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.

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.
Adult ; Diabetes Insipidus, Nephrogenic/genetics* ; Exons ; Female ; Frameshift Mutation ; Humans ; Hydrochlorothiazide/therapeutic use* ; Infant, Newborn ; Male ; Pedigree ; Receptors, Vasopressin/genetics*

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.

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.

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.

Objective:To compare the clinical diagnosis and outcomes of preterm infants with bronchopulmonary dysplasia (BPD) under two different diagnostic criteria.Methods:A retrospective study was performed in 157 preterm infants who were admitted to Neonatal Intensive Care Unit of the Children′s Hospital, Zhejiang University School of Medicine from January 2015 to December 2018. Enrolled infants, with gestational age <32 weeks and survived >14 days, met the 2001 National Institute of Child Health and Human Development(NICHD) definition of moderate and severe BPD or died between 14 days of postnatal age and 36 weeks owing to persistent parenchymal lung disease and respiratory failure. The severities of BPD were revaluated according to the 2018 revised definition of BPD proposed by NICHD. Characteristics and outcomes of these infants were compared with the two different diagnostic criteria with t-test, nonparametric test or Chi-square test. Results:In the 157 enrolled infants (100 males), severities of BPD were classified as moderate in 62, severe in 84 and unclassifiable in 11 according to the 2001 NICHD criteria, while grade Ⅰ in 51, Ⅱ in 29, Ⅲ in 66 and ⅢA in 11 infants respectively according to the 2018 NICHD criteria. Duration of oxygen therapy, positive pressure ventilation and endotracheal intubation in grade Ⅱinfants of 2018 criteria were much longer than that in moderate infants of 2001 criteria (80 (65, 95) vs. 65 (59, 77) d, 52 (38, 58) vs.30 (19, 48) d, 10 (2, 17) vs.4 (0, 12) d, Z=-2.995, -3.750, -2.073, all P<0.05). Mortality of moderate and severe infants in 2001 criteria was 10.3% (15/146), while mortality of BPD in 2018 criteria was 16.6% (26/157). Mortality of grade Ⅲ and ⅢA BPD in 2018 criteria was much higher than mortality of severe BPD in 2001 criteria (33.8% (26/77) vs. 17.9%(15/84), χ 2=5.357, P<0.05). Conclusion:Definition and classification of BPD based on 2001 NICHD criteria may cause missed or unclassified cases, resulting in the underestimation of the morbidity and mortality of infants with severe BPD.

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.

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.

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.

Objective To summarize clinical experience of using extracorporeal membrane oxygenation (ECMO) in rescuing children with acute fulminant myocarditis (AFM).Method Data of 12 children with acute fulminate myocarditis (6 boys and 6 girls,median age 8.3 (0.6,13.0) years,median weight 33.1 (6,61) kg) who were rescued with ECMO in Children's Hospital,Zhejiang University from September 2009 to August 2015 were analyzed retrospectively.The analysis focused on the intervene timing of ECMO for the cardiogenic shock and hypoperfusion caused by heart failure and(or) lethal arrhythmia and the essentials of ECMO cardiopulmonary resuscitation (ECPR) for cardiac arrest in pediatric AFM were summarized.Result The median ECMO duration was 110(22,240) h.Ten cases survived and 2 were dead of the total of 12 patients.Six ECPR patients survived and 2 were dead in the total of 8 ECPR patients.The complication of 10 survivors were cannula site bleeding (3 cases),hypematremia and intracranial hemorrhage (1 case),limping (1 case),hoarse voice (1 case),and cerebral injury (1 case).Conclusion The key points of improving ECMO rescuing outcome for the AFM children are grasping the ECMO intervene timing and training skilled ECMO team.For ECPR patients,keeping effective chest compressions resuscitation is the key to achieve survival and improve the quality of life.