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Abstract
Purpose
Because children with asplenia have an increased risk of fulminant infection associated with a high fatality, chemoprophylaxis, and vaccinations against encapsulated bacteria are recommended. However, there have been few reports of the burden of severe bacterial infection and the current status of chemoprophylaxis and immunization among children with asplenia in Korea.
Methods
We conducted a retrospective study including children with asplenia who were treated at our institute between January 1997 and December 2016.
Results
From a total of 213 children with asplenia, 114 (53.5%) had congenital asplenia and 58 (27.2%) had functional asplenia. The remaining 41 (19.3%) had acquired asplenia with the median age at splenectomy being 12.2 years (range, 5.0 to 16.9 years); the most common cause of splenectomy was hereditary spherocytosis (39.0%). The chemoprophylaxis rate was 16.4%. The immunization rates were 44.1% for pneumococcus, 53.0% for Haemophilus influenzae type B, and 10.7% for meningococcus. The incidence of invasive bacterial infection among children with asplenia was 0.28/100 person-year; a total of six episodes (2.8%) were observed in five patients with congenital asplenia and one patient with functional asplenia. The median age for these infections was 15 months (range, 4 to 68 months). Five of the six episodes were bacteremia, and the other was meningitis. The most common pathogen was Streptococcus pneumoniae (n=3), followed by H.influenzae (n=1). Three of the six patients (50.0%) died, all of whom had pneumococcal bacteremia. None of the six had chemoprophylaxis or proper vaccinations.
Figures and Tables
Fig. 1
Chemoprophylaxis and pneumococcal vaccination status by year at diagnosis. As there have been no cases of invasive bacterial infection since 2008, asplenic children were divided into two groups by diagnosed year, respectively: ones who were diagnosed with asplenia between 1997 and 2007 and the others who were diagnosed with asplenia between 2008 and 2016. The rate of chemoprophylaxis was 10.2% (13/127) among children who were diagnosed with asplenia between 1997 and 2007, but 30.2% (26/86) were received chemoprophylaxis among those who were diagnosed between 2008 and 2016. Similarly, in pneumococcal vaccination, 22.6% (19/84) were vaccinated completely among children who were diagnosed with asplenia between 1997 and 2007, but 58.3% (49/84) were vaccinated completely among those who were diagnosed between 2008 and 2016.
Table 1
Characteristics of Children with Acquired, Congenital, or Functional Asplenia
Values are presented as number (%) or median (range).
*The most common cause of splenectomy was hereditary spherocytosis (n=16, 39.0%) followed by idiopathic thrombocytopenic purpura (n=11, 26.8%), splenic disease (n=6, 14.6%), trauma (n=5, 12.2%), and others (n=2, 4.9%). Three out of six patients with splenic disease had a splenic cyst, two had splenic infarction and one had splenic torsion. One out of others was pancreatitis and the other was secondary splenomegaly by portal vein thrombosis.
†Being received prophylactic antibiotics at least 12 months among patients who observed for 12 months or longer.
Abbreviations: CCHD, complex congenital heart disease; NA, not applicable.
Table 2
Characteristics of Episodes of Invasive Bacterial Infection in Patients with Asplenia
*Immunization status at infection.
Abbreviations: Pnc, pneumococcal conjugate or polysccharide vaccine; Hib, Haemophilus influenzae type B vaccine; Mnc, meningococcal vaccine; AVSD, atrioventricular septal defect; BA, biliary atresia; HLHS, hypoplastic left heart syndrome; IAA, interruption of aortic arch; TGA, transposition of great arteries; PA, pulmonary atresia; DORV, double outlet of right ventricle; TAPVR, total anomalous of pulmonary venous return.
Table 3
Vaccination Status of Asplenic Children against Encapsulated Bacteria
Values are presented as number (%).
*Pnc: 23-valent polysaccharide vaccine or protein-conjugate vaccine against Streptococcus pneumoniae.
†Hib: Protein-conjugate vaccine against Haemophilus influenzae type B.
‡Mnc: Protein-conjugate vaccine against Neisseria meningitidis serogroup A, C, W-135, and Y.
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