Neonatal surgical pathologies in developing countries are characterized by high mortality. The aim of this work was to describe the characteristics of the newborn and its surgical pathologies treated in the neonatal d...Neonatal surgical pathologies in developing countries are characterized by high mortality. The aim of this work was to describe the characteristics of the newborn and its surgical pathologies treated in the neonatal department of the Gabriel Touré University Hospital in Bamako (Mali). Patients and method: This is a retrospective study of 626 files of newborns hospitalized over a period of 3 years (January 2016 to December 2018) for a surgical pathology, whether operated on or not. We have studied the etiological, therapeutic and evolutionary aspects. Results: During the study period, we collected 626 patients who had surgical pathology, i.e. a frequency of 5.35%. The average therapeutic consultation time was 2 days with extremes (1 and 30 days). Pathologies of the abdominal wall and digestive tract represented 57.9% (n = 84) followed by pathologies of the spine 26.9% (n = 39). The mean treatment time was 4.5 days. Twenty-three point sixteen (23.16%) newborns could be operated on. The neonatal mortality rate was 12.4% (n = 18). Mortality was postoperative (100%) with a mean time to onset of 5 days. Abdominal wall pathologies were responsible for 8.7% (n = 13) of deaths. Conclusion: Surgical pathologies of the newborn are characterized by delayed treatment with high mortality. Improving their prognosis requires early diagnosis (prenatal diagnosis) and adequate management.展开更多
Introduction: The United Nations Sustainable Development Goals (SDGs) aim to decrease the global maternal mortality ratio to below 70 per 100,000 live births and eliminate preventable deaths of newborns and children u...Introduction: The United Nations Sustainable Development Goals (SDGs) aim to decrease the global maternal mortality ratio to below 70 per 100,000 live births and eliminate preventable deaths of newborns and children under the age of five in all countries by 2030. The pediatric department at Spécialisé Mère-Enfant Blanche Gomes Hospital (HSMEBG) is divided into two sectors, one catering to children aged one month to four years and the other dedicated to children aged five to 17 years. According to department records, over the past three years, there has been an average of 1050 hospitalizations per year, with an average duration of five days. Objectives: This study aims to describe the socio-demographic characteristics of children who died while in the pediatrics department of the HSMEBG and analyze the factors associated with their deaths. Methodology: A retrospective analytical cross-sectional study was conducted, collecting data over a three-year period, covering the years 2019, 2020 and 2021. Data were collected from medical records of deceased children using Excel software version 2016, and statistical calculations and logistic regression were performed using Epi info software version 7.2.5.0. Results: During the three years of operation, the pediatric department at HSMEBG recorded 3060 new admissions, of which 271 resulted in death, representing an overall frequency of 8.8%. December and January had the highest mortality rates, accounting for 15.5% and 12.5%, respectively. Out of the 271 recorded deaths, 143 (52.77%) occurred in children under the age of one, and 230 (84.87%) occurred in children under the age of five. The average age at death was 2.4 years, ranging from one month to 17 years. The sex ratio was 1. More than half of the deaths (51.66%) occurred during the night, and 165 (60.89%) sought medical help more than three days after the onset of symptoms. Weekend deaths accounted for nearly half (45.7%) of the cases. Upon admission, slightly over half of the children (55.72%) had impaired consciousness, 219 (80.81%) presented with respiratory distress, and 194 (71.59%) had a fever. The average time from admission to administering the first medication was 72 minutes. Respiratory infections were the leading cause of death, accounting for 83 (30.26%) cases, followed by severe forms of malaria (anemic and neurological) at 23.25%. Among the 271 recorded deaths, 33 (12.18%) received no treatment before their demise, and 136 (50.18%) died within the first 24 hours of hospitalization. The average duration of hospitalization for patients who spent less than 24 hours was 15 hours, while those who died after the 24th hour had an average hospital stay of five days, ranging from one to 41 days. Children under the age of five who were admitted with impaired consciousness had roughly double the risk of dying compared to those without this condition (p = 0.001). Conclusion: The overall mortality rate in the pediatric department at HSMEBG is 8.8%. Acute respiratory infections are the primary cause of death. Improving this rate necessitates reducing consultation and treatment durations.展开更多
Background: Moderate to severe hypoxic-ischemic encephalopathy (HIE) in neonates is often treated with hypothermia. However, some neonates may experience epileptic seizures during therapeutic hypothermia (TH). Data on...Background: Moderate to severe hypoxic-ischemic encephalopathy (HIE) in neonates is often treated with hypothermia. However, some neonates may experience epileptic seizures during therapeutic hypothermia (TH). Data on the electrophysiologic and evolutionary aspects of these seizures are scarce in African countries. Objectives: To determine the types of epileptic seizures caused by HIE in neonates in Brazzaville;to describe the evolution of background EEG activities during TH and rewarming;to report the evolution of epileptic seizures. Methods: This was a cross-sectional, descriptive study conducted from January 2020 to July 2022. It took place in Brazzaville in the Neonatology Department of the Blanche Gomez Mother and Child Hospital. It focused on term neonates suffering from moderate or severe HIE. They were treated with hypothermia combined with phenobarbital for 72 hours. Results: Among 36 neonates meeting inclusion criteria, there were 18 boys and 18 girls. Thirty-one (86.1%) neonates had grade 2 and 5 (13.9%) grade 3 HIE. In our neonates, HIE had induced isolated electrographic seizures (n = 11;30.6%), electroclinical seizures (n = 25;69.4%), and 6 types of background EEG activity. During TH and rewarming, there were 52.8% of patients with improved background EEG activity, 41.7% of patients with unchanged background EEG activity, and 5.5% of patients with worsened background EEG activity. At the end of rewarming, only 9 (25%) patients still had seizures. Conclusion: Isolated electrographic and electroclinical seizures are the only pathological entities found in our studied population. In neonates with moderate HIE, the applied therapeutic strategy positively influences the evolution of both seizures and background EEG activity. On the other hand, in neonates with severe HIE, the same therapeutic strategy is ineffective. .展开更多
Split hand/foot malformation (SHFM), formerly known as ectrodactyly is a rare congénital anomaly, its incidence varies from 1/8.500 to 1/25.000 live birth. It mainly affects the development of the limbs, its clin...Split hand/foot malformation (SHFM), formerly known as ectrodactyly is a rare congénital anomaly, its incidence varies from 1/8.500 to 1/25.000 live birth. It mainly affects the development of the limbs, its clinical variability is standard, can present as an isolated feature or as a syndrome associated with other congenital anomalies. Our objective was to present the two cases of SHFM, and to review the literature on the clinical aspects and discuss a probable origin. The father went to school and is a driver because the malformations concerned only the fingers, were less severe, and did not prevent the realization of certain simple gestures of the daily life. On the other hand, the malformations of the fingers of the newborn were severe and the absence of the thumbs compromised the later prehension function. Also the association of a microglossia and a cleft palate contributed to a weight loss that justified hospitalization. The clinical presentation of split hands and feet is variable and the prognosis depends on the type of anomaly. Familial cases suggest a probable genetic origin. Genetic testing is necessary to establish genetic counseling.展开更多
Melnick-Needles syndrome is an osteo-dysplasia caused by a mutation of a gene, FLNA, coding for filamine A. It is at the origin of a set of complex congenital malformations, mainly of interest to the members, but also...Melnick-Needles syndrome is an osteo-dysplasia caused by a mutation of a gene, FLNA, coding for filamine A. It is at the origin of a set of complex congenital malformations, mainly of interest to the members, but also to the cranio-facial region. Melnick-Needles syndrome was first described in 1966 by two Americans John Melnick, a radiologist, and Carl F Needles, a pediatrician. They described cases of families of several generations who had a characteristic severe congenital bone disorder. They thought that human-to-human transmission had occurred in one case. We report a case of Melnick-Needles syndrome which is a pathology with very high mortality especially in a male subject. This was an observational study of a case received in consultation with the pediatric service of the Souro Sanou University Hospital Center in Bobo Dioulasso in Burkina Faso. It was a 3-year-old boy born in a low-term pregnancy without any prenatal consultation, imaging test and prenatal biology performed. The exact pathogenesis of this condition is not established but is linked to a mutation of the Filamine A gene linked to sexual chromosomes X. Pathology is rare, around a hundred cases have been reported worldwide. Its incidence, according to the literature is around 1/100,000. The sex ratio is at 7. The malformations of this acquired embryo-fetopathy are multiple, polymorphic and asymmetrical. The diagnosis of this pathology is suspected by the clinic and established by molecular biology by sequencing the genes responsible for the mutation. However, imagery, in particular the scanner has a major role by its protocol with multi-planar reconstructions and its analysis in double windowing which allow a better description of the malformations of this syndrome. Its management is complex, and multidisciplinary, and the unfortunate prognosis in our context is because of the precarious technical platform. In our case despite the obvious malformations presented by the patient, the parents consulted late in a health center because of the automation and especially the socio-cultural constraints which incriminate this type of polymalformation as being a curse. The patient underwent abdominal surgery and even the operating procedures were simple, he died four months after leaving the hospital, due to an unspecified cause.展开更多
Despite substantial progress in neonatal care over the past two decades leading to improved survival of extremely premature infants, extreme prematurity continues to be associated with long term neurodevelopmental imp...Despite substantial progress in neonatal care over the past two decades leading to improved survival of extremely premature infants, extreme prematurity continues to be associated with long term neurodevelopmental impairments. Cerebral white matter injury is the predominant form of insult in preterm brain leading to adverse neurological consequences. Such brain injury pattern and unfavorable neurologic sequelae is commonly encountered in premature infants exposed to systemic inflammatory states such as clinical or culture proven sepsis with or without evidence of meningitis, prolonged mechanical ventilation, bronchopulmonary dysplasia, necrotizing enterocolitis and chorioamnionitis. Underlying mechanisms may include cytokine mediated processes without direct entry of pathogens into the brain, developmental differences in immune response and complex neurovascular barrier system that play a critical role in regulating the cerebral response to various systemic inflammatory insults in premature infants. Understanding of these pathologic mechanisms and clinical correlates of such injury based on serum biomarkers or brain imaging findings on magnetic resonance imaging will pave way for future research and translational therapeutic opportunities for the developing brain.展开更多
Respiratory syncytial virus(RSV) is the most frequent and important cause of lower respiratory tract infection in infants and children. It is a seasonal virus, with peak rates of infection occurring annually in the co...Respiratory syncytial virus(RSV) is the most frequent and important cause of lower respiratory tract infection in infants and children. It is a seasonal virus, with peak rates of infection occurring annually in the cold season in temperate climates, and in the rainy season, as temperatures fall, in tropical climates. High risk groups for severe RSV disease include infants below six mo of age, premature infants with or without chronic lung disease, infants with hemodynamically significant congenital heart disease, infants with immunodeficiency or cystic fibrosis, and infants with neuromuscular diseases. Mortality rates associated with RSV infection are generally low in previous healthy infants(below 1%), but increase significantly in children with underlying chronic conditions and comorbidities. Following early RSV lower respiratory tract infection, some patients experience recurrent episodes of wheezing mimicking early childhood asthma with persistence of lung function abnormalities until adolescence. There is currently no RSV vaccine available, but promising candidate vaccines are in development. Palivizumab, a monoclonal RSV antibody that is the only tool for immunoprophylaxis in high-riskinfants, lowers the burden of RSV infection in certain carefully selected patient groups.展开更多
In the epidemiologic context of maternal obesity and type 2 diabetes(T2D),the incidence of gestational diabetes has significantly increased in the last decades.Infants of diabetic mothers are prone to various neonatal...In the epidemiologic context of maternal obesity and type 2 diabetes(T2D),the incidence of gestational diabetes has significantly increased in the last decades.Infants of diabetic mothers are prone to various neonatal adverse outcomes,including metabolic and hematologic disorders,respiratory distress,cardiac disorders and neurologic impairment due to perinatal asphyxia and birth traumas,among others.Macrosomia is the most constant consequence of diabetes and its severity is mainly influenced by maternal blood glucose level.Neonatal hypoglycemia is the main metabolic disorder that should be prevented as soon as possible after birth.The severity of macrosomia and the maternal health condition have a strong impact on the frequency and the severity of adverse neonatal outcomes.Pregestational T2 D and maternal obesity significantly increase the risk of perinatal death and birth defects.The high incidence of maternal hyperglycemia in developing countries,associated with the scarcity of maternal and neonatal care,seriously increase the burden of neonatal complications in these countries.展开更多
The aim of this article is to examine the effect of omega-3(ω-3) long-chain polyunsaturated fatty acids(LCPUFAs) intake on retinopathy of prematurity(ROP) by reviewing the experimental and clinical trials conducted o...The aim of this article is to examine the effect of omega-3(ω-3) long-chain polyunsaturated fatty acids(LCPUFAs) intake on retinopathy of prematurity(ROP) by reviewing the experimental and clinical trials conducted on animal models and infants.LCPUFAs demonstrate cytoprotective and cytotherapeutic actions contributing to a number of anti-angiogenic and neuroprotective mechanisms within the retina.Their intake appears to have a beneficial effect on ischemia,oxidative stress,inflammation and cellular signaling mechanisms,influencing retinal cell gene expression and cellular differentiation.ω-3 LCPUFAs may modulate metabolic processes that activate molecules implicated in the pathogenesis of vasoproliferative and neurodegenerative retinal diseases such as ROP.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
Necrotizing enterocolitis [NEC] is an inflammatory disease of intestine largely occuring in preterm infants with a wide range of damage from minimal injury limited to mucosa to extensive necrosis of bowel wall and per...Necrotizing enterocolitis [NEC] is an inflammatory disease of intestine largely occuring in preterm infants with a wide range of damage from minimal injury limited to mucosa to extensive necrosis of bowel wall and perforation. Despite advancements in neonatal care, mortality remains high [30% - 50%] and controversy still persists with regards to the most appropriate management of neonates with necrotizing enterocolitis. The main factors thought to be involved in the pathogenesis of NEC are: relatively hyper-reactive state of premature intestine, enteral feeding and bacterial colonization. In this review, we discuss current knowledge about the epidemiology, pathophysiology, imaging, medical and surgical management of necrotizing enterocolitis and describe novel strategies for prevention and treatment.展开更多
Objective:To study the correlation of immune response and inflammatory response with human cartilage glycoprotein 39 (YKL-40) content in children with recurrent pneumonia. Methods: Children who were treated in the Cen...Objective:To study the correlation of immune response and inflammatory response with human cartilage glycoprotein 39 (YKL-40) content in children with recurrent pneumonia. Methods: Children who were treated in the Central Hospital of Enshi Autonomous Prefecture due to recurrent pneumonia between May 2014 and October 2017 were selected as the experimental group A of the research, children who were treated in the Central Hospital of Enshi Autonomous Prefecture due to acute pneumonia during the same period were selected as the experimental group B of the study, and children who received physical examination in the Central Hospital of Enshi Autonomous Prefecture during the same period were selected as the control group. Serum was collected to determine the contents of YKL-40, immune molecules and inflammatory molecules, and peripheral blood was collected to determine the expression of immune molecules and inflammatory molecules.Results: Serum YKL-40, IFN-γ, HMGB1, TNF-α, MCP4 and CysLTs levels as well as peripheral blood TLR4 and NF-κB expression intensity of experiment group A and experiment group B were higher than those of control group whereas peripheral blood TIM1, TIM3 and CD19 expression intensity as well as serum IL-4 levels were lower than those of control group, and the change of above indexes in experimental group A was more significant than that in experimental group B;peripheral blood TIM1, TIM3 and CD19 expression intensity as well as serum IL-4 level of experimental group A with high YKL-40 were significantly lower than those of experimental group A with low YKL-40 whereas serum IFN-γ, HMGB1, TNF-α, MCP4 and CysLTs levels as well as peripheral blood TLR4 and NF-κB expression intensity were significantly higher than those of experimental group A with low YKL-40.Conclusions: The abnormal secretion of YKL-40 in children with recurrent pneumonia is closely related to the disorder of immune response and the exceissive activation of inflammatory response.展开更多
Objective: The objective of the study was to determine the incidence, origin, and character of cerebral lesions in monochorionic twins with twin-to-twin transfusion syndrome treated with fetoscopic laser surgery. Stud...Objective: The objective of the study was to determine the incidence, origin, and character of cerebral lesions in monochorionic twins with twin-to-twin transfusion syndrome treated with fetoscopic laser surgery. Study design: This was a prospective study of monochorionic twins with twin-to-twin transfusion syndrome treated with fetoscopic laser surgery and monochorionic twins without twin-to-twin transfusion syndrome delivered at our center between June 2002 and September 2005, using cranial ultrasonography. Results: Incidence of antenatally acquired severe cerebral lesions in the twin-to-twin transfusion syndrome group was 10% (8/84) and 2% (2/108) in the non-twin-to-twin transfusion syndrome group (P = .02) . Incidence of severe cerebral lesions at discharge was 14% (12/84) in the twin-to-twin transfusion syndrome group and 6% (6/108) in the non-twin-to-twin transfusion syndrome group (P = .04). Antenatal injury was responsible for severe cerebral lesions in 67% (8/12) of the twin-to-twin transfusion syndrome group. Conclusion: Incidence of severe cerebral lesions in twin-to-twin transfusion syndrome treated with fetoscopic laser surgery is high and results mainly from antenatal injury.展开更多
Objective:To evaluate the changes in serum index levels after monosialoganglioside injection combined with conventional treatment of neonatal hypoxic ischemic encephalopathy. Methods:A total of 70 children with neonat...Objective:To evaluate the changes in serum index levels after monosialoganglioside injection combined with conventional treatment of neonatal hypoxic ischemic encephalopathy. Methods:A total of 70 children with neonatal hypoxic ischemic encephalopathy treated in our hospital between February 2013 and February 2016 were selected and randomly divided into observation group and control group, control group received clinical routine treatment and observation group accepted monosialoganglioside injection combined with conventional treatment. After 1 week of treatment, serum levels of apoptosis factors, nerve function indexes, oxidation/anti-oxidation indexes and disease severity indexes of two groups of patients were detected.Results:Serum PDCD5, sFas, sFasL, NSE, S100-β, MDA, NO, NOS, H-FABP, NPY, caspase-1 and ET-1 levels of observation group were lower than those of control group while BDNF, NGF, SOD, GSH-PX, IGF-1 and GH levels were higher than those of control group.Conclusion: Monosialoganglioside injection can enhance the overall treatment effect and promote the realization of homeostasis in children with HIE.展开更多
Objectives: We tested the hypothesis that twice weekly prophylactic dosing of fluconazole prevents invasive candidiasis without promoting resistant Candida sp ecies in high-risk, preterm infants. Study design: We comp...Objectives: We tested the hypothesis that twice weekly prophylactic dosing of fluconazole prevents invasive candidiasis without promoting resistant Candida sp ecies in high-risk, preterm infants. Study design: We compared our previous dos ing schedule (Group A) to a less frequent dosing schedule of twice a week (Group B) of fluconazole prophylaxis for up to 6 weeks in a prospective, randomized, d ouble-blind clinical trial in preterm infants weighing<1000 grams at birth and with an endotracheal tube and/or central vascular catheter over a 24-month peri od. Weekly surveillance cultures were obtained on study patients. Results: Candi da colonization was documented in 5 (12%) of 41 Group A and in 4 (10%) of 40 G roup B infants. Candida sepsis developed in two (5%) of Group A and one (3%) o f Group B infants (risk difference, -0.02; 95%confidence interval, -0.14-0.1 0; P = .68). All fungal isolates remained sensitive to fluconazole, and no drug side effects were documented. Conclusions: Twice weekly dosing of prophylactic f luconazole can decrease Candida colonization and invasive infection, cost, and p atient exposure in high-risk, preterm infants weighing < 1000 grams at birth. W e speculate that lower and less frequent dosing may delay or prevent the emergen ce of antifungal resistance.展开更多
Objective:To assess the correlation of signs of myocardial damage to serum cardiac troponin I(cTnI)and creatine kinase MB isoenzyme(CK-MB)concentrations.Methods:Blood samples were collected from 25 term asphyxiated ne...Objective:To assess the correlation of signs of myocardial damage to serum cardiac troponin I(cTnI)and creatine kinase MB isoenzyme(CK-MB)concentrations.Methods:Blood samples were collected from 25 term asphyxiated neonates and 25 controls at 12 h of age by immunoassay.The asphyxiated neonates were followed up until discharge or death.Results:Asphyxiated neonates had significantly higher concentrations of cTnI and CK-MB than controls(P<0.001).Serum cTnI concentrations were significantly higher in asphyxiated neonates who developed hypotension,heart failure or those had low ejection fraction(P<0.01).Serum cTnI concentrations were significantly higher in asphyxiated who died than those who survived(P<0.01).There was no significant difference in serum CK-MB mass concentrations between asphyxiated neonates with and without these complications.Conclusion:Unlike CK-MB,serum cTnI concentrations are significantly higher in asphyxiated neonates who died or developed cardiac dysfunction.展开更多
Background: The importance of non-glucose carbohydrates, especially mannose and inositol, for normal development is increasingly recognized. Whether pregnancies complicated by abnormal glucose transfer to the fetus al...Background: The importance of non-glucose carbohydrates, especially mannose and inositol, for normal development is increasingly recognized. Whether pregnancies complicated by abnormal glucose transfer to the fetus also affect the regulation of non-glucose carbohydrates is unknown. In pregnant sheep, maternal insulin infusions were used to reduce glucose supply to the fetus for both short(2-wk) and long(8-wk) durations to test the hypothesis that a maternal insulin infusion would suppress fetal mannose and inositol concentrations. We also used direct fetal insulin infusions(1-wk hyperinsulinemic-isoglycemic clamp) to determine the relative importance of fetal glucose and insulin for regulating non-glucose carbohydrates.Results: A maternal insulin infusion resulted in lower maternal(50%, P < 0.01) and fetal(35-45%, P < 0.01) mannose concentrations, which were highly correlated(r2= 0.69, P < 0.01). A fetal insulin infusion resulted in a 50% reduction of fetal mannose(P < 0.05). Neither maternal nor fetal plasma inositol changed with exogenous insulin infusions.Additionally, maternal insulin infusion resulted in lower fetal sorbitol and fructose(P < 0.01).Conclusions: Chronically decreased glucose supply to the fetus as well as fetal hyperinsulinemia both reduce fetal non-glucose carbohydrates. Given the role of these carbohydrates in protein glycosylation and lipid production, more research on their metabolism in pregnancies complicated by abnormal glucose metabolism is clearly warranted.展开更多
Respirovirus infection can cause viral pneumonia and acute lung injury(ALI). The interleukin-1(IL-1) family consists of proinflammatory cytokines that play essential roles in regulating immune and inflammatory respons...Respirovirus infection can cause viral pneumonia and acute lung injury(ALI). The interleukin-1(IL-1) family consists of proinflammatory cytokines that play essential roles in regulating immune and inflammatory responses in vivo. IL-1 signaling is associated with protection against respiratory influenza virus infection by mediation of the pulmonary anti-viral immune response and inflammation. We analyzed the infiltration lung immune leukocytes and cytokines that contribute to inflammatory lung pathology and mortality of fatal H1N1 virus-infected IL-1 receptor 1(IL-1R1) deficient mice. Results showed that early innate immune cells and cytokine/chemokine dysregulation were observed with significantly decreased neutrophil infiltration and IL-6, TNF-α, G-CSF, KC, and MIP-2 cytokine levels in the bronchoalveolar lavage fluid of infected IL-1R1^(-/-) mice in comparison with that of wild type infected mice. The adaptive immune response against the H1N1 virus in IL-1R1^(-/-) mice was impaired with downregulated anti-viral Th1 cell, CD8+ cell, and antibody functions, which contributes to attenuated viral clearance. Histological analysis revealed reduced lung inflammation during early infection but severe lung pathology in late infection in IL-1R1^(-/-) mice compared with that in WT infected mice. Moreover, the infected IL-1R1^(-/-) mice showed markedly reduced neutrophil generation in bone marrow and neutrophil recruitment to the inflamed lung. Together, these results suggest that IL-1 signaling is associated with pulmonary anti-influenza immune response and inflammatory lung injury, particularly via the influence on neutrophil mobilization and inflammatory cytokine/chemokine production.展开更多
Perinatal brain injury(PBI)is one of the most important causes of lifelong deficits in cognition,behavior,social interaction and motor skills,as well as epilepsy.PBI encompasses,but is not limited to,hypoxic-ischemic ...Perinatal brain injury(PBI)is one of the most important causes of lifelong deficits in cognition,behavior,social interaction and motor skills,as well as epilepsy.PBI encompasses,but is not limited to,hypoxic-ischemic encephalopathy(HIE),intraventricular hemorrhage,periventricular leukomalacia and encephalopathy展开更多
Williams-Beuren Syndrome (WB-S) occurs in approximately 1/7500 live births. It is characterized by typical facial features, congenital heart defects and mild mental retardation. Around 75% - 80% of all patients have s...Williams-Beuren Syndrome (WB-S) occurs in approximately 1/7500 live births. It is characterized by typical facial features, congenital heart defects and mild mental retardation. Around 75% - 80% of all patients have some kind of cardiovascular disorder being supravalvular aortic stenosis and pulmonary artery stenosis the most frequent. This syndrome is due to a contiguous gene deletion (1- to 2-megabase deletion on the long arm of chromosome 7), including the entire elastin gene and 20 additional genes. We present a case of a two year old boy with WB-S and Tetralogy of Fallot, a very infrequent association. Diagnosis of WB-S could be made because of typical facial features. Characteristic WB-S deletion was present. Genetic study to rule out 22q11 deletion was also performed.展开更多
文摘Neonatal surgical pathologies in developing countries are characterized by high mortality. The aim of this work was to describe the characteristics of the newborn and its surgical pathologies treated in the neonatal department of the Gabriel Touré University Hospital in Bamako (Mali). Patients and method: This is a retrospective study of 626 files of newborns hospitalized over a period of 3 years (January 2016 to December 2018) for a surgical pathology, whether operated on or not. We have studied the etiological, therapeutic and evolutionary aspects. Results: During the study period, we collected 626 patients who had surgical pathology, i.e. a frequency of 5.35%. The average therapeutic consultation time was 2 days with extremes (1 and 30 days). Pathologies of the abdominal wall and digestive tract represented 57.9% (n = 84) followed by pathologies of the spine 26.9% (n = 39). The mean treatment time was 4.5 days. Twenty-three point sixteen (23.16%) newborns could be operated on. The neonatal mortality rate was 12.4% (n = 18). Mortality was postoperative (100%) with a mean time to onset of 5 days. Abdominal wall pathologies were responsible for 8.7% (n = 13) of deaths. Conclusion: Surgical pathologies of the newborn are characterized by delayed treatment with high mortality. Improving their prognosis requires early diagnosis (prenatal diagnosis) and adequate management.
文摘Introduction: The United Nations Sustainable Development Goals (SDGs) aim to decrease the global maternal mortality ratio to below 70 per 100,000 live births and eliminate preventable deaths of newborns and children under the age of five in all countries by 2030. The pediatric department at Spécialisé Mère-Enfant Blanche Gomes Hospital (HSMEBG) is divided into two sectors, one catering to children aged one month to four years and the other dedicated to children aged five to 17 years. According to department records, over the past three years, there has been an average of 1050 hospitalizations per year, with an average duration of five days. Objectives: This study aims to describe the socio-demographic characteristics of children who died while in the pediatrics department of the HSMEBG and analyze the factors associated with their deaths. Methodology: A retrospective analytical cross-sectional study was conducted, collecting data over a three-year period, covering the years 2019, 2020 and 2021. Data were collected from medical records of deceased children using Excel software version 2016, and statistical calculations and logistic regression were performed using Epi info software version 7.2.5.0. Results: During the three years of operation, the pediatric department at HSMEBG recorded 3060 new admissions, of which 271 resulted in death, representing an overall frequency of 8.8%. December and January had the highest mortality rates, accounting for 15.5% and 12.5%, respectively. Out of the 271 recorded deaths, 143 (52.77%) occurred in children under the age of one, and 230 (84.87%) occurred in children under the age of five. The average age at death was 2.4 years, ranging from one month to 17 years. The sex ratio was 1. More than half of the deaths (51.66%) occurred during the night, and 165 (60.89%) sought medical help more than three days after the onset of symptoms. Weekend deaths accounted for nearly half (45.7%) of the cases. Upon admission, slightly over half of the children (55.72%) had impaired consciousness, 219 (80.81%) presented with respiratory distress, and 194 (71.59%) had a fever. The average time from admission to administering the first medication was 72 minutes. Respiratory infections were the leading cause of death, accounting for 83 (30.26%) cases, followed by severe forms of malaria (anemic and neurological) at 23.25%. Among the 271 recorded deaths, 33 (12.18%) received no treatment before their demise, and 136 (50.18%) died within the first 24 hours of hospitalization. The average duration of hospitalization for patients who spent less than 24 hours was 15 hours, while those who died after the 24th hour had an average hospital stay of five days, ranging from one to 41 days. Children under the age of five who were admitted with impaired consciousness had roughly double the risk of dying compared to those without this condition (p = 0.001). Conclusion: The overall mortality rate in the pediatric department at HSMEBG is 8.8%. Acute respiratory infections are the primary cause of death. Improving this rate necessitates reducing consultation and treatment durations.
文摘Background: Moderate to severe hypoxic-ischemic encephalopathy (HIE) in neonates is often treated with hypothermia. However, some neonates may experience epileptic seizures during therapeutic hypothermia (TH). Data on the electrophysiologic and evolutionary aspects of these seizures are scarce in African countries. Objectives: To determine the types of epileptic seizures caused by HIE in neonates in Brazzaville;to describe the evolution of background EEG activities during TH and rewarming;to report the evolution of epileptic seizures. Methods: This was a cross-sectional, descriptive study conducted from January 2020 to July 2022. It took place in Brazzaville in the Neonatology Department of the Blanche Gomez Mother and Child Hospital. It focused on term neonates suffering from moderate or severe HIE. They were treated with hypothermia combined with phenobarbital for 72 hours. Results: Among 36 neonates meeting inclusion criteria, there were 18 boys and 18 girls. Thirty-one (86.1%) neonates had grade 2 and 5 (13.9%) grade 3 HIE. In our neonates, HIE had induced isolated electrographic seizures (n = 11;30.6%), electroclinical seizures (n = 25;69.4%), and 6 types of background EEG activity. During TH and rewarming, there were 52.8% of patients with improved background EEG activity, 41.7% of patients with unchanged background EEG activity, and 5.5% of patients with worsened background EEG activity. At the end of rewarming, only 9 (25%) patients still had seizures. Conclusion: Isolated electrographic and electroclinical seizures are the only pathological entities found in our studied population. In neonates with moderate HIE, the applied therapeutic strategy positively influences the evolution of both seizures and background EEG activity. On the other hand, in neonates with severe HIE, the same therapeutic strategy is ineffective. .
文摘Split hand/foot malformation (SHFM), formerly known as ectrodactyly is a rare congénital anomaly, its incidence varies from 1/8.500 to 1/25.000 live birth. It mainly affects the development of the limbs, its clinical variability is standard, can present as an isolated feature or as a syndrome associated with other congenital anomalies. Our objective was to present the two cases of SHFM, and to review the literature on the clinical aspects and discuss a probable origin. The father went to school and is a driver because the malformations concerned only the fingers, were less severe, and did not prevent the realization of certain simple gestures of the daily life. On the other hand, the malformations of the fingers of the newborn were severe and the absence of the thumbs compromised the later prehension function. Also the association of a microglossia and a cleft palate contributed to a weight loss that justified hospitalization. The clinical presentation of split hands and feet is variable and the prognosis depends on the type of anomaly. Familial cases suggest a probable genetic origin. Genetic testing is necessary to establish genetic counseling.
文摘Melnick-Needles syndrome is an osteo-dysplasia caused by a mutation of a gene, FLNA, coding for filamine A. It is at the origin of a set of complex congenital malformations, mainly of interest to the members, but also to the cranio-facial region. Melnick-Needles syndrome was first described in 1966 by two Americans John Melnick, a radiologist, and Carl F Needles, a pediatrician. They described cases of families of several generations who had a characteristic severe congenital bone disorder. They thought that human-to-human transmission had occurred in one case. We report a case of Melnick-Needles syndrome which is a pathology with very high mortality especially in a male subject. This was an observational study of a case received in consultation with the pediatric service of the Souro Sanou University Hospital Center in Bobo Dioulasso in Burkina Faso. It was a 3-year-old boy born in a low-term pregnancy without any prenatal consultation, imaging test and prenatal biology performed. The exact pathogenesis of this condition is not established but is linked to a mutation of the Filamine A gene linked to sexual chromosomes X. Pathology is rare, around a hundred cases have been reported worldwide. Its incidence, according to the literature is around 1/100,000. The sex ratio is at 7. The malformations of this acquired embryo-fetopathy are multiple, polymorphic and asymmetrical. The diagnosis of this pathology is suspected by the clinic and established by molecular biology by sequencing the genes responsible for the mutation. However, imagery, in particular the scanner has a major role by its protocol with multi-planar reconstructions and its analysis in double windowing which allow a better description of the malformations of this syndrome. Its management is complex, and multidisciplinary, and the unfortunate prognosis in our context is because of the precarious technical platform. In our case despite the obvious malformations presented by the patient, the parents consulted late in a health center because of the automation and especially the socio-cultural constraints which incriminate this type of polymalformation as being a curse. The patient underwent abdominal surgery and even the operating procedures were simple, he died four months after leaving the hospital, due to an unspecified cause.
文摘Despite substantial progress in neonatal care over the past two decades leading to improved survival of extremely premature infants, extreme prematurity continues to be associated with long term neurodevelopmental impairments. Cerebral white matter injury is the predominant form of insult in preterm brain leading to adverse neurological consequences. Such brain injury pattern and unfavorable neurologic sequelae is commonly encountered in premature infants exposed to systemic inflammatory states such as clinical or culture proven sepsis with or without evidence of meningitis, prolonged mechanical ventilation, bronchopulmonary dysplasia, necrotizing enterocolitis and chorioamnionitis. Underlying mechanisms may include cytokine mediated processes without direct entry of pathogens into the brain, developmental differences in immune response and complex neurovascular barrier system that play a critical role in regulating the cerebral response to various systemic inflammatory insults in premature infants. Understanding of these pathologic mechanisms and clinical correlates of such injury based on serum biomarkers or brain imaging findings on magnetic resonance imaging will pave way for future research and translational therapeutic opportunities for the developing brain.
文摘Respiratory syncytial virus(RSV) is the most frequent and important cause of lower respiratory tract infection in infants and children. It is a seasonal virus, with peak rates of infection occurring annually in the cold season in temperate climates, and in the rainy season, as temperatures fall, in tropical climates. High risk groups for severe RSV disease include infants below six mo of age, premature infants with or without chronic lung disease, infants with hemodynamically significant congenital heart disease, infants with immunodeficiency or cystic fibrosis, and infants with neuromuscular diseases. Mortality rates associated with RSV infection are generally low in previous healthy infants(below 1%), but increase significantly in children with underlying chronic conditions and comorbidities. Following early RSV lower respiratory tract infection, some patients experience recurrent episodes of wheezing mimicking early childhood asthma with persistence of lung function abnormalities until adolescence. There is currently no RSV vaccine available, but promising candidate vaccines are in development. Palivizumab, a monoclonal RSV antibody that is the only tool for immunoprophylaxis in high-riskinfants, lowers the burden of RSV infection in certain carefully selected patient groups.
文摘In the epidemiologic context of maternal obesity and type 2 diabetes(T2D),the incidence of gestational diabetes has significantly increased in the last decades.Infants of diabetic mothers are prone to various neonatal adverse outcomes,including metabolic and hematologic disorders,respiratory distress,cardiac disorders and neurologic impairment due to perinatal asphyxia and birth traumas,among others.Macrosomia is the most constant consequence of diabetes and its severity is mainly influenced by maternal blood glucose level.Neonatal hypoglycemia is the main metabolic disorder that should be prevented as soon as possible after birth.The severity of macrosomia and the maternal health condition have a strong impact on the frequency and the severity of adverse neonatal outcomes.Pregestational T2 D and maternal obesity significantly increase the risk of perinatal death and birth defects.The high incidence of maternal hyperglycemia in developing countries,associated with the scarcity of maternal and neonatal care,seriously increase the burden of neonatal complications in these countries.
文摘The aim of this article is to examine the effect of omega-3(ω-3) long-chain polyunsaturated fatty acids(LCPUFAs) intake on retinopathy of prematurity(ROP) by reviewing the experimental and clinical trials conducted on animal models and infants.LCPUFAs demonstrate cytoprotective and cytotherapeutic actions contributing to a number of anti-angiogenic and neuroprotective mechanisms within the retina.Their intake appears to have a beneficial effect on ischemia,oxidative stress,inflammation and cellular signaling mechanisms,influencing retinal cell gene expression and cellular differentiation.ω-3 LCPUFAs may modulate metabolic processes that activate molecules implicated in the pathogenesis of vasoproliferative and neurodegenerative retinal diseases such as ROP.
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Necrotizing enterocolitis [NEC] is an inflammatory disease of intestine largely occuring in preterm infants with a wide range of damage from minimal injury limited to mucosa to extensive necrosis of bowel wall and perforation. Despite advancements in neonatal care, mortality remains high [30% - 50%] and controversy still persists with regards to the most appropriate management of neonates with necrotizing enterocolitis. The main factors thought to be involved in the pathogenesis of NEC are: relatively hyper-reactive state of premature intestine, enteral feeding and bacterial colonization. In this review, we discuss current knowledge about the epidemiology, pathophysiology, imaging, medical and surgical management of necrotizing enterocolitis and describe novel strategies for prevention and treatment.
文摘Objective:To study the correlation of immune response and inflammatory response with human cartilage glycoprotein 39 (YKL-40) content in children with recurrent pneumonia. Methods: Children who were treated in the Central Hospital of Enshi Autonomous Prefecture due to recurrent pneumonia between May 2014 and October 2017 were selected as the experimental group A of the research, children who were treated in the Central Hospital of Enshi Autonomous Prefecture due to acute pneumonia during the same period were selected as the experimental group B of the study, and children who received physical examination in the Central Hospital of Enshi Autonomous Prefecture during the same period were selected as the control group. Serum was collected to determine the contents of YKL-40, immune molecules and inflammatory molecules, and peripheral blood was collected to determine the expression of immune molecules and inflammatory molecules.Results: Serum YKL-40, IFN-γ, HMGB1, TNF-α, MCP4 and CysLTs levels as well as peripheral blood TLR4 and NF-κB expression intensity of experiment group A and experiment group B were higher than those of control group whereas peripheral blood TIM1, TIM3 and CD19 expression intensity as well as serum IL-4 levels were lower than those of control group, and the change of above indexes in experimental group A was more significant than that in experimental group B;peripheral blood TIM1, TIM3 and CD19 expression intensity as well as serum IL-4 level of experimental group A with high YKL-40 were significantly lower than those of experimental group A with low YKL-40 whereas serum IFN-γ, HMGB1, TNF-α, MCP4 and CysLTs levels as well as peripheral blood TLR4 and NF-κB expression intensity were significantly higher than those of experimental group A with low YKL-40.Conclusions: The abnormal secretion of YKL-40 in children with recurrent pneumonia is closely related to the disorder of immune response and the exceissive activation of inflammatory response.
文摘Objective: The objective of the study was to determine the incidence, origin, and character of cerebral lesions in monochorionic twins with twin-to-twin transfusion syndrome treated with fetoscopic laser surgery. Study design: This was a prospective study of monochorionic twins with twin-to-twin transfusion syndrome treated with fetoscopic laser surgery and monochorionic twins without twin-to-twin transfusion syndrome delivered at our center between June 2002 and September 2005, using cranial ultrasonography. Results: Incidence of antenatally acquired severe cerebral lesions in the twin-to-twin transfusion syndrome group was 10% (8/84) and 2% (2/108) in the non-twin-to-twin transfusion syndrome group (P = .02) . Incidence of severe cerebral lesions at discharge was 14% (12/84) in the twin-to-twin transfusion syndrome group and 6% (6/108) in the non-twin-to-twin transfusion syndrome group (P = .04). Antenatal injury was responsible for severe cerebral lesions in 67% (8/12) of the twin-to-twin transfusion syndrome group. Conclusion: Incidence of severe cerebral lesions in twin-to-twin transfusion syndrome treated with fetoscopic laser surgery is high and results mainly from antenatal injury.
文摘Objective:To evaluate the changes in serum index levels after monosialoganglioside injection combined with conventional treatment of neonatal hypoxic ischemic encephalopathy. Methods:A total of 70 children with neonatal hypoxic ischemic encephalopathy treated in our hospital between February 2013 and February 2016 were selected and randomly divided into observation group and control group, control group received clinical routine treatment and observation group accepted monosialoganglioside injection combined with conventional treatment. After 1 week of treatment, serum levels of apoptosis factors, nerve function indexes, oxidation/anti-oxidation indexes and disease severity indexes of two groups of patients were detected.Results:Serum PDCD5, sFas, sFasL, NSE, S100-β, MDA, NO, NOS, H-FABP, NPY, caspase-1 and ET-1 levels of observation group were lower than those of control group while BDNF, NGF, SOD, GSH-PX, IGF-1 and GH levels were higher than those of control group.Conclusion: Monosialoganglioside injection can enhance the overall treatment effect and promote the realization of homeostasis in children with HIE.
文摘Objectives: We tested the hypothesis that twice weekly prophylactic dosing of fluconazole prevents invasive candidiasis without promoting resistant Candida sp ecies in high-risk, preterm infants. Study design: We compared our previous dos ing schedule (Group A) to a less frequent dosing schedule of twice a week (Group B) of fluconazole prophylaxis for up to 6 weeks in a prospective, randomized, d ouble-blind clinical trial in preterm infants weighing<1000 grams at birth and with an endotracheal tube and/or central vascular catheter over a 24-month peri od. Weekly surveillance cultures were obtained on study patients. Results: Candi da colonization was documented in 5 (12%) of 41 Group A and in 4 (10%) of 40 G roup B infants. Candida sepsis developed in two (5%) of Group A and one (3%) o f Group B infants (risk difference, -0.02; 95%confidence interval, -0.14-0.1 0; P = .68). All fungal isolates remained sensitive to fluconazole, and no drug side effects were documented. Conclusions: Twice weekly dosing of prophylactic f luconazole can decrease Candida colonization and invasive infection, cost, and p atient exposure in high-risk, preterm infants weighing < 1000 grams at birth. W e speculate that lower and less frequent dosing may delay or prevent the emergen ce of antifungal resistance.
文摘Objective:To assess the correlation of signs of myocardial damage to serum cardiac troponin I(cTnI)and creatine kinase MB isoenzyme(CK-MB)concentrations.Methods:Blood samples were collected from 25 term asphyxiated neonates and 25 controls at 12 h of age by immunoassay.The asphyxiated neonates were followed up until discharge or death.Results:Asphyxiated neonates had significantly higher concentrations of cTnI and CK-MB than controls(P<0.001).Serum cTnI concentrations were significantly higher in asphyxiated neonates who developed hypotension,heart failure or those had low ejection fraction(P<0.01).Serum cTnI concentrations were significantly higher in asphyxiated who died than those who survived(P<0.01).There was no significant difference in serum CK-MB mass concentrations between asphyxiated neonates with and without these complications.Conclusion:Unlike CK-MB,serum cTnI concentrations are significantly higher in asphyxiated neonates who died or developed cardiac dysfunction.
基金supported by National Institutes of Health training grant T32 HD007186-32 (W Hay, PI and PD)supported by NIH Grants R01DK088139 and K08HD060688+5 种基金American Diabetes Association Junior Faculty Award 7-08-JF-51(PJR, PI)provided by the UC Denver DERC (P30DK57516 J. Hutton, PI)supported as a Scholar by NIH Building Interdisciplinary Careers in Women ’ s Health Scholar Award K12HD057022 (J. Regensteiner, PI)a Children ’ s Hospital Colorado Research Institute Research Scholar Award (PI)supported by NIH K01DK090199 (PI) and as a trainee on NIH training grant T32 HD007186-32 (W Hay, PI and PD)
文摘Background: The importance of non-glucose carbohydrates, especially mannose and inositol, for normal development is increasingly recognized. Whether pregnancies complicated by abnormal glucose transfer to the fetus also affect the regulation of non-glucose carbohydrates is unknown. In pregnant sheep, maternal insulin infusions were used to reduce glucose supply to the fetus for both short(2-wk) and long(8-wk) durations to test the hypothesis that a maternal insulin infusion would suppress fetal mannose and inositol concentrations. We also used direct fetal insulin infusions(1-wk hyperinsulinemic-isoglycemic clamp) to determine the relative importance of fetal glucose and insulin for regulating non-glucose carbohydrates.Results: A maternal insulin infusion resulted in lower maternal(50%, P < 0.01) and fetal(35-45%, P < 0.01) mannose concentrations, which were highly correlated(r2= 0.69, P < 0.01). A fetal insulin infusion resulted in a 50% reduction of fetal mannose(P < 0.05). Neither maternal nor fetal plasma inositol changed with exogenous insulin infusions.Additionally, maternal insulin infusion resulted in lower fetal sorbitol and fructose(P < 0.01).Conclusions: Chronically decreased glucose supply to the fetus as well as fetal hyperinsulinemia both reduce fetal non-glucose carbohydrates. Given the role of these carbohydrates in protein glycosylation and lipid production, more research on their metabolism in pregnancies complicated by abnormal glucose metabolism is clearly warranted.
基金supported by the National Natural Science Foundation of China(31300143,31570900)the Applied Basic Research Foundation of Yunnan Province,China(2015FB139)the Chinese Academy of Medical Science(CAMS)Innovation Fund for Medical Sciences(2016-I2M-1-014)
文摘Respirovirus infection can cause viral pneumonia and acute lung injury(ALI). The interleukin-1(IL-1) family consists of proinflammatory cytokines that play essential roles in regulating immune and inflammatory responses in vivo. IL-1 signaling is associated with protection against respiratory influenza virus infection by mediation of the pulmonary anti-viral immune response and inflammation. We analyzed the infiltration lung immune leukocytes and cytokines that contribute to inflammatory lung pathology and mortality of fatal H1N1 virus-infected IL-1 receptor 1(IL-1R1) deficient mice. Results showed that early innate immune cells and cytokine/chemokine dysregulation were observed with significantly decreased neutrophil infiltration and IL-6, TNF-α, G-CSF, KC, and MIP-2 cytokine levels in the bronchoalveolar lavage fluid of infected IL-1R1^(-/-) mice in comparison with that of wild type infected mice. The adaptive immune response against the H1N1 virus in IL-1R1^(-/-) mice was impaired with downregulated anti-viral Th1 cell, CD8+ cell, and antibody functions, which contributes to attenuated viral clearance. Histological analysis revealed reduced lung inflammation during early infection but severe lung pathology in late infection in IL-1R1^(-/-) mice compared with that in WT infected mice. Moreover, the infected IL-1R1^(-/-) mice showed markedly reduced neutrophil generation in bone marrow and neutrophil recruitment to the inflamed lung. Together, these results suggest that IL-1 signaling is associated with pulmonary anti-influenza immune response and inflammatory lung injury, particularly via the influence on neutrophil mobilization and inflammatory cytokine/chemokine production.
文摘Perinatal brain injury(PBI)is one of the most important causes of lifelong deficits in cognition,behavior,social interaction and motor skills,as well as epilepsy.PBI encompasses,but is not limited to,hypoxic-ischemic encephalopathy(HIE),intraventricular hemorrhage,periventricular leukomalacia and encephalopathy
文摘Williams-Beuren Syndrome (WB-S) occurs in approximately 1/7500 live births. It is characterized by typical facial features, congenital heart defects and mild mental retardation. Around 75% - 80% of all patients have some kind of cardiovascular disorder being supravalvular aortic stenosis and pulmonary artery stenosis the most frequent. This syndrome is due to a contiguous gene deletion (1- to 2-megabase deletion on the long arm of chromosome 7), including the entire elastin gene and 20 additional genes. We present a case of a two year old boy with WB-S and Tetralogy of Fallot, a very infrequent association. Diagnosis of WB-S could be made because of typical facial features. Characteristic WB-S deletion was present. Genetic study to rule out 22q11 deletion was also performed.
