Helen Lee

Acute Kidney Injury During Vancomycin Therapy in Critically Ill Children

To determine the rate, risk factors, and outcome of vancomycin–associated acute kidney injury (AKI) in critically ill children.

Three New α-Thalassemia Point Mutations Ascertained Through Newborn Screening

We report three new α-thalassemia (thal) point mutations detected during newborn screening for hemoglobinopathies. The first mutation is a single nucleotide deletion (−A) that abolishes the translation initiation codon of the α2-globin gene, detected in a newborn of Hmong ethnicity who carried the Southeast Asian α0-thal deletion (αTα/– –SEA). The second mutation, a frameshift caused by a single nucleotide deletion in exon 2 of the α1-globin gene [codon 78 (−C)], was detected in a Black/Chinese newborn who also carried the Southeast Asian α0-thal deletion (ααT/– –SEA). The third mutation was a frameshift in exon 3 of the α2-globin gene, codons 113/114 (−C). This mutation was detected in a newborn who carried the 3.7 kb α+-thal deletion (αTα/–α3.7).

Drug use density in critically ill children and newborns: analysis of various methodologies.

Objective: To compare in the pediatric, cardiac, and neonatal intensive care units, three methods of assessing vancomycin and linezolid drug use density by number of: defined daily doses (DDDs), prescribed daily doses, and days of drug use per 100 patient days. Design: Retrospective study. Setting: A tertiary care children’s hospital. Patients: We reviewed the charts of patients admitted to the cardiac intensive care unit and neonatal intensive care unit in 2005 who were treated with vancomycin, and those admitted to the pediatric intensive care unit who were treated with vancomycin or linezolid during 2004 and 2005. Interventions: None. Measurements and Main Results: The number of patients, treatment days, total amount of vancomycin/linezolid, total intensive care unit admissions, and patient days were recorded. We used the World Health Organization definition of DDD for vancomycin and linezolid (2000 and 1200 mg, respectively). The prescribed daily dose for each intensive care unit was calculated for each year by dividing the total amount of the medication administered by the total number of treatment days. The drug use densities were then calculated as the total DDDs, prescribed daily doses, and days of drug use per 100 patient days. The vancomycin use densities were significantly different among the three intensive care units when compared by each method. They were significantly lower in all three units when expressed as DDDs per 100 patient days. The vancomycin drug use density in the pediatric intensive care unit was significantly decreased during 2005 compared with 2004 by all three methods. Conclusions: In critically ill children, drug use density of vancomycin is significantly less when evaluated by the DDD method compared with the prescribed daily dose method, a more appropriate method in children. However, the simplest and most accurate method of assessing drug use density is the number of days of drug use method, which allows comparison of drug use density between different pediatric facilities or clinical units.

Three New β-Globin Gene Promoter Mutations Identified Through Newborn Screening

We report three new β-globin gene promoter mutations identified in newborns with hemoglobin (Hb) profiles consistent with Hb S/β+-thalassemia (thal) (Hbs FSA). All three mutations are in close proximity to the conserved ATAA sequence located at positions −31 to −28 relative to the mRNA Cap site. Two cases involved single base substitutions at positions −25 (G→C) and −32 (C→T). The remaining case involved the deletion of two bases (−AA) at positions −27 and −26.

4. A challenging case of refractory BehÇet's disease in an adolescent with sight threatening uveitis

patient.Asignificantlyelevatedtroponinhowever,couldnotbeexplained bya tachyarrhythmiaalone.One hypothesis fordevelopinganacutecoronary syndrome, is the vasodilatation role of TNF in the maintenance of myocardial vascular perfusion through the induction of nitric oxide. It is also capable of inhibiting apoptosis of myocardiocytes and attenuation of cardiac stimulation by the sympathetic nervous system through breceptors. The administration of Infliximab, which is a potent anti-TNF antibody can neutralise both soluble and membrane-bound TNF which can suspend these homeostatic mechanisms, resulting in deprivation of first linedefencesandleadingtocoronaryvasoconstrictionandhypoperfusion. Why patients without prior cardiovascular disease develop such symptomsisstillunclearandmaybeafurtherscopeforresearch. KeyLearning Points:Potentanti-TNFagents have the theoretical ability of causing coronary vasoconstriction and hypoperfusion. Albeit rare, patients presenting with chest pain, during or after the infusion should have the appropriate coronary biochemistry and investigations performed.Youngpeoplewithnoriskofcardiovasculardiseasecandevelop transient acute coronary syndrome in response to potent anti-TNF agents. Doubling the frequency of infusion in attempt to regain control of disease after secondary failure should be done with caution and may increasetherisk. Disclosure: K.M. Achilleos: None. P. Bale: None. A. Shastri: None. N. Puvanachandra:None.K.Armon:None.

P45 What does a tertiary paediatric and adolescent service look like today

Anne-Marie McMahon, Daniel Hawley, Ruud Verstegen, Rachel Tattersall, Helen Lee, Clare Nash, Jenny Edgerton, Francesca Peech, Samantha Bull, Elizabeth Deugo, Gisella Cooper, Shirley Armstrong, Kathryn Smith, Nicola Webb, Samantha Leach, Oliver Ward, Catherine Dunbar, Jeanette Hall, Maxine Mutten, Caroline Curran, Shirley Rhodes, Tracy Rew, Barbara Smith and Anne-Marie McMahon Paediatric Rheumatology, Sheffield Children’s Hospitals, Sheffield, UNITED KINGDOM