Influence of Dahuang Zhechong Pills Combined with Compound Gosspol Acetate Tablets on Serum Markers of Endometriosis Patiens After Operation with Laparoscopy / 中国中医药信息杂志

Objective To explore the clinical efficacy of Dahuang Zhechong Pills combined with compound gosspol acetate tablets for endometriosis (EM) after operation with laparoscopy; To observe changes in serum markers. Methods Totally 98 EM patients after operation with laparoscopy were divided into the control group (49 cases) and observation group (49 cases). All patients had laparoscopic conventional anti-infective, anti-inflammatory, nutritional support, maintenance of electrolyte and acid-base balance, for 14 d. The control group treated with compound gosspol acetate tablets, one tablet each time, once a day, orally after dinner; the observation group was given Dahuang Zhechong Pills on the basis of control group, two pills per time, twice a day, orally. The treatment course was 6 months. The clinical efficacy and symptom scores were evaluated in the two groups. The levels of MMP-3, CA-125, EMAb, IgG, FSH, E2 and LH were detected before and after treatment in the two groups. The adverse reaction was observed during the treatment. Results After the treatment, the total effective rate was 87.7％(43/49) in observation group, and 73.4％ (36/49) in control group, with statistical significance (P<0.05). After the treatment, the symptom scores were significantly lower in both groups, and the observation group was lower than the control group (P<0.05). After the treatment, the levels of MMP-3, CA-125, EMAb, FSH, E2 and LH decreased in both groups (P<0.05). The levels of MMP-3, CA-125, EMAb, FSH, E2 and LH in the observation group were lower than the control group (P<0.05). There were no serious adverse reactions in the two groups during the treatment period. Conclusion Dahuang Zhechong Pills combined with compound gosspol acetate tablets in the treatment of EM after operation with laparoscopy has a good clinical curative effect and can decrease the levels of MMP-3, CA-125, EMAb and estrogen levels.

A preliminary study of an inherited macrothrombocytopenia disorder with abnormal large granules / 中华血液学杂志

<p><b>OBJECTIVE</b>To study the platelet morphology and function of an inherited macrothrombocytopenia disorder with abnormal large granules.</p><p><b>METHODS</b>Platelet size and structure were investigated by both light microscopy and electron microscopy. The platelet membrane expression of GP I b, GP II b, GPIII a, P-selectin and CD63 were analyzed by using respective monoclonal antibodies. Platelet 5-hydroxy-tryptamine was measured with spectrophotofluorometer.</p><p><b>RESULTS</b>Both the patient and her father had large granules in their platelets, with exocytosis being easily observed. The expressions of GP I b, GP II b and GP II a on the platelets were in normal range, while P-selectin and CD63 were somewhat increased. The abnormal large granules were not the alpha granules, lysosomes or dense bodies.</p><p><b>CONCLUSION</b>Both morphological and functional abnormalities of the platelets from the patient are clearly distinguishable from other hereditary giant platelet disorders. It would probably represent a novel platelet disorder.</p>

A novel genetic defect in a Chinese family with inherited coagulation factor XIII deficiency / 中华血液学杂志

<p><b>OBJECTIVE</b>To identify the genetic defect of inherited coagulation factor (F) deficiency in a Chinese family and to explore its molecular mechanism.</p><p><b>METHODS</b>The activity and antigen of plasma F were measured by photometric test and enzyme-linked immunosorbent assay, and rocket-electrophoresis, respectively. All the exons and exon-intron boundaries of the FA subunit gene were amplified by PCR and then DNA sequencing was performed. Restriction endonuclease analysis was used for the PCR products of the family members and 80 healthy donors to exclude gene polymorphism.</p><p><b>RESULTS</b>Rapid dissolution of the proband's fibrin clot occurred within 30 minutes, and antigen of his plasma F was significantly decreased, two compound heterozygous missense mutations (a C to T transition at nucleotide 177,246 which caused Arg703Trp, and a A to G transition at nucleotide 177,286 which caused His716Arg) in exon 15 of FA subunit gene were found. The possibility of gene polymorphism was excluded by restriction endonuclease analysing. Each of these two missense mutations was respectively found in his mother and father. Molecular modeling based on 3D crystallographic data predicted that the mutant protein decreased stability and was likely to be rapidly degraded.</p><p><b>CONCLUSIONS</b>The inherited F deficiency in the Chinese family is caused by two compound heterozygous missense mutations-Arg703Trp and His716Arg in the FA subunit, which to our knowledge, are reported for the first time.</p>

Congenital afibrinogenemia associated with a novel nonsense mutation in the FGA gene / 中华血液学杂志

<p><b>OBJECTIVE</b>To identify the genetic defect underlying congenital afibrinogenemia in a Chinese family.</p><p><b>METHODS</b>Plasma fibrinogen (Fg) was assessed by both Clauss method and immunonephelometry. Genomic DNA was isolated from peripheral blood of the proband and 13 members of her family. All the exons and exon-intron boundaries of the three fibrinogen genes (FGA, FGB, FGG) were amplified by PCR followed by direct sequencing. Restriction endonuclease analysis was performed for the PCR products of the family members and 50 healthy donors to exclude gene polymorphism.</p><p><b>RESULTS</b>No Fg was detected in the plasma of the proband and her father by Clauss method, while low levels (< 0.02 g/L) were detected by immunonephelometry. A homozygous C to T mutation was found in the two cases at nucleotide 3108 in exon 4 of FGA gene, resulting in a null mutation which encoded severely truncated alpha-chains owing to its premature termination at the Gln 150 codon. The C-->T mutation eliminated a unique recognition site for restriction enzyme RsaI. The PCR amplified fragments of the proband and her father could not be digested by RsaI, showing that they are homozygous. Her mother and some family members are heterozygous at this site since the fragment could partly be digested, while the same fragment of controls could be completely digested as expected.</p><p><b>CONCLUSION</b>The Gln (CAG)-->150stop (TAG) nonsense mutation in FGA gene is a novel genetic defect of congenital afibrinogenemia which, to our knowledge, has not been described before.</p>