Bilateral Pneumothoraces in a 3-Month-Old Infant During an Apnea Test in the Diagnosis of Brain Death.

We present the case of a 3-month-old boy who suffered bilateral pneumothoraces secondary to insufflation of oxygen into the endotracheal tube during the apnea test as part of brain death testing. Although rare, awareness of this potential complication of the apnea test is of particular importance in pediatric patients who have narrow endotracheal tubes because resistance to expiratory flow increases exponentially as lumen diameter decreases.

Burden of Cardiomyopathic Genetic Variation in Lethal Pediatric Myocarditis.

BACKGROUND: Acute myocarditis (AM) is a well-known cause of sudden death and heart failure, often caused by prevalent viruses. We previously showed that some pediatric AM correlates with putatively damaging variants in genes related to cardiomyocyte structure and function. We sought to evaluate whether deleterious cardiomyopathic variants were enriched among fatal pediatric AM cases in New York City compared with ancestry-matched controls. METHODS: Twenty-four children (aged 3 weeks to 20 years) with death due to AM were identified through autopsy records; histologies were reviewed to confirm that all cases met Dallas criteria for AM and targeted panel sequencing of 57 cardiomyopathic genes was performed. Controls without cardiovascular disease were identified from a pediatric database and matched by genetic ancestry to cases using principal components from exome sequencing. Rates of putative deleterious variations (DV) were compared between cases and controls. Where available, AM tissues underwent viral analysis by polymerase chain reaction. RESULTS: DV were identified in 4 of 24 AM cases (16.7%), compared with 2 of 96 age and ancestry-matched controls (2.1%, P=0.014). Viral causes were proven for 6 of 8 AM cases (75%), including the one DV+ case where tissue was available for testing. DV+ cases were more likely to be female, have no evidence of chronic inflammation, and associate with sudden cardiac death than DV- cases. CONCLUSIONS: Deleterious variants in genes related to cardiomyocyte integrity are more common in children with fatal AM than controls, likely conferring susceptibility. Additionally, genetically mediated AM may progress more rapidly and be more severe.

Death in a Carbon Dioxide Therapy Bath: A Case Report and Review of the Literature.

Carbon dioxide (CO2) therapy is the subcutaneous or transcutaneous administration of CO2 for therapeutic purposes. Carbon dioxide therapy is used for localized lipolysis, to treat chronic skin conditions, and is a safe treatment. Full-body CO2 baths are offered in European spa centers, in which the clients are placed into full body bags infused with CO2 at an optimal concentration range between 1000 and 1400 mg/L (516 000-722 500 ppm). Commercially manufactured, air-tight bags and accompanying apparatus designed to provide CO2 baths can be purchased for home use. Few human CO2-related deaths have been reported. They have been mostly accidental, consisting of persons trapped in a closed environment in the presence of "dry ice" or solid CO2. There have been no reported deaths of a human undergoing a CO2 therapy at home. We present a case of a middle-aged male found at home completely inside an air-sealed bag wrapped tightly around his body. The bag was connected to a working pump and a CO2 gas tank. The pump was connected through an inflow and outflow circuit to the bag. The inflow tubing for CO2 gas delivery was partially disrupted, while the outflow tubing was intact. The autopsy and toxicology were unremarkable. The cause of death was determined to be asphyxia by vitiated atmosphere as evident by the displacement of oxygen by CO2 and low pressure created inside a "CO2 therapy bath." The manner of death was accidental.

Neuropathologic Examination in Sudden Unexpected Deaths in Infancy and Childhood: Recommendations for Highest Diagnostic Yield and Cost-Effectiveness in Forensic Settings.

In infants and toddlers (less than four years of age), determination of cause and manner of death often requires a complete autopsy. Few evidence-based guidelines exist regarding optimum nervous system sectioning in this population. Over a six-month interval and using a comprehensive section protocol, we categorized cases having neuropathological findings that were critical (Class A), contributory (Class B), or noncontributory (Class C) to the final cause and manner of death. We further evaluated which sections helped make this determination. Among 53 cases (44 infants, 9 toddlers; 26 girls, 27 boys), Class A neuropathology was noted in nine (16.9%). Seven infants had meningoencephalitis (2/7, 28.6%), craniospinal trauma (3/7, 42.8%), brainstem necrosis suggesting Leigh Disease (1/7, 14.3%), and hydrocephalus in Dandy-Walker malformation (1/7, 14.3%); two toddlers had inflicted craniospinal trauma (2/2, 100%). Class B factors were identified in 11/53 (20.8%), including recent hypoxic-ischemic lesions (2/11, 18.2%), meningitis or dural venous sinus thrombosis in systemic sepsis (2/11, 18.2%), multicystic encephalopathy following peripartum asphyxia (2/11, 18.2%), and microcephaly and delayed myelination (Cri-du-Chat Syndrome) (1/11, 9.09%). Class B also included three toddlers (3/11, 27.2%) with features of hippocampal dysgenesis, two in the setting of febrile seizures. Class C comprised normal brains (3/53, 5.7%), and those with findings of uncertain significance, such as white matter and brainstem gliosis (30/53, 56.6%). The sections most valuable for detection of relevant pathology, and thus recommended for routine sampling, were: 1) bilateral hippocampus; 2) cerebral cortex and leptomeninges; and 3) pons or medulla.

Bartonella henselae endocarditis and glomerulonephritis with dominant C3 deposition in a 21-year-old male with a Melody transcatheter pulmonary valve: case report and review of the literature.

We report a case of a 21-year-old young man with underlying congenital heart disease who developed Bartonella henselae endocarditis of the right ventricular outflow tract (RVOT) conduit of his Melody transcatheter (percutaneous) pulmonary valve (TPV), with an initial presentation of glomerulonephritis with a dominant C3 pattern, with renal failure and circulating cryoglobulins. There are few reports of a glomerulonephritis with a dominant C3 pattern presenting as a manifestation of B. henselae endocarditis. While most cases of B. henselae endocarditis affect the aortic valve, in this case the valve damage was to the RVOT of the Melody TPV, a percutaneous transcatheter valve delivery system that had previously replaced his pulmonary homograft, which had become dysfunctional as a result of prior Streptococcus viridans endocarditis. The pulmonary homograft had been in place since childhood as a result of a Ross procedure to repair his congenital aortic stenosis. The patient's renal failure significantly improved after surgical resection of the infected RVOT and institution of appropriate antibiotic therapy.

Adult and child automated immature granulocyte norms are inappropriate for evaluating early-onset sepsis in newborns.

AIM: Automated haematology analysers are increasingly being used. Normal ranges for automated immature granulocyte counts (IG%) are described in adults and children as <1%, but are not reported for newborns, who often have complete blood count with differential in evaluation for early-onset sepsis. Therefore, this study aimed to describe IG% during the first 48 hours of life (HOL) in newborns and determine the clinical factors affecting IG%. METHODS: We carried out retrospective chart reviews for newborns ≥35 weeks gestational age with one or more complete blood count with differential in the first 48 HOL. Clinical history and automated haematology results were reviewed. RESULTS: Forty-seven of 215 subjects had two or more complete blood counts within 48 h. In the first 48 HOL, IG% ranged from 0 to 8.4% (95th percentile 5.2%). At <12 h, 70% of samples had IG% >1%. IG% appears to decrease over time. Earlier hour of life and higher birth weight were independently associated with higher IG%. CONCLUSION: Immature granulocyte counts in newborns appeared to be higher than reported for other age groups. Use of adult and child norms for IG% would not be appropriate for newborns being evaluated for early-onset sepsis.

Histopathology of the stimulated Vagus nerve: primum non nocere.

A primary concern in the utilization of implantable neural interfaces for the treatment of medical diseases is to follow the Hippocratic dictum: First, do no harm. If we are to avoid harm to the Vagus nerve in our use of stimulatory electrodes in the treatment of heart failure, we must understand the structural and functional elements that comprise peripheral nerves, their susceptibility to various types of injury that might be expected to occur secondary to functional electrical stimulation and how to separate the various components of the response of peripheral nervous system elements to stresses that may occur in the complex interactions that take place between electrode and nerve. To this end, we review the functional histology of peripheral nerve, followed by a consideration of salient types of nerve injuries, which have been elucidated through the combination of careful observations of human disease and well-constructed experimental models. We then examine the extant literature on stimulation-induced nerve injury in light of recent developments in the understanding of electropermeabilization of biological membranes. Finally, we briefly discuss our experience using the CardioFit™ electrode on the canine Vagus nerve.