Objective To primarily explore the efficacy and adverse effects of the combination of fiudarabine,cytarabine and granulocyte colony-stimulating factor(G-CSF)(FLAG regime)therapy for relapsed and refractory acute leukemia in children.Methods Ten children were treated with the FLAG regime for relapsed and refractory acute myeloid leukemia (AML)and acute lymphoblastic leukemia(ALL)from Feb.2007 to Mar.2010.There were 8 male and 2 female,with mean age 8 years(ranging from 4 to 12 years).AML was diagnosed in 8 children,AML-M2 in 5 cases,AML-M4 in 3 cases.ALL was diagnosed in 2 children,both were B-ALL.Six children had refractory disease,and 4 cases were in relapse.FLAG regime included:fludarabine 25 mg?m-2?d-1,days 1-5;cytarabine 2 g?m-2?d-1,days 1-5;G-CSF 150-300 ?g?d-1,from day 0 to neutrophils ≥0.5?109 L-1.Results Complete remission was obtained in 6 children(60%),partial remission was obtained in 1 child(10%),and 3 children were considered non-response(30%).The total effective rate was 70%.For 8 children with AML,6 children had achieved complete remission(75%),2 children had non-response(25%).While in children with ALL,1 child got partial remission,and the other one had non-response.Myelosuppression and infections due to neutropenia were the most frequent adverse effects,severe nonhematologic toxicity were not observed in these children.And there were no chemotherapy-related death.Conclusions The FLAG regime is effective in treatment of children with relapsed and refractory acute leukemia,especially for the children with the relapsed and refractory AML.The adverse effects from this regime were well tolerated.FLAG regime can give children with relapsed and refractory acute leukemia another chance.

Objective To evaluate the role of imaging diagnosis in surgical treatment of primary aldosteronism(PA). MethodsFrom Jan 1995 to Dec 2004,245 patients with PA were hospitalized in our hospital.Before the operations,all the patients underwent B-ultrasonography and CT scaning,240 received intravenous pyelography and 75 MRI.The preoperative imaging diagnosis were compared with the findings during the operations and postoperative pathologic results. Results Compared with the findings during the operations,the accuracy rates of localized diagnosis for PA with B-ultrasonography,CT scanning and MRI were 92.7%,98.2% and 90.4%,respectively.Compared with the postoperative pathologic results,the accuracy rates of qualitative diagnosis for aldosterone-producing adenoma(APA) with B-ultrasonography,CT scanning and MRI were 83.0%,90.7% and 72.2%,respectively. Conclusion The comprehensive imaging data are helpful in the localized diagnosis of PA.Correct preoperative qualitative diagnosis of APA is the key step for the surgical treatment for PA.

OBJECTIVETo explore the relation between the positive rates of autoantibodies against beta(1) adrenergic receptor (beta1-receptor)and (M2-receptor) with urinary albumin excretion rate (UAER) in type 2 diabetes patients with refractory hypertension.

METHODSAutoantibodies against beta(1)- and M(2)-receptor as well as autoantibodies were determined in type 2 diabetes patients with (n = 136) or without (n = 111) refractory hypertension, hypertensive patients without renal failure (n = 60) and healthy control subjects (n = 40, control) by ELISA.

RESULTSThe positive rates of the autoantibodies against beta1-receptors (44.9%) and M(2)-receptor (37.5%) in patients with type 2 diabetes with refractory hypertension were significantly higher than those in patients with type 2 diabetes without refractory hypertension (27.9% and 24.3%, respectively, all P < 0.05), in patients with hypertension without renal failure (11.7% and 15.0%, all P < 0.01) and in healthy controls (8.3% and 7.5%, all P < 0.01). In type 2 diabetes patients with refractory hypertension and renal failure (UAER > or = 200 microg/min), the positive rates of the autoantibodies against beta(1)-receptor (87.1%, 27/31) and against M(2)-receptor (67.7%, 21/31) were significantly higher than those in type 2 diabetes patients with refractory hypertension but without renal failure (UAER 20 - 199 microg /min, 46.7%, 28/60 and 41.7%, 25/60, respectively, all P < 0.05).

CONCLUSIONThe serum beta(1)- and M (2)-receptor autoantibodies are positively associated with the UAER level and suggest that these autoantibodies against beta(1) and M(2)-receptor may play important roles in the pathogenesis of the type 2 diabetes with refractory hypertension.

Aged ; Albuminuria ; etiology ; Autoantibodies ; analysis ; Diabetes Mellitus, Type 2 ; complications ; immunology ; Female ; Humans ; Hypertension ; complications ; immunology ; Male ; Middle Aged ; Receptor, Muscarinic M2 ; immunology ; Receptors, Adrenergic, beta-1 ; immunology

Astrocytoma ; genetics ; metabolism ; Brain Neoplasms ; genetics ; metabolism ; Frizzled Receptors ; genetics ; metabolism ; Glioblastoma ; genetics ; metabolism ; Glioma ; genetics ; metabolism ; Humans ; Paraffin Embedding ; RNA, Messenger ; metabolism ; Receptors, G-Protein-Coupled ; genetics ; metabolism ; Signal Transduction ; Wnt2 Protein ; genetics ; metabolism ; beta Catenin ; genetics ; metabolism

OBJECTIVETo describe the epidemiological features of cleft lip with or without cleft palate (CL +/- P) in Chinese perinatals.

METHODSFrom 1996 through 2000, hospital-based cluster sampling method was adopted for collecting data. During that period all live or still births with 28 weeks of gestation or more delivered in monitoring hospitals were assessed within 7 days after birth.

RESULTSThe birth prevalence rates of cleft lip (CL) and of cleft lip with cleft palate (CLP) were 5.03/10,000, 8.97/10,000 respectively, then the rate of CL +/- P was 14.0/10,000. The prevalence rates in urban and rural area, in male and female births were 13.28/10,000 and 15.57/10,000, 16.06/10,000 and 11.40/10,000 respectively. Significant difference was found among maternal-age-specific prevalence rates, and the highest one was observed in >or= 35 maternal age group. 87.25% of CL +/- P was isolated forms. No secular trend was found during that period. The perinatal fatality rate of CL +/- P was 19.04%, and the rate in isolated forms was 12.69%, but the rate in syndromic CL +/- P was as high as 62.60%.

CONCLUSIONSNo decline trend in prevalence rate of CL +/- P was observed during 1996 approximately 2000. Compared with prevalence rates of CL +/- P in some foreign countries, it was higher in China during same period.

Adult ; China ; epidemiology ; Cleft Lip ; epidemiology ; Cleft Palate ; epidemiology ; Female ; Humans ; Incidence ; Male ; Maternal Age ; Pregnancy ; Time Factors

OBJECTIVETo understand the trend of incidence of conjoined twins and its epidemiological characteristics in China during 1996 to 2004.

METHODSA descriptive epidemiology of conjoined twinning in China from 1996 to 2004 was investigated by using data from National Center for Birth Defects Monitoring Program (NCBDMP). Data were collected from more than 400 hospital-based units involved in the program. The monitored subjects were the hospitalized births from 28 weeks gestation to a period within 7 days after delivery.

RESULTS122 conjoined twins were found in 4,282,536 births with an incidence rate of 0. 28 per 10,000. Incidence of conjoined twins was 0.20 per 10,000 and 0.47 per 10,000 in the rural and urban areas, respectively, and the statistical significant difference (P < 0.05) was observed., Incidence of conjoined twins was for 0.17 per 10,000 and 0.36 per 10,000 in boys and girls, respectively. Conjoined twins were more common among females than males, the live-born rate was 41.32%, and the perinatal mortality was 82.6%. 53.7% conjoined twins can be diagnosed prenatally.

CONCLUSIONThe results indicate that the incidence of conjoined twins in China is higher than that in Western countries. It is necessary to improve the prenatal diagnostic techniques and management.

Adult ; China ; epidemiology ; Congenital Abnormalities ; epidemiology ; Female ; Humans ; Infant, Newborn ; Male ; Pregnancy ; Pregnancy Trimester, Second ; Pregnancy Trimester, Third ; Sampling Studies ; Twins, Conjoined

OBJECTIVETo study the epidemiological features of cleft palate (CP) in Chinese perinatals.

METHODSData from 1996 to 2000 were collected through Chinese Birth Defects Monitoring Network a hospital based congenital malformation registry system. During that period all live or still births with 28 weeks of gestation or more were assessed within 7 days after delivery.

RESULTS499 perinatals with CP were identified among 2,218,616 births, then the overall prevalence rates of CP were 2.25/10,000, rates in isolated and syndromic CP were 1.75/10,000, 0.50/10,000 respectively. The rates in urban and rural area, in male and female births were 2.27/10,000 and 2.19/10,000, 1.93/10,000 and 2.57/10,000 respectively. The highest but nonsignificant rate (2.84/10,000) was found in maternal age group of more than 35 years. An increased trend was found both in overall CP and syndromic CP during that period. Geographic variation was found among provinces. The perinatal mortality rate of CP was 14.9%, and the rate of isolated forms was 7.3%, while the rate of syndromic CP was as high as 41.4%.

CONCLUSIONThe increasing prevalence rate of syndromic CP can account for the secular trend of overall CP. Female predominance was only observed in isolated forms of CP among Chinese perinatals. Geographic variation was identified too. Due to poor birth quality, perinatals suffering from syndromic CP had poor prognosis.

Adult ; China ; epidemiology ; Cleft Palate ; epidemiology ; etiology ; Female ; Humans ; Incidence ; Male ; Pregnancy ; Sex Factors

OBJECTIVETo study the epidemiological characteristics of congenital hydrocephalus in Chinese perinatal.

METHODSFrom 1996 to 2004, data gained from Chinese Birth Defects Monitoring Network were used to depict the epidemiology of congenital hydrocephalus in China. All perinatal born in hospitals had an access within 7 days after delivery. The affected cases were divided into two groups-isolated and syndromic hydrocephalus. And prevalence rates were calculated by year, by sex, by birth area (urban versus rural), by maternal age group and by geographic area (north versus south). Of the affected, fetal age at birth, birth weight, perinatal outcome and prenatal diagnosis were analysed.

RESULTSAll 3012 perinatal with congenital hydrocephalus were identified among 4,282,536 births, then an overall prevalence rate was 7.03/10,000, rates of isolated and syndromic hydrocephalus were 5.67/10,000 and 1.36/10,000 respectively. Furthermore, the annual prevalence rates of hydrocephalus presented an increasing trend during that period. The rates in male and female births, in urban and rural area, were 7.09/10,000 and 6.76/10,000, 5.49/10,000 and 10.10/10,000 respectively. There were significant differences among maternal-age-specific prevalence rates, the highest (11.42/10,000) was in an age < 20 years group. For total and isolated hydrocephalus, higher rates were found in north part of China. On the contrary, a higher rate of syndromic hydrocephalus was observed in south part of China. Among the infants with hydrocephalus, the ratio of preterm delivery and of low birth weight were 57.97% and 50.92% respectively. The ratio of congenital hydrocephalus diagnosed antenatally, which could be an indicator representing the capability of detecting the malformation both prenatally and postnatally, showed an upward trend similar to the prevalence rates. The perinatal fatality rates of the total, isolated and syndromic hydrocephalus were 87.75%, 88.66% and 83.91% correspondingly.

CONCLUSIONBased on comparison between prevalence rates in China and those reported in foreign countries, our country might be listed into a higher epidemic region of the congenital hydrocephalus. Significant differences were identified between rural and urban areas, between north and south parts of China. The improvement ability in prenatal and postnatal diagnosis should be partly accounted for the increasing prevalence rates of hydrocephalus in Chinese perinatal. The poor birth quality of the affected predicts poor prognosis.

China ; epidemiology ; Congenital Abnormalities ; epidemiology ; Female ; Fetal Death ; Humans ; Hydrocephalus ; epidemiology ; Infant, Newborn ; Male ; Pregnancy ; Pregnancy Trimester, Second ; Pregnancy Trimester, Third ; Prevalence ; Rural Population ; Urban Population

OBJECTIVETo understand the trend of incidence of omphalocele and its epidemiological characteristics in China during 1996 to 2000.

METHODSSurveillance data of omphalocele were collected from 460 hospitals at county level or above county level, involving all births with 28-week gestation to 7 days after delivery, including live births, fetal deaths and stillbirths in 31 provinces, autonomous regions and municipalities all over the country during 1996 to 2000.

RESULTSOverall incidence of omphalocele in China was 1.52 per 10 000 live births, with an increasing trend during 1996 to 2000. Incidence of omphalocele was 1.40 per 10 000 and 1.83 per 10 000 in the rural and urban areas, respectively, with a statistically significant difference (P < 0.05), and 1.54 per 10 000 and 1.41 per 10 000 in boys and girls, respectively. Babies of omphalocele associated with other malformation accounted for 30.77% of the total cases. Perinatal fatality rate of omphalocele was 51.18%, with prenatal diagnostic rate of 31.07%.

CONCLUSIONSPrevalence of omphalocele appeared an increasing trend in China during the period from 1996 to 2000. Occurrence of omphalocele was more frequent in rural areas than that in urban areas. Perinatal fatality rate in babies with omphalocele was higher and fatality of associated omphalocele was higher than that of simple one. It is suggested that management of perinatal care and level of prenatal diagnosis for omphalocele should be improved.

China ; epidemiology ; Female ; Hernia, Umbilical ; epidemiology ; Humans ; Incidence ; Infant, Newborn ; Male ; Rural Health

Objective To investigate the incidence and its secular trends of gastroschisis in Chinese perinatal infants.Methods Data on perinatal infants was collected at hospitals under surveillance program in Chinese Birth Defects Monitoring Network from 1996 to 2007.Data on incidence,trend and related factors of gastroschisis in perinatal infants were carried out.Both x2 test and Poisson regression model were used to test the differences between residential areas,sex and maternal age.Both x2 trends test and Poisson regression model were applied to analyze the trends.Results A total of 6 308 594 perinatal infants were monitored during 1996-2007,including 1601 infants with gastroschisis to show the incidence as 2.54 per 10 000 births.The overall prevalence of gastroschisis in China did not change remarkably during the period of our research.The incidence rates of gastroschisis were significantly different between urban and rural areas,between different sex and different maternal age groups.The incidence of gastroschisis was lower in urban area than in rural area (RR=0.58) and lower in female fetuses than in male fetuses (RR=0.76),highest in the group younger than 20 years of age,which was 11.43 times than incidence of the 30-34 age group (RR=11.432).Conclusion The overall prevalence of gastroschisis in China did not show remarkable change during 1996-2007 but the incidence of gastroschisis a bit increased in the area of study and significant differences were seen in different sex,regions and maternal age groups.Mothers aged younger than 20 years old appeared to be a significant risk factor for the occurrence of gastroschisis.