Table of Content

15 February 2021 Volume 39 Issue 2

  

Value of Oxford pathological classification in children with Henoch-Schönlein purpura nephritis

TIAN Yingying, SHAO Xiaoshan, LI Yuhong, et al

Journal of Clinical Pediatrics. 2021, 39(2):  81.  doi:10.3969/j.issn.1000-3606.2021.02.001

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Objective? To evaluate the value of the five pathological indexes (MESTC) of Oxford classification in clinical indicators and prognosis of Henoch-Sch?nlein purpura nephritis (HSPN) in children. Methods The clinicopathological data of children with HSPN diagnosed by clinical and renal biopsy from January 2014 to February 2019 were collected. Pathology reports and other data were then reclassified according to the Oxford pathological classification by two professional nephropathologists. The correlations between the five indicators of Oxford classification and clinical indicators and prognosis in children with HSPN were analyzed. Results A total of 102 children with HSPN were collected, including 55 males and 47 females, with an average age of onset of 8. 72 years ( 3. 9 - 15 years). According to the Oxford typing score, they were divided into mesangial hypercellularity (M) ( 89 cases of M 1 and 13 cases of M 0 ), endothelial hypercellularity (E) ( 62 cases of E 1 and 40 cases of E 0 ), segmental glomerulosclerosis (S) ( 39 cases of S 1 and 63 cases of S 0 ), tubular/interstitial lesions (T) ( 37 cases of T 1/T 2 and 65 cases of T 0 ), and crescent (C) ( 39 cases of C 1/C 2 and 63 cases of C 0 ). M, E, S and C lesions were correlated with International Study of Kidney Disease in Children (ISKDC) classification (P< 0. 05 ). The proportion of hematuria proteinuria in E 1 and C 1/C 2 groups was lower than that in the E 0 and C 0 groups; the proportion of nephrotic syndrome type was higher in the E 1 group than in the E 0 group, and differences were statistically significant (P< 0. 05 ). Urea nitrogen in group M 1 was higher than that in group M 0, urine protein in group E 1 was higher than that in group E 0, urea nitrogen in group S 1 was higher than that in group S 0, uric acid and creatinine in group T 1/T 2 were all higher than that in group T 0, and uric acid, creatinine and urine protein in group C 1/C 2 were all higher than that in group C 0, and differences were all significant (all P are correlated with various clinical indicators and prognosis of children with HSPN, which suggested that the Oxford classification has certain clinical application value in children with HSPN.

Clinical and pathological analysis of 1002 children with chronic kidney disease

FENG Shipin, WANG Li, LIU Xi, et al

Journal of Clinical Pediatrics. 2021, 39(2):  87.  doi:10.3969/j.issn.1000-3606.2021.02.002

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Objective? To investigate the clinical and pathological features of children with chronic kidney disease (CKD). Methods? The clinical and pathological data of 1002 hospitalized children with CKD were retrospectively analyzed. Results? Among the 1002 children with CKD included in the study, the male to female ratio was 1 . 7: 1 and the median age of onset was 7 years. 973 patients with CKD I, 4 patients with CKD II, 7 patients with CKD III, 6 patients with CKD IV, and CKD V 12 cases. The deposit disease is mainly acquired glomerulopathy in 390 cases of primary nephrotic syndrome ( 38 . 9%), 372 cases of Henoch-Sch?nlein purpura nephritis ( 37. 1%), and 100 cases of IgA nephropathy ( 10%). Pathological classification included 108 cases of nephropathy and 594 cases of non-minor lesions. 990 cases were fully followed up, 12 were lost and 4 died, with a median follow-up of 894 days. Conclusion? Acquired glomerular disease is the main cause of CKD in children. The pathological changes are mainly non-minimal lesions. Early diagnosis and intervention are the key factors to delay the progression to end-stage renal failure in children with CKD.

Neonatal distal renal tubular acidosis caused by ATP6V0A4 gene mutation: a case report and five-year follow-up results

ZHAO Xiaoying, LIU Fang

Journal of Clinical Pediatrics. 2021, 39(2):  91.  doi:10.3969/j.issn.1000-3606.2021.02.003

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Objective? To explore the genotype, clinical characteristics and long-term development of neonatal distal renal tubular acidosis (DRTA). Methods? The clinical data of a DRTA child were retrospectively analyzed. Results A girl, born at full term by vaginal delivery, suffered from no weight gain, poor sleep, crying and mild dehydration after birth. Repeated blood gas tests suggested severe hyperchloric acidosis, hypokalemia, alkaline urine and renal calcium deposition. Renal ultrasonography showed nephrocalcinosis. High-throughput gene sequencing revealed compound heterozygous variants in ATP 6 V 0 A 4 gene including a frameshift variant of c. 2351 dupT from the father (the coding region No. 2351 was inserted into thymine, which resulted in amino acid changes of p.F 785 Ifs* 28 ) and a frameshift variant of c. 1504 dupT from the mother (the coding region No. 1504 was inserted into thymine, which resulted in amino acid changes of p.Y 502 Lfs* 22 ). This variant was a pathogenic variant and lead to the diagnosis of autosomal recessive DRTA. After 5 years of treatment with citrate mixture, the children's growth and development caught up to normal, and with no obvious complications. Conclusion? For neonatal DRTA, genetic risk factors should be alerted and gene sequencing should be carried out to make a definite diagnosis when necessary. Intervention should be given as early as possible.

Childhood atypical hemolytic uremic syndrome caused by CFHR family gene variation: a case report and literature review

CUI Jieyuan, ZHANG Dongfeng, LI Chunzhen, et al

Journal of Clinical Pediatrics. 2021, 39(2):  95.  doi:10.3969/j.issn.1000-3606.2021.02.004

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Objective? To analyze the clinical characteristics of atypical hemolytic uremic syndrome (aHUS) caused by CFHR family gene variation in a child. Methods? The clinical data and the results of related gene mutation analysis by second-generation sequencing technology were retrospectively analyzed in a child with aHUS. Results? The 8 -year-old girl presented with ochriasis and had hematuria after respiratory infection. Laboratory examination showed hemolytic anemia, thrombocytopenia, acute kidney injury and decrease in complement C 3 . No abnormalities were found in complement C 4 . Renal pathology showed thrombotic microangiopathy. The child was clinically diagnosed with aHUS. Gene analysis showed homozygous deletion of CFHR1 and CFHR3 genes and a heterozygous indel mutation (c.333_3del, p.1112Ffs*18 ) in CFHR2 genes. Genetic analysis of the mother and brother revealed the same results as those in the child, and the father only had homozygous deletion of CFHR1 and CFHR3 genes. The parents are not consanguineous. Hematuria related screening results of parents and younger brother were normal. Conclusion? A case of childhood aHUS caused by homozygous deletion of CFHR1 and CFHR3 and a heterozygous deletion of CFHR2 is reported for the first time.

Mizoribine and benazapril-associated hyperuricemia and acute kidney injury: a case report with review of the literature

HE Xu, XIA Zhengkun

Journal of Clinical Pediatrics. 2021, 39(2):  99.  doi:10.3969/j.issn.1000-3606.2021.02.005

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Objective? To investigate the clinical characteristics and pathogenesis of hyperuricemia and acute kidney injury (AKI) induced by mizoribine (MZR). Methods? The clinical data of a case of IgA nephropathy with hyperuricemia induced by oral administration of MZR was retrospectively reviewed. Results? A 13 -year-old boy with IgA nephropathy was diagnosed for 5 months. The basic renal function was normal. Transient hyperuricemia and acute renal injury were found after combined use of benazepril and MZR. The uric acid clearance rate (UACl) was significantly reduced to 3.66 mL·min-1 ·1.73 m-2 , the fractional excretion of sodium (FENa) was less than one ( 0. 4%), and the fractional excretion of uric acid (FEUA) was increased to 17 . 1%, highly suggestive of pre-renal AKI. After stopping the above drugs, he received hydration treatment, then the renal function recovered rapidly. Conclusion? Hyperuricemia induced by MZR can not only cause post-renal AKI, but also reduce renal perfusion. When combined with angiotensin converting enzyme inhibitor, the effect of MZR on uric acid metabolism and the adverse reactions should be found in time for further adjustment of the medication.

Clinical features and prognostic analysis of complicated urinary tract infection in children

SUN Jinshan, CHEN Chaoying

Journal of Clinical Pediatrics. 2021, 39(2):  102.  doi:10.3969/j.issn.1000-3606.2021.02.006

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Objective? To study the clinical features of complicated urinary tract infection (CUTI) in children, and to provide a reference for clinical and treatment of the disease. Methods? Clinical data of children with complex urinary tract infections from January 2015 to December 2019 were collected for retrospective analysis. Results? A total of 128 children were diagnosed, including 75 males and 53 females. Fever ( 111 cases, 86 . 7%) was the main manifestation of CUTI in infants, and vesicoureteral reflux ( 83 cases, 64 . 8%) was the most common. Compared with the group ≥ 3 years old, the proportion of vesicoureteral reflux was higher in the group < 3 years old, while the proportion of neurogenic bladder and renal insufficiency was lower, and the difference was statistically significant (P< 0 . 05 ). A total of 72 pathogenic bacteria were isolated, of which Escherichia coli was the first ( 17 strains, 23 . 6%). Drug sensitivity indicated that Escherichia coli was the most resistant to ampicillin ( 100%), but was more sensitive to meropenem, piperacillin/tazobactam and imipenem. Neurogenic bladder and vesicoureteral reflux were risk factors for urinary tract infection recurrence in children with CUTI (OR= 13 . 09 , 5 . 40 , P < 0 . 05 , respectively). The recurrence rate of urinary tract infection in the high-grade reflux group ( 76 . 6%) was higher than that in the low-grade reflux group ( 52 . 8%), and the recurrence rate in the low-grade reflux prophylactic antibiotics group ( 37 . 5%) was lower than that in the non-antibiotics group ( 83 . 3%), with statistically significant differences (P< 0 . 05 ). Thirty-five ( 35 ) cases of children with CUTI received surgical treatment to solve the urinary tract deformity, and the recurrence rate of urinary tract infection in children with CUTI after surgery ( 11 . 4%) was lower than that before surgery ( 37 . 1%), with statistically significant difference (P< 0 . 05 ). Conclusion? The clinical manifestations of CUTI in children are not typical, and fever with unknown cause is more common. Escherichia coli is the main pathogen. Vesicoureteral reflux and neurogenic bladder are high risk factors for recurrence. Prophylactic antibiotic therapy in children with low-grade vesicoureteral reflux and surgical treatment of urinary tract deformity significantly reduce the recurrence rate.

Diagnostic value of contrast-enhanced voiding urosonography in grading of vesicoureteral reflux

YANG Lefei, WU Wei, ZOU Xiangyu, et al

Journal of Clinical Pediatrics. 2021, 39(2):  108.  doi:10.3969/j.issn.1000-3606.2021.02.007

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Objective? To assess the accuracy of contrast-enhanced voiding urosonography (CeVUS) in diagnosing and grading of vesicoureteral reflux (VUR) and the consistency of the grading between the ultrasound observers. Methods? Thirtynine patients ( 79 Pyeloureteric units) examined by CeVUS and voiding cystourethrography (VCUG) were retrospectively analyzed. Based on the results of VCUG as the gold standard, the specificity, sensitivity and accuracy of grading diagnosis of CeVUS were calculated, and the consistency of different ultrasonic signs and grading evaluation of VUR between two ultrasound doctors was analyzed by Kappa test. Results? Compared with VCUG, the specificity and sensitivity of CeVUS in 79 PUUs was 90 . 6% and 89. 4%, respectively. The accuracy of grading of 42 pyeloureteral units with reflux was 90 . 5%, the accuracy of grade Ⅱ, Ⅲ, Ⅳ, V was 100%, 80 . 0%, 93 . 8% and 90 . 9%, respectively. The judgment of ureter development, ureter distortion, renal pelvis development, degree of renal pelvic dilatation, calyceal development, degree of calyceal dilatation, loss of renal pelvis contour between observers, the values of κ were 1 , 1 , 1 , 0 . 66 ( 0 . 52 - 0 . 80 ), 0 . 91 ( 0 . 82 - 1 . 00 ), 0 . 79 ( 0 . 70 - 0 . 89 ), and 0 . 69 ( 0 . 56 - 0 . 81 ) ( 95% confidence interval in brackets), respectively. The consistency of overall grading judgment was 0 . 87 ( 0 . 80 - 0 . 93 ). Conclusion? This study suggested that CeVUS with no ionizing radiation has a high accuracy in diagnosing and grading of VUR in children. It should be the first choice for the diagnosis and follow-up tool of VUR. When using CeVUS grading system for grading VUR, the overall consistency of the grading between the observers was excellent, which shows the feasibility of the CeVUS grading system even with different doctors.

Impact of spina bifida occulta in children on recurrence and treatment of monosymptomatic nocturnal enuresis

TAN Junjie, YU Li

Journal of Clinical Pediatrics. 2021, 39(2):  113.  doi:10.3969/j.issn.1000-3606.2021.02.008

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Objective? To investigate the effect of spina bifida occulta on the recurrence of monosymptomatic nocturnal enuresis in children and its treatment. Methods? A total of 186 children with monosymptomatic nocturnal enuresis from September 2018 to September 2019 were selected, of them 98 children with spina bifida occulta were selected and randomly grouped into a control group (desmopressin group, n= 41 ) and an observation group (desmopressin combined with oxybutynin group, n= 57 ), and all children were given dry bed training. The treatment effect and adverse effects of the children were observed after 6 months of treatment and 3 months of drug withdrawal. Results? After 6 months of treatment, the control group had a complete effect rate of 61. 0% and a total effective rate of 68. 3%; the observation group had a complete effect rate of 80. 7% and a total effective rate of 91.2%. The differences in the complete effect rate (χ2 =4.65, P=0.031) and the total effective rate (χ2 =8.37, P= 0 . 004 ) between the two groups were statistically significant. Three months after discontinuation of the drug, 11 ( 26 . 8%) of the 41 control children relapsed and 6 ( 10. 5%) of the 57 observed children relapsed, with a statistically significant difference between the two groups (χ2 = 4 . 42 , P= 0 . 036 ). Conclusion? Spina bifida occulta was found to be highly prevalent in children with monosymptomatic nocturnal enuresis and to be prone to recurrence of enuresis during treatment. The combination of desmopressin with oxybutynin for the treatment was effective on increasing the complete response rate and efficiency, decreasing the recurrence rate, and on providing good compliance and safety.

Allergic bronchopulmonary aspergillosis in childhood cystic fibrosis: a case report and literature review

WANG Fei, YANG Nan, CHEN Ning, et al

Journal of Clinical Pediatrics. 2021, 39(2):  117.  doi:10.3969/j.issn.1000-3606.2021.02.009

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Objective? To analyse the characteristics of allergic bronchopulmonary aspergillosis (ABPA) in children with cystic fibrosis (CF). Methods? The clinical data of a child with ABPA and CF was retrospectively analyzed and related literatures in database within 10 years were analyzed. Results? An 11 -year-old boy was diagnosed with asthma. He was admitted to the hospital because of “cough, wheezing”, and ABPA was confirmed. The genetic test identified mutation in CFTR gene and the concentration of chloride ions in sweat was 93 mmol/L. The final diagnosis was ABPA with CF. Seven cases of ABPA with CF were reported in detail in PubMed, CNKI, and Wanfang data. Five children born in European and American countries mostly were diagnosed CF by screening at early age as manifested repeated infections, respiratory symptoms, and pancreatic insufficiency. The clinical manifestation is varied among different gene mutations. Two cases of ABPA with CF reported in China were first diagnosed with ABPA, and CF was confirmed by gene examination and sweat chloride ion test. Conclusion? The disease process and clinical manifestations of CF complicated with ABPA in children are different related to the different mutations. The clinical manifestations of CF children in China are not typical and genetic testing should be conducted for children diagnosed with ABPA. Children with CF should be monitored for the presence of ABPA.

Juvenile xanthogranuloma presenting as a solitary tracheal mass in a child: a case report and literature review

CHEN Lili, LIN Lihua, ZHOU Yi, et al

Journal of Clinical Pediatrics. 2021, 39(2):  121.  doi:10.3969/j.issn.1000-3606.2021.02.010

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Objective? To investigate the clinical characteristics and treatment of juvenile xanthogranuloma ( JXG) presenting as a solitary tracheal mass in children. Methods? Clinical data of a patient admitted to the first affiliated hospital of Xiamen University with tracheal JXG in March 2019 was retrospectively analyzed, and related literatures were reviewed . Results? A 20-month-old girl, had a course of more than 2 months with repeated stridor and inspiratory dyspnea. She was treated with inhalation budesonide and alternating albuterol in addition to a systemic steroid, but relief was minimal. Evaluation of the subglottic area by bronchoscopy identified a light-yellow mass with a diameter of about 0 . 8 cm on the lateral wall of the trachea below the glottis which resulted in 60% of the trachea obstructed. The tumor was removed by laser under the microlaryngoscope, and locally treated with CO2 cryotherapy. Histopathological and immunohistochemical examination confirmed the diagnosis of JXG involvement of the trachea. Follow up for 2 months showed no recurrence. With the “juvenile xanthogranuloma, children” as keywords, the CNKI, Wangfang digital knowledge service platform and PubMed were searched for relevant articles from the establishment to August 2020 . Eight cases of children with JXG of trachea or larynx were reported. Including this case, all the 9 patients had recurrent wheezing as the main manifestation, and the conventional anti-infection and antiasthmatic treatment did not have a good effect. Some of the cases were surgically excised, some were conservatively treated after tracheotomy, waiting for the natural degeneration of the lesion, and most of them had a good prognosis. Conclusion? JXG of trachea or larynx is rarely seen in children. JXG can remit spontaneously in some cases, therefore surgical treatment was only required when severe airway obstruction occurs. Laser resection of tumor combined with CO2 cryosurgery can not only relieve airway obstruction, but also reduce the formation of local granulation tissue.

Clinical characteristics and genetic analysis of two 45, X / 46, X, + mar male children

WANG Xiaoxia, YU Fei

Journal of Clinical Pediatrics. 2021, 39(2):  125.  doi:10.3969/j.issn.1000-3606.2021.02.011

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Objective? To report clinical features and genetic analysis of two cases of 45, X/ 46, X, +mar male children. Methods? Two hospitalized children with short stature were analyzed. The medical history and clinical data were collected, and karyotype analysis of peripheral blood was performed. The second-generation sequencing of sexual development related genes was performed in case one, and the whole genome copy number variation sequencing was performed in case two. Relevant literature was reviewed. Results? Two male children, one was 10 years and 7 months old, and the other was 3 years and 1 month old. Physical examination showed short stature, and complicated with gonadal development delay. Case one also presented with varicocele and partial empty sella revealed by cranial MRI, the karyotype of peripheral blood was 45, X [ 31 ] / 46, X, +mar [ 69 ]. The secondgeneration sequencing showed that the copy number of SRY gene on the short arm of Y chromosome was repeated, and USP9Y gene at long arm was deleted. In case two, the karyotype of the peripheral blood was 45, X [ 5 ] / 46, X, +mar [ 75 ]. The whole genome copy number variation sequencing test showed that the karyotype of 46, XY, AZFb + AZFc region of Y chromosome was completely deleted. Conclusion? Children with short stature should be paid close attention to the morphology and function of their external genitalia. If necessary, karyotype analysis or genetic analysis should be performed to avoid misdiagnosis.

Genetic analysis of copy number variation in 90 children with developmental delay by CNV-seq

FENG Yu, YOU Shiqiong, LU Hongyong, et al

Journal of Clinical Pediatrics. 2021, 39(2):  129.  doi:10.3969/j.issn.1000-3606.2021.02.012

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Objective? To detect copy number variation sequencing (CNV) in children diagnosed with developmental delay (DD). Methods? Clinical data of children diagnosed with DD in Shanxi Provincial Children's Hospital (Shanxi Maternal and Child Health Hospital) from 2017 to 2019 were collected, and all patients had karyotype tested. CNV-seq was used to detect CNVs in these patients. ClinVar, DECIPHER, OMIM, DGV and other databases were used as references for data annotation, and the pathogenicity of CNV is classified according to ACMG guideline. Related articles were retrieved through the PubMed database. Results? Ninety patients were tested and the diagnostic rate was 20% ( 18/ 90 ). In which 15 patients were found have pathogenic CNVs,and three patients were found have likely pathogenic CNV. In addition, 32 patients carried copy number variation of unknown significance. Conclusion? This study confirmed that CNVs were an important cause of DD, and the CNVs > 1Mb in length are more likely pathogenic. In view of this, we recommend that CNVs testing be the first-line test in children with DD. CNV-Seq is a more efficient CNVs detection method based on high-throughput sequencing.

Genetic analysis of a family congenital generalized lipodystrophy

LI Yuanlong, LU Jun, CHEN Xiuling

Journal of Clinical Pediatrics. 2021, 39(2):  134.  doi:10.3969/j.issn.1000-3606.2021.02.013

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Objective? To explore the clinical characteristics and gene mutation characteristics of congenital generalized lipodystrophy (CGL). Methods? Clinical data and genetic results of a twin with CGL were retrospectively analyzed. Results? Both of the twins were male at the age of 4 months old, presented with similar features including the disappearance of adipose tissue throughout the body, hepatosplenomegaly, and a small amount of pigmentation all over the body. Laboratory examination revealed hypertriglyceridemia. DNA extracted from peripheral blood from the elder brother of the twin and from their parents was used for whole exome gene sequencing to identify mutations verified by Sanger sequencing. The results showed that the elder brother had a homozygous mutation of c. 974 dup (p.ile 326 hisfster 12 ) in the BSCL 2 gene, which was classified as pathogenic. Both parents carry a heterozygous mutation of c. 974 dup. Ten members (generation I, II, and III) of the family were tested for this mutation, and the younger brother of the twin was also found a homozygous mutation of c. 974 dup, and his grandmother and grandfather, uncle, and their sibling brother are all carriers of the mutation, which is consistent with the autosomal recessive inheritance. Conclusion? Two cases of identical twins with CGL 2 were reported as the first ones in China.

Meconium calprotectin level and its influencing factors in very low birth weight infants

XU Jinglin, TONG Xiaomei, WANG Ruiquan, et al.

Journal of Clinical Pediatrics. 2021, 39(2):  138.  doi:10.3969/j.issn.1000-3606.2021.02.014

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Objective? To explore the fecal calprotectin (FC) level in meconium and its influencing factors in very low birth weight infants. Methods? The first meconium after birth in very low birth weight infants hospitalized from June 2018 to May 2019 was collected. Immunofluorescence method was performed to detect quantitatively the FC levels in meconium, and general data during maternal pregnancy and infancy were collected. Results? A total of 87 very low birth weight infants ( 45 boys and 42 girls) were included. The median gestational age was 30 . 3 weeks ( 29 . 1 ~ 31 . 1 ), the median birth weight was 1300 g ( 1200 ~ 1450 ), and the median FC level in meconium was 165 . 1 ( 57 . 4 ~ 408 . 2 ) μg/g. The FC level in meconium in the group of pregnant women with preeclampsia and fetal distress was significantly higher than that in the group of pregnant women without preeclampsia (P 0 . 05 ). Conclusion? The meconium FC level in very low birth weight infants is high, and widely varies among individuals. Prenatal complications of preeclampsia and intrauterine distress may influence meconium FC levels in very low birth weight infants.

Clinical analysis of combined chemotherapy with cytarabine for refractory Langerhans cell histiocytosis

TANG Wenjing, YU Jie

Journal of Clinical Pediatrics. 2021, 39(2):  142.  doi:10.3969/j.issn.1000-3606.2021.02.015

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Objective? To evaluate the efficacy of combined chemotherapy with cytarabine (Ara-C) for children with refractory Langerhans cell histiocytosis (LCH). Methods The clinical data of patients with refractory LCH in Children's Hospital of Chongqing Medical University were retrospectively analyzed to evaluate the curative effect. Results A total of 11 patients with refractory LCH were included, with a male-to-female ratio of 6 to 5 , the median diagnostic age was 22 months ( 5 - 157 months), with median follow-up duration of 32 months ( 17 - 75 months). All of the patients were MS-LCH. After first-line treatment, 9 patients had disease progression ( 8 patients had skeletal progression), and 2 patients had stable disease. All patients were treated with combined Ara-C chemotherapy. The overall response rate was 91% ( 1 patient was cured, 9 patients were improved, and 1 patient was in a stable state), the overall survival rate was 100%. For patients with central nervous system (CNS) involvement (n= 10 ), 9 patients were accompanied by CNS mass at the first diagnosis, and 5 of the 9 patients had a complete regression of the CNS mass after the treatment. And two patients had diabetes insipidus as sequelae. Conclusion Ara-C combined with chemotherapy for refractory LCH had a high response rate, and it also had a better effect for patients with CNS involvement.

Retrospective analysis of 31 cases of multiple magnetic foreign bodies ingestion in children

ZHOU Fang, WANG Ruifeng, ZHOU Liang, et al

Journal of Clinical Pediatrics. 2021, 39(2):  147.  doi:10.3969/j.issn.1000-3606.2021.02.016

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Objective? To explore the clinical characteristics and prognosis of multiple magnetic foreign bodies in the gastrointestinal tract in children. Methods? Clinical data of 31 patients who swallowed multiple magnets and received endoscopic management and/or surgery in Henan Children’s Hospital from January 2014 to July 2020 were retrospectively analyzed. Results? The enrolled patients included 23 boys and 8 girls with the median age of 34 months. Patients yoanger than 3 years old accounted for 58.1% (n=18 ). The duration from ingestion to hospital visit ranged from 5 h to 60 d. The median number of magnets was 5 with a range from 2 to 24 . The common type of foreign bodies was Bucky ball magnet (n= 27 , 87 . 1%). There are 27 children suffered from multiple gastrointestinal perforations (range: 2 - 8 ), and 4 children (n = 4 , 12 . 9%) had no digestive duct injury. The majority of perforation was located at the small intestine ( 17/ 27 , 63 . 0%), including simple small intestinal perforation ( 12/ 27 , 44 . 4%), followed by gastric perforation and esophagus-gastric perforation ( 5/ 27 , 18 . 5%). The most common presenting signs and symptoms in children with perforation were abdominal pain (n= 14 , 51 . 9%) , vomiting (n= 11 , 40 . 7%), fever (n = 5 , 18 . 5%), and bloating (n= 4 , 14 . 8%). However, six patients ( 6/ 27 , 22 . 2%) had no clinical symptoms. Endoscopic foredgn body extraction was successful in 32.3% (n=10) of cases; while surgery was required in 21 patients (67.7%), including 9 cases failed endoscopic treatment. All patients with gastrointestinal perforation were fully recovered, including 6 cases ( 6/ 27 , 22 . 2%) treated with indwelling gastrointestinal decompression tube and enteral nutrition via nasojejunal tube, another 21 cases ( 21/ 27 , 77 . 8%) underwent surgical repair. One patient had intestinal obstruction and underwent a second operation, and the remaining patients’ courses were uneventful during the follow-up period. Conclusion The ingestion of multiple magnets is harmful for children’s health because of its severe digestive tract injury, such as perforation. Early diagnosis and treatment are important. The combination of endoscopy and surgery is necessary. If magnetic foreign bodies cannot be removed by endoscopy, surgical intervention is suggested as soon as possible to avoid serious complications.

Progress in research on neuroprotective effect of sirtuin 3 in regulating microglia against brain injury in premature infants

WEI Jia

Journal of Clinical Pediatrics. 2021, 39(2):  152.  doi:10.3969/j.issn.1000-3606.2021.02.017

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With the development of perinatal medicine, the survival rate of preterm infants has been increasing year by year, but the incidence of brain injury is still high. Although neurorehabilitation can improve brain injury to a certain extent, neurological dysfunction of varied degrees remains inevitably. Recent studies have shown that sirtuin 3 can regulate microglia in the brain to reduce neuronal damage, and has great potential in protecting premature infants from brain injury. This article reviews the neuroprotective mechanism of sirtuin 3 through microglia on brain injury in premature infants, and to provide a theoretical basis for treatment.

Progress in diagnosis and treatment of hereditary renal tubular alkalosis in children

HAN Tingting, WANG Wenhong

Journal of Clinical Pediatrics. 2021, 39(2):  157.  doi:10.3969/j.issn.1000-3606.2021.02.018

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? Hereditary renal tubular alkalosis in children is a rare renal tubular disease. It is a clinical syndrome caused by protein dysfunction resulted from single gene mutation of ion channel in renal tubular epithelial cells. Bartter syndrome, Gitelman's syndrome and Liddle's syndrome are among the more common types, all of which are hereditary potassium loss diseases clinically characterized by renal tubular alkalosis. In this paper, the pathogenesis, clinical manifestations and the latest progress in diagnosis and treatment were reviewed.