Hemorrhagic Disease of the Newborn: A Case Series Illustrating Preventable Harm.

Newborns are susceptible to postnatal Vitamin K deficiencies from limited placental transfer, gastrointestinal absorption, and bioavailability in breast milk and formula preparations. For over 50 years, the American Academy of Pediatrics has recommended prophylactic vitamin K to prevent hemorrhagic disease in newborns. Yet, public skepticism contributes to increasing refusal rates. We present three cases of vitamin K-dependent bleeding following parental refusal of postnatal prophylaxis. Two patients experienced intracranial hemorrhage with resultant neurological devastation and mortality, respectively. The third child presented with symptomatic hematuria. Perinatal providers must partner with families and advocate vitamin K prophylaxis to limit unnecessary morbidity and mortality.

Heliox Prescribing Trends for Pediatric Critical Asthma.

BACKGROUND: Children with asthma exacerbations requiring pediatric ICU (PICU) admission, known as critical asthma (CA), are prescribed a variety of therapeutic interventions including heliox. Delivered invasively and noninvasively, heliox is employed to enhance deposition of aerosolized medications, improve obstructive pulmonary pathophysiology, and avoid complications associated with invasive mechanical ventilation. We used the Virtual Pediatric Systems database to update estimates of heliox prescription and explore for relationships between heliox and mechanical ventilation frequency and duration. METHODS: We performed a retrospective cohort study using data from 97 PICUs among children 3-17 y of age admitted for CA from 2013-2019. The primary outcome was heliox prescribing rates and trends. Subgroup analyses assessed mechanical ventilation rates and duration by heliox exposure. RESULTS: Of 43,238 subjects studied, 1,070 (2.5%) were prescribed heliox. Mean heliox prescribing rates fell from 4.11% in 2013 to 2.37% in 2019. Heliox use was greater from centers in the South (2.6%) and Midwest (3.3%) as compared to the West (1.6%) and Northeast United States (1.6%, P < .001). In the subgroup assessing mechanical ventilation frequency, mechanical ventilation rates were 273/39,739 (0.7%) and greater for those provided heliox (1.9% vs 0.7%, P < .001). In the subgroup assessing mechanical ventilation duration, no differences in median mechanical ventilation duration were observed (4.94 [interquartile range [IQR] 3.04-6.36] vs 4.63 [IQR 3.11-7.30] d; P = .35) for those with and without heliox. In exploratory adjusted models, noninvasive heliox was not associated with mechanical ventilation. Mortality was rare (206/43,238 [0.47%]) and predominantly among subjects intubated prehospitalization (188/206 [91.3%]). CONCLUSIONS: Heliox as adjunctive therapy for children with CA is uncommon (2.5%) and not associated with mechanical ventilation or decreased mechanical ventilation duration in adjusted models. Updated estimates provided herein inform prospective controlled trial development to better define the role of heliox for CA.

Heliox for Pediatric Critical Asthma: A Multicenter, Retrospective, Registry-Based Descriptive Study.

BACKGROUND: In cases of critical asthma (CA), heliox may be applied as an adjunctive rescue therapy to avoid invasive mechanical ventilation (MV), improve deposition of aerosolized medications, and enhance laminar airflow through obstructed airways. Using the Pediatric Health Information System (PHIS) registry, we evaluate heliox prescribing and explored for differences in MV rates and hospital length of stay (LOS) among children with and without heliox exposure. METHODS: We performed a retrospective cohort study using PHIS data from 42 pediatric intensive care units among children 5-17 years of age admitted for CA from 2010 through 2019. Primary outcomes were heliox prescribing rates and trends. Secondary outcomes were invasive MV rates and LOS assessed in a subgroup of children receiving ≥ 1 adjunctive intervention(s). RESULTS: Of the 19,780 studied, heliox was prescribed in 12.5% and linearly declined from 16.1% in 2010 to 5.6% in 2019. The overall MV rate was 12.8% and was lower in subjects receiving heliox alone (4.9%) compared to heliox plus alternative adjunctive therapies [31.2%] or children receiving non-heliox adjunctive therapies [22.1%], P < .01). Accounting for MV, no difference in LOS was observed. In exploratory adjusted models, MV free hospitalization was associated with heliox-only exposure (OR: 0.33, 95% CI: 0.17-0.63, P < .01) and exposure to multiple adjunctive therapies was associated with MV (OR: 2.48, 95% CI: 1.56-3.94, P < .01). CONCLUSIONS: In this multicenter retrospective study from 42 children's hospitals, heliox prescribing for CA declined over the last decade. Subjects receiving multiple adjunctive therapies more commonly required invasive MV perhaps indicating a greater severity of illness. At this time, prospective trials needed to identify the role of heliox for pediatric CA.

High-flow nasal cannula and bilevel positive airway pressure for pediatric status asthmaticus: a single center, retrospective descriptive and comparative cohort study.

INTRODUCTION: We aimed to describe patient characteristics and clinical outcomes for children hospitalized for status asthmaticus (SA) receiving high-flow nasal cannula (HFNC) or bilevel positive airway pressure (BiPAP). METHODS: We performed a single center, retrospective cohort study among 39 children admitted for SA aged 5-17 years from January 2016 to May 2019 to a quaternary pediatric intensive care unit (PICU). Cohorts were defined by BiPAP versus HFNC exposure and assessed to determine if differences existed in demographics, anthropometrics, comorbidities, asthma severity indices, historical factors, duration of noninvasive ventilation, and asthma-related clinical outcomes (i.e. length of stay, mechanical ventilation rates, exposure to concurrent sedatives/anxiolysis, and rate of adjunctive therapy exposure). RESULTS: Thirty-three percent (n = 13) received HFNC (33%) and 67% (n = 26) BiPAP. Children receiving BiPAP had greater age (10.9 ± 3.7 vs. 6.8 ± 2.2 years, P < 0.01), asthma severity (proportion with severe NHLBI classification: 38% vs. 0%, P < 0.01; median pediatric asthma severity score: 13[12,14] vs. 10[9,12], P < 0.01), previous PICU admissions (62% vs. 15%, P = 0.01), frequency of prescribed anxiolysis/sedation (42% vs. 8%, P = 0.02), and median duration of continuous albuterol (1.7[1,3.1] vs. 0.9[0.7,1.6] days, P = 0.03) compared to those on HFNC. Those on HFNC more commonly were treated comorbid bacterial pneumonia (69% vs. 19%, P < 0.01). No differences in NIV duration, mortality, mechanical ventilation rates, or LOS were observed. CONCLUSIONS: Our data suggest a trial of BiPAP or HFNC appears well tolerated in children with SA. Prospective trials are needed to establish modality superiority and identify patient or clinical characteristics that prompt use of HFNC over BiPAP.

Volatile anesthetic agents for life-threatening pediatric asthma: A multicenter retrospective cohort study and narrative review.

BACKGROUND: Volatile anesthetic agents are described as rescue therapy for children invasively ventilated for critical asthma. Yet, data are currently limited to case series. AIMS: Using the Virtual Pediatric Systems database, we assessed children admitted to a pediatric intensive care unit invasively ventilated for life-threatening asthma and hypothesized ventilation duration and mortality rates would be lower for subjects exposed to volatile anesthetics compared with those without exposure. METHODS: We performed a multicenter retrospective cohort study among nine institutions including children 5-17 years of age invasively ventilated for asthma from 2013 to 2019 with and without exposure to volatile anesthetics. Primary outcomes were ventilation duration and mortality. Secondary outcomes included patient characteristics, length of stay, and anesthetic-related adverse events. A subgroup analysis was performed evaluating children intubated ≥2 days. RESULTS: Of 203 children included in study, there were 29 (14.3%) with and 174 (85.7%) without exposure to volatiles. No differences in odds of mortality (1.1, 95% CI: 0.3-3.9, p > .999) were observed. Subjects receiving volatiles experienced greater median difference in length of stay (4.8, 95% CI: 1.9-7.8 days, p < .001), ventilation duration (2.3, 95% CI: 1-3.3 days, p < .001), and odds of extracorporeal life support (9.1, 95% CI: 1.9-43.2, p = .009) than those without volatile exposure. For those ventilated ≥2 days, no differences were detected in mortality, ventilation duration, length of stay, arrhythmias, or acute renal failure. However, the odds of extracorporeal life support remained greater for those receiving volatiles (7.6, 95% CI: 1.3-44.5, p = .027). No children experienced malignant hyperthermia or hepatic failure after volatile exposure. CONCLUSIONS: For intubated children for asthma, no differences in mechanical ventilation duration or mortality between those with and without volatile anesthetic exposure were observed. Although volatiles may represent a viable rescue therapy for severe cases of asthma, definitive, and prospective trials are still needed.

Repair of Forearm Muscle Herniation Using Local Fascial Flap: A Case Report.

Forearm muscle herniation is a rare but known cause of symptomatic pain in the upper extremity caused by compression or strangulation of the muscle belly through a defect in the overlying fascia. Because of the rarity of this condition, optimal treatment is still widely unknown and debated. To date, there are various treatment methods published, including rest, physiotherapy, primary repair, fasciotomy, fascia lata inlay, onlay or wrap-around, mesh graft, and acellular porcine collagen matrix. In this study, a 61-year old man underwent an ulnar nerve transposition to correct cubital tunnel syndrome, resulting in subsequent symptoms of muscle herniation on the volar aspect of the forearm. Prominent muscle herniation was visible a few weeks after the onset of symptoms and surgical correction of the fascial defect was performed using a local fascial flap. Postoperatively, the patient's herniation symptoms resolved without signs of ulnar nerve entrapment. The rationale for this treatment option is discussed.

Long-term safety of dichloroacetate in congenital lactic acidosis.

We followed 8 patients (4 males) with biochemically and/or molecular genetically proven deficiencies of the E1α subunit of the pyruvate dehydrogenase complex (PDC; 3 patients) or respiratory chain complexes I (1 patient), IV (3 patients) or I+IV (1 patient) who received oral dichloroacetate (DCA; 12.5 mg/kg/12 h) for 9.7 to 16.5 years. All subjects originally participated in randomized controlled trials of DCA and were continued on an open-label chronic safety study. Patients (1 adult) ranged in age from 3.5 to 40.2 years at the start of DCA administration and are currently aged 16.9 to 49.9 years (mean ± SD: 23.5 ± 10.9 years). Subjects were either normal or below normal body weight for age and gender. The 3 PDC deficient patients did not consume high fat (ketogenic) diets. DCA maintained normal blood lactate concentrations, even in PDC deficient children on essentially unrestricted diets. Hematological, electrolyte, renal and hepatic status remained stable. Nerve conduction either did not change or decreased modestly and led to reduction or temporary discontinuation of DCA in 3 patients, although symptomatic worsening of peripheral neuropathy did not occur. We conclude that chronic DCA administration is generally well-tolerated in patients with congenital causes of lactic acidosis and is effective in maintaining normal blood lactate levels, even in PDC-deficient children not consuming strict ketogenic diets.