Non-compaction cardiomyopathy in a 5-generation Chinese family / 中华心血管病杂志

<p><b>OBJECTIVE</b>Familial left ventricular noncompaction(LVNC) is quite rare. We screened for the presence of LVNC and related clinical characteristics in a 5-generation Chinese family.</p><p><b>METHODS</b>Comprehensive medical history was obtained from 40 members in a 5-generation Chinese family. Systemic clinical investigations including echocardiography (UCG), routine and ambulatory electrocardiogram (ECG), X-rays were performed in 33 family members. Cardiovascular magnetic resonance image (MRI) was carried out in 2 family members.</p><p><b>RESULTS</b>Sudden cardiac death (including 1 occurred while following-up) was reported in 7 family members (17.5%, 7/40). LVNC was diagnosed in 10 out of the 33 family members (30.3%) and heart enlargement was evidenced in 3, heart failure in 2, complete left branch conductive block in 3, serious sick sinus syndrome (SSS) treated with permanent pacemaker implantation in 1 and paroxysmal supraventricular tachycardia treated with radiofrequency ablation procedure in 1 out of these 10 LVNC patients. Primary pedigree analysis revealed that offspring from female patients were at the highest risk to be affected by LVNC (15/18, 83.3%) while LVNC was absent in offspring of male LVNC patients (0/8). Moreover, clinical heart failure symptoms and arrhythmias were more severe in female LVNC patients than in male LVNC patients.</p><p><b>CONCLUSION</b>Primary familial investigation reveals the matrilineal inheritance of familial LVNC in this 5-generation Chinese family, further investigations are warranted to explore the potential mutations in the mitochondrial genome responsible for LVNC in this family.</p>

Classification and functional study of peripheral blood dendritic cells in patients with coronary artery disease with different atherosclerotic plaques / 南方医科大学学报

<p><b>OBJECTIVE</b>To study the quantitative and functional changes of peripheral blood dendritic cells (DCs) and their subsets in the leukocyte population in patients with coronary artery disease (CHD) with different coronary artery plaques and explore the relation between DCs and coronary plaque development.</p><p><b>METHODS</b>Thirty CHD patients were divided into SAP (10 cases), UAP (10 cases) and ACS (10 cases) groups, with another 10 patients having negative result in coronary angiography as the control group. Intravascular ultrasound (IVUS) was performed to identify the nature of the plaques. The percentage and absolute number of peripheral blood DCs and DC subsets were measured by flow cytometry. The functional status of the DCs was analyzed by enzyme-linked immunosorbent assay (ELISA) and flow cytometry.</p><p><b>RESULTS</b>In the SAP group, IVUS found stable plaques in 8 cases and unstable plaques in 2 cases; in UAP group, 7 patients had unstable plaques, 2 had stable plaques, and 1 had plaque rupture. Plaque rupture, unstable plaques and stable plaques were found in 6, 3 and 1 patients in ACS group, respectively. In comparison with patients with stable plaques, those with unstable plaques had significantly increased percentages and number of DCs, mDCs and mDC1 (P<0.05), while the mDC2s and pDCs showed no obvious difference between them (P>0.05). The percentages and number of DCs, mDCs, mDC1s and pDCs were significantly decreased in patients with ruptured plaques (P<0.05). In peripheral blood monouclear cells cultured for 7 days, the CD83 expression was significantly higher in unstable and rupture plaque groups than in stable plaque group, and no significant difference was found between stable plaque group and the control group (P>0.05). In unstable and rupture plaque groups, co-culture with 2x10(5)/ml DCs evoked strong proliferation of the T cells in comparison with the stable plaque group, but no difference was found between the stable plaque and the control groups (P>0.05). Significantly higher levels of interleukin-2 and interferon-alpha were detected in the supernatant of the mixed lymphocyte reaction in unstable and ruptured plaque groups than in stable plaque and control groups, without obvious difference between the latter two groups.</p><p><b>CONCLUSION</b>The percentage and absolute number of peripheral blood DCs and their functional status suggest the alterations of the coronary artery plaques in CHD patients.</p>

The effect of telomerase reverse transcriptase on 5-HT induced pulmonary artery smooth muscle cells proliferation / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the effect of telomerase reverse transcriptase (TERT) to the proliferation of 5-HT induced pulmonary artery smooth muscle cells (PASMCs).</p><p><b>METHODS</b>The PASMCs proliferation experiment was performed to detect the effort on PASMCs of 5-HT or ASODN TERT (antisense oligoribonucleotides TERT designed according to the rat TERT mRNA sequence of gene bank). The immunohistochemistry staining experiment and the in situ hybridization experiment were to detect the TERT protein and mRNA expression with 5-HT or ASODN TERT. FITC marked ASODN TERT experiment was done to research the distribution of ASODN TERT in PASMCs.</p><p><b>RESULTS</b>5-HT promoted PASMCs proliferation in a dose-dependent manner (10(-9) - 10(-5) mol/L). 5-HT also significantly increased TERT expression at protein and mRNA levels as shown by immunohistochemistry staining and the in situ hybridization studies. This effect could be blocked by ASODN TERT in a time and dose-dependent manner.</p><p><b>CONCLUSIONS</b>Our experiments show TERT is one of the key factors in the procession of 5-HT induced PASMCs proliferation. ASODN TERT might be a potential therapy agent for pulmonary hypertension.</p>

Melatonin decreases expression of transforming growth factor-?_1 in renal cortex of diabetic rats / 中华内分泌代谢杂志

Transforming growth factor-?_1 (TGF-?_) mRNA or protein expression in renal cortex of diabetic rats was assessed by real-time quantitative RT-PCR with SYBR Green,immunohistochemistry or Western blot.After melatonin treatment,the expressions of TGF-?_1 mRNA and protein were decreased,suggesting that beneficial effect of melatonin may result from its antioxidative property and inhibiting TGF-?_1 expressions.