Objective To assess the efficacy of drug therapy for Henoch-Schonlein purpura nephritis (HSPN) in children. Methods Medical electronic databases and other sources were searched without language restriction. According to including and excluding cretria,articles were evaluated ( Shekelle PG, et al). Randomized control trials (RCTs) were assessed according to the Juni assessment. Software Rev Man 4.2 was used to assess control trials, and other studies were reviewed and describe. Results Sixty-four papers were included out of 1948 papers. There were 1 article in grade Ⅰa, 6 in grade Ⅰb, 10 in grade Ⅱa, 34 in grade Ⅱb, and 13 in grade Ⅲ. We assessed the efficacy of steroids combined with immunosuppressants (76 cases) vs steroids alone (87 cases) by analyzing 1 RCT (Ⅰb) and 4 control trials (Ⅱa). There were significant differences in cure rate (RD=-0.39, 95% CI: -0.53－-0.25) and effective power (RD＝-0.43, 95％CI: -0.66－-0.20) The efficacies of steroids combined with cyclophosphamide (CTX) pulse therapy (57 cases) vs steroids alone (49 cases) were revealed significant differences in cure rate (RD=-0.48, 95% CI:-0.74－-0.23) and effective power (RD=-0.60, 95% CI: -0.81－-0.40). CTX used alone was not effective compared to supported therapy from a RCT. Steroids combined with other drugs were confined to non-randomized control trials, so efficacy was difficult to evaluate. Conclusions Drug therapy for HSPN in children is still lack of consistent and common regimens. Steroids combined with immunosuppressants were effective for severe HSPN in children, in which steroids combined with CTX were the most effective; CTX used alone was not effective compared to supported therapy. Nevertheless, multicentre and large-scale RCTs are still needed. In addition, other drug regimens were limited to non-randomized control trials and difficult to evaluate.
Objective Group A rotavirus is the most important pathogen leading to dehydrating diarrhea in young children in worldwide scope. This study aimed to study the epidemiology of rotaviruses in hospitalized children in Shanghai, China during the years 2001－2005. The information will provide important data and theoretical bases for study and production of rotavirus vaccine. Methods Systematic sampling was applied for collection of 363 fecal samples from hospitalized children with rotavirus diarrhea in children's hospital, Fudan University. ELISA was used to detect rotavirus in stool samples, and then nested RT-PCR was applied for determination of rotavirus genotypes. Result The largest proportion of children with rotavirus diarrhea was in the 6－11 month age group, followed by the 12－23 months age group(29.8% and 26.7% respectively). 79.9% of children with rotavirus diarrhea were younger than 2 years of age. 97 rotavirus positive samples were detected in November, accounting for 26.7%, the other predominant months were October(49 samples, 13.1%) and December(54 samples, 14.9%). 62.3% of children with rotavirus diarrhea were detected during October to January of the next year. Among G genotypes of rotavirus in 2001, G3(47.1%) was the most predominant, followed by G1 at 28.8%, G4 at 8.0% and G2 at 6.9%. In the year of 2002, G3 was accounted for 62.0%, followed by G1(21.0%) and type frequency was 5.0% for G2 and G4 together. G3 increased to 74.6% in 2003 and G1 was the secondly prevailing type but decreased to 18.6%. All the other samples in 2003 could not be typed. During the year of 2004, G3 type continued to increase to 85% and G1 dropped to 1.7%. G3 accounted for 87.7% in 2005 but G1 turned up to 7%. Several mixed infection of G types were detected in 2001, 2002 and 2005 such as combination of any two types of G1, G2 and G3. Only one G9 was detected in 2001 in all the 363 samples. The dominant P type in 2001 was P［8］(79.3%), followed by P［4］(6.9%) and P［6］(2.3%). For the year of 2002, P［8］ was accounted for 52%, followed by P［4］ (11.0%). 19.0% of the samples contained mixed types of P［4］ and P［8］, and 6% were mixed infection of P［4］, P［6］ and P［8］. P［8］ was still the most dominant P type in 2003 with high frequency of 91.5% and all the others were mixed types of P［4］ and P［8］. 81. 2% of samples in 2004 were P［8］ and the others could not be typed. During 2005, P［8］ was accounted for 87.7% and 3.5% of P［6］ was found. 4 of P［9］ were only detected in 2001. The most predominant combination of genotype was P［8］G3 from 2001 to 2005, with high frequency exceeding 50% each year except frequency of 36.0% in 2002. Other common stains included P［8］G1in 2001 and 2004, accounting for 38.6% and 18.6% respectively. Combination of G3 and mixed infection of P types in 2002 and combination of G3 and untypable P types in both 2004 and 2005 were also found. Other minor genotypes included P［8］G4 (12.2%) in 2001 and combination of P［8］ and untypable G types. Conclusions The characterization of rotavirus genotypes demonstrated some circulating characteristics from year to year in Shanghai district in recent 5 years. G3 and P［8］ and combination of P［8］G3 were the most predominant circulating genotypes. Some other common strains and unknown strains were also present in different years.
Objective To analyze the psychometric character of Symbolic Play Test(SPT): reliability, validity and item difficulty. Methods 484 childern (33.4±12.3，13-72 months) participated in the study, of which 30 (25.4±7.1，15-38 months)were normal children, 138(30.1±11.3，13-63 months) were children with cerebral palsy, and 316(36.2±12.2，15-72 months) were children with mental retardation or language delay. Internal reliability was analyzed in the sample of total 484 children.12 children were included for test-retest reliability study with a interval of 4 to 11 days.18 children were sampled for interscorer reliability analysis(among three scorers). Parallel validity for SPT was determined by using Bayley Scales of Infant Development-Ⅱ( BSID-Ⅱ) in 30 normal children and Reynell Developmental Language Scales Ⅲ (RDLSⅢ) in 87 children. Construct validity of SPT was confirmed by comparing the differences of SPT equivalent age in three groups(normal children, children with cerebral palsy and children with mental retardation or language delay) with the same chronological age level. Item difficulty order of 24 items in SPT was ascertained in the total 484 sample group by Rasch analysis. Also the item difficulty order of our study was compared with that of the English second edition. Results SPT had good internal reliability (Cronbach α= 0.897 2) as well as good test-retest (ICC=0.944 9，95%CI：0.808 6-0.984 1) and interscorer (ICC=0.997 2，95%CI： 0.992 6-0.999 0)reliability. Significant correlations were found between SPT raw score and BSID-Ⅱmental and psychomotor raw scores(r=0.755 4, 0.718 5，respectively ). There also existed good correlation between SPT score and RDLS-Ⅲ score(r=0.765 0). 30 children aging from 15 to 38 months were randomly selected from group of cerebral palsy and group of mental retardation or language delay respectively. Comparing with the normal children group, there was no difference in chronological age among three groups. But SPT equivalent ages were significantly delayed in groups of cerebral palsy and mental retardation or language delay than in normal children group. Good consistency (Spearman r=0.9240) was found between the item difficulty order of our study and that of the English second edition. Conclusions Symbolic Play Test has a satisfactory psychometric character, with good internal reliability, test-retest reliability, interscorer reliability, parallel validity, Construct validity and stable item difficulty order. SPT can be utilized to assess the intellectual ability and language potential in young children.
Objective To provide reliable methods for the gene diagnosis of spinal muscular atrophy(SMA) patients. Methods Exon 7 and 8 of SMN gene were tested by PCR-RFLP and PCR-SSCP in 30 patients who were clinically diagnosed as SMA and in their parents. 50 normal children were tested as control. Results In PCR-RFLP,Two segments (188 bp,164 bp ) in normal children and one segment (164 bp) in SMA patients when exon 7 was detected; And two segments (188 bp,125 bp) in normal children and one segment (125 bp) who were found when exon 8 was detected.In PCR-SSCP the bands denoted SMN1 exon7 and exon8 missed in SMA patients. SMN1 gene exon7 and 8 are both missed in 22 of 30 patients, only exon 7 missed in 4 patients. No missing of exon7 and/or exon8 was found in the left 4 patients. Nobody was showed exon8 missing only. 16 SMAⅠ patients(94.1%) and 10 SMAⅡpatients(100%) were found SMN1 gene missing. Abnormal bands were found in two of the three SMAⅢ patients who did not lose SMN1 gene exon 7or 8 . One SMA patient's father missed SMN1 gene exon 7or 8 homozygously. No one lost SMN1 gene exon 7or 8 in 50 normal children， but one child missed SMN2 gene exon7 and 8. We found that PCR-RFLP and PCR-SSCP had the same results in testing deletion of SMN1 exon7 and exon 8. And in two SMA patients without deletion of SMN1 gene exon 7or 8 by PCR-RFLP，we found abnormal bands in their parents by PCR-SSCP. Conclusions The test of missing of SMN1 exon 7 and 8 is maybe helpful to early diagnosis, prenatal diagnosis, differentiate diagnosis and exact diagnosis. Both PCR-RFLP and PCR-SSCP are useful assistant ways in diagnosis of SMA more exactly. Gene diagnosis of SMA by testing absence of SMN E7 and E8 is convenient,and can be accepted by the parents.
Objective To evaluate the preliminary value of realtime three dimensional echocardiography (RT3DE) in diagnosing congenital noncompaction of ventricular myocardium (NCVM) in children. Methods Four cases of NCVM diagnosed by two dimensional echocardiography in Children′s Hospital of Fudan University from July 2006 to December 2006 were included. RT3DE was used to observe the structure of trabecula and recess and calculate the ejection fraction of left ventricle. Left ventricular synchronicity was assessed by the parameters from time-volume curve. Results ① NCVM morphology observed by RT3DE: According to Van Praagh sequential analysis method, all of the four cases were single NCVM without combining cardiac anomaly. Four-chamber volumetric view showed left ventricle was involved entirely in one case while the middle part and apex of left ventricle were implicated in the other three cases. But in no case right ventricle was involved. ② Quantitative diagnosis of NCVM by RT3DE: From ventricular short axis volumetric view, the non-compacted myocardial layers at the end of systole (X′s) were 1.0-2.0 cm, while the compacted myocardial layers (Y′s) were 0.3-0.4 cm in length. So the ratio of non-compacted to compacted myocardial layers at the end of systole (X′s/Y′s) was 3.3-5.0. The non-compacted myocardial layers at the end of diastole (X′d) were 1.1-2.2 cm, while the compacted myocardial layers (Y′d) were 0.4-0.5 cm. So the ratio at the end of diastole (X′d/Y′d) was 2.6-4.4. Whether at the end of systole or at the end of diastole, the ratioes of non-compacted to compacted myocardial layers were all more than 2. ③ Left ventricular systolic function of NCVM assessed by RT3DE: The left ventricular diastolic volume was 15.1-44.0 mL, while the systolic volume was 7.8-22.8 mL. So the left ventricular ejection fraction was 21.2%-56.4%. Cardiac function of all cases was compromised in different degrees. ④ Left ventricular synchronicity of NCVM assessed by RT3DE: Percentage ratio of Tmsv standard deviation in 16 cardiac segments among cardiac cycle was 2.65%-19.30%. Percentage ratio of Tmsv maximum difference in 16 cardiac segments among cardiac cycle was 8.75%-78.20%. Synchronical parameters was increased in different degrees, indicating systolic movement was not so harmonic in NCVM cases. Conclusions RT3DE was a useful tool in observing the compromised range of NCVM accurately. Moreover, the degree of gravity, cardiac function and ventricular synchronicity were evaluated precisely by RT3DE.
Objective To explore the application of pulse transit time (PTT) in evaluating obstructive sleep apnea hypopnea syndrome(OSAHS) in children and to introduce the principle and manipulation of PTT. PTT is the time the pulse wave (PW) travells between two arterial sites (normally heart to finger). Methods One hundred and twenty-five children suspected of OSAHS underwent All-night PTT test. The following parameters were measured: chest and abdominal wall movement by respiratory inductance plethysmography, heart rate by electrocardiogram, PTT and arterial oxygen saturation were recorded using ECG and pulse oximetry. Nasal airflow was measured by nasal catheter and thermistor. The apnea events and hypopnea events were determined by nasal/mouth airflow. The obstructive and central events were differentiated by combining the changes of PTT and nasal/mouth airflow. Then PTT values were recorded and analyzed with a statistical software program (Version 11.0; SPSS). Results All of the 125 children (age range, 2 to 12 years; mean age5 years and 8 months old; 85 male children, 40 female children) underwent PTT monitoring. The results were as followings: The mean maximum obstructive apnea duration was (32.02±20.80)s ［male （30.47±21.97）s ,female （34.26±18.22）s respectively］. The mean maximum central apnea duration was （14.45±6.55）s［male （14.34±7.59）s, female （14.78±4.54）s, respectively］. The mean hypopnea duration was （57.04±25.22）s［male （53.80±21.95）s, female （62.2±30.68）s, respectively］. The obstructive apnea index (OAI) was 6.00±6.44（male 5.44±7.37, female 7.33±3.93, respectively）,central apnea index (CAI) was 0.85±1.00（male 0.79±1.07, female 0.98±0.87, respectively）, hypopnea index (HI) was 5.68±6.04（male 5.72±5.0, female 5.60 ±7.84, respectively）, saO2 nadir was 83.26±12.88% ［male （81.97±12.93）%，female （85.58±15.87）% , respectively］, arousal index was 26.39±12.46（male 28.09±13.58, female 22.75±9.29, respectively）.The causes of sleeprelated breathing disorder included upper airway resistance syndrome (UARS) in 13 cases（10.4％，13/125）, mild OSAHS in 41 cases（32.8％，41/125）, moderate OASHS in 47 cases（37.6％，47/125）, severe OSAHS in 22 cases（17.6％，22/125）, normal in 2case（1.6％，2/125）. No significant difference was found for maximum apnea duration, maximum hypopnea duration, AI, HI, saO2 nadir values in boys and girls. Conclusions PTT should be a useful method for diagnosis of OSAHS, it could distinguish the central from obstructive apnea events. PTT could be clinically applied in polysomnographic studies in children suspected of OSAHS.
Objective To explore:① Role of various dosage ectogenic anti- TGF-β1 in hyperoxic lung fibrosis in neonatal rats.② Effect of the expression level of TGF-β1 on hyperoxic lung fibrosis of rats treated with various dosage ectogenic anti- TGF-β1. Methods 120 Sprague-Dawley(SD) rats younger than 12 hours old were enrolled in this study. The neonatal rats were divided into 6 groups randomly: normal concentration O2 control group(group Ⅰ);>95%O2 control group(groupⅡ); >95%O2+ anti- TGF-β1 0.1mg·kg-1 (groupⅢ); >95%O2+ anti- TGF-β1 0.2 mg·kg-1 (group Ⅳ); >95%O2+ anti- TGF-β1 0.4 mg·kg-1 (groupⅤ); >95%O2+ anti- TGF-β1 0.8mg·kg-1 (group Ⅵ). GroupⅠwas placed in the common room . Group Ⅱ,Ⅲ，Ⅳ，Ⅴand Ⅵ were placed into oxygen cabins and exposed to >95% O2 for 3 days，at same time，neonatal rats received nebulization therapy with various dosage anti- TGF-β1 once a day 3 days,7 days,14 days and 28 days after exposure to high concentration oxygen(O2>95%), the changes of the pathologic alteration in lung were measured，and the expression levels of TGF-β1 were measured by immunohistochemistry. Results On day 3 and day 7, the lungs of group Ⅱ,Ⅲ，Ⅳ，Ⅴand Ⅵ neonatal rats showed acute inflammations. Microvessel hyperemia, edema,inflammatory cell infiltration, hemorrhage were found by using light microscope and transmission electron microscope but the expression levels of TGF-β1 were obviously increased in the lungs of group Ⅱ,Ⅲand Ⅳ rats. On day 14, edema,inflammatory cell infiltration,hemorrhage were reduced while marked fibrosis changes were seen in lung, the expression levels of TGF-β1 were hightest; On day 28 there were obvious inflammations and expanded alveolus alternations and strong express of TGF-β1 in groupⅡ, Ⅲ and Ⅳ than other groups. There was no obviously inflammation and expression of TGF-β1 in group Ⅰ,There was no significant fibrosis on day 14 and day 28 in group Ⅴ and Ⅵ comparing with the control,the expression level of TGF-β1 was not increased on day 14 and day 28 in group Ⅴ,and no ECM and lung fibrosis were found by using light microscope and transmission electron microscope. Conclusions Hyperoxia could cause acute or chronic lung injury. Hyperoxia could obviously increase the expression level of TGF-β1 and cause lung fibrosis, which suggested TGF-β1 has a key role in fibrosis. Anti- TGF-β1 could inhibit the ALI and lung fibrosis. Anti- TGF-β1 could protect against lung fibrosis and exceptional alveolus development, which suggested Anti-TGF-β1 may have a key role in hyperoxic-induced lung fiborsis.
【abstract】The clinical findings and the imaging features of 2 cases with asphyxiating thoracic dysplasia were described.Combining with literature, pathogenesis、diagonosis、differential diagnosis、the progresss of treatment and progonosis were also described .
