This study was aimed to investigate the pro coagulation effects of hemocoagulase atrix and its effective components (batroxobin and factor X activator) on plasma of normal subjects and patients with bleeding disorders and their mechanisms. Activated partial thromboplastin time (APTT) and prothrombin time (PT) were measured. The factor (F)X activation and thrombin generation were analyzed by using chromogenic substrate method. The results showed that the plasma APTT of normal subjects was shortened by hemocoagulase atrix, batroxobin and FX activator, and the effect of FX activator was found to be concentration-dependent (r = 0.889, P < 0.05). The prolonged APTT of plasma from patients with bleeding disorders could be corrected by hemocoagulase atrix, batroxobin and FX activator, but PT showed no great changes resulted from the treatments. FX activator could promote FX activation and thrombin generation, while neither hemocoagulase atrix nor batroxobin showed such abilities. It is concluded that hemocoagulase atrix promotes coagulation process, and corrects coagulation abnormalities in patients with bleeding disorders, its main component batroxobin directly acts on fibrinogen, and FX activator promotes thrombin generation through activating FX.
Adult ; Batroxobin ; pharmacology ; Blood Coagulation ; drug effects ; Blood Coagulation Disorders ; blood ; Case-Control Studies ; Cysteine Endopeptidases ; pharmacology ; Factor X ; metabolism ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Proteins ; pharmacology ; Partial Thromboplastin Time ; Thrombin ; metabolism ; Young Adult

OBJECTIVETo investigate the clinical manifestations, pathologic features and laboratory findings in two Proteus syndrome patients with giant hemangiomas in the spleen and chronic DIC.

METHODSUltrasound imaging and magnetic resonance imaging (MRI) were used for analysing the characteristics of the giant hemangiomas in the spleen. The spleen specimen was examined pathologically for the feature of the hemangioma. Homostatic tests were performed by routine laboratory methods.

RESULTSTwo Proteus syndrome patients with giant hemangiomas in the spleen causing chronic DIC (Kasabach-Merritt syndrome) were first reported. They were recovered after splenectomy.

CONCLUSIONProteus syndrome when accompanied giant hemangioma could cause chronic DIC. Significantly decreased plasma fibrinogen level in this case might be helpful for the differential diagnosis from DIC caused by other diseases.

Adolescent ; Disseminated Intravascular Coagulation ; etiology ; Female ; Hemangioma, Cavernous ; complications ; diagnostic imaging ; surgery ; Humans ; Proteus Syndrome ; complications ; Splenectomy ; Splenic Neoplasms ; complications ; diagnostic imaging ; surgery ; Ultrasonography

OBJECTIVETo evaluate the clinic effect of abdominal needling (AN) on post-cardiosurgical operational gastrointestinal dysfunction.

METHODSSixty patients were equally assigned to two groups, the AN group and the control group. AN applied on the AN group was conducted by needling at Xiawan (Ren 10), Qihai (Ren 6), Qipang, Guanyuan (Ren 4), Qixue, Shuifen (Ren 9), Tianshu (St. 25) and Daju (St. 27), starting from the first day after operation, once every day with the needle retained time of 20 min, 5 days as one therapeutic course. The control group was treated with the conventional post-operational measurement with intravenous injection of 20 mg Omeprazole once a day, for 3 successive days. The score on symptoms as anorexia, weakness, abdominal distension, constipation, laziness to speak, nausea and vomiting, weakened borborygmus, pale-yellow complexion and edema, etc. , were observed.

RESULTSThe symptom scores in the aspects of anorexia, weakness, abdominal distension, constipation, weakened borborygmus, nausea and vomiting in the AN group were significant difference when compared with before treatment and the control group (P <0.01). After treatment the improvement in the control group only showed in weakness and pale-yellow complexion (P <0.01). Observation on gastric emptying showed that 24 h gastric paresis occurred in 17 patients (56.7%) of the AN group, and 22 (73.3%) of the control group; the 24 h and 72 h barium residue (strip) in the AN group was 10.60 +/- 4.61 and 8.53 +/- 4.08 respectively, while in the control group 17.07 +/- 3.99 and 15.83 +/- 4.19; the 24 h, 72 h gastric emptying rate in the former was (47.00 +/- 23.07) % and (57.33 +/- 20.37) %, and in the latter (14.67 +/- 19.95) % and (20.83 +/- 20.97) %, all showed significant difference between them (P <0.05 or P <0.01). Levels of motilin in the two groups after operation were decreased showing significant difference in comparison with before operation (P < 0.01), and AN group was raised after teratment, also showing significant difference between groups (P <0.01).

CONCLUSIONAN can strengthen gastrointestinal peristalsis, promote gastrointestinal emptying, recover quickly gastrointestinal hormone to the normal range. Besides, it is safe and effective, with no pain or only slight pain, easy in locating the acupoint accurately, standard prescription, high compliance of patients, and convenient for clinical operating, therefore, it is worthy of spreading in clinical practice.

Acupuncture Points ; Acupuncture Therapy ; Adolescent ; Adult ; Aged ; Cardiac Surgical Procedures ; adverse effects ; Female ; Gastrointestinal Diseases ; etiology ; physiopathology ; therapy ; Gastrointestinal Tract ; physiopathology ; Humans ; Male ; Middle Aged ; Postoperative Complications ; physiopathology ; therapy ; Young Adult

OBJECTIVETo identify clinical and laboratory abnormalities and genetic defect of Fechtner syndrome in a Chinese family.

METHODSThe characteristic morphological features of platelets and leukocytes were examined on blood smears with Wright's-Giemsa staining and ultrastructure of platelet and leukocyte were investigated under electron microscope. Genomic DNA was isolated from peripheral blood of the proband and 9 members of his family. All the exons and exon-intron boundaries of the MYH9 gene were amplified by PCR followed by direct sequencing.

RESULTSPatients presented the characteristic clinical features including macrothrombocytopenia, leukocyte inclusions and/or hereditary nephritis. A heterozygous C to T mutation was found in the proband and three members of his family at nucleotide 5981 in exon 40 of MYH9 gene, resulting in a nonsense mutation which encoded truncated protein due to premature termination at the Arg 1933 codon.

CONCLUSIONIt is the first report of a Chinese family with Fechtner syndrome. The Arg (CGA) 1933--> stop (TGA) nonsense mutation in MYH9 gene is a causative genetic defect.

Adult ; Codon, Nonsense ; DNA Mutational Analysis ; Exons ; genetics ; Humans ; Inclusion Bodies ; genetics ; Male ; Molecular Motor Proteins ; genetics ; Myosin Heavy Chains ; genetics ; Nephritis, Hereditary ; genetics ; Pedigree ; Syndrome ; Thrombocytopenia ; genetics

OBJECTIVETo identify gene mutations involved in five cases of inherited factor V (FV) deficiency.

METHODSActivity of FV was determined by one-stage clotting assay using FV-deficiency plasma, and FV antigen by an ELISA assay. All the exons and exon-intron boundaries of FV gene were amplified by PCR and then DNA sequencing. Restriction enzyme analysis was used to analyze the probands, their family members and healthy volunteers.

RESULTSBoth activity and antigen of FV in the 5 patients were extremely lower compared with that of normal mixed plasma. Six mutations were identified in these 5 patients, G69969T (G2079V), C45533T (R712Ter), C46796T (R1133Ter), G45366A (C656Y), C46253T (R952C) and G16088C (D68H), the latter three were novel mutations reported for the first time and the C46253T (R952C) was the first missense mutation reported in B domain. The result of sequencing or restriction enzyme analysis showed that the three novel missense mutations were not caused by single nucleotide polymorphisms.

CONCLUSIONGene mutations in 5 type I inherited FV deficiency of patients including 2 nonsense mutations and 4 missense mutations identified which led to the instability of FV protein and the reducing of FV: Ag in the plasma.

Adolescent ; Adult ; Child ; DNA Mutational Analysis ; Exons ; genetics ; Factor V ; genetics ; metabolism ; Factor V Deficiency ; blood ; genetics ; Female ; Humans ; Male ; Mutation ; Pedigree ; Phenotype ; Young Adult

OBJECTIVETo study the clinical features and ABCG5/ABCG8 gene mutations of three pedigrees of phytosterolemia presented with macrothrombocytopenia and hemolysis.

METHODSErythrocyte and platelet morphology were examined under light microscope. Plasma sterol levels were measured by high pressure/performance liquid chromatography method. All of ABCG5 and ABCG8 exons and intron-exon boundaries were directly sequenced to identify mutations, the corresponding gene mutation sites of three families members and healthy individuals were detected.

RESULTSAll the patients presented macrothrombocytopenia, hemolysis, splenomegaly and xanthomas. The blood smears showed large platelets, some as large as erythrocytes, and abnormal erythrocyte shapes, such as stomatocytes. Plasma concentrations of phytosterols, especially sitosterol were markedly elevated (30 fold) in the affected patients. Four mutations were identified in these three pedigrees, ABCG5 C20896T (R446X) and A20883G, ABCG8 del43683-43724 and del1938C-1939G/ins1938T. The latter three were novel mutations reported for the first time.

CONCLUSIONSPhytosterolemia associated with macrothrombocytopenia and hemolysis is a new subtype of this disease. Plasma phytosterols and related gene analysis should be performed when ever an unexplained macrothrombocytopenia, especially combined with haemolysis or/and stomatocytosis.

ATP Binding Cassette Transporter, Sub-Family G, Member 5 ; ATP Binding Cassette Transporter, Sub-Family G, Member 8 ; ATP-Binding Cassette Transporters ; genetics ; Adult ; Blood Platelets ; cytology ; DNA Mutational Analysis ; Erythrocytes, Abnormal ; Female ; Hemolysis ; genetics ; Humans ; Hypercholesterolemia ; genetics ; pathology ; Intestinal Diseases ; genetics ; pathology ; Lipid Metabolism, Inborn Errors ; genetics ; pathology ; Lipoproteins ; genetics ; Male ; Middle Aged ; Mutation ; Pedigree ; Phytosterols ; adverse effects ; blood ; genetics ; Platelet Count ; Thrombocytopenia ; genetics ; pathology

OBJECTIVETo investigate the diagnostic value of contrast-enhanced ultrasonography (CEUS) in preoperative T-staging of gastric cancer.

METHODSA sulfur hexafluoride-filled microbubble ultrasound contrast agent and a continuous real-time imaging technique of contrast pulse sequencing were used. Normal gastric wall was examined by CEUS in 8 healthy volunteers and the results were compared with the findings on multislice computed tomography. Sixty-two patients with gastric cancer proved by biopsies who received preoperative CEUS examination were involved in this study, and the CEUS result was compared with postoperative pathological findings.

RESULTSThe normal gastric wall presented a one-layer structure in the portal venous phase and a three-layer structure in the arterial and equilibrium phase including a slightly hyper-enhanced inner layer, a hypo-enhanced intermediate layer, and a markedly hyper-enhanced outer layer, which corresponded histologically to the mucosal, submucosal, and muscular-serosal layer, respectively. The accuracy of transabdominal ultrasonography and CEUS in determining the T stage of gastric cancer was 72.9% and 88.1% respectively, and the difference was statistically significant (chi(2)=4.37, P=0.036).

CONCLUSIONSCEUS shows the normal gastric wall as a one- or a three-layer structure, which provides a theory base for CEUS in preoperative T-staging of gastric cancer. CEUS is a useful diagnostic method for preoperative T-staging of gastric cancer.

Adult ; Aged ; Contrast Media ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Staging ; methods ; Stomach Neoplasms ; diagnostic imaging ; pathology ; Sulfur Hexafluoride ; Ultrasonography ; methods

OBJECTIVETo evaluate the clinical value of double contrast-enhanced ultrasonography using oral and intravenous contrast agents in preoperative staging of gastric cancer.

METHODSSixty-two patients with biopsy-proven gastric cancer were enrolled into this study, and were examined by double contrast-enhanced gastric ultrasonography preoperatively. The results were compared with postoperative pathologic findings.

RESULTSThe accuracy of oral contrast-enhanced gastric ultrasonography and double contrast-enhanced ultrasonography in determining the T stage of gastric cancer was 72.9% (T1: 66.7%, T2: 60.0%, T3: 76.9%, T4: 71.4%) and 88.1% (T1: 66.7%, T2: 80.0%, T3: 89.7%, T4: 100%), respectively, with a statistically significant difference between the two methods (P = 0.036). The sensitivity, specificity, accuracy and Youden index of oral contrast-enhanced gastric ultrasonography and double contrast-enhanced ultrasonography in assessment of lymph node metastasis were 74.5%, 66.7%, 72.9%, and 0.41 versus 89.4%, 75.0%, 86.4%, 0.76, respectively. No significant difference in the accuracy of assessment for lymph node metastasis was observed (P > 0.05).

CONCLUSIONDouble contrast-enhanced ultrasonography is useful for preoperative staging of gastric cancer, especially for T staging.

Adult ; Aged ; Contrast Media ; Endosonography ; methods ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Staging ; methods ; Preoperative Care ; Prospective Studies ; Stomach Neoplasms ; diagnostic imaging ; pathology ; surgery ; Sulfur Hexafluoride

This study was purposed to applicate the bone marrow indirect Coombs test and investigate its clinical significancies in diagnosis of immuno-related pancytopenia (IRP). 30 patients with pancytopenia including 22 cases of IRP and 8 cases of idiopathic cytopenia of undetermined significance (ICUS), and 15 patients with iron-deficiency anemia as controls were enrolled in this study. After incubation of the bone marrow supernatant of IRP patients and bone marrow nucleated cell (BMNC) of controls was used as experiment group, while the incubation of BMNC and bone marrow supernatant of controls was used as control group. After incubation for 45 min, the positive rate of membrane antibodies in bone marrow hematopoietic cells (CD15(+), GlyCoA(+) and CD34(+)cells) was detected by flow cytometry, and correlation analysis of positive rate with clinical data of patients were analyzed. The results showed that among 30 patients with pancytopenia (16 positive and 14 negative for bone marrow direct Coombs test) 16 cases showed positive for bone marrow indirect Coombs test, with positive rate 53.33. In the experiment group, the median positive rate of CD15(+)IgM was 0.34, which was significantly higher than that in control group (0.20, P < 0.05); the median positive rates of CD34(+) IgG and IgM were 0.64 and 0.21 respectively, which were significantly higher than those in control group (0.00, P < 0.05) and (0.00, P < 0.05); the positive rates of GlyCoA(+)IgG and IgM were (0.83 ± 0.75) and (2.12 ± 1.98) respectively, which were significantly higher than those in control group [(0.47 ± 0.43), P < 0.05, (0.68 ± 0.64), P < 0.01]; the positive rates of CD15(+) IgG and IgM were positively correlated with the ratio of CD5(+)B cells. The positive rates of GlyCoA(+) IgG and IgM negatively correlated with the Hb level, percentage of reticulocytes, the ratio of bone marrow erythroid lineage and DC1/DC2 positively correlated with the ratio of CD5(+)B cells and indirect bilirubin level. It is concluded that antibodies (IgG or IgM) aiming at the bone marrow hematopoietic cells exist in the supernatant of some IRP and ICUS patients, and may act on the membrane protein of the normal BMNC. These antibodies correlate with the prognosis of IRP.
Adolescent ; Adult ; Aged ; Bone Marrow Cells ; immunology ; Child ; Coombs Test ; Female ; Humans ; Male ; Middle Aged ; Pancytopenia ; diagnosis ; immunology ; Young Adult

OBJECTIVETo analyze the phenotype and genotype of a family with inherited dysfibrinogenemia.

METHODSAssays of coagulation, including activated partial thromboplastin time (APTT), prothrombin time (PT) and thrombin time (TT), were carried out with Stago Compact in the proband and his family members. The activity and antigen of fibrinogen in plasma were determined by Clauss and immunoturbidimetry, respectively. Fibrinogen and its constituent were analyzed by Western blot with nonreducing 4%-20% SDS-polyacrylamide gel electrophoresis (PAGE). All exons and exon-intron boundaries of fibringen genes FGA, FGB and FGG were analyzed by PCR and then direct sequencing.

RESULTSThe proband had normal APTT and PT, but prolonged TT. The activity of fibrinogen in plasma was decreased while its antigen level was normal. These abnormalities were also found in his mother and a sister. Genetic analysis revealed heterozygous G1233A in the exon 2 of FGA originating from his mother, which resulted in Arg16His missense mutation.

CONCLUSIONInherited dysfibrinogenemia was caused by Arg16His mutation in exon 2 of FGA, and this is the first case reported in a Chinese family.

Fibrinogen ; genetics ; Genotype ; Humans ; Mutation ; Pedigree ; Phenotype