• Pediatric Infection and Vaccine
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• v.19 no.3
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• pp.89-110
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• 2012

Meningococcal Disease, manifesting as meningitis and septicemia, is a life-threatening bacterial infection that results in significant morbidity and mortality, particularly in childhood. Its epidemic potential and limited opportunities for clinical intervention due to its rapid course present unique public health and clinical challenges. Incidence is highest in infants and young children, with a secondary peak of risk in adolescents. Approximately 10% of cases are fatal and survivors can be left with serious and permanent sequelae including amputations, hearing loss and cognitive impairment. Transmission is only from human-to-human, by infected respiratory tract secretions or saliva and therefore crowding poses a tremendously elevated risk for disease development. Military recruits and university students are at high risk due to the high carriage rate in adolescents, their behavior patterns and close contact. Menveo$^{(R)}$ (Novartis Vaccines and Diagnostics), a novel quadrivalent meningococcal conjugate vaccine directed against meningococcal serogroups A, C, W-135 and Y, has been shown to be immunogenic and well tolerated in all age groups and was recently licensed for use in Korea. Recent cases and deaths among military recruits drew public attention to their elevated risk and the Korean government has recommended vaccination of all new military recruits. Many Korean students seek to attend school, university, or language institutes in countries where routine meningococcal vaccination is required - clinicians should be aware of such requirements to ensure that students are vaccinated prior to arrival in the destination country.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.101-103
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• 2005

Meningococcal disease is not rarely associated with abnormalities of the complement system. We experienced a case of C9 deficiency with meningococcal meningitis from a 12-year-old girl. Identification of complement deficiency has implications for management, including family studies, prophylaxis, vaccination, and altered threshold for infection screening and treatment.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.721-724
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• 2009

Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to the loss of complement lytic function, and affected patients show increased susceptibility to recurrent meningococcal meningitis and systemic Neisseria gonorrhoeae infection. In September 2003, an 11-year-old female patient presented at our outpatient department with high fever, lower leg pain, headache, and petechiaes. She rapidly progressed to coma but later achieved full recovery due to prompt treatment. Her final diagnosis was meningococcal sepsis and arthritis. Her elder brother also had a similar bacterial meningoencephalitis history, which encouraged us to perform analyses for complement component and gene mutations. Resultantly, both the brother and sister were found to have the same mutation in the C7 gene. Subsequently, vaccinations of the meningococcal vaccine meningococcal vaccine ($Menomune^{(R)}$) were administered. However, in September 2006, the brother expired due to acute micrococcus meningoencephalitis. At present, the 16-year-old female patient is healthy. Here, we report a Korean family with a hereditary C7 deficiency with susceptibility to meningococcal infections due to C7 gene mutation.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.45-51
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• 2006

Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called 'nephritic factor'(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

• Pediatric Infection and Vaccine
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• v.24 no.3
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• pp.160-167
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• 2017

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. Conclusions: Although there is an increased risk of a severe infection proper vaccinations and chemoprophylaxis are still lacking. Physicians should be encouraged to implement appropriate chemoprophylaxis and immunizations for patients with asplenia.