features include renal involvement, initial lung involvement, cardiac involvement, positive baseline ANCA and persistently elevated ANCA. 4 Relapse rate can be decreased by intensive treatment within the first 6 months of disease, 4 prompting change of therapy in this patient when poor prognostic factors persisted. Case series and open label trials document im- pressive response to rituximab, including two prospective studies with complete response in 9/10 and 10/10 patients, respectively. 5,6 This case demonstrates the existence of cardiac involvement in paediatric WG, associated with a poor prognosis. During the course of this 14-year-old girl’s illness, treatment was intensified in response to the persistence of poor prognostic factors for disease. Despite this, this patient sadly died. Although a post- mortem was not carried out, the temporal existence of myo- carditis associated with clinical deterioration in the absence of infection, suggests that the presence of cardiac involvement was instrumental in this girl’s collapse and death. It is important to consider the possibility of sub-clinical cardiac disease in paediatric WG. Recognition of cardiac involve- ment should prompt aggressive treatment, although optimum management remains unclear. Acknowledgements The authors would like to thank the patient’s family for allow- ing us to publish this article. We would also like to thank Dr Gordan Gladman, Consultant Cardiologist Royal Liverpool Children’s Hospital, for his help with this report. Dr Flora McErlane 1 Dr Liza J McCann 2 1 Specialist Registrar Paediatric Rheumatology 2 Consultant Paediatric Rheumatologist Department of Rheumatology Alder Hey Children’s NHS Foundation Trust Eaton Road, West Derby Liverpool, UK References 1 Frosch M, Foell D. Wegener granulomatosis in childhood and adolescence. Eur. J. Pediatr. 2004; 163: 425–34. 2 Morelli S, Gurgo di Castelmenardo AM, Conti F, Sgreccia A, Alessandri C, Bernardo ML. Cardiac involvement in patients with Wegeners granulomatosis. Rheumatol. Int. 2000; 19: 209–12. 3 Oliveira GH, Seward JB, Tsang TS, Specks U. Echocardiographic findings in patients with Wegener granulomatosis. Mayo Clin. Proc. 2005; 80: 1435–40. 4 Koldingsnes W, Nossent J. Baseline features and initial treatment as predictors of remission and relapse in Wegener’s granulomatosis. J. Rheumatol. 2003; 30: 80–8. 5 Stasi R, Stipa E, Del Poeta G, Amadori S, Newland AC, Provan D. Long term observation of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis treated with rituximab. Rheum 2006; 45: 1432–6. 6 Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U. Rituximab for refractory Wegener’s granulomatosis: report of a prospective open-label pilot trial. Am. J. Respir. Crit. Care Med. 2006; 173: 180–7. 23 December 2008 Dear Editor, STREPTOCOCCUS PNEUMONIAE: UNCOMMON CAUSE OF FATAL NEONATAL SEPSIS Neonatal Streptococcus pneumoniae infection is a recognised but uncommon cause of neonatal sepsis. In a retrospective analysis, the incidence of neonatal pneumococcal sepsis was 0.06/1000 live births and other reports suggest that 1–8% of culture posi- tive neonatal sepsis is caused by S. pneumoniae. 1,2 The clinical course closely resembles early onset group B Streptococcal infection making it difficult to differentiate these two entities clinically. However, the case fatality rate with S. pneumoniae infections has been reported as 14.3–35% and should be con- sidered as a cause of fulminant non-responsive sepsis. 2,3 We report a case of a term infant who deteriorated rapidly and succumbed shortly after birth as a result of severe S. pneumoniae sepsis. A 4586-g male neonate was born by NVD at term following an uneventful pregnancy. The mother was GBS negative with no known gestational diabetes and no identifiable risk factors for sepsis. Prophylactic antibiotics were not administered during labour. The neonate breathed spontaneously at birth but was soon observed to be dusky so supplemental oxygen was com- menced. His Apgar scores were 7/1 and 7/5, respectively. At 6 min of age, he became apnoeic and bradycardic, requiring intubation and aggressive resuscitation. He was admitted to the NICU and commenced on conventional ventilation with high pressures being required. Despite volume expansion and pres- sors, he remained bradycardic with undetectable blood pressure. He failed to respond to all supportive measures and died 80 min after delivery. Because of time restraints, antibiotic therapy was not administered during resuscitation. Investigations revealed Hb: 109 g/L, WCC: 12.6 ¥ 10 9 /L, Plate- lets: 151 ¥ 10 9 /L and CRP: <2 mg/L. His chest X-ray was normal. A single blood culture was sterile. However, S. pneumoniae was isolated from swabs taken from the foetal and maternal sides of the placenta. Post-mortem samples grew S. pneumoniae in the lung tissue and gastric secre- tions. The cultured S. pneumoniae was susceptible to penicillin. S. pneumoniae (Pneumococcus) is a gram positive coccus sur- rounded by a polysaccharide capsule. Pneumococcal infections are a major public health problem and cause significant mor- bidity and mortality worldwide. Perinatal infections with S. pneumoniae are rare but again cause significant morbidity and mortality. 1,2 Pneumococcus is a rare inhabitant of the female genital tract. 3 Some workers recommend pre-emptive treat- ment for pneumococcal sepsis where S. pneumoniae is isolated from the genital tract samples, 4 although standard screening methods for GBS do not favour its isolation. Geelen and col- leagues reported the largest series of seven cases and discussed features of additional cases reported in the literature. 5 They hypothesised that the majority of infants are colonised during delivery and present in the first 48 h of life with a mortality of about 50%. Early onset sepsis had a higher mortality than late onset sepsis. 2 Some reports suggest that early onset pneumo- coccal sepsis maybe an increasing problem especially in devel- oping countries, 3 and this has been attributed to an increase in Letters to the Editor Journal of Paediatrics and Child Health 45 (2009) 684–687 © 2009 The Authors Journal compilation © 2009 Paediatrics and Child Health Division (Royal Australasian College of Physicians) 686