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Objective:To investigate the clinical features, treatment, and prognosis of transient hyperammonemia of the newborn (THAN).Methods:Data of two infants with severe THAN admitted to the Department of Neonatology of Shanghai Children's Hospital in September 2021 and August 2022 were retrospectively investigated. Clinical data of confirmed THAN cases (blood ammonia>400 μmol/L) were collected from relevant literature retrieved from the Wanfang Database, China National Knowledge Infrastructure, Chinese Medical Journal Database, and PubMed up to July 2022. A descriptive method was used for statistical analysis.Results:A total of 24 cases were involved (two in the present study, and 22 in 12 retrieved articles), including 19 (79.2%) premature newborns and five term infants. The average birth weight was (2 237±608) g and the average onset time was 27 h (4-55 h) after birth. The early clinical symptoms included respiratory distress and hyporesponsiveness (drowsiness, lethargy, coma or hypotonia) in 18 cases (75.0%), metabolic acidosis in 11 cases (45.8%), hypocalcemia in seven cases (29.2%), pupil fixation/dilation in six cases (25.0%), convulsion in five cases (20.8%), apnea in three cases (12.5%) and sinus bradycardia in one case (4.2%). The serum ammonia levels were 1 422.8 μmol/L (547.2-4 494.1 μmol/L). Treatments included peritoneal dialysis plus exchange transfusion in eight cases (33.3%), exchange transfusion in seven cases (29.2%), continuous renal replacement therapy (CRRT) in four cases (16.7%), arginine in two cases (8.3%), peritoneal dialysis in two cases (8.3%), and CRRT+peritoneal dialysis in one case (4.2%). During follow-ups of four months (one month to six years), 13 cases (54.2%) showed no abnormalities in development; two (8.3%) had a neurodevelopmental delay, and six (25.0%) died. The follow-up of the other three cases (12.5%) were not reported in the literature.Conclusions:The early clinical manifestation of severe THAN is atypical. A good prognosis can be expected through early exclusion of possible hyperammonemia-related genetic metabolic diseases and lowering the serum ammonia level. Long-term follow-up is needed for neurological evaluation.
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Objective:To investigate the effectiveness and advantage of visual laryngoscope in the treatment of patients with sudden cardiac arrest who need spontaneous respiration tracheal intubation.Methods:Totally 60 patients who suffered from cardiac arrest and needed spontaneous respiration tracheal intubation were enrolled from June 2020 to February 2021 in the Affilicated Hospital of Chifeng University. Theywere randomlydivided into two groups-traditional laryngoscope (TL) group and visual laryngoscope (VL) group, with30 patients in each group. Then the success rate of glottis exposure, the operative time, success rate and complication rate of tracheal intubation were compared between the two groups. Subgroup assessment between the junior emergency resident doctor (A group) and the senior emergency resident doctor (B group) was conducted.Results:According to Cormack Lehan grades, the success rate of glottis exposure in VL group was higher than that in TL group ( P>0.05), and the success rate of Grade I in VL group was significant higer than that in TL group: 56.70%(17/30) vs. 30.00%(9/30), P<0.05. The trial times of successful intubation cases and the operative time of successful intubation cases were significantly less than those in TL group (1.30 vs 1.67, P = 0.049) and (56.37 s vs 67.12 s, P<0.05). In the subgroup, the one-time success rate of tracheal intubation in A-TL group was significantly lower than that in B-TL group (4/15 vs. 11/15, P<0.05), while the one-time success rate of tracheal intubation in A-VL group was 60.00%, which is lower than that in B-VL group ( P>0.05). The operative time consumed for successful intubation in A-TL group was significantly longer than that in B-TL group: 78.00 s vs. 55.57 s, P<0.05, while the operative time in A-VL group was a little longer than that in B-VL group ( P>0.05). Conclusions:Visual laryngoscope used in spontaneous respiration tracheal intubation can not only increase the success rate of glottis exposure, decrease trial times and shorten operative time of intubation, but also improve the success rate and decrease the complication rate of emergency tracheal intubation.
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Objective:To study the correlations of neonatal hemodynamic parameters with gestational age (GA) and birth weight (BW) using non-invasive ultrasound cardiac output monitor (USCOM).Method:From March to September 2019, neonates with stable hemodynamics admitted to the Department of Neonatology of our hospital were enrolled in this prospective study. According to their GA, they were assigned into <29 w group, 29~33 w group, 34~36 w group and ≥37 w group. According to their BW, they were assigned into <1 000 g group, 1 000~1 499 g group, 1 500~2 499 g group and ≥2 500 g group. Cardiac output (CO), cardiac index (CI), stroke volume (SV), myocardial contractility (inotropy, INO), flow time corrected (FTC), systemic vascular resistance index (SVRI) and heart rate (HR) were measured using USCOM. The univariate linear regression method was used to analyze the correlation of hemodynamic parameters with different GA and BW.Result:A total of 120 neonates with stable hemodynamics were enrolled, including 69 males and 51 females. The average GA was (34.2±3.8)w and the average BW was (2 221±860) g. SV ( r=0.489, P<0.001), CO ( r=0.681, P<0.001), CI ( r=0.348, P<0.001), FTC ( r=0.266, P=0.003), INO ( r=0.446, P<0.001)and HR ( r=-0.322, P<0.001) showed significant linear correlations with GA. No linear correlation existed between SVRI ( r=-0.052, P=0.574) and GA. SV ( r=0.603, P<0.001), CO ( r=0.852, P<0.001), CI ( r=-0.390, P<0.001), INO ( r=0.576, P<0.001) and HR ( r=-0.440, P<0.001) showed significant linear correlations with BW. No significant linear correlations existed between SVRI ( r=-0.076, P=0.409) or FTC ( r=0.090, P=0.329) and BW. Conclusion:USCOM can monitor neonatal hemodynamic parameters in real-time.Hemodynamic parameters including SV, CO, CI and INO are significantly different among newborns with different GA and BW and these parameters are linearly correlated with GA and BW.
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Objective:To study the gene expression of nuclear factor erythroid-2-related factor 2 (Nrf2), glutathione-S-transferase (GST) and interleukin-1 β (IL-1β) in A549 cells exposed to hyperoxia and cell apoptosis after siRNA interference with Nrf2.Method:Normal A549 cell lines were assigned into normoxia+siRNA group, normoxia+control group, hyperoxia+siRNA group and hyperoxia+control group according to whether siRNA interference was used and the exposure environment (normoxia/hyperoxia). The hyperoxia environment contained 95%O 2 and 5%CO 2. The levels of mRNA expression of Nrf2, GST and IL-1β were detected using quantitative real-time polymerase chain reaction (qRT-PCR). Flow cytometry was used to examine cell apoptosis of the hyperoxia+control group and hyperoxia+siRNA group at different time points. Analysis of variance (ANOVA) was used to test the relative gene expression and apoptosis of A549 cells. Result:(1) Compared with the normoxia+control group, the expression of Nrf2 and GST in the hyperoxia+control group was significantly increased ( P<0.05), and the expression of IL-1β was significantly decreased ( P<0.05); the expression of Nrf2 and GST in the normoxia+siRNA group decreased significantly ( P<0.05), while the expression of IL-1β increased significantly ( P<0.05). (2) Compared with the normoxia+siRNA group, Nrf2 expression in the hyperoxia+siRNA group showed no significant changes ( P=0.230), GST expression increased slightly ( P=0.057), and IL-1β expression decreased slightly ( P=0.112). (3) Compared with the hyperoxia+control group, the expression of Nrf2 and GST in the hyperoxia+siRNA group decreased significantly ( P<0.05), and the expression of IL-1β increased significantly ( P=0.042). (4) Compared with the hyperoxia+control group, the apoptosis of A549 cells in the hyperoxia+siRNA group increased significantly at 24 h, 48 h and 72 h ( P<0.05). Conclusion:After interfering with Nrf2, siRNA may regulate the expression of GST and IL-1β, preventing oxidative stress, reducing inflammatory response and inhibiting apoptosis.
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Objective:To compare the effect of SMOF lipids composed of soybean oil, medium chain triglycerides, olive oil, and fish oil with medium-long chain mixed fat emulsions(Lipofundin) on parenteral nutrition-associated cholestasis(PNAC) in premature infants.Methods:Clinical data were collected from premature infants hospitalized in the neonatal intensive care unit of Shanghai Children′s Hospital from January 2018 to December 2019 with gestational age ≤34 weeks, birth weight ≤2 000 g, and duration of parenteral nutrition ≥14 days.They were devided into SMOF lipid group and Lipofundin group, and the incidence of PNAC, neonatal necrotizing enterocolitis(NEC), bronchopulmonary dysplasia(BPD), retinopathy of prematurity(ROP), periventricular-intraventricular hemorrhage(PVH-IVH), late-onset sepsis and liver function were compared between two groups.Results:The incidence of PNAC in the SMOF lipid group was significantly lower than that in Lipofundin group( P=0.042). The average level of ALT and AST in SMOF lipid group were markedly lower than those in Lipofundin group( P<0.05). The time to reach full enteral feeding of SMOF lipid group was shorter than that of Lipofundin group( P=0.005). There was no significant difference in the incidence of NEC, BPD, ROP, PVH-IVH, and late-onset sepsis between two groups( P>0.05). Conclusion:Compared with lipofundin, SMOF lipid can reduce the incidence of PNAC in premature infants, and has no significant effect on the incidence of NEC, BPD, ROP, PVH-IVH and late-onset sepsis.
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Objective:To investigate the clinical and molecular genetic characteristics of 6q24-related transient neonatal diabetes mellitus (6q24-TNDM).Methods:The clinical data of two neonates with 6q24-TNDM admitted to Shanghai Children's Hospital, Shanghai Jiao Tong University in 2017, were retrospectively collected. The methylation levels of 16 cytidine-phosphate-guanosine (CpG) sites from the methylated differentially modified region (DMR) in 6q24 were quantitatively analyzed by pyrosequencing.Results:Case 1, aged 5 d, was born at 37 +4 gestational weeks due to fetal growth restriction, and case 2 was 11-days old and born at 38 +2 gestational weeks. Both infants were male and small for age. They were born through a cesarean section. The birth weight of case 1 and case 2 were 2 340 g and 2 600 g, respectively. They were admitted due to hyperglycemia with blood glucose of 12.95 and 8.00 mmol/L on admission, respectively. Physical examination showed slightly poor skin elasticity and thin subcutaneous fat. Laboratory examination revealed lower serum insulin (<1.39 and 3.94 pmol/L) and peptide C (0.05 and 0.14 nmol/L) levels, positive results of urine glucose, negative tests for urine ketone, serum anti-glutamic acid decarboxylase antibody, anti-insulin antibody, and islet cell antibody in both cases. Normal size of the pancreas was observed by ultrasonography. The infants were improved and were discharged after subcutaneous insulin infusion for more than two weeks. The treatment was discontinued at 69 d and 42 d postnatally for case 1 and case 2. Prenatal diagnosis of the two infants showed normal karyotypes and uniparental disomy of chromosome 6 indicated by single nucleotide polymorphism chip. No pathogenic mutations were detected by next-generation sequencing after admission. The methylation levels of 16 CpG sites in DMR of 6q24 in the two cases, which were quantitatively analyzed by pyrosequencing, were lower than 10% (normal value in healthy matched controls: 40%), indicating an obvious hypomethylation. Conclusions:For children with TNDM who are small for gestational age at birth, presenting hyperglycemia with decreased serum insulin and C-peptide levels, pyrosequencing can be used to quantitatively analyze the methylation levels of CpG sites in 6q24 DMR, which can quickly and directly assist in the diagnosis of 6q24-TNDM, thereby contributing to the treatment and prognosis assessment.
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Objective:To investigate the clinical and molecular genetic features of neonatal congenital Netherton syndrome (NS) caused by mutations in serine protease inhibitor of Kazal type 5 ( SPINK5) gene. Methods:This study retrospectively analyzed the clinical data of an NS neonate admitted to Shanghai Children's Hospital in November 2018. SPINK5 gene was analyzed using high-throughput sequencing and Sanger sequencing. Relevant articles were retrieved from various databases including China National Knowledge Infrastructure, Wanfang and PubMed, and the reported cases who were diagnosed as NS within two months after birth with SPINK5 gene sequencing results were reviewed. Clinical features, gene mutations, treatment and follow-up results of NS were summarized using descriptive statistical analysis. Results:The patient presented with diffuse erythema and desquamation, sparse hair and repeated infections shortly after birth. Laboratory tests revealed elevated IgE (111 IU/ml) and "invagination-like" change in the hair under optical microscope. SPINK5 gene analysis found that there were compound heterozygous mutations of c.2468dup (p.Lys824Glufs*4) and c.377_378del (p.Tys126*) in the child. The pedigree analysis found that the two mutations were respectively inherited from the father and the mother, which supported the diagnosis of NS caused by SPINK5 gene mutation. Though skin rash improved after comprehensive treatments including anti-infection therapy, gamma globulin injection and skincare, the patient suffered from recurrent infection and was discharged from the hospital after giving up treatment and died of infection at two months old. Eleven NS cases were retrieved from literature and altogether 12 cases were analyzed here. The most common clinical manifestations in the 12 patients were early skin diffuse erythema and desquamation (12/12), infection (8/12), dry hair (7/12), hypernatremia dehydration (7/12), high IgE (5/12), growth retardation (4/12), respiratory failure (3/12), atopic constitution (2/12), diarrhea (2/12), dysphagia (1/12), hypothermia (1/12), wheezing (1/12), hypertension (1/12), liver failure (1/12) and metabolic alkalosis (1/12). Conclusions:NS is caused by SPINK5 gene mutation with atypical manifestations in neonates. Neonates with diffuse erythema and desquamation of the skin, repeated infections, dry hair and especially with high blood IgE should be considered the possibility of NS. Genetic testing is conducive to early diagnosis, guiding treatment decisions and providing a basis for genetic counseling.
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Objective:To investigate the expression of long non-coding RNA MALAT1, interleukin 6(IL-6) and apoptosis induced factor(AIF) in peripheral venous blood of premature infants with bronchopulmonary dysplasia (BPD) and its clinical significance.Methods:Preterm infants admitted to the Department of Neonatology, Shanghai Children′s Hospital from January 2015 to December 2016 were enrolled.The selection criteria included gestational age (GA) ≥28 weeks and ≤32 weeks, and birth weight (BW) < 1 500 g. According to the diagnosis, they were divided into BPD group (20 cases) and control group (20 cases). The clinical data of the two groups of premature infants were collected and analyzed, and the levels of MALAT1, IL-6 and AIF in the blood of 40 premature infants were detected by real-time polymerase chain reaction (RT-PCR). T test was used to compare gestational age, birth weight, MALAT1, IL-6 and AIF between the two groups. Results:(1)There was no significant differences in sex ( χ2=1.76), gestational age ( t= 0.17) and birth weight ( t=1.25) of premature infants in BPD group, compared with the control group (all P >0.05). (2)Compared with the control group, the expression of MALAT1 in the peripheral blood of premature infants in BPD group were significantly increased (0.273 4±0.067 3 vs. 0.375 5±0.081 9, P<0.05). (3)Compared with the control group, the expression of IL-6 in the peripheral blood of premature infants in BPD group were obviously decreased (1.448 8±0.191 8 vs.4.444 6±0.165 7, P<0.05). (4)Compared with the control group, the expression of AIF in the peripheral blood of premature infants in BPD group were remarkably decreased(0.006 8±0.002 0 vs.0.004 5±0.001 9, P<0.05). Conclusions:MALAT1 and IL-6 levels of long non-coding RNA in BPD and non-BPD preterm infants are different, which may be related to the incidence of BPD.IL-6 may be a predictor of BPD, and MALAT1 may protect premature infants with BPD.
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Objective To evaluate the safety,feasibility,complications and outcome of continuous renal replacement therapy (CRRT) in neonates weighting less than 3 000 g.Method A total of 6 neonates weighting less than 3 000 g treated with CRRT in the Department of Neonatology,Shanghai Children's hospital,from January 2015 to December 2017 were studied.The birth weight,primary disease,indications of CRRT,treatment duration,age,complications and outcome of the neonates were collected and analyzed.Serum creatinine (Scr),blood urea nitrogen (BUN) and blood ammonia were analyzed before and after CRRT.T test was used for statistical analysis of the data.Result (1) Among the 6 neonates,2 were full-term infants and 4 were premature infants.The average gestational age of the neonates was (35.0± 2.1) weeks and the average birth weight was (2 542±586) g.(2) The catheterization was successful in all of the 6 neonates.The model for CRRT was continuous veno-venous hemofiltration dialysis,and the duration was 50(48,154)h,the neonates' age of CRRT was 3.0(2.0,4.5)days.The primary disease included 3 perinatal asphyxia,1 hemolytic uremic syndrome,1 ornithine transcarboxylase deficiency,1 jejunal atresia.There were 5 patients with acute kidney injury and fluid overload,and another one with hyperammonemia.(3) Compared with before CRRT,serum creatinine,urea nitrogen and serum ammonia all decreased significantly and reached the normal range after CRRT.(4)The complications of CRRT in the 6 neonates included 2 hypotension,1 hypokalemia,1 hypocalcemia and 1 hypophosphatemia.Catheter related infection,blockage and other complications had not occurred.(5) After treatment,3 patients survived,1 witdrew and 2 died.Conclusion The application of CRRT in neonates with weight less than 3 000 g is safe and feasible,the prognosis and survival rate of which can be improved with fewer and controllable complications.
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Objective To study the clinical efficacy and safety of extracorporeal membrane oxygenation (ECMO) in critically ill neonates.Method From November 2016 to September 2018,the clinical data of 5 cases who received ECMO treatment in NICU of our hospital were retrospectively analyzed.The indication of ECMO was reversible respiratory failure irresponsive to conventional therapy.The treatment mode was V-A ECMO.Oxygenation index (OI),vasoactive-inotropic score,blood lactate before and 24 h after ECMO were recorded.Complications of ECMO were also studied.Paired t-test was used to compare the pre and post treatment parameters.Result Among the 5 cases,4 cases were male and 1 case was female.3 cases were diagnosed with meconium aspiration syndrome,2 cases pulmonary hypertension.OI[(9.5 ± 1.8) vs.(60.6 ± 19.4)],vasoactive-inotropic score[(19.5 ± 12.0) points vs.(204.0 ± 143.8) points]and blood lactate [(2.8 ± 1.5) mmol/L vs.(9.6 ± 3.6) mmol/L]) were all significantly decreased at 24 h after ECMO treatment (P ＜ 0.05).During follow-up,3 cases survived,2 cases died.All the 5 cases showed thrombocytopenia,3 cases developed renal failure and received continuous renal replacement therapy,1 case got intracranial hemorrhage.2 of the 3 survived cases developed neurological impairment and need long term follow-up and rehabilitation therapy.Conclusion ECMO treatment has remarkable effects on critically ill neonates and may actually save lives,but the risk of complications are quite high.
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Objective To study efficacy and safety of continuous renal replacement therapy (CRRT) in the treatment of neonatal sepsis-related acute kidney injury (AKI).Method From June 2011 to June 2018,neonates with sepsis-related AKI hospitalized in the neonatal intensive care unit of our hospital and treated with CRRT were enrolled.Before CRRT,12 h,24 h,48 h after CRRT and by the end of CRRT,their clinical data including renal function,acid-base balance,electrolytes,blood pressure (BP)and the change of hemodynamic indexes were retrospectively analysed.The efficacy and safety of CRRT was evaluated.Kruskal-wallis H test was used for statistical analysis.Result A total of 9 cases of sepsis-related AKI neonates were enrolled in the study,all treated with continuous veno-venous hemofiltration dialysis.5 cases had oliguria,2 cases fluid overload and 2 cases shock.The duration of CRRT was 49 ～ 110 h (76.2 ±23.5) h.12 h after CRRT,BP were maintained at 40 ～60 mmHg and stable during the treatment,the blood pH value increased to 7.35 ～ 7.45 and the oxygenation index reached 200 mmHg.24 h after CRRT,the oxygenation index rose to more than 300 mmHg.Serum potassium,urea nitrogen and creatinine levels decreased significantly after 12 h of CRRT,and reached the normal range after 24 h of CRRT.After 24 h of CRRT,the urine volume significantly increased.Venous catheterization was performed successfully in 9 cases.2 cases had thrombocytopenia,1 case catheterization obstruction and 1 case hypotension during CRRT.No complications such as hypothermia,hemorrhage,thrombosis or infection occurred.All 9 patients were cured and discharged.Conclusion CRRT is safe and effective for the treatment of neonatal sepsis-related AKI.
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Objective To explore the efficacy of continuous renal replacement therapy (CRRT) in the treatment of neonatal acute kidney injury (AKI).Methods Totally 17 critically ill neonates treated with CRRT were selected who were hospitalized at Department of Neonatology,Shanghai Children's Hospital,Children's Hospital Affiliated to Shanghai Jiaotong University,from June 2012 to June 2017,and among them there were 15 cases with AKI,and the clinical data of these 15 patients were retrospectively analyzed,while 15 AKI neonates were treated with CRRT combined with conventional treatment.The model for CRRT was continuous veno-venous hemofiltration dialysis (CVVH-DF) in 13 cases,plasma exchange (PE) in 2 cases.The changes of blood pressure(BP),renal function,electrolyte,acid-base balance index and hemodynamic indicators were analyzed respectively before CRRT treatment,12 h,24 h,48 h after treatment and by the end of CRRT treatment.The efficacy of CRRT treatment was evaluated in these 15 AKI neonates.Results Gestational age of 15 AKI newborns was 33 +4-40 +1 weeks,admission day age was 2-28 days,birth weight was 2.25-4.00 kg.Primary diseases were severe asphyxia in 6 cases,neonatal septicemia in 5 cases,congenital hereditary metabolic disease in 2 cases,traumatic asphyxia in 1 case,and liver failure in 1 case.CRRT treatment persisted for 49-190 hours.BP value [(50.8 ± 6.57) mmHg(1 mmHg =0.133 kPa)] could reach normal level after 12 h CRRT treatment,and blood pH value (7.31 ± 0.25) increased significantly after 12 h CRRT treatment,while blood K+[(5.51 ±1.86) mmoL/L],urea nitrogen (BUN) [(9.5 ±3.7) mmol/L],creatinine(Cr) [(93± 14)μmol/L] significantly decreased after 12 h CRRT treatment,and reached the normal range [K + (4.78 ± 2.95)mmol/L,BUN (7.5 ±2.1) mmol/L,Cr (54 ± 13) μmol/L] after 24 h treatment,but urine volume[(0.8 ±0.2)mL/(kg· h)] significantly increased after 24 h treatment.Partial pressure of oxygen/fraction of inspired oxygen reached 200 mmHg after 12 h treatment and more than 300 mmHg after 24 h treatment.CRRT treatment of 15 AKI neonates turned out to be effective.Conclusions CRRT can effectively improve the internal environment of AKI neonates and reduce the death rate of neonatal AKI,which can provide an effective adjuvant treatment measures for the treatment of AKI neonates.
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Objective@#To investigate the principles of diagnosis and treatment of breast cancer during pregnancy.@*Methods@#Clinical data of patients with breast cancer during pregnancy admitted to Obstetrics and Gynecology Hospital of Fudan University between January 2012 to July 2017 were analyzed retrospectively. A total of 17 patients were diagnosed with breast cancer in pregnancy, the median age was 32 years (range from 25 to 45 years old), pathological staging revealed 2 patient with stage 0, 1 with stage Ⅱa, 7 with stage Ⅱb, 1 with stage Ⅲa, 2 with stage Ⅲc, 4 with stage Ⅳ.@*Results@#Thirteen patients received surgical treatment in pregnancy, the gestational age at surgery was (27.7±4.6) weeks; 2 patients with ductal carcinoma in situ received mastectomy, 11 patients with breast cancer underwent modified radical mastectomy. In patients undergoing surgery during pregnancy, no prophylactic contractions were used in 4 patients who had been treated earlier, there were 2 patients with frequent contractions within 24 hours after operation in these patients. Follow-up 9 patients were given oral nifedipine to prevent contractions, no obvious contractions occurred after the operation. Seven patients received chemotherapy during pregnancy; the chemotherapy of 4 cases of triple negative breast cancer was weekly paclitaxel sequential epirubicin and cyclophosphamide, the chemotherapy of the other three patients was docetaxel sequential epirubicin and cyclophosphamide. Fifteen patients underwent cesarean section to terminate pregnancy, 2 patients underwent spontaneous labor. The gestational age of birth was (36.9 ±1.3) weeks. Less than 35 weeks of termination of pregnancy occurred in one patient, the fetus was delivered to the neonatal intensive care unit due to neonatal respiratory distress syndrome, and suffered from congenital dysaudia. The prognosis of the other 16 survived infants was good. The median follow-up time was 10 months (range from 4 to 27) months, in 13 patients of stage 0 to Ⅲc, one patient were diagnosed with bone metastasis at 12 months after surgery, the remaining 12 patients had no disease progression, the progression free survival rate was 12/13, the overall survival rate was 13/13. Among the 4 patients with stage Ⅳ, one died in 7 months after delivery, one had new liver metastasis in 8 months after delivery. The remaining 2 patients were in stable condition.@*Conclusions@#Breast cancer in pregnancy can be treated effectively, multidisciplinary cooperation and detailed assessment of maternal-fetal risks and benefits are necessary. Chemotherapy during pregnancy is safe for maternal-fetal, but it needed a large sample of clinical studies and long-term follow-up. The neonatal outcome was associated with gestational age, and therefore premature delivery was avoided as much as possible during treatment.
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ObjectiveTo investigate the timing and efficacy of continuous renal replacement therapy (CRRT) in neonatal acute kidney injury (AKI).MethodsNineteen AKI neonates treated with CRRT were enrolled during hospitalization in the Department of Neonatology of the Children's Hospital of Shanghai from June 2011 to June 2018. Their clinical data were retrospectively analyzed. According to their baseline renal function, these neonates were divided into two groups using an improved RIFLE (Risk, Injury, Failure, Loss and End-stage renal disease) standard: AKI stage 1-2 group and AKI stage 3 group. CRRT included continuous veno-venous hemodiafiltration (CVVHDF) and plasma exchange (PE). Several parameters included blood pressure (BP), renal function, electrolyte, blood gas and hemodynamic indicators were analyzed before, 12 h, 24 h, and 48 h after the initiation of CRRT and at the end of CRRT. Changes in neonatal renal function before, 24 h after the initiation of CRRT and at the end of CRRT were compared between the two groups. Efficacy of CRRT was evaluated, and clinical outcomes were analyzed. Kruskal-WallisH-test ort-test was applied for statistic analysis.Results(1) Among the 19 neonates with AKI, there were 12 in stage 1-2 and seven in stage 3. Seventeen cases were treated with CVVHDF, and the other two underwent plasma exchange. The duration of CRRT was 49-190 h with an average of (89.2±33.9) h. (2) After 12 h of CRRT, the blood pressure of all 19 AKI neonates returned to normal (40-60 mmHg, 1 mmHg=0.133 kPa) and was maintained at that level during the treatment. The blood pH value also increased to a normal range (7.35-7.45) at the same time. The oxygenation index reached 200 mmHg after 12 h of CRRT and rose to over 300 mmHg after 24 h. The levels of serum potassium, urea nitrogen, and creatinine decreased significantly after 12 h of CRRT and reached the normal range after 24 h of CRRT. After 24 h of CRRT, the urine volume significantly increased. (3) Serum levels of urea nitrogen and creatinine in neonates with AKI stage 1-2 decreased significantly after 24 h of CRRT. At any time points before and after CRRT (24 h before, 24 h after and at the end of CRRT), serum levels of urea nitrogen and creatinine in AKI stage 3 neonates were higher than those in AKI stage 1-2 neonates [urea nitrogen: (15.8± 4.1) mmol/L vs (10.2±5.1) mmol/L, (11.5±2.4) mmol/L vs (6.3±2.3) mmol/L, (9.8±2.1) mmol/L vs (5.1± 2.2) mmol/L,t=2.468, 2.226 and 2.171, respectively; creatinine: (184±32) μmol/L vs (152±26) μmol/L, (110±35) μmol/L vs (87±25) μmol/L, (63±12) μmol/L vs (44±9) μmol/L,t= 2.404, 2.423 and 3.972, respectively; allP<0.05]. (4) Venous catheterization was successful in the 19 AKI neonates. Three cases were complicated with thrombocytopenia, two with obstruction and two with hypotension during CRRT. Complications such as hypothermia, hemorrhage, thrombosis, and infection were not reported. (5) Among the 19 AKI neonates, 12 (including five of severe asphyxia, five of septic sepsis and two of inherited metabolic disorders and in metabolic crisis) were cured and discharged. The other seven cases (two in stage 1-2 and five in stage 3) lived through the oliguria stage but died after their family members gave up the treatment.ConclusionsCRRT is a safe and effective management for neonatal AKI. The optimal opportunity for CRRT treatment in AKI neonates should be at stage 1-2.
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Objective To investigate the diagnostic value of cerebrospinal fluid protein in the assess-ment of neurological outcome in preterm infants with sepsis. Methods A total of 80 preterm infants with sepsis were enrolled in the department of neonatology of Shanghai Children's Hospital from June 2014 to June 2016. The lumbar puncture was completed within 24 hours after diagnosis of sepsis,and the results of ce-rebrospinal fluid protein were obtained. The prognosis of neurological development was assessed according to Gesell Developmental Quotient ( DQ) at 6 months of adjusted gestational age. DQ> 85 was used as an indi-cator of good prognosis group. DQ≤85 was assigned to the poor prognosis group. The differences in protein content of cerebrospinal fluid between these two groups were retrospectively analyzed. The receiver operating characteristic ( ROC) curve was used to evaluate the diagnostic value of cerebrospinal fluid in evaluating the prognosis of preterm infants with sepsis. Results Cerebrospinal fluid protein content of poor prognosis group was higher than those in good prognosis group[(2005. 56 ± 582. 85)mg/L vs. (1367. 92 ± 362. 29)mg/L, t= -6. 019,P<0. 01]. The area under the ROC curve was 0. 819(95％CI 0. 711 -0. 927,P<0. 05). The optimal threshold of cerebrospinal fluid protein was 1560 mg/L with specificity of 75. 5％ and sensitivity of 81. 5％. Conclusion Cerebrospinal fluid protein content has certain diagnostic value on the assessment of sepsis premature neurological prognosis.
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Objective To observe the expressions of long noncoding RNA (IncRNA)metastasis-associated lung adenocarcinoma transcript 1 (MALAT1) and nuclear factor etythroid-2 related factor 2 (Nrf2) in lung tissues of hyperoxia-exposed premature neonatal rats,and explore the role of MALAT1 and Nrf2 in hyperoxia-induced lung injury.Methods Pregnant Sprague-Dawley (SD) rats were given cesarean section on the 21st day of gestation.After feeding for 24 h,a total of 80 premature rats were randomly divided into the air group and hyperoxia group.The rats in the air group were fed in the indoor environment[fraction of inspiration O2 (FiO2) =210 mL/L] and those in the hyperoxia group were fed in a high-oxygen box (FiO2 ＞ 850 mL/L).Eight premature rats from each group were sacrificed and lung tissue samples were collected at 5 experimental time points (1st,4th,7th,10th,14th day),respectively.Hematoxylin-eosin staining was used to observe pathological changes in lung tissues.Real-time quantitative polymerase chain reaction (qPCR) and Western blot were used to detect the expression level of MALAT1and Nrf2.Results Compared with the air group,the degree of alveolarization in lung tissues of the hyperoxia group rats was reduced,and radial alveolar count (RAC) decreased on the 1st day,but there was no significant difference between 2 groups (P ＞0.05),when they decreased on the 4th(3.14 ± 0.23),7th(5.25 ± 0.38),10th (4.41 ± 0.44),14th (3.41 ± 0.13) day of exposure,the differences were statistically significant (all P ＜ 0.05).Compared with the air group,the RNA expression of MALAT1 of hyperoxia group preterm rats decreased after the 1 st day(0.527 ± 0.124) of exposure,increased after the 4th (0.538 ±0.128),7th (0.748 ±0.071) day,decreased after the 10th (0.519 ± 0.081)day,and the differences were statistically significant (all P ＜ 0.05),but it continually became weak on the 14th day,but there was no significant difference between 2 groups (P ＞ 0.05).Compared with the air group,the mRNA expression of Nrf2 of hyperoxia group preterm rats decreased after the 1st day(0.791 ± 0.031) of exposure,increased after the 4th (0.977 ± 0.189),7 th (1.369 ± 0.100),10th (1.094 ± 0.104) day,and the differences were statistically significant (all P ＜ 0.05),and it decreased after the 14th day,but there was no significant difference between 2 groups (P ＞0.05).The hyperoxia group had significantly higher expression of free Nrf2 protein and lower expression of Nrf2-Keapl protein than those of the air group at all time points.Within the hyperoxia group,the RNA expression of MALAT1 was positively correlated with RAC and Nrf2 (r =0.517,0.533,all P ＜ 0.001).Conclusions Lung injury is gradually aggravated over the time of hyperoxia exposure.The level of lncRNA MALAT1 is associated with the severity of lung injury and the level of Nrf2 mRNA,suggesting that IncRNA MALAT1 and the Nrf2 target gene signaling pathway might be involved in the development of hyperoxia-induced lung injury in neonatal premature rats together.
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Objective To evaluate the application value of bedside noninvasive hemodynamic monitoring in the diagnosis and treatment of neonatal septic shock.Methods The purchase time and use of Ultrasound Cardiac Output Monitor (USCOM) to monitor hemodynamic status were taken as the grouping condition,and the infants admitted to Department of Neonatology in Shanghai Children's Hospital from March 2014 to December 2016 were divided into 3 groups,16 of USCOM's pre-purchased septic shock infants were taken as non-USCOM monitoring group,20 patients with septic shock received USCOM monitoring as USCOM monitoring group,the other 20 non-septic shock neonates were assigned as a control group,whose primary diseases were premature,neonatal jaundice or neonatal pneumonia.Systolic volume (SV),cardiac output (CO),heart rate (HR),cardiac index (CI) and systemic vascular resistance index (SVRI) in USCOM monitoring group and control group were recorded.The doses of dopamine,dobutamine,epinephrine or norepinephrine and the time of vasoactive drug administration were compared between the USCOM monitoring group and non-USCOM monitoring group.The data of 3 groups were analyzed statistically.Results Compared with the control group,the hemodynamic parameters of the USCOM monitoring group before treatment such as CO [(0.68 ± 0.44)L/min vs.(0.44 ± 0.17) L/min,t =3.306,P =0.004],CI [(4.40 ± 1.88) L/(min · m2) vs.(3.00 ±0.40) L/(min · m2),t =3.328,P =0.004],SV [(3.90 ±2.39) cm3 vs.(3.08 ±0.31) cm3,t =2.227,P =0.038]and HR [(166.09 ± 26.20) times/min vs.(145.35 ± 16.16) times/min,t =2.750,P =0.013] were increased,while the SVRI showed an obvious decline [(795.88 ± 450.19) d · s/(cm5 · m2) vs.(1 160.61 ± 49.59)d · s/(cm5 · m2),t =-2.898,P =0.009],and the differences were statistically significant.While in the USCOM monitoring group after treatment,the CO [(0.56 ± 0.28) L/min vs.(0.68 ± 0.44) L/min,t =2.456,P =0.024] and CI [(3.65 ± 1.10) L/ (min · m2) vs.(4.40 ± 1.88) L/ (min · m2),t =2.614,P =0.017] were decreased significantly compared with those in USCOM monitoring group before treatment.Compared with non-USCOM monitoring group,the doses of dopamine [(45.72 ± 28.80) mg/kg vs.(85.83 ± 69.33) mg/kg,t =2.352,P =0.005],dobutamine [(12.81 ±26.18) mg/kg vs.(85.83 ±69.33) mg/kg,t =4.351,P =0.002],epinephrine [(0.11 ±0.33) mg/kg vs.(0.90± 1.75) mg/kg,t=1.986,P =0.014],and the time of vasoactive drug use [(68.10 ±34.37) h vs.(167.75 ± 117.14) h,t =3.626,P =0.001] were decreased significantly in USCOM monitoring group.The doses of norepinephrine [(1.91 ± 3.79) mg/kg vs.(0.47 ± 0.90) mg/kg,t =-1.481,P =0.046] were increased significantly in USCOM monitoring group.Conclusion The noninvasive hemodynamic monitoring plays an important role in the diagnosis and treatment of septic shock in neonates by clarifying the hemodynamic status of shock and guiding the rational use of vasoactive drugs so as to improve the successful rescue rate.
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Objective:To explore the relationship between unexplained palpitation in children and head-up tilt test (HUTT).Methods:A total of 142 children with the main symptom of unexplained palpitation were admitted to the Specialist Out-Patient Clinic of Children's Cardiovascular Disease from Sept.2008 to Feb.2017 in the Second Xiangya Hospital,Central South University.Among them,63 cases were male,79 cases were female,with the mean age of (10.12±2.88) years old.The detailed history,physical examinations,conventional 12 electrocardiogram,chest X-ray,echocardiography,myocardial enzymes and thyroid function were all examined.The disorders of heart disease,systemic disease and drug effect were ruled out.The HUTT inspection was then given to them.Results:Among the 142 palpitation cases,79 cases were HUTT positive (55.6％) and 63 cases were HUTT negative (44.4％).The age in HUTT positive patients was older than that in HUTT negative patients (P＜0.05),with no significant difference in gender (P＞0.05).There were three types of hemodynamic changes in HUTT positive patients.Among them,38 cases were postural orthostatic tachycardia syndrome (48.1％),36 cases were the vasovagal syncope vasodepressive type (45.6％) and 5 cases were the vasovagal syncope mixed type (6.3％).There were no hemodynamic types for vasovagal syncope cardioinhibitory type,orthostatic hypotension and orthostatic hypertension.Conclusion:Among the clinically unexplained palpitations children,more than half are caused by unbalanced autonomic nervous function.HUTT can help clear the cause of unexplained palpitations.
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<p><b>OBJECTIVE</b>To carry out rapid genetic diagnosis for a child affected with citrullinemia type Ⅰ.</p><p><b>METHODS</b>Peripheral venous blood samples were obtained from the two-day-old child and his parents as well as 100 healthy controls. Serum ammonia and citrulline was determined by biochemical test and tandem mass spectrometry. Sixteen pairs of primers were designed for high-resolution melting (HRM) analysis of all exons and adjacent intronic sequences of the ASS1 gene in the proband, parents and healthy controls. Suspected mutations were confirmed by DNA sequencing, while the mRNA transcripts of the ASS1 gene were determined by reverse transcription (RT)-PCR. Functional impact of the mutation sites was predicted with PolyPhen-2 and SIFT Blink software.</p><p><b>RESULTS</b>Blood ammonia and citrulline of the proband have respectively reached 286 μmol/L and 487.69 μmol/L, which far superseded the normal values. HRM analysis and DNA sequencing have identified in the child a homozygous c.380G>A (p.R127Q) mutation in exon 6 of the ASS1 gene, in addition with a homozygous IVS8+60G>A substitution in intron 8, while his parents were heterozygous carriers for both mutations. RT-PCR assay indicated that the IVS8+60G>A mutation did not result in abnormal splicing of the ASS1 gene transcripts. Bioinformatic analysis suggested that the site for p.R127Q was conserved among 45 species of vertebrates and may play a crucial role in citrulline metabolism.</p><p><b>CONCLUSION</b>The severe urea cycle disorder in the proband was probably due to the compound homozygous R127Q and IVS8+60G>A mutations of the ASS1 gene.</p>
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Objective To explore the protective effect of long non-coding RNA(lncRNA) metasta-sis associated in lung denocarcinoma transcript 1 (MALAT1) involved in hyperoxia-induced lung injury in preterm infants.Methods This study had downloaded chip data set GSE25286 (Mouse GEO Genome 430 2.0 Array) from gene expression database gene expression omnibus (GEO),according to the state of hyperoxia exposure,the MALAT1 mRNA expression in rats normal lung tissues and hyperoxic lung tissues was compared at day 14th and 29th.In chip data set GSE43830(Human Exon 1.0 ST Arrays) from GEO,the expression of multi-ple genes[cell division cycle 6(CDC6),death effector domain containing 2(DEDD2),and Cyclin B1 (CCNB1)] in WI38 cells(lung fibroblasts) was compared before and after MALAT1 was knockout.At the same time,the peripheral blood samples of premature infants were collected to verify.Totally 40 premature infants were hospitalized in the department of neonatology in our hospital from Jan 2015 to Dec 2016,the pe-ripheral blood samples of 40 premature infants were collected.RNA was extracted and Real time-PCR was performed after reverse transcription,clinical data of these 40 cases were retrospectively analyzed. Results (1) By using Affymetrix Expression console and Affymetrix Transcriptome analysis console software source files of the chip of pretreatment and difference expression gene screening,the expression of lncRNA MALAT1 gene in lung tissues of hyperoxia lung injury mice significantly upregulated[fold change(FC) =2.33,P=0.001].(2) After MALAT1 in WI38 cell was knockout,MALAT1 expression was significantly reduced(FC= -15.6,P=0.000),the expression of CDC6(FC= -2.37,P=0.001) and CCNB1(FC=-2.16,P=0.002) were down regulated,DEDD2 expression was up regulated(FC =2.46,P =0.000). (3) The results of peripheral blood samples from preterm infants showed that the expression of MALAT1 was significantly increased in preterm infants with hyperoxia-induced lung injury(0.375 5 ± 0.081 9,t =4.634, P=0.015),compared with normal preterm infants(0.273 4 ± 0.067 3).Conclusion Through inhibiting cell apoptosis,lncRNA MALAT1 can protect preterm infants with hyperoxia-induced lung injury,it may provide a new strategy for prevention and treatment of hyperoxia-induced lung injury in premature infants.