ECLS for Legionella: All Ages Welcome in the ELSO Registry.

We describe the use of extracorporeal life support (ECLS) for Legionellosis in the pediatric and adult populations and report complications, morbidity, and mortality by conducting a retrospective review of patients from the Extracorporeal Life Support Organization registry, including two cases at our pediatric institution. A total of 194 patients with ECLS for Legionella pneumophila infection who received ECLS were included in the analysis. Overall survival was 71%. Lower body weight and VA ECLS were associated with lower survival. ECLS complications including central nervous system (CNS) hemorrhage, cardiopulmonary resuscitation, pulmonary infection, and documented infections were rare but more common among nonsurvivors. Mortality in children (64%) was significantly higher than for adults (27%, p = 0.015). Infants fared very poorly with a mortality of 88%. As demonstrated, ECLS can successfully support patients with severe respiratory failure caused by L. pneumophila infection. Consideration should be given to its use in high-risk pediatric patients with severe acute hypoxemic respiratory failure of unclear etiology. ECLS is a beneficial tool for appropriate candidates with rare disease. Pulmonary respite may provide time for diagnosis and lung recuperation.

Oral injuries and occult harm in children evaluated for abuse.

OBJECTIVE: To determine the frequency of occult trauma in children with oral injury evaluated for physical abuse. DESIGN: This was a retrospectively planned secondary analysis of a prospective, observational study. SETTING: Emergency departments supported by 20 US child abuse teams in the Examining Siblings to Recognize Abuse (ExSTRA) network. PATIENTS: Children <120 months old evaluated for physical abuse. INTERVENTIONS: Analysis of index children with oral injury on initial examination. MAIN OUTCOME MEASURES: Rates of physician-recognised oral injury, as well as frequency and results of occult injury testing. Perceived abuse likelihood was described on a 7-point scale (7=definite abuse). RESULTS: Among 2890 child abuse consultations, 3.3% (n=96) of children had oral injury. Forty-two per cent were 0-12 months old, 39% 1-3 years old and 18% >3 years old. Oral injury was the primary reason for evaluation for 32 (33%). Forty-three per cent (42/96) had frenum injuries. Skeletal surveys were obtained for 84% and 25% of these identified occult fractures. Seventy-five per cent had neuroimaging; 38% identified injuries. Forty-one per cent of children with oral injuries had retinal examinations; 24% of exams showed retinal haemorrhages. More occult injuries were found in children with oral injuries than other ExSTRA subjects. A high level of concern for abusive injury was present in 67% of children with oral injury versus 33% without. CONCLUSIONS: Children with oral injury are at high risk for additional occult abusive injuries. Infants and mobile preschoolers are at risk. Young children with unexplained oral injury should be evaluated for abuse.

Evacuation of Intensive Care Units During Disaster: Learning From the Hurricane Sandy Experience.

OBJECTIVE: Data on best practices for evacuating an intensive care unit (ICU) during a disaster are limited. The impact of Hurricane Sandy on New York City area hospitals provided a unique opportunity to learn from the experience of ICU providers about their preparedness, perspective, roles, and activities. METHODS: We conducted a cross-sectional survey of nurses, respiratory therapists, and physicians who played direct roles during the Hurricane Sandy ICU evacuations. RESULTS: Sixty-eight health care professionals from 4 evacuating hospitals completed surveys (35% ICU nurses, 21% respiratory therapists, 25% physicians-in-training, and 13% attending physicians). Only 21% had participated in an ICU evacuation drill in the past 2 years and 28% had prior training or real-life experience. Processes were inconsistent for patient prioritization, tracking, transport medications, and transport care. Respondents identified communication (43%) as the key barrier to effective evacuation. The equipment considered most helpful included flashlights (24%), transport sleds (21%), and oxygen tanks and respiratory therapy supplies (19%). An evacuation wish list included walkie-talkies/phones (26%), lighting/electricity (18%), flashlights (10%), and portable ventilators and suction (16%). CONCLUSIONS: ICU providers who evacuated critically ill patients during Hurricane Sandy had little prior knowledge of evacuation processes or vertical evacuation experience. The weakest links in the patient evacuation process were communication and the availability of practical tools. Incorporating ICU providers into hospital evacuation planning and training, developing standard evacuation communication processes and tools, and collecting a uniform dataset among all evacuating hospitals could better inform critical care evacuation in the future.

Genetic analyses for predictors of radiation response in glioblastoma.

PURPOSE: Radiotherapy (RT) for patients with glioblastoma improves survival and is recommended for nearly all patients with this diagnosis. However, the response to RT is variable in this patient population. Prior studies have suggested that underlying genetic alterations in the tumor may account for some of this treatment-related heterogeneity. It has been previously reported that epidermal growth factor receptor (EGFR) gene amplification and TP53 mutation correlate with the response to RT in patients with glioblastoma. METHODS AND MATERIALS: We sought to identify molecular markers that could predict the response to RT, progression-free survival after RT, and overall survival among 75 glioblastoma patients treated with RT at a single institution. Genetic analyses assessed EGFR amplification, TP53 mutations, CDKN2A/p16 deletion, and losses of chromosomes 1p, 10q, and 19q. RESULTS: Unlike previous reports, no association of EGFR amplification with response to RT, progression-free survival, or overall survival was found. Moreover, no association was found between these endpoints and the other genetic alterations assayed (TP53 mutation, CDKN2A/p16 deletion, loss of heterozygosity 1p, loss of heterozygosity 10q, and loss of heterozygosity 19q). However, in accordance with recent observations that the prognostic effects of genetic alterations in glioblastoma may depend on patient age, we observed age-dependent prognostic effects of TP53 and CDKN2A/p16 alterations in our patient population. For patients > or = 57 years old, those harboring TP53 mutations had a decreased overall survival compared with patients without TP53 mutations. Similarly, deletion of CDKN2A/p16 in patients > or = 57 years was associated with decreased progression-free survival after RT and a trend toward a shorter time to progression after RT compared with similar patients without the deletion. CONCLUSION: These data contrast with previous studies regarding the significant prognostic effect of EGFR with respect to RT response. Although our observations regarding the age-dependent prognostic effects of TP53 and CDKN2A/p16 are consistent with a prior report regarding these alterations, the present results should be considered preliminary, given the small sample size.

Age-dependent prognostic effects of genetic alterations in glioblastoma.

PURPOSE: Although the genetic alterations in glioblastoma have been well characterized, reports regarding their prognostic effects have been inconsistent. EXPERIMENTAL DESIGN: In this series of 140 consecutive cases of glioblastoma treated at a single center, we analyzed the frequency, age dependency and prognostic effects of TP53 mutation, CDKN2A/p16 deletion, EGFR amplification, as well as loss of chromosome 1p, chromosome 10q, and chromosome 19q. The complete set of genetic alterations was available on 60 of 140 patients. RESULTS: In this cohort of glioblastoma cases, TP53 mutation was significantly associated with patient age. The prognostic effects of TP53 mutation, EGFR amplification, CDKN2A/p16 alterations, and loss of chromosome 1p were dependent on the age of the patient. CONCLUSIONS: This is the first observation that the prognostic effects of TP53, 1p, and CDKN2A/p16 alterations are dependent on patient age. These observations concerning the interactions of age and genetic changes in glioblastoma suggest that tumorigenic pathways to glioblastoma vary with the age of the patient and that future molecular marker studies should carefully evaluate the potential age-dependent prognostic effects of these biological variables. The inconsistent or negative prognostic effects of molecular markers reported in prior studies of glioblastoma may be because different effects at different ages may have resulted in a cancellation of an overall effect in the entire cohort.

Facial numbness in a man with inguinal and retroperitoneal masses.

BACKGROUND: A 37-year-old Brazilian man was admitted to Massachusetts General Hospital for evaluation of left-sided facial numbness, left-sided ataxia, dizziness, and vertigo. Seven weeks prior to admission, he reported numbness of the left oral cavity and tongue after a dental procedure. Three weeks prior to presentation, he developed left-sided incoordination with dizziness and vertigo. One week later, he noticed difficulty using a box cutter at work and presented to the emergency department for evaluation. A CT scan without contrast revealed no abnormalities and he was discharged home. Three days prior to admission, the patient developed diplopia. Cranial MRI revealed a 1.6 cm irregular enhancing mass in the left middle cerebellar peduncle. Neurologic examination was significant for diminished sensation over the left face to pinprick, left-sided dysmetria, and mild lateral instability of the trunk while walking. INVESTIGATIONS: MRI, lumbar puncture, CT scans of the abdomen and pelvis, needle biopsy of the lymph node, and paraneoplastic antibodies. DIAGNOSIS: Possible paraneoplastic demyelination. MANAGEMENT: Orchiectomy, adjuvant radiation, and corticosteroids.