Objective To evaluate the clinical value of Bristol stool form scale(BSFS) for bowel preparation in pediatric patients. Methods Data of 202 pediatric patients undergoing colonoscopy were collected from May 2016 to December 2016 at Children's Hospital of Fudan University. All patients received polyethylene glycol ( PEG)-4000 with clear fluid diet for bowel preparation. BSFS was used to record the stool form, and Boston bowel preparation scale ( BBPS) was used to evaluate the quality of bowel cleansing.Differences in BBPS score between the enema group with BSFS 6 and the no enema group with BSFS 7 were studied. Based on the data types, t ( or t') test and chi-square test were used to analyze the influencing factors for colon preparation respectively. Those factors of statistical significance were studied with multivariate logistic regression analysis. Results The mean defecation time of pediatric patients during bowel preparation was 14. 4±6. 8. One hundred and sixty-five (81. 7％) patients were assessed as 7 points in BSFS, 37 ( 18. 3％) were 6 points in BSFS with supplemented enema, and 154( 76. 2％) patients achieved adequate bowel preparation. No significant differences were observed between the no enema group and enema group in the BBPS scores [75. 2％(124/165) VS 81. 1％(30/37), χ2=0. 587, P=0. 526]. Significant factors for inadequate colon preparation were constipation history (χ2=32. 588, P=0. 000 ) , total time of defecation(t=3. 432,P=0. 001) and total time of BSFS 7 (t'=2. 877,P=0. 005). Multivariate logistic regression analysis showed constipation history ( P = 0. 000, OR = 12. 620, 95％CI:4. 850-32. 800 ) was independent risk factor for inadequate colon preparation. Conclusion Patients of BSFS 6 points could receive warm saline enema as a remedy. Patients with total time of defecation less than 15 and total time of BSFS ( 7 points) less than 8 are liable for the possibility of inadequate bowel preparation. It is also suggested that for patients with constipation history, the time of bowel preparation should be prolonged for microscopic visual field clarity.

OBJECTIVE: To explore the genetic basis of a child with Very early onset inflammatory bowel disease (VEOIBD). METHODS: A female child who had presented at the Children's Hospital of Fudan University on May 23, 2018 due to occurrence of diarrhea and fever 6 days after birth was selected as the study subject. Clinical data of the child was collected. Family-based whole-exome sequencing (WES) was carried out. Candidate variant was verified by Sanger sequencing and PCR of the patient and her parents. RESULTS: The child had developed the symptoms 6 days after birth, with main manifestations including diarrhea, fever, failure to thrive, rectovestibular fistula and hypothyroidism. An enterostomy was performed at the age of 3.5 months due to severe intestinal adhesion and obstruction. Based on her clinical manifestations, colonoscopic finding, and results of biopsies, she was diagnosed with VEOIBD in conjunct with congenital hypothyroidism. Replacement treatment of levothyroxine was given since one month of age. Family-based WES revealed that the child has harbored compound heterozygous variants of the DUOX2 gene, namely c.2654G>T (p.R885L) and c.505C>T (p.R169W), in addition with a heterozygous c.301C>T (p.R101W) variant of the IL10RA gene. Re-analysis of the WES data revealed that the patient also had a 333 bp deletion spanning exon 1 of the IL10RA gene (Chr11: 117857034_117857366). CONCLUSION For patients with VEOIBD, genetic testing is recommended. Presence of additional DUOX2 gene variants might have exacerbated the clinical symptoms in this patient. Above finding has facilitated genetic counseling and prenatal diagnosis for this family, and raised clinicians' awareness of this rare disease.
Female ; Humans ; Infant ; Pregnancy ; Diarrhea ; Dual Oxidases/genetics* ; Exons ; Failure to Thrive ; Inflammatory Bowel Diseases/genetics*

Objective:To construct a program for improving care ability of caregivers for children with home enteral tube feeding (HETF) based on timing theory, and explore its preliminary effects.Methods:Based on the framework of timing theory and literature review, intervention measures to improve the care ability of caregivers for children with HETF were extracted and summarized to form a preliminary program draft. Fourteen caregivers with experience in caring for children with HETF were subjected to qualitative interviews to supplement the content of the program, and the program was revised through expert meetings to form the final draft. Non-synchronous control method was adopted, and 89 children with HETF and 89 caregivers of them admitted to the Children's Hospital of Fudan University from March 2022 to February 2023 were continuously included as study subjects. The children and their caregivers included from March to August 2022 were in control group ( n=42), and the children and their caregivers included from September 2022 to February 2023 were in intervention group ( n=47), and the plan was preliminarily applied. Family Caregiver Task Inventory (FCTI) was used to evaluate the impact of the program on improving caregiver care ability. Age specific body weight z-values, height specific body weight z-values, and complications were used to evaluate the impact of the program on the growth and development of children and the incidence of tube feeding complications. The data was collected at the time of enrollment and one, two, and three months after discharge. Results:There were 66 children who completed the whole study, including 33 children in the control group and the intervention group, and 33 caregivers in each group. The application results showed that the total score of FCTI in the intervention group decreased from (25.91±2.94) at enrollment to (5.85±2.60) at three months after discharge, while the total score of FCTI in the control group decreased from (26.12±4.34) at enrollment to (12.52±3.60) at three months after discharge, and the total score of FCTI in both groups decreased over time. At one, two, and three months after discharge, the total FCTI score of the intervention group was lower than that of the control group, and the difference was statistically significant ( P<0.05). At three months after discharge, the incidence of complications in children with HETF in the intervention group was lower than that in the control group with a statistical difference ( P<0.05) . Conclusions:The program for improving care ability of caregivers for children with HETF based on timing theory is scientific and provides basis for the management of home tube feeding of children.

Objective:To describe the implementation status of home enteral nutrition (HEN) in pediatric patients, providing reference for the management of HEN in pediatric patients.Methods:This study was a cross-sectional study. From March 2020 to May 2021, convenience sampling was used to select 161 pediatric patients who were discharged from the Children's Hospital of Fudan University and underwent HEN as participants. The survey questionnaire was designed to collect clinical data based on the research purpose.Results:Among the 161 pediatric patients who underwent HEN, congenital malformations, digestive system diseases, neurological diseases, malignant tumors, and respiratory system diseases accounted for 38.5% (62/161), 21.7% (35/161), 19.3% (31/161), 11.2% (18/161), and 9.3% (15/161), respectively. A total of 120 pediatric patients were followed up and recorded with nutritional supplements, 49 pediatric patients received whole protein nutritional supplements, 19 received amino acid nutritional supplements, 16 received whole protein nutritional supplements with homemade homogenization, 15 received short peptide nutritional supplements, seven received breast milk with whole protein nutritional supplements, five received homemade homogenization, five received animal milk, two received breast milk, and two received amino acid nutritional supplements with homemade homogenization. A total of 118 pediatric patients were followed up with tube feeding, including 107 cases using intermittent feeding, nine cases using continuous feeding, and two cases using intermittent combined continuous feeding. There were 46 children with tube slippage, nine cases of vomiting, six cases of tube blockage, three cases of abdominal pain and bloating, two cases of diarrhea, one case of nasal redness, one case of aspiration pneumonia, and one case of exudation around the stoma. After a short-term HEN, 74 cases continued to receive tube feeding, 49 cases were successfully removed from the tube and switched to oral feeding, three cases were switched to tube and oral combined feeding, five cases died due to severe illness.Conclusions:Pediatric patients undergoing HEN have a wide range of diseases and a high incidence of tube slippage. Education should be provided to family caregivers to enhance their ability to identify and handle complications, as well as to make correct choices in feeding formulations and methods. We should also establish a sound follow-up system, closely monitor the nutritional status of pediatric patients, and actively assist in preventing complications.

Objective:To evaluate the efficacy and safety of bendamustine monotherapy in Chinese patients with relapsed/refractory (R/R) B cell non-Hodgkin lymphoma (B-NHL) .Methods:This prospective, multicenter, open label, single-arm, phase Ⅱ study investigated bendamustine’s efficacy and safety in Chinese patients with R/R B-NHL. A total of 78 patients with B-NHL in 11 hospitals in China from March 2012 to December 2016 were included, and their clinical characteristics, efficacy, and survival were analyzed.Results:The median age of all patients was 58 (range, 24-76) years old, and 69 (88.4% ) patients had stage Ⅲ/Ⅳ disease. 61 (78.2% ) patients were refractory to previous treatments. Patients received a median of 4 (range, 1-10) cycles of bendamustine treatment. The overall response rate was 61.5 (95% CI 49.8-72.3) % , the median response duration was 8.3 (95% CI 5.5-14.0) months, and the complete remission (CR) rate was 5.1 (95% CI 1.4-12.6) % . In the full analysis set, median progression-free survival (PFS) and median OS were 8.7 (95% CI 6.7-13.2) months and 25.5 months (95% CI 14.2 months to not reached) , respectively, after a median follow-up of 33.6 (95% CI 17.4-38.8) months. Lymphopenia (74.4% ) , neutropenia (52.6% ) , and leukopenia (39.7% ) , thrombocytopenia (29.5% ) and anemia (15.4% ) were the most common grade 3-4 hematologic adverse events (AE) . The most frequent non-hematologic AEs included nausea (43.6% ) , vomiting (33.3% ) , and anorexia (29.5% ) . Univariate and multivariate analysis showed that <4 cycles of bendamustine treatment was a poor prognostic factor for PFS ( P=0.003) , and failure to accept fludarabine containing regimen was a poor prognostic factor for OS ( P=0.009) . Conclusion:Bendamustine monotherapy has good efficacy and safety in the treatment of patient with R/R B-NHL.