Table of Content

15 October 2021 Volume 39 Issue 10

  

contents

Journal of Clinical Pediatrics. 2021, 39(10):  720.  doi:10.3969/j.issn.1000-3606.2021.10.000

Abstract ( 217 )   PDF (597KB) ( 58 )  

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A study on myocardial injury and cardiac function changes during the perioperative period of permanent pacemaker implantation in children

ZHOU Yunguo, ZHU Diqi, JI Wei

Journal of Clinical Pediatrics. 2021, 39(10):  721.  doi:10.3969/j.issn.1000-3606.2021.10.001

Abstract ( 375 )   PDF (1563KB) ( 217 )  

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Objective To investigate perioperative myocardial injury and cardiac function changes in children undergoing permanent pacemaker implantation. Methods The clinical data of children who underwent permanent pacemaker implantation for various types of bradycardia from November 2018 to October 2019 were reviewed. The blood troponin I (cTnI), creatine kinase (CK)-MB isoenzyme, and N-terminal pro-B-type natriuretic peptide (NT-proBNP) of the children were compared before the pacemaker implantation operation, 1 day after and 1 week after the operation. The left ventricular end diastolic diameter (LVEDD) Z value and left ventricular ejection fraction (LVEF) by transthoracic echocardiography before and 1 week after the operation were compared. Results Twenty-one children ( 8 boys and 13 girls) were included, with an age of 8 . 0 ( 4 . 0 - 11 . 0 ) years and a weight of 26 . 1 ( 15 . 3 - 45 . 8 ) kg. Dual-chamber pacemakers were implanted in 15 cases and single-chamber pacing in 6 cases. There were statistically significant differences in the levels of cTnI and CK-MB in 21 children before, 1 day after and 1 week after pacemaker implantation (P<0 . 001). The levels of cTnI and CK-MB were the highest one day after operation, which were higher than those before and one week after operation, respectively, with statistical significance (P<0 . 05 ). The LVDD-Z and LVEF values of 21 children one week after pacemaker implantation were lower than those before implantation, and the difference was statistically significant (P<0.05 ). Conclusions Children with bradycardia may experience shortterm, mild and reversible myocardial damage after implantation of a permanent pacemaker, but it has a protective effect on heart function.

Value of SNP array analysis in diagnosis of fetal congenital heart disease

WANG Guixi, SUN Kun, KONG Linghui, et al

Journal of Clinical Pediatrics. 2021, 39(10):  726.  doi:10.3969/j.issn.1000-3606.2021.10.002

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Objective To explore the application value of single nucleotide polymorphism (SNP) microarray in the prenatal diagnosis of fetal congenital heart disease. Methods A total of 200 pregnant women and fetuses were selected for prenatal diagnosis due to fetal congenital heart disease and/or positive ultrasound soft indicators. Chromosome karyotype and SNP array analysis were performed in amniotic fluid cells to analyze the detection rate of the two techniques and to determine the nature of copy number variation. Results Only 1 case of chromosome abnormality was detected by common karyotype, and 22 cases were detected by SNP array technique, among which 11 cases were pathogenic copy number variation (CNVs) and 11 cases were CNVs with unclear clinical significance. Conclusions SNP technology can significantly increase the detection rate, and has important application value for fetuses with congenital heart disease with normal karyotypes.

Analysis of etiology and clinical features of left bundle branch block in 43 children

LI Wei, HUANG Ping, ZHANG Li, et al

Journal of Clinical Pediatrics. 2021, 39(10):  729.  doi:10.3969/j.issn.1000-3606.2021.10.003

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Objective To investigate the etiology, clinical features and prognosis of left bundle branch block (LBBB) in children. Method The clinical data of children diagnosed with LBBB from January 2013 to December 2018 were retrospectively analyzed. Results There were 43 patients with LBBB ( 35 boys and 8 girls), and the median age was 36 months ( 9 - 72 months). There were 36 cases of complete LBBB and 7 cases of left anterior branch block. Cardiac enlargement occurred in 36 cases and cardiac function decreased in 16 cases. Twelve patients had a history of surgery for congenital heart disease (CHD), including ventricular septal defect (VSD) repair in 7 cases and complicated CHD in 5 cases. Closure of VSD was performed in 12 cases. There were 6 cases of cardiomyopathy, 4 cases of myocarditis, 4 cases of CHD before operation, and 3 normal children. There were 1 cases of craniocerebral trauma and 1 cases of drowning. LBBB occurred in 12 cases 1 - 7 days after the closure of VSD. After treatment with methylprednisolone and myocardial nutrition, 8 cases recovered immediately; 4 cases were not recovered after treatment, and sinus rhythm was restored after surgical removal of occluder and repair of VSD from 3 to 8 days after interventional occlusion. LBBB was found in 12 cases of CHD after surgery. After treatment with hormones, nourishing myocardium and other drugs, all 8 cases returned to sinus rhythm, 1 case still had LBBB; 3 cases developed LBBB after repair of VSD, which gradually progressed to degree III atrioventricular block, and permanent cardiac pacemakers were implanted. The median follow-up time was 24 months ( 12 - 49 months). One patient returned to normal early after VSD occlusion, but delayed LBBB appeared at 7 months follow-up. At the end of the follow-up, one patient still had LBBB after surgery for complicated CHD and one patient with fulminant myocarditis still had LBBB accompanied by cardiac enlargement and cardiac dysfunction. One case of dilated cardiomyopathy complicated with LBBB died due to aggravation of heart failure at the age of 2 months. The remaining children had no discomfort, no changes in electrocardiogram, no abnormalities in cardiac function and left ventricular ejection fraction. Conclusions The most common causes of LBBB in children are surgery for CHD and closure of VSD. Clinical diagnosis and treatment should be made as soon as possible, and close follow-up should be conducted.

Kawasaki disease complicated with pancreatitis and low T3 syndrome: a case report

DENG Haimei, MIN Li, WU Jinzhi, et al

Journal of Clinical Pediatrics. 2021, 39(10):  733.  doi:10.3969/j.issn.1000-3606.2021.10.004

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Objective To investigate the clinical manifestations, diagnosis and treatment of Kawasaki disease complicated with pancreatitis and low T3 syndrome. Methods The clinical data of Kawasaki disease complicated with pancreatitis and low T3 syndrome in one child were retrospectively analyzed, and the relevant literature was reviewed. Results A 4-year-old girl presented with Kawasaki disease and pancreatitis was treated at our facility. During her stay, she developed Kawasaki disease shock syndrome, low T3 syndrome, and gamma globulin resistance. After two times of gamma globulin and hormone therapy, she had no fever, but she still constantly complained abdominal pain. Through the treatment of fasting, water prohibition, suppression of pancreatic enzyme secretion, and gastrointestinal nutrition by nose jejunum tube implantation, the child was recovered and discharged. The prognosis of the child was good after six months’ follow-up. Conclusion Kawasaki disease complicated with pancreatitis and low T3 syndrome suggests a critical condition requiring early identification, intervention and follow-up of echocardiography and thyroid function.

Acute kidney injury caused by Kawasaki disease shock syndrome: a case report

ZHANG Haiyang, LUO Lili, LI Deyuan, et al

Journal of Clinical Pediatrics. 2021, 39(10):  736.  doi:10.3969/j.issn.1000-3606.2021.10.005

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Objective To explore the clinical features and treatment of Kawasaki disease shock syndrome (KDSS) with acute kidney injury (AKI). Method The clinical data of KDSS with AKI in a child was reviewed and analyzed. Results A 12 -year-old girl presented with fever and vomiting at the onset of Kawasaki disease. Acute kidney injury developed gradually after septic shock was diagnosed. The fever subsided 10 days later, urine output recovered after 5 continuous renal replacement treatment (CRRT), and blood pressure stabilized. The urea nitrogen and creatinine returned to normal. Later re-examination of cardiac color Doppler echocardiography indicated coronary artery dilation, and then the diagnosis was revised as KDSS. The patient was treated with aspirin and discharged from the hospital. Long-term follow-up showed that the coronary dilatation disappeared. Conclusions KDSS should be diagnosed from septic shock at the early stage. Once AKI is complicated, the active use of CRRT can effectively improve the prognosis.

The evaluation value of serum cholinesterase in children with severe adenovirus pneumonia

PENG Hongyan, HONG Jie, YANG Wenmin, et al

Journal of Clinical Pediatrics. 2021, 39(10):  740.  doi:10.3969/j.issn.1000-3606.2021.10.006

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Objective To explore the evaluation value of serum cholinesterase (SCHE) in children with severe adenovirus pneumonia. Methods The clinical data of 93 patients with severe adenovirus pneumonia admitted to the intensive care unit from January 2016 to September 2020 were collected. According to the SCHE level, the patients were divided into SCHE normal group (SCHE≥4650 U/L, n=39 ) and SCHE reduced group (SCHE

Clinical and genetic analysis of 31 children with spinal muscular atrophy

CHEN Yuyi, HAN Yunli, LI Xing, et al

Journal of Clinical Pediatrics. 2021, 39(10):  745.  doi:10.3969/j.issn.1000-3606.2021.10.007

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Objective To investigate the clinical phenotype and genotype characteristics of spinal muscular atrophy (SMA). Methods The clinical data of 31 children with SMA diagnosed from February 2014 to May 2019 were retrospectively analyzed. Results Among the 31 SMA children, the male to female ratio was 1 . 8 : 1 . The age of onset was as followed: 18 months in 2 cases ( 6 . 5 %). The initial symptoms were as followed: muscle hypotonia in 13 cases ( 41 . 9 %), muscle strength decline in 9 cases ( 29 . 0 %), abnormal gait in 5 cases ( 16 .1 %), and growth retardation in 4 cases ( 12 .9 %). Myasthenia was mainly found in the proximal body, the lower limb was more serious than the upper limb, and the tendon reflex weakened or disappeared. There was no change in sensation or cognition. The multiplex ligation-dependent probe amplification (MLPA) was used for gene testing. Among the 31 children, 29 ( 93 . 5 %) had homozygous deletion of exon 7 and exon 8 in SMN 1 gene, and only deletion of exon 7 was found in 2 children ( 6 . 5 %), all of whom were type 2 . The results showed that there was no significant difference between different phenotypes of SMA and SMN 1 gene deletion types (P>0 . 05 ). The SMN 2 gene copy number of SMA type 2 and 3 was higher than that of SMA type 1 , and SMN 2 gene copy number of SMA type 3 was significantly higher than that of SMA type 2 . The distribution of SMN 2 copy number among different SMA phenotypes was statistically significant (P< 0 . 05 ). The parents of 30 children ( 96 . 8 %) were found to have loss of heterozygosity of SMN1 gene. In the remaining cases, the father has SMN1 heterozygous deletion, which was not detected by the mother. Conclusions The detection and analysis of SMN 1 gene is of diagnostic significance in children with SMA. The clinical phenotypic severity of SMA is inversely proportional to the increase of copy number of SMN 2 gene.

Spinal muscular atrophy with congenital bone fractures-2: two cases in one family and literature review

YAO Lingsong, LIN Xinzhu, SHEN Wei

Journal of Clinical Pediatrics. 2021, 39(10):  750.  doi:10.3969/j.issn.1000-3606.2021.10.008

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Objective To explore the clinical features of spinal muscular atrophy with congenital bone fractures- 2 (SMABF 2 ). Methods The clinical data of 2 newborns with SMABF 2 caused by ASCC 1 gene variation were retrospectively analyzed and the relevant literatures were reviewed. Results Two children were siblings with a 4 -year age difference, and the younger brother was the proband. They were admitted to the hospital due to postnatal respiratory distress, hypotonia and arthrogryposis multiplex congenital. Because of respiratory failure, two newborns were given mechanical ventilation support after birth. Two children had finger flexion deformity, hyperextension and contracture of bilateral wrist joint and knee joint and bilateral club-foot. Whole body X-ray images showed poor skeletal development, rarefaction of bone, thin and gracile ribs. The proband suffered a fracture of the left tibiofibula, and his older brother suffered a fracture of the middle part of the right humerus. The whole exon sequencing analysis of the proband showed a novel homozygous missense variation of c. 913 C>T (p. 305 His>Tyr) in the ASCC 1 gene (NM_ 0011988002 . 2 , OMIM: 616867 ), which was located at 10 Q 22 . 1 . Both parents are carriers of heterozygous variation. ASCC 1 gene variation has not been reported in China. Conclusion SMABF 2 is an autosomal recessive inheritance disease, and ASCC 1 gene variation is the pathogenic factor.

Clinical and genetic analysis of two brothers in a family with Pelizaeus-Merzbache disease

NIU Guohui, ZHANG Xiaoli, LI Linchen, et al

Journal of Clinical Pediatrics. 2021, 39(10):  754.  doi:10.3969/j.issn.1000-3606.2021.10.009

Abstract ( 488 )   PDF (1633KB) ( 197 )  

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Objective To explore the clinical characteristics, imaging and genetic features of two brothers in a core family with Pelizaeus-Merzbache disease. Methods The clinical data of two brothers in a core family with Pelizaeus-Merzbache disease were retrospectively analyzed and followed up. Results Both the proband (male, 7 years old) and his younger brother ( 5 years old ) showed motor retardation since childhood, and so far they cannot stand and walk alone, but their intellectual development is basically unaffected. The brain magnetic resonance imaging (MRI) T 2 -weighted image showed diffuse hyperintensities in white matter. Both the proband and the younger brother had a hemizygous mutation of c. 259 delC in the proteolipid protein 1 gene. The proband’s mother was acarrier. Combined with family history, clinical manifestations and gene detection data, the type of the two brothers with Pelizaeus-Merzbache disease in a family was confimed. After 3 years followup, the motor function of both brothers was regressed. The proband can only stand with support for a short time, and the muscle strength of the limbs was grade III. the younger brother of the proband had deformed ankles and obviously talipes equinovarus， and cannot stand with support. In contrast, there was no obvious regression in their intelligence. Conclusion The two brothers had typical clinical features of Pelizaeus-Merzbache disease. With combination of clinical features and gene sequencing, they were diagnosed as Pelizaeus-Merzbache disease.

Clinical analysis of eight children with undifferentiated embryonal sarcoma of the liver in children and literature review

TANG Jingjing, MA Yihui, XU Xueju, et al

Journal of Clinical Pediatrics. 2021, 39(10):  758.  doi:10.3969/j.issn.1000-3606.2021.10.010

Abstract ( 598 )   PDF (1154KB) ( 159 )  

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Objective To analyze the clinical features, treatment and prognosis of undifferentiated embryonal sarcoma of the liver (UESL) in children. Methods The clinical data of 8 children with UESL admitted from November 2012 to June 2019 were retrospectively analyzed. Kaplan-Meier survival analysis was used to calculate the survival rate. Result There were 8 children ( 5 boys and 3 girls). The median age of onset was 7 years ( 8 months to 10 years). The tumors of the 8 children were all located in the right lobe of the liver, and the median maximum diameter was 13 . 5 cm ( 10 - 20 cm). Ultrasonography showed intrahepatic solid cystic mass with mixed echogenicity. All patients underwent complete resection of the tumor, and 3 patients had tumor rupture before surgery. Six cycles of alternating chemotherapy were performed between AVCP regimen (cisplatin, vincristine, adriamycin and cyclophosphamide) and IEV regimen (ifosfamide, etoposide and vincristine). The median follow-up time was 47 ( 24 - 90 ) months, 7 children were in complete remission, and 1 died of recurrence. The 5 -year event free survival (EFS) was ( 79 . 25±9 . 31 ) %. Conclusions Preoperative tumor rupture may occur in children with UESL. Complete resection combined with chemotherapy can achieve long-term disease-free survival.

Diagnosis and treatment of consumptive hypothyroidism secondary to infantile hepatic hemangiomatosis: a case report

XIE Lichun, YOU Jingyu, Li Guodong, et al

Journal of Clinical Pediatrics. 2021, 39(10):  761.  doi:10.3969/j.issn.1000-3606.2021.10.011

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Objective To investigate the diagnosis and treatment of infantile hepatic hemangioma (IHH). Methods The clinical data of an infant with hepatic multiple focal hemangioma presenting with recurrent anemia and congenital hypothyroidism was retrospectively analyzed and the related literatures were reviewed. Results Congenital hypothyroidism was diagnosed 2 days after birth in an infant baby girl, and oral levothyroxine treatment showed poor efficacy. At the age of 2 months, the hemoglobin was 68 g/L, and the reticulocyte count increased, and the blood routine examination suggested normal cellular anemia. Total bilirubin, unconjugated bilirubin, bile acid, glycholic acid and γ-glutamyl transpeptidase increased significantly, thyroid stimulating hormone increased significantly, and free T 3 decreased significantly. Ultrasonography showed that there were several hypoechoic masses scattered in the liver parenchyma, the largest one was located in the right lobe of liver with a size of about 8 . 9 cm×5 . 3 cm. Abdominal CT showed a significantly enlarged liver with a size of 117 mm×145 mm× 90 mm. In the liver parenchyma, there were abnormal enhancements of round shape, presenting multiple cystic lesions of varying sizes and slightly high-density septa surrounding the cystic lesions. The child was diagnosed with hepatic hemangioma and was given propranolol plus levothyroxine treatment. After 6 months of followup, abdominal ultrasonography showed that the number and size of hepatic hemangiomas had been reduced by half, and the levels of liver enzymes and bile acids had dropped to normal ranges. Therefore, the dosage of levothyroxine was gradually reduced, and the thyroid function of the child returned to normal. At present, the growth and development of the child are similar to those of normal age. Conclusions Infantile hepatic hemangioma can lead to serious complications, timely diagnosis and treatment can significantly improve the prognosis.

Hereditary hypomagnesemia caused by TRPM6 gene variation: a case report and literature review

WANG Li, SU Zhe, JIAO Yanhua, et al

Journal of Clinical Pediatrics. 2021, 39(10):  765.  doi:10.3969/j.issn.1000-3606.2021.10.012

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Objective To analyze the diagnosis and treatment of hereditary hypomagnesemia. Methods The clinical data of hereditary hypomagnesemia of one child were retrospectively analyzed and the related literatures were reviewed. Results The boy was admitted at 6 months of age due to convulsion caused by hypomagnesemia, and the blood magnesium level was only maintained at about 0 . 6 mmol/L after magnesium supplementation. Whole exome sequencing showed that there were compound heterozygous variations, c. 2495 A>G (p.Tyr 832 Cys) and c. 3357 C>A (p. Cys 1119* ), in TRPM 6 gene. The child was treated with oral magnesium for a long period of time and grew up l during follow-up. Conclusions Compound heterozygous variation in TRPM 6 gene (c. 2495 A>G and c. 3357 C>A) was responsible for hereditary hypomagnesemia 1 (intestinal). Diagnose and treatment for patients in time may avoid irreversible neurocognitive impairment.

Hereditary coagulation factor Ⅻ deficiency caused by compound heterozygous variation: a case report and literature review

HU Can, TIAN Xin, HE Xiangling, et al

Journal of Clinical Pediatrics. 2021, 39(10):  768.  doi:10.3969/j.issn.1000-3606.2021.10.013

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Objective To strengthen the understanding of hereditary factor Ⅻ (FⅫ) deficiency. Methods The clinical data of hereditary coagulation factor Ⅻ (FⅫ) deficiency caused by compound heterozygous variation in a child were retrospectively analyzed and the related literatures in recent 10 years were reviewed. Results A girl was found to have abnormal coagulation function due to inguinal hernia surgery at the age of 6 . She has no bleeding, chronic disease history or family history. The coagulation function showed that activated partial thromboplastin time (APTT) was significantly prolonged, prothrombin time (PT) was normal, and APTT correction test (immediate and delayed) could be corrected. The plasma coagulation factor activity (Ⅻ:C) was 1 . 3 % (reference range: 50 %- 150 %). Two heterozygous variations were detected in F12 gene in the child by gene sequencing. The variation of c.G 1681 A (nucleotide 1681 in coding region changing from G to A) in exon 14 came from the mother, and the variation of c.G 1330 T (nucleotide 1330 in coding region changing from G to T) in exon 11 came from the father. The parents of the child were carriers, and they had no spontaneous bleeding. It was determined as pathogenic variation by ACMG. Conclusion Genetic testing is helpful for the diagnosis of hereditary FⅫ deficiency and the genetic variation information of hereditary FⅫ deficiency can be found.

Clinical and genetic analysis of acute necrotizing encephalopathy with trilaminar appearance of insular in one family

TIAN Maoqiang, PENG Longying, LIU Shuyi, et al

Journal of Clinical Pediatrics. 2021, 39(10):  771.  doi:10.3969/j.issn.1000-3606.2021.10.014

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Objective To investigate the clinical and genetic characteristics of acute necrotizing encephalopathy (ANE) with trilaminar appearance (TA) of insular. Methods The clinical data of patients from one family diagnosed with ANE were collected and analyzed. Results The proband was a boy; he started development of convulsion and disturbance of consciousness at the age of 4 . 5 years, and a history of febrile convulsion at the age of 1 year. Head MRI showed symmetrical multifocal lesions with TA in the insular lobe. There were 6 patients in the family with onset ages ranging from 6 months to 50 years. The main symptoms of the patients were convulsion after fever or status convulsions and disturbance of consciousness, and 3 patients died of status convulsions. Whole-exon sequencing analysis confirmed that the pathogenic gene was RANBP2 (NM_ 006267 ; c. 1754 C>T [p.T 585 M]). Immunotherapy and energy support could improve the prognosis. Conclusion This study reported for the first time that TA can appear in the insula.

IPEX syndrome: a case report and literature review

WANG Jun, ZHOU Li, SUN Mengjiao, et al

Journal of Clinical Pediatrics. 2021, 39(10):  775.  doi:10.3969/j.issn.1000-3606.2021.10.015

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Objective To analyze the clinical and genetic characteristics of immune dysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome. Methods The clinical data and genetic test results of 1 patient with IPEX syndrome were analyzed, and related literatures were reviewed. Results The boy developed immune thrombocytopenia at 14 months of age. He had recurrent respiratory tract infection with secondary immune thrombocytopenia and hepatitis, accompanied by splenomegaly and lymphadenopathy. Genetic test found that the child had a hemizygotic variation in FOXP 3 gene, and nucleotide 767 changed from thymine T to cytosine C (c. 767 T >C), resulting in amino acid 256 changed from methionine to threonine (p.M 256 T). Pedigree verification showed that there was no variation at this locus in the father and a heterozygous variation at this locus was found in the mother. After one month of rapamycin treatment, the platelet level rose significantly and the liver function returned to normal. However, the patient died 3 months later due to severe infection. Conclusions IPEX syndrome is a multi-organ autoimmune disease with high mortality. Immunosuppressive therapy followed by allogeneic hematopoietic stem cell transplantation is the current optimal treatment.

Clinical and genetic analysis of hyper-IgE syndrome in two patients

REN Liying, LIU Haichao, GUO Qi

Journal of Clinical Pediatrics. 2021, 39(10):  779.  doi:10.3969/j.issn.1000-3606.2021.10.016

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Objective To explore the clinical and genetic features of hyper-IgE syndrome with recurrent infection caused by DOCK8 gene variation. Methods The clinical data of hyper-IgE syndrome in 2 children were analyzed retrospectively, including whole exome sequencing, Sanger verification of pathogenic site, and the expression level of DOCK8 gene in peripheral blood. Results Both children, 1 boy and 1 girl aged 5 years 2 months and 4 years 8 months respectively, were visited for recurrent infection, persistent rash or pustules. The children had no special facial features. The serum IgE level was significantly increased, and the eosinophils in peripheral blood were significantly increased. There were compound heterozygous variants of IVS2+1G>A and c.1729G>A (p.A577T) in DOCK8 gene of the girl, which came from her parents respectively. The DOCK8 gene of the boy had compound heterozygous variation of paternal C.2248G>A (p.E750K) and maternal C.1685T>G (p.L562R). Bioinformatics analysis showed that the three missense variants of p. A 577T, p.E 750K and p.L 562R were conserved among different species. The results of real-time PCR showed that the expression level of DOCK8 gene in peripheral blood of the girl decreased significantly. Conclusion DOCK8 gene variation is a common cause of hyper-IgE syndrome with recurrent infection, which expands the DOCK8 gene variation spectrum.

The efficacy and safety of levetiracetam and phenytoin in the treatment of children with convulsive status epilepticus: a meta-analysis

YANG Li, DONG Xianzhe, ZHANG Lan

Journal of Clinical Pediatrics. 2021, 39(10):  782.  doi:10.3969/j.issn.1000-3606.2021.10.017

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Objective To comprehensively evaluate the efficacy and safety of levetiracetam (LEV) versus phenytoin (PHT) in the treatment of convulsive status epilepticus (CSE) in children. Methods The PubMed, Web of Science, Cochrane Library, Embase, CNKI, Wanfang Database, China Biomedical Literature Database (CBM) and VIP Chinese science and technology periodical database (VIP) were retrieved. The retrieval period is from the database construction to July 17 , 2020 . Prospective randomized controlled trials (RCTs) of intravenous PHT (control group) and intravenous LEV (intervention group) for the treatment of children with CSE were screened. The data that met the inclusion criteria were extracted and their quality was evaluated, and meta-analysis was performed using Rev Man 5 . 3 software. Results A total of 7 RCTs were included, including 1647 children with CSE. The meta-analysis results showed that the complete control rate of clinical seizures within 60 minutes in the LEV group was slightly higher than that in the PHT group, but the difference was not statistically significant (RR=1 . 06 , 95%CI:1.00-1.13, P=0. 06 ). There was no statistical difference in clinical recurrence rate within 24 hours (RR=0.93, 95%CI: 0.50-1.74, P=0 . 83 ), mortality rate (RR=0.89, 95%CI: 0.18-4.48, P=0 . 89 ), ICU admission rate (RR=1 . 11 , 95 %CI: 0 . 96 - 1 . 28 , P=0 . 17 ) between the LEV group and the PHT group. The total incidence of adverse events in the LEV group was lower than that in the PHT group, and the difference was statistically significant (RR=0 . 74 , 95 %CI: 0 . 60 - 0 . 92 , P=0 . 006 ). Conclusions LEV is comparable to the second-line preferred drug PHT in children with CSE after benzodiazepine treatment failure; furthermore, LEV has low overall incidence of adverse events and may be considered as a suitable alternative to PHT.

Progress in diagnosis and treatment of epilepsia partialis continua in children

ZHOU Zhuying, WU Jing, ZHANG Zhijie, et al

Journal of Clinical Pediatrics. 2021, 39(10):  788.  doi:10.3969/j.issn.1000-3606.2021.10.018

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Epilepsia partialis continua (EPC) is a kind of focal status epilepticus caused by abnormal discharge of cortical neurons, which leads to localized and continuous muscle contraction (interval time is not more than 10 seconds, duration time is not less than 1 hour). The etiology of EPC determines its clinical characteristics, treatment and prognosis. This paper reviews the progress of diagnosis and treatment of EPC from the perspective of etiology, so as to improve the understanding of EPC.

Research progress for diagnosis and treatment of multisystem inflammatory syndrome in children

WANG Liang

Journal of Clinical Pediatrics. 2021, 39(10):  792.  doi:10.3969/j.issn.1000-3606.2021.10.019

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Multisystem inflammatory syndrome in children (MIS-C) is a severe inflammatory syndrome diagnosed as Kawasaki-like disease manifested by toxic shock and cardiogenic or vascular paralytic shock. MIS-C usually has atypical onset age (older) and ethnic background (non-Asian). MIS-C is a severe condition and can progress rapidly and deteriorate in a short time. MIS-C is potentially associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV- 2 ). Since the pathogenesis of MIS-C is unclear, the treatments of MIS-C are based on treatment strategies in Kawasaki disease or coronavirus disease 2019 (COVID- 19 ), and it is reported some of new treatment strategies are effective. This article reviews the status and progress of diagnosis and treatment for MIS-C.

The diagnosis and management of hypertriglyceridemic pancreatitis in children

ZHANG Qin, MIAO Hongjun

Journal of Clinical Pediatrics. 2021, 39(10):  797.  doi:10.3969/j.issn.1000-3606.2021.10.020

Abstract ( 233 )   PDF (1131KB) ( 136 )  

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