Post-transplant lymphoproliferative disorder in paediatric patients: the Irish perspective-a single centre experience.

BACKGROUND: Post-transplant lymphoproliferative disease (PTLD) is a serious complication of both solid organ and haematopoietic stem cell transplantation in children. Its incidence has increased over the last decade as a result of more potent immunosuppressive regimens. Many treatments have been explored however optimal therapy remains controversial. AIMS: We report on the diagnosis, treatment and outcome of ten patients who were diagnosed with PTLD in Our Lady's Hospital for Sick Children in Dublin between 2004 and 2015 inclusive. METHODS: Data were collected by retrospective review of patient medical records. RESULTS: 9 out of ten of our patients are alive and disease free following treatment for PTLD with rituximab alone or in combination with chemotherapy. CONCLUSION: The outcome of paediatric patients treated for PTLD at our institution is at least comparable to published international series and supports the use of rituximab ± low dose chemotherapy in the treatment of this malignancy.

Rapid rise in incidence of Irish paediatric inflammatory bowel disease.

AIMS: To describe the change in incidence of paediatric inflammatory bowel disease (IBD) observed at the National Centre for Paediatric Gastroenterology, Hepatology and Nutrition, and to determine whether the presenting disease phenotype and disease outcomes have changed during the past decade. METHODS: The annual incidence of IBD in Irish children aged <16 years was calculated for the years 2000-2010. Two subsets of patients, group A (diagnosed between 1 January 2000 and 31 December 2001), and group B (diagnosed between 1 January and 31 December 2008) were phenotyped according to the Paris Classification. Phenotype at diagnosis and 2-year follow-up were then compared. RESULTS: 406 new cases of IBD were identified. The incidence was 2.5/100 000/year in 2001, 7.3 in 2008 and 5.6 in 2010, representing a significant increase in the number of new cases of Crohn's disease (CD) and ulcerative colitis (UC). There were 238 cases of CD; 129 of UC; and 39 of IBD unclassified. Comparing groups A and B, no differences were found in disease location at diagnosis or, for CD, in its behaviour. CONCLUSIONS: There has been a substantial and sustained increase in the incidence of childhood UC and CD in Ireland over a relatively short period of time. However, disease phenotype at diagnosis has not changed. At 2 years follow-up, CD appears to progress less frequently than in some neighbouring countries. These variations remain unexplained. Prospective longitudinal studies will help to elucidate further the epidemiology of childhood IBD.

Hepatitis B virus infection in children.

Recent increases in Hepatitis B virus (HBV) infection prompted us to characterize HBV-infected children in Ireland and to audit management, by reviewing prospectively gathered data. Of 46 children (29 [63%] male), median age at presentation was 8.1 years (range 0.6-17.6), monitoring duration was 22.5 months (range 1-101), 23/46 (50%) were European (including 9 [19.6%] Irish), 15 (32.6%) African and 9 (19.6%) Asian. Acquisition was vertical (25/46 [54.3%]), horizontal (5/46 [10.9%]), unknown (16/46 [34.8%]). HBV-DNA was >100,000,000 cpm in 20/32 (62.5%) with chronic infection. Hepatitis B e antigen (HBeAg) was detected in 32/44 (72.7%). We estimate that universal neonatal vaccination (UNV-HBV) could have prevented 22% of cases, and could limit further horizontal HBV spread. This supports the recent introduction of UNV-HBV.