Development of dried blood spot quality control materials for adenosine deaminase severe combined immunodeficiency and LC-MS/MS method for their characterization.

Adenosine deaminase severe combined immunodeficiency (ADA-SCID) is an autosomal recessive disorder in which a lack of ADA enzyme prevents the maturation of T- and B-cells; early intervention is crucial for restoring immune function in affected neonates. ADA is responsible for purine metabolism and-in its absence-adenosine, deoxyadenosine, and S-adenosylhomocysteine build up and can be detected in the blood. Preparing dried blood spot (DBS) quality control (QC) materials for these analytes is challenging because enrichments are quickly metabolized by the endogenous ADA in normal donor blood. Adding an inhibitor, erythro-9-(2-hydroxy-3-nonyl) adenine (EHNA), has been previously reported to minimize enzyme activity, although this adds additional cost and complexity. We describe an alternative method using unnatural L-enantiomer nucleosides (L-adenosine and 2'-deoxy-L-adenosine) which eliminates the need for enzyme inhibition. We also present a novel method for characterization of the materials using liquid chromatography mass spectrometry to quantify the analytes of interest.

Nutrition management after pediatric solid organ transplantation.

Survival rates for pediatric transplant recipients and organ grafts have increased due to improvements in surgical techniques and with immunosuppressant treatment therapies. Interdisciplinary management after pediatric organ transplantation is essential to assist not only with the complex medical issues and complications that can result from immunosuppressant therapy but also with the achievement of normal growth and development. Impaired growth is a complication frequently experienced by pediatric transplant patients. The presence or absence of impaired growth is affected by the length of illness prior to transplant, graft function, the use of corticosteroids, and the development of infectious complications after surgery. A review of posttransplant nutrition assessment, nutrition requirements, and nutrition goals is provided. In addition, a case series of experiences with nutrition management of pediatric solid organ transplant recipients is described.