球囊扩张术在小儿食道狭窄及贲门失弛缓中的应用分析

目的比较球囊扩张术(EBD)治疗小儿食道狭窄及贲门失弛缓的疗效,分析预后影响因素。方法 2012年1月-2014年12月共28例患儿纳入研究。其中,食道狭窄22例,贲门失弛缓6例,利用扩张球囊进行治疗。结果 28例患儿共接受57次球囊扩张术,随访12~36个月,28例全部成功。其中,23例患儿症状完全缓解(82.14%),4例患儿部分缓解(14.28%),1例无效(3.57%),1例复发(3.57%)。治疗成功率、有效率、并发症发生率及复发率两组患儿比较差异无统计学意义。食道狭窄患儿中,多个狭窄患儿并发症较单个狭窄患儿多,狭窄直径越小并发症越多,比较差异有统计学意义(P<0.05);治疗效果与狭窄直径和狭窄个数显著相关(P<0.05)。贲门失弛缓组患儿中,发病年龄及明确诊断前症状持续的时间均对EBD治疗效果没有显著影响。结论 EBD是小儿食道狭窄及贲门失弛缓安全有效的治疗手段;狭窄直径和狭窄个数是影响食道狭窄治疗效果最重要的因素,而第一次球囊扩张与食道闭锁手术之间的时间则是术后食道狭窄患儿最重要的影响因素。

Accuracy of multi-echo Dixon sequence in quantification of hepatic steatosis in Chinese children and adolescents

BACKGROUND Nonalcoholic fatty liver disease(NAFLD) is currently the outstanding cause of chronic liver disease in children and adolescents, especially in overweight and obese groups. Liver biopsy is the reference standard to diagnose NAFLD but invasive, thus it is not the best choice in clinical diagnosis and follow-up.Magnetic resonance(MR) is widely used in clinical trials to noninvasively quantify liver fat content in adults and children in foreign countries. While currently, it is rarely used in Chinese children and adolescents. We postulated that quantifying hepatic steatosis by MR could be extended to children and adolescents in China.AIM To investigate the accuracy of MR imaging(MRI) in quantifying liver fat with MR spectroscopy(MRS) as a reference. A secondary goal was to assess the prevalence of NAFLD in overweight and obese Chinese children and adolescents.METHODSThere were 86 children and adolescents enrolled in this study, including 65 overweight and obese children and 21 healthy children. The participants underwent MRI and MRS. MRI and MRS were performed using multi-echo Dixon and HISTO sequences, respectively, to calculate hepatic proton density fat fraction(PDFF). Hepatic steatosis was diagnosed using MRS-PDFF > 5% as the threshold. Spearman's analysis was used to evaluate the correlation between MRI and MRS. The agreement between these two methods was assessed by BlandAltman analysis.RESULTS The MRI-PDFF in the MRS region of interest and the entire liver was 9.9% ±10.3% with a range of 0.3%-39.9%, and 10.6% ± 9.4% with a range of 1.9%-38.9%,respectively. The MRS-PDFF was 9.1% ± 10.0%, with a range of 0.5%-37.8%. The incidence of hepatic steatosis detected by MRS-PDFF was 46.5%(40/86) of all participants, all of whom belonged to the overweight and obese group.Spearman's analysis indicated an excellent correlation between multi-echo Dixon and MRS(r > 0.9, P < 0.01). Bland-Altman analysis also demonstrated a good agreement between these two methods.CONCLUSION Multi-echo Dixon shows an excellent correlation and agreement with MRS in quantifying liver fat content and could be a potential tool to detect hepatic steatosis in Chinese children and adolescents.

Infant cholestasis patient with a novel missense mutation in the AKR1D1 gene successfully treated by early adequate supplementation with chenodeoxycholic acid: A case report and review of the literature

Steroid 5β-reductase [aldo-keto reductase family 1 member D1(AKR1D1)] is essential for bile acid biosynthesis. Bile acid deficiency caused by genetic defects in AKR1D1 leads to life-threatening neonatal hepatitis and cholestasis. There is still limited experience regarding the treatment of this disease. We describe an infant who presented with hyperbilirubinemia and coagulopathy but normal bile acid and γ-glutamyltransferase. Gene analysis was performed using genomic DNA from peripheral lymphocytes from the patient, his parents, and his elder brother. The patient was compound heterozygous for c.919C>T in exon 8 and exhibited a loss of heterozygosity of the AKR1D1 gene, which led to an amino acid substitution of arginine by cysteine at amino acid position 307(p.R307C). Based on these mutations, the patient was confirmed to have primary 5β-reductase deficiency. Ursodeoxycholic acid(UDCA) treatment did not have any effect on the patient. However, when we changed to chenodeoxycholic acid(CDCA) treatment, his symptoms and laboratory tests gradually improved. It is therefore crucial to supplement with an adequate dose of CDCA early to improve clinical symptoms and to normalize laboratory tests.

Prevention program for the COVID-19 in a children’s digestive endoscopy center

The pneumonia caused by the coronavirus disease-2019(COVID-19)outbreak in Wuhan,China constitutes a public health emergency of international concern.The gastrointestinal symptoms of vomiting,diarrhea and abdominal pain and the detection of COVID-19 nucleic acid from fecal specimens in a small number of patients suggest the possibility of transmission via the gastrointestinal tract.People of all ages are vulnerable to this virus,including children.Digestive endoscopy is an invasive procedure during which children cannot wear masks;therefore,they have higher risks of exposure to COVID-19,and the digestive endoscopy center is a relatively high-risk area for COVID-19 infection.Based on these factors and in combination with related policies and regulations,a prevention and control program for the COVID-19 pneumonia in a children's digestive endoscopy center was established to prevent the COVID-19 nosocomial infection.