Increase in invasive Streptococcus pneumoniae infections in children with sickle cell disease since pneumococcal conjugate vaccine licensure

Invasive pneumococcal disease (IPD) in children with sickle cell disease has decreased with prophylactic penicillin, pneumococcal polysaccharide vaccine, and pneumococcal protein-conjugate vaccine usage. We report 10 IPD cases since pneumococcal protein-conjugate vaccine licensure, including a recent surge of non-vaccine serotypes. IPD continues to be a serious risk in sickle cell disease.     

Invasive pneumococcal disease in children prior to implementation of the conjugate vaccine in the Zurich region,Switzerland

Objective: To describe symptoms, disease manifestations and outcome of invasive pneumococcal disease in children prior to implementation of the pneumococcal vaccine. Patients and methods: Analysis of children younger than 16 years of age with invasive pneumococcal disease (IPD; n = 119). Children with culture‐confirmed IPD, without underlying illness at risk for invasive disease, were included. Results: IPD in 90 children (age: median 2, mean 3.2 years) included 15 with meningitis, 16 with septicaemia, 14 with bacteraemia, 24 with pneumonia and 21 with skin, bone and joint infections. Symptoms of IPD most often described were fever and gastrointestinal symptoms (abdominal pain, vomiting, or diarrhoea), and coughing. More than 90% of children with pneumonia were coughing. Most importantly, clinical signs significantly predictive for severe IPD included tachycardia for sepsis, tachypnea for pneumonia, and meningeal signs for meningitis. Leukocyte, neutrophil and platelet counts were lower and C‐reactive protein concentrations were higher on admission in children with complicated than in children with uncomplicated IPD but, due to wide overlap of these numbers, the difference was not of prognostic help to predict clinical course and outcome. Overall, 40% of children with IPD manifested complications and IPD showed a mortality rate of 6.6%. Conclusions: IPD is a serious disease with a high complication rate and mortality. The clinical signs tachycardia, tachypnea, and meningism were highly predictive for severe IPD. The initial clinical presentation and laboratory evaluation were mostly unpredictable with respect to complications and outcome in contrast to the clinical signs.     

Effect of the pneumococcal conjugate vaccine on pneumococcal carriage in Turkish children

Background: The aim of our study was to evaluate the effect of the seven‐valent pneumococcal conjugate vaccine which has recently been included in the national immunization schedule on the nasopharyngeal carriage of Streptococcus pneumoniae in a group of healthy Turkish children. This is the first study determining the efficacy of this vaccine in Turkey. Methods: One hundred and thirty‐eight children who had completed their pneumococcal vaccination series and 109 unvaccinated control subjects aged 12–59 months were included in the study between October 2007 and April 2008. A single nasopharyngeal swab sample was obtained from each subject. Results: S. pneumoniae was isolated in 32 (12.9%) of 247 subjects. No significant differences were detected in pneumococcal carriage rate between the vaccinees and controls (10.1% vs 16.5%). Prevalence of vaccine type (VT) carriage was statistically lower in the vaccinated group than the controls while non‐vaccine type carriage (NVT) was similar. Most frequently isolated vaccine serotype was 23F in the vaccinated group and 19F in the non‐vaccinated group. Of the isolated S. pneumoniae, 13.3% were penicillin susceptible and 86.7% were non‐susceptible. Vaccinees and controls did not differ statistically with respect to carriage rate of penicillin‐resistant S. pneumoniae. All the pneumococcal isolates were susceptible to ceftriaxone, vancomycin, rifampicin and quinolones. Conclusion: Seven‐valent conjugate vaccine induces long‐term protection against carriage of VT S. pneumoniae in Turkish children. The ability of the conjugate vaccine to reduce transmission of antibiotic resistant S. pneumoniae may be possible if its introduction is coupled with a reduction in inappropriate use of antibiotics.     

Invasive pneumococcal disease in Danish children, 1996-2007, prior to the introduction of heptavalent pneumococcal conjugate vaccine

Aim: The aim of this study was to document the epidemiology, microbiology and outcome of invasive pneumococcal disease (IPD) among children <16 years with quality surveillance data, just prior to the introduction of the heptavalent pneumococcal conjugate vaccine (PCV7) into the Danish routine immunization programme October 2007.
Methods: Clinical and microbiological records on cases of IPD in children <16 years admitted to Hvidovre Hospital, Denmark 1996–2007, were retrospectively reviewed.
Results: We identified 106 cases of IPD. The annual incidence of IPD was 11 per 100 000 in children <16 years, but considerably higher, 62 per 100 000, in children <2 years. Additionally, of the children with pneumococcal meningitis 86% were <2 years. We observed no fatalities. A total of 10% developed sequelae, but of the patients with pneumococcal meningitis 27% developed sequelae. Nine patients had known risk factors. The Streptococcus pneumoniae serotype was available for 81 cases. Seventy-five percent of the IPD cases in children aged <2 years were caused by one of the serotypes contained within PCV7, compared to only 24% in children ≥2 years.
Conclusion: Our data indicate that an estimated 75% of all IPD cases among children <2 years are caused by PCV7 serotypes and might therefore be prevented by PCV7 vaccination.     

Reversible posterior leuko‐encephalopathy in children with sickle cell disease

Children with sickle cell disease (SCD) have high risk of neurologic morbidity and mortality, such as strokes, silent infarcts and TIA's. A retrospective review of magnetic resonance imaging and magnetic resonance angiography identified eight children with radiological and clinical characteristics of reversible posterior encephalopathy (RPLS). These patients had no evidence of previous cerebral infarcts or vasculopathy. Three have died during the 5‐year follow up; one developed a stroke and one a conditional TCD. RPLS needs to be considered in the differential diagnosis of children with SCD that present with acute neurological changes, especially if they are already been hospitalized. Pediatr Blood Cancer 2009;52:373–375. © 2008 Wiley‐Liss, Inc.     

Side effects following COVID-19 vaccination in pediatric patients with sickle cell disease

Vulnerable patient populations have seen decreased rates of vaccination against SARS-CoV2-19 (COVID-19) due to hesitancies and distrust, magnified by a paucity of data for certain populations. The rate of COVID-19 vaccination in children with sickle cell disease (SCD) remains low despite the risk for severe complications, resulting in continued infections and hospitalizations from COVID-19. We sought to describe vaccine reactions, including vaso-occlusive crises, emergency department visits, and hospitalizations, in children with SCD. Our findings will start to provide the necessary vaccine side effect data to inform patients, caregivers, and clinicians considering the COVID-19 primary vaccination series.     

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non‐SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.     

Cochlear implantation in children with sickle cell disease

Sickle cell disease (SCD) is associated with sensorineural hearing loss (SNHL). Although the hearing loss is usually mild, some develop severe‐to‐profound hearing loss, in whom cochlear implants (CI) may be an option. We present the cases of two children with SCD who developed bilateral severe‐to‐profound SNHL and underwent cochlear implantation. One patient became profoundly deaf after an acute episode of dizziness. Imaging indicated bilateral cochlear ossification, making subsequent cochlear implant surgery challenging. The second patient developed bilateral severe‐to‐profound SNHL following acute vaso‐occlusive crises. She went on to have uncomplicated cochlear implant surgery. These cases illustrate the variable manner in which children with SCD may develop SNHL, and the difficulties associated with managing such cases. We recommend that children with SCD should undergo regular audiological assessment. Furthermore, clinicians should be aware of the risk of cochlear fibrosis and ossification and ensure prompt assessment following an acute vaso‐occlusive crisis or unexplained vestibulocochlear event.     

Invasive pneumococcal infections in children with sickle cell disease in the era of penicillin prophylaxis, antibiotic resistance, and 23-valent pneumococcal polysaccharide vaccination

Rates and severity of pneumococcal infections in children with sickle cell disease were examined before licensure of pneumococcal-conjugated vaccine (PVC). Rates of peak invasive infection rates in 1-year-old children with hemoglobin SS and mortality in those 0 to 10 years of age were 36.5 to 63.4 and 1.4 to 2.8 per 1000 person-years, respectively (>10 and 100 times as frequent as in the general population). Overall, 71% of serotyped isolates (n=80) were PVC serotypes and 71% of nonvaccine serotype strains were penicillin-sensitive. Clinical presentation in children with hemoglobin SS (n=71; more with hypotension) and hemoglobin SC (n=18; more with acute chest syndrome, otitis media) differed. Penicillin nonsusceptibility (38% of isolates) varied between geographic study sites. Penicillin prophylaxis appeared less effective against intermediate and resistant strains. Of all infected children, meningitis developed in 20% and 15% died (hemoglobin SS, n=15 and 11; hemoglobin SC, n=1 each). Factors associated with death included age >4 years (58%), serotype 19F, and not being followed by a hematologist (42% each). The pneumococcal-polysaccharide vaccine was 80.4% effective within 3 years after vaccination (95% CI, 39.7, 93.6). Children with sickle cell disease of all ages may benefit from PVC boosted with polysaccharide vaccination.