Report of a case with Joubert syndrome and literature review / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore the clinical feature, imaging and their diagnostic value for Joubert syndrome (JS).</p><p><b>METHOD</b>The clinical data, imaging feature, and 31 references from China Biomedical literature database (CBMdise) were reviewed and analyzed.</p><p><b>RESULT</b>The age of onset of 32 patients including male 20 and female 12 ranged from 3 days to 6 years (mean 2.2 years). All the 32 patients with Joubert syndrome showed "slow growth" and "reduced muscle tension", 26 cases (81.3%) showed "gasp for breath", 26 cases (81.3%) showed "unusual motion of eyeball", 2 cases (6.3%) showed additional fingers (toes), 6 cases (18.8%) showed stretching tongue with agape. The typical imaging features of Joubert syndrome included "molar tooth sign", "midline cleavage" between cerebellar hemispheres and "bat-wing" like fourth ventricle, all the 32 patients with Joubert syndrome showed "midline cleavage", "molar tooth sign" was present in 29 cases (90.1%), and "bat-wing" like fourth ventricle in 30 cases (93.8%).</p><p><b>CONCLUSION</b>Joubert syndrome is a rare congenital brain malformation. The typical clinical manifestations included "gasp for breath", "reduced tension of muscle", "slow growth" and "unusual motion of eyeball", and at the same time the patients had the following typical imaging features of brain: "molar tooth sign", "midline cleavage" and "bat-wing" like fourth ventricle.</p>

Manifestation of lower extremity atherosclerosis in diabetic patients with high ankle-brachial index / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The ankle brachial index (ABI) is a simple, inexpensive, noninvasive tool that correlates well with angiographic disease severity and functional symptoms. The aim of this study was to identify the manifestation of lower extremity atherosclerotic lesions in patients with high ABI by retrospective clinical study.</p><p><b>METHODS</b>A cohort of 184 diabetic patients, (63 +/- 14) years old, 144 males, who underwent simultaneously ABI testing and low extremity arterial duplex ultrasound within one week, were enrolled randomly into this study. According to the ABI value, they were divided into three groups: the high, normal and low ABI groups. The severity and location of atherosclerotic lesions in the lower extremity were determined based on the results of low extremity artery duplex ultrasound. The chi-square test was used to compare the atherosclerosis severity grade and lesion location across the three groups.</p><p><b>RESULTS</b>The prevalence of low extremity artery occlusion was significantly lower in the high ABI group than in the low ABI group (3.3% vs. 63.5%, P < 0.01), and the main atherosclerotic lesions were diffuse dot-like hyperechogenicity spots or small plaques (86.7%). In addition, the atherosclerotic lesions were mostly found in the distal segment of the lower extremity in patients with high ABI (46.3%).</p><p><b>CONCLUSION</b>A high ABI may be an integrative marker for intimal and medial calcification, which has a high positive predictive value for artery calcification.</p>

Long-term efficacy of calcitonin and bisphosphonates on renal osteopathy in maintenance hemodialysis patients / 中华肾脏病杂志

Objective To observe the efficacy of ealcitonin and bisphosphonates on renal osteopathy of maintenance hemodialysis (MHD)patients. Methods Forty-three MHD patients were raindomly divided into two groups: A group and B group. All the patients were routinely received oral calcium carbonate 1.0 g tid and calcitriol 0.25 μg qd. Calcitonin (20U) hypodermic injection was given three times a week additionally during hemodialysis in A group. Patients in B group received bisphosphonates 70 mg once a week based on the therapy of A group. Serum levels of intact parathyroid hormone (iPTH), calcium, phosphorus, alkaline phosphatase (AKP), bone mass density (BMD) of lumbar spine and femoral neck, and the degree of bone ache (visual analogue scale, VAS) were assessed before the therapy and 3, 6 and 12 months after treatment. The adverse reactions were recorded during treatment. Results The levels of AKP and iPTH in both two groups decreased significantly after treatment. The above values of pre-treatment and 12 months after treatment were as follows: AKP(U/L)of A group 244.05±41.99 and 148.35±27.71,of B group 245.60±40.86 and 143.40±28.03;PTH(ng/L) of A group 697.5±119.7 and 267.4±45.9,of B group 708.2±120.3 and 277.6±41.9 (all P<0.05). While the levels of calcium and phosphorus did not change obviously during treatment (P>0.05). BMD was not improved at 3, 6 mouths and became better at 12 mouths after treatment. As compared to pre-treatment, BMD of lumbar spine(g/cm2) in A group was 1.062±0.223 vs 1.202±0.251 ,in B group 1.033±0.152 vs 1.189±0.225; BMD of femoral neck (g/cm2)in A group was 0.993±0.108 vs 1.067±0.095,in B group 0.947±0.083 vs 1.018 ±0.217 (all P<0.05). The scores of VAS also decreased significantly at 3, 6, 12 months after treatment(P<0.05). No severe adverse reaction was found during the treatment. Conclusions Utilization of calcitonin and combination with bisphosphonates during bemodialysis can effectively preserve the BMD and prevent bone loss in MHD patients and is well tolerated. No significant difference of therapeutic effect is observed between using ealcitonin or combination with bisphosphonates.

Fenestration of the anterior cerebral artery detected by magnetic resonance angiography / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Fenestration of the proximal anterior cerebral artery (ACA) A1 segment is a rare anatomic variation. The purpose of the this study was to report the incidence of fenestration in the proximal segment of the anterior cerebral artery and to delineate its configurations on cranial MR angiography.</p><p><b>METHODS</b>Magnetic resonance angiography (MRA) was performed in 762 patients using 1.5 T imagers during the period July 2007 through September 2008. All images were obtained by the three-dimensional time-of-flight (3D TOF) technique. Volume rendering (VR) images in the horizontal rotation view were displayed stereoscopically. The presence of fenestration in the proximal segment of the anterior cerebral artery was identified and evaluated retrospectively by MRA.</p><p><b>RESULTS</b>Six patients (four men and two women, 15 to 63 years of age, median age 50 years) had proximal ACA fenestration. The appearance rate of ACA fenestration was 0.8% (6/762). All 6 fenestrations were located at the A1 segment: three of them were with a slit-like shape and three were with a convex-lens-like shape, 5 of the right A1 segment, 1 of the left A1 segment.</p><p><b>CONCLUSION</b>Recognizing ACA fenestration is important to interpret cranial MR angiographys and helpful to make a plan for neurosurgical procedures or neurological intervention.</p>

Study on the mechanism of the interaction between montmorillonite and bacterium / 药学学报

<p><b>AIM</b>To investigate the mechanism of the interaction between montmorillonite and bacteria by studying the reactions of different charges of montmorillonites with bacteria.</p><p><b>METHODS</b>Bacteriostatic test: one loop of E. coli and Staphylococcus aureus at the concentration of 1 x 10(6).mL-1 was incubated to the plate culture medium containing different concentrations of montmorillonite, and incubated 24 h to observe the growth of bacteria. Bacterial adsorptive test: different amounts of montmorillonite were added into the artificially simulated intestinal solution (containing bacteria 1 x 10(7).mL-1). After the culture, the bacterial colonies were counted.</p><p><b>RESULTS</b>The results showed that montmorillonite per se showed no bacteriostatic or bactericidal effect, but after exchange with metal ion and functional groups which inhibits bacteria, then it showed these activities. Adsorption was the main way between montmorillonite and bacteria. The special way of fixing bacteria into the "carriage" of montmorillonite gel which carry this structure was its pharmacological basis of curing diarrhea. The adsorption effect was related to layer charge density of the montmorillonites.</p><p><b>CONCLUSION</b>Montmorillonite showed adsorption ability of bacteria with minus related to its layer charge, but has no bacteriostatic and bactericidal effect.</p>