Clinical practice guidelines: management of severe bronchiolitis in infants under 12 months old admitted to a pediatric critical care unit.

PURPOSE: We present guidelines for the management of infants under 12 months of age with severe bronchiolitis with the aim of creating a series of pragmatic recommendations for a patient subgroup that is poorly individualized in national and international guidelines. METHODS: Twenty-five French-speaking experts, all members of the Groupe Francophone de Réanimation et Urgence Pédiatriques (French-speaking group of paediatric intensive and emergency care; GFRUP) (Algeria, Belgium, Canada, France, Switzerland), collaborated from 2021 to 2022 through teleconferences and face-to-face meetings. The guidelines cover five areas: (1) criteria for admission to a pediatric critical care unit, (2) environment and monitoring, (3) feeding and hydration, (4) ventilatory support and (5) adjuvant therapies. The questions were written in the Patient-Intervention-Comparison-Outcome (PICO) format. An extensive Anglophone and Francophone literature search indexed in the MEDLINE database via PubMed, Web of Science, Cochrane and Embase was performed using pre-established keywords. The texts were analyzed and classified according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. When this method did not apply, an expert opinion was given. Each of these recommendations was voted on by all the experts according to the Delphi methodology. RESULTS: This group proposes 40 recommendations. The GRADE methodology could be applied for 17 of them (3 strong, 14 conditional) and an expert opinion was given for the remaining 23. All received strong approval during the first round of voting. CONCLUSION: These guidelines cover the different aspects in the management of severe bronchiolitis in infants admitted to pediatric critical care units. Compared to the different ways to manage patients with severe bronchiolitis described in the literature, our original work proposes an overall less invasive approach in terms of monitoring and treatment.

End-Stage Respiratory Failure Secondary to Bronchiolitis Obliterans Syndrome Induced by Toxic Epidermal Necrosis, Also Known as Lyell Syndrome: A Case Report.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but serious dermatologic diseases. They can be associated with systemic manifestations such as bronchiolitis obliterans syndrome (BOS). SJS/TEN-induced BOS is associated with a poor prognosis, and no guidelines exist regarding its management. Several case reports have described the association between SJS/TEN and BOS, with few patients undergoing lung transplantation as a last resort therapy. Unfortunately, in the published reports, none of the transplanted patients were observed for a long period of time after the transplantation; therefore, the long-term mortality as well as the risk of recurrence of BOS could not be inferred from these reports. CASE REPORT: We present the case of a young patient diagnosed with SJS complicated by BOS and end-stage respiratory failure refractory to corticosteroid therapy. She underwent bilateral lung transplantation with an outstanding outcome at 5-year follow-up. CONCLUSION: SJS/TEN-induced BOS might have a favorable evolution and long-term outcomes following lung transplantation. However, prospective studies are needed to confirm this finding.

ZIKA vasculitis: A new cause of stroke in children?

Viral infectious vasculitis is a cause of stroke in children. Zika virus infection is often asymptomatic. Neurological complications have however been reported: Guillain-Barré, ADEM, meningoencephalitis, myelitis, microcephaly in the fetus of infected mothers. We discuss the case of a child presenting acute infection with ZIKV that was associated with a stroke. A 10-months-old boy without medical history presented after 2days of fever and cutaneous rash, conjunctivitis on day 5, a right hemiparesis on day 6. Brain MRI found an ischemic stroke in the left superficial MCA territory with distal occlusion of left M1 portion of the MCA. Specific real-time reverse PCR detected Zika virus in serum. There are no known cases of cerebral infarction associated with Zika infection. However, cases of cerebral infarcts associated with dengue vasculitis have rarely been described. It has been recently showed that there is a tropism of the Zika virus for endothelial cells. This could be responsible for stroke, possibly particularly in young children.

Peculiar Clinical Presentation of Coxsackievirus B4 Infection: Neonatal Restrictive Cardiomyopathy.

Introduction Restrictive cardiomyopathy in fetuses and neonates is extremely rare and has a poor outcome. Its etiology in neonates is elusive: metabolic diseases (e.g., Gaucher, Hurler syndrome), neuromuscular disorders (e.g., muscular dystrophies, myofibrillar myopathies), or rare presentation of genetic syndromes (e.g., Coffin-Lowry syndrome) account for a minority of the cases, the majority remaining idiopathic. Case Study We report the case of a 17-day-old male infant presenting cardiogenic shock following a restrictive dysfunction of the left ventricle. Postmortem investigations revealed coxsackievirus B4 myocarditis with histological lesions limited to the left heart. However, polymerase chain reaction (PCR) for coxsackievirus B4 was positive in the left as well as in the right ventricular samples. Conclusion In conclusion, coxsackievirus myocarditis is a cause of restrictive cardiomyopathy, and its diagnosis should involve PCR screening as a more sensitive technique.

Invasive pneumococcal disease in children can reveal a primary immunodeficiency.

BACKGROUND: About 10% of pediatric patients with invasive pneumococcal disease (IPD) die from the disease. Some primary immunodeficiencies (PIDs) are known to confer predisposition to IPD. However, a systematic search for these PIDs has never been carried out in children presenting with IPD. METHODS: We prospectively identified pediatric cases of IPD requiring hospitalization between 2005 and 2011 in 28 pediatric wards throughout France. IPD was defined as a positive pneumococcal culture, polymerase chain reaction result, and/or soluble antigen detection at a normally sterile site. The immunological assessment included abdominal ultrasound, whole-blood counts and smears, determinations of plasma immunoglobulin and complement levels, and the evaluation of proinflammatory cytokines. RESULTS: We included 163 children with IPD (male-to-female ratio, 1.3; median age, 13 months). Seventeen children had recurrent IPD. Meningitis was the most frequent type of infection (87%); other infections included pleuropneumonitis, isolated bloodstream infection, osteomyelitis, endocarditis, and mastoiditis. One patient with recurrent meningitis had a congenital cerebrospinal fluid fistula. The results of immunological explorations were abnormal in 26 children (16%), and a PID was identified in 17 patients (10%), including 1 case of MyD88 deficiency, 3 of complement fraction C2 or C3 deficiencies, 1 of isolated congenital asplenia, and 2 of Bruton disease (X-linked agammaglobulinemia). The proportion of PIDs was much higher in children aged >2 years than in younger children (26% vs 3%; P < .001). CONCLUSIONS: Children with IPD should undergo immunological investigations, particularly those aged >2 years, as PIDs may be discovered in up to 26% of cases.

Next-generation sequencing (NGS) as a fast molecular diagnosis tool for left ventricular noncompaction in an infant with compound mutations in the MYBPC3 gene.

Left ventricular noncompaction (LVNC) is a clinically heterogeneous disorder characterized by a trabecular meshwork and deep intertrabecular myocardial recesses that communicate with the left ventricular cavity. LVNC is classified as a rare genetic cardiomyopathy. Molecular diagnosis is a challenge for the medical community as the condition shares morphologic features of hypertrophic and dilated cardiomyopathies. Several genetic causes of LVNC have been reported, with variable modes of inheritance, including autosomal dominant and X-linked inheritance, but relatively few responsible genes have been identified. In this report, we describe a case of a severe form of LVNC leading to death at 6 months of life. NGS sequencing using a custom design for hypertrophic cardiomyopathy panel allowed us to identify compound heterozygosity in the MYBPC3 gene (p.Lys505del, p.Pro955fs) in 3 days, confirming NGS sequencing as a fast molecular diagnosis tool. Other studies have reported neonatal presentation of cardiomyopathies associated with compound heterozygous or homozygous MYBPC3 mutations. In this family and in families in which parental truncating MYBPC3 mutations are identified, preimplantation or prenatal genetic screening should be considered as these genotypes leads to neonatal mortality and morbidity.

Supraclavicular ultrasound-guided catheterization of the subclavian vein in pediatric and neonatal ICUs: a feasibility study.

OBJECTIVES: To assess the feasibility of ultrasound-guided supraclavicular catheterization of the subclavian vein in pediatric and neonatal ICU. DESIGN: Retrospective cohort. SETTING: Ten-bed pediatric medicosurgical ICU and 15-bed neonatal ICU. PATIENTS: Children and newborns undergoing supraclavicular ultrasound-guided subclavian vein catheterization from March 2010 to September 2010. MEASUREMENTS: The placement of central venous catheter in ICU was carried out either by one of the experts in ultrasound-guided puncture of the unit or by a novice completely supervised by the expert. The success and the early complications were recorded. A comparison between novice and expert operators was also made. RESULTS: Forty-two catheters were placed in 40 children. The median age and median weight were, respectively, 6.5 months and 6.5 kg. The success rate was 97.6% (one failure) and the early complication rate was 4.7% (one pneumothorax and one arterial puncture); 61% of children breathed spontaneously during the catheter placement. No significant difference was found between expert and novice operators. CONCLUSIONS: Supraclavicular ultrasound-guided catheterization of the subclavian vein in pediatric and neonatal ICU seems to be a promising technique in the context of emergency. It is safe, reliable, with few early complications. Furthermore, it does not compromise the airways of the patient owing to the low level of sedation needed for its placement.

[Anti-PF4 antibodies and thrombophlebitis in a child with cerebral venous thrombosis]. / Anticorps anti-PF4 et thrombopénie chez un enfant avec thrombophlébite cérébrale.

We report the case of a 13-year-old girl, with a cerebral venous thrombosis treated initially by heparin. In front of this early thrombocytopenia, in spite of the absence of preliminary treatment by heparin, the "heparin-dependent" anti-PF4 antibodies ELISA assay was realized and turned out to be strongly positive. In spite of the negativity of the specific functional tests, the heparinotherapy was substituted by a treatment by danaparoïd of sodium, allowing a clinico-biological improvement. Because of the low score of HIT imputability, we realized a specific antibodies anti-PF4 alone assay which was strongly positive. This specific positivity probably explains the positivity of the antibodies anti PF4 "heparin-dependent" assay. The originality of this case lies in the young age of the patient and in the probably pathogenic character of these unusual antibodies, in addition markers of an auto-immune disease.