ABSTRACT
Objective To study the clinical characteristics,treatment and prognosis of neonatal VACTERL association.Method The clinical data of newborns diagnosed with VACTERL association from January 2010 to December 2015 were collected and retrospectively analyzed.Result A total of 33 patients diagnosed with VACTERL association were included,including 23 males and 10 females.Among them,17 cases were term infants,15 cases premature infants and 1 case of overdue birth,with an admission age of 1 to 24 days.The most common deformities were cardiac anomalies (C) in 27 cases (81.8％),followed by anal atresia/anorectal malformation (A) in 25 cases (75.8％),renal deformity (R) in 24 cases (72.7％),limb abnormalities (L) in 20 cases (60.6％),Tracheoesophageal fistula (TEF) in 8 cases (24.2％) and vertebral abnormalities (V) in 3 cases (9.1％).11 cases (33.3％) had other deformities.Among these 33 patients,24 cases had 3 types of malformations and 9 cases had 4 types of malformations.The most common combination was ACR (n =8).20 patients had no abnormalites on chromosome karyotype test including 2 patients had normal gene microarray results.16 patients received surgical treatment during neonatal period and 13 of them recovered and discharged.Among the other 17 cases received no surgery,only 1 patient improved and discharged.A telephone follow-up was proceeded in 14 discharged cases at 1 year old.Among them,13 cases had good prognosis,however,the remaining one was dead.Conclusion VACTERL association is a rare non-random combination of multiple malformations.The early discovery and appropriately treatment after diagnosis will improve the prognosis and prevent death.Doctors should reinforce the ability to detect various types of deformities and examine the chromosome and gene properly.
ABSTRACT
Objective To investigate the effect of di-(2-ethylhexyl) phthalate (DEHP) on the postnatal lung development in newborn rats.Methods A total of 60 newborn Sprague-Dawley rats (weighing 5.0-8.0 g) in five age groups were studied in the first experiment.The rats were divided based on the different postnatal ages:postnatal day (PND)I,PND4,PND7 and PND14.A total of 45 newborn Sprague-Dawley rats (weighing 5.0-8.0 g) were randomly divided into three groups according to the dosage of DEHP administered in the second experiment.The newborn rats were administered DEHP through intraperitoneal injection at 10 (low-dose subgroup),100 (medium-dose subgroup) or 750 (high-dose subgroup) mg/kg daily from PND1 to PND13.The rats were sacrificed on PND14.Pups were sacrificed with lethal dose injection of pentobarbital sodium.The lung was removed.The right middle lobes were used for analysis.The tissue was processed for histology and lung sections were stained with HE for light microscopic (LM) morphometric measurement.The analysis was performed by means of a digital image analysis system,including pulmonary interstitial area ratio (IAR) and total length density of all segments.One-way ANOVA,LSD and Dunnet T3 methods were used for statistical analysis.Results In the normal controls,IAR decreased significantly by (31.97±5.03) ％,(30.05±3.57)％,(25.33± 1.83)％ and(22.01 ±2.19)％,respectively,from PND1 to PND14 (P＜0.05 or P＜0.01).IAR in medium-and high-dose subgroups increased significantly by (24.11 ±2.78)％ and (26.53± 3.42)％,respectively on PND 14.The total length density of all segments in unit area lung volume increased significantly by 0.047 8±0.003 7,0.050 0±0.002 9,0.071 2±0.003 0 and 0.084 4±0.004 3,respectively from PND1 to PND14 (P＜0.01).In the DEHP treated animals,when compared with the control group,IAR was significantly higher on PND14 (P＜0.05 or P＜0.01),while the total length density of all segments in unit area lung volume was significantly decreased (P＜0.05 or P＜0.01).Length density in medium-and high-dose subgroups were higher than that of low-dose subgroup by 0.082 9±0.001 8,0.077 2±0.002 0 and 0.071 3±0.003 7,respectively on PND14 (P＜0.05 or P＜0.01).Conclusions Medium-and high-dose DEHP affect the postnatal lung development in rats in a dose-dependent mode.