Objective To evaluate the quality of cryoprecipitates prepared from fresh frozen plasma via deepfreeze method and siphonage method thawing at 4℃ water bath,and to select a proper preparation method for cryoprecipitates.Methods Sixty samples of cryoprecipitate prepared by deepfreeze method,and 60 samples of cryoprecipitate prepared by siphonage method were selected randomly.Factor FⅧ,fibrinogen(Fg) in cryoprecipitates were measured by coagulometer,and the qualification rates were calculated.Results The concentration of FⅧ(IU/pack) in the cryoprecipitates prepared via deepfreeze method and siphonage method were(70.30?23.68),and(161.62?39.45),respectively;the qualification rate of FⅧ prepared via the two methods were 43.33%(26/60),and 93.33%(56/60),respectively(P0.05).Conclusion Siphonage method,with a higher qualification rate of FⅧ,is simple and fast,which deserves a popular application.

Acute hepatic failure was induced in rats with intraperitoneal administration of D-galactosamine.Serum and tissue amino acid profiles of the animals were studied. Except for arginine, serum levels of all amino acids were elevated. This is in variance with the findings of Fischer and other investigators. In their studies, the decreased levels of BCAA could be related to the administration of dextrose. In our study, the serum BCAA/AAA ratio was decreased and it was correlated negatively with the dosage of D-galactosamine (r =-0.789). Amino acid profiles in the liver,kidney and muscles were significantly altered. The chracteristics of the changes indicated that enhanced proteolysis of tissue proteins was the major factor accounting for the elevation of serum amino acid levels. Hepatic coma might be related to the increase of aromatic amino acids in the brain. It is usually difficult for glycine, cystine, asparagine and threonine to penetrate the blood-brain-barrier. Elevation of their levels in the brain might be a consequence of increased permeability of the blood-brain-barrier. Further studies are needed to clarify the role of GABA in hepatic coma.

Objective To investigate the expression of nm23-H1 in human benign and malignant colorectal lesions and the relationship between invasion,metastasis in colorectal carcinoma.Methods The expression of nm23-H1 were determined by ABC immunohistochemical technique in 80 cases human colorectal carcinoma,and compared with that in adenoma and normal mucus.Result Positivity expression rates of nm23-H1 were 66.7% in colorectal carcinoma.No significant difference of the expression of nm23-H1 in normal mucus,adenoma and cancerous tissue (P>0.05).The positive rates of nm23-H1 expression in different pathological grading,lymph node metastasis and post-operative survival (3 years) were showed significant difference (P<0.05).Conclusions It is suggested that the deletion of nm23-H1 low expression might play an important role in the carcinogenesis,development,metastasis and prognostic value of human colorectal carcinoma.

Objective To investigate mutation spectrums of α- and β-haemoglobin genes in thalassemia patients and carriers in Yunnan province,and to establish procedures on prenatal gene diagnosis.MethodsTotally 10 033 counseling couples and pregnant women,and 22 cases of children with moderate or severe thalassemia were recruited from 5 parts of Yunnan Province,middle,western,eastern,southern and northern areas, during July 2009 to July 2011.Medical records, including results of haemoglobin electrophoresis,blood routine examination,and gene diagnosis of subjects were collected and saved in an database in Excel software by the Key Laboratory for Birth Defects and Genetic Diseases.Using multiple gap-PCR and PCR-reversed dot blotting kits, DNA samples collected from 1077 cases of haematological positive thalassemia patients and carriers were tested to determine common mutations of the α-or β-haemoglobin genes.The codon regions of haemoglobin genes were sequenced by the Sanger sequencing in cases that the mutation tests were negative.Mutation spectrums of α- and β-haemoglobin genes were concluded.Prenatal gene diagnosis was offered to fetuses who had risk of thalassemia major.Results( 1 ) In 1077 cases of haemological screen positive subjects,deletions and mutations of α-haemoglobin gene were tested in 119 subjects among 347 cases suspected as α-thalassemia patients and carriers.Five kinds of deletions and mutations on α-haemoglobin gene were found.In 104 subjects,four kinds of common deletions and mutations onα-haemoglobin gene were determined:--SEA, -α3.7, αCS α,-α4.2.Other 14 subjects were double heterozygotes with haemoglobin H disease and severe α-thalassemia phenotypes.A rare mutation of insertion and deletion in α2 haemoglobin gene intron,α301-24-301-23 indel,was found in one carrier subject.(2)In 1077 cases of haemological screen positive subjects,deletions and mutations of β-haemoglobin gene were tested in 297 subjects among 730 cases suspected as β-thalassemia patients and carriers.Sixteen kinds of β-haemoglobin gene mutations were found,including 7 cases of rare abnormal haemoglobinopathy patients with β-haemoglobin gene mutations.In one case with β + phenotype patient,the Codon 5 (-CT)mutation at β-haemoglobin gene was found (firstly reported in China ). (3) Three fetuses with high riskS of α-thalassemia were accepted for prenatal diagnosis.One case of Hb Bart's hydrops syndrome fetus with the genotype --SEA/--SEA,and one case of mild α-thalassemia fetus with the genotype αCS α/αα were found.Another one fetus was found with normal α-haemoglobin.In 6 fetuses accepted for prenatal diagnosis due to high risks of β-thalassemia,one case of β-thalassemia major with the genotype CD17( A→T)/-28 (A→G) was found,3 fetuses were heterozygote carriers,and 2 fetuses had normal genotypes without mutations found in their parents.Medical terminations for 2 fetuses with severe thalassemia were made according to the choice of pregnant women.Other 7 pregnancies continued to term.Anemia or growth retardation was not found in the 7 infants when following up after given-birth 6 to 12 months.Conclusions The mutation spectrums of α- and β-haemoglobin genes of thalassemia patients and αarriers.in Yunnan province are special,in which β-haemoglobin gene exits more polymorphism in the mutation spectrum.Carrier screening in pregnant women,and offering prenatal gene diagnosis to the high risk pregnancies should be an efficient strategy to prevent thalassemia major.

Objective To observe the changes of cerebral blood flow velocities and carbon dioxide (CO_2) reactivity of cerebral vessels in children after cardiopulmonary resuscitation,and the relationship between the changes of cerebral blood flow and outcome.Methods The waveforms,cerebral blood flow velocities of left middle cerebral artery and extracranial internal carotid artery were monitored by Transcranial Doppler (TCD) in 35 post-resuscitation children. Glasgow coma score (GCS) was estimated at the same time.They were divided into three groups according to the diastolic blood flow velocities:low perfusion group,high perfusion group and near-normal perfusion group.CO_2 reactivity of cerebral vessels was induced by hyperventilation in 26 children under ventilation.Results In both low and high perfusion groups,GCS and CO_2 reactivity of cerebral vessels were significantly lower than that of near-normal perfusion group (P＜0.05);the numbers of died and withdrawing treatment patients were significantly higher than that of near-normal perfusion group (P＜0.01).Conclusion TCD monitoring is a useful technique for estimating cerebral perfusion,outcome and CO_2 reactivity of cerebral vessels of children after cardiopulmonary resuscitation.A secondary ischemic event may be caused by a forced hyperventilation therapy in the condition without TCD monitoring in post-resuscitation patients.

ObjectiveTo investigate the incidence and risk factors in retinopathy of prematurity (ROP) at matched gestational age.Methods Data collected by the medical records of neonates from November 2007 to December 2010 in our neonatal database were analyzed.Patients'information was recorded from birth to discharge from NICU.Data included age after birth (hours),gestational age,body weight at birth,treatment,and maternal demographics.The statistical study was carried out by SPSS version 13.0 software.Mann - Whitney U test was used for numerate data.Fisher's exact probability test and Pearson's chi - square test were used to compare quantitative variables between independent groups.P values were considered significant when they were less than 0.05 ( two - sided).ResultsThe incidence of ROP was 5.38％.Gestational age of ROP group and control group was matched ( P =0.387 ),but difference in body weight at birth was significant ( P =0.045 ).Age after birth was significantly different ( P =0.013 ).Apnea and anemia were significantly different between two groups (P ＜ 0.01). The differences in duration of hospitalization,NCPAP and use of antibiotics between two groups were significant ( P ＜ 0.002,0.000 and 0.000,respectively).ROP group differed from control group greatly in hospitalization expenses.Multiple stepwise logistic regression analysis showed that oxygen supplenentation ( P =0.0237 ) and infection ( P =0.0118) were risk factors of ROP. ConclusionsThe incidence of ROP in NICU in Beijing Children's Hospital was 5.38％.Inhalation of oxygen supplementation and infection were risk factors of ROP.

ObjectiveTo investigate key techniques and intervention in reducing birth defects. Method Down's syndrome (DS), trisomy-18 (Edwards syndrome, ES), neural tube defects (NTD), Duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA), thalassemia, and glucose-6-phosphate dehydrogenase deficiency (G6PD) were chosen as target disease. From Jan. 2007 to Dec. 2009, the condition of intake folie acid were investigated in 5004 pregnant women in Panlong District and Wuhua District of Kunming City. All of the 27 660 pregnant women undergoing prenatal examination were enrolled into the study from the First People's Hospital of Yunnan Province, the Second People's Hospital of Yunnan Province, the First People's Hospital of Qujing City, the Second People's of Qujing City, Qujing Women and Children's Hospital, People's Hospital of Lincang City, Kunming Maria Women's Hospital, Maternal and Infant's Care Unit of Panlong District of Kunming City, Maternal and Infant's Hospital of Dali City. The screening was performed on serum of those pregnant women at 8 -20 +6 gestational weeks. Prenatal cytogenetic analysis and fetal ultrasonogrspy were performed on the high risk or indicated women after genetic counseling. DNA analysis was administered on those women with family or childbearing history of DMD,SMA,thalassemia,orG6PD. Outcomeof pregnancywasfolloweduptoevaluatetheeffectof intervention. ResultsApproximately 30. 10％ (1506/5004) of pregnant women were administered by oral folic acid during perinatal period. Two thousand three hundred and thirteen women with high risks of DS,ES, or NTD fetuses were observed among 27 660 undergoing maternal serum screening. Two thousand and ninety-six pregnant women including two twins pregnant women were performed cytogenetic analysis. Other 67 pregnant women at high risk of DMD, SMA, thalassemia, and G6PD accepted genetic counseling and prenatal gene analysis. Two thousand one hundred and sixty-three pregnant women (2165 fetuses) underwent prenatal examination. One hundred and two cases chromosome abnormalities, 17 cases NTD, 4 cases DMD, 1 cases α-thalassemia major were found. All of the 91 fetuses with major birth defects were terminated after genetic counseling. Another affected DS fetus in a twin pregnancy dead intrauterine at 24 gestational weeks. Thirty-two women bearing fetuseswithbalancedtranslocations orinversionscontinuedtheir pregnancies. Totally 2071 normal term fetuses were born in the prenatal diagnosis group. Two fetuses with normal chromosome were lost within 1 week after amniocentesis. Four affected DS fetuses were born from their high risk mothers who refused further prenatal diagnosis service. In a random sampling follow-up cohort of 5000 mothers at low risk, none of affected child suffering target diseases was found. The DS detection rate of maternal serum screening was 84％ (27/32), with the false positive rate was 6. 153％ (1702/27 660).ConclusionsFolic acid intake before conception and in the first trimester would reduce the risk of birth defects, only 1/3 reproductive women took folie acid actively. Maternal serum screening could effectively detect high risk of DS, ES and NTD. The genetic counseling is critical in women at high risk or who had family history of inherited disorders. The prenatal screening and diagnosis combined with routine obstetric care could reduce the incidence of major birth defects, which should become prenatal care strategy in our country.

Nutcracker syndrome is caused by external compression of the left renal vein and is characterized by various symptoms, such as hematuria and left flank pain. However, long-standing gross hematuria is uncommon. We report the case of a 23-year-old woman who had had painless gross hematuria for several months. Kidney biopsy revealed thin basement membrane disease, but this did not explain the gross hematuria with proteinuria. Renal venography and Doppler ultrasonography showed increased diameter and velocity ratios between the left renal vein and inferior vena cava. Cystoscopy revealed that the hematuria originated from the left ureteral orifice. These results indicate coexisting Nutcracker syndrome. Nutcracker syndrome with thin basement membrane disease should be considered in the differential diagnosis of a patient who has long-standing gross hematuria.

Objective:To investigate the method and effect of correcting upper eyelid depression with released orbital diaphragmatic fat.Methods:From January 2017 to June 2020, there were 26 female patients with upper eyelid depression in this department of Hebei Chengde Stomatological Hospital. The patients aged 22-54 years, with average 43 years. An external mass orbital diaphragm fat release reverse folding repositioning with fat placement in the upper lid depression was performed in 26 patients. An incision about 4 mm long was made in the middle and lateral of the orbital diaphragm to ensure the integrity of the orbital diaphragm as far as possible. The photos of beauty seekers before and 6 months after operation were compared to observe the correction effect of upper eyelid depression and the shape of upper eyelid.Results:All 26 patients had one-stage healing of the surgical incision without infection, with inconspicuous incisional scarring. At 6 months postoperatively, 18 patients had satisfactory results; 1 patient had improved results due to insufficient fat; and 1 patient had poor results due to too little fat and poor filler placement, which was later improved by autologous fat grafting.Conclusions:External mass orbital fat release reverse folding repositioning for correction of upper eyelid depressions is a surgical procedure with obvious results, little trauma, and following the principle of anatomical recovery, which is worthy of clinical application.

Objective: To survey the conduction and evaluate the effectiveness of extracorporeal membrane oxygenation (ECMO) therapy in pediatric intensive care unit (PICU) in China mainland. Methods: In a questionnaire-based survey, we retrospectively reviewed the application of ECMO in children's hospital and general hospital in China mainland to summarize and analyze the categories of diseases and prognosis of children treated with ECMO therapy. Results: By December 31, 2017, a total of 23 hospitals using ECMO, including 22 tertiary referral hospitals and 1 secondary hospital, among which 16 were children′s hospitals and 7 were general hospitals. Thirty-seven ECMO equipment was available. A total of 518 patients treated with ECMO, within whom 323 (62.4%) successfully weaned from ECMO and 262 (50.6%) survived to discharge. Among 375 pediatric patients, 233 (62.1%) were successfully weaned from ECMO and 186 (49.6%) survived to discharge. Among 143 newborn patients, 90 (62.9%) successfully weaned from ECMO, 76 (53.1%) survived to discharge. ECMO was applied in veno-arterial (VA) mode to 501 (96.7%) patients, veno-venous (VV) mode to 14 (2.7%) patients, and VV-VA conversion mode to 3 (0.6%) patients. Sixty-nine patients required extracorporeal cardiopulmonary resuscitation (ECPR), including 20 newborn patients (29.0%) and 38 pediatric patients (71.0%), who were all with cardiovascular disease. Neonatal respiratory distress syndrome (26/61), persistent pulmonary hypertension of the newborn (PPHN) (12/61), and meconium aspiration syndrome (MAS) (11/61) are the most common pulmonary diseases in newborn patients; among whom, infants with PPHN had highest survival rate (10/12), followed by MAS (9/11). Among newborn patients with cardiovascular diseases, those who admitted were after surgery for congenital cardiac disease were the most common (54/82), while those with septic shock had the highest survival rate (2/3). In pediatric pulmonary diseases, acute respiratory distress syndrome was the most common (42/93), while plastic bronchitis was with the highest survival rate (4/4), followed by viral pneumonia (13/16). Among pediatric cardiovascular diseases, congenital cardiac defect was the most common (124/282), while fulminant myocarditis had the highest survival rate (54/77). Conclusion The application of ECMO as a rescue therapy for children with severe cardiopulmonary failure has dramatically developed in China mainland.