Normal Anatomical Features and Variations of the Vertebrobasilar Circulation and Its Branches: An Analysis with 64-Detector Row CT and 3T MR Angiographies

Purpose. To determine the normal anatomical features and variations of the vertebrobasilar circulation and its branches in patients who underwent multidetector computed tomography (CT) or magnetic resonance (MR) angiographies of the brain. Methods. 135 patients (male, 83 and female, 52; mean age, 50.1 years) who underwent CT or MR angiographies of the vertebrobasilar vasculature for various reasons were analyzed retrospectively. The right and left distal vertebral arteries (VAs), posterior inferior cerebellar arteries (PICAs), anterior inferior cerebellar arteries (AICAs), superior cerebellar arteries (SCAs), posterior cerebral arteries (PCAs), and posterior communicating arteries (PCoAs) were analyzed individually. Results. In 24.4% of the cases (33/135) right PICA, in 19.3% of the cases (26/135) left PICA, in 17.8% of the cases (24/135) right AICA, and in 18.5% of the cases (25/135) left AICA were absent. In cases without PICA or AICA, there was a statistically significant, moderately or well-developed AICA or PICA on the same side, respectively . The most common variation was isolated absence of right PICA and was seen in 17.8% of the cases. Conclusions. The anatomic features of the branches of the vertebrobasilar circulation may be different from well-known normal anatomy. CT and MR angiographies allow a precise and detailed evaluation of vertebrobasilar circulation

Anticancer Effect of a Novel Palladium-Saccharinate Complex of Terpyridine by Inducing Apoptosis on Ehrlich Ascites Carcinoma (EAC) in Balb-C Mice

Background/Aim: [Pd(sac)(terpy)](sac)center dot 4H(2)O (sac=saccharinate and terpy=2,2':6',2 ''-terpyridine) is newly-synthesized palladium(II) (Pd) complex. We investigated the antiproliferative and apoptotic effects of this complex on Ehrlich ascites carcinoma (EAC). Materials and Methods: EAC cells were administered to 33 Balb/c mice. Mice were divided randomly into four groups: control, cisplatin. Pd(II) complex and paclitaxel. Control group animals received 0.9% NaCl; other groups received treatments cisplatin, Pd(II) complex and paclitaxel on days 7 and 12. At day 14, animals were sacrificed. Expression of active caspase-3, p53 and proliferating cell nuclear antigen (PCNA) was investigated and apoptosis was evaluated by terminal deoxynucleotidyltransferase (TdT)-mediated nick-end labelling (TUNEL) technique. Results: Expression of p53 and PCNA were found to be decreased (p<0.0001), cells with active caspase-3 and TUNEL-positive cells were found to be increased (p<0.0001) in all treatment groups. Conclusion: Like cisplatin and paclitaxel, this Pd(II) complex has a strong anticancer activity against EAC by inducing apoptosis and suppressing proliferation in vivo

Evolution and long-term outcomes of combined immunodeficiency due to CARMIL2 deficiency

Background Biallelic loss-of-function mutations in CARMIL2 cause combined immunodeficiency associated with dermatitis, inflammatory bowel disease (IBD), and EBV-related smooth muscle tumors. Clinical and immunological characterizations of the disease with long-term follow-up and treatment options have not been previously reported in large cohorts. We sought to determine the clinical and immunological features of CARMIL2 deficiency and long-term efficacy of treatment in controlling different disease manifestations. Methods The presenting phenotypes, long-term outcomes, and treatment responses were evaluated prospectively in 15 CARMIL2-deficient patients, including 13 novel cases. Lymphocyte subpopulations, protein expression, regulatory T (Treg), and circulating T follicular helper (cT(FH)) cells were analyzed. Three-dimensional (3D) migration assay was performed to determine T-cell shape. Results Mean age at disease onset was 38 +/- 23 months. Main clinical features were skin manifestations (n = 14, 93%), failure to thrive (n = 10, 67%), recurrent infections (n = 10, 67%), allergic symptoms (n = 8, 53%), chronic diarrhea (n = 4, 27%), and EBV-related leiomyoma (n = 2, 13%). Skin manifestations ranged from atopic and seborrheic dermatitis to psoriasiform rash. Patients had reduced proportions of memory CD4(+) T cells, Treg, and cT(FH) cells. Memory B and NK cells were also decreased. CARMIL2-deficient T cells exhibited reduced T-cell proliferation and cytokine production following CD28 co-stimulation and normal morphology when migrating in a high-density 3D collagen gel matrix. IBD was the most severe clinical manifestation, leading to growth retardation, requiring multiple interventional treatments. All patients were alive with a median follow-up of 10.8 years (range: 3-17 years). Conclusion This cohort provides clinical and immunological features and long-term follow-up of different manifestations of CARMIL2 deficiency

Evolution and long-term outcomes of combined immunodeficiency due to CARMIL2 deficiency

Background Biallelic loss-of-function mutations in CARMIL2 cause combined immunodeficiency associated with dermatitis, inflammatory bowel disease (IBD), and EBV-related smooth muscle tumors. Clinical and immunological characterizations of the disease with long-term follow-up and treatment options have not been previously reported in large cohorts. We sought to determine the clinical and immunological features of CARMIL2 deficiency and long-term efficacy of treatment in controlling different disease manifestations. Methods The presenting phenotypes, long-term outcomes, and treatment responses were evaluated prospectively in 15 CARMIL2-deficient patients, including 13 novel cases. Lymphocyte subpopulations, protein expression, regulatory T (Treg), and circulating T follicular helper (cT(FH)) cells were analyzed. Three-dimensional (3D) migration assay was performed to determine T-cell shape. Results Mean age at disease onset was 38 +/- 23 months. Main clinical features were skin manifestations (n = 14, 93%), failure to thrive (n = 10, 67%), recurrent infections (n = 10, 67%), allergic symptoms (n = 8, 53%), chronic diarrhea (n = 4, 27%), and EBV-related leiomyoma (n = 2, 13%). Skin manifestations ranged from atopic and seborrheic dermatitis to psoriasiform rash. Patients had reduced proportions of memory CD4(+) T cells, Treg, and cT(FH) cells. Memory B and NK cells were also decreased. CARMIL2-deficient T cells exhibited reduced T-cell proliferation and cytokine production following CD28 co-stimulation and normal morphology when migrating in a high-density 3D collagen gel matrix. IBD was the most severe clinical manifestation, leading to growth retardation, requiring multiple interventional treatments. All patients were alive with a median follow-up of 10.8 years (range: 3-17 years). Conclusion This cohort provides clinical and immunological features and long-term follow-up of different manifestations of CARMIL2 deficiency