheter in use (type Broviac or Port; CVAD). Referring to the current surveillance definitions a significant proportion of all BSI in pediatric patients with febrile neutropenia is categorized as CVAD-associated BSI. This state of the art review summarizes the epidemiology and the distinct pathogen profile of BSI in pediatric cancer patients from the perspective of infection surveillance. Problems in executing the current surveillance definition in this patient population are discussed and a new concept for the surveillance of BSI in pediatric cancer patients is outlined.

Pediatric tracheomalacia

Tracheomalacia (TM) is defined as an increased collapsibility of the trachea due to structural anomalies of the tracheal cartilage and/or posterior membrane. Tracheomalacia has a wide range of etiologies but is most commonly present in children born with esophageal atresia and tracheal esophageal fistula. Clinical symptoms can range from minor expiratory stridor with typical barking cough to severe respiratory distress episodes to acute life-threatening events (ALTE). Although the majority of children have mild-to-moderate symptoms and will not need surgical intervention, some will need life-changing surgical treatment. This article examines the published pediatric literature on TM, discusses the details of clinical presentation, evaluation, diagnosis, and a variety of treatments.

Manejo del reflujo gastroesofágico en niños. Funduplicatura de Nissen convencional y por laparoscopia en los últimos 15 años en un centro especializado

IntroductionNissen fundoplication (NF) is the most used and effective technique for the treatment of gastroesophageal reflux in children. The laparoscopic approach (LNF) is safe, with low morbidity and high success rate, although some cases require a conventional approach (CNF). The aim of the study is to compare the results between LNF and CNF in our centre. Material and methodsA retrospective review was performed on patients <14years after NF between 2000 and 2015. A comparison was made of the complications, hospital stay, and follow-up for both approaches. ResultsOf the total 75 NF performed, 49 (65.3%) were LNF, 23 (30.7%) CNF, and 3 (4.0%) reconversions. Concomitant laparoscopic gastrostomy was performed in 10.7%, and open gastrostomy in 5.3% of cases. Prior to NF, 10.7% had a gastrostomy. The mean age was 4 years and 68.7% were male. Of the diagnoses, 36% had encephalopathy, 14.7% hiatal hernia, 5.4% oesophageal atresia, and 5.4% an acute life-threatening event. No differences were found in operation time. More than two-thirds (36%) had complications, which were more frequent in the CNF (OR=3.30, 95%CI: 1.1-9.6). The hospital-stay decreased by 9 days in the LNF (95%CI: 5.5-13.5). Mean follow-up was 26 months (95%CI: 20.9-31.6). Mortality during follow-up was of 5.3% (5 respiratory failure, 1 sudden cardiac death, and 2 due to complications of the encephalopathy), 4.2% required re-fundoplication, 15.8% had symptomatic improvement, and 64.0% had absence of symptoms. ConclusionsThe LNF is an effective technique for the treatment of gastroesophageal reflux, with lower morbidity and shorter hospital stay than CNF. It is recommended as the first surgical option.

Early T-cell precursor acute lymphoblastic leukaemia in children treated in AIEOP centres with AIEOP-BFM protocols: a retrospective analysis

Early T-cell precursor acute lymphoblastic leukaemia was recently recognised as a distinct leukaemia and reported as associated with poor outcomes. We aimed to assess the outcome of early T-cell precursor acute lymphoblastic leukaemia in patients from the Italian Association of Pediatric Hematology Oncology (AIEOP) centres treated with AIEOP-Berlin-Frankfurt-Münster (AIEOP-BFM) protocols. In this retrospective analysis, we included all children aged from 1 to less than 18 years with early T-cell precursor acute lymphoblastic leukaemia immunophenotype diagnosed between Jan 1, 2008, and Oct 31, 2014, from AIEOP centres. Early T-cell precursors were defined as being CD1a and CD8 negative, CD5 weak positive or negative, and positive for at least one of the following antigens: CD34, CD117, HLADR, CD13, CD33, CD11b, or CD65. Treatment was based on AIEOP-BFM acute lymphoblastic leukaemia 2000 (NCT00613457) or AIEOP-BFM acute lymphoblastic leukaemia 2009 protocols (European Clinical Trials Database 2007-004270-43). The main differences in treatment and stratification of T-cell acute lymphoblastic leukaemia between the two protocols were that in the 2009 protocol only, pegylated L-asparaginase was substituted for Escherichia coli L-asparaginase, patients with prednisone poor response received an additional dose of cyclophosphamide at day 10 of phase IA, and high minimal residual disease at day 15 assessed by flow cytometry was used as a high-risk criterion. Outcomes were assessed in terms of event-free survival, disease-free survival, and overall survival. Early T-cell precursor acute lymphoblastic leukaemia was diagnosed in 49 patients. Compared with overall T-cell acute lymphoblastic leukaemia, it was associated with absence of molecular markers for PCR detection of minimal residual disease in 25 (56%) of 45 patients; prednisone poor response in 27 (55%) of 49 patients; high minimal residual disease at day 15 after starting therapy in 25 (64%) of 39 patients (bone marrow blasts ≥ 10%, by flow cytometry); no complete remission after phase IA in 7 (15%) of 46 patients (bone marrow blasts ≥ 5%, morphologically); and high PCR minimal residual disease (≥ 5 × 10(-4)) at day 33 after starting therapy in 17 (85%) of 20 patients with markers available. Overall, 38 (78%) of 49 patients are in continuous complete remission, including 13 of 18 after haemopoietic stem cell transplantation, with three deaths in induction, five deaths after haemopoietic stem cell transplantation, and three relapses. Severe adverse events in the 2009 study were reported in 10 (30%) of 33 patients with early T-cell precursor acute lymphoblastic leukaemia versus 24 (15%) of 164 patients without early T-cell precursor acute lymphoblastic leukaemia and life-threatening events in induction phase IA occurred in 4 (12%) of 33 patients with early T-cell precursor acute lymphoblastic leukaemia versus 7 (4%) of 164 patients without early T-cell precursor acute lymphoblastic leukaemia. No difference was seen in the subsequent consolidation phase IB of protocol I. Early T-cell precursor acute lymphoblastic leukaemia is characterised by poor early response to conventional induction treatment. Consolidation phase IB, based on cyclophosphamide, 6-mercaptopurine, and ara-C at conventional (non-high) doses is effective in reducing minimal residual disease. Although the number of patients and observational time are limited, patients with early T-cell precursor acute lymphoblastic leukaemia treated with current BFM stratification and treatment strategy have a favourable outcome compared with earlier reports. The role of innovative therapies and haemopoietic stem cell therapy in early T-cell precursor acute lymphoblastic leukaemia needs to be assessed. None.

Sudden Unexpected Postnatal Collapse of a Neonate During Skin-to-Skin Contact

Skin-to-skin contact (SSC) is a well-documented benefits manner for neonates and mothers. Neonates are placed on their mothers’ chest after birth immediately which will contribute to the transition from fetal to extra-uterine life such as stabilizing babies’ respiration, body temperature and blood sugar. Besides, SSC also helps mothers’ recovery after delivery. However, some acute life-threatening events happened during SSC. Herein, we reported the sudden unexpected collapse of a well-appearing full-term baby occurred during SCC and to make health care providers and parents aware of this possible risk.

Establishing a Role for Polysomnography in Hospitalized Children

BackgroundChildren with medical complexity have a high prevalence of sleep disorders. However, outpatient polysomnography to evaluate for these conditions may be difficult to perform because of lack of skilled nursing care. The aim of this study was to explore polysomnography indications in hospitalized children and assess its impact on patient care. MethodsData from 85 inpatient polysomnographies of 70 children hospitalized between March and December 2014 were retrospectively collected. ResultsSixty percent of patients were boys with ages 6.5 ± 6 years. Chronic respiratory failure was present in 33.8%, airway obstruction due to defects of the tracheobronchial tree or craniofacial abnormalities in 54.3%, neurological complications of the perinatal period in 22.9%, genetic syndromes and neurodegenerative disorders in 31.4%, congenital myopathies in 5.7%, metabolic diseases in 4.3% and congenital cyanotic heart defects in 4.3%. Indications for polysomnography included assessment of chronic pulmonary disease (60%), ventilator requirements (41.2%), apnea/desaturation (23.5%), and acute life-threatening events (1.2%). Abnormal results were found in 89.4%. The observed diagnosis was obstructive sleep apnea in 64.7%, signs of chronic lung disease in 34.1%, hypoventilation in 9.4%, periodic breathing in 3.5%, and periodic limb movement of sleep in 4.7%. The following interventions were performed: adjustment of ventilator parameters (45.8%), positive airway pressure initiation (24.7%), otorhinolaryngology referral (30.6%), supraglottoplasty (2.4%), tracheostomy decannulation (2.4%), and tracheostomy placement (3.5%). Nine patients had available follow-up polysomnograms, all showing improvement in sleep variables after adherence to recommended interventions. ConclusionsIn individuals with complex medical disorders, inpatient polysomnographies give invaluable information to guide immediate medical decision making and should be strongly considered if resources allow this.

Metabolic disease in 10 patients with sudden unexpected death in infancy or acute life-threatening events.

In order to determine the associations between sudden unexpected death in infancy (SUDI) or acute life-threatening events (ALTE) and inborn errors of metabolism, particularly organic acidemia and fatty acid oxidation disorders, we evaluated clinical features in patients with SUDI or ALTE. The subjects were infants between the ages of 7 days and 3 years who developed SUDI or ALTE between January 2004 and December 2013. They were then diagnosed as having inborn errors of metabolism on gas chromatography-mass spectrometry (GC/MS) and/or tandem mass spectrometry (MS/MS). The age distribution, onset forms, and clinical findings were evaluated during the acute phase. Inborn errors of metabolism were detected in three of 196 patients with SUDI, and in seven of 167 patients with ALTE. Of these 10 patients, nine had a history of poor feeding and somnolence during the neonatal period, and symptoms of infection such as cough, fever or vomiting during infancy. Routine laboratory tests during an acute phase indicated hyperammonemia, liver dysfunction, increased blood creatine kinase, acidosis, positive ketone bodies in urine or blood, or hypoglycemia. When SUDI or ALTE are encountered in the emergency unit, it is essential that a detailed medical history is taken, particularly with regard to the neonatal period, and that specific abnormalities are investigated on routine laboratory tests. Moreover, samples such as urine, serum, and filter paper blood specimens should be collected for GC/MS and/or MS/MS of organic acids and acylcarnitines, to identify inborn metabolic disorders.

Lessons learned: critical care management of patients with Ebola in the United States.

This report will describe the preparations for and the provision of care of two patients with Ebola virus disease in the biocontainment unit at the University of Nebraska Medical Center. Patient medical records. Not applicable. Not applicable. Not applicable. Safe and effective care of patients with Ebola virus disease requires significant communication and planning. Adherence to a predetermined isolation protocol is essential, including proper donning and doffing of personal protective equipment. Location of the patient care area and the logistics of laboratory testing, diagnostic imaging, and the removal of waste must be considered. Patients with Ebola virus disease are often dehydrated and need adequate vascular access for fluid resuscitation, nutrition, and phlebotomy for laboratory sampling. Advanced planning for acute life-threatening events and code status must be considered. Intensivist scheduling should account for the significant amount of time required for the care of patients with Ebola virus disease. With appropriate precautions and resources, designated hospitals in the United States can safely provide care for patients with Ebola virus disease.

An analysis of common indications for bronchoscopy in neonates and findings over a 10-year period.

There is a perceived increase in the number of microdirect laryngoscopies and bronchoscopies (MLBs) required on premature infants, infants with syndromic conditions, and those with complex congenital heart defects. Determining which neonates with certain underlying conditions require more aggressive interventions like tracheostomy, intubation, or supraglottoplasty might be useful for future preoperative planning and counseling of the families of newborns with complex medical conditions involving the airway. To evaluate features and findings in neonates undergoing MLB in the first 28 days of life over a 10-year period and compare these findings with past publications. Retrospective case series of 162 consecutive patients 28 days or younger undergoing MLB between January 1, 2002, and December 31, 2012, at a tertiary care children's hospital. Microdirect laryngoscopy and bronchoscopy. Frequencies of common indications and findings in neonates undergoing MLB. To determine if findings on MLB had significant associations with gestational age, neonates with cardiac defects or syndromic conditions, and neonates who had at least 1 other comorbid condition, χ2 and Fisher exact tests were performed. Similar associations were analyzed between neonates with other comorbid conditions and need for interventions such as supraglottoplasty, tracheostomy, and intubation. Of the 162 patients, 101 were male (55.5%). The mean age at their procedure was 14.1 days. The mean weight of patients at MLB was 3.31 kg, and 32 (17.6%) were premature. Congenital conditions were noted in 114 patients (62.6%), and of these, 55 (30.2%) had congenital cardiac disease and 30 (16.4%) had syndromic conditions. Common indications for surgery were respiratory distress (n = 145 [79.7%]), stridor (n = 102 [56.0%]), and cyanosis or an acute life-threatening event (n = 67 [36.8%]). The most common findings at the time of surgery were laryngomalacia (n = 71 [39.0%]), subglottic stenosis (n = 58 [31.9%]), and tracheomalacia (n = 47 [25.8%]). Seventeen neonates (9.3%) required a tracheostomy, and 11 (6.0%) required a supraglottoplasty. Neonates with congenital heart defects were statistically significantly more likely to require long-term intubation (n = 9 [16.4%]; P = .03). Those with syndromic conditions were more likely to require long-term intubation and tracheostomy (n = 7 [23.3%] [P = .004] and n = 7 [23.3%] [P = .01], respectively). Premature infants who required MLB had a decreased incidence of laryngomalacia (n = 7 [21.9%]; P = .03), and those with comorbid conditions, an increased incidence (n = 43 [33.3%]; P = .01). There was minimal morbidity directly associated with the procedure. Neonates undergoing MLB most commonly presented with respiratory distress and stridor and were most commonly found to have laryngomalacia and subglottic stenosis. More than half of the patients studied had other comorbid conditions. Those with cardiac defects and syndromic conditions were more likely to require intubation, and those with syndromic conditions were more likely to eventually undergo tracheostomy.