AIM: To investigate the preventive effect of PNS on chronic hypoxic pulmonary hypertension in rats. METHODS: Pulmonary arterial pressure observation, hematocrit (Hct)measurement, biochemical analysis and transmission electron microscopy were conducted to investigate the role of PNS. RESULTS: (1)Mean pulmonary arterial pressure(mPAP), right ventricular mean pressure(RVMP) and Hct were significantly higher in hypoxia group (H group) than that of control group (C group) and were much lower in hypoxia with PNS group (HT group) than that in H group;(2)Nitric oxide (NO 2-/NO 3-) concentration and nitric oxide synthase(NOS) activity in the plasma and the lung tissue, total superoxide dismutase(T-SOD) and copper/zinc-containing enzyme (Cu/ZnSOD) activities in the plasma were all significantly lower in H group than that in C group and were much higher in HT group than that in H group, but NO 2-/NO 3- concentration and NOS activity were still markedly decreased in comparison with C group;(3)Injury of endothelial cells in pulmonary arteriole was improved obviously in HT group compared with H group. CONCLUSIONS: These results suggest that PNS reduces the increase in mPAP, probably through adjusting NO level, anti-damaging effect of free radicals, inhibiting the injury of endothelial cells and decreasing Hct.

Objective To evaluate the clinical manifestations, metal metabolism, imaging characteristics and treatment response in patients with delayed Wilson disease (WD). Methods Patients with untreated WD (40 with delayed onset and 40 with non?delayed onset) were enrolled. Twenty healthy people were included as normal controls. All patients were evaluated with modified Young scale neural symptom scores, grade of Child liver function and mental symptoms rating scale, magnetic resonance imaging (MRI) scan, magnetic sensitive imaging (susceptibility weighted imaging, SWI), metal metabolism. Corrected phase (CP) was measured at SWI. After 2 week treatment, neurologic symptoms, liver function, and metal metabolism were reviewed. Results The total score of neurological symptoms in WD patients with delayed onset was lower than that of non?delayed onset (13.00 ± 6.87 vs. 21.13 ± 5.53, P=0.033). The scores of SCL?90 and HAMA depression scales in patients with delayed onset were lower than those of non?delayed onset. On T2 weighted imaging, areas including substantia nigra and thalamus, the caudate nucleus, globus pallidus, putamen presented high signal rate in patients with delated onset than those with non?delayed (P=0.022, 0.037, 0.022, 0.037, 0.029 respectively). The SWI CP values of cangbai sphere and shell nucleus in patients with delayed onset were lower than those with non?delayed onset. Patients with delayed onset had higher urinary copper than those with non?delayed onset before and after treatment (P=0.040, 0.036). After treatment, the score of abnormal tremor and gait in patients with delayed onset was decreased (P=0.037, 0.044), while as the occurrence of neurological symptoms was increased by 10%, and the liver function level in patients with delayed WD was decreased in 3 cases. Conclusions The brain of WD patients with delayed onset is mainly composed of metal deposits, however the cell damage is not apparent. Clinical symptoms are characterized by significant liver injury, but relatively mild neurological and psychiatric symptoms. Patients with delayed WD have higher urinary copper excretion than those with non?delayed WD. Chelating agents improves the neurological symptoms in patients with delayed onset.