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06 May 2024, Volume 39 Issue 5 Previous Issue   

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Attention to necrotizing enterocolitis in children SHENG Qing-feng, LU Li, LYU Zhi-bao 2024, 39(5): 321-324.  DOI: 10.19538/j.ek2024050601 Abstract ( )   Necrotizing enterocolitis （NEC） remains one of the leading causes of death of the newborn，especially in the preterm and low birth weight neonates. How to identify and accurately determine the severity of the disease early，how to grasp the surgical opportunity of children with NEC，and explore effective intervention measures have become the hot topic of current clinical research. New discoveries in the basic research including intestinal mucosal barrier and inappropriate inflammatory response. Current work will shed light on explaining the pathogenesis of NEC，potential therapeutic methods，and ultimately improve outcomes in NEC.

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Nutritional management after surgery for neonatal necrotizing enterocolitis LAI Deng-ming, JIN Jing-yi, TOU Jin-fa 2024, 39(5): 325-330.  DOI: 10.19538/j.ek2024050602 Abstract ( )   Neonatal necrotizing enterocolitis (NEC) is the most common intestinal disease leading to neonatal mortality，with 20% -50% of patients requiring surgical intervention. Short bowel syndrome is one of the most serious complications after NEC surgery. Nutritional management affects postoperative recovery. Despite the development of pediatric nutritional skills，nutrition -related complications such as electrolyte disturbances，nutrient imbalances，and parenteral nutrition associated liver disease still exist. This article aims to describe the aspects of postoperative nutritional assessment，nutrition modality selection and complication prevention of NEC postoperative nutrition management，and then provide theoretical support for nutritional treatment and bowel function rehabilitation of NEC.

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Early diagnosis and surgical timing of necrotizing enterocolitis LI Wei, TANG Wei-bing 2024, 39(5): 330-334.  DOI: 10.19538/j.ek2024050603 Abstract ( )   Necrotizing enterocolitis （NEC） is a necrotizing inflammation of the intestine unique to the neonatal period，primarily affecting premature infants with a high fatality rate. Due to its inconspicuous initial clinical symptoms，early diagnosis can be challenging. In addition to relying on clinical manifestations and traditional diagnostic methods such as laboratory tests and imaging examinations，future research will focus on enhancing the accuracy of NEC's early diagnosis by integrating multiple biological markers. The clinicians should perform surgical intervention as soon as possible before perforation takes place or the condition worsens by identifying closely associated indicators in order to reduce mortality in children with NEC.

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Current status of surgical treatment for neonatal necrotizing enterocolitis DU Jing-bin, HUANG Jin-shi 2024, 39(5): 334-338.  DOI: 10.19538/j.ek2024050604 Abstract ( )   Necrotizing enterocolitis (NEC) is a common disease in the newborn period. In the past，the traditional cognition believed that surgical intervention was only needed when abdominal and intestinal perforation occured. However，with the continuous understanding of the pathological course of necrotizing enterocolitis，people began to look for early surgical intervention methods. At present，the relative indications for requiring surgical intervention are mainly based on the parameters of clinical manifestations，laboratory examination and radiological examination. Once the clinical condition of the child is found to deteriorate，surgical intervention is required. The surgical management of NEC includes the following types. The abdominal placement of abdominal drainage tube: the simple placement of abdominal drainage can be used as the final choice for some children with poor general conditions who can’t tolerate surgery or with unstable vital signs in the rescue. Open exploration: intestinal stomy or anastomosis can be done after necrotic bowel resection，especially in children with NEC with localized lesions，who can have intestinal anastomosis. In severe，multifocal NEC，due to the more diseased bowel tubes， jejunostomy with high position or "clip and drop back " technology is used to improve the survival rate of children；Use of laparoscopy in NEC: few cases are reported at home and abroad. This technique exacerbates intraabdominal CO2 pressure in children with emergencies，increases the risk of anesthesia and internal environmental deterioration in the children. There are no additional data to support the widespread use of this technology in children with NEC.

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Status quo and dilemma in the diagnosis and treatment of necrotizing enterocolitis totalis CAO Xu-qing, SHEN Chun 2024, 39(5): 338-341.  DOI: 10.19538/j.ek2024050605 Abstract ( )   Necrotizing enterocolitis totalis （NEC-T） is one of the most severe forms of necrotizing enterocolitis，with rapid progression，a high risk of poor prognosis，and a lack of universally accepted treatment strategies. This article summarized the risk factors，treatment decision，prognosis and possible ethical issues in the management of NEC-T，and presented some difficulties and dilemma in the course of clinical diagnosis and treatment.

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Progress in the diagnosis and treatment of intestinal stenosis after neonatal necrotizing enterocolitis LIU Yuan-mei, TANG Cheng-yan 2024, 39(5): 342-345.  DOI: 10.19538/j.ek2024050606 Abstract ( )   Necrotizing enterocolitis （NEC） in newborns is a common acute abdominal disease characterized by diffuse necrosis of the local intestinal mucosa or wall，mainly affecting the terminal ileum or colon. In recent years，with the improvement in early diagnosis and treatment of NEC，the mortality rate of NEC has been decreasing year by year，but the incidence of complications after treatment has increased. Intestinal stenosis is one of the most common complications after NEC. Due to the lack of specificity in clinical symptoms，it is easy to be missed or misdiagnosed，leading to serious consequences such as growth and development delay，intestinal perforation，and sepsis. This article will review the clinical characteristics，diagnosis，treatment，and prognosis of intestinal stenosis after NEC，aiming to provide reference for the early diagnosis and treatment of intestinal stenosis after NEC.

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Research progress on surgical intervention of necrotizing enterocolitis in premature and low birth weight infants REN Hong-xia, WU Ying 2024, 39(5): 346-350.  DOI: 10.19538/j.ek2024050607 Abstract ( )   Necrotizing enterocolitis（NEC） is a common gastrointestinal disease that occurs mostly in premature infants and low birth weight infants. It is manifested as diffuse or limited necrosis of intestinal mucosa，muscle layer or even all intestinal wall in the small intestine and colon. It is also one of the main causes of death in premature infants and low birth weight infants. With the progress of medicine，especially the development of perinatal medicine and intensive care medicine，the survival rate of premature infants and low birth weight infants has been greatly increased，and the corresponding incidence of NEC in premature and low birth weight infants has also increased. If NEC cannot be controlled by conservative treatment，timely surgical intervention is required. The surgical indication and treatment timing of neonatal necrotizing enterocolitis are controversial，more so for premature and low birth weight infants. This review focuses on the clinical features，surgical indications and operation methods of premature and low birth weight infants with NEC requiring surgical intervention.

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Clinical reflection on the surgical indications of NEC LI Tian-yu, ZHANG Zhi-bo 2024, 39(5): 350-355.  DOI: 10.19538/j.ek2024050608 Abstract ( )   Necrotizing enterocolitis （NEC） is an inflammatory necrotizing disease of the intestinal tract involving the ileum and colon，which mainly occurs in low birth weight preterm infants. NEC often progresses rapidly and remains one of the most common diseases that seriously threaten the lives of preterm babies. Generally，NEC is first treated medically and conservatively，including intestinal rest application of antibiotics，and management of complications，etc. However，some cases still progress rapidly despite the most aggressive medical treatment and eventually need to be operated on. The ideal surgical opportunity，indications，and methods are still inconclusive，which will be further discussed in this paper on the basis of our clinical experience，with the purpose of improving the survival of NEC infants.

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A review of surgical treatment strategies and prognosis of neonatal necrotizing enterocolitis ZHONG Wei, XIE Xiao-li, HE Qiu-ming 2024, 39(5): 355-358.  DOI: 10.19538/j.ek2024050609 Abstract ( )   Necrotizing enterocolitis （NEC），an acquired inflammatory bowel disease， almost exclusively affects the newborns，which is one of the leading causes of neonatal death，with nearly half of NEC patients requiring surgical treatment. At present，there are various surgical options for the acute stage of NEC，but there is no consistents recommendation standard. Surgeons often make choices based on the patient's specific circumstances and individual preferences. In this paper，we will review the common surgical strategies for NEC currently used.

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Analysis of clinical features，gene mutations and prognostic characteristics of 19 children with primary distal renal tubular acidosis WANG Qiao, CAO Bing-yan, ZHAO Yun, et al 2024, 39(5): 359-366.  DOI: 10.19538/j.ek2024050610 Abstract ( )   To investigate the clinical charateristics and treatment outcome of children with primary distal tubular acidosis （dRTA），analyze the difference among children with different gene phenotypes，and to improve the understanding of primary dRTA in children. Methods    From April 2011 to November 2022，19 patients diagnosed with primary dRTA were included. Clinical and laboratory characteristics were summarized and compared among genotype. Outcome factors were also analyzed. Results    There were 19 patients （8 males and 11 females）. The age of onset ranged from 10 days to 4 years. The last follow-up age was 8 months to 17 years and one month. Growth retardation was the most common clinical symptom. Height or weight below - 2SD accounted for 78.94%. All patients had hypokalemia，hyperchloremic metabolic acidosis，alkaline urine，increased urine calcium and renal medullary calcinosis. Twenty mutations of SCL4A1，ATP6V0A4 and ATP6V1B1 gene were found in the 19 patients，and 9 of them were novel mutations. The proportion of patients with SLC4A1 recessive inheritance and SLC4A1 dominant inheritance，ATP6V0A4 and ATP6VB1 inheritance was 31.58%，15.79%，36.84% and 15.79%，respectively. The onset age of patients with SLC4A1 mutations was significantly higher than patients with ATP6V0A4 and ATP6VB1 mutations （P<0.001）. Bicarbonate level of SLC4A1 recessive inherited patients was significantly lower than others （P=0.01）. Combination rate of bone abnormalities：in SLC4A1 dominant inherited patients it was 66.67%，in SLC4A1 recessive inherited patients it was 100%，in ATP6V1B1 group in was 33.33%，and in ATP6V0A4 group it was 0；there was statistical difference among the four groups （P<0.001）. Three patients had sensorineural hearing loss （1 of ATP6V0A4，2 of ATP6V1B1）. The proportion of patients with ATP6V0A4 mutations combined with sensorineural hearing loss was significantly lower than that reported abroad. After treatment with citric acid preparation，68.42% of the patients had a height and weight of more than - 2SD，which were significantly positively correlated with the time of treatment （related coefficients 0.627），but had no significant relationship with genotype. After treatment， blood potassium remained normal，and 71.43% of patients' blood bicarbonate remained normal. Only one patient had eGFR lower than 90ml/min/1.73m2 after 8 years of treatment. There was no significant genotypic difference in treatment outcome. In addition，the allele frequency of SLC4A1 c.2102G>A （p.G701D） was 83.33%，which was a hot spot mutation in SLC4A1 recessive patients. All patients carrying this mutation in this study came from southern China. ATP6V0A4 c.580C>T （p.R194X） mutation gene frequency was 28.57%，which was considered to be a hot mutation in Chinese population，and all patients with this mutation came from North China. Conclusion    Different genotypes of primary dRTA have different phenotypes. Patients with SLC4A1 mutation have a late onset age and more bone involvement. Patients with SLC4A1 recessive mutations often suffer from severe acidosis. After appropriate treatment，the condition of patients with all genotypes improved，and prognosis in childhood is good without end point. This study reveals 9 new mutations，which have enriched the human gene mutation database. The ATP6V0A4 c.580C>T （p.R194X） mutation and SLC4A1 c.2102G>A （p.G701D） are consistent with the hot spot mutation of Chinese population，and there are hot spot regional differences. There is no end event under the current follow-up age and current treatment.

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Clinical and genetic characteristics of 22 cases of non - 21 OHD congenital adrenal hyperplasia HE Qin, ZHANG Li, HUANG Ke, et al 2024, 39(5): 367-374.  DOI: 10.19538/j.ek2024050611 Abstract ( )   Objective    To analyze the clinical features and molecular genetic changes of congenital adrenal hyperplasia（CAH） with non-21 -OHD，and to investigate the correlation between clinical phenotype and genotype of different types of CAH. Methods    A total of 60 patients diagnosed with congenital adrenal hyperplasia were collected from Endocrinology Department of Children′s Hospital of Zhejiang University School of Medicine from July 2017 to April 2022. There were 22 cases of CAH with non 21-OHD. Clinical manifestation，laboratory examination and gene testing results were retrospectively analyzed. Results    Among the 60 CAH patients diagnosed by clinical manifestations and genetic diagnosis，22 were non-21-OHD patients，of whom 11 （11/22） were girls and 11 （11/22） were boys，aged from 19 days to 14.5 years. There were 8 cases of 11β-hydroxylase deficiency （11β-OHD） （8/22），7 cases of 17α-hydroxylase deficiency （17α-OHD） （7/22），5 cases of congenital lipoid adrenal hyperplasia （CLAH）（5/22），and 1 case of P450 oxidoreductase deficiency （PORD）（1/22）；1 case Of 3β -hydroxysteroid dehydrogenase type 2 deficiency （3βHSD2D）（1/22）. External genital dysplasia and pigmentation were found in all 5 types of diseases，and the age of diagnosis in the 17α-OHD group was the latest. Among the 5 related genes （CYP11B1，CYP17A1，StAR，POR，HSD3B2） found in 22 patients，a total of 28 gene mutations were detected，including 10 new mutations that had not been reported before （CYP11B1 gene c.715_731del，c.240-2A>G ，c.1359dupG，c.64C>T，c.346T>C，chr8:143957113-143994301 ，chr8：143957206-143994456 fragment deletion， CYP17A1 gene c.1304T>C，StAR gene c.491C>A，HSD3B2 gene c.484G>A ）. The c.1459_1467del （p.D487_F489del） with the highest frequency accounted for 50% （7/14） of CYP17A1 alleles. Conclusion    Genetic testing is important for the early diagnosis and treatment of non-21-OHD. The detection rate of 17α-OHD is close to that of 11β-OHD. And p.D487_F489del and p.Y329Kfs are the main variants in the study. A homozygous mutation of c.484G>A （P.Glu162Lys） is found in 3β-hydroxysteroid dehydrogenase type 2 deficiency，which has not been previously reported.

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Caroli syndrome in children：A clinical analysis of 10 cases GAO Tian-jiao, REN Xiao-xia, HAN Ya-nan, et al 2024, 39(5): 375-379.  DOI: 10.19538/j.ek2024050612 Abstract ( )   Objective    To explore the clinical features of Caroli syndrome in children，including clinical symptoms，signs，laboratory indicators，and imaging pathological and genetic，features. Methods    The clinical data of 10 children with Caroli syndrome diagnosed in Xi'an children's Hospital from January 2018 to June 2021 were analyzed retrospectively. Results    The age range of the 10 children was 2 ~ 9 years old，with an average age of 6.2 years old. All patients visited hospital due to gastrointestinal bleeding，manifested as hematemesis and melena. The levels of white blood cells，red blood cells，hemoglobin，hematocrit，and platelet were reduced. Alanine aminotransferase and aspartate aminotransferase levels were normal. Imaging examination was mainly characterized by intrahepatic bile duct dilation with portal hypertension. All 10 cases had infantile polycystic kidney disease. Liver biopsy in 4 children showed congenital hepatic fibrosis. The susceptibility genes for digestive system diseases in 3 children suggested polycystic kidney and hepatic disease 1 （PKHD1） gene heterozygous mutation（containing three newly identified mutation sites）. Endoscopic ligation of esophageal varices was performed in 5 cases. Postoperative follow-up lasted for half a year，and there was no bleeding again. Gastroscope re-examination showed that esophageal varices was significantly reduced. Conclusion    The main manifestation of Caroli syndrome in children is gastrointestinal bleeding. Imaging features，pathology and genetic examination should be taken into account to avoid missed diagnosis or misdiagnosis.

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Randomized controlled，multi-center clinical study of Sechangzhixiesan in the treatment of acute watery diarrhea in children GUO Sheng-xuan, MA Rong, HU Si-yuan, et al 2024, 39(5): 380-385.  DOI: 10.19538/j.ek2024050613 Abstract ( )   Objective    To evaluate the clinical efficacy and safety of Sechangzhixiesan in the treatment of acute watery diarrhea in children. Methods    Stratified block randomized，double-blind method was adopted. A total of 248 children with acute watery diarrhea were enrolled in 17 research centers from December 2020 to March 2023. They were divided into an experimental group of 123 cases and a control group of 125 cases. They were given Sechangzhixiesan and montmorillonite powder respectively to test the non-inferiority of the drugs. The effecton diarrhea，duration of diarrhea，abdominal pain duration，onset time of drug effect in stopping diarrhea，onset time of abdominal pain relief， and safety indicators were compared between the two groups. Results    A total of 245 cases were included in the full analysis set （FAS），including 122 cases in the experimental group and 123 cases in the control group. The main efficacy index was the efficacy （effective rate） of diarrhea for 3 days，which was 83.61% in the experimental group and 85.37% in the control group. The rate ratio of the two groups and the 95%CI （experimental group / control group） was 0.979（0.879～1.09）；the non-inferiority test was established，the experimental group was not inferior to the control group， and the FAS and PPS analysis conclusions were consistent. There were no statistically significant differences in the duration of diarrheaor or onset time of drug effect in stopping diarrhea; the duration of abdominal pain and the onset time of abdominal pain were shorter in the experimental groups than in the control group， and the difference was statistically significant； the conclusions of FAS and PPS analysis were consistent. In terms of safety，there was no statistically significant difference in the incidence of adverse events/adverse reactions between the two groups. Adverse reactions occurred in 1 case in each group，both of which were constipation，and it was a known adverse reaction in the species. Conclusion    Sechangzhixiesan is non-inferior to montmorillonite powder in treating acute watery diarrhea in children，having obvious analgesic effect，and it exhibits good safety，implying its promising clinical application value.

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Multi-center randomized controlled clinical study of Danmu extract syrup in the treatment of children with influenza TIAN Zhi-fan, WANG Xue-feng, LIU Han-min, et al 2024, 39(5): 386-394.  DOI: 10.19538/j.ek2024050614 Abstract ( )   Objective    To evaluate the clinical efficacy and safety of Danmu extract syrup in the treatment of children with influenza. Methods    A stratified block randomization，positive drug parallel control，and multicenter clinical trial design was used to enroll 480 children from 6 centers with influenza. They were randomly divided into a treatment group and a control group，with 240 cases in each group. The treatment group was treated with Danmu extract syrup，while the control group was treated with oseltamivir phosphate granules，both for 5 days. Observe the clinical recovery time，complete fever relief time，resolution time of main symptoms （pharyngalgia，cough，headache），Canadian Acute Respiratory Disease and Influenza Scale （CARIFS） score，traditional Chinese medicine syndrome efficacy，incidence rate of complications，severe and critical illness，and safety indicators of the two groups of children. Results    The FAS results showed that the main efficacy indicators were as follows.  The clinical recovery time of both groups was 4 days，and the complete fever relief time of both groups was 36 hours. After COX regression analysis，the inter-group HR and 95% CI were estimated，and after adjusting for the influence of central factors，0.67 was used as the non-inferiority standard. The treatment group was not inferior to the control group. Secondary efficacy indicators were as follows. The disappearance time of pharyngeal pain symptoms in the treatment group was 3 days， lower than that in the control group （4 days），with a statistically significant difference （P<0.01）. There was no statistically significant difference in the disappearance time of cough and headache symptoms between the two groups （P>0.05）. After medication，the CARIFS symptom dimension score in the treatment group was better than that in the control group，with a statistically significant difference （P<0.01）. The scores in CARIFS functional dimension and influencing parental dimension in the treatment group were better than those in the control group，but there was no statistically significant difference between the two groups （P>0.05）. The recovery rate of traditional Chinese medicine syndrome in the treatment group was 75.32%， higher than that in the control group，which was 60.50%，with a statistically significant difference （P<0.01）. There was no statistically significant difference in the total effective rate of traditional Chinese medicine syndrome between the two groups （P>0.05）. In addition，there was no statistically significant difference in the incidence of complications，severe and critical illnesses，or adverse events and reactions between the two groups （P>0.05）. Conclusion    Danmu extract syrup has a significant effect on children's influenza，which is not inferior to oseltamivir phosphate granules，and helps to alleviate the symptoms of sore throat，shorten the course of influenza，and improve the health of children. Its clinical application is safe， and it is suitable for popularization.

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Ureaplasma parvum meningitis complicated with hydrocephalus in premature infants：A case report and literature review FENG Meng-ting, JI Qiong, WANG Bao-ying, et al 2024, 39(5): 395-400.  DOI: 10.19538/j.ek2024050615 Abstract ( )   To explore the clinical features，laboratory examination and treatment of neonatal ureaplasma parvum meningitis. The clinical data and laboratory and diagnosis and treatment process of one case of hydrocephalus caused by ureaplasma parvum infection were retrospectively analyzed，and the relevant literature was reviewed. The female baby with 27 weeks of gestational age. She had dyspnea after birth，accompanied by signs of infection and bleeding tendency. Intracranial hemorrhage and cerebrospinal fluid analysis abnormalities were found in early postnatal period. Both blood and cerebrospinal fluid cultures were negative. The cerebrospinal fluid finding did not improve after 28d of antibiotic treatment，and the ventricular dilatation and hydrocephalus appeared，so the baby was transferred to our hospital. After admission，the Ommaya reservoir was implanted and the cerebrospinal fluid was drained daily. Cerebrospinal fluid metagenomic next-generation sequencing was performed to confirm the diagnosis of ureaplasma parvum meningitis，and intravenous treatment with azithromycin was performed for 21 days. Hydrocephalus was improved and cerebrospinal fluid finding returned to normal. Cerebrospinal fluid continued to be drained daily after discharge. Lateral ventriculoperitoneal shunt was performed at 6 months after birth. The patient was followed up to 12 months after birth，and the child has normal ventricular size and normal mental and motor development.