RESUMO
The Chinese Neonatal Network(CHNN) was established in 2018 with the mission of establishing a national collaboration platform, conducting high-quality and collaborative research, and ultimately improving the quality of neonatal-perinatal care and health in China.At present, 112 hospitals across the country have joined CHNN.CHNN has established a national standardized cohort of very premature infants/very low birth weight infants with >10 000 enrollments each year, has been leading data-driven collaborative quality improvement initiatives, conducting multicenter clinical studies, and performing multi-level training programs.Guided by the principles of collaboration and sharing, data-driven, continuous improvement, and international integration, CHNN has become an important platform for clinical and research collaboration in neonatal medicine in China.
RESUMO
OBJECTIVE@#To detect potential variant in a male neonate affected with congenital nephrogenic diabetes insipidus (CNDI).@*METHODS@#Clinical data of the patient was collected. Genomic DNA was extracted from peripheral blood samples from the child and his parents. The whole coding regions of the arginine vasopressin V2 receptor (AVPR2) gene were amplified by PCR and subjected to Sanger sequencing.@*RESULTS@#The patient presented recurrent fever and polyuria after birth. Multiple blood gas analyses indicated hypernatremia. Ultrasound showed bilateral hydronephrosis and hydroureter. The patient was partially responsive to hydrochlorothiazide. DNA analysis identified a hemizygous frameshift variant c.890-899delACCCGGAGGC in exon 2 of the AVPR2 gene in the proband. His mother was heterozygous for the same variant.@*CONCLUSION@#The c.890-899delACCCGGAGGC variant of the AVPR2 gene probably underlies the CNDI in the child. Above discovery has enriched to spectrum of CNDI associated variants.
Assuntos
Adulto , Feminino , Humanos , Recém-Nascido , Masculino , Diabetes Insípido Nefrogênico/genética , Éxons , Mutação da Fase de Leitura , Hidroclorotiazida/uso terapêutico , Linhagem , Receptores de Vasopressinas/genéticaRESUMO
Enterovirus is a common cause of neonatal virus infection, which is primarily transmitted by fecal-oral and respiratory routes and can cause nosocomial infection and outbreak in nursery or neonatal intensive care unit.Most infants with enterovirus infection are asymptomatic or just have mild self-limiting symptoms.Severe infection is less common but with significantly increased mortality, and may be complicated by the encephalitis/meningoencephalitis, myocarditis and/or hepatitis.Early recognition and prompt isolation of infants with enterovirus infection are important strategies for nosocomial infection control.
RESUMO
The clinical manifestation, laboratory findings, and imaging examination of a baby with familial glucocorticoid deficiency were summarized. The patient presented achypnea, cyanosis, and pigmentation of the whole body skin, no convulsion and hypoglycemia found. Laboratory findings revealed low blood cortisol and high blood ACTH levels. A 1-bp homozygous deletion(c.106+ 1delG) in intron 3 of melanocortin 2 receptor accessory protein(MRAP) gene in the patient was found. His parents were found to be heterozygous carrier for the same mutation, without any clinical manifestation.
RESUMO
Objective:To explore the clinical and genetic characteristics of a Chinese baby with perinatal hypophosphatasia (HPP) and his parents for better understanding of the disease.Methods:The clinical data of the patient with HPP was carefully collected. The laboratory and radiographic examination data were taken for this baby patient. Sequencing for all the twelve tissue-nonspecific alkaline phosphatase(ALPL) exons and the flanking exon-intron junctions were performed in the proband and his parents with their genomic DNA from peripheral blood.Results:The blood level of alkaline phosphatase was decreased in this patient while serum calcium level was increased. His bone revealed chondrodysplasia-like change. Compound heterozygous mutations were found in the proband, with c. 346G>A(p.A116T) in exon 5 and c. 1171C>T(p.R391C) in exon 10. His father and mother were without clinical manifestation while respectively carried c. 346G>A(p.A116T and c. 1171C>T(p.R391C) missense mutations, suggesting an autosomal recessive inheritance in this family.Conclusion:Perinatal HPP has a high mortality rate. Skeletal deformities, hypercalcemia, and low level of ALP are important in the differential diagnosis of perinatal HPP.
RESUMO
For provincial children's hospitals and children's hospitals affiliated to medical colleges and universities with a high level of the diagnosis and treatment,they are the center of referral and need to retain pediatricians and build a high-level team of pediatricians.With the authors' own experience in Cincinnati Children's Hospital Medical Center in the US,this article briefly introduces the current clinical operation mode in American children's hospitals,especially the consultation system mainly composed of attending physicians and the mode of continuing medical education based on both clinical and scientific research.Such experience provides a reference for pediatricians in China.
RESUMO
Clinical jaundice is present in the majority of the newborns, while bilirubin encephalopathy is one of the most serious complications.Preterm birth is high risk factors of bilirubin encephalopathy.Premature infants may develop bilirubin-induced central nervous system(CNS)injury even at total serum bilirubin(TSB)levels conventionally considered to be non-neurotoxic, and less frequently show the typical abnormal neuromotor clinical signs.Preterm birth is one of the risk factors of bilirubin encephalopathy.The potential for bilirubin-induced neurotoxicity in the premature neonate remains a clinical concern, while efforts to improve current clinical assessment and early diagnosis are certainly needed.Recently research on the molecular and cellular mechanism of bilirubin neurotoxicity and the clinical manifestations of bilirubin-induced CNS injury in the preterm neonate has made great progress, and it has been found that laboratory index and neuroimaging techniques are closely associated with early diagnosis of bilirubin-induced neurotoxicity in the preterm neonate.
RESUMO
Objective@#To evaluate the effectiveness and safety of the use of noninvasive high-frequency oscillation ventilation (nHFOV) in very low birth weight infants.@*Method@#A total of 36 cases received nHFOV between January 2016 and October 2016 in Children′s Hospital, Zhejiang University School of Medicine, including 24 males and 12 females, with the gestational age of (27.5±2.5) weeks and birth weight of(980±318)g. The data of the ventilator settings, side effects, and changes of the respiratory function before and after nHFOV were collected and analyzed retrospectively. Nonparametric tests or t tests or χ2 tests were used.@*Result@#Thirty-two (89%) out of the 36 cases successfully avoided intubation or re-intubation after using of nHFOV. nHFOV was used as the rescue treatment after failure of other noninvasive ventilation in 17 cases, and as the prophylactical treatment preventing re-intubation after extubation in the remaining 19 cases. There were significant decreases in the incidences of apnea and desaturation(SpO2<0.85), the level of PaCO2, and the FiO2 24 h after the initiation of the nHFOV as the rescue therapy((1.2±1.1)vs.(6.3±2.1)episodes , (1.1±1.2) vs.(4.3±1.5) episodes, (43±8) vs.(56±10) mmHg, 0.30±0.07 vs. 0.39±0.11, respectively; 1 mmHg=0.133 kPa, t=7.562, 8.913, 4.179, 3.437 respectively, all P<0.01). No significant changes were found in FiO2 and PaCO2 levels 24 h after initiation of nHFOV as the prophylactical therapy after extubation (0.42±0.12 vs.0.40±0.10, (49±8)vs.(48±7)mmHg, t=0.872 and 0.501 respectively, both P>0.05), except for the significant decreases in the mean airway pressure ((7.9±2.6)vs.(9.6±1.6)cmH2O, 1 cmH20=0.098 kPa, t=2.198, P=0.041). There were 4 cases suffered from nasal septum injury, while no other nHFOV related complications were noted.@*Conclusion@#nHFOV can be applied in preterm infants as a rescue treatment after the failure of other noninvasive ventilation, or prophylactically used in patients who have high risk of re-intubation.
RESUMO
Obgective To analyze the demographic data,non-specific items,pathogens and antibiotic sensitivity between the children with early-onset and late-onset sepsis,in order to guide the diagnosis and treatment of neonatal sepsis.Methods Three hundred and fifty-two cases with positive blood culture were retrospectively recruited and divided into an early-onset group and a late-onset sepsis group according to the onset of sepsis.Results Of 352 cases,144 cases (40.91%) were the early-onset children while 208 cases (59.09%) were the late-onset children,and in the late-onset group,108 cases occurred due to nosocomial infection.Most neonates of the early-onset term were term infants [107/144 cases (74.31%)],while the preterm infants [77/208 cases (37.02%)] and low birth weight infants[70/208 cases(33.65%)] accounted for the majority of the late-onset group.The asphyxia,perinatal intrauterine distress,meconium-staining amniotic fluid and premature rupture of fetal membranes ≥ 18 h occurred more frequently in the early-onset group [21/144 cases (14.58%),14/144 cases (9.72%),26/144 cases (18.06%),31/144 cases (21.53%)],respectively,while those in the late-onset group were [17/208 cases (8.17%),9/208 cases(4.33%),13/208 cases(6.25%),17/208 cases(8.17%)],respectively,there were significant differences (x2 =4.622,3.886,5.950,13.345,all P < 0.05) between 2 groups.In the early-onset group abnormal temperature[72/208 cases(34.62%)vs 30/144 cases(20.83%)],vomiting or abdominal distention[109/208 cases (52.40%) vs 35/144 cases (24.31%)],lethargy [79/208 cases (37.98%) vs 38/144 cases (26.39 %)] and umbilicalitis or skin pustule [33/208 cases (15.87 %) vs 11 / 1 44 cases (7.64 %)] occurred more frequently in late-onset group,and there were significant differences (x2 =7.853,8.763,5.153,5.265,all P < 0.05).Besides,more cases in the late-onset group had elevated immature neutrophil vs total neutrophil count ratio [27/184 cases (14.67%)] and C-reactive protein value [76/206 cases (36.89%)],compared with those in early-onset group [9/133 cases (6.77%),38/143 cases(26.57%)],and there were significant differences (x2 =4.794,4.087,allP < 0.05).Compared with early-onset group,patients in the late-onset group were more likely to suffer from suppurative meningitis [17.79% (37/208 cases) vs 8.33% (12/144 cases);x2 =6.348,P < 0.05].In terms of pathogens,the main pathogens in the early-onset group were gram negative bacteria[39.58% (57/144 cases),including detection of Klebisella pneumoniae in 21 cases and E.coli in 20 cases] and coagulase negative staphylococcus[32.64% (47/144 cases)].In late-onset group,the main pathogens were gram positive bacteria [58.65% (122/208 cases)],including detection of coagulase negative staphylococcus in 90 cases(43.27%) and E.coli [17.79% (37/208 cases)].There was no significant difference in prognosis between 2 groups(x2 =1.187,P =0.552).Conclusions Early-onset sepsis and late onset sepsis differ in the clinical manifestation and laboratory findings.Distinguishing neonatal early-onset and late onset septicemia is of clinical significance in choosing appropriate antibiotics.
RESUMO
Persistent pulmonary hypertension of the newborn is a critical illness in neonatal period.Rational treatments are based on the correct diagnosis and differential diagnosis. In this paper, the clinical manifestations and diagnosis of persistent pulmonary hypertension of the newborn, the relationship between the relevant clinical diagnostic procedures, auxiliary examination method and the common need to identify the disease are described, with emphasis on Echocardiographic diagnosis of pulmonary hypertension.
RESUMO
The incidence of aberrant thymus in the posterior mediastinum is very uncommon. It is difficult to exclude malignancy before surgical procedure. In a six-month-old male coughing for two weeks prior to admission a posterior mediastinal mass was found incidentally by chest roentgenogram. Thoracotomy was performed. Histologic study revealed normal thymic tissue. When a mass located in the posterior mediastinum, ectopic thymus should be included in differential diagnosis. Imaging techniques may spare thoracotomy. Ectopic thymus has a benign clinical course, and surgical resection is not recommended
RESUMO
Objective To evaluate the role of using non-invasive ventilation with bi-level positive airway pressure (BiPAP) in order to reduce the need of re-intubation in pediatric patients with respiratory failure after cardiac surgery. Method From January 2007 to December 2007, 25 patients aged from three months to 11 years with median 2.3 years operated on for cardiac surgery with respiratory insufficiency after extubation and re-intubation indicated were enrolled in this study. They were put on non-invasive nasal (mask) BiPAP ventilation before re-intubation. The arterial blood gas, A-aDO2 and PaO2/FiO2 were measured. In addition, clinical data including heart rate, respiratory rate, and the product of heart rate and systolic pressure were recorded before and after BiPAP. The software SPSSD 13.0 was used to process by ANOVA test for statistical analysis. Meanwhile, the outcome of these patients was analyzed. Results Twenty-five patients with 30 episodes of respiratory insufficiency were treated with BiPAP ventilation with median duration of 1.96 days ranged from 0.03 to 12 days. Of these respiratory failure episodes, 25 ones (83.3%) could be controlled by BiPAP and the needs of re-intubation were avoided. Five episodes of respiratory failure in 4 patients could not be quelled and the endo-tracheal tubes were inserted in these patients. All patients were saved with a median of mechanical ventilation duration of 3.4 days and ICU stay of 10.6 days. No major complications were observed. The heart rate, respiratory rate and the rate-pressure product were decreased significantly one hour after BiPAP (P < 0.05 all). Meanwhile, patients showed rapid improvement of oxygenation. The pH, SpO>2 and PaO2/FiO2 were increased significantly and A-aDO2 was decreased significantly (P < 0.05 all). The PaCO2, was decreased significantly four hours after BiPAP (P < 0.05). Conclusions Non-invasive nasal mask BiPAP can be used safely and effectively in children after cardiac surgery to improve oxygenation/ventilation, decreasing the work of breathing. It may be particularly useful in patients with high risk of re-intubation.
RESUMO
Persistent pulmonary hypertension of newborn (PPHN) is a clinical syndrome,characterized by pulmonary arterial pressure elevated relative to systemic blood pressure,leading to extrapulmonary right-to-left shunting of blood across the ductus arteriosus and foramen ovale,causing severe hypoxemia.Pulmonary vascular remodeling is pathognomonic of hypoxia-induced PPHN.The regulatory mechanism and cell composition of vascular wall were dysfunction,which led to vascular wall thickened.The review focus on three aspects,such as endothelial cells,smooth muscle cells,and adventitial cells,described the relation and the possible mechanisms between hypoxia-induced PPHN and pulmonary vascular remodeling.
RESUMO
Objective To compare the effects of oral Sildenafil(SIL)and intravenous prostaglandin E_1(Alprostadil,PGE_1)in treatment of postoperative pulmonary hypertension(PH)associated with congenital heart defects(CHD).Methods Data were collected from 24 children with postoperative mPAP≥35 mmHg in our hospital between August 2004 and March 2005.These children were randomly divided into three groups:8 children(group A)initially received SIL(0.35 mg/kg,orally by nasal gastric tube),followed by the addition of intravenous PGE_1(20 ng/kg per min)at 40 min.The second group of 8 children(group C)initially received intravenous PGE_1 followed by the addition of SIL at 20 min(dose as above).And remains(group B)were placebo-controlled.The changes of hemodynamic variables,arterial blood gas,lung static compliance(C)and work of breathing(W)were measured after drugs were given.Results Compared with group B,the mPAP and mPAP/mSAP was significantly reduced(P
RESUMO
<p><b>OBJECTIVE</b>To establish the specific 16S-23S rRNA gene spacer regions in different bacteria using polymerase chain reaction (PCR), restriction fragment length polymorphism (RFLP), DNA cloning and sequences analysis.</p><p><b>METHODS</b>A pair of primers were selected from highly conserved sequences adjacent to the 16S-23S rRNA spacer region. Bacterial DNA from sixty-one strains of standard bacteria and corresponding clinical isolates representative of 20 genera and 26 species was amplified by PCR, and further analyzed by RFLP, DNA cloning and sequences analysis. Furthermore, all specimens were examined by bacterial culturing and PCR-RFLP analysis. The evaluation of these assays in practical clinic practice was also discussed.</p><p><b>RESULTS</b>Restriction enzyme analysis revealed one, two or three bands or more observed among the 26 different standard strains. The sensitivity of PCR reached 2.5 colony-forming unit (CFU), and there was no cross reaction with human genomic DNA, fungus or virus. Fourteen species could be distinguished immediately by PCR, while another 10 species were further identified by Hinf I or Alu I digestion. The only difference between K.pneumoniae and E. durans was located at the site of the 779th nucleotide according to the sequence analysis and only XmaIII digestion could distinguish one from another. Of 42 specimens from septicemic neonates, 15 were identified as positive by blood culture at a rate of 35.7%. However, 27 specimens identified as positive by PCR, with a rate of 64.2%, a method significantly more effective than blood culture (P < 0.01). Of 6 cerebrospinal fluid (CSF) specimens, one tested positive for S.epidermidis was also positive by PCR, two culture negative were positive by PCR and diagnosed as S.epidermidis according to the DNA pattern. One positive for C.neoformans was negative by PCR. The other two specimens were negative by both PCR and culture.</p><p><b>CONCLUSIONS</b>The method of detecting bacterial 16S-23S rRNA spacer regions using PCR-RFLP techniques was specific, sensitive, rapid and accurate in providing a new technique for detecting pathogens in clinical bacterial infections.</p>
Assuntos
Humanos , Bactérias , Genética , DNA Bacteriano , Química , DNA Ribossômico , Química , Genes de RNAr , Reação em Cadeia da Polimerase , Polimorfismo de Fragmento de Restrição , RNA Ribossômico 16S , Genética , RNA Ribossômico 23S , Genética , Sensibilidade e Especificidade , Análise de Sequência de DNARESUMO
<p><b>OBJECTIVE</b>To determine whether lead affects brainstem auditory evoked potentials (BAEPs) in low-to-moderate lead exposed children.</p><p><b>METHODS</b>BAEPs were recorded from 114 asymptomatic children aged 1 - 6 years. Average values were calculated for peak latency (PL) and amplitude (Amp). Whole blood lead (PbB) levels were assessed by graphite furnace atomic absorption spectroscopy. Based on their PbB levels, subjects were divided into low lead (PbB < 100 micro g/L) and high lead subgroups (PbB > or = 100 micro g/L).</p><p><b>RESULTS</b>The PbB levels of the 114 subjects ranged from 32.0 to 380.0 micro g/L in a positively skewed distribution. The median of PbB levels was 90.0 micro g/L while the arithmetic average was 88.0 micro g/L. Of the subjects, 43.0% (49/114) had levels equal to or greater than 100 micro g/L. Bilateral PLs I, V, and III of the left ear in the high lead subgroup were significantly longer than those in the low lead subgroup (P < 0.05). A positive correlation was found between PbB levels and bilateral PLs I, V and III of the left ear (P < 0.05), after controlling for age and gender as confounding factors. A significant and positive correlation between PbB levels and PL I of the left ear, even when PbB levels were lower than 100 micro g/L, in the low subgroup (r = 0.295, P = 0.019) was also found.</p><p><b>CONCLUSIONS</b>Lead poisoning in children younger than 6 years old is a very serious problem to which close attention should be paid. The indications that lead prolongs partial PLs may imply that lead, even at PbB levels lower than 100 micro g/L, impairs both the peripheral and the central portions of the auditory system. BAEPs may be a sensitive detector of subclinical lead exposure effects on the nervous system in children.</p>
Assuntos
Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Potenciais Evocados Auditivos do Tronco Encefálico , Chumbo , Sangue , Toxicidade , Intoxicação por ChumboRESUMO
Objective: To investigate the changes of haemodynamics, pulmonary mechanics and blood gas in volume controlled and pressure controlled ventilatory patterns during one-lung ventilation (OLV). Methods: 20 patients with patent ductus arteriosus (PDA) underwent left thoracotomy PDA ligation with right OLV. The patients were divided into three groups: two-lung ventilation with volume controlled (TLV-VCV), one-lung ventilation with volume controlled (OLV-VCV), and one-lung ventilation with pressure controlled (OLV-PCV). After two-lung ventilation with VCV, one-lung ventilation was started by VCV and the ventilation mode was then switched to PCV. All measurements were made 25 min after initiation of the ventilation mode. The respiratory mechanics index was measured by side stream spirometry (SSS), including peak airway pressure (Ppeak), plateau pressure (Pplat), airway resistance (Raw), lung compliance (Cdyn) and inspiratory and expiratory minute ventilation (Mvi, Mve). Cardiac output (CO), stroke volume (SV), systemic vascular resistance (SVR), left ventricular ejectiontime (LVETi), and aortic blood flow acceleration (ACC) were also measured, by using the transesophageal Doppler (TED) monitor. Arterial blood gases was determined in every ventilation mode. Results: Ppeak、Pplat and Raw were significantly higher during OLV-VCV than that during TLV-VCV (P
RESUMO
AIM: To evaluate the contribution of inducible nitric oxide synthase (iNOS) and nitrotyrosine to acute lung injury (ALI) in rats with meconium aspiration. METHODS: 16 health male Sprage-Dawley rats were randomized to control group and meconium group, followed by intratracheally administration of 1 mL/kg saline or 1 mL/kg 20% human newborn meconium suspension. The animals were killed after 24 h of treatment. The measurements included bronchoalveolar lavage fluid (BALF) cell count, pulmonary myoloperoxidase (MPO) activity and nitric oxide (NO) level. Western bloting was used to determine the expression of pulmonary nitrotyrosine-a specific “footprint” of peroxynitrite and iNOS. RESULTS: Compared to control group, the rats in the meconium group had increased BALF cell counts (4.04?1.01)?10~9cells/L vs (0.53?0.19)?10~9cells/L:, pulmonary MPO activity (1.49?0.22)U/g wet lung tissue vs (0.62?0.16) U/g wet lung tissue:, NO level (12.77?5.00) mmol/g protein vs (4.89?1.32) mmol/g protein:, increased expression of nitrotyrosine and iNOS (0.46?0.19 and 1.49?0.60 vs 0.15?0.04 and 0.09?0.04, respectively), all P
RESUMO
AIM: To evaluate the role and mechanisms of recombinant human superoxide dismutase (rhSOD) in meconium-induced acute lung injury (ALI) by evaluating pulmonary MIP-1? and NF-?B expression. METHODS: 24 health male Sprage-Dawley rats were randomized to 3 groups (8, each group), followed by intratracheal (IT) administration with (1) saline at (1 mL/kg) (control group); (2) 20% human newborn meconium suspension at 1 mL/kg, followed by saline at 1 mL/kg (Mec/saline group); (3) 20% human newborn meconium suspension at 1mL/kg, followed by rhSOD at 20 mg/kg (Mec/rhSOD group). The animal was killed 24 h after treatment. The measurements included the bronchoalveolar lavage (BAL) cell count, RT-PCR analysis of pulmonary MIP-1? mRNA expression, Western blotting analysis of pulmonary NF-?B expression. RESULTS: Meconium-induced ALI was characterized by increased BAL cell count, increased expressions of pulmonary MIP-1? mRNA and NF-?B protein [(4.68?1.40)?10~9 cells/L vs (0.53?0.19)?10~9 cells/L, 3.60?0.75 vs 1.56?0.33, 0.72?0.31 vs 0.23?0.12, respectively in control rats, all P
RESUMO
AIM: To evaluate effects of inhaled nitric oxide(iNO) on adhesion molecule CD11b expression on lung neutrophils in experimental meconium aspiration syndrome(MAS) rabbits treated with conventional mechanical ventilation under room air or 100%O 2. METHODS: Animals were randomly allocated to 8 groups( n= 48) of 6 each: two MAS model groups(under room air or 100%O 2 without iNO treatment), 6 treatment groups were treated with continuous NO inhalation at a dose of 0.2?10 -6 mol/L,0.33?10 -6 mol/L or 0.67?10 -6 mol/L respectively for 12 hours under room air or 100%O 2. Mean systemic arterial pressure(SAP) and methemoglobin (MeHb) were performed at basement time, 0, 2, 4, 12 hours. Expression of CD11b on neutrophils in the bronchoalveolar lavage fluid(BALF) was detected with flow cytometry. RESULTS: SAP, MeHb at different time among different groups were within the normal scale. CD11b expression on the neutrophils in the BALF significantly decreased in groups of inhalation 0.33?10 -6 mol/L or 0.67?10 -6 mol/L NO, compared with the two MAS model groups. ( ?s : under 21%O 2, 0.33?10 -6 mol/L NO,121?20 vs 392?204; 0.67?10 -6 mol/L NO,112?30 vs 392?204;under 100%O 2,0.33?10 -6 mol/L NO,113?24 vs 293?65; 0.67?10 -6 mol/L 102?114 vs 293?65, P 0 05). No statistic difference was observed between groups inhaled 0.33?10 -6 mol/L NO and 0.67?10 -6 mol/L NO. CONCLUSION: 0.33?10 -6 mol/L or 0.67?10 -6 mol/L NO inhalation down-regulated the CD11b expression on the neutrophils in BALF to reduce the sequestration of neutrophils in rabbit lung.