S Hoare1, O El-Shazali, J E Clark, A Fay, A J Cant. 1. Paediatric Infectious Diseases Unit, Newcastle General Hospital, Newcastle upon Tyne, UK. simon.hoare@dial.pipex.com
Abstract
BACKGROUND AND AIMS: The incidence of complement abnormalities in the UK is not known. It is suggested in at least three major paediatric textbooks to test for abnormalities of the complement system following meningococcal disease (MCD). METHODS: Over a four year period, surviving children with a diagnosis of MCD had complement activity assessed. A total of 297 children, aged 2 months to 16 years were screened. RESULTS: All children except one had disease caused by B or C serogroups. One child, with group B meningococcal septicaemia (complicated by disseminated intravascular coagulation and who required ventilation and inotropic support) was complement deficient. C2 deficiency was subsequently diagnosed. She had other major pointers towards an immunological abnormality prior to her MCD. CONCLUSION: It is unnecessary to screen all children routinely following MCD if caused by group B or C infection. However, it is important to assess the previous health of the child and to investigate appropriately if there have been previous suspicious infections, abnormal course of infective illnesses, or if this is a repeated episode of neisserial infection.
BACKGROUND AND AIMS: The incidence of complement abnormalities in the UK is not known. It is suggested in at least three major paediatric textbooks to test for abnormalities of the complement system following meningococcal disease (MCD). METHODS: Over a four year period, surviving children with a diagnosis of MCD had complement activity assessed. A total of 297 children, aged 2 months to 16 years were screened. RESULTS: All children except one had disease caused by B or C serogroups. One child, with group B meningococcal septicaemia (complicated by disseminated intravascular coagulation and who required ventilation and inotropic support) was complement deficient. C2 deficiency was subsequently diagnosed. She had other major pointers towards an immunological abnormality prior to her MCD. CONCLUSION: It is unnecessary to screen all children routinely following MCD if caused by group B or C infection. However, it is important to assess the previous health of the child and to investigate appropriately if there have been previous suspicious infections, abnormal course of infective illnesses, or if this is a repeated episode of neisserial infection.
Authors: Rosa Maria Dellepiane; Laura Dell'Era; Paola Pavesi; Paolo Macor; Mara Giordano; Luca De Maso; Maria Cristina Pietrogrande; Massimo Cugno Journal: Orphanet J Rare Dis Date: 2016-05-17 Impact factor: 4.123