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Table of Content

    15 September 2019 Volume 37 Issue 9
      
    Application of modified sequential method in febrile neonates
    LI Pingping, HUANG Xuefei, HUANG Xuxu, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  641.  doi:10.3969/j.issn.1000-3606.2019.09.001
    Abstract ( 254 )   PDF (1182KB) ( 306 )  
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    Objective To establish a modified sequential method based on clinical and laboratory indicators for assessment of whether febrile neonates were complicated with infection and evaluate its application value. Methods The clinical data of 230 febrile neonates admitted to the NICU from October 2015 to October 2017 were retrospectively analyzed. A modified sequential method based on clinical and laboratory indicators was established to evaluate whether febrile neonates were infected or not, and to compare it with the laboratory scoring method. Results In the 230 full-term febrile neonates, 112 (48.7%) were diagnosed with sepsis. The general condition, age, urinary routine, procalcitonin, C-reactive protein and neutrophil absolute count of children with sepsis were evaluated and classified according to the modified sequential method. The incidence of sepsis neonates was significantly higher than that of non-sepsis neonates. The sensitivity, negative predictive value and negative likelihood ratio of modified sequential method in the diagnosis of sepsis were 98.2%, 94.8% and 0.02, and the laboratory score was 66.1%, 72.9% and 0.40, respectively. Two children with sepsis were misjudged as non-sepsis by modified sequential method, and 38 children with sepsis were misjudged as non-sepsis by laboratory scoring method. Conclusions Modified sequential method can identify infected and non-infected children. It has high sensitivity for infected newborns and higher application value than laboratory score.
    Clinical analysis of early onset sepsis caused by Listeria monocytogenes in 12 neonates
    REN Jing, SUN Bin, WANG Ying
    Journal of Clinical Pediatrics. 2019, 37(9):  646.  doi:10.3969/j.issn.1000-3606.2019.09.002
    Abstract ( 284 )   PDF (1181KB) ( 306 )  
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    Objective To explore the clinical characteristics, diagnosis and treatment of early onset sepsis caused by Listeria monocytogenes in neonates. Methods The clinical data of early onset sepsis with positive blood culture of Listeria monocytogenes in 12 neonates admitted to NICU from January 2011 to June 2018 were retrospectively analyzed. Results Among the 12 cases, 8 were males, 4 were females, and 10 were premature. The main clinical manifestations were abnormal body temperature, shortness of breath, distress or apnea, convulsions, rashes and pulmonary hemorrhage. Blood culture results showed Listeria, and Listeria was also found in cerebrospinal fluid culture in one case. The antibiotics, including meropenem, penicillins and vancomycin, were used alone or in combination, and 4 cases were improved and 8 cases died. Conclusion Neonatal sepsis caused Listeria monocytogenes is a very serious infectious disease, which is characterized by acute onset, severe infection and high mortality.
    Feeding intolerance in twin anemia-polycythemia sequence in 3 newborns
    HU Jing, WANG Jimei
    Journal of Clinical Pediatrics. 2019, 37(9):  649.  doi:10.3969/j.issn.1000-3606.2019.09.003
    Abstract ( 291 )   PDF (1113KB) ( 179 )  
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    Objective To explore the feeding status in newborns with twin anemia-polycythemia sequence (TAPS). Methods The clinical data of TAPS with feeding intolerance in 3 newborns were retrospectively analyzed. Results In 3 preterm monochorionic twins who were diagnosed with TAPS after birth, feeding intolerance occurred during the feeding process in three donors, and the feeding process was smooth in 3 recipients. Conclusion If there is chronic intrauterine twin-twin transfusion in the monochorionic twins, postpartum neonatal feeding intolerance should be closely watched, especially in the donor twins.
    Relevant factors of recurrent primary nephrotic syndrome in children in Ningxia
    FU Yanhong, MA Liyan, WANG Lian, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  652.  doi:10.3969/j.issn.1000-3606.2019.09.004
    Abstract ( 211 )   PDF (1196KB) ( 213 )  
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     Objective To explore the related factors of recurrent primary nephrotic syndrome (PNS) in children. Method The clinical data of recurrent PNS in children were retrospectively analyzed. According to the recurrence situation, the children were divided into frequency recurrence group and non-frequency recurrence group. The differences between the two groups were analyzed. Results Ninety-two children with recurrent PNS were enrolled, with a median age of 3.54 years and a maleto-female ratio of 3.18:1. There were 76 cases of simple type and 16 cases of nephritis type. And there were 60 Han people and 32 Hui people. There were 55 cases in the non-frequent relapses group and 37 cases in the frequent relapses group. There were differences in proteinuria elimination time, initial recurrence time, clinical type and hematuria between the two groups (all P<0.05). Cox proportional hazards multiple regression showed that the initial recurrence time (OR= 0.90, 95% CI: 0.85~0.96) and proteinuria elimination time (OR= 1.01, 95% CI: 1.00~1.01) were associated with frequent relapses of PNS (P<0.05). After making frequent relapse curves for the two variables, it was found that the frequent relapses were more likely in the children whose urine protein became negative for more than 12 days at the initial treatment and who relapsed within 7.5 months after the initial remission. Conclusion The proteinuria elimination time and initial recurrence time may be significantly associated with frequent relapses of PNS in children.
    The clinical value of fractional exhaled nitric oxide in children with bronchiolitis
    YUAN Lin, LI Lijuan, ZHUO Zhiqiang
    Journal of Clinical Pediatrics. 2019, 37(9):  657.  doi:10.3969/j.issn.1000-3606.2019.09.005
    Abstract ( 284 )   PDF (1176KB) ( 226 )  
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     Objective To explore the significance and application value of fractional exhaled nitric oxide (FeNO) in children with bronchiolitis. Methods A total of 49 hospitalized children with primary bronchiolitis admitted from January to September 2018 were selected as study subjects. According to the respiratory syncytial virus (RSV) test results, they were divided into RSV group (27 cases) and non-RSV group (22 cases). In addition, 17 healthy children of the same age were selected as the healthy controls. Nitric oxide in exhaled breath was detected by tidal breathing method in each group. Children in RSV group and non-RSV group were tested again in the remission period (1-2 weeks) and the recovery period (4-6 weeks), and the results were compared. Results There was no difference in sex and age among RSV group, non-RSV group and control group (P>0.05). The FeNO levels in both RSV group and non-RSV group were statistically significantly different among the acute, remission and recovery phases (P<0.05). During the recovery period, there was significant difference in the FeNO levels among RSV group, non-RSV group and healthy control group (P<0.05), with RSV group having the highest FeNO levels. There was no difference in the FeNO levels among the three groups in either acute phase or remission phase (P>0.05). Conclusions The FeNO levels in children with bronchiolitis having or not having RSV infection in both acute and remission phases were not different from those of healthy children at the same age, and were higher during the recovery phase than those of healthy children at the same age.
    Clinical and genetic analysis of Noonan syndrome in 7 children
    LIU Ziqin, CHEN Xiaobo, SONG Fuying
    Journal of Clinical Pediatrics. 2019, 37(9):  661.  doi:10.3969/j.issn.1000-3606.2019.09.006
    Abstract ( 433 )   PDF (1168KB) ( 423 )  
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    Objective To explore the clinical and genetic characteristics of Noonan syndrome in children. Methods The clinical data of Noonan syndrome in 7 children diagnosed from January to December 2018 were collected. Familial analysis and interpretation were completed by whole exon sequencing and Sanger sequencing. Results Four boys and 3 girls visited the clinic for their short stature and the median age of diagnosis was 3 years and 3 months (8 months to 13 years and 1 month). Six children had characteristic features of Noonan syndrome, such as pigmentation, curly hair, webbed neck, palmthrough, scoliosis and cryptorchidism. Six children had mental retardation. Four children had a peak GH concentration>10 ng/ mL in provocation test, and there was no abnormality of insulin-like growth factor. Abnormal cardiac structure was founded in 5 cases, including 2 cases of pulmonary stenosis, 2 cases of atrial septal defect, one case of mitral insufficiency and one case of hypertrophic ventricular septal. Missense mutations of PTPN11 gene were found in all 7 children, including 6 de novo mutations and one paternal mutation, and all these mutations were considered to be pathogenic according to the ACMG Practice Guideline. Conclusions Growth failure is the main presentation in patients with Noonan syndrome. Short stature, special facial features and mental retardation are common manifestations. Many cardiovascular phenotypes occur in Noonan syndrome. Noonan syndrome is caused by missense mutations in PTPN11, mainly de novo mutation.
    Localization of pathogenic gene and mutation detection of GEFS+ gene in children with Dravet syndrome
    ZHANG Hua, CHEN Haidan, CHEN Zeyan, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  665.  doi:10.3969/j.issn.1000-3606.2019.09.007
    Abstract ( 384 )   PDF (1467KB) ( 248 )  
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    Objective To analyze the genetic characteristics of Dravet syndrome in children in Hainan Province. Methods The peripheral blood of 18 children with Dravet syndrome and their family members in Hainan Province from 2015 to 2017 were collected. The PCR amplification and Sanger sequencing were performed for SCN1A gene detection. The GenomeStudio software was used for the localization and linkage analysis of pathogenic genes. For children who were not found SCN1A gene mutation by Sanger sequencing, the multiple connection dependent probe amplification (MLPA) method was used to analyze the deletion or repetition of the SCN1A gene fragment and to further screen the mutation of the parents' SCN1A gene and analyze the source of the mutation. Results The results of gene location scan in 18 cases of Dravet syndrome and their parents fully conformed to the Mendel genetic relationship between the parents and children. The children with SCN1A gene mutation could be located in three candidate mutation regions of chromosome 5, 9 and 22. The region of chromosome 5 was located between SNP Rs4957954 and Rs728937; the maximum positive LOD value (2.13) was obtained at Rs1459085. The region of chromosome 9 is located between SNP Rs720974 and Rs1220087, and the maximum positive LOD value (1.92) was obtained at is 1.92 at Rs71332677. The region of chromosome 22 was located between SNP Rs756658 and Rs713751, and the maximum positive LOD value (1.91) was obtained at Rs374225. In the 18 children, SCN1A gene mutation was found in 12 children, among whom 6 children had heterozygous variation (G→A) at the 5383rd base in the CDS region, 4 children had homozygous variation (T→C) at exon 13 and 2292nd base in CDS region, and 2 children had homozygous variation (A→T) in non-coding region of SCN1A gene. Conclusions The gene localization of the children with Dravet syndrome in Hainan fully conforms to the Mendelian genetic relationship, and it is speculated that Rs4957954 to Rs728937, Rs720974 to Rs1220087, and Rs756658 to Rs713751 are the possible pathogenic regions of Dravet syndrome.
    infantile spasm associated with DEPDC5 gene mutation: case report of 3 infants and literature review
    CHEN Liqing, LIU Yan
    Journal of Clinical Pediatrics. 2019, 37(9):  669.  doi:10.3969/j.issn.1000-3606.2019.09.008
    Abstract ( 414 )   PDF (2901KB) ( 287 )  
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    Objective To explore the clinical features of infantile spasm associated with DEPDC5 gene mutation. Method The clinical data of infantile spasm associated with DEPDC5 gene mutation in 3 infants from October 2016 to May 2018 were retrospective analyzed. Results In 2 girls the age at onset was 1 month and at 4 months respectively. In 1 boy the age at onset was one month. The c.3092C > A (p.Pro1031His) and c.20A > G (p.Tyr7Cys) of DEPDC5 gene were found in 2 girls, and they were heterozygous mutations and not completely extraneous. The c.280-1G > A, splicing, , was found to be a new mutation in the boy. Cranial magnetic resonance imaging was normal in all 3 patients, but PET-CT showed cortical dysplasia in the left temporal region in the boy. The seizures were controlled by the antiepileptic drugs combined with hormone therapy in 2 girls, while the antiepileptic drugs and hormone therapy were not effective in the boy and the seizures were controlled after surgical treatment. Conclusions DEPDC5 gene may be the pathogenic gene of infantile spasm, and it has a certain genotype-phenotype correlation. Aminohexenoic acid has a certain therapeutic effect on DEPDC5 mutation-related infantile spasm. Surgical treatment should be considered for patients with refractory focal epilepsy caused by mutation of DEPDC5 gene.
    Congenital nephrotic syndrome caused by NPHS1 gene mutation: two case report
    LIAO Panli, LUAN Jiangwei, ZHU Gaohong, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  673.  doi:10.3969/j.issn.1000-3606.2019.09.009
    Abstract ( 666 )   PDF (1962KB) ( 505 )  
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     Objective To explore the clinical and gene mutation characteristics of congenital nephrotic syndrome caused by NPHS1 gene mutation. Methods The clinical characteristics of congenital nephrotic syndrome caused by mutation of NPHS1 gene in 2 children were retrospectively analyzed, and the related literature was reviewed. Results The age at onset was 3 months after birth in both girls who presented nephrotic syndrome without family history of renal disease. The whole exon detection was performed in all the children and their parents and both girls had NPHS1 gene complex heterozygous mutation. In case 1, c.2629_c.2630 delAAinsT (exon 19) was from her father and c.1315+1 (IVS 10) G > A was from her mother, and the two mutations have not been reported in the literature. In Case 2, c.2205 (exon16) _c.2206insTGGAC (exon16) was from her mother, but no report was found; C. 3478C >T (exon27) was from her father, which has been reported in the literature. According to the ACMG score, c.1315+1 (IVS10) G>A, c.2205 (exon 16)_c.2206 insTGGAC (exon 16), c.3478C > T (exon 27) were all pathogenic, and c.2629_c.2630 del AAinsT (exon 19) was possibly pathogenic. Conclusion Children with congenital nephrotic syudrome should be considered for NPHS1 gene testing.
    Mandibuloacral dysplasia type A: a familial report and literature review
    XU Xuemei, CHEN Yao, LI Xin, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  677.  doi:10.3969/j.issn.1000-3606.2019.09.010
    Abstract ( 266 )   PDF (2046KB) ( 168 )  
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     Objective To explore the clinical features of mandibuloacral dysplasia type A (MADA) caused by LMNA gene mutation. Methods The clinical data of LMNA homozygous mutation in a child and the results of gene detection in the family were retrospectively analyzed, and the related literature was reviewed. Results A 7-year-old girl suffered from postnatal growth restriction, specific facial features (round face, double eyelid pigmentation, beak-shaped nose, crack with crowded dentition, malocclusion, tooth hypoplasia), skeletal deformities (micrognathia, bilateral clavicle hypoplasia, short rod-shaped finger/toe end), sparse and lusterless hair, pigmented skin with poor elasticity, poor finger/toenail development, limited movement and incomplete squats due to stiff elbows and knees, thin subcutaneous fat of limbs and trunk, normal muscle strength, and excellent academic performance. The parents and the older sister of the child had normal phenotypes, and the younger brother had similar but less severe clinical manifestations. High throughput sequencing analysis revealed homozygous missense mutation c.1580G>A, p.Arg527His in the LMNA gene (NM-170707.3) of both the child and her younger brother, and heterozygous mutation of this locus was carried by both her parents. Conclusion MADA has characteristic clinical manifestations, and genetic testing can further confirm the diagnosis.
    EDA gene mutation in hypohidrotic ectodermal dysplasia in a family
    LIANG Rujia, LIU Fang
    Journal of Clinical Pediatrics. 2019, 37(9):  682.  doi:10.3969/j.issn.1000-3606.2019.09.011
    Abstract ( 213 )   PDF (1741KB) ( 259 )  
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     Objective To explore the genetic variation and genetic counseling in X-linked hypohidrotic ectodermal dysplasia families. Methods The proband's pathogenic gene and its mutation sites were identified by second generation sequencing, and verified by Sanger sequencing. According to the genetic diagnosis results, prenatal molecular diagnosis was performed for the second fetus of the proband's mother. Results The proband was male and was found to have fever and special facial features 10 days after birth. It was found the heterozygous mutation of EDA gene, c.682_683delCCinsA, a new mutation, and the mother had the same mutation. The second fetus underwent amniocentesis, and the above mutation was not found in the gene test. Conclusions Gene detection is helpful to identify the genetic causes of hypohidrotic ectodermal dysplasia in children. On this basis, genetic counseling and prenatal gene diagnosis can be performed for the family of children with hypohidrotic ectodermal dysplasia.
    Peroxisome biogenesis disorder 1B: a case report
    LAN Li, TAN Qianqian, WANG Chunhui, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  685.  doi:10.3969/j.issn.1000-3606.2019.09.012
    Abstract ( 305 )   PDF (1700KB) ( 441 )  
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    Objective To explore the clinical and genetic characteristics of peroxisome biogenic disorders (PBD) caused by peroxin 1 (PEX1) complex heterozygous mutation. Method The clinical data of PBD in a child were retrospectively analyzed and the related literature was reviewed. Results A 4-years- and 6-months-old boy had mental retardation and no other obvious abnormalities. Gene detection found that there were two unreported heterozygous mutations in PBD related PEX1 gene in children, c.539A> c (p.lys180thr) and c.2704_2708delTTTAT (p.phe902fs), which were consistent with the autosomal recessive genetic pattern. The child was therefore diagnosed with PBD1B. Conclusions The clinical phenotype of patients with PBD was diverse, and its severity was related to the mutation type of PEX1 gene. Genetic testing could confirm the diagnosis.
    Succinic semialdehyde dehydrogenase deficiency caused by ALDH5A1 gene mutation: a case report and literature review
    HOU Hongliang, ZHANG Hewei, CAO Xujun, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  688.  doi:10.3969/j.issn.1000-3606.2019.09.013
    Abstract ( 434 )   PDF (1412KB) ( 331 )  
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    Objective To explore the clinical characteristics, gene mutation and pathogenesis of succinic semialdehyde dehydrogenase deficiency (SSADHD) caused by ALDH5A1 gene mutation. Method The clinical data and gene sequencing results of SSADHD in a child were retrospectively analyzed, and the related literature was reviewed. Results A 3-year- and 6-month-old girl presented with recurrent episodes of paroxysmal dystonia. However, the brain magnetic resonance imaging, video electroencephalogram, blood biochemical examination and hematuria genetic metabolism screening all found no abnormalities. Gene sequencing showed that there were two heterozygous mutations in the exon region of ALDH5A1 gene, c.112G > A (p.A38T) (pathogenicity is not yet clear) and c.1529C > T (p.S510F) (reported pathogenic mutation). The two heterozygous mutations were derived from their parents respectively, which were complex heterozygous mutations and consistent with autosomal recessive inheritance. Therefore, the child was diagnosed with SSADHD caused by ALDH5A1 gene mutation. A total of 26 related literatures were retrieved, and 75 ALDH5A1 gene mutations located in Exon1~Exon11 were reported, including missense mutations, deletion mutations, insertion mutations, splicing mutations and nonsense mutations. Conclusion ALDH5A1 gene mutation is closely related to SSADHD, and gene detection is helpful for the diagnosis of SSADHD.
    Clinical characteristics of CASK gene mutation in a child
    ZHANG Yi, YAO Ruen, XU Yufei, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  693.  doi:10.3969/j.issn.1000-3606.2019.09.014
    Abstract ( 600 )   PDF (1490KB) ( 718 )  
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    Objective To explore the clinical characteristics of CASK gene mutation. Methods The clinical data and gene test results of a child with mental retardation, microcephaly and pontocerebellar dysplasia caused by CASK gene mutation were reviewed. Results The male patient was 3 months and 27 days old. The main clinical manifestations were microcephaly, congenital laryngeal cartilage dysplasia and tracheomalacia, growth retardation, feeding difficulties, high limb muscle tension and repeated convulsions without fever. Chromosome microarray analysis did not detect clinically significant gene copy number deletion, duplication and large fragment homozygote phenomena. High-throughput sequencing and Sanger sequencing showed that the children carried a hemizygous frameshift mutation in CASK gene, c.1818_1821dupAACT, p.t608nfs *16, which was a possible pathogenic variation. Conclusion A case of mental retardation, microcephaly and pontocerebellar dysplasia caused by CASK mutation was found.
    The clinical feature and HEXA gene mutation analysis of Tay-Sachs disease in a child
    YANG Zhigang, WANG Yuan, Chen Gouhong
    Journal of Clinical Pediatrics. 2019, 37(9):  697.  doi:10.3969/j.issn.1000-3606.2019.09.015
    Abstract ( 343 )   PDF (1609KB) ( 223 )  
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     Objective To explore the clinical manifestations of Tay-Sachs disease and the characteristics of HEXA gene mutation. Methods The clinical data of Tay-Sachs disease diagnosed by gene testing in a child were retrospectively analyzed and the related literature was reviewed. Results A 1-year-7-month-old boy mainly presented with psychomotor retardation, startle reaction and seizures. Ophthalmoscopy showed macular cherry erythema, and hexosaminidase A activity was only 0.1 nmol/(mg·h). Target sequence capture and second-generation sequencing revealed two heterozygous mutations, c.1445A>T and c.1052T>C, in the exon region of the HEXA gene in the child, causing amino acid changes p. E482V and p.L351P respectively. The results of Sanger sequencing confirmed that the two mutations were from father and mother respectively and were compound heterozygous mutation. Conclusion Tay-Sachs disease is a rare neurodegenerative disease. Enzyme activity and genetic testing can help to clarify the diagnosis.
    X-linked Charcot-Marie-Tooth disease type 1 with convulsion: a case report and literature review
    Li Zhiyi, Cao Yanli, Jin Ruifeng, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  700.  doi:10.3969/j.issn.1000-3606.2019.09.016
    Abstract ( 278 )   PDF (1324KB) ( 169 )  
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    Objective To explore the pathogenesis of X-linked Charcot-Marie-Tooth disease type 1 (CMT1X) and the possible causes of convulsion. Method The clinical manifestations of CMT1X with convulsions in a child were retrospectively analyzed and the related literature was reviewed. Results The boy, aged 7 years and 6 months, presented with reversible cerebral white matter lesions and convulsion as the first symptom. Genetic testing revealed a mutation in the gap junction protein beta 1 (GJB1) gene, c.425G>A (p.R142Q), and the child was diagnosed as CMT1X. The clinical symptoms of the child were different from those reported previously. Conclusions CMT1X, with convulsion as the first symptom, is caused by channel dysfunction caused by GJB1 gene mutation, and the same mutations may present different clinical manifestations.
    The Smith-Magenis syndrome: a case report and literature review
    ZHANG Liyi, CAO Yuhong, ZHANG Guangyun, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  704.  doi:10.3969/j.issn.1000-3606.2019.09.017
    Abstract ( 257 )   PDF (1336KB) ( 361 )  
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    Objective To explore the clinical characteristics of Smith-Magenis syndrome (SMS). Methods The clinical data and the results of molecular genetics detection of SMS in a child were retrospectively analyzed and the related literature was reviewed. Results A boy, aged 5 years and 9 months, suffered from psychomotor retardation. He had sleep disorders since childhood including trouble falling asleep and shortened sleep cycles. He also had neurobehavioral symptoms including hyperactivity and self-injurious behaviors including bumping head, scratching and yanking fingernails. The patient had special facial features such as low hairline, broad forehead, flat face, low nasal bridge, tented upper lip and enamel dysplasia, brachydactyly, short and broad hands. Head magnetic resonance imaging showed enlargement of cerebral ventricle and cavum septum pellucidum. Chromosome microarray analysis showed a deletion of 17p11.2 with a length of 3.07 Mb [arr 17p11.2 (17,143,150-20,213,815) ×1]. The molecular karyotype was ascertained as 46, XY and Smith-Magenis syndrome was diagnosed finally. Conclusions For children with psychomotor retardation, distinct facial phenotype, sleep disorders, behavioral disorders, Smith-Magenis syndrome should be considered. Chromosomal microarry analysis contributes to the early diagnosis of SmithMagenis syndrome.
    Combined extracorporeal membrane oxygenation in the treatment of Kawasaki disease shock syndrome:  a case report and literature review
    ZHAO Jian, XIE Lijian, XIAO Tingting, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  708.  doi:10.3969/j.issn.1000-3606.2019.09.018
    Abstract ( 176 )   PDF (1233KB) ( 122 )  
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    Objective To explore the clinical value of extracorporeal membrane oxygenation (ECMO) in the treatment of severe Kawasaki disease shock syndrome (KDSS) complicated with cardiogenic shock. Methods The clinical characteristics of KDSS complicated with cardiogenic shock in a child and the process of treatment with ECMO were retrospectively analyzed, and the related literature was reviewed. Results A 4-year-old girl diagnosed with Kawasaki disease did not response to intravenous gamma globulin (IVIG) therapy. Diarrhea, listlessness, decreased blood pressure and multiple organ dysfunction of respiratory, cardiovascular, digestive and renal systems occurred on the 6th day of the course of the disease. The condition was still critical after fluid resuscitation, which was in line with the recommended indications of ECMO cardiac support. After the treatment of veno-arterial (V-A) mode ECMO, the patient's condition was relieved and discharged. Conclusion Early combined ECMO support therapy may be one of the effective methods for KDSS with cardiogenic shock.
    Colostrum oral smear/drip in the prevention of necrotizing enterocolitis in premature infants: a meta-analysis
    WANG Qi, ZHANG Xianhong, WEI Lu, et al
    Journal of Clinical Pediatrics. 2019, 37(9):  712.  doi:10.3969/j.issn.1000-3606.2019.09.019
    Abstract ( 177 )   PDF (1474KB) ( 57 )  
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    Objective To explore the efficacy and safety of colostrum oral smear or drip in the prevention of necrotizing enterocolitis in premature infants. Methods Systematic retrieval of PubMed, Ovid-Embase, Web of Science, Cochrane Library, CNKI, Wan Fang database and VIP database was conducted to collect the randomized controlled trials (RCTs) of colostrum oral smear/drip in premature neonates. The retrieval period was from the establishment of the database to January 2019. Two reviewers independently screened the literature, extracted the data and evaluated the quality of the literature. Meta-analysis was performed using RevMan5.3 software. Results Seven RCTs were included (368 subjects) totally. Meta-analysis showed that there were no statistical differences in incidence of necrotizing enterocolitis (OR=0.85, 95% CI: 0.40~1.79, P=0.67), length of stay (WMD=-7.09, 95% CI:-16.84~2.67, P=0.15), mortality (OR=0.64, 95% CI: 0.20~2.06, P=0.46) between colostrum oral smear/drip group and control group, while significant differences were found in the time to reach full enteral feeding (WMD= -1.90, 95% CI: -2.15~1.65, P<0.01) and days of antibiotic use (WMD= -2.57, 95% CI: -4.13~1.01, P<0.01). Conclusions Oral smear/drip of colostrum could not reduce the incidence and mortality of necrotizing enterocolitis in premature infants, and could not shorten the hospitalization days of premature infants, but it could shorten the time to reach full enteral feeding and the days of antibiotic use in hospitalized premature infants.
    A brief discussion on the pharmacologic characteristics of common inhaled glucocoticoids
    CUI Yongyao
    Journal of Clinical Pediatrics. 2019, 37(9):  718.  doi:10.3969/j.issn.1000-3606.2019.09.020
    Abstract ( 151 )   PDF (1016KB) ( 142 )  
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