Creutzfeldt-Jakob disease(CJD)is a rapidly progressive and fatal neurodegenerative disorder caused by prions,with certain infectivity and iatrogenic transmission risks.With the rapid progress and application of new dia-gnostic biomarkers and detection methods,as well as the construction and improvement of surveillance and reporting systems,the detection of CJD in patients domestically and internationally has shown an increasing trend year by year.Due to its long incubation period and heterogeneity of early symptoms,early identification and diagnosis of the disease is difficult,increasing the risk of transmission within medical institutions.Currently,there is a lack of con-sensus on the infection prevention and control of CJD.In order to timely identify and diagnose CJD as well as effec-tively block its transmission in medical institutions,this consensus summarizes 15 clinical concerns and formulates 24 specific recommendations based on the latest domestic and international research findings and clinical evidence,as well as combines with clinical practice,aiming to standardize healthcare-associated infection prevention and control measures for CJD and reduce its transmission risk in medical institutions.

Creutzfeldt-Jakob disease(CJD)is a rapidly progressive and fatal neurodegenerative disorder caused by prions,with certain infectivity and iatrogenic transmission risks.With the rapid progress and application of new dia-gnostic biomarkers and detection methods,as well as the construction and improvement of surveillance and reporting systems,the detection of CJD in patients domestically and internationally has shown an increasing trend year by year.Due to its long incubation period and heterogeneity of early symptoms,early identification and diagnosis of the disease is difficult,increasing the risk of transmission within medical institutions.Currently,there is a lack of con-sensus on the infection prevention and control of CJD.In order to timely identify and diagnose CJD as well as effec-tively block its transmission in medical institutions,this consensus summarizes 15 clinical concerns and formulates 24 specific recommendations based on the latest domestic and international research findings and clinical evidence,as well as combines with clinical practice,aiming to standardize healthcare-associated infection prevention and control measures for CJD and reduce its transmission risk in medical institutions.

OBJECTIVES: To investigate the clinical application of endoscopic esophageal dilation in the treatment of corrosive esophageal strictures in children. METHODS: A retrospective analysis was performed on the clinical data of 15 children with corrosive esophageal strictures who underwent endoscopic esophageal dilation in Children's Hospital, Zhejiang University School of Medicine. The clinical features, treatment modality of endoscopic esophageal dilation, number of dilations, complications, and prognosis were reviewed. RESULTS: A total of 96 esophageal dilations were performed in the 15 children with corrosive esophageal strictures, with a median of 6 dilations per child. Among them, 9 children (60%) underwent 6 or more dilations. The children with a stricture length of >3 cm had a significantly higher number of dilations than those with a stricture length of ≤3 cm (P<0.05). The children with strictures in a single segment had a significantly better treatment outcome than those with strictures in multiple segments (P=0.005). No complication was observed during all sessions of dilation. The overall effective rate (including significant improvement and improvement) of endoscopic esophageal dilation treatment was 87%, with 2 cases of failure. CONCLUSIONS Endoscopic esophageal dilation is an effective and relatively safe treatment method for corrosive esophageal strictures in children, and children with strictures in a single segment tend to have a better treatment outcome than those with strictures in multiple segments.
Child ; Humans ; Esophageal Stenosis/therapy* ; Constriction, Pathologic/complications* ; Dilatation/methods* ; Caustics ; Retrospective Studies ; Treatment Outcome

A girl aged 12 years and 2 months presented with recurrent abdominal pain and vomiting for more than 2 years and arthrodynia for 3 months. She was diagnosed with recurrent acute pancreatitis with unknown causes and had been admitted multiple times. Laboratory tests showed recurrent significant increases in fasting serum triglyceride, with elevated immunoglobulin and positive antinuclear antibody. The girl was improved after symptomatic supportive treatment. The girl developed arthrodynia with movement disorders 3 months before, and proteinuria, hematuria, and positive anti-double-stranded DNA antibody were observed. The renal biopsy was performed, and the pathological examination and immunofluorescence assay suggested diffuse lupus nephritis (type Ⅳ). She was finally diagnosed with systemic lupus erythematosus (SLE), lupus nephritis (type Ⅳ), and recurrent acute pancreatitis. Pancreatitis was suspected to be highly associated with SLE. She was treated with oral hydroxychloroquine sulfate and intravenous methylprednisolone sodium succinate and cyclophosphamide. Arthrodynia was partially relieved. She was then switched to oral prednisone acetate tablets. Intravenous cyclophosphamide and pump infusion of belimumab were regularly administered. Now she had improvement in arthrodynia and still presented with proteinuria and hematuria. It is concluded that recurrent acute pancreatitis may be the first clinical presentation of SLE. For pancreatitis with unknown causes, related immunological parameters should be tested, and symptoms of the other systems should be closely monitored to avoid delaying the diagnosis.
Abdominal Pain ; Acute Disease ; Antibodies, Antinuclear ; Cyclophosphamide ; Female ; Hematuria ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Pancreatitis ; Proteinuria ; Triglycerides ; Vomiting

Objective: To analyze the follow-up and clinical effect of multidisciplinary treatment on the children with spinal muscular atrophy (SMA). Methods: The clinical data including nutritional status, respiratory function, bone health and motor function of 45 children with SMA who received multidisciplinary management 1-year follow-up in the Children's Hospital, Zhejiang University School of Medicine from July 2019 to October 2021 were retrospectively collected. Comparisons before and after management were performed using paired-samples t-test or Wilcoxon rank-sum test, etc. Results: The age of 45 patients (25 boys and 20 girls) was 50.4 (33.6, 84.0) months at the enrollment, with 6 cases of type 1, 22 cases of type 2, and 17 cases of type 3 respectively. After the multidisciplinary management, the cases of SMA patients with malnutrition decreased from 22 to 12 (P=0.030), the level of vitamin D were significantly increased ((45±17) vs. (48±14) nmol/L, t=-4.13, P<0.001). There was no significant difference in the forced vital capacity %pred, the forced expiratory volume at 1 second %pred, and the peak expiratory flow %pred ((76±19)% and (76±21)%, (81±18)% and (79±18)%, (81±21)% and (78±17)%; t=-0.24, 1.36, 1.21; all P>0.05). The Cobbs angle of scoliosis also improved significantly (8.0°(0°, 13.0°) vs. 10.0°(0°, 18.5°), Z=-3.01, P=0.003). The Hammersmith functional motor scale expanded scores of children with SMA type 2 and type 3 both showed significant elevation (11.0 (8.0, 18.0) vs. 11.0 (5.0, 18.5) scores, 44.0 (36.5, 53.0) vs. 44.0 (34.0, 51.5) scores, Z=2.44, 3.11, P=0.015, 0.002). Conclusion: Multidisciplinary management is beneficial for delaying the progression of the multi-system impairments of SMA patients, such as malnutrition, restrictive ventilation dysfunction and scoliosis.
Child ; Male ; Female ; Humans ; Child, Preschool ; Scoliosis ; Retrospective Studies ; Follow-Up Studies ; Muscular Atrophy, Spinal ; Malnutrition

Inflammatory bowel disease (IBD) is a chronic recurrent non-specific inflammatory disease in the intestinal tract. About 10%-56% of children with Crohn's disease and about 10% of children with ulcerative colitis have growth retardation. This study reports four adolescents with IBD and growth hormone deficiency who were diagnosed with Crohn's disease. There were three boys and one girl, with an age of 11.0-13.9 years and a disease duration of 11-85 months at diagnosis. The four patients had the involvement of the small intestine only, the colon only, both the small intestine and the upper gastrointestinal tract, and both the small intestine and the colon respectively. The pediatric Crohn's disease activity index ranged from 27.5 to 45 points. All four patients had a height-for-age Z-score (HAZ) of <-2, and the growth hormone provocative test suggested growth hormone deficiency. Of all four patients, two received recombinant human growth hormone combined with infliximab, one received infliximab only, and one received recombinant human growth hormone combined with mercaptopurine. All four patients had an improvement in HAZ after treatment.
Adolescent ; Child ; Colitis, Ulcerative ; Crohn Disease ; Female ; Growth Hormone ; Humans ; Inflammatory Bowel Diseases ; Infliximab ; Male

OBJECTIVETo study the clinical features and prognosis of gastrointestinal injury caused by foreign bodies in the upper gastrointestinal tract in children.

METHODSA retrospective analysis was performed for the clinical data of 217 children who were diagnosed with foreign bodies in the upper gastrointestinal tract complicated by gastrointestinal injury by gastroscopy from January 2011 to December 2016, including clinical features, gastroscopic findings, complications, and prognosis.

RESULTSAmong the 217 children, 114 (52.5%) were aged 1-3 years. The most common foreign body was coin (99/217, 45.6%), followed by hard/sharp-edged food (45/217, 20.7%) and metal (35/217, 16.1%). The most common gastrointestinal mucosal injury was ulceration (43.8%), followed by erosion (33.2%). Compared with other foreign bodies, button cells were significantly more likely to cause esophageal perforation (P<0.01). The esophagus was the most commonly injured organ (207/217, 95.4%). Of all the 217 children, 24 (11.1%) experienced infection. The children with perforation caused by foreign bodies had a significantly higher incidence rate of infection than those with ulceration caused by foreign bodies (P=0.003). Of all the 217 children, 204 (94.0%) underwent successful endoscopic removal of foreign bodies. Among these children, 98 were hospitalized due to severe mucosal injury and were given anti-infective therapy, antacids, and supportive care including enteral nutrition through a nasogastric tube and/or parenteral nutrition. Of all the children, 10 left the hospital and were lost to follow-up, and all the other children were improved and discharged.

CONCLUSIONSMost cases of foreign bodies in the upper gastrointestinal tract occur at 1-3 years of age. Coin, hard/sharp-edged food, and metal are the most common foreign bodies. Button cells are more likely to cause esophageal perforation. The incidence rate of secondary infection increases with the increasing severity of gastrointestinal mucosal injury. Children undergoing endoscopic removal of foreign bodies and enteral nutrition through a nasogastric tube tend to have a good prognosis.

Female ; Food ; adverse effects ; Foreign Bodies ; diagnosis ; etiology ; therapy ; Humans ; Infant ; Male ; Metals ; adverse effects ; Prognosis ; Retrospective Studies ; Upper Gastrointestinal Tract ; injuries

OBJECTIVETo investigate the impact of timing of nasojejunal feeding tube placement and enteral nutrition on clinical outcomes in children with acute pancreatitis.

METHODSA retrospective analysis was performed on the clinical data of 31 children with acute pancreatitis, who received nasojejunal feeding between January 2008 and July 2013, to investigate the relationship of abdominal symptoms/signs and serum amylase level with the tolerability of catheterization and success rate of enteral nutrition. The treatment outcome and incidence of adverse reactions and complications were compared between the early enteral nutrition group ( ≤7 days from the onset of the disease) and late enteral nutrition group (>7 days from the onset of the disease).

RESULTSAbdominal symptoms/signs and serum amylase level were independent of the tolerable rate of catheterization and success rate of enteral nutrition. Compared with the late enteral nutrition group, the early enteral nutrition group had a shortened time to normal serum amylase level, significantly reduced incidence of systemic complications, length of hospital stay, and hospitalization expenses, and less weight gain. The tolerable rate of catheterization and success rate of enteral nutrition showed no significant difference between the two groups. Similarly, no significant differences were found in the increase in albumin level after enteral nutrition, duration of enteral nutrition, incidence of adverse reactions, and incidence of local complications.

CONCLUSIONSAbdominal symptoms/signs and serum amylase level cannot be used as a measure of whether nasojejunal feeding tube placement and enteral nutrition can be performed. Early enteral nutrition can better improve clinical outcomes in children with acute pancreatitis, and it is feasible.

Acute Disease ; Adolescent ; Child ; Child, Preschool ; Enteral Nutrition ; Female ; Humans ; Intubation, Gastrointestinal ; Male ; Pancreatitis ; therapy ; Retrospective Studies ; Time Factors

OBJECTIVETo evaluate the feasibility and effectiveness of placement of nasojejunal feeding tube and nasojejunal nutrition feeding in children with acute pancreatitis.

METHODTwenty-two patients (of whom 13 had severe acute pancreatitis and 9 acute mild pancreatitis) who needed nutritional intervention were selected. They were from Department of Gastroenterology and Surgery during the years 2009 - 2012, and they were at high nutritional risk after STONGkid nutrition risk screening. The average age of them was 5 - 15 years (9.1 years ± 2.8 years). Assisted by endoscopy, the nasojejunal feeding tube was placed in 22 of 24 patients (in 2 cases of recurrent pancreatitis the tubes were placed again after extubation). Besides the use of regular fasting, antacids, inhibitors of trypsin secretion, and anti-infective treatment, 23 cases of all children got nasojejunal nutrition treatment as well. The outcome measures included the success rate, complications of endoscope-assisted nasojejunal tube placement. The children's tolerance and nutrition indicators (weight, blood lymphocytes count, erythrocytes count, serum albumin, serum creatinine, blood urea nitrogen) were observed before and after enteral nutrition therapy.

RESULTMalnutrition evaluation was done 24 times before treatment among 22 patients, incidence of malnutrition was 33% in 22 cases. Placement of nasojejunal tube placement was attempted for a total of 24 times and was successful on first placement in 22 cases, in two cases the placement was successful on the second placement, so the success rate of the first attempt for placement was 92%. No significant complications were observed in any of the cases. Twenty-three of 24 cases were given standardized enteral nutrition (one case was not given enteral nutrition therapy but underwent ERCP due to obstructive jaundice). Twenty-two of 23 cases could tolerate enteral nutrition well, only 1 case was unable to tolerate enteral nutrition due to the pancreas schizophrenia, paralytic ileus. The treatment of jejunal feeding success rate was 96%. The feeding duration was 2 - 74 d (27.0 d ± 18.3 d). The adverse reactions include plugging of the tube in two cases, constipation in two cases, five cases had abdominal pain, diarrhea in 2 cases, vomiting in 2 cases and 1 case of jejunum retention. No case had nasopharynx ulcers, gastrointestinal perforation, gastrointestinal bleeding, re-feeding syndrome and infection etc. Blood erythrocytes count, serum creatinine, blood urea nitrogen were not significantly changed. Twenty of 23 cases were cured, 2 cases were improved and 1 case was unchanged.

CONCLUSIONEndoscope-assisted nasojejunal tube placement for children with acute pancreatitis is safe and feasible. Nasojejunal nutrition therapy is effective for acute pancreatitis patients who are at severe nutritional risk, especially for the improvement of the nutritional status of children.

Abdominal Pain ; etiology ; Acute Disease ; Adolescent ; Child ; Child, Preschool ; Endoscopy, Gastrointestinal ; Enteral Nutrition ; adverse effects ; instrumentation ; methods ; Feasibility Studies ; Female ; Humans ; Intubation, Gastrointestinal ; adverse effects ; methods ; Jejunum ; Male ; Malnutrition ; etiology ; therapy ; Pancreatitis ; therapy ; Severity of Illness Index ; Treatment Outcome ; Vomiting ; etiology

Adolescent ; Anti-Bacterial Agents ; adverse effects ; therapeutic use ; Bacteria ; drug effects ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Helicobacter Infections ; drug therapy ; Helicobacter pylori ; Humans ; Intestines ; microbiology ; Male ; Probiotics ; pharmacology