SARS-CoV-2 variants are associated with different clinical courses in children with MIS-C.

BACKGROUND: Recent infection with SARS­CoV­2 in children has been associated with multisystem inflammatory syndrome in children (MIS-C). SARS­CoV­2 has undergone different mutations. Few publications exist about specific variants and their correlation with the severity of MIS-C. METHODS: This was a single-center, retrospective study including all patients admitted with MIS-C at Rady Children's Hospital-San Diego between May 2020 and March 2022. Local epidemiologic data, including viral genomic information, were obtained from public records. Demographics, clinical presentation, laboratory values, and outcomes were obtained from electronic medical records. RESULTS: The analysis included 104 pediatric patients. Four MIS-C waves were identified. Circulating variants in San Diego during the first wave included clades 20A to C. During the second wave, there were variants from clades 20A to C, 20G, 21C (Epsilon), 20I (Alpha), and 20J (Gamma). The third wave had Delta strains (clades 21A, 21I, and 21J), and the fourth had Omicron variants (clades 21K, 21L, and 22C). MIS-C presented with similar symptoms and laboratory findings across all waves. More patients were admitted to the pediatric intensive care unit (PICU) (74%) and required inotropic support (63%) during the second wave. None of the patients required mechanical circulatory support, and only two required invasive ventilatory support. There was no mortality. CONCLUSIONS: The various strains of SARS-CoV-2 triggered MIS-C with differing severities, with the second wave having a more severe clinical course. Whether the differences in disease severity across variants were due to changes in the virus or other factors remains unknown.

Infants Admitted to US Intensive Care Units for RSV Infection During the 2022 Seasonal Peak.

Importance: Respiratory syncytial virus (RSV) is the leading cause of lower respiratory tract infections (LRTIs) and infant hospitalization worldwide. Objective: To evaluate the characteristics and outcomes of RSV-related critical illness in US infants during peak 2022 RSV transmission. Design, Setting, and Participants: This cross-sectional study used a public health prospective surveillance registry in 39 pediatric hospitals across 27 US states. Participants were infants admitted for 24 or more hours between October 17 and December 16, 2022, to a unit providing intensive care due to laboratory-confirmed RSV infection. Exposure: Respiratory syncytial virus. Main Outcomes and Measures: Data were captured on demographics, clinical characteristics, signs and symptoms, laboratory values, severity measures, and clinical outcomes, including receipt of noninvasive respiratory support, invasive mechanical ventilation, vasopressors or extracorporeal membrane oxygenation, and death. Mixed-effects multivariable log-binomial regression models were used to assess associations between intubation status and demographic factors, gestational age, and underlying conditions, including hospital as a random effect to account for between-site heterogeneity. Results: The first 15 to 20 consecutive eligible infants from each site were included for a target sample size of 600. Among the 600 infants, the median (IQR) age was 2.6 (1.4-6.0) months; 361 (60.2%) were male, 169 (28.9%) were born prematurely, and 487 (81.2%) had no underlying medical conditions. Primary reasons for admission included LRTI (594 infants [99.0%]) and apnea or bradycardia (77 infants [12.8%]). Overall, 143 infants (23.8%) received invasive mechanical ventilation (median [IQR], 6.0 [4.0-10.0] days). The highest level of respiratory support for nonintubated infants was high-flow nasal cannula (243 infants [40.5%]), followed by bilevel positive airway pressure (150 infants [25.0%]) and continuous positive airway pressure (52 infants [8.7%]). Infants younger than 3 months, those born prematurely (gestational age <37 weeks), or those publicly insured were at higher risk for intubation. Four infants (0.7%) received extracorporeal membrane oxygenation, and 2 died. The median (IQR) length of hospitalization for survivors was 5 (4-10) days. Conclusions and Relevance: In this cross-sectional study, most US infants who required intensive care for RSV LRTIs were young, healthy, and born at term. These findings highlight the need for RSV preventive interventions targeting all infants to reduce the burden of severe RSV illness.

Severe alcohol withdrawal in an adolescent male.

Ethanol remains one of the most frequently abused agents by adolescents, exceeding all others except for vaping nicotine, and use is rising. With increased ethanol use comes a greater risk for dependence and potential for alcohol withdrawal syndromes (AWS). Pediatric AWS is extremely rare and poorly characterized in the literature. Pediatric acute care practitioners may have limited exposure to AWS. We report the case of a 16-year-old male with a history of polysubstance abuse who presented with mild AWS and progressed rapidly to delirium tremens. His withdrawal was initially refractory to high dose benzodiazepine therapy but responded well to phenobarbital. This case highlights how rapidly and dangerously AWS can progress if not aggressively treated. Given the rise in adolescent alcohol use and potential for life threatening symptoms, practitioners, especially in acute care specialties such as emergency medicine, critical care, and hospital medicine, would benefit from additional familiarity with AWS diagnoses and management strategies.

Variation in Early Anakinra Use and Short-Term Outcomes in Multisystem Inflammatory Syndrome in Children.

OBJECTIVE: Evidence regarding effectiveness of interleukin-1 receptor antagonism in multisystem inflammatory syndrome in children (MIS-C) is lacking. We characterized variation in initial treatment with anakinra and evaluated cardiovascular outcomes associated with adding anakinra to standard initial therapy. METHODS: We conducted a retrospective cohort study of MIS-C cases in a US surveillance registry from November 2020 to December 2021. Day 0 was the first calendar day of immunomodulatory treatment. Factors associated with initial anakinra use (days 0-1) were identified. We compared cases in patients ages 2-20 years receiving intravenous immunoglobulin (IVIG) and glucocorticoids versus anakinra plus IVIG and/or glucocorticoids on days 0-1, using inverse probability weighting to balance disease severity. Primary outcomes were vasopressor requirement on day 3 and impaired left ventricular ejection fraction on days 3-4. The secondary outcome was 50% reduction in C-reactive protein on day 3. RESULTS: Among 1,516 MIS-C cases at 44 sites, 193 (13%) patients received anakinra alone or with other immunomodulators as initial treatment (range 0-74% by site). Site accounted for 59% of residual variance in anakinra use. After balancing disease severity, initial treatment with anakinra plus IVIG and/or glucocorticoids (n = 121) versus IVIG plus glucocorticoids (n = 389) was not associated with significant differences in vasopressor requirement (25.6% versus 20.1%, respectively; risk ratio [RR] 1.27 [95% confidence interval (95% CI) 0.88-1.84]), ventricular dysfunction (33.7% versus 25.7%, respectively; RR 1.31 [95% CI 0.98-1.75]), or C-reactive protein reduction. CONCLUSION: We identified substantial variation in initial anakinra use in a real-world population of children with MIS-C, but no average short-term improvement in cardiovascular outcomes associated with early addition of anakinra to IVIG and/or glucocorticoids compared to IVIG and glucocorticoids alone.

Predictive Factors for Delayed Surgical Intervention in Children With Epidural Hematomas.

BACKGROUND: Optimal treatment of children with traumatic intracranial epidural hematomas (EDHs) is unknown. We sought to identify clinical and radiographic predictors of delayed surgical intervention among children with EDH admitted for observation. METHODS: We retrospectively identified patients younger than 15 years with acute traumatic EDHs evaluated at our level 1 pediatric trauma center. We excluded patients with penetrating head injuries, recent surgical evacuation of EDH, or depressed skull fracture requiring surgical repair and assigned the remaining subjects to the immediate surgery group if they underwent immediate surgical evacuation, to the supportive-therapy-only group if they underwent observation only, and to the delayed surgery group if they underwent surgery after observation. We abstracted clinical and laboratory findings, surgical interventions, and neurological outcome and measured EDH dimensions and volumes, adjusting for cranial size. We compared clinical and radiographic characteristics among groups and performed receiver-operator characteristic analyses of predictors of delayed surgery. RESULTS: Of 172 patients with EDH, 103 patients met the inclusion criteria, with 6 (6%) in the immediate surgery group, 87 (84%) in the supportive-therapy-only group, and 10 (10%) in the delayed surgery group. Headache, prothrombin time of >14 seconds, EDH maximal thickness of ≥1.1 cm, volume of ≥14 mL, EDH thickness/cranial width index of ≥0.08 and EDH volume/cranial volume index of ≥0.18, and mass effect were associated with delayed surgical intervention. There was no difference in length of stay or functional impairment between the immediate and delayed surgery groups. However, patients in delayed surgery group were more likely to have subjective symptoms at discharge than those in immediate surgery group. CONCLUSIONS: Among patients with EDH admitted for observation, larger EDH, mass effect, headaches, and prothrombin time of >14 seconds were associated with delayed surgical intervention. A larger-scale study is warranted to identify independent predictors of delayed surgery in children under observation for EDH.

Changes in Distribution of Severe Neurologic Involvement in US Pediatric Inpatients With COVID-19 or Multisystem Inflammatory Syndrome in Children in 2021 vs 2020.

Importance: In 2020 during the COVID-19 pandemic, neurologic involvement was common in children and adolescents hospitalized in the United States for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related complications. Objective: To provide an update on the spectrum of SARS-CoV-2-related neurologic involvement among children and adolescents in 2021. Design, Setting, and Participants: Case series investigation of patients reported to public health surveillance hospitalized with SARS-CoV-2-related illness between December 15, 2020, and December 31, 2021, in 55 US hospitals in 31 states with follow-up at hospital discharge. A total of 2253 patients were enrolled during the investigation period. Patients suspected of having multisystem inflammatory syndrome in children (MIS-C) who did not meet criteria (n = 85) were excluded. Patients (<21 years) with positive SARS-CoV-2 test results (reverse transcriptase-polymerase chain reaction and/or antibody) meeting criteria for MIS-C or acute COVID-19 were included in the analysis. Exposure: SARS-CoV-2 infection. Main Outcomes and Measures: Patients with neurologic involvement had acute neurologic signs, symptoms, or diseases on presentation or during hospitalization. Life-threatening neurologic involvement was adjudicated by experts based on clinical and/or neuroradiological features. Type and severity of neurologic involvement, laboratory and imaging data, vaccination status, and hospital discharge outcomes (death or survival with new neurologic deficits). Results: Of 2168 patients included (58% male; median age, 10.3 years), 1435 (66%) met criteria for MIS-C, and 476 (22%) had documented neurologic involvement. Patients with neurologic involvement vs without were older (median age, 12 vs 10 years) and more frequently had underlying neurologic disorders (107 of 476 [22%] vs 240 of 1692 [14%]). Among those with neurologic involvement, 42 (9%) developed acute SARS-CoV-2-related life-threatening conditions, including central nervous system infection/demyelination (n = 23; 15 with possible/confirmed encephalitis, 6 meningitis, 1 transverse myelitis, 1 nonhemorrhagic leukoencephalopathy), stroke (n = 11), severe encephalopathy (n = 5), acute fulminant cerebral edema (n = 2), and Guillain-Barré syndrome (n = 1). Ten of 42 (24%) survived with new neurologic deficits at discharge and 8 (19%) died. Among patients with life-threatening neurologic conditions, 15 of 16 vaccine-eligible patients (94%) were unvaccinated. Conclusions and Relevance: SARS-CoV-2-related neurologic involvement persisted in US children and adolescents hospitalized for COVID-19 or MIS-C in 2021 and was again mostly transient. Central nervous system infection/demyelination accounted for a higher proportion of life-threatening conditions, and most vaccine-eligible patients were unvaccinated. COVID-19 vaccination may prevent some SARS-CoV-2-related neurologic complications and merits further study.

Utilizing extracorporeal membrane oxygenation and surfactant in the management of severe acute respiratory distress syndrome due to hydrocarbon pneumonitis.

Severe cases of hydrocarbon aspiration requiring Extracorporeal Membrane Oxygenation (ECMO) are rarely reported in pediatrics, and 90% of hospitalized patients have a relatively benign clinical course. We describe a 14 month-old female with accidental hydrocarbon ingestion and aspiration due to organic makeup brush cleaner that suffered severe ARDS and multiorgan failure, successfully managed with ECMO and surfactant. She was decannulated after a total of 72 hours on ECMO, extubated on hospital day 15 (HD 15), and discharged home in her normal state of health after one month in the hospital. ECMO and adjunctive therapies such as surfactant may be helpful in the management of severe hydrocarbon pneumonitis and there are limited reports of ECMO as a supportive method for these pediatric patients.

Peripheral IV Administration of Hypertonic Saline: Single-Center Retrospective PICU Study.

OBJECTIVES: To determine the frequency and characteristics of complications of peripherally administered hypertonic saline (HTS) through assessment of infiltration and extravasation. DESIGN: Retrospective cross-sectional study. SETTING: Freestanding tertiary care pediatric hospital. PATIENTS: Children who received HTS through a peripheral IV catheter (PIVC). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We conducted a single-center retrospective review from January 2012 to 2019. A total of 526 patients with 1,020 unique administrations of HTS through a PIVC met inclusion criteria. The primary endpoint was PIVC failure due to infiltration or extravasation. The indication for the administration of HTS infusion was collected. Catheter data was captured, including the setting of catheter placement, anatomical location on the patient, gauge size, length of time from catheter insertion to HTS infusion, in situ duration of catheter lifespan, and removal rationale. The administration data for HTS was reviewed and included volume of administration, bolus versus continuous infusion, infusion rate, infusion duration, and vesicant medications administered through the PIVC. There were 843 bolus infusions of HTS and 172 continuous infusions. Of the bolus administrations, there were eight infiltrations (0.9%). The continuous infusion group had 13 infiltrations (7.6%). There were no extravasations in either group, and no patients required medical therapy or intervention by the wound care or plastic surgery teams. There was no significant morbidity attributed to HTS administration in either group. CONCLUSIONS: HTS administered through a PIVC infrequently infiltrates in critically ill pediatric patients. The infiltration rate was low when HTS is administered as a bolus but higher when given as a continuous infusion. However, no patient suffered an extravasation injury or long-term morbidity from any infiltration.

Expanding the phenotypic and molecular spectrum of NFS1-related disorders that cause functional deficiencies in mitochondrial and cytosolic iron-sulfur cluster containing enzymes.

Iron-sulfur cluster proteins are involved in critical functions for gene expression regulation and mitochondrial bioenergetics including the oxidative phosphorylation system. The c.215G>A p.(Arg72Gln) variant in NFS1 has been previously reported to cause infantile mitochondrial complex II and III deficiency. We describe three additional unrelated patients with the same missense variant. Two infants with the same homozygous variant presented with hypotonia, weakness and lactic acidosis, and one patient with compound heterozygous p.(Arg72Gln) and p.(Arg412His) variants presented as a young adult with gastrointestinal symptoms and fatigue. Skeletal muscle biopsy from patients 1 and 3 showed abnormal mitochondrial morphology, and functional analyses demonstrated decreased activity in respiratory chain complex II and variably in complexes I and III. We found decreased mitochondrial and cytosolic aconitase activities but only mildly affected lipoylation of pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase enzymes. Our studies expand the phenotypic spectrum and provide further evidence for the pathogenicity and functional sequelae of NFS1-related disorders with disturbances in both mitochondrial and cytosolic iron-sulfur cluster containing enzymes.

Clinical application of cell-free next-generation sequencing for infectious diseases at a tertiary children's hospital.

BACKGROUND: Children affected by infectious diseases may not always have a detectable infectious etiology. Diagnostic uncertainty can lead to prolonged hospitalizations, inappropriately broad or extended courses of antibiotics, invasive diagnostic procedures, and difficulty predicting the clinical course and outcome. Cell-free plasma next-generation sequencing (cfNGS) can identify viral, bacterial, and fungal infections by detecting pathogen DNA in peripheral blood. This testing modality offers the ability to test for many organisms at once in a shotgun metagenomic approach with a rapid turnaround time. We sought to compare the results of cfNGS to conventional diagnostic test results and describe the impact of cfNGS on clinical care in a diverse pediatric population at a large academic children's hospital. METHODS: We performed a retrospective chart review of hospitalized subjects at a tertiary pediatric hospital to determine the diagnostic yield of cfNGS and its impact on clinical care. RESULTS: We describe the clinical application of results from 142 cfNGS tests in the management of 110 subjects over an 8-month study period. In comparison to conventional testing as a reference standard, cfNGS was found to have a positive percent agreement of 89.6% and negative percent agreement of 52.3%. Furthermore, 32.4% of cfNGS results were directly applied to make a clinical change in management. CONCLUSIONS: We demonstrate the clinically utility of cfNGS in the management of acutely ill children. Future studies, both retrospective and prospective, are needed to clarify the optimal indications for testing.

Contemporary management of pediatric open skull fractures: a multicenter pediatric trauma center study.

OBJECTIVE: The authors sought to evaluate the contemporary management of pediatric open skull fractures and assess the impact of variations in antibiotic and operative management on the incidence of infectious complications. METHODS: The records of children who presented from 2009 to 2017 to 6 pediatric trauma centers with an open calvarial skull fracture were reviewed. Data collected included mechanism and anatomical site of injury; presence and depth of fracture depression; antibiotic choice, route, and duration; operative management; and infectious complications. RESULTS: Of the fractures among the 138 patients included in the study, 48.6% were frontal and 80.4% were depressed; 58.7% of patients underwent fragment elevation. The average duration of intravenous antibiotics was 4.6 (range 0-21) days. Only 53 patients (38.4%) received a single intravenous antibiotic for fewer than 4 days. and 56 (40.6%) received oral antibiotics for an average of 7.3 (range 1-20) days. Wounds were managed exclusively in the emergency department in 28.3% of patients. Two children had infectious complications, including a late-presenting hardware infection and a superficial wound infection. There were no cases of meningitis or intracranial abscess. Neither antibiotic spectrum or duration nor bedside irrigation was associated with the development of infection. CONCLUSIONS: The incidence of infectious complications in this population of children with open skull fractures was low and was not associated with the antibiotic strategy or site of wound care. Most minimally contaminated open skull fractures are probably best managed with a short duration of a single antibiotic, and emergency department closure is appropriate unless there is significant contamination or fragment elevation is necessary.

The Utility of Infectious and Neurodiagnostic Testing in Children With Complex Febrile Seizures Requiring Mechanical Ventilation.

A retrospective cohort analysis was performed on 79 consecutive patients between 6 months and 5 years admitted to a tertiary hospital with a diagnosis of complex febrile seizures requiring mechanical ventilation from 2011 to 2017 to determine the utility of infectious and neurologic diagnostics. Intubation was used as a proxy for severity of illness. The overall intensive care unit stay was short (95% intubated <24 hours, 88% admitted <3 days). No life-threatening infections were identified, and none required surgical interventions. Electroencephalogram (EEG) was obtained on 43%, 26% of which were abnormal. Sixty-six percent of patients were discharged on rescue benzodiazepine and 20% with maintenance antiseizure medications. Duration of follow-up averaged 4 years (range 1 month to 9 years); 8 patients (10%) were subsequently diagnosed with epilepsy. Our findings suggest that extensive diagnostic evaluations may not be necessary for children with complex febrile seizures requiring mechanical ventilation although the role of EEG is less understood.

A Four-Year-Old with History of Kawasaki Disease Presenting in Acute Shock.

We present a case in which emergency medical services (EMS) intervened on a critically ill child with known giant coronary aneurysms as sequela to her severe complicated Kawasaki disease. This patient's severe shock ultimately ended in cardiac arrest and death. We discuss the keys to recognition, and critical importance to early intervention of pediatric shock in prehospital care. We also detail the cardiac ramifications of Kawasaki disease, steps for prompt identification of high risk complaints in these patients, and opportunities for treatment.

Age-Related Changes in Upper Airway Anatomy Via Ultrasonography in Pediatric Patients.

PURPOSE: Ultrasonography is a portable, noninvasive tool that may be used to evaluate the upper airway. The purpose of our study was to present a systematic approach to identify salient features of the pediatric airway and determine whether ultrasonography can identify anatomical changes that occur with growth and development. METHODS: We present a prospective, observational trial where patients included were between 1 day and 10 years of age presenting for elective surgery who had no known history of unfavorable airway pathology. We sequentially obtained 5 ultrasound views under anesthesia: (1) sagittal sternal notch view of the trachea, (2) sagittal longitudinal view of trachea (LT), (3) axial view at the level of the vocal cords (AVC), (4) axial view at the level of the cricoid membrane (AC), and (5) sagittal longitudinal submental space view (SM). A broadband linear array transducer was used to identify airway structures and perform measurements. RESULTS: Eighty-four percent of enrolled patients underwent airway imaging and were analyzed using multiple regression and Spearman correlation (ρ). In view 1, tracheal diameter via sagittal sternal notch view was immeasurable because of air disturbance. In the LT view, the distance from the skin to the cricothyroid membrane (LT1) did not statistically increase with age in days (P = 0.06); however, the distance from the cricoid to thyroid cartilage (LT2) did correlate to age (P < 0.001; 99% confidence interval [CI], 1.8 × 10-5, 7.7 × 10-5; ρ = 0.77, P = 0.001). We found a statistically significant relationship between age and the distance between the anterior and posterior commissures (AVC2; P < 0.001; 99% CI, 1.0 × 10-4, 1.7 × 10-4; ρ = 0.80, P < 0.001), the distance from the skin to the posterior commissure (AVC3; P < 0.001; 99% CI, 9.6 × 10-5, 2.0 × 10-4; ρ = 0.73, P < 0.001), the distance to the cricoid cartilage (AC; P < 0.001; 99% CI, 2.0 × 10-5, 7.7 × 10-5; ρ = 0.66, P < 0.001), and the distance from the tongue base to the soft palate (SM2; P < 0.001; 9% CI, 1.8 × 10-4, 3.9 × 10-4; ρ = 0.85, P < 0.001). There were no significant relationships between age and AVC1 (P = 0.16) and SM1 (P = 0.44). CONCLUSIONS: Airway ultrasound is a feasible tool to evaluate the pediatric airway in children younger than 10 years; however, the detection of age-related changes of certain structures is limited to select measurements.

Use of Simulation Strategies to Predict Subtherapeutic Meropenem Exposure Caused by Augmented Renal Clearance in Critically Ill Pediatric Patients With Sepsis.

OBJECTIVES: The objectives of this study were to 1) define extent and potential clinical impact of increased or decreased renal elimination of meropenem in children with sepsis, based on analysis of renal function during the first 2 days of PICU stay; and 2) estimate the risk of subtherapeutic meropenem exposure attributable to increased renal clearance. METHODS: This retrospective study evaluated patients with a diagnosis of sepsis, receiving meropenem from the PICU at Rady Children's Hospital San Diego from 2015-2017. Meropenem exposure was estimated by using FDA-approved doses (20 and 40 mg/kg/dose) on day 1 and day 2 of PICU stay, based on a population pharmacokinetic (PK) model. For this population with sepsis, we assessed time-above-minimum inhibitory concentration (T>MIC) for pathogen MICs. RESULTS: Meropenem treatment was documented in 105 episodes of sepsis with a 48% rate of pathogen detection. By day 2, increased eGFR (>120 mL/min/1.73 m2) was documented in 49% of patients, with 17% meeting criteria for augmented renal clearance ([ARC] >160 mL/min/1.73 m2) and 10%, for decreased function. Simulations documented that 80% of PICU patients with ARC did not achieve therapeutic meropenem exposure for Pseudomonas aeruginosa with a MIC of 2, using standard doses to achieve a pharmacodynamic goal of 80% T>MIC. CONCLUSIONS: Approximately 3 of every 20 children with sepsis exhibited ARC during the first 48 hours of PICU stay. Simulations documented an increased risk for subtherapeutic meropenem exposure, suggesting that higher meropenem doses may be required to achieve adequate antibiotic exposure early in the PICU course.

Perioperative hypothermia in neonatal intensive care unit patients: effectiveness of a thermoregulation intervention and associated risk factors.

BACKGROUND: Hypothermia in neonatal intensive care unit patients is associated with morbidity. Perioperative normothermia is the standard of care. AIMS: We hypothesized that a quality improvement intervention (transport protocol, transport education, ongoing monitoring) would decrease the incidence of perioperative hypothermia. Secondarily, we hypothesized that patients undergoing surgery at a postmenstrual age of <37 weeks or at a weight of <1.5 kg would be at higher risk for perioperative hypothermia. METHODS: Lean Six Sigma methodology was used to institute a quality improvement intervention. In a retrospective chart review, we identified 708 cases for which the neonatal intensive care unit was the preoperative and postoperative destination and documented patient characteristics, including postoperative temperature. Cardiac surgical cases and cases with no postoperative temperature record were excluded. RESULTS: Patients in the postintervention group had a statistically significant decrease in hypothermia compared to those in the preintervention group (P < 0.001; OR: 0.17; 95% CI: 0.09-0.31). The absolute risk of hypothermia was 23% in the preintervention group and 6% in the postintervention group. Weight <1.5 kg on day of surgery (P = 0.45; OR: 0.63; 95% CI: 0.16-2.24) and postmenstrual age (P = 0.91; OR: 1.07; 95% CI: 0.33-3.98) were not risk factors. Odds of hypothermia were increased in patients undergoing interventional cardiology procedures (P = 0.003; OR: 17.77; 95% CI: 2.07-125.7). CONCLUSIONS: Perioperative hypothermia is a challenge in the care of neonatal intensive care unit patients; however, a thermoregulation intervention can decrease the incidence with sustained results. Future studies can examine why certain procedures have a tendency toward increased perioperative hypothermia, determine the relative value of quality improvement interventions, and characterize the morbidity and mortality associated with perioperative hypothermia in neonatal intensive care unit patients.

Long-term immune reconstitution after anti-CD52-treated or anti-CD34-treated hematopoietic stem cell transplantation for severe T-lymphocyte immunodeficiency.

BACKGROUND: Results of treatment of severe T-lymphocyte immunodeficiencies by means of hematopoietic stem cell (HSC) transplantation have improved. T cell-depleted haploidentical transplantations are successful if there is no HLA-identical donor. Methods to remove T lymphocytes include addition of anti-CD52 antibodies and CD34(+) HSC selection. OBJECTIVE: Assessment of long-term immune function is important after these treatments. We looked at immune reconstitution in 36 survivors for more than 2 years after HSC transplantation for severe T-lymphocyte immunodeficiencies and compared engraftment quality between the 2 T-lymphocyte depletion methods. METHODS: Chimerism, T- and B-lymphocyte subsets, immunoglobulin levels, and specific antibody production at last follow-up were examined. The chi(2) (Fisher exact test) and Wilcoxon rank sum analyses were used to compare the groups. RESULTS: Nineteen patients received anti-CD52-treated and 19 anti-CD34-treated HSCs. More anti-CD52-treated patients had full donor myeloid chimerism (P = .025). All patients had full donor T-lymphocyte chimerism. There was no difference in donor B-lymphocyte chimerism, but significantly more anti-CD52-treated patients had class-switched memory B lymphocytes (P = .024), normal IgG levels, and normal responses to tetanus and Haemophilus influenzae type B vaccination. More anti-CD52-treated patients with common gamma chain or Janus-associated kinase 3 severe combined immunodeficiency had donor B lymphocytes. CONCLUSION: Long-term T-lymphocyte function is good with either treatment method, with a low incidence of graft-versus-host disease. The results imply more incomplete donor chimerism in anti-CD34-treated patients with less B-lymphocyte function.

Family study of the inheritance of pectus excavatum.

BACKGROUND: The most common congenital deformity of the chest wall is pectus excavatum, a malformation that is present in between 1 in 400 and 1 in 1000 live births and causes the body of the sternum to be displaced, producing a depression. There are many different shapes of the pectus, and multiple factors probably contribute to the final form. The etiology of pectus excavatum is uncertain, but a familial tendency has been found in clinical experience, where it may be seen in more than one sibling. Pectus excavatum is commonly associated with connective tissue disorders such as Marfan and Ehlers Danlos syndromes. Extensive literature review failed to identify articles documenting families with multiple affected members. PURPOSE: The purpose of this study was to collect evidence that pectus excavatum is familial and may be an inherited disorder. METHODS: Using the Children's Surgical Specialty Group database at Children's Hospital of The King's Daughters, families with more than one affected individual were selected. With Institutional Review Board-approved informed consent, 34 families agreed to participate. Family histories were obtained, and a 4-generation pedigree was constructed for each family. Forty questions were asked about each individual's medical history, and comprehensive systems review included features of connective tissue-related problems. Inheritance patterns for each family were determined by pedigree analysis. RESULTS: A total of 14 families suggested autosomal dominant inheritance, 4 families suggested autosomal recessive inheritance, and 6 families suggested X-linked recessive inheritance. Ten families had complex inheritance patterns. Pectus excavatum occurred more frequently in males than in females (1.8:1). Long arms, legs, and fingers; high-arched palate; mitral valve prolapse; heart arrhythmia; scoliosis; double jointedness; flexibility; flat feet; childhood myopia; poor healing; and easy bruising were commonly associated with pectus excavatum. CONCLUSIONS: Pedigree analysis of 34 families provides evidence that pectus excavatum is an inherited disorder, possibly of connective tissue. Although some families demonstrate apparent Mendelian inheritance, most appear to be multifactorial.