2 months. There were no operative deaths. Four patients were converted intraoperatively to an open decompression,

all for intraoperative bleeding; only one required a blood transfusion. Average operating time was 189 minutes, and the average length of stay was 3.5 days. CA intervention was required in six patients, including three intraoperative procedures (1 patch angioplasty, 1 celiac bypass, I percutaneous angioplasty) and six late procedures (2 percutaneous angioplasties, 3 percutaneous stents, I celiac bypass). One complication occurred, a severe case of pancreatitis that developed I week after discharge. On follow-up, 14 of 15 patients subjectively reported significant improvement, and one patient

remains symptomatic with no diagnosis.

Conclusion: Laparoscopic decompression of the CA may be a useful therapy for CACS, but there is potential for vascular injury, and adjunctive selleckchem CA intervention is often required. Surgeons should consider laparoscopic CA decompression as a therapeutic alternative for CACS and should participate in the rare of patients with this diagnosis. (J Vasc Surg 2009; 50:124-33.)”
“BACKGROUND

Chronic mucocutaneous candidiasis may be manifested Y-27632 mouse as a primary immunodeficiency characterized by persistent or recurrent infections of the mucosa or the skin with candida species. Most cases are sporadic, but both autosomal dominant inheritance and autosomal recessive inheritance have been described.

METHODS

We performed genetic studies in 36 members of a large, consanguineous five-generation family, in which 4 members had recurrent fungal infections and an additional

3 members died during adolescence, 2 after invasive infection of the brain with candida species. All 36 family members were enrolled in the study, and 22 had blood samples taken for DNA analysis. Homozygosity mapping was used to locate the mutated gene. In the 4 affected family members (patients) and the 18 unaffected members we sequenced CARD9, the gene encoding the caspase recruitment domain-containing protein 9, carried out T-cell phenotyping, and performed functional studies, with the use of either leukocytes from the patients or a reconstituted murine model of the genetic BV-6 in vivo defect.

RESULTS

We found linkage (lod score, 3.6) to a genomic interval on chromosome 9q, including CARD9. All four patients had a homozygous point mutation in CARD9, resulting in a premature termination codon (Q295X). Healthy family members had wild-type expression of the CARD9 protein; the four patients lacked wild-type expression, which was associated with low numbers of Th17 cells (helper T cells producing interleukin- 17). Functional studies based on genetic reconstitution of myeloid cells from Card9(-/-) mice showed that the Q295X mutation impairs innate signaling from the antifungal pattern-recognition receptor dectin-1.