Paediatric autoimmune neutropaenia.

Neutropaenia is defined as an absolute neutrophil count (ANC) of less than 1500 cells/µL and is often divided into mild (ANC: 1000 to 1500 cells/µL), moderate (ANC: 500 to 1000 cells/µL) and severe (ANC: >500 cells/µL) neutropaenia. Autoimmune neutropaenia is arguably one of the most clinically relevant forms of neutropaenia and is defined by an ANC of <500 cells/µL alongside the confirmed presence of anti-neutrophil antibodies. It is a rare subset of neutropaenia boasting an incidence of just 1 in 100 000 infants and is thought to be caused as a result of a 'surveillance escape event' of the immune system. This case report identifies key issues in the diagnosis and monitoring of paediatric patients with recurrent neutropaenia and reviews current literature relating to its management in hospital and community settings.

Outcome of patent ductus arteriosus ligation in premature infants in the East of England: a prospective cohort study.

BACKGROUND: Surgical ligation of patent ductus arteriosus is considered when medical treatment fails or is contraindicated. This study aims to determine the mortality and morbidity of preterm neonates referred for patent ductus arteriosus ligation. METHODS: A prospective study was conducted in the East of England to follow the outcome of premature infants under 37 weeks' gestation undergoing patent ductus arteriosus ligation. A standardised proforma was used to collect information before and after the procedure. RESULTS: A total of 102 premature infants were recruited, and patent ductus arteriosus ligation was performed in 92. Surgical complications occurred in 8.7% (8/92), which included pneumothorax (5/8), recurrent laryngeal nerve palsy (2/8), and chylothorax (1/8). Morbidity outcome data were not available for all infants. The incidence of chronic lung disease was 88% (88/99); intraventricular haemorrhage was 49% (49/100); necrotising enterocolitis 39% (39/99), and retinopathy of prematurity 42% (41/97). The overall mortality rate in our study was 7.8% (8/102). Mortality rate in infants who had patent ductus arteriosus ligation was 4.3% (4/92). The 30-day survival rate after ligation was 99% (91/92). Beyond 30 days post-ligation, three infants died from other causes that were not directly related to surgery. CONCLUSION: Patent ductus arteriosus ligation in premature infants is associated with low mortality and complication rates; however, there is a high incidence of neonatal morbidity. Surgical capacity for patent ductus arteriosus ligation needs to be carefully planned nationally as the duration of ''waiting time'' and transport to another surgical centre could adversely affect outcomes in this high-risk population.