Thrombosis and hemorrhage experienced by hospitalized children with SARS-CoV-2 infection or MIS-C: Results of the PICNIC registry.

INTRODUCTION: Coagulopathy and thrombosis associated with SARS-CoV-2 infection are well defined in hospitalized adults and leads to adverse outcomes. Pediatric studies are limited. METHODS: An international multicentered (n = 15) retrospective registry collected information on the clinical manifestations of SARS-CoV-2 and multisystem inflammatory syndrome (MIS-C) in hospitalized children from February 1, 2020 through May 31, 2021. This sub-study focused on coagulopathy. Study variables included patient demographics, comorbidities, clinical presentation, hospital course, laboratory parameters, management, and outcomes. RESULTS: Nine hundred eighty-five children were enrolled, of which 915 (93%) had clinical information available; 385 (42%) had symptomatic SARS-CoV-2 infection, 288 had MIS-C (31.4%), and 242 (26.4%) had SARS-CoV-2 identified incidentally. Ten children (1%) experienced thrombosis, 16 (1.7%) experienced hemorrhage, and two (0.2%) experienced both thrombosis and hemorrhage. Significantly prevalent prothrombotic comorbidities included congenital heart disease (p-value .007), respiratory support (p-value .006), central venous catheter (CVC) (p = .04) in children with primary SARS-CoV-2 and in those with MIS-C included respiratory support (p-value .03), obesity (p-value .002), and cytokine storm (p = .012). Comorbidities prevalent in children with hemorrhage included age >10 years (p = .04), CVC (p = .03) in children with primary SARS-CoV-2 infection and in those with MIS-C encompassed thrombocytopenia (p = .001) and cytokine storm (p = .02). Eleven patients died (1.2%), with no deaths attributed to thrombosis or hemorrhage. CONCLUSION: Thrombosis and hemorrhage are uncommon events in children with SARS-CoV-2; largely experienced by those with pre-existing comorbidities. Understanding the complete spectrum of coagulopathy in children with SARS-CoV-2 infection requires ongoing research.

Autosomal Recessive Agammaglobulinemia Due to a Homozygous Mutation in PIK3R1.

The role of class IA phosphoinositide 3 kinases (PI3Ks) in immune function and regulation continues to expand with the identification of greater numbers of genetic variants. This case report is the second reported case of a homozygous premature stop codon within the PIK3R1 gene leading to autosomal recessive agammaglobulinemia. The proband, born to consanguineous parents, presented at 10 months of age with a history of oropharyngeal petechiae and bleeding from the mouth, gums, and tear ducts. Initial investigations revealed thrombocytopenia, neutropenia and the absence of B cells. Further genetic testing via a custom next-generation sequencing panel confirmed the presence of a homozygous mutation in PIK3R1, c.901 C>T, a premature stop codon at amino acid position 301. Given their many roles in immune regulation, recessive mutations in the PlK3R1 gene should be considered in infants presenting with hypogammaglobulinemia or agammaglobulinemia, particularly in the setting of parental consanguinity.

Group A Streptococcal Septic Hip Arthritis in a Child With Spastic Triplegic Cerebral Palsy.

Reports of septic hip arthritis in children with cerebral palsy are exceedingly rare. This case report describes a 10-year-old boy with spastic triplegic cerebral palsy (Gross Motor Functional Classification System), who presented with fever and irritability. This case highlights the difficulties in diagnosing septic joint arthritis in patients with cerebral palsy who are nonverbal and have limited mobility. A high index of suspicion is necessary in this population when presented with fever and new limitations in mobility.

Epidemiology of haemophilus influenzae invasive disease in Jamaica, 1990-1993

Haemophilus influenzae (H influenzae) invasive disease was studied retrospectively over a four-year period in children admitted to the Bustamante Hospital for Children in Kingston, Jamaica. A total of 86cases were identified. The mean estimated annual incidence of H influenzae invasive disease in Kingston and St. Andrew was 39 per 100,000 children under 5 years of age. The majority (77percent) of cases were in the under 2-year age group. A distinct seasonal pattern was noted, with a significantly higher proportion of patients (57-73percent) presenting in the cooler months. Meningitis was the most common clinical diagnosis, accounting for 76percent of the cases. Poor outcome was demonstrated in 21.5percent of patients with meningitis. Sensitivity testing of H influenzae isolates revealed a resistance rate of 26percent for ampicillin and 7percent for chloramphenicol. The epidemiological findings in this study provide reasonable guidelines for empiric antibiotic therapy and also support the need to seriously consider vaccine prophylaxis in Jamaican children.(Au)

The epidemiology of Haemophilus influenzae invasive disease n the Kingston and St. Andrew Region, Jamaica - abstract

Haemophilus influenzae invasive disease was studied retrospectively over a four year period in children admitted to the Bustamante Hospital for Children in Kingston, Jamaica. A total of 86 cases wwere identified. The estimated annual incidence of H. influenzae invasive disease in Kingston and St Andrew ranged from 39 to 45 per 100,000 children <5 years of age. The majority (77 percent ) of cases were in the < 2 year age group. A distinct seasonal pattern was noted, with a significantly higher proportion of patients (57 - 73 percent ) presenting in the cooler months. Similar seasonal variations have been described in the literature. Meningitis was the most clinical diagnosis, accounting for 76 percent of the cases. Poor outcome was demonstrated in 21.5 percent of patients with meningitis. Sensitivity testing of Haemophilus isolates revealed a resistance rate oof 26 percent for ampicillin and 7 percent for chloramphenicol. The epidemiological findings in this study do not differ significantly from those described in the literature for many developed countries. (AU)