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John J Zone

Publications and source records attributed to John J Zone.

21 records · Page 2Linked to original sources
PubMed2002

HLA DQA1-DQB1 genotypes in Bedouin families with celiac disease.

Celiac disease (CD) has a strong genetic association with human leukocyte antigens (HLA). The primary susceptibility for CD is HLA-DQA1*05 DQB1*02 (also known as DQ2), with the remainder of cases primarily HLA-DQA1*03 DQB1*03 (also known as DQ8). In a set of nine Bedouin multiplex celiac disease families and one simplex, we genotyped DNA samples at HLA DQA1 and DQB1. Nineteen celiac disease patients had at least one DQA1*05 DQB1*02 genotype (= DQ2), 4 affecteds had the second most common genotype of DQA1*03 DQB1*0302 (= DQ8), 9 were DQ2 and DQ8, and 4 had at least one copy of DQB1*02 without the DQA1*05 genotype. Using transmission disequilibrium testing, we observed a significant over-representation in affecteds of the DQA1*05 DQB1*02 genotype (p = 0.0089), as well as over-representation of the DQA1*03 DQB1*0302 genotype (p = 0.078). The HLA DQA1 DQB1 high-risk genotypes associated with celiac disease are similar in these Bedouin families with CD to what is observed in Northern and Southern Europeans.

Alleles↗
PubMed2002

Treatment of toxic epidermal necrolysis with intravenous immunoglobulin in children.

BACKGROUND: Toxic epidermal necrolysis (TEN) is an acute illness characterized by rapid onset of skin necrosis and high mortality. Standard treatment is primarily aimed at supportive care in a burn unit setting. OBJECTIVE: We evaluated the outcome of 8 pediatric patients treated for TEN with intravenous immunoglobulin (IVIg) over a 3-year period. METHODS: We performed a retrospective analysis of pediatric patients with a diagnosis of TEN between 1999 and 2001, obtained from a computerized database. RESULTS: Mean body surface involvement of 8 patients treated with IVIg was 67%. The average length of hospitalization was 13.6 days, with an average delay in treatment of 3.2 days. The average time to arrest in progression of lesions was 2.1 days and to complete re-epithelialization, 8.1 days. The mortality rate was 0%. The majority of complications were infectious. CONCLUSION: IVIg is a safe and effective treatment for TEN in the pediatric population. Randomized trials are needed to further evaluate the efficacy of IVIg compared with other modalities.

Adolescent↗