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Objective:To investigate the rate and timing of antenatal corticosteroid administration in the singleton preterm infants in our hospital, and explore the relationship between the timing of antenatal corticosteroid administration associated with the causes of preterm delivery and with neonatal outcomes.Methods:The study was a retrospective chart review of clinical data regarding singleton preterm neonates and their mothers from January 2016 and June 2020 at Peking University International Hospital.Optimal administration timing was defined as the first dose of antenatal dexamethasone given ≥48 h and ≤7 d before delivery.Suboptimal administration timing included any antenatal dexamethasone timing(<48 h or >7 d) that did not meet the optimal criteria.Antenatal dexamethasone administration timings were compared among preterm delivery with different causes.The neonatal outcomes of the optimal and suboptimal administration timing groups were compared.Results:The percentage of antenatal dexamethasone use was 89.16%, with 51.35% receiving optimal dexamethasone.Women with premature rupture of membranes were most likely to receive optimal dexamethasone(63.79%), followed by women with complications of pregnancy and other disorders(54.29%). The optimal dexamethasone rate of the women with cervical incompetence and preterm labor was relatively low(20% and 28%, respectively). The incidence of respiratory distress syndrome(RDS) of optimal administration timing group was lower than that in suboptimal administration timing group among neonates at <34 weeks of gestation( P<0.05). But there was no significant difference in the incidence of severe RDS, bronchopulmonary dysplasia, and need for pulmonary surfactant between two groups( P>0.05). The preterm infants with a gestational age between 34 and 34 + 6 weeks had no severe RDS or bronchopulmonary dysplasia.Compared with suboptimal administration timing group, the incidence of RDS and need for pulmonary surfactant of optimal administration timing group did not decrease significantly( P>0.05). Conclusion:The causes of preterm delivery affect the timing of antenatal dexamethasone administration.Optimizing the timing of antenatal dexamethasone administration can reduce the incidence of RDS among neonates less than 34 weeks of gestation.
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Objective To explore the clinical features and gene mutations of blepharophimosis-ptosis-intellectual-disability syndrome (BPID). Methods The clinical data, diagnosis and treatment of a child with BPID in neonatal intensive care unit (NICU) were reviewed. Based on the literature retrieved from PubMed database, the common classification, clinical features, diagnosis and genetic counseling of BPID and its affiliated blepharophimosis-mental retardation syndromes (BMR) were reviewed. Results This male infant was 39 weeks of gestational age with birth weight of 1920 g, and was admitted to NICU 15 min after birth due to dyspnea. The main clinical manifestations were facial deformity such as biepharophimosis, ptosis and micromandible, inspiratory dyspnea with laryngeal cartilage softening, malformations of the thorax and feeding difficulties. A heterozygous mutation in UBE3B gene was identified by complete exon sequencing and he was diagnosed of BPID, a rare genetic disorder. Reviewing the literature, there was no relevant report in domestic. While one foreign literature was found to report 5 patients from 4 families having a subtype of BMR, a kind of autosomal recessive diseases caused by mutations in the UBE3B gene. Conclusion BPID is a rare clinical entity of BMR. Complete exon sequencing can be used to diagnose the disease.
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OBJECTIVE: To explore different doses of sodium(s)-2-(dithiocarboxylato((2R,3R,4R,5R,6R)-2,3,4,5,6-pentahydroxyhexyl) amino)-4-(methylthio) butanoate(GMDTC) for removing cadmium. METHODS: Thirty-five male New Zealand rabbits were randomly divided into blank control group,GMDTC high dose control group,model control group,ethylene diamine tetraacetic acid(EDTA) control group and GMDTC low,medium and high dose groups,five rabbits in each group. The blank control group and GMDTC high dose control group were given 0. 90% normal saline solution intravenously; model control group,EDTA control group and GMDTC low,medium and high dose group were given 2 μmol/kg of cadmium chloride(CdCl_2) and 40 μmol/kg of β-mercaptoethanol mixed solution intravenously,5. 0mL/kg body weight(bw),once a day for five days. On the forty-one day of the experiment(the fist day of GMDTC treatment),the control group and the model control group were injected 0. 90% normal saline solution 250 mL via ear vein,the EDTA control group was given EDTA solution at the dose of 93. 5 mg/kg bw with 250 mL 0. 90% normal saline solution,also via ear vein; the GMDTC high dose control group,and the GMDTC low,medium and high dose groups were given 250 mL GMDTC solution at the concentration of 108.0,12.0,36.0 and 108. 0 mg/kg bw with 0. 90% normal saline by intravenous infusion,once a day,6 times a week for four consecutive weeks. The urine β_2-microglobulin(MG),renal cadmium,blood cadmium,and urinary cadmium before and after the treatment were detected. RESULTS: The body weight of New Zealand rabbits increased with the increasing feed time(P < 0. 01). The levels of β_2-MG before treatment increased in model control group,EDTA control group,GMDTC low,medium and high dose groups than that in the blank control group(P < 0. 01). The levels of renal cadmium after treatment in GMDTC medium and high dose groups decreased compared with those in the blank control group and EDTA control group respectively(P < 0. 05). The blood cadmium after treatment in EDTA control group,GMDTC low,medium and high dose groups were decreased compared with those before treatment in the same group respectively(P < 0. 05),meanwhile decreased than the blood cadmium after treatment in the model control group respectively(P < 0. 05). The blood cadmium after treatment had not a statistically significant difference among the EDTA control group,GMDTC medium and high dose groups(P < 0. 05). At all the time points(1,6,8,13,15,20,22 and 28 days after treatment),the urinary cadmium after treatment in EDTA control group and the three GMDTC dose groups increased compared to the model control group at the same time(P < 0. 05). The urinary cadmium after treatment increased with GMDTC dose increased at the other six time points,expect on 20 and 22 days after treatment(P < 0. 05). The blood cadmium removal rates after treatment were 70. 06%,74. 86% and 78. 05% and the renal cadmium removal rates were 14. 27%,27. 95% and 61. 24% in GMDTC low,medium and high dose groups,respectively. CONCLUSION: The intravenous infusion of GMDTC at the dose of 108. 0 mg/kg bw effectively removed cadmium in cadmium poisoning rabbit. This dose had no obvious toxic effect and was equivalent to human dose of 36. 0mg/kg bw which meets the requirement of new drug property.