Objective To investigate the variations of matrix metalloproteinase 2 (MMP2) and type Ⅳcollagen expression in newborn rat alveoli at different stage during pregnancy and the influence of dexamethasone (DXM). Methods Twenty-four Sprague-Dawley (SD) pregnant rats were randomly divided into control group and observe group (12 in each group). Normal saline (0.5 ml) or DXM [0.8 mg/(kg?d)] were injected muscularly to these two groups, respectively, on the 18th,19th and 20th day after conception. All rats were delivered normally at term. The lung tissue were examined in 20 days gestation fetal rats and newborn rats on the 1st, 4th, 10th and 14th day after birth. Immunohistochemical studies were performed for MMP2 and type Ⅳ collagen. Reverse transcriptase-polymerase chain reaction (RT-PCR) was used to test the MMP2 mRNA expression. Results The MMP2 expression of day 4 rats (20.14?3.39) was stronger than that of fetal rats lung (16.35?2.36), and decreased in day 10 rats(15.93?1.94). while the general MMP2 expression in the observe group was significantly less than the control(P0.05). The expression of type Ⅳcollagen was stronger than the control group. Conclusion DXM applied in later stage of gestation inhibits the expression of MMP2, reduces the degradation of type Ⅳcollagen, which affect basement membrane and alveoli development.

Objective To summarize the clinical characteristics of neonatal asplenia syndrome to improve the understanding of the disorder. Methods Newborns with neonatal asplenia syndrome admitted to the neonatal department of our Hospital from June 2008 to July 2015 were recruited and their gender, birth weight, gestational age, clinical manifestations, imaging features, laboratory examinations, treatments and prognosis were analyzed. Results A total of 8 newborns with asplenia syndrome were studied. All the patients presented with cyanosis at birth, 5 of them with heart murmur, 3 with apical impulse in the right ( dextrocardia) thorax and complex congenital heart diseases were found in all of them by echocardiogram examinations. 2 of them were diagnosed congenital heart diseases before birth by the fetal ultrasound. 2 cases combined with congenital imperforate anus and cutaneous fistulas. 1 case was found right renal agenesis and Howell-Jolly was seen in another case. All the 8 patientsˊ parents refused medical advices about continuing treatments and left hospital. Among them, 4 patients died in the neonatal period, 2 lost in follow-up and only 2 survived, one for 19 months and the other for 4 years. Conclusions Patients with asplenia syndrome presented absence of spleen, visceral inversion, severe cardiovascular malformations and other malformations. When the cyanosis, heart murmur and abnormal position of apical impulse were found in the newborns, asplenia syndrome should be considered and imaging examinations such as chest X-ray, ultrasound, visceral angiography and more should be ordered immediately, in order to make an early diagnosis of the disease.

[Objective]To evaluate FANCF gene methylation status and its roles in the pathogenesis acute myeloid leukemia (AML).[Methods]Genomic DNA from primary 30 AML patients and 21 AML cell lines were subjected to FANCF methylation analysis by PCR based restriction enzyme digestion assay.FANCF protein expression was detected by Western blot.In addition,FANCF gene methylation status was further analyzed using bisulfate sequencing.[Results] No FANCF methylation was found in primary AML patients.One (4.76 ％) AML cell line contained FANCF methylation in the promoter region.The AML cell line was hypersensitive to MMC with absence of FANCF protein expression.[Conclusion] FANCF methylation is a rare event in AML,and does not contribute to the initiation of AML, but may contribute to the clonal transformation and cellular phenotype maintenance in some AML cell lines.

Objective To review the clinical experience of diagnosis and treatment of the congenital diaphragmatic hernia in newborn infants. Methods Thirty-three neonates were diagnosed having congenital diaphragmatic hernia in our hospital from Jan. 1,2004 to Sept. 30, 2009. The clinical data was retrospectively reviewed. Results 21 cases were treated surgically and 17 survived, while 4 cases died. The main cause of death was congenital pulmonary dysplasia. Another 12 cases refused to accept surgical treatment and they all died, one died shortly after he was born. Four cases who had been diagnosed by prenatal ultrasonography were survived. Conclusion The mortality of congenital diaphragmatic hernia in neonates was still high. Prenatal diagnosis of the congenital diaphragmatic hernia is very important and the cooperation between the obstetrics, neonatology and cardiothoracic surgery will improve the survival rate of congenital diaphragmatic hernia in newborn infants.

Objective To summarize the clinical features of neonatal lower respiratory tract infection of respiratory syncytial virus (RSV). Methods Data of newborns admitted into Deparrment of Neonatology of Yuying Children Hospital of Wenzhou Medical College from January to December 2010 with RSV lower respiratory tract infection were retrospectively analyzed. The characteristics of clinical manifestation,examination,treatment and prognosis were studied.Factors affecting the duration of hospitalization were analyzed with x2 test. Results Of 354 cases of neonatal lower respiratory tract infection,75 (21.2％) were RSV positive,whose attack time was from 5 to 25 days with an average of 13.2 days; and there were two preterm infants (2.7％).The chief clinical manifestations were cough (100.0％,75/75),followed by shortness of breath (56.0％,42/75),spittle (52.0％,39/75) and fever with temperature ≥38.0 ℃ (25.3％,19/75).The white blood cell count of one newborn was higher than 20.0× 109/L; 52 cases (69.3％) had higher blood lymphocyte percentage (＞ 0.48) ; eight cases (10.7％) had higher level of C-reactive protein (≥ 8 mg/L).Among the 58 patients who underwent blood gas analysis,28 (48.3％) had carbon dioxide retention (partial pressure of carbon dioxide≥ 45 mm Hg). Chest X-ray results within 48 hours after hospitalization suggested that 52.0％ patients (n =39) had pneumonia and 20.0％ (n=15) had bronchitis.Among 69 patients who had sputum cultures,20 cases were positive.And for the 11 cases who had blood cultures,three were positive for Staphylococcus epidermidis.Finally,73 cases were cured,two were improved and no death was reported.The duration of hospitalization was from 3 to 17 days with an average of 8.4 days.Neonates with RSV lower respiratory tract infection who had higher C-reactive protein (≥8 mg/L),carbon dioxide retention,nasal oxygen catheter or assisted ventilation had higher percentage of longer hospital stay (≥7 days). Conclusions Compared with infants,the clinical characteristics of RSV lower respiratory tract infections in neonates are atypical,which are mainly menifested by cough,shortness of breath,spittle,but rare wheezing.There are no effective interventions against RSV infection except for supportive therapy.

AIM: To observe the effects and safety of clopidogrel used in percutanous coronary intervention (PCI) while acute occlusions of coronary arteries happened. METHODS: 92 patients with acute occlusions of coronary arteries were treated through PCI and they were randomized equally into following groups: clopidogrel group (CPG group) and ticlopidine group (TCP group). Each patient was treated with clopidogrel 25 mg bid or ticlopidine 250 mg bid for 4 weeks after PCI. RESULTS: Compared with the control group, there was not significantly difference in platelet aggregation ( 45.36 ? 2.98 vs 51.60 ? 2.56 ) and clinical effects ( 97.95 % vs 93.02 %) after 3 weeks. The proportion of patients with adverse events who had to release the treatment was 2.04 % (CPG group) and 16.27 % (TCP group). CONCLUSION: The clinical effect of clopidogrel is similar to ticlopidine and shows less adverse events.

Objective To study the clinical characteristics,treatment and prognosis of neonatal VACTERL association.Method The clinical data of newborns diagnosed with VACTERL association from January 2010 to December 2015 were collected and retrospectively analyzed.Result A total of 33 patients diagnosed with VACTERL association were included,including 23 males and 10 females.Among them,17 cases were term infants,15 cases premature infants and 1 case of overdue birth,with an admission age of 1 to 24 days.The most common deformities were cardiac anomalies (C) in 27 cases (81.8％),followed by anal atresia/anorectal malformation (A) in 25 cases (75.8％),renal deformity (R) in 24 cases (72.7％),limb abnormalities (L) in 20 cases (60.6％),Tracheoesophageal fistula (TEF) in 8 cases (24.2％) and vertebral abnormalities (V) in 3 cases (9.1％).11 cases (33.3％) had other deformities.Among these 33 patients,24 cases had 3 types of malformations and 9 cases had 4 types of malformations.The most common combination was ACR (n =8).20 patients had no abnormalites on chromosome karyotype test including 2 patients had normal gene microarray results.16 patients received surgical treatment during neonatal period and 13 of them recovered and discharged.Among the other 17 cases received no surgery,only 1 patient improved and discharged.A telephone follow-up was proceeded in 14 discharged cases at 1 year old.Among them,13 cases had good prognosis,however,the remaining one was dead.Conclusion VACTERL association is a rare non-random combination of multiple malformations.The early discovery and appropriately treatment after diagnosis will improve the prognosis and prevent death.Doctors should reinforce the ability to detect various types of deformities and examine the chromosome and gene properly.

Objective To investigate the effect of di-(2-ethylhexyl) phthalate (DEHP) on the postnatal lung development in newborn rats.Methods A total of 60 newborn Sprague-Dawley rats (weighing 5.0-8.0 g) in five age groups were studied in the first experiment.The rats were divided based on the different postnatal ages:postnatal day (PND)I,PND4,PND7 and PND14.A total of 45 newborn Sprague-Dawley rats (weighing 5.0-8.0 g) were randomly divided into three groups according to the dosage of DEHP administered in the second experiment.The newborn rats were administered DEHP through intraperitoneal injection at 10 (low-dose subgroup),100 (medium-dose subgroup) or 750 (high-dose subgroup) mg/kg daily from PND1 to PND13.The rats were sacrificed on PND14.Pups were sacrificed with lethal dose injection of pentobarbital sodium.The lung was removed.The right middle lobes were used for analysis.The tissue was processed for histology and lung sections were stained with HE for light microscopic (LM) morphometric measurement.The analysis was performed by means of a digital image analysis system,including pulmonary interstitial area ratio (IAR) and total length density of all segments.One-way ANOVA,LSD and Dunnet T3 methods were used for statistical analysis.Results In the normal controls,IAR decreased significantly by (31.97±5.03) ％,(30.05±3.57)％,(25.33± 1.83)％ and(22.01 ±2.19)％,respectively,from PND1 to PND14 (P＜0.05 or P＜0.01).IAR in medium-and high-dose subgroups increased significantly by (24.11 ±2.78)％ and (26.53± 3.42)％,respectively on PND 14.The total length density of all segments in unit area lung volume increased significantly by 0.047 8±0.003 7,0.050 0±0.002 9,0.071 2±0.003 0 and 0.084 4±0.004 3,respectively from PND1 to PND14 (P＜0.01).In the DEHP treated animals,when compared with the control group,IAR was significantly higher on PND14 (P＜0.05 or P＜0.01),while the total length density of all segments in unit area lung volume was significantly decreased (P＜0.05 or P＜0.01).Length density in medium-and high-dose subgroups were higher than that of low-dose subgroup by 0.082 9±0.001 8,0.077 2±0.002 0 and 0.071 3±0.003 7,respectively on PND14 (P＜0.05 or P＜0.01).Conclusions Medium-and high-dose DEHP affect the postnatal lung development in rats in a dose-dependent mode.

Objective To review the trends of survival rates and complications in extremely low birth weight (ELBW) infants and to improve the prognosis of ELBW infants.Method From January 1999 to December 2015,ELBW infants in our hospital were retrospectively studied.Their survival rates and complications were compared among groups with different birth weight,and the risk factors for survival were identified using multivariate unconditional logistic regression analysis.Result A total of 243 ELBW infant were collected.The median gestational age of ELBW infant was 27.3 weeks (23 ～ 34 weeks),and their median birth weight was 890 g (490 ～ 995 g).Excluding 40 cases refused treatment,the cure and survival rates of the remaining 203 ELBW infants were 43.8％ (89/203) and 65.0％ (132/203),respectively.The survival rate in ELBW infant with birth weight ＜ 600 g was 0/3,increased to 70.8％ (68/96) when birth weight was 900 ～ 999 g,with an ascending trend with increased birth weight (x2 trend =12.673,P ＜0.001).The most common complications of 243 cases were neonatal respiration distress syndrome [87.7％ (213/243)],sepsis [45.3％ (110/243)],intraventricular hemorrhage [37.4％ (91/243)],bronchopulmonary dysplasia [36.6％ (89/243)] and pheumonia [36.6％ (89/243)].The incidence of complications (including intracerebral hemorrhage and hydrocephalus),decreased with increased birth weight.Multivariate unconditional logistic regression analysis found that birth weight below 800 g (＜ 700 g:OR =22.333,95％ CI 1.493 ～ 334.148,P =0.024;700 ～ 799 g:OR =3.573,95％ CI 1.075 ～ 11.874,P =0.038),stage Ⅲ necrotizing enterocolitis (OR =8.803,95％ CI 1.308 ～ 59.244,P =0.025),stage Ⅲ and Ⅳ of intraventricular hemorrhage (OR =8.902,95％ CI 1.127 ～ 70.338,P =0.038) and mechanical ventilation (OR =3.597,95％ CI 1.043 ～ 12.410,P =0.043) were risk factors affecting the ELBW infant's survival.Conclusion As birth weight increases,the survival rate also increases,and the rate of complications decreases.Birth weight,stage Ⅲ necrotizing enterocolitis,stage Ⅲ and Ⅳ intraventricular hemorrhage and mechanical ventilation are risk factors for the ELBW infant's survival.

Objective:To investigate the clinical characteristics and pathogenic gene mutation of lateral meningocele syndrome(LMS).Methods:We retrospectively collected the clinical manifestations, laboratory examination, imaging examinations, and genetic analysis of a neonate with LMS which was diagnosed at the Department of Neonatology of the Second Affiliated Hospital of Wenzhou Medical University in May 2020. Relevant literature up to February 2021, retrieved from PubMed, OMIM, CNKI, Wanfang, and CQVIP database with the terms of "lateral meningocele syndrome", " NOTCH3", were reviewed to summarize the clinical characteristics, pathogenesis, and genetic etiology of this disease. Results:A full-term male newborn was admitted to our hospital due to feeding difficulty 7 d after birth. The clinical characteristics included hypotonia, dysphagia, hypertension, lateral spinal meningocele, craniofacial anomaly, and cryptorchidism. Abnormal spinal MRI and brainstem evoked potential were also observed. Whole exome sequencing revealed a heterozygous frameshift variation c.6667_6686del(p.Ala2223Profs*12) of NOTCH3 gene located in 19p13.12, which was not detected in the parents. Only 12 English literature were retrieved, with 17 patients from 15 pedigrees. Out of the 18 patients including the index case, 10 were genetically diagnosed as LMS. The age at diagnosis ranged from 15 d to 55 years. Regarding the clinical features, multiple lateral thoracolumbar spinal meningoceles (18/18) was the most common one, followed by retrognathia and low-set ears (16/18), eyelid ptosis and down slanting palpebral fissures (15/18), hypotonia (13/18), hypertension (11/18), developmental delay (9/18), mixed or conductive hearing loss (9/18), cardiovascular dysplasia (7/18), and cryptorchidism (7/10). A total of nine NOTCH3 gene variants were detected, all were heterozygous variants, including six frameshift and three nonsense variants. Conclusions:LMS is caused by NOTCH3 gene mutation with the clinical characteristics including multiple lateral thoracolumbar spinal meningoceles, craniofacial dysmorphisms, hypotonia, hypertension, developmental delay, difficulty in feeding, cryptorchidism, etc.