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06 July 2024, Volume 39 Issue 7 Previous Issue   

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Attention should be paid to the standardized diagnosis and treatment of gastrointestinal bleeding in children YANG Hong-bin, FANG Ying 2024, 39(7): 481-484.  DOI: 10.19538/j.ek2024070601 Abstract ( )   Gastrointestinal bleeding is a common symptom of pediatric digestive system，but it is difficult to diagnose and treat with different bleeding sites，different onset ages，different manifestations and different etiologies.There is a shortage of pediatricians in digestive endoscopy in China，the development of children's digestive endoscopy technology is different in various regions，and the popularity rate of gastroenteroscopy and treatment is not high，so many children with gastrointestinal bleeding can not be checked in time to determine the cause，which may delay the time for treatment.It is necessary to pay attention to the normalization of diagnosis and treatment of gastrointestinal bleeding in children，improve the level of diagnosis and treatment，improve the prognosis of the disease，and reduce the medical burden.

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Identification of pseudo-gastrointestinal bleeding in children YAN Wei-hui, WANG Ying 2024, 39(7): 485-488.  DOI: 10.19538/j.ek2024070602 Abstract ( )   Distinguishing true and false gastrointestinal bleeding is an essential skill that every pediatrician needs to master.The main causes of pseudo-gastrointestinal bleeding are bleeding from the mouth，nose，throat，respiratory tract，and exogenous food or drug interference. In most cases，it is easier to distinguish true from false gastrointestinal bleeding by history inquiry，physical examination，and fecal occult blood test， avoiding unnecessary invasive examination and surgery as much as possible. However，there are some misdiagnoses and emergencies in clinical practice.If bleeding from the mouth，nose，pharynx，respiratory tract and digestive tract is ruled out after adequate clinical evaluation，the possibility of malingery or factitious disorders should be considered.

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Endoscopic diagnosis and treatment of gastroesophageal varices in children YU Fei-hong, WU Jie 2024, 39(7): 489-492.  DOI: 10.19538/j.ek2024070603 Abstract ( )   Gastroesophageal varices and variceal bleeding are one of the gastrointestinal emergencies.Gastroscopy is the golden standard for screening esophageal and gastric varices and assessing the risk of bleeding.Endoscopic treatment includes endoscopic injection sclerotherapy，endoscopic variceal ligation and endoscopic glue injection therapy. The article introduces endoscopic diagnosis and treatment of gastroesophageal varices in children to provide clinical reference.

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Endoscopic diagnosis and treatment of lower gastrointestinal bleeding in children  ZHAO Hong-mei, LI Can-lin 2024, 39(7): 492-497.  DOI: 10.19538/j.ek2024070604 Abstract ( )   Lower gastrointestinal bleeding typically refers to bleeding from the intestines below the Trietz ligament，which includes small intestine bleeding and colon bleeding. It is relatively common in children， and although there is relatively less research on lower gastrointestinal bleeding compared to upper gastrointestinal bleeding，and there are few guiding documents and consensus.With the rapid development of pediatric endoscopy technology in recent years，especially small bowel endoscopy，capsule endoscopy，and various endoscopic treatment methods，most children with lower gastrointestinal bleeding can be effectively diagnosed and treated.However，there are still a few cases that are difficult to diagnose and treat clinically.This article summarizes the clinical evaluation，diagnosis，and treatment methods of pediatric lower gastrointestinal bleeding，aiming to standardize the diagnosis and treatment process of pediatric lower gastrointestinal bleeding.

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Perioperative management of gastrointestinal bleeding in children QI Ling-li, HUANG Xin-xing, ZHONG Yuan, et al 2024, 39(7): 497-502.  DOI: 10.19538/j.ek2024070605 Abstract ( )   Gastrointestinal bleeding is a common disease in children，which can be divided into upper gastrointestinal bleeding and lower gastrointestinal bleeding according to the location of bleeding. Digestive endoscopy is an important approach to diagnosing the cause of gastrointestinal bleeding.The earlier the endoscopy is conducted，the higher the diagnostic rateis. Therefore，when the hemodynamics is stable，digestive endoscopy should be performed as soon as possible. When hemodynamics is unstable，fluid resuscitation should be activated and restrictive transfusion strategy should be adopted. Adequate bowel preparation is important for the quality of colonoscopy，enteroscopy，and capsule endoscopy.In this review，we summarize the perioperative management of gastrointestinal bleeding in children，and focuses on bowel preparation.

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Multidisciplinary diagnosis and treatment strategy for acute and critical gastrointestinal bleeding in children REN Xiao-xia, FANG Ying 2024, 39(7): 502-506.  DOI: 10.19538/j.ek2024070606 Abstract ( )   Gastrointestinal bleeding is a common disease in children with complex etiology and different etiology at different ages.It may be the digestive tract manifestation of systemic diseases or local lesions of the digestive tract. Most  gastrointestinal bleeding can be alleviated or cured by internal medical treatment，but clinical acute gastrointestinal bleeding is life-threatening，often requiring the multidisci-plinary diagnosis and treatment of emergency department，gastroenterology department，interventional vascular department，ICU，general surgery department and others，and effective diagnostic technology and treatment means are needed to save the lives of children.

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Nursing care for gastrointestinal bleeding in children FENG Yuan, SHI Yu-run, YAO Jia-meng, et al 2024, 39(7): 506-508.  DOI: 10.19538/j.ek2024070607 Abstract ( )   The incidence of gastrointestinal bleeding，one of the most common critical conditions in children's digestive diseases，has gradually increased in recent years，seriously affecting children's life and health.This paper summarizes the nursing points of gastrointestinal bleeding in children by sorting out the research status in the nursing for children with gastrointestinal bleeding at home and abroad，aiming to alleviate the clinical symptoms and shorten the hospitalization time of children with gastrointestinal bleeding and to provide guidance for clinical nursing and improve the quality of nursing for children with gastrointestinal bleeding.

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Value of multiple polymerase chain reaction test in cerebrospinal fluid for pathogenic diagnosis of community-acquired bacterial meningitis：a meta-analysis LI Yi-xuan , GUO Ling-yun, GAO Cheng-feng, et al 2024, 39(7): 509-517.  DOI: 10.19538/j.ek2024070608 Abstract ( )   Objective    To systematically evaluate the value of multiple polymerase chain reaction（mPCR）in cerebrospinal fluid for pathogenic diagnosis of community-acquired bacterial meningitis（CABM）. Methods    Wanfang，Sinomed，Pubmed，Cochrane Library and Embase databases were searched from the establishment of the database to September 2021 according to the retrieval strategy. Two researchers conducted literature screening，quality assessment and data extraction independently. Meta-analysis was performed using Stata11.0 to calculate the combined sensitivity，specificity，positive likelihood ratio，negative likelihood ratio and diagnostic odds ratio. The subject receiver operating characteristic（SROC）curve was drawn and the area under the curve（AUC）was also calculated.Subgroup analysis was performed according to study type，pathogen species and affected population. Sensitivity analysis was performed by one-by-one exclusion method，and publication bias was evaluated by Deek's funnel plot method.Results    A total of 279 articles were retrieved，and 4679 cases of 19 articles were finally included according to exclusion and inclusion criteria. Meta-analysis showed that mPCR in CSF could increase the positive rate of bacterial culture pathogens by 0.89%～49.51%. The combined sensitivity and specificity of mPCR in CSF were 0.91［95% CI 0.75-0.97］ and 0.88［95%CI 0.78-0.94］，and the positive and negative likelihood ratios were 7.7［95% CI 3.9-15.2］ and 0.11［95% CI 0.04-0.31］，respectively. The diagnostic odds ratio was 72 ［95% CI 18-295］and the AUC was 0.95［95% CI 0.93-0.97］. Subgroup analysis showed that mPCR detection had high diagnostic efficiency for three common pathogens of CABM（Streptococcus pneumoniae，Haemophilus and Meningococcal）and children. Sensitivity analysis showed that the results of this study were stable and credible. Conclusion    The mPCR test in cerebrospinal fluid has high diagnostic efficiency in the diagnosis of CABM，and can be used as an auxiliary method for the pathogenic diagnosis of CABM.

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Clinical characteristics of epilepsy after herpes simplex virus encephalitis in children：an analysis of 14 cases YAN Shuang, WU Miao-juan, SUN Dan 2024, 39(7): 518-523.  DOI: 10.19538/j.ek2024070609 Abstract ( )   Objective    To summarize the clinical features of epilepsy after herpes simplex virus encephalitis （HSE） in children.Methods    The data of 14 children with herpes simplex encephalitis diagnosed in the Department of Neurology，Wuhan Children′s Hospital，Tongji Medical College，Huazhong University of Science&Technology from March 2017 to June 2022 were retrospectively collected，and their clinical manifestations，cerebrospinal fluid examination，EEG and imaging were analyzed and summarized.After that，the patients were followed up for 10 months to 5 years to evaluate whether they had postencephalitic epilepsy（PEE） ，then they were divided into post encephalitis epilepsy group and non epilepsy group，to compare the clinical data include cerebrospinal fluid，VEEG and imaging parameters in the acute phase of encephalitis. Results    Seven of the 14 children were diagnosed with postencephalitic epilepsy including 5 males and 2 females with an onset age of 4-110 months（media 11 months）months.All the children were treated with two or more antiepileptic drugs，and 3 of them had refractory epilepsy. All patients had residual sequelae such as cognitive and motor disorders, and there was no statistically significant difference between the PEE and non PEE groups after HSE. Conclusion    There is a high possibility of secondary epilepsy after herps simplex virus encephalitis， with some developing into refractory epilepsy. Acute phase seizures or status epilepticus may be high-risk factors for postencephalitic epilepsy.

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Baseline nutritional status of children on ketogenic diet and the awareness of ketogenic dietary therapies in the primary caregivers GONG Xiao-yan, LU Zhong-ying, ZHANG Yun-jian, et al 2024, 39(7): 524-529.  DOI: 10.19538/j.ek2024070610 Abstract ( )   Objective    To investigate the baseline nutritional status of children prior to commencing of ketogenic diet therapies（KDTs）and the level of awareness regarding KDTs among primary caregivers，providing a foundation for subsequent effective treatment and exploration of long-term management strategies. Methods    A prospective cohort study design was adopted.The study included children who underwent KDTs at the Department of Neurology，Children's Hospital of Fudan University from July 2021 to January 2024 and their primary caregivers. The children underwent nutritional screening and assessment.A questionnaire survey was administered to the primary caregivers to assess the daily dietary behaviors of the children and their caregivers’ awareness of KDTs in the early stages. Results    A total of 104 children were enrolled in this study，achieving a questionnaire recovery rate of 100%.All children exhibited nutritional risks. Regarding daily dietary behaviors，picky eating and partial eating problems were observed in 55.3% of the children.During initial stages of KDTs，specialist visits at the Department of Neurology accounted for knowledge acquisition by 89.4% of primary caregivers；WeChat/internet/books or patient groups contributed 25.0% and 10.6%，respectively. Regarding the implementation of KDTs，a significant majority（72.1%）of primary caregivers expressed concerns regarding their children's potential challenges in accepting such a specialized dietary regimen.Moreover，28.8% expressed concerns regarding their lack of ability in managing the clinical implementation process，while 22.1% had concerns about accurately comprehending the teachings provided by the KDTs team. In terms of expected therapeutic outcomes，all primary caregivers anticipated effective seizure control for their children，with an additional 79.8% hoping for improvements in cognitive behavior and 77.9% expecting a reduction or cessation of all antiepileptic medications simultaneously. Conclusion    Children initiating KDTs are at a moderate to high nutritional risk，with some experiencing issues related to undernutrition or overnutrition.The proportion of unbalanced dietary intake is high. The primary caregivers have  a high expectation for curative effect. The KDTs team should pay attention to the nutritional status of children throughout the routine diagnostic and therapeutic process，while intensifying the dissemination and education on ketogenic diet therapy to ensure its safe and efficacious implementation.

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LIG4 syndrome caused by gene mutation： the clinical and genetic characteristics LIU Hong-hong, WANG Yi-zhong, XIAO Yong-mei, et al 2024, 39(7): 530-538.  DOI: 10.19538/j.ek2024070611 Abstract ( )   Objective    To explore the clinical and genetic characteristics of LIG4 syndrome. Methods    We retrospectively analyzed the clinical data of 3 patients with LIG4 syndrome who were admitted to ShangHai Children’S Hospital from Jun 2014 to Aug 2018，and reviewed related articles. Results    All 3 cases were female，presented with recurrent chronic diarrhea or recurrent respiratory infections.The clinical manifestations were microcephaly，a special“bird-head-like”face，growth retardation，and repeated infections.The blood routine showed that the lymphocyte count was significantly reduced，the immune function showed that the counts of T and B lymphocytes were decreased，and immunodeficiency disease was considered clinically. The gene detection revealed that P1 had homozygous mutation of LIG4 gene，c.833G>T（p.R278L），with severe clinical phenotype；P2 had LIG4 gene complex heterozygous mutations，c.883G>T（p.R278L）and c.1144-1145delCT（p.L382EfsX5）；P3 had LIG4 gene complex heterozygous mutations，c.28-29delGT（p.vall0fs）and c.1341G>T（p.Trp447cys），where the frameshift gene mutation site，c.28-29delGT（p.vall0fs），had not been reported.P1 died of severe infection and multiple organ dysfunction syndrome. P2 had preventive anti-infection treatment and received regular human immunoglobulin infusion，who still had recurrent infection and lost follow-up.P3 underwent peripheral blood hematopoietic stem cell transplantation，and established normal immune function.She did not have recurrent infection during 2-year follow-up period. A total of 46 cases of LIG4 syndrome had been published worldwide，including 13 cases and 1 family report of LIG4 syndrome in China. Conclusion    Immunodeficiency diseases should be considered in the patients with chronic multiple infections accompanied by T and B lymphocytes decrease，growth retardation，and microcephaly.Gene testing helps confirm the diagnosis and prenatal screening.Hematopoietic stem cell transplantation is effective for immune reconstitution.

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Analysis of short-term scientific weight loss effect and persistence in overweight and obese children WANG Hai-hong, YAN Jie, XIA Lu-lu, et al 2024, 39(7): 539-543.  DOI: 10.19538/j.ek2024070612 Abstract ( )   Objective    To explore the effect of short-term scientific weight loss on overweight and obese children and the factors affecting sustainability. Methods    The subjects were 30 overweight and obese children who participated in weight-loss summer camp organized by the Department of Clinical Nutrition，Beijing Children's Hospital affiliated to the Capital Medical University from July to August in 2023，the data and information were collected by questionnaire survey，camp-based index monitoring and follow-up survey before，during and two weeks after the camp. Results    After short-term intervention，the weight，body fat and waist circumference of all the children were decreased. About 29 cases（96.7%） of the parents and their children had new knowledge about healthy diet.About 27 cases（90.0%） of the children could keep the weight loss，only 3 cases（10%）gained more than 1.5 kg. About 16 cases（53.3%） of the children did not gain weight or further loss，continued to lose more than 1.5 kg in 9 cases（30.0%）.There were 14 cases（46.7%）who gained weight after the camp.The results of multivariate  analysis showed that not adhering to healthy eating habits after the camp was an important influencing factor（P<0.001）. Conclusion    The short-term scientific comprehensive intervention of diet and exercise has a good effect on weight loss in overweight and obese children，in addition to strengthening health education to promote awareness of healthy life，we should also pay attention to the late stage of habit formation supervision and guidance.

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Diagnostic value of liver biopsy in children with inherited metabolic liver disease FAN Yan-lei, LI Zhong-yue 2024, 39(7): 544-548.  DOI: 10.19538/j.ek2024070613 Abstract ( )   Inherited metabolic liver disease is a kind of common liver disease in children.Although the prevalence rate of single disease is low，the overall incidence is high.The diagnosis relies on multiple clinical data，including clinical symptoms and signs，laboratory examination，imaging，liver biopsy and gene detection. Liver biopsy is an invasive procedure，and its diagnostic value is not consistent in different genetic metabolic liver diseases.Not all liver diseases need liver biopsy，and genetic metabolic liver diseases do not necessarily have their characteristic pathological manifestations.Therefore，clinicians should master the indications strictly to maximize the clinical value of liver biopsy.

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A case of MYO5B gene mutation manifested as jaundice and pruritus and literature review CUI Xiao-shuang, WANG Guo-li, ZHANG Jing, et al 2024, 39(7): 549-554.  DOI: 10.19538/j.ek2024070614 Abstract ( )   Retrospective analysis of a case with jaundice and pruritus caused by MYO5B gene mutation admitted to Beijing Children's Hospital in March 2021，To investigate the cholestasis phenotype of MYO5B gene mutation，Summarize clinical characteristics and treatment by literature review. Patient（girl）10-month-old presented with pruritus，jaundice，pale stool and liver swelling，without gastrointestinal symptom. Total and direct serum bilirubin elevated，normal gamma-glutamyltransferase（GGT）activity，a increase of transaminases，elevated total serum bile acid.Liver biopsies：swelling of liver cells，mild dilation of liver sinuses，mild perisinusoidal fibrosis，intracellular cholestasis，Biliary thrombus in bile duct，focal lymphocyte infiltration. MYO5B compound heterozygous mutation：c.2254C>T（p.Q752X），c.1618A>C（p.K540Q）.Ursodeoxycholic acid and Cholestyramine were ineffective，Long term follow-up showed malnutrition and no signs of diarrhea. For patients with low GGT cholestatic symptoms developing in late infancy，attention should be paid to the MYO5B gene mutation，which can manifest as simple cholestasis without obvious gastrointestinal symptoms.If oral medications are poor effect，biliary diversion or liver transplantation should be considered.

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Clinical manifestations and gene mutation analysis of a patient with giant axonal neuropathy and literature review WANG Li, DUAN Li-fen, BIAN Cheng, et al 2024, 39(7): 555-560.  DOI: 10.19538/j.ek2024070615 Abstract ( )   The analysis revealed the clinical phenotypic and genetic characteristics of a patient with Giant axonal neuropathy，combined with the clinical phenotype and gene mutation characteristics of GAN patients reported in China，we provided a reference for the diagnosis of GAN and genetic counseling.Retrospectively analyzed the clinical data and other auxiliary inspection materials of a GAN patient with chromosomal 16q23.2 partial deletions，and gene detection is performed on the child and his parents.The clinical data and pathogenic mutations of GAN patients in China were summarized in this study based on related databases.The proband is a seven years and six months old boy with duck walking，and a history of seizures.Gene detection shown that the chromosome 16q23.2 region was partially deleted，Combined with clinical manifestations，the child was diagnosed with GAN.The summary of 12 Chinese GAN patients' clinical manifestations revealed that the average onset age is three years old，and all of them showed symptoms with unexpected lower limbs，gait abnormalities，and walking obstacles in their early childhood.Our patient is deleted by a gene fragment，and other are point mutation.The location of the mutation is widely distributed in three domains of GAN genes. Partial deletion of GAN gene leads to Giant axonal neuropathy in this case.This study further complements the genetic mutant and summarizes the clinical phenotypes for GAN patients in China，and provide a reference for GAN clinical diagnosis and genetic counseling.