Biomedical subjects
Lenora M Noroski
Publications and source records attributed to Lenora M Noroski.
Burkholderia gladioli osteomyelitis in association with chronic granulomatous disease: case report and review.
We describe a case of insidious small bone osteomyelitis and soft tissue abscess with Burkholderia gladioli in a 6-year-old Caucasian boy with chronic granulomatous disease. DNA sequencing of the 16S ribosomal RNA gene confirmed the bacterial identification. Clinical cure was achieved with a combination of antimicrobial therapy and surgical debridement. A review of infections caused by Burkholderia spp., other than Burkholderia cepacia complex, in pediatric patients with chronic granulomatous disease is provided.
Live viral vaccines in patients with partial DiGeorge syndrome: clinical experience and cellular immunity.
Partial DiGeorge syndrome (pDGS) is an inherited primary immunodeficiency syndrome (incidence, 1:3000 live births) primarily affecting cellular immune function; partial, infers thymic hypoplasia with detectable circulating T-lymphocytes and adequate function. No guidelines exist regarding the recommendations for use of live viral vaccines (LVVs) in this extensive population of pediatric patients. We reviewed the experience with live viral vaccines in our cohort of patients with pDGS. Of 53 patients, 25 (47%) had received a live viral vaccine. No significant adverse events were recorded in association with administration of live viral vaccines. There was no statistically significant difference between cellular immune function at initial presentation between those patients that received live viral vaccines and those that did not. Adequate cellular immune function was documented for 15 of the 25 LVV recipients at the time of vaccine administration without significant change from baseline. These observations suggest that live viral vaccines appear safe in patients with pDGS and stable immune function.
Long-term assessment of T-cell populations in DiGeorge syndrome.
BACKGROUND: Patients with DiGeorge syndrome present with a broad range of T-cell deficiency. Partial DiGeorge syndrome (pDGS) is a preferred designation for patients with detectable T-cell function. Among immunology experts, there is no uniform opinion on the necessity of T-cell precautions for pDGS patients. Few studies have addressed the natural course of their immune function over time. OBJECTIVE: The objective of this study was to describe the natural history of immune parameters in pDGS. METHODS: We reviewed the medical records of 45 pDGS patients. Peripheral blood T-cell subsets counts and percentages were recorded at 1, 6, 12, 18, 24, 30, 48, 60, 72, 96, and 120 months of age, and the rates of change of T-cell measurements over the follow-up period (slopes) were calculated for each individual. Humoral immunity was evaluated by quantification of immunoglobulins and by testing antibody titers to recall antigens. RESULTS: T-cell subsets counts from pDGS patients were generally lower than those of age-matched normal populations but were not severely depressed (ie, CD4+ T-cell percentage less than 15%). The median of the slopes for CD3+, CD4+, and CD8+ T-cell percentages were -0.7%, -0.8%, and -0.1%/month, respectively, in the first year of age and 0.1%/month for each subpopulation from 12 to 120 months of age. Lymphoproliferative responses to phytohemagglutinin were adequate at all ages. Immunoglobulin deficiencies or inadequate production of specific antibodies were not detected. CONCLUSIONS: In our pDGS patient cohort, a significant deterioration of T-cell number or function did not occur over time. Clinical implications of this finding include the possibility of discontinuing T-cell deficiency precautions and frequency of reevaluations of pDGS patients with stable and adequate immune function.