Yang et al reported an immunocompetent infant with gastrointestinal cytomegalovirus disease secondary to measles infection.We express our opinion about the diagnosis and treatment of this rare disease.
Free radicals (FRs) generation is an unavoidable consequence of the life in an oxygen-rich atmosphere. FRs can be considered a double-edged sword. Beneficial effects of FRs occur at moderate concentrations and involve...Free radicals (FRs) generation is an unavoidable consequence of the life in an oxygen-rich atmosphere. FRs can be considered a double-edged sword. Beneficial effects of FRs occur at moderate concentrations and involve physiological roles in cellular responses to noxia, as in defense against infectious agents, in the function of a number of cellular signaling pathways and the induction of a mitogenic response. The over-production of FRs and the insufficiency of an antioxidant mechanism result in oxidative stress (OS), a deleterious process and important mediator of damage to cell structures and tissues. It occurs at birth in all newborns as a consequence of the hyperoxic challenge after the transition from the hypoxic intrauterine environment to extrauterine life. During the perinatal period, OS can be magnified by others predisposing conditions such as hyperoxia, hypoxia, ischemia, hypoxia-reperfusion, inflammation and high levels of non-protein bound iron. Epidemiological studies linked OS occurring during fetal stages and early infancy with adverse health outcomes later in life, indicating that OS is an early event in the etiology of these chronic diseases. Newborns, especially if preterm, are particularly susceptible to OS and damage due to the increased generation of FRs, the lack of adequate antioxidant protection, and the inability to induce antioxidant defenses during the hyperoxic challenge at birth. This impairment of the oxidative balance has been thought to be the common factor of pathologies grouped together as “free radical disease in the neonate” that include retinopathy of prematurity (which may lead to blindness in severe cases), bronchopulmonary dysplasia (a particularly debilitating pulmonary lesion of the preterm infant), periventricular leukomalacia (an important cause of severe neurodisability) and necrotizing enterocolitis. In this review we discuss in detail these perinatal diseases. Particularly, we analyze the current knowledge about the role of OS in their pathogenesis.展开更多
Parenteral nutrition associated liver disease (PNALD) is a significant complication in infants receiving long-term parenteral nutrition (PN). Chronic administration of PN has been associated with its development. Our ...Parenteral nutrition associated liver disease (PNALD) is a significant complication in infants receiving long-term parenteral nutrition (PN). Chronic administration of PN has been associated with its development. Our purpose is to characterize our incidence of PNALD over an extended period and identify risk factors for its development, including administration of soybean-based injectable lipid emulsions (ILEs) as we transit to novel ILEs</span><span style="font-family:Verdana;"> in our practice</span><span style="font-family:Verdana;">. Infants receiving 30 days or more of PN were included. PNALD was defined as a direct bilirubin ≥ 2 mg/dL. Data collected included: patient demographics, clinical and enteral feeding characteristics. Macronutrient intake was recorded using these cut-offs: glucose infusion rate (GIR) of ≤14 mg/kg/min or above, protein doses of ≤3 g/kg/day or above and lipid doses of ≤2 g/kg/day or above.</span><span style="font-family:""> </span><span style="font-family:Verdana;">A total of 349 infants were included, with an annual incidence of PNALD ranging between 34</span><span style="font-family:Verdana;">% </span><span style="font-family:Verdana;">-</span><span style="font-family:""> </span><span style="font-family:Verdana;">54%. Infants with PNALD were younger by gestation (27 vs. 29.5 weeks) and smaller by birthweight (900 vs. 1248 grams). Sepsis, GI disease including necrotizing enterocolitis and bowel resection were significantly associated with an increased risk for development of PNALD. PNALD infants received lower protein doses (3.0 vs 3.3 g/kg/day, p = 0.014) while receiving higher GIR (11.4 vs 10.7 mg/kg/min, p = 0.012) compared to non-PNALD infants. Low birth weight, sepsis and bowel resection remain strong indicators of risk for PNALD. No single macronutrient increased our infants’ risk for PNALD. The use of newer ILEs when available should be evaluated for their impact on PNALD development.展开更多
Background:To achieve successful management of infants with congenital heart disease(CHD)together with pulmonary hypertension(PH),postoperative care,especially feeding care is vital in addition to surgery.Postoperativ...Background:To achieve successful management of infants with congenital heart disease(CHD)together with pulmonary hypertension(PH),postoperative care,especially feeding care is vital in addition to surgery.Postoperative feeding is comprised of three stages:feeding in the intensive care unit,feeding in the general ward and family feeding,in which the general ward is considered as the“transitional stage”.At present,there is little research on the optimal mode of feeding care for the transitional stage,and there is no universally recognized and accepted protocol.Methods:We retrospectively analyzed 114 CHD infants with PH who underwent family-centered(FC)feeding care from July 2017 to December 2018,and prospectively studied 122 CHD infants with the same baseline level who adopted the improved mode,nurse-parent-driven(NPD)feeding mode from January 2019 to June 2020.The feasibility and efficacy of NPD as a“transitional”feeding nursing mode in CHD infants with PH were compared with the FC cohort by observing and analyzing the stress of family caregivers,feedingrelated complications,the proportion of breastfeeding,improvement of nutritional status,acquisition of knowledge and skills of feeding care,inpatient’s satisfaction rating and prognosis.Results:When compared with the FC feeding care,the NPD mode significantly reduced the burden of family caregivers,improved the rate of feeding care knowledge and skills and inpatient’s satisfaction rating,reduced the incidence of improper feeding-related complications,and enhanced the proportion of breastfeeding and nutritional status of infants at the“transitional stage”(all P<0.05).The self-assessment score of care ability of family caregivers and weight gain of children in the NPD group were significantly higher than those in the FC group(all P<0.05)during the follow-up.Conclusions:As a transitional mode of feeding in CHD infants with PH,NPD feeding care is superior to the conventional FC mode,which therefore can be adopted as a standard protocol in clinical practice.展开更多
The prefrontal cortex of a human full termnewborn infant just after accidental death was studies by mcans of immunocytochemical technique with antibody directedagainst GABA(Immunonuclear Corp.)and ABC kit(Vector)。GAB...The prefrontal cortex of a human full termnewborn infant just after accidental death was studies by mcans of immunocytochemical technique with antibody directedagainst GABA(Immunonuclear Corp.)and ABC kit(Vector)。GABA-containing neurons were found over all layers and all were nonpyramidal cells.The laminar distribution of GABA-containing ne-urons was not even between different layers,density in laye Ⅱ was prominently higher than any other layers,density in layer Ⅲ and layer Ⅳ was higher than that in layer V and Ⅵ.展开更多
BACKGROUND Anti-D antibody is not the common cause of Rh-isoimmunization in Chinese neonatal jaundice.Recent change in national population policy has followed by an increase in Rh-isoimmunization related hemolytic dis...BACKGROUND Anti-D antibody is not the common cause of Rh-isoimmunization in Chinese neonatal jaundice.Recent change in national population policy has followed by an increase in Rh-isoimmunization related hemolytic disease of the newborn(HDN).Unfortunately,regional status of Rh-HDN is unavailable.We hypothesize that Rh-HDN in our region is most commonly due to anti-E antibody.AIM To investigate the prevalence of hemolytic disease of the newborn due to Rhisoimmunization in Hefei City.METHODS Retrospective review of data obtained from Children’s Hospital of Anhui and Hefei Blood Center between January 2017 and June 2019.Status of minor blood group antibody was studied in the corresponding mothers.RESULTS Totally 4138 newborns with HDN admitted during the study period and 116(2.8%)received blood exchange transfusion(BET).Eighteen newborns(0.43%)with proven Rh-incompatible HDN were identified.All were not the first-born baby.Thirteen mothers were RhD(+)(72%)and five were RhD(-).The distribution of Rh-related antibodies in mothers was ten anti-E(55%),five anti-D(27%),and for one anti-C,anti-c,and anti-E/c(6%)each.Thirteen(72.2%)were qualified for BET,relative risk for BET was 28.9 as compared to other types of HDN,but only 10 received due to parenteral refusal.All(100%)RhD related HDN received BET which is not significantly different from RhE related HDN(81.8%).CONCLUSION As expected,all Rh-incompatible HDN newborns were not the first-born.Contrary to the Caucasian population,anti-D induced HDN is not the most common etiology.In our region,anti-E(11/18,61%)is the most common cause of Rh-HDN.展开更多
A Chinese woman of blood group B,D-and her husband of blood group AB,CCDeewere examined.The woman had not been transfused before.Their first two babiesdied.Anti-Hro and anti-e were found in the mother’s serum.During ...A Chinese woman of blood group B,D-and her husband of blood group AB,CCDeewere examined.The woman had not been transfused before.Their first two babiesdied.Anti-Hro and anti-e were found in the mother’s serum.During her third pregnancy,the titer of antibodies went up quickly,approximately one titer per month.After 36 weeksof pregnancy,the baby was delivered by Caesarean section.The cord blood Hb was 88g/L,his red blood cell count 2.7×10<sup>12</sup>/L,and total biIirubin 114.6 mol/L.The baby was ofblood group AB,and CDe-D-genotype.Exchangetransfusion was begun 2.5 hours afterbirth.O,ccDEE washed red cells together with group AB plasma were used.Two dayslater,7Oml washed O,ccDEE concentrated red cells were administered.The baby is aliveand in good health.展开更多
Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the ...Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.展开更多
AIM To evaluate the prevalence, risk factors and outcome of acute kidney injury(AKI) in very low birth weight(VLBW) infants. METHODS In this retrospective study of VLBW infants, we analyzed the prevalence of AKI, as d...AIM To evaluate the prevalence, risk factors and outcome of acute kidney injury(AKI) in very low birth weight(VLBW) infants. METHODS In this retrospective study of VLBW infants, we analyzed the prevalence of AKI, as defined by changes in serum creatinine and urine output, associated risk factors and outcomes.RESULTS A total of 293 VLBW infants(mean gestational age 28.7 wk) were included, of whom 109 weighed less than 1000 g at birth. The overall prevalence of AKI was 11.6%(22% in infants with a birth weight under 1000 g and 5.4% those heavier). A total of 19(55%) affected infants died, with a mortality rate of 58% in infant less than 1000 g and 50% in those heavier. After adjusting for confounding variables, only necrotizing enterocolitis(NEC) remained associated with AKI, with odds ratio of 4.9(95%CI: 1.9-18.6). Blood pressure and glomerular filtration rate(GFR) were not different between affected infants and the others upon discharge from hospital. A normal GFR was documented in all affected infants at one year of age.CONCLUSION Using Kidney Disease Improving Global Outcomes definition of AKI, it occurred in over 10% of VLBW infants,more commonly in infants with lower birth weight. NEC was an independent associated risk factor. Renal function, as defined by GFR, was normal in all surviving affected infants 10 to 12 mo later.展开更多
Introduction The distribution of congenital heart disease (CHD) in sub-Saharan Africa is highly imprecise and varies from one region to another due to the inequality of diagnostic facilities. The aim of this stud...Introduction The distribution of congenital heart disease (CHD) in sub-Saharan Africa is highly imprecise and varies from one region to another due to the inequality of diagnostic facilities. The aim of this study was to determine the in-hospital prevalence of congenital heart disease in children at the Mother-Child hospital of Bingerville (HME) by specifying the diagnostic, therapeutic and evolutionary aspects. Materials and methods We conducted a retrospective, descriptive, cross-sectional study at HME of Bingerville from January 2021 to December 2022. All newborns with congenital heart disease confirmed by echocardiography were included in the study. Results Of 656 admissions to the neonatology department over the study period, congenital heart disease accounted for 14.9% (98/656) of cases. In our series, 76.7% were diagnosed before the 1st week of life, with a mean chronological age of 5.18 days and extremes of 0 and 46 days. There were as many male patients (50%) as female (50%), i.e. a sex ratio of 1. These newborns were premature in 60.2% of cases, with a mean and median gestational age of 34 weeks’ amenorrhea. Most were left-right shunts (90.8%). Persistent ductus arteriosus (PDA) (48.9%) predominated, followed by atrial septal defect (38.7%), ventricular septal defect (13.3%), common trunk artery (CTA) (3.1%) and open septal pulmonary atresia (OSPA) (1%) as the primary cyanogenic heart disease. Pulmonary arterial hypertension (PAH) (50%) was primary in 38.8% and secondary (61.2%). The mortality rate was 30.6%, and all CTA patients died (100%), with a significant statistical relationship (p = 0.027). Progression under treatment was marked by clinical stabilization (68/98) in 69.4% of cases. Conclusion: Congenital heart disease is relatively common at the Bingerville HME. Access to echocardiography should be facilitated in neonatology departments for rapid diagnosis and optimal management of congenital heart disease in newborns.展开更多
Background Newborn screening(NBS)is an important and successful public health program that helps improve the long-term clinical outcomes of newborns by providing early diagnosis and treatment of certain inborn disease...Background Newborn screening(NBS)is an important and successful public health program that helps improve the long-term clinical outcomes of newborns by providing early diagnosis and treatment of certain inborn diseases.The develop-ment of next-generation sequencing(NGS)technology provides new opportunities to expand current newborn screening methodologies.Methods We designed a a newborn genetic screening(NBGS)panel targeting 135 genes associated with 75 inborn disorders by multiplex PCR combined with NGS.With this panel,a large-scale,multicenter,prospective multidisease analysis was conducted on dried blood spot(DBS)profiles from 21,442 neonates nationwide.Results We presented the positive detection rate and carrier frequency of diseases and related variants in different regions;and 168(0.78%)positive cases were detected.Glucose-6-Phosphate Dehydrogenase deficiency(G6PDD)and phenylketonuria(PKU)had higher prevalence rates,which were significantly different in different regions.The positive detection of G6PD variants was quite common in south China,whereas PAH variants were most commonly identified in north China.In addi-tion,NBGS identified 3 cases with DUOX2 variants and one with SLC25A13 variants,which were normal in conventional NBS,but were confirmed later as abnormal in repeated biochemical testing after recall.Eighty percent of high-frequency gene carriers and 60%of high-frequency variant carriers had obvious regional differences.On the premise that there was no significant difference in birth weight and gestational age,the biochemical indicators of SLC22A5 c.1400C>G and ACADSB c.1165A>G carriers were significantly different from those of non-carriers.Conclusions We demonstrated that NBGS is an effective strategy to identify neonates affected with treatable diseases as a supplement to current NBS methods.Our data also showed that the prevalence of diseases has significant regional charac-teristics,which provides a theoretical basis for screening diseases in different regions.展开更多
Gastroesophageal reflux(GER)in children is very common and refers to the involuntary passage of gastric contents into the esophagus.This is often physiological and managed conservatively.In contrast,GER disease(GERD)i...Gastroesophageal reflux(GER)in children is very common and refers to the involuntary passage of gastric contents into the esophagus.This is often physiological and managed conservatively.In contrast,GER disease(GERD)is a less common pathologic process causing troublesome symptoms,which may need medical management.Apart from abnormal transient relaxations of the lower esophageal sphincter,other factors that play a role in the pathogenesis of GERD include defects in esophageal mucosal defense,impaired esophageal and gastric motility and clearance,as well as anatomical defects of the lower esophageal reflux barrier such as hiatal hernia.The clinical manifestations of GERD in young children are varied and nonspecific prompting the necessity for careful diagnostic evaluation.Management should be targeted to the underlying aetiopathogenesis and to limit complications of GERD.The following review focuses on up-to-date information regarding of the pathogenesis,diagnostic evaluation and management of GERD in children.展开更多
There are around 15 million preterm newborns(PT) eve ry year(Atienza-Navarro et al., 2020).With these figures in mind,prematurity represents a major health problem worldwide and it is a leading cause of infant mortali...There are around 15 million preterm newborns(PT) eve ry year(Atienza-Navarro et al., 2020).With these figures in mind,prematurity represents a major health problem worldwide and it is a leading cause of infant mortality,accounting for up to 35%of all deaths among newborns(Atienza-Navarro et al.,2020) and up to 18% of the deaths among children under 5 years of age.展开更多
Wilson disease is an autosomal recessive disorder of copper metabolism that can cause fatal neurological and hepatic disease if not diagnosed and treated. The youngest child with normal liver function reported so far ...Wilson disease is an autosomal recessive disorder of copper metabolism that can cause fatal neurological and hepatic disease if not diagnosed and treated. The youngest child with normal liver function reported so far is an 8-mo-old Japanese boy with low ceruloplasmin levels, and the youngest child with elevated aminotransferase ever reported so far is a 9-mo-old Korean boy with confirmed by genetic testing. Here we report an 8-mo-old Chinese boy presented with elevated liver enzymes, and low serum ceruloplasmin level. Genetic analysis of ATP7 B gene detected two heterozygous disease causing mutations(c.2621C>T/p.A874 V and c.3809A>G/p.N1270S), and parental origins were determined. Persistent elevation of serum aminotransferase in this infant was normalized after zinc therapy. To our best knowledge, this is the youngest patient with elevated liver enzymes ever reported worldwide. We hope that this will raise awareness among pediatricians, leading to earlier diagnosis, timely treatment, and better clinical outcome.展开更多
Background: Congenital heart disease (CHD) is the most common congenital malformations with high mortality and morbidity. The prevalence of CHD reported previously ranged from 4 per 1000 live births to 50 per 1000 ...Background: Congenital heart disease (CHD) is the most common congenital malformations with high mortality and morbidity. The prevalence of CHD reported previously ranged from 4 per 1000 live births to 50 per 1000 live births. In this cross-sectional study, we aimed to document the prevalence of CHD in Langfang district of Hebei Province, China by analyzing data collected by hospitals located in 11 the counties of the district, as supported by a public health campaign. Methods: A total of 67,718 consecutive 3-month-old infants were included from July 19,2012 to July 18, 2014. Structural abnormalities were diagnosed based on echocardiography findings, including two-dimensional and color Doppler echocardiography results. Results: Of the 67,718 infants, 1554 were found to have cardiac structural abnormalities. The total prevalence of CHD was 22.9 per 1000 live births, a value significantly higher than the previously reported prevalence of 8 cases per 1000 live births. The top five most common cardiac abnormalities were as follows: atrial septal defect (ASD, 605 cases, 8.93‰); ventricular septal defect (550 cases, 8.12‰); patent ductus arteriosus (228 cases, 3.37‰); pulmonary stenosis (66 cases, 0.97‰); and tetralogy of Fallot (32 cases, 0.47‰). The CHD prevalence differed by gender in this study (x^2 = 23.498, P 〈 0.001), and the majority of ASD cases were females. Regional differences in prevalence were also found (x^2 = 24.602, P 〈 0.001); a higher prevalence was found in urban areas (32.2 cases per 1000 live births) than in rural areas (21. 1 cases per 1000 live births). There was a significant difference in the prevalence of CHD in preterm versus full-term infants (x^2 - 133.443, P 〈 0.001 ). Prevalence of CHD in infants of maternal aged 35 years or over was significantly higher (x^2 86.917, P 〈 0.001). Conclusions: The prevalence of CHD in Langfang district was within the range reported using echocardiography. Echocardiography can be used to early diagnose the CHD.展开更多
文摘Yang et al reported an immunocompetent infant with gastrointestinal cytomegalovirus disease secondary to measles infection.We express our opinion about the diagnosis and treatment of this rare disease.
文摘Free radicals (FRs) generation is an unavoidable consequence of the life in an oxygen-rich atmosphere. FRs can be considered a double-edged sword. Beneficial effects of FRs occur at moderate concentrations and involve physiological roles in cellular responses to noxia, as in defense against infectious agents, in the function of a number of cellular signaling pathways and the induction of a mitogenic response. The over-production of FRs and the insufficiency of an antioxidant mechanism result in oxidative stress (OS), a deleterious process and important mediator of damage to cell structures and tissues. It occurs at birth in all newborns as a consequence of the hyperoxic challenge after the transition from the hypoxic intrauterine environment to extrauterine life. During the perinatal period, OS can be magnified by others predisposing conditions such as hyperoxia, hypoxia, ischemia, hypoxia-reperfusion, inflammation and high levels of non-protein bound iron. Epidemiological studies linked OS occurring during fetal stages and early infancy with adverse health outcomes later in life, indicating that OS is an early event in the etiology of these chronic diseases. Newborns, especially if preterm, are particularly susceptible to OS and damage due to the increased generation of FRs, the lack of adequate antioxidant protection, and the inability to induce antioxidant defenses during the hyperoxic challenge at birth. This impairment of the oxidative balance has been thought to be the common factor of pathologies grouped together as “free radical disease in the neonate” that include retinopathy of prematurity (which may lead to blindness in severe cases), bronchopulmonary dysplasia (a particularly debilitating pulmonary lesion of the preterm infant), periventricular leukomalacia (an important cause of severe neurodisability) and necrotizing enterocolitis. In this review we discuss in detail these perinatal diseases. Particularly, we analyze the current knowledge about the role of OS in their pathogenesis.
文摘Parenteral nutrition associated liver disease (PNALD) is a significant complication in infants receiving long-term parenteral nutrition (PN). Chronic administration of PN has been associated with its development. Our purpose is to characterize our incidence of PNALD over an extended period and identify risk factors for its development, including administration of soybean-based injectable lipid emulsions (ILEs) as we transit to novel ILEs</span><span style="font-family:Verdana;"> in our practice</span><span style="font-family:Verdana;">. Infants receiving 30 days or more of PN were included. PNALD was defined as a direct bilirubin ≥ 2 mg/dL. Data collected included: patient demographics, clinical and enteral feeding characteristics. Macronutrient intake was recorded using these cut-offs: glucose infusion rate (GIR) of ≤14 mg/kg/min or above, protein doses of ≤3 g/kg/day or above and lipid doses of ≤2 g/kg/day or above.</span><span style="font-family:""> </span><span style="font-family:Verdana;">A total of 349 infants were included, with an annual incidence of PNALD ranging between 34</span><span style="font-family:Verdana;">% </span><span style="font-family:Verdana;">-</span><span style="font-family:""> </span><span style="font-family:Verdana;">54%. Infants with PNALD were younger by gestation (27 vs. 29.5 weeks) and smaller by birthweight (900 vs. 1248 grams). Sepsis, GI disease including necrotizing enterocolitis and bowel resection were significantly associated with an increased risk for development of PNALD. PNALD infants received lower protein doses (3.0 vs 3.3 g/kg/day, p = 0.014) while receiving higher GIR (11.4 vs 10.7 mg/kg/min, p = 0.012) compared to non-PNALD infants. Low birth weight, sepsis and bowel resection remain strong indicators of risk for PNALD. No single macronutrient increased our infants’ risk for PNALD. The use of newer ILEs when available should be evaluated for their impact on PNALD development.
基金This work was supported by the Science and Technology Project of Science and Technology Bureau of Wenzhou(Y20170467)the Science and Technology Project of Medical and Health of Zhejiang Province(2017RC021)+1 种基金Key Discipline Program of Pediatric Surgery of Health Bureau of Zhejiang Province(No.11-ZC27)Research Center for diagnosis and treatment of cardiac and vascular disease of Zhejiang,China(JBZX-202001).
文摘Background:To achieve successful management of infants with congenital heart disease(CHD)together with pulmonary hypertension(PH),postoperative care,especially feeding care is vital in addition to surgery.Postoperative feeding is comprised of three stages:feeding in the intensive care unit,feeding in the general ward and family feeding,in which the general ward is considered as the“transitional stage”.At present,there is little research on the optimal mode of feeding care for the transitional stage,and there is no universally recognized and accepted protocol.Methods:We retrospectively analyzed 114 CHD infants with PH who underwent family-centered(FC)feeding care from July 2017 to December 2018,and prospectively studied 122 CHD infants with the same baseline level who adopted the improved mode,nurse-parent-driven(NPD)feeding mode from January 2019 to June 2020.The feasibility and efficacy of NPD as a“transitional”feeding nursing mode in CHD infants with PH were compared with the FC cohort by observing and analyzing the stress of family caregivers,feedingrelated complications,the proportion of breastfeeding,improvement of nutritional status,acquisition of knowledge and skills of feeding care,inpatient’s satisfaction rating and prognosis.Results:When compared with the FC feeding care,the NPD mode significantly reduced the burden of family caregivers,improved the rate of feeding care knowledge and skills and inpatient’s satisfaction rating,reduced the incidence of improper feeding-related complications,and enhanced the proportion of breastfeeding and nutritional status of infants at the“transitional stage”(all P<0.05).The self-assessment score of care ability of family caregivers and weight gain of children in the NPD group were significantly higher than those in the FC group(all P<0.05)during the follow-up.Conclusions:As a transitional mode of feeding in CHD infants with PH,NPD feeding care is superior to the conventional FC mode,which therefore can be adopted as a standard protocol in clinical practice.
文摘The prefrontal cortex of a human full termnewborn infant just after accidental death was studies by mcans of immunocytochemical technique with antibody directedagainst GABA(Immunonuclear Corp.)and ABC kit(Vector)。GABA-containing neurons were found over all layers and all were nonpyramidal cells.The laminar distribution of GABA-containing ne-urons was not even between different layers,density in laye Ⅱ was prominently higher than any other layers,density in layer Ⅲ and layer Ⅳ was higher than that in layer V and Ⅵ.
文摘BACKGROUND Anti-D antibody is not the common cause of Rh-isoimmunization in Chinese neonatal jaundice.Recent change in national population policy has followed by an increase in Rh-isoimmunization related hemolytic disease of the newborn(HDN).Unfortunately,regional status of Rh-HDN is unavailable.We hypothesize that Rh-HDN in our region is most commonly due to anti-E antibody.AIM To investigate the prevalence of hemolytic disease of the newborn due to Rhisoimmunization in Hefei City.METHODS Retrospective review of data obtained from Children’s Hospital of Anhui and Hefei Blood Center between January 2017 and June 2019.Status of minor blood group antibody was studied in the corresponding mothers.RESULTS Totally 4138 newborns with HDN admitted during the study period and 116(2.8%)received blood exchange transfusion(BET).Eighteen newborns(0.43%)with proven Rh-incompatible HDN were identified.All were not the first-born baby.Thirteen mothers were RhD(+)(72%)and five were RhD(-).The distribution of Rh-related antibodies in mothers was ten anti-E(55%),five anti-D(27%),and for one anti-C,anti-c,and anti-E/c(6%)each.Thirteen(72.2%)were qualified for BET,relative risk for BET was 28.9 as compared to other types of HDN,but only 10 received due to parenteral refusal.All(100%)RhD related HDN received BET which is not significantly different from RhE related HDN(81.8%).CONCLUSION As expected,all Rh-incompatible HDN newborns were not the first-born.Contrary to the Caucasian population,anti-D induced HDN is not the most common etiology.In our region,anti-E(11/18,61%)is the most common cause of Rh-HDN.
文摘A Chinese woman of blood group B,D-and her husband of blood group AB,CCDeewere examined.The woman had not been transfused before.Their first two babiesdied.Anti-Hro and anti-e were found in the mother’s serum.During her third pregnancy,the titer of antibodies went up quickly,approximately one titer per month.After 36 weeksof pregnancy,the baby was delivered by Caesarean section.The cord blood Hb was 88g/L,his red blood cell count 2.7×10<sup>12</sup>/L,and total biIirubin 114.6 mol/L.The baby was ofblood group AB,and CDe-D-genotype.Exchangetransfusion was begun 2.5 hours afterbirth.O,ccDEE washed red cells together with group AB plasma were used.Two dayslater,7Oml washed O,ccDEE concentrated red cells were administered.The baby is aliveand in good health.
文摘Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.
文摘AIM To evaluate the prevalence, risk factors and outcome of acute kidney injury(AKI) in very low birth weight(VLBW) infants. METHODS In this retrospective study of VLBW infants, we analyzed the prevalence of AKI, as defined by changes in serum creatinine and urine output, associated risk factors and outcomes.RESULTS A total of 293 VLBW infants(mean gestational age 28.7 wk) were included, of whom 109 weighed less than 1000 g at birth. The overall prevalence of AKI was 11.6%(22% in infants with a birth weight under 1000 g and 5.4% those heavier). A total of 19(55%) affected infants died, with a mortality rate of 58% in infant less than 1000 g and 50% in those heavier. After adjusting for confounding variables, only necrotizing enterocolitis(NEC) remained associated with AKI, with odds ratio of 4.9(95%CI: 1.9-18.6). Blood pressure and glomerular filtration rate(GFR) were not different between affected infants and the others upon discharge from hospital. A normal GFR was documented in all affected infants at one year of age.CONCLUSION Using Kidney Disease Improving Global Outcomes definition of AKI, it occurred in over 10% of VLBW infants,more commonly in infants with lower birth weight. NEC was an independent associated risk factor. Renal function, as defined by GFR, was normal in all surviving affected infants 10 to 12 mo later.
文摘Introduction The distribution of congenital heart disease (CHD) in sub-Saharan Africa is highly imprecise and varies from one region to another due to the inequality of diagnostic facilities. The aim of this study was to determine the in-hospital prevalence of congenital heart disease in children at the Mother-Child hospital of Bingerville (HME) by specifying the diagnostic, therapeutic and evolutionary aspects. Materials and methods We conducted a retrospective, descriptive, cross-sectional study at HME of Bingerville from January 2021 to December 2022. All newborns with congenital heart disease confirmed by echocardiography were included in the study. Results Of 656 admissions to the neonatology department over the study period, congenital heart disease accounted for 14.9% (98/656) of cases. In our series, 76.7% were diagnosed before the 1st week of life, with a mean chronological age of 5.18 days and extremes of 0 and 46 days. There were as many male patients (50%) as female (50%), i.e. a sex ratio of 1. These newborns were premature in 60.2% of cases, with a mean and median gestational age of 34 weeks’ amenorrhea. Most were left-right shunts (90.8%). Persistent ductus arteriosus (PDA) (48.9%) predominated, followed by atrial septal defect (38.7%), ventricular septal defect (13.3%), common trunk artery (CTA) (3.1%) and open septal pulmonary atresia (OSPA) (1%) as the primary cyanogenic heart disease. Pulmonary arterial hypertension (PAH) (50%) was primary in 38.8% and secondary (61.2%). The mortality rate was 30.6%, and all CTA patients died (100%), with a significant statistical relationship (p = 0.027). Progression under treatment was marked by clinical stabilization (68/98) in 69.4% of cases. Conclusion: Congenital heart disease is relatively common at the Bingerville HME. Access to echocardiography should be facilitated in neonatology departments for rapid diagnosis and optimal management of congenital heart disease in newborns.
基金the Foundation of National Key R&D Program of China of Research on Application Demonstration and Evaluation of Comprehensive Prevention And Control Technology of Birth Defects(Grant No.2018YFC1002700)Zhejiang R&D Research Project Research on New Technologies for Birth Health,Birth Safety and Perinatal Disease Diagnosis and Treatment(Grant No.2021C03099).
文摘Background Newborn screening(NBS)is an important and successful public health program that helps improve the long-term clinical outcomes of newborns by providing early diagnosis and treatment of certain inborn diseases.The develop-ment of next-generation sequencing(NGS)technology provides new opportunities to expand current newborn screening methodologies.Methods We designed a a newborn genetic screening(NBGS)panel targeting 135 genes associated with 75 inborn disorders by multiplex PCR combined with NGS.With this panel,a large-scale,multicenter,prospective multidisease analysis was conducted on dried blood spot(DBS)profiles from 21,442 neonates nationwide.Results We presented the positive detection rate and carrier frequency of diseases and related variants in different regions;and 168(0.78%)positive cases were detected.Glucose-6-Phosphate Dehydrogenase deficiency(G6PDD)and phenylketonuria(PKU)had higher prevalence rates,which were significantly different in different regions.The positive detection of G6PD variants was quite common in south China,whereas PAH variants were most commonly identified in north China.In addi-tion,NBGS identified 3 cases with DUOX2 variants and one with SLC25A13 variants,which were normal in conventional NBS,but were confirmed later as abnormal in repeated biochemical testing after recall.Eighty percent of high-frequency gene carriers and 60%of high-frequency variant carriers had obvious regional differences.On the premise that there was no significant difference in birth weight and gestational age,the biochemical indicators of SLC22A5 c.1400C>G and ACADSB c.1165A>G carriers were significantly different from those of non-carriers.Conclusions We demonstrated that NBGS is an effective strategy to identify neonates affected with treatable diseases as a supplement to current NBS methods.Our data also showed that the prevalence of diseases has significant regional charac-teristics,which provides a theoretical basis for screening diseases in different regions.
基金Supported by the Research Grant Contract Allocated for Basic Research from the Chulalongkorn University,No.HEA663000047.
文摘Gastroesophageal reflux(GER)in children is very common and refers to the involuntary passage of gastric contents into the esophagus.This is often physiological and managed conservatively.In contrast,GER disease(GERD)is a less common pathologic process causing troublesome symptoms,which may need medical management.Apart from abnormal transient relaxations of the lower esophageal sphincter,other factors that play a role in the pathogenesis of GERD include defects in esophageal mucosal defense,impaired esophageal and gastric motility and clearance,as well as anatomical defects of the lower esophageal reflux barrier such as hiatal hernia.The clinical manifestations of GERD in young children are varied and nonspecific prompting the necessity for careful diagnostic evaluation.Management should be targeted to the underlying aetiopathogenesis and to limit complications of GERD.The following review focuses on up-to-date information regarding of the pathogenesis,diagnostic evaluation and management of GERD in children.
基金supported by the following funds:To PAM:predoctoral fellowship.Instituto de Investigacion Biomedica de la Provincia de Cadiz (INIBICA)To MGA:Agencia Andaluza del Conocimiento.Proyectos I+D+I—Programa Operativo FEDER Andalucia 2014-2020 fondos FEDER (SOL2018001071 89-TRA)+1 种基金Agencia Estatal de Investigacion.Ministerio de Educacion y Ciencia.Programa Estatal de Generacion de Conocimiento y Fortalecimiento Cientifico y Tecnologico del Sistema de I+D+i y del Programa Estatal de I+D+i Orientada a los Retos de la Sociedaddel Plan Estatal de Investigacion Cientifica y Tecnica y de Innovacion 2017-2020 (PID2020-115499RB-I00/AEI/10.13039/501100011033)。
文摘There are around 15 million preterm newborns(PT) eve ry year(Atienza-Navarro et al., 2020).With these figures in mind,prematurity represents a major health problem worldwide and it is a leading cause of infant mortality,accounting for up to 35%of all deaths among newborns(Atienza-Navarro et al.,2020) and up to 18% of the deaths among children under 5 years of age.
基金Supported by National Natural Science Foundation of China,No.81070281
文摘Wilson disease is an autosomal recessive disorder of copper metabolism that can cause fatal neurological and hepatic disease if not diagnosed and treated. The youngest child with normal liver function reported so far is an 8-mo-old Japanese boy with low ceruloplasmin levels, and the youngest child with elevated aminotransferase ever reported so far is a 9-mo-old Korean boy with confirmed by genetic testing. Here we report an 8-mo-old Chinese boy presented with elevated liver enzymes, and low serum ceruloplasmin level. Genetic analysis of ATP7 B gene detected two heterozygous disease causing mutations(c.2621C>T/p.A874 V and c.3809A>G/p.N1270S), and parental origins were determined. Persistent elevation of serum aminotransferase in this infant was normalized after zinc therapy. To our best knowledge, this is the youngest patient with elevated liver enzymes ever reported worldwide. We hope that this will raise awareness among pediatricians, leading to earlier diagnosis, timely treatment, and better clinical outcome.
文摘Background: Congenital heart disease (CHD) is the most common congenital malformations with high mortality and morbidity. The prevalence of CHD reported previously ranged from 4 per 1000 live births to 50 per 1000 live births. In this cross-sectional study, we aimed to document the prevalence of CHD in Langfang district of Hebei Province, China by analyzing data collected by hospitals located in 11 the counties of the district, as supported by a public health campaign. Methods: A total of 67,718 consecutive 3-month-old infants were included from July 19,2012 to July 18, 2014. Structural abnormalities were diagnosed based on echocardiography findings, including two-dimensional and color Doppler echocardiography results. Results: Of the 67,718 infants, 1554 were found to have cardiac structural abnormalities. The total prevalence of CHD was 22.9 per 1000 live births, a value significantly higher than the previously reported prevalence of 8 cases per 1000 live births. The top five most common cardiac abnormalities were as follows: atrial septal defect (ASD, 605 cases, 8.93‰); ventricular septal defect (550 cases, 8.12‰); patent ductus arteriosus (228 cases, 3.37‰); pulmonary stenosis (66 cases, 0.97‰); and tetralogy of Fallot (32 cases, 0.47‰). The CHD prevalence differed by gender in this study (x^2 = 23.498, P 〈 0.001), and the majority of ASD cases were females. Regional differences in prevalence were also found (x^2 = 24.602, P 〈 0.001); a higher prevalence was found in urban areas (32.2 cases per 1000 live births) than in rural areas (21. 1 cases per 1000 live births). There was a significant difference in the prevalence of CHD in preterm versus full-term infants (x^2 - 133.443, P 〈 0.001 ). Prevalence of CHD in infants of maternal aged 35 years or over was significantly higher (x^2 86.917, P 〈 0.001). Conclusions: The prevalence of CHD in Langfang district was within the range reported using echocardiography. Echocardiography can be used to early diagnose the CHD.