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OBJECTIVE@#To investigate the possible causes of abnormal hemoglobin electrophoresis results.@*METHODS@#The hemoglobin electrophoresis results of 5 696 patients in the First Affiliated Hospital of Chengdu Medical College from September 2018 to July 2021 were collected, and the abnormal results and clinical significance were analyzed.@*RESULTS@#The results of 486 patients (accounting for 8.53%) were abnormal, of which 300 cases had increased HbA2, 135 cases had decreased HbA2, 44 cases had increased F alone, and 7 cases had abnormal hemoglobin bands. Among the 486 patients, 246 patients were thalassemia gene positive (the positive rate was 50.62%), including 29 cases of α thalassemia, 208 cases of β thalassemia and 9 cases of αβ thalassemia. Among the patients with elevated HbA2, 68.67% were detected β thalassemia, 3.00% αβ thalassemia, 9.33% were suspected to be caused by macrocytosis, 6.33% by thyroid dysfunction, and 12.67% by uncertainty of the method. Among the patients with reduced HbA2, 21.48% were detected α thalassemia, 60.00% iron deficiency anemia, 8.15% were suspected to be caused by thyroid dysfunction, and 10.37% by uncertainty of the method. Among the patients with elevated F alone, the results of thalassemia gene detection were negative, 40.91% of them were suspected to be caused by macrocytosis, 27.27% by hereditary persistence of fetal hemoglobin, 29.55% by special physiological condition of pregnant women, and 2.27% by hyperthyroidism. Abnormal hemoglobin bands were detected in 7 patients, including 4 cases of hemoglobin D, 2 cases of hemoglobin E, and 1 case of hemoglobin J.@*CONCLUSION@#Thalassemia, iron deficiency anemia, macrocytosis such as megaloblastic anemia and non-severe aplastic anemia, thyroid dysfunction, hereditary persistence of fetal hemoglobin, abnormal hemoglobin diseases, the uncertainty of the method are all important causes of abnormal hemoglobin electrophoresis results. In clinical work, the patient's indicators should be comprehensively analyzed to determine the possible cause.
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Humains , Femelle , Grossesse , bêta-Thalassémie/génétique , Anémie par carence en fer , Hémoglobine foetale/analyse , alpha-Thalassémie , Électrophorèse des protéines sanguines , Hémoglobine A2/analyse , Hémoglobines anormales/analyseRésumé
ObjectiveTo observe the effect of polysaccharides from root, stem, leaf and fruit of Schisandra chinensis on exercise endurance in the aging mice induced by D-galactose. MethodMale ICR mice were randomly assigned into six groups: blank control group, model group, root polysaccharide group, stem polysaccharide group, leaf polysaccharide group and fruit polysaccharide group. The mice were administrated with distilled water or root, stem, leaf and fruit polysaccharide (total sugar content of 35 mg·kg-1) by gavage. Thirty minutes after the administration, the blank control group was subcutaneously injected with normal saline, and the other groups with D-galactose (300 mg·kg-1), once daily for 6 weeks. The anti-fatigue effects were evaluated by rotarod test, forelimb grip strength test, and weight-loaded swimming test. The fatigue and oxidation indicators such as blood urea nitrogen (BUN), serum lactic acid (LD), lactic dehydrogenase (LDH), creatine kinase (CK), superoxide dismutase (SOD), malondialdehyde (MDA), glutathione peroxidase (GSH-Px), and reactive oxygen species (ROS) were measured by chemical colorimetry. The protein levels of pro-apoptotic protein B cell lymphoma-2 (Bcl-2)-associated X protein (Bax), anti-apoptotic Bcl-2 and cleaved cysteinyl aspartate-specific protease-3 (cleaved Caspase-3) in mouse skeletal muscle were detected by Western blot. ResultIn the rotarod test, the time on rod was shorter in the model group than in the blank control group (P<0.01) and the root, stem and fruit polysaccharide groups (P<0.01). In the forelimb grip strength test, the forelimb grip strength in the model group was lower than that in the blank control group (P<0.01) and the root, stem, leaf and fruit polysaccharide groups (P<0.01). In the weight-loaded swimming test, the weight-loaded swimming time in the model group was shorter than that in the blank control group (P<0.01) and the root, stem, leaf and fruit polysaccharide groups (P<0.01). Compared with those in the blank control group, the BUN, LD, LDH and CK levels significantly increased in the model group (P<0.05, P<0.01). The increases in BUN and LDH levels were decreased by the root, stem and fruit polysaccharides (P<0.05, P<0.01) and those in LD and CK by the root, stem, leaf and fruit polysaccharides (P<0.05, P<0.01). Compared with the blank control group, the model group showed decreased SOD and GSH-Px activities (P<0.01) and increased MDA and ROS content (P<0.01). Compared with the model group, the root, stem, and fruit polysaccharide increased the SOD activity (P<0.05, P<0.01) and decreased ROS content (P<0.01). The root and stem polysaccharides decreased the MDA content (P<0.01) and increased the GSH-Px activity (P<0.05, P<0.01). Compared with the blank control group, the model group showed up-regulated protein levels of Bax and cleaved Caspase-3 and down-regulated protein level of Bcl-2 (P<0.01). Compared with the model group, the root, and stem polysaccharides down-regulated the protein levels of cleaved Caspase-3 (P<0.05) and up-regulated protein level of Bcl-2 (P<0.01). ConclusionThe polysaccharides from the root, stem, leaf, and fruit of S. chinensis have anti-fatigue effect in D-galactose-induced aging mice. The polysaccharides may exert such effect by improving the antioxidant capacity and inhibiting the apoptosis of skeletal muscle cells.
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OBJECTIVE@#To investigate the morphological, histological and ultrastructural changes of acute closed rupture of Achilles tendon, in order to clarify the pathological basis of the injury and to explore the significance.@*METHODS@#From January 2015 to January 2019, 35 patients with acute Achilles tendon rupture who underwent the minimally invasive Achilles tendon suture technique were retrospectively analyzed. Among these patients, 12 cases in acute open Achilles tendon rupture group included 10 males and 2 females, with an average age of (35.1±9.7) years old ranging from 19 to 50, and the time from injury to operation was 2 to 8 hours with an average of(5.6±1.8);23 cases in acute closed Achilles tendon rupture group included 21 males and 2 females, with an average age of (35.5±6.6) years old ranging from 18 to 50, and the time from injury to operation was 3 to 15 hours with an average of (7.5±3.1). The gross appearance and imaging findings of the broken end of Achilles tendon tissue in the two groups were compared by naked eye observation and foot and ankle MRI at 4 to 6 hours before operation. HE staining, scanning and fluoroscopic electron microscopy, immunohistochemistry(Sirius red staining) were performed on the intraoperative Achilles tendon tissue specimens at 1 to 2 days after operation, the collagen fiber degeneration and local fat infiltration, collagen fiber shape, cell morphology and function, and the distribution of typeⅠand type Ⅲ collagen fibers in Achilles tendon were compared between the two groups.@*RESULTS@#Compared with the acute open Achilles tendon rupture group, the acute closed Achilles tendon rupture group had poor elasticity, hard texture, moderate edema, irregular shape of Achilles tendon broken end, horsetail shape, and more calcification around the broken end. HE staining results:the collagen fibers in the Achilles tendon of the acute open Achilles tendon rupture group were arranged irregularly, with hyaline degeneration and fat infiltration;The results of electron microscopy showed that collagen arranged disorderly and fibroblasts atrophied in the acute closed Achilles tendon rupture group. Immunohistochemical(Sirius staining) results:the proportion of collagenⅠin the acute open Achilles tendon rupture group and the acute closed Achilles tendon rupture group was(91.12±4.34)% and(54.71±17.78)% respectively, and the proportion of collagen Ⅲ was (8.88±4.34)% and (45.29±17.78)% respectively. The content of collagenⅠin the acute closed Achilles tendon rupture group was lower than that in the acute open Achilles tendon rupture group, and the content of collagen Ⅲ in the acute closed Achilles tendon rupture group was higher than that in the acute open Achilles tendon rupture group(P<0.05).@*CONCLUSION@#The morphology, histology and ultrastructure of the acute closed ruptured Achilles tendon are significantly altered compared with the normal Achilles tendon. The original fine and orderly spatial structure cannot be maintained, part of collagen Ⅰ is replaced by collagen Ⅲ, and the toughness and strength of the tendon tissue decreased, which may be the feature of degeneration of the Achilles tendon and an important pathological basis for closed Achilles tendon rupture.
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Adulte , Femelle , Humains , Mâle , Tendon calcanéen/chirurgie , Études rétrospectives , Rupture/chirurgie , Techniques de suture , Traumatismes des tendons/chirurgie , Résultat thérapeutiqueRésumé
Excessive exercise makes the body consume more oxygen and produce excessive free radicals. The increased free radicals lead to oxidative stress injury and dysfunctions in liver tissue. Our previous study showed that Anwulignan, an active monomer in Schisandra sphenanthera Rehd. et Wils. (Schisandra), had anti-fatigue effects in mice. However, whether Anwulignan has a protective effect on liver damage in exhausted mice and the mechanism underlying remain elusive. An exhaustive swimming mice model was used to study the protective effects of Anwulignan on liver damage. The involvement of the nuclear factor (erythroid-derived 2)-like 2 (NRF2)/antioxidant responsive element (ARE) antioxidative pathway in Anwulignan-mediated anti-fatigue was analyzed using NRF2 inhibitor ML385 in HepG2 cells treated with H2O2. Animal welfare and experimental process follow the regulations of the Animal Ethics Committee of Beihua University. Anwulignan significantly lowered serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels, reduced liver tissue damages, increased superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), and catalase (CAT), and decreased malondialdehyde (MDA) and 8-hydroxy-2 deoxyguanosine (8-OHdG) contents in the livers of exhausted mice, demonstrating a strong antioxidant effect. Furthermore, Anwulignan up-regulated the NRF2/ARE antioxidant pathway in liver tissue, increased B-cell lymphoma 2 (Bcl-2) expression, and decreased Bcl-2-like protein 4 (Bax) and caspase3 expression. In HepG2 cells, Anwulignan improved the cell viability and SOD activity, reduced reactive oxygen species (ROS) and MDA contents, up-regulated the expression of the NRF2/ARE signaling pathway and Bcl-2, and decreased Bax and caspase3 expression in the cells. Furthermore, pretreated ML385 partly abolished all these effects of Anwulignan. Anwulignan protects the liver from damage in the exhausted mice by its antioxidant effects and related to its activation of the NRF2 pathway.
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Objective: to prepare neogambogic acid nanoliposomes [GNA-NLC] and study its pharmacokinetics [PK] in rats
Study Design: an experimental study
Place and Duration of Study: mudanjiang Medical University, Mudanjiang, China, from January 2016 to October 2017
Methodology: GNA-NLC was prepared by emulsion evaporation-low temperature solidification. The entrapment efficiency, average particle size, and zeta potential were investigated. Male Wistar rats were injected with 1 mg/mL gambogic acid and GNA-NLC into the caudal vein respectively, and the plasma concentration was determined by UPLCMS/MS. The pharmacokinetic parameters of the two agents were compared
Results: GNA-NLC prepared in this study were mostly spherical spheroids with an average particle size of 146.35 +/- 1.72 nm, polydispersity coefficient of 0.26 +/- 0.02, zeta potential of -28.24 +/- 0.13 MV, entrapment efficiency of 84.63%, and drug loading capacity of 4.23%. DSC showed that neogambogic acid nanoparticles had formed and neogambogic acid was amorphous in the matrix. The pharmacokinetics results in rats showed that GNA-NLC plasma concentration was significantly higher than that of common preparation of gambogic acid, with a half-life period of 10.14 +/- 0.03 hours, 4.57 times that of gambogic acid. AUC0 - 24hof gambogic acid in GNA-NLC lipidosome was 58.36 +/- 0.23 [micro]g/h/mL, 4.83 times that of gambogic acid
Conclusion: GNA-NLC can be prepared successfully by emulsion evaporation-low temperature solidification. The method is simple and easy to control. The GNA-NLC has a long cycle, and high blood concentration, sustained release compared with the raw material gambogic acid
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Orphan drugs are medicines or vaccines intended to treat, prevent or diagnose a rare disease. As the number of patients is limited, registration and developmening to enhance the understanding of rare dermatosis and related orphan drugs, this article chooses some presentative diseases in different categories of dermatosis to review, and explains the pathogenesis, therapeutic drugs and related mechanisms.
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<p><b>BACKGROUND</b>The dyschromatoses are a group of disorders characterized by simultaneous hyperpigmented macules together with hypopigmented macules. Dyschromatosis universalis hereditaria (DUH) and dyschromatosis symmetrica hereditaria are two major types. While clinical and histological presentations are similar in these two diseases, genetic diagnosis is critical in the differential diagnosis of these entities.</p><p><b>METHODS</b>Three patients initially diagnosed with DUH were included. The gene test was carried out by targeted gene sequencing. All mutations detected on ADAR1 and ABCB6 genes were analyzed according to the frequency in control database, the mutation types, and the published evidence to determine the pathogenicity.</p><p><b>RESULTS</b>Family pedigree and clinical presentations were reported in 3 patients from two Chinese families. All patients have prominent cutaneous dyschromatoses involving the whole body without systemic complications. Different pathogenic genes in these patients with similar phenotype were identified: One novel mutation on ADAR1 (c. 1325C>G) and one recurrent mutation in ABCB6 (c. 1270T>C), which successfully distinguished two diseases with the similar phenotype.</p><p><b>CONCLUSION</b>Targeted gene sequencing is an effective tool for genetic diagnosis in pigmentary skin diseases.</p>
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Adolescent , Enfant , Femelle , Humains , Mâle , Transporteurs ABC , Génétique , Adenosine deaminase , Génétique , Asiatiques , Diagnostic différentiel , Prédisposition génétique à une maladie , Génétique , Pedigree , Troubles de la pigmentation , Diagnostic , Génétique , Protéines de liaison à l'ARN , Génétique , Maladies génétiques de la peau , Diagnostic , GénétiqueRésumé
<p><b>BACKGROUND</b>H syndrome (OMIM 612391) is a recently described autosomal recessive genodermatosis characterized by indurated hyperpigmented and hypertrichotic skin, as well as other systemic manifestations. Most of the cases occurred in the Middle East areas or nearby countries such as Spain or India. The syndrome is caused by mutations in solute carrier family 29, member 3 (SLC29A3), the gene encoding equilibrative nucleoside transporter 3. The aim of this study was to identify pathogenic SLC29A3 mutations in a Chinese patient clinically diagnosed with H syndrome.</p><p><b>METHODS</b>Peripheral blood samples were collected from the patient and his parents. Genomic DNA was isolated by the standard method. All six SLC29A3 exons and their flanking intronic sequences were polymerase chain reaction (PCR)-amplified and the PCR products were subjected to direct sequencing.</p><p><b>RESULTS</b>The patient, an 18-year-old man born to a nonconsanguineous Chinese couple, had more extensive cutaneous lesions, involving both buttocks and knee. In his genomic DNA, we identified a novel homozygous insertion-deletion, c. 1269_1270delinsA, in SLC29A3. Both of his parents were carriers of the mutation.</p><p><b>CONCLUSIONS</b>We have identified a pathogenic mutation in a Chinese patient with H syndrome.</p>
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Adolescent , Humains , Mâle , Malformations multiples , Diagnostic , Génétique , Asiatiques , Prédisposition génétique à une maladie , Mutation , Transporteurs de nucléosides , Génétique , Malformations cutanées , Diagnostic , GénétiqueRésumé
The volatile components of roots and stems of Zanthoxylum nitidum were investigated by supercritical fluid carbon dioxide extraction (SFE-CO2) and gas chromatography-mass spectrometry(GC-MS). Thirty-one and fifty-one compounds were identified in the supercritical extracts from roots and stems of Z. nitidum, respectively, and total twenty-seven compounds were the common constituents. Among them, the major constituents in root and stem supercritical extracts were spathulenol (18.49 and 26.18%), n-hexadecanoic acid (14.24% and 12.79%), ar-tumerone (6.95% and 8.88%), oleic acid (8.39% and 5.71%) and hexanoic acid (4.39% and 7.78%). The in-vitro MTT assay showed that the volatile components of roots and stems of Z. nitidum did not exhibited any cytotoxic activity against human cancer Huh-7 and normal IEC-6 cells. These results indicated the same nature of the volatile constituents in the root and stem of Z. nitidum. This investigation may provide further evidence for expansion of medicinal parts of Z. nitidum.
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Animaux , Humains , Souris , Lignée cellulaire tumorale , Survie cellulaire , Chromatographie en phase supercritique , Médicaments issus de plantes chinoises , Chimie , Toxicité , Chromatographie gazeuse-spectrométrie de masse , Méthodes , Racines de plante , Chimie , Tiges de plante , Chimie , Zanthoxylum , ChimieRésumé
<p><b>OBJECTIVE</b>To observe the distribution difference of magnetic resonance imaging (MRI) signals in osteonecrosis of the femoral head (ONFH) patients of different TCM syndrome types.</p><p><b>METHODS</b>Recruited 29 ONFH patients were assigned to the tendon and vessel stagnation group (14 cases) and the Gan-Shen deficiency group (15 cases) according to TCM syndrome typing. The distribution difference of their MRI signals of MRIT1WI, T2WI + fs, T1 and T2 combined signal were compared.</p><p><b>RESULTS</b>There was statistical difference in T1W1 signal distribution between the two TCM syndrome types (P = 0.04). There was no statistical difference in T2WI + fs or T1 and T2 combined signal between the two TCM syndrome types (P = 0.42, P = 0.15). MRI signals in the tendon and vessel stagnation group were mainly manifested as fat-like signals,while they were mainly manifested as mixed signals in the Gan-Shen deficiency group.</p><p><b>CONCLUSION</b>Distribution difference of MRI signals exists between ONFH patients of different TCM syndrome types.</p>
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Adulte , Sujet âgé , Femelle , Humains , Mâle , Adulte d'âge moyen , Diagnostic différentiel , Nécrose de la tête fémorale , Diagnostic , Anatomopathologie , Imagerie par résonance magnétique , Médecine traditionnelle chinoiseRésumé
<p><b>OBJECTIVE</b>To isolate and identify active neuraminidase constituents of Polygonum cuspidatum against influenza A (H1N1) influenza virus.</p><p><b>METHOD</b>On the basis of the bioassay-guided fractionation,such chromatographic methods as silica gel, sephadex LH-20 and HPLC were adopted to isolate active constituents of extracts from Polygonum cuspidatum, and their molecular structures were identifiied on the basis of their spectral data such as NMR and MS and physico-chemical properties.</p><p><b>RESULT</b>Seven compounds were isolated from the ethyl acetate extract of P. cuspidatum and identified as 2-methoxystypandrone (1), emodin (2), resveratrol (3), polydatin (4), emodin-8-O-beta-D-glucopyranoside (5), (E)-3, 5, 12-trihydroxystilbene-3-O-beta-D-glucopyranoside-2'-(3", 4", 5"-trihydroxybenzoate) (6) and catechin-3-O-gallate (7), respectively. Among them, the NA test showed that compounds 3, 6 and 7 had inhibitory effect against NAs activity, with IC50 values of 129.8, 44.8 and 21.3 micromol x L(-1), respectively. Moreover, the further CPE test showed compounds 6 and 7 had significant inhibitory effect against H1N influenza virus (EC50 = 5.9, 0.9 micromol x L(-1), respectively), with very low cytotoxicity to the host cells, their therapeutic selective index(SI) in MDCK cells ranged from 56 to 269.</p><p><b>CONCLUSION</b>The neuraminidase inhibitors against H1N1 anti-influenza virus isolated from extracts of P. cuspidatum on the basis of the bioassay-guided fractionation are significant in specifying their therapeutic material basis and drug R&D against influenza.</p>
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Humains , Lignée cellulaire , Médicaments issus de plantes chinoises , Chimie , Pharmacologie , Antienzymes , Chimie , Pharmacologie , Polygonum cuspidatum , Chimie , Sous-type H1N1 du virus de la grippe A , Grippe humaine , Virologie , Structure moléculaire , SialidaseRésumé
<p><b>BACKGROUND</b>To study the arboviruses carried by mosquitoes collected in Hebei Province.</p><p><b>METHODS</b>Samples were collected from mosquito active sites and stored in liquid nitrogen till use. Pools of 20 to 30 mosquitoes were ground after sterilization, centrifugal supernant was inoculated onto C6/36 cell, cytopathic effect was observed for three sequential passages. Positive isolates were identified by IFA and RT-PCR.</p><p><b>RESULTS</b>Totally 1310 mosquitoes were collected from two villages of She county, Hebei province. They were divided into 46 pools and ground respectively. Thirteen positive isolates were obtained. Two isolates reacted with alphaviral antibodies and were amplified by alphaviral primers, nucleotide sequence showed the highest homology (98%) to Getah virus (AY702913.1), so the two isolates were identified as Getah virus.</p><p><b>CONCLUSION</b>Getah virus was isolated from mosquitoes in Hebei Province. This is the first report of isolating Getah virus from inland of China.</p>