Table of Content

15 June 2021 Volume 39 Issue 6

  

contents

Journal of Clinical Pediatrics. 2021, 39(6):  400.  doi:10.3969/j.issn.1000-3606.2021.06.000

Abstract ( 209 )   PDF (519KB) ( 146 )  

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Paying attention to the rational use of inhaled glucocorticoids in children

WU Jiaying, ZOU Xiaojian, HONG Jianguo

Journal of Clinical Pediatrics. 2021, 39(6):  401.  doi:10.3969/j.issn.1000-3606.2021.06.001

Abstract ( 378 )   PDF (1049KB) ( 349 )  

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Establishment of predicted value of lung function determined by impulse oscillometry in children in Shanghai

WU Jinhong, ZHANG Hao, DONG Xiaoyan, et al

Journal of Clinical Pediatrics. 2021, 39(6):  405.  doi:10.3969/j.issn.1000-3606.2021.06.002

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Objective To establish the predicted value of lung function determined by impulse oscillometry (IOS) in children ( 5 ~ 11 years old) in Shanghai. Methods A total of 746 healthy children aged 5 to 11 in Shanghai were included. The Master Screen IOS pulmonary function device (Jaeger Co, Germany) was used to detect the respiratory total impedance (Zrs) , response frequency (Fres), viscosity resistance ( Rrs ) and elastic resistance (Xrs) at various oscillation frequencies, and the indexes above were analyzed. The stepwise multivariate regression was used to establish the regression equation of related parameters of IOS in different genders. Results The median age of 746 children ( 510 boys and 236 girls) was 8 years, ranging from 5 to 11 years. The height of male in 5 -year-old group was higher than that of female, and the weight of male in 5 -, 7 - and 10 -year-old group was higher than that of female, and the differences were statistically significant (P< 0 . 05 ). The differences in the main IOS parameters between different age and height groups are statistically significant regardless of sex (P< 0 . 05 ). The values of Zrs, Fres, R5 and R20 decreased with the increase of age and height, while X5 increased with the increase of age and height. The stepwise multivariate regression analysis showed that IOS parameters were related to height, age and weight, and most IOS parameters were most closely related to height (the absolute value of regression coefficient was the largest). Conclusions The study revealed the normal predicted value of pulmonary function by IOS in healthy children in Shanghai. In the evaluation of the results of the lung function measurements, the use of this predicted value equation is more in line with the characteristics of Chinese children than that of foreign countries.

Effect of antenatal corticosteroids on respiratory diseases in late-preterm infant

WANG Hui, CAO Lifang, ZHANG Xuefeng

Journal of Clinical Pediatrics. 2021, 39(6):  410.  doi:10.3969/j.issn.1000-3606.2021.06.003

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Objective To explore the effect of antenatal corticosteroids on respiratory diseases in late-preterm infants. Methods The clinical data of late-preterm infants delivered from January 2016 to June 2020 and their mothers were retrospectively analyzed. According to prenatal application of dexamethasone, the infants were divided into hormone group and control group, and the incidence of respiratory diseases in late preterm infants with different gestational ages was compared. Multivariate logistic regression analysis was used to analyze the risk factors of post-natal respiratory diseases in late preterm infants. Results In the gestational age group of 36 ~ 36+ 6 weeks, the rate of cesarean section in the hormone group was higher than that in the control group. In the gestational age group of 35 ~ 35+ 6 weeks and 36 ~ 36+ 6 weeks, the rate of premature rupture of membranes in the hormone group was lower than that in the control group, and the differences were statistically significant (P

A retrospective study of non-Magill forceps-assisted less invasive pulmonary surfactant administration (LISA) technique

LIU Xiao, CHEN Pingyang, TAN Xin, et al

Journal of Clinical Pediatrics. 2021, 39(6):  415.  doi:10.3969/j.issn.1000-3606.2021.06.004

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Objective To compare the non-Magill forceps-assisted less invasive pulmonary surfactant (PS) administration (LISA) technique and the intubation surfactant and extubation (InSurE) technique. Methods The clinical data of 172 preterm infants (< 32 weeks of gestational age) with neonatal respiratory distress syndrome (NRDS) treated with PS were retrospectively analyzed. In the LISA group (n= 106 ), PS was injected with non-Magill forceps-assisted LISA technique, while in the InSurE group (n= 66 ), PS was injected with InSurE technique. The clinical characteristics of the two groups were compared and the effects of the two methods on prognosis were analyzed. Results In the LISA group, gestational age was smaller, body weight was lower, proportion of male and usage of poractant alfa were more than those in the InSurE group. The differences were statistically significant (P< 0 . 05 ). After the propensity score matching (PSM), a total of 43 pairs of cases were successfully matched, there was no significant difference between the two groups in baseline data (P> 0 . 05 ). There were no significant differences between the two groups after PSM in the main outcomes (mortality and incidence of bronchopulmonary dysplasia) and secondary outcomes (oxygen consumption time, noninvasive ventilation time, mechanical ventilation time, mechanical ventilation rate within 72 hours after birth, mechanical ventilation rate 72 hours after birth and total mechanical ventilation rate, etc.) (all P>0.05). Conclusions Compared with InSurE technology, non-Magill forceps-assisted LISA technique is safe and feasible, but whether it can reduce the incidence of BPD and mortality remains to be further investigated.

Mycoplasma pneumoniae pneumonia complicated with cardiac thrombus and pulmonary embolism: a case report and literature review

HUANG Kunling, NIU Bo, LU Sukun, et al

Journal of Clinical Pediatrics. 2021, 39(6):  421.  doi:10.3969/j.issn.1000-3606.2021.06.005

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Objective To explore the clinical characteristics, diagnosis and treatment of Mycoplasma pneumoniae pneumonia (MPP)complicated with cardiac thrombus and pulmonary embolism in children. Methods The clinical data of MPP complicated with cardiac thrombus and pulmonary embolism in a child were retrospectively analyzed, and the cases of MPP complicated with cardiac thrombus and pulmonary embolism in CNKI, Wanfang database and PubMed were searched and analyzed. Results A 4 -year-old boy presented with fever and cough; his serum Mycoplasma pneumoniae (MP) antibody titer was higher than 1 : 1280 , and D-dimer was significantly increased. CT showed a large area of inflammatory consolidation in the left lung, and echocardiography showed a mass in the right ventricular cavity. The patient received combined treatment of right ventricle tumor extraction, bronchoscopic lavage and anti-infection, and pulmonary inflammation was improved for a time. Subsequently, the child's condition worsened, and chest enhanced CT showed left lower pulmonary embolism. After anticoagulant therapy, pulmonary inflammation and pulmonary embolism were improved. The postoperative pathology of the right ventricle mass of the child showed inflammatory necrotic tissue. The child was finally diagnosed of MPP with intracardiac thrombosis and pulmonary embolism. Four cases of MPP complicated with intracardiac thrombosis were reported in domestic and foreign literatures, and all of them were boys aged from 4 to 9 years old. Intracardiac thrombosis was found in 3 children 4 - 11 days after onset, 4 children had positive anti-cardiolipin antibody, 2 had elevated D-dimer, 2 underwent surgery, and 2 received anticoagulation therapy. All children were cured and anti-cardiolipin antibodies turned negative at 3 months of followup. Conclusions When MP infection causes lobar pneumonia or pleural effusion, D-dimer levels and anticardiolipin antibodies should be dynamically monitored and so embolism or thrombosis can be timely detected. Early diagnosis and timely treatment will lead to a good prognosis.

The etiological analysis of hydronephrosis with urinary tract infection in 98 children

GU Songlei, SHEN Tong, YANG Xiaoqing

Journal of Clinical Pediatrics. 2021, 39(6):  425.  doi:10.3969/j.issn.1000-3606.2021.06.006

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Objective To explore the etiology of hydronephrosis with urinary tract infection in children. Methods The clinical data of 98 children hospitalized with urinary tract infection who were found to have renal pelvis separation by urinary tract color Doppler ultrasonography from 2010 to 2020 were retrospectively analyzed. Results Based on the urinary tract dilation grading system, hydronephrosis was graded in 98 children with urinary tract infection: normal in 42 cases, grade 1 in 17 cases, grade 2 in 28 cases and grade 3 in 11 cases. Thirty-six children ( 64 . 3 %) with hydronephrosis grade 1 , 2 and 3 were complicated with urinary malformation, the incidence was higher than that in the normal group ( 18 cases, 42 . 9 %), and the difference was statistically significant (χ 2 =4.45, P= 0 . 035 ). The urinary malformations in hydronephrosis mainly included 16 cases of ureteropelvic junction obstruction, 16 cases of vesicoureteral reflux and 8 cases of vesicoureteral junction stricture. The ureteropelvic junction obstruction and vesicoureteral junction stricture were mainly on the left side, while vesicoureteral reflux was mainly bilateral, and the difference was statistically significant (P> 0 . 05 ). There was significant difference in operation rate among different grades of hydronephrosis (P< 0 . 05 ). Conclusion The common etiology of hydronephrosis is urinary malformation, among which the obstruction of the ureteropelvic junction and vesicoureteral reflux is the most common, followed by vesicoureteral junction stricture. The classification of hydronephrosis and urinary tract malformations can be considered as the basis for diagnosis and treatment.

Clinical-pathological feature of poststreptococcal glomerulonephritis with the nephrotic-range proteinuria in children

TU Juan, CHEN Chaoying, XIA Hua, et al

Journal of Clinical Pediatrics. 2021, 39(6):  429.  doi:10.3969/j.issn.1000-3606.2021.06.007

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Objective To explore the clinical-pathologic characteristic, prognosis and efficacy of steroid in acute poststreptococcal glomerulonephritis (APSGN) with the nephrotic-range proteinuria in children. Methods The clinical data of 50 cases of APSGN with nephritic proteinuria in recent 10 years were retrospectively analyzed. Results The median onset age of 50 children ( 35 boys and 15 girls) was 8 . 7 ( 6 . 8 - 10 . 0 ) years. The main clinical manifestations were hypertension ( 43 cases), gross hematuria ( 38 cases), edema ( 37 cases), acute kidney injury ( 34 cases) and hypoproteinemia ( 27 cases). All the 16 children who underwent renal biopsy had endocapillary proliferative glomerulonephritis, 15 had renal tubular lesions of varying degrees, 8 had renal interstitial changes, 7 had extensive foot process fusion, 5 had crescent formation, and 3 had extensive capillary loop compression and narrowing. The quantitative 24-hour urine protein of children with hypertension and gross hematuria is higher than that of children without hypertension and gross hematuria, and the difference is statistically significant (P< 0 . 05 ). The glomerular filtration rate (eGFR) of children with renal interstitial disease was lower than that of children without renal interstitial disease (Z=2.79 , P= 0 . 005 ). Kaplan-Meier survival curve showed that the recovery of renal function in the glucocorticoid treatment group was better than that in the no-treatment group, and the difference was statistically significant (χ 2 = 6 . 11 , P= 0 . 013 ). Conclusion APSGN with nephrotic-range proteinuria can be accompanied by acute kidney injury and obvious tubular interstitial damage, and glucocorticoid is helpful to the recovery of renal function

Progressing of nephronophthisis with different NPHP gene variations to end-stage renal disease in 3 cases

XU Xinxing, SUN Zhouyun, DENG Fan, et al

Journal of Clinical Pediatrics. 2021, 39(6):  433.  doi:10.3969/j.issn.1000-3606.2021.06.008

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Objective To explore the clinical characteristics of nephronophthisis caused by different NPHP gene variations. Methods The clinical data of nephronophthisis caused by different NPHP gene variations in 3 children were retrospectively analyzed. Results All of the 3 cases were female and had manifestations of anemia. Case 1 had extrarenal manifestations such as visceral inversion and abnormal liver function. Renal pathology of case 2 and 3 showed glomerular fibrosis, destruction of the integrity of the tubular basement membrane, tubule atrophy and infiltration of inflammatory cells in the renal interstitium. Case 1 and 2 had a family history of kidney disease. The younger brother of case 3 carried the same variation but did not have any clinical manifestations. NPHP gene variations were identified in all 3 cases by genetic testing. In case 1 , there were heterozygous variants of c. 388 A>C p.K 130 Q and c. 1465 G>A, p.V 489 M in NEK 8 /NPHP 9 gene, both of which were newly discovered. Case 2 had whole deletion of NPHP 1 gene. In case 3 , there was a homozygous variation of c.3218 T>G, p.L1073 * in NPHP3 gene, and the same homozygous variation site was found in her younger brother. All 3 patients had received renal transplantation and were under follow-up. Conclusions Nephronophthisis is a kind of clinical and genetic heterogeneous disease with no specific clinical manifestations, and genetic testing is helpful for diagnosis. Renal transplantation is an effective treatment for the progression from nephronophthisis to end-stage renal disease. The heterozygous missense variation of c.388A>C p.K 130 Q and the heterozygous variation of c.1465 G>A, p.V 489 M are newly discovered NPHP gene variants

Clinical features and risk factors of neuropsychiatric lupus in children

SHI Danying, LI Xing, HAN Yunli, et al

Journal of Clinical Pediatrics. 2021, 39(6):  437.  doi:10.3969/j.issn.1000-3606.2021.06.009

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Objective To explore the clinical features and risk factors of neuropsychiatric systemic lupus erythematosus (NPSLE) in children. Methods The clinical data of SLE in children hospitalized from January 2013 to December 2018 were retrospectively analyzed. Results A total of 315 children with SLE were included. 58 ( 11 boys and 47 girls) were diagnosed with NPSLE and an average age was 10 . 45 ± 2 . 63 years. There were 14 kinds of neuropsychiatric symptoms, including headache ( 21 cases), epileptic seizure ( 19 cases), cerebrovascular accident ( 15 cases), demyelinating syndrome ( 13 cases) and emotional disorder ( 10 cases). Fifty-eight children with NPSLE underwent cranial imaging examination, and 53 cases ( 91 . 4 %) were abnormal; 44 cases underwent EEG examination, and 38 cases ( 86 . 4 %) were abnormal; 39 cases underwent cerebrospinal fluid examination, and 34 cases ( 87 . 2 %) were abnormal. The incidence of platelet count < 100 × 109 /L was higher in children at first-onset of NPSLE than that in children without NPSLE, and the difference was statistically significant (P< 0 . 05 ). The case fatality rate of the 23 children ( 21 . 7 %) who developed disease from January 2013 to December 2015 was higher than that of the 35 children (case fatality rate of 2 . 9 %) who developed disease from January 2016 to December 2018 , and the difference was statistically significant (P< 0 . 05 ). Conclusions The most common symptoms of NPSLE are headache and seizures, and children with decreased platelet count have earlier neuropsychiatric symptoms. The mortality rate of children with NPSLE has decreased in recent years. Low platelet count, positive anticardiolipin antibody, and nonstandard hormone therapy are the risk factors for NPSLE in children with SLE.

Clinical characteristics and prognosis of recurrent immune cerebellar ataxia in children

LIAO Yinting, CHEN Wenxiong, HOU Chi, et al

Journal of Clinical Pediatrics. 2021, 39(6):  442.  doi:10.3969/j.issn.1000-3606.2021.06.010

Abstract ( 459 )   PDF (1072KB) ( 344 )  

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Objective To analyze the clinical features, treatment and prognosis of recurrent immune cerebellar ataxia in children. Methods Clinical data of relapsed immune cerebellar ataxia in 11 children diagnosed from January 2016 to June 2020 were collected and analyzed. Results The median onset age of 11 children ( 4 boys and 7 girls) was 2 . 2 years ( 0 . 8 - 10 . 8 years). Seven children had prodromal infection or vaccination during the first course of the disease. All the 11 children had abnormal gait or unsteadiness, 8 had intentional tremor, 5 had dyslexic articulation, 5 had body wobble and 5 had nystagmus. Among the 11 children, 5 children were positive for systemic autoantibodies, 2 had elevated CSF leukocytes, and 2 were positive for paraneoplastic syndrome antibodies. Two children had slow background activity of electroencephalogram. During the course of recurrence, cerebellar atrophy was showed in 3 children by cranial magnetic resonance (MRI) and cerebellar hypometabolism was showed in 2 children by PET-CT. The median follow-up time was 27 months ( 10 - 48 months) in the 11 children who had 21 relapses in total and the median recurrence interval was 4 months ( 3 - 24 months). The causes of recurrence were infection ( 10 times), hormone reduction ( 7 times), Mycophenolate Mofetil reduction ( 1 time) and no obvious inducement ( 3 times). Intravenous infusion of human immunoglobulin and methylprednisolone were the preferred treatment for all the children at the first onset, and their symptoms disappeared or were relieved. After rituximab immunosuppressive therapy, recurrence was reduced in 6 children who had repeated recurrence. Mediastinal ganglioneuroblastoma was found in 2 children with recurrence and surgical resection was performed, and then the symptoms disappeared or alleviated. The median follow-up time after the last attack in the 11 children was 13 months ( 1 - 35 months). Four cases had complete remission, 4 cases had broad basal gait, 3 cases had slight wobble of trunk and limbs, and 2 cases had unclear articulation. The annual recurrence rate after immunosuppressive therapy decreased from 1 . 6 to 0 . 19 . Conclusions The most common predisposing factor of recurrent immune cerebellar ataxia in children is infection. It happens mostly in female. Human immunoglobulin and hormone shock therapy were effective at the first onset, and immunosuppressive therapy could improve the condition and reduce the recurrence in patients with multiple relapses. Some patients with tumors should be treated etiologically.

Clinical and genetic analysis of molybdenum cofactor deficiency: a case report

ZHANG Guangyu, LI Sansong, YANG Lei, et al

Journal of Clinical Pediatrics. 2021, 39(6):  446.  doi:10.3969/j.issn.1000-3606.2021.06.011

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Objective To explore the clinical and genetic features of molybdenum cofactor deficiency. Method The clinical data of molybdenum cofactor deficiency in a patient was retrospectively analyzed. Result A 6 -month-old girl was born with no abnormalities. However, she had feeding difficulties and convulsion immediately after birth, and gradually developed microcephaly and spastic tetraplegia, etc. Whole exome sequencing showed compound heterozygous variation of c. 473 T>G (p.Leu 158 *) and c. 472 _ 477 del (p.Leu 158 _Lys 159 del) in MOCS2 gene, which were inherited from her father and mother respectively. According to the ACMG guidelines, the c. 473 T>G variation was classified as pathogenic, while the c. 472 _ 477 del variation was classified as likely pathogenic. Conclusion The child was diagnosed with molybdenum cofactor deficiency caused by heterozygous variation in the MOCS 2 gene.

Study on a family of dilated cardiomyopathy caused by SRCAP gene mutation

LI Yanping, ZHANG Yongwei, CHEN Juan, et al

Journal of Clinical Pediatrics. 2021, 39(6):  449.  doi:10.3969/j.issn.1000-3606.2021.06.012

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Objective To analyze the correlation between the genotype and clinical phenotype of the family with dilated cardiomyopathy caused by SRCAP gene mutation. Methods To retrospectively analyze the clinical data of a child with idiopathic dilated cardiomyopathy, use whole exome sequencing technology to find the pathogenic gene, and verify with Sanger sequencing, use I-TASSER software to predict whether the pathogenic gene affects protein structure and Features. Results The whole exome sequencing of a 16 -month male child with DCM found that SRCAP c. 452 - 453 del, pPhe 151 Cysfs* 71 gene mutations were found. The family verified that the mutations were new heterozygous mutations through Sanger sequencing technology, and the inheritance mode was autosomal dominant. Genetic. Cysteine at position 151 of SRCAP gene is highly conserved among different species. I-TASSER software predicts 3230 residues of wild-type protein and 220 residues of mutant protein. Hydrophilicity analysis wild-type hydrophilic Sum (5:3226) = -1449.44, mutant hydrophilic Sum (5:216) =-126.55. Conclusion SRCAPc. 452 - 453 del, pPhe 151 Cysfs* 71 gene mutation is a new site mutation, which leads to premature termination of peptide chain synthesis, protein structure truncation and significant changes in hydrophilicity and hydrophobicity. Combining genotype and clinical phenotype analysis, consider The SRCAPc. 452 - 453 del mutation at this site is a new pathogenic mutation of DCM. The clinical characteristics were recurrent respiratory tract infection, high myocardial enzyme spectrum, decreased myocardial contractility, hyperlactic disease, backward language development, no malignant arrhythmia.

Monitoring of indicators in the treatment of 21- hydroxylase deficiency in adolescent children

XU De, LU Wenli

Journal of Clinical Pediatrics. 2021, 39(6):  453.  doi:10.3969/j.issn.1000-3606.2021.06.013

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Objective To explore the hormone levels in adolescent children with 21 -hydroxylase deficiency ( 21 -OHD), and to screen the high specific indicators. Methods The clinical data of 21 -OHD in adolescent children were collected. According to the degree of masculinity and bone age, the children were divided into the well-controlled group and the poorly-controlled group, and the differences of body mass index (BMI), hydrocortisone dose and steroid hormone levels between the two groups were compared. The receiver operating characteristic (ROC) curve was used to define the cutoff value for poor control. Results The mean age of puberty development in 43 included children with 21 -OHD was 8 . 1 ± 2 . 1 years ( 3 . 3 ~ 12 . 9 years). Among them, there were 14 boys and 16 girls with simple virilizing (SV) type. Salt-wasting (SW) type was found in 8 boys and 5 girls. In both men and women, the levels of 17 -hydroxyprogesterone ( 17 -OHP), adrenocorticotropic hormone (ACTH), androstenedione (AD) and AD/testosterone (T) in the poorly-controlled group were higher than those in the well-controlled group; the T level of poorly-controlled group was higher than that of well-controlled group, and the differences were statistically significant (P< 0 . 05 ). ROC curve analysis indicated that 17 -OHP combined with AD/T had the highest diagnostic value (AUC, 0 . 907 for boy and 0 . 814 for girl); when the cut-off value of 17 -OHP and AD/T for boy was 14 . 88 ng/mL and 4 . 17 , the sensitivity and specificity were 0 . 824 and 0 . 889 respectively; when the cut-off value of 17 -OHP and AD/T for girl was 27 . 23 ng/mL and 4 . 63 , the sensitivity and specificity were 0 . 848 and 0 . 646 respectively. Conclusions In adolescence, 17 -OHP combined with AD/T has certain value in monitoring of replacement therapy, but the steroid hormone level and AD/T ratio vary in patients with different types and genders, so individualized adjustment of the treatment plan is required.

The clinical phenotype and gene variation of two children with Kabuki syndrome

WENG Lingwei , XU Yuyan, LIN Kexin, et al

Journal of Clinical Pediatrics. 2021, 39(6):  459.  doi:10.3969/j.issn.1000-3606.2021.06.014

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Objective The clinical and molecular genetic characteristics of Kabuki syndrome (KS). Methods The clinical data of 2 children with KS who were treated with growth retardation was analyzed etrospectively. Using the nextgeneration sequencing technology, the children and their parents were sequenced with short stature-related genome or whole exome and whole genome copy number variation (CNV) detection. Results Two female children aged 36 months and 3 months old went to the doctor due to feeding difficulties and growth retardation. The clinical manifestations are special appearance, the length and weight Z scores for age are less than -2 . 5 , developmental delay, scoliosis, and abnormal head MRI. Case 1 has an ectopic right kidney, and case 2 has atrial septal defect. The results of genetic testing and analysis show KMT 2 D gene exon34 c. 10139 delA (p.K 3380 Sfs* 12 ) heterozygous mutation. KDM 6 A gene exon 16 c. 1909 - 1912 delTCTA (p.Ser 637 Thrfs* 53 ) heterozygous mutation. All are frameshift mutations. They are new and pathogenic mutations. Conclusions It is suggesting that children with growth retardation, feeding difficulties with growth retardation and special facial features should undergo genetic examination as soon as possible to confirm the diagnosis.

Genetic analysis of Menkes disease in 2 children

PENG Wei, WANG Sanmei, MA Ning, et al

Journal of Clinical Pediatrics. 2021, 39(6):  463.  doi:10.3969/j.issn.1000-3606.2021.06.015

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Objective To explore the genetic variation characteristics of Menkes disease. Method The clinical data and ATP 7 A gene test results of Menkes disease in 2 children were retrospectively analyzed. Results Case 1 was a 2 -month-old boy with a homozygous variation of c. 4077 dupT in exon 21 of ATP 7 A gene. Case 2 was a 4 -month-old boy with a homozygous variation of c. 2354 delC in exon 10 of ATP 7 A gene. The pedigree of the two children confirmed that the parents were wild type. These two variants were not reported in HGMD database, and were classified as pathogenic variants according to ACMG guidelines. Conclusion The genetic analysis of the two families enriched the variation spectrum of ATP 7 A gene in Chinese, and also provided help for clinicians in early diagnosis and genetic counseling.

Research progress of the relationship between childhood asthma and chronic obstructive pulmonary disease

XIE Yili

Journal of Clinical Pediatrics. 2021, 39(6):  467.  doi:10.3969/j.issn.1000-3606.2021.06.016

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Asthma and chronic obstructive pulmonary disease (COPD) are common chronic airway diseases with airflow limitation in childhood and adulthood. Studies have found that asthma in childhood increases the risk of developing COPD in adulthood, and changes in lung function are an important predictor of COPD risk. The establishment of long-term monitoring and management of pulmonary function for children with asthma is of great significance for assessing the risk of irreversible airway obstruction in adulthood. The article reviews the research progress of childhood asthma and COPD.

Short-chain fatty acids and intestinal mucosal immunity in food allergy

LI Xinyue

Journal of Clinical Pediatrics. 2021, 39(6):  471.  doi:10.3969/j.issn.1000-3606.2021.06.017

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The pathogenesis, diagnostic criteria, and treatment options of food allergy still need to be further explored. Short-chain fatty acids (SCFAs) are one of the metabolites of the intestinal microbiome, and the researches on their immunomodulatory effects are gradually increasing and have been applied to the clinical treatment of certain immune-related diseases. In order to gain a comprehensive understanding of the intestinal immunomodulation and potential therapeutic role of SCFAs in food allergy, this paper will briefly review the intestinal mucosal immune mechanism in food allergy, the link between SCFAs and food allergy, and the immunomodulatory role of SCFAs in it.

Progress in diagnosis and treatment of idiopathic membranous nephropathy in children

WANG Nannan

Journal of Clinical Pediatrics. 2021, 39(6):  476.  doi:10.3969/j.issn.1000-3606.2021.06.018

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Membranous nephropathy (MN) is an immune complex mediated glomerular disease and mostly manifests as nephrotic syndrome. In the pediatric population, the MN in children is more often secondary to hepatitis B virus infection or systemic lupus erythematosus. In recent years, the incidence of pediatric idiopathic MN is increasing. M-type phospholipase A 2 receptor (PLA 2 R) and thrombospondin type- 1 domain-containing 7 A (THSD 7 A) antibody were initially found in adults. They can be used as biomarkers for the diagnosis of adult idiopathic MN, judgment of disease activity, evaluation of prognosis and assessment of therapeutic effect. Now they are also important indicators for the diagnosis and monitoring of pediatric MN. Conservative support is recommended for patients with non-nephrotic level of proteinuria and low risk of disease progression; immunosuppressive therapy should be given to patients with persistent massive proteinuria, severe complications or deterioration of renal function. This paper reviews the diagnosis and treatment of idiopathic membranous nephropathy in children.