Treatment and prognosis of severe hyperbilirubinemia in full-term infants meeting exchange transfusion criteria: a multicenter retrospective study / 中华围产医学杂志

Objective:To investigate the prognosis of severe hyperbilirubinemia in full-term infants who met the exchange transfusion criteria and were treated by blood exchange transfusion and phototherapy.Methods:A total of 168 full-term infants with severe hyperbilirubinemia who met the criteria for exchange transfusion and were hospitalized in the Neonatology Department of seven tertiary hospitals in Hebei Province from June 2017 to December 2018 were retrospectively included. According to the treatment protocol, they were divided into two groups: exchange transfusion group (38 cases) and phototherapy group (130 cases). Two independent sample t-test and Chi-square test were used to compare the clinical manifestations and follow-up results between the two groups. Multivariate logistic regression was used to analyze the risk factors for poor prognosis. Results:Neonatal severe hyperbilirubinemia in the exchange transfusion and phototherapy group were both mainly caused by hemolytic disease [42.1%(16/38) and 29.2%(38/130)], sepsis [28.9%(11/38) and 11.5%(15/130)] and early-onset breastfeeding jaundice [15.8%(6/38) and 11.5%(15/130)]. Total serum bilirubin level on admission in the exchange transfusion group was significantly higher than that in the phototherapy group [(531.7±141.3) vs (440.0±67.4) μmol/L, t=3.870, P<0.001]. Moreover, the percentage of patients with mild, moderate and severe acute bilirubin encephalopathy in the exchange transfusion group were higher than those in the phototherapy group [15.8%(6/38) vs 3.8%(5/130), 7.9%(3/38) vs 0.8%(1/130), 13.2%(5/38) vs 0.0%(0/130); χ2=29.119, P<0.001]. Among the 168 patients, 135 were followed up to 18-36 months of age and 12 showed poor prognosis (developmental retardation or hearing impairment) with four in the exchange transfusion group (12.9%, 4/31) and eight in the phototherapy group (7.7%, 8/104). Multivariate logistic regression analysis showed that for full-term infants with severe hyperbilirubinemia who met the exchange transfusion criteria, phototherapy alone without blood exchange transfusion as well as severe ABE were risk factors for poor prognosis ( OR=14.407, 95% CI: 1.101-88.528, P=0.042; OR=16.561, 95% CI: 4.042-67.850, P<0.001). Conclusions:Full-term infants who have severe hyperbilirubinemia and meet the exchange transfusion criteria should be actively treated with blood exchange transfusion, especially for those with severe ABE, so as to improve the prognosis.

Influence of combined drug therapy by intratracheal infusion on clinical prognosis of premature infants with severe RDS / 中国医师进修杂志

Objective To investigate the influence of combined drug therapy by intratracheal infusion on clinical prognosis of premature infants with severe respiratory distress syndrome (RDS). Methods One hundred and twenty-eight premature infants with severe RDS were chosen in the period from August 2015 to December 2017 in central hospital of Hebei Handan and randomly divided into 2 groups including control group (64 children) treated with pulmonary surfactant (PS) by intratracheal infusion and experimental group (64 children) treated with PS and budesonide by intratracheal infusion. The invasive ventilation time, withdrawal time, total hospitalization time, the levels of blood oxygen index before and after treatment and the complications incidence of two groups were compared. Results The invasive ventilation time, withdrawal time and total hospitalization time in experimental group were significantly shorter than those in control group:(3.50 ± 0.72) d vs.(4.84 ± 0.98) d, (29.52 ± 3.97) d vs. (35.21 ± 5.10) d, (45.26 ± 6.27) d vs. (53.85 ± 8.04) d, and there were significant differences (P<0.05). The levels of blood oxygen index after treatment of experimental group were significantly better than those of control group (P < 0.05). The incidence of total bronchopulmonary dysplasia(BPD), retinopathy, necrotizing enterocolitis, pulmonary hemorrhage, sepsis, intracranial hemorrhage and ventilator-associated pneumonia (VAP) in two groups had not significant differences (P>0.05). But the moderate to severe BPD in experimental group was significantly lower than that in control group: 15.63％(10/64) vs. 45.31％(29/64), P < 0.05. Conclusions Combinated drug therapy by intratracheal infusion in the treatment of premature infants with severe RDS can efficiently promote disease recovery process, improve the lung function and be helpful to prevent the moderate and severe degree BPD.

Comprehensive Quality Evaluation of Flos Farfarae from Different Habitats / 中国中医药信息杂志

Objective To compare the quality of Flos Farfarae from different habitats, and provide basis for the utilization and development of Tussilago farfara. Methods The contents of rutin and tussilagone were determinated by HPLC, the 100-bud dry weight and bud color were weighed and observed. And the data of different samples were compared and statistical analysed. Results The content of rutin, tussilagone and 100-bud dry weight in Flos Farfarae from different place has a significant difference, and there was a significant positive correlation between rutin and tussilagone. Principal component and factor analysis showed that the quality of Flos Farfarae from Yushe, Ningwu, Guangling was better than other areas. Conclusion The quality of Flos Farfarae from different areas is difference, and wild T. farfara in Yushe, Ningwu, Guangling could be used as high quality germplasm.

Chronic sublethal hypoxia: challenge to premature brain in structual and neurological development / 国际儿科学杂志

With the advance of modern neonatal management, the increase of survival of infants born with ELBW has resulted in collateral increase in incidence of infants with serious chronic lung disease, typically brnchopulmonary dysplasia (BPD). Long-term sensory, motor and cognitive impairments are common outcomes in survivals with moderate and severe BPD and may persist during school years and adolescence. Increasing evidence suggest that BPD exerts a significant effect on brain growth and development and may be associated with chronic sublethal hypoxia which compond the risk of extended brain injury and NS complications such as cerebral palsy. Animal studies have demonstrated progressive gliosis and cerebral ventriculomegaly, injured subcortical white matter and corpus callosum, dysynchrony synaptic development and disrupted neurotransmitssion in the hypoxia newborn brain. In this literature we built upon the review of neurogical and congnitive outcome in preterm infants with BPD and structural, functional and neurochemical alterations in ainimals following clinical and experimental hypoxia respectively, which may underlie the primary or potential mle for chronic sublethal hypoxia on premature brain development.

The genotype and phenotype studies of 40 Chinese patients with X-linked adrenoleukodystrophy(X-ALD) / 北京大学学报(医学版)

Obiective:To elucidate the phenotype and the genotype-phenotype correlations in Chinese patients with X-linked adrenoleukodystrophy(X-ALD).Methods:Clinical features of 40 Chinese patients with X-ALD were studied and mutation spectrums were investigated by polymerase chain reaction (PCR) and sequencing. Results:Among these patients, four were siblings from two unrelated families, the others were unrelated. There were 31 cases with childhood cerebral (CCALD), 8 cases with adolescent cerebral (ACALD) and 1 case with adrenomyeloneuropathy (AMN). Visual impairment, which presented in 12 cases (30%), was the most common initial symptom. Nine (69%) of 13 cases who had hydrocortisone and ACTH measured showed adrenal insufficiency. By follow-up date, 19 cases (47.5%) were dead. The interval from onset to death varied from 1 to 6 years and the average were 3.3 years. The mean age at death was 10.5 years. Eleven cases (27.5%) were in vegetable state. The mean interval from onset to apparently vegetable state was 2.8 years (range from 1 to 6 years). Four cases had progressive neurological disability. Four cases were lost follow-up. One case with CCALD and one case with ACALD progressed slowly. The courses of the disease of these two patients were 5 years and 15 years respectively. Thirty five mutations were identified in 40 cases. Most were located within exon 1-3 (40%, 16/40) and exon 6-8 (42%, 17/40). There is a distinct clustering of missense mutations in exon 6 (17%, 7/40). Five types of mutations were associated with CCALD, three with ACALD and a missense mutation was identified in the patients with AMN. The two patients with long disease courses had a missence mutation c.1559 T>A and a nonsense mutation c.1785 G>A respectively. The siblings with similar manifestations and onset age were observed in two families, whose mutations were c.887 A>G and c.1028 G>T. Conclusion:The phenotypes, disease severity and rate of neurodegeneration could not be predicted by the nature of mutations.