Neonatal hyperinsulinism/hyperammonemia syndrome is a genetic disease result from glutamate dehydrogenase gene mutations.The clinical manifestations are hypoglycemia,hyperinsulinemia and mild hyperammonemia.Hypoglycemia may occur quickly due to eating protein.It is a rare neonatal disease that was easily ignored or delayed diagnosis and treatment causing serious sequelae of nervous system.This review summarized pathogenesis,clinical manifestation and diagnosis of the disease.

As a new group of stem cells found in the human breast in the 21st century, stem cells in human breast milk have the potential to differentiate into ectoderm, mesoderm, and endoderm cells.It is not only rich in sources, but also can be obtained in a non-invasive way.At present, there are relatively few ethical disputes.First, as a potential source of stem cells, it has a great prospect in the field of alternative medicine and regenerative medicine.Second, it can promote newborns early growth and development, repair the damaged tissues, and prevent as well as treat some early diseases.This review summarized stem cells from human breast milk, from aspects of distribution, acquisition, and functional characteristics, and discussed their application in newborns.

Objective:To compare the clinical characteristics of congenital chylothorax in preterm and term infants.Method:From January 2011 to December 2019, the clinical data of infants with congenital chylothorax admitted to our hospital were retrospectively analyzed. The infants were assigned into preterm group (<37 weeks) and term group (≥37 weeks) according to their gestational age. The general information, clinical manifestations, laboratory results, treatment and prognosis of the two groups were compared.Result:A total of 34 infants with congenital chylothorax were included, including 11 premature infants and 23 term infants. No significant differences existed in gender, delivery mode, prenatal diagnosis of pleural effusion, congenital heart disease/chromosome abnormality, birth asphyxia, dyspnea, fetal edema, and location of effusion between the two groups ( P>0.05). Compared with term group, preterm group had significantly fewer leukocytes [3 245(1 007, 7 403)×10 6/L vs. 10 214(6 233,16 458)×10 6/L] and lower protein level [(28.1±7.6) g/L vs. (33.3±6.3) g/L] in the pleural fluid ( P<0.05). No significant differences existed in the proportion of pleural lymphocytes between the two groups ( P>0.05). The proportion of mechanical ventilation (MV) in the preterm group was statistically higher than that the term group [100%(11/11) vs. 65.2%(15/23)], and the duration of MV was statistically longer than the term group [(16(10,25) d) vs. (1(0,11) d)] ( P<0.05). No significant differences existed between the two groups in the application of other treatment options (thoracentesis/drainage, fasting, octreotide and erythromycin pleural injection), time needed for the disappearance of effusion, duration of hospital stay and cure/improvement rate ( P>0.05). Conclusion:Preterm infants may have lower leukocyte count and protein level in the pleural effusion than the term infants. Both preterm and term infants have higher proportion of lymphocytes in the pleural effusion fluid. Although most preterm infants need ventilator support after delivery, most of them achieve complete remission after conservative treatment and the overall prognosis is as good as term infants.

Objective:Induced pluripotent stem cells (iPSCs) cell lines were established using peripheral blood mononuclear cells (PBMCs) from a patient suffering from neonatal respiratory distress syndrome (NRDS) who carried Adenosine triphosphate-binding cassette transporter A3 ( ABCA3) compound heterozygous mutations. Methods:Cell experimental research.Peripheral venous blood was collected and PBMCs were isolated and cultured in vitro. PBMCs were transfected with non-integrated Sendai vector carrying reprogramming factors.The chromosome karyotypes of the established iPSCs were analyzed.Immunofluorescence and flow cytometry were used to detect pluripotency markers of stem cells and verify their differentiation potential.Sanger sequencing was performed to analyze gene mutations.In addition, short tandem repeat (STR) analysis was performed, polymerase chain reaction(PCR) and agarose gel electrophoresis were used to detect virus residual. Results:Karyotype analysis of established iPSCs cell lines showed normal diploid 46, XY karyotype.Immunofluorescence showed positive staining of stem cell pluripotency markers OCT4, SSEA4, Nanog and Sox2.Flow cytometry was used to detected stem cell pluripotency markers and showed expression of TRA-1-60, SSEA-4 and OCT4.After differentiation into all three germ layers, immunofluorescence was performed to detect ectoderm (Pax-6), mesoderm (Brachyury) and endoderm alpha-fetoprotein markers, and the results showed positive staining, which confirmed that the iPSCs had the potential to differentiate.Sanger sequencing showed c. 3997_3998del and c. 3137C>T compound heterozygous mutations.STR analysis showed they originate from PBMCs, and no Sendai virus residual was detected by PCR and agarose gel electrophoresis.Conclusions:In this study, PBMCs from patient carrying ABCA3 compound heterozygous mutations was used to establish iPSCs cell lines.The research lays a foundation for the study of pathogenesis, therapeutic drug screening and cell therapy of NRDS caused by ABCA3 gene mutations.

The cultivation of medical humanistic quality is indispensable in the standardized training of pediatric residents, and it is urgent to explore new educational methods to improve their medical humanistic quality level. In this study, 60 standardized pediatricians participated in the standardized training, 36 in the experimental group received innovative interactive medical humanities education, while 24 in the control group were set up to receive traditional medical humanities education. Short-term and long-term test scores were conducted by questionnaire at the beginning of the standardized training and 2 years later. The results showed that there was no significant difference between the experimental group and the control group in the self-scores of professional quality, moral cultivation, communication skills, legal knowledge and innovative spirit (P>0.05) , but the scores of teaching teachers were improved except innovative spirit (P<0.05) . In addition, compared with the control group, the number of pediatricians with professional honor increased, the doctor-patient communication ability improved, the medical disputes reduced, and the family satisfaction improved in experimental group were increased (P<0.05) . These results indicated that innovative interactive medical humanistic education is an effective method to improve the medical humanistic quality of pediatric residents in standardized training.