Department of Pediatrics, Nishi-Kobe Medical Center, 5-7-1 Kojidai, Nishi-ku, Kobe, 651-2273, Japan.
J Infect Chemother. 2012 Aug;18(4):576-80. doi: 10.1007/s10156-011-0341-z. Epub 2011 Nov 17.
Isolated congenital asplenia (ICA) is a rare condition at risk for overwhelming infection. When complicated by invasive infection, the mortality remains high, at greater than 60%. We describe a girl with ICA who developed recurrent meningitis by three different pathogens. The first, meningitis by Escherichia coli, occurred 4 days after premature birth. The other two pathogens were serotype 6B Streptococcus pneumoniae and Haemophilus influenzae type b (Hib), at 18 and 25 months of age, respectively. The patient was successfully treated with prompt antimicrobial therapy in all episodes. Serum anti-polyribosylribitol phosphate (PRP) and anti-6B-type pneumococcal antibodies were below the levels for protective activity after natural infections. Although anti-PRP antibody was significantly increased after Hib vaccination, two (6B and 19F) of seven serotype-specific pneumococcal antibodies were not elevated to protective levels after the second 7-valent pneumococcal conjugate vaccine (PCV7). We, therefore, added a third PCV7. To our knowledge, this is the first neonatal ICA patient with invasive infection and the first case of bacterial meningitis occurring three times. Our findings indicate that monitoring of immune responses after natural infections and vaccinations, and reevaluations of vaccine schedule, are important for ICA patients to prevent subsequent invasive infections.
孤立性先天性脾缺如（ICA）是一种罕见的易发生感染性休克的疾病。当并发侵袭性感染时，死亡率仍然很高，超过 60%。我们描述了一例 ICA 女孩，她由三种不同的病原体引起反复发作性脑膜炎。第一次是大肠杆菌性脑膜炎，发生在早产 4 天后。另外两种病原体是 6B 型肺炎链球菌和乙型流感嗜血杆菌（Hib），分别在 18 个月和 25 个月时感染。所有发作均通过及时的抗菌治疗成功治愈。血清抗多聚核糖醇磷酸（PRP）和抗 6B 型肺炎球菌抗体在自然感染后的保护活性低于水平。尽管 Hib 疫苗接种后抗 PRP 抗体显著增加，但在第二次 7 价肺炎球菌结合疫苗（PCV7）接种后，7 种血清型特异性肺炎球菌抗体中有两种（6B 和 19F）未升高至保护性水平。因此，我们又接种了第三剂 PCV7。据我们所知，这是首例新生儿 ICA 侵袭性感染病例，也是首例发生三次细菌性脑膜炎的病例。我们的发现表明，对自然感染和疫苗接种后的免疫反应进行监测，并重新评估疫苗接种方案，对于预防 ICA 患者随后发生侵袭性感染非常重要。
