Appendicitis combined with Meckel’s diverticulum obstruction, perforation, and inflammation in children: Three case reports

BACKGROUND Meckel’s diverticulum is a common congenital malformation of the small intestine,with the three most common complications being obstruction,per-foration,and inflammation.To date,only a few cases have been reported world-wide.In children,the clinical symptoms are similar to appendicitis.As most of the imaging features are nonspecific,the preoperative diagnosis is not precise.In addition,the clinical characteristics are highly similar to pediatric acute appendicitis,thus special attention is necessary to distinguish Meckel’s diver-ticulum from pediatric appendicitis.Patients with poor disease control should undergo laparoscopic exploration to avoid serious complications,including intestinal necrosis,intestinal perforation and gastrointestinal bleeding.CASE SUMMARY This report presents three cases of appendicitis in children combined with intestinal obstruction,which was caused by fibrous bands(ligaments)arising from the top part of Meckel's diverticulum,diverticular perforation,and diver-ticular inflammation.All three patients,aged 11-12 years,had acute appendicitis as their initial clinical presentation.All were treated by laparoscopic surgery with a favorable outcome.A complete dataset including clinical presentation,dia-gnostic imaging,surgical information,and histopathologic findings was also provided.CONCLUSION Preoperative diagnosis of Meckel’s diverticulum and its complications is challenging because its clinical signs and complications are similar to those of appendicitis in children.Laparoscopy combined with laparotomy is useful for diagnosis and treatment.

Challenges and dilemmas in pediatric hepatic Wilson’s disease

Wilson disease is an autosomal recessive disorder affecting the ATP7B gene located on chromosome 13q.This leads to copper deposition in various organs,most importantly in the liver and brain.The genetic mutations are vast,well reported in the West but poorly documented in developing countries.Hence the diagnosis is made with a constellation of clinico-laboratory parameters which have significant overlap with other liver diseases and often pose a significant dilemma for clinicians.Diagnostic scoring systems are not fool-proof.The availability and affordability of chelators in developing countries impact the drug compliance of patients.While D-penicillamine is a potent drug,its side effects lead to drug discontinuation.Trientine is cost-prohibitive in developing countries.There is no single test to assess the adequacy of chelation.Exchangeable urinary copper is an essential upcoming diagnostic and prognostic tool.In the presence of cirrhosis,hypersplenism clouds the assessment of myelosuppression of drugs.Similarly,it may be difficult to distinguish disease tubulopathy from druginduced glomerulonephritis.Neurological worsening due to chelators may appear similar to disease progression.Presentation as fulminant hepatic failure requires rapid workup.There is a limited window of opportunity to salvage these patients with the help of plasmapheresis and other liver-assisted devices.This review addresses the challenges and clinical dilemmas faced at beside in developing countries.

Disease impact on the quality of life of children with inflammatory bowel disease

To assess the impact of disease characteristics on the quality of life (QOL) in children with inflammatory bowel diseases (IBD).METHODSThis was a cross-sectional study conducted at the First Department of Pediatrics of the University of Athens at the “Aghia Sophia” Children’s Hospital. Children diagnosed with Crohn’s disease (CD) or ulcerative colitis (UC), who were followed as outpatients or during a hospitalization, participated, after informed consent was obtained from their legal representative. QOL was assessed by the IMPACT-III questionnaire. Demographic data and disease characteristics were also collected. Statistical analyses included parametric (Student’s t-test and Pearson’s r) and non-parametric (Mann-Whitney test, Fisher’s test and Spearman’s rho) procedures.RESULTSNinety-nine patients (UC: 37, 73.0% females, CD: 62, 51.6% females), aged 12.8 ± 2.6 years were included. Overall, as well as, sub-domain scores did not differ between UC and CD (overall score: 73.9 ± 13.3 vs 77.5 ± 11.2, respectively, P = 0.16). In the entire sample, total score was related to physician’s global assessment (PGA, patients classified as “mild/moderate” active disease had, on average, 14.8 ± 2.7 points lower total scores compared to those “in remission”, P < 0.001) and age at IMPACT completion (Pearson’s r = 0.29, P = 0.05). Disease activity assessed by the indices Pediatric Ulcerative Colitis activity index, Pediatric Crohn’s disease activity index or PGA was significantly associated with all subdomains scores. Presence of extraintestinal manifestations had a negative impact on emotional and social functioning domains.CONCLUSIONDisease activity is the main correlate of QOL in children with IBD, underlining the importance of achieving and sustaining clinical remission

Comparison of the use of wireless capsule endoscopy with magnetic resonance enterography in children with inflammatory bowel disease

BACKGROUND Magnetic resonance enterography (MRE) and wireless capsule endoscopy (WCE) are equally accepted modalities for noninvasive screening of small bowel involvement (SBI) in children with Crohn’s disease (CD) and indeterminate colitis (IC) albeit there is a paucity of data comparing the two and thereby guiding the clinician in selecting the ideal diagnostic approach. Therefore, the goal of this study is to provide additional evidence for capsule endoscopy role in the evaluation of established Crohn’s disease exacerbation compared to MRE in relation to Pediatric Crohn's Disease Activity Index (PCDAI), and histological indices. AIM To prospectively compare the findings of MRE and WCE and their agreement with PCDAI or histology in children with CD or IC. METHODS Consecutive patients diagnosed with CD and IC were screened for inclusion. After informed consent, patient’s demographic and clinical data was abstracted. The current pediatric disease activity index (PCDAI) and endoscopic findings were included. Patients underwent MRE and WCE including preprocedural patency capsule within a maximum of 7 d of each other. Pathological presence of active small bowel disease in ileal and duodenal biopsies were collected if the endoscopy was performed within 2 mo of the WCE study. Patients who failed to pass the PC were excluded from the study. WCE was read by two different experienced gastroenterologists (Attard TM and Colombo JM) blinded to each other's findings and to the findings on MRE (Mardis NJ). Agreement between WCE reviewers, WCE and MRE findings and concordance between positive PCDAI and SBI based on MRE compared with WCE was computed. RESULTS Forty-five patients were included in the study, 18 withdrew and 27 (20 males and 20 CD), mean age (standard deviation) 13.46 (2.4) years, completed the study protocol. There were no instances of capsule retention. Concordance between gastroenterologist reviewers was excellent for the diagnosis of small intestinal CD with good correlation between the two Lewis scores (r=0.875, P<0.001). Concordance between WCE and MRE was poor (69%). In CD patients, when both MRE and WCE were compared using PCDAI>10 as the standard reference reflecting active small intestinal CD, the sensitivity of MRE and WCE were 100% and 83% respectively and the specificity of MRE and WCE were 57.14% and 78.6%, respectively. If the histology in ileum or/and duodenum was used as the reference for active small bowel involvement, WCE had a higher specificity as compared to MRE (83.3% vs 50%). In patients with Crohn’s disease, those with a positive PCDAI (>10) were more likely to have a positive WCE as compared to those with a negative PCDAI (83% vs 21%;P=0.018). CONCLUSION We suggest that MRE and WCE have a complementary role in the assessment of SBI in CD. WCE detected SBI with a much higher specificity while MRE had a higher sensitivity.

Risk of venous thromboembolism in children and adolescents with inflammatory bowel disease: A systematic review and meta-analysis

BACKGROUND A two-to three-fold increased risk of venous thrombotic events(VTE) has been demonstrated in patients with inflammatory bowel disease(IBD) compared to the general population, but less is known about the risk of VTE in child-and pediatric-onset IBD. In recent years, several studies have reported the rising incidence rate of VTE in juvenile patients with IBD, and the related risk factors have been explored.AIM To evaluate the risk of VTE in children and adolescents with IBD.METHODS Articles published up to April 2021 were retrieved from Pub Med, Embase, Cochrane Library, Web of Science, Sino Med, CNKI, and WANFANG. Data from observational studies and clinical work were extracted. The outcome was the occurrence of VTE according to the type of IBD. The available odds ratio(OR) and the corresponding 95% confidence interval(CI) were extracted to compare the outcomes. Effect size(P), odds ratio(OR), and 95%CI were used to assess the association between VTE risk and IBD disease. Subgroup analyses stratified by subtypes of VTE and IBD were performed.RESULTS Twelve studies(7450272 IBD patients) were included in the meta-analysis. Child and adolescent IBD patients showed increased VTE risk(P = 0.02, 95%CI: 0.01-0.03). Subgroup analyses stratified by IBD(ulcerative colitis(UC): P = 0.05, 95%CI: 0.03-0.06;Crohn’s disease(CD): P = 0.02, 95%CI: 0.00-0.04) and VTE subtypes(portal vein thrombosis: P = 0.04, 95%CI: 0.02-0.06;deep vein thrombosis: P = 0.03, 95%CI: 0.01-0.05;central venous catheter-related thrombosis: P = 0.23, 95%CI: 0.00-0.46;thromboembolic events: P = 0.02, 95%CI: 0.01-0.03) revealed a significant correlation between VTE risk and IBD. Patients with IBD were more susceptible to VTE risk than those without IBD(OR = 2.99, 95%CI: 1.45-6.18). The funnel plot was asymmetric, suggesting the presence of significant publication bias. Pediatric and adolescent IBD patients have an increased VTE risk. UC and CD patients exhibited a high risk of VTE. The risk of VTE subtypes was increased in IBD patients.CONCLUSION The current meta-analysis showed that the incidence and risk of VTE are significantly increased in pediatric and adolescent IBD patients. Thus, IBD might be a risk factor for VTE in children and young adults. High-quality prospective cohort studies are necessary to confirm these findings.

Magnetic resonance imaging in children and adolescents with chronic inflammatory bowel disease

Inflammatory bowel diseases(IBD) represent challenges, both from a diagnostic, and therapeutic point of view. Deep-seated anatomic structures are difficult to assess by ultrasound technique alone. As radiation-free alternative cross-sectional imaging method, magnetic resonance imaging of the intestinal structures is costly and time-consuming. Examination of pediatric patients imply additional considerations: reduction of body motions in younger children and consideration of the most appropriate preparation, and examination technique. The demanding Sellink technique is the only means for appropriately distending the lesser intestine in order to detect small bowel strictures. Oral intake of contrast medium(CM) alone shows its limitations regarding distensibility. The need for intravenous contrast media application needs to be considered, too. Active inflammation of both intestinal wall, and mesentery can be demonstrated accurately. Nevertheless, viable alternatives to CM application is desirable, considering non-negligible adverse reactions. Recent data suggest diffusion weighted imaging might fill this diagnostic gap. Irrespective of sequence technique chosen, bowel movement remains a major obstacle. Antispasmolytics in their function as smooth muscle relaxants help in improving image quality, however, their use in children might be off-label. Optimal preparation for the examination and appropriate imaging technique allow for diagnosing typical patterns of changes in IBD, such as bowel wall thickening, ulcers, mural stratification, strictures, creeping fat, and comb sign, and lymphadenopathy. The article gives a detailed overview of current significance of magnetic resonance imaging pediatric patients suffering from IBD, considering indications, limitations, and safety aspects.

Association between orofacial granulomatosis and Crohn's disease in children:Systematic review

AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn&#x02019;s disease.

Acute liver failure with hemolytic anemia in children with Wilson’s disease:Genotype-phenotype correlations?

BACKGROUND Wilson’s disease(WD)is a rare autosomal recessive inherited disorder of copper metabolism.Acute liver failure(ALF)and hemolytic anemia represent the most severe presentation of WD in children.No clear genotype-phenotype correlations exist in WD.Protein-truncating nonsense,frame-shift,or splice-site variants may be associated with more severe disease.In contrast,missense variants may be associated with late-onset,less severe disease,and more neurological manifestations.Recently,a gene variant(HSD17B13:TA,rs72613567)with a possible hepatic protective role against toxins was associated with a less severe hepatic phenotype in WD.AIM To analyze the possible genotype-phenotype correlations in children with WD presented with ALF and non-immune hemolytic anemia.METHODS The medical records of children with WD diagnosed and treated in our hospital from January 2006 to December 2020 were retrospectively analyzed.The clinical manifestations(ALF with non-immune hemolytic anemia or other less severe forms),laboratory parameters,copper metabolism,ATP7B variants,and the HSD17B13:TA(rs72613567)variant were reviewed to analyze the possible genotype-phenotype correlations.RESULTS We analyzed the data of 51 patients with WD,26 females(50.98%),with the mean age at the diagnosis of 12.36±3.74 years.ALF and Coombs-negative hemolytic anemia was present in 8 children(15.67%),all adolescent girls.The Kayser-Fleisher ring was present in 9 children(17.65%).The most frequent variants of the ATP7B gene were p.His1069Gln(c.3207A>G)in 38.24% of all alleles,p.Gly1341Asp(c.4021G>A)in 26.47%,p.Trp939Cys(c.2817G>T)in 9.80%,and p.Lys844Ter(c.2530A>T)in 4.90%.In ALF with hemolytic anemia,p.Trp939Cys(c.2817G>T)and p.Lys844Ter(c.2530A>T)variants were more frequent than in other less severe forms,in which p.His1069Gln(c.3207A>G)was more frequent.p.Gly1341Asp(c.4021G>A)has a similar frequency in all hepatic forms.For 33 of the patients,the HSD17B13 genotype was evaluated.The overall HSD17B13:TA allele frequency was 24.24%.Its frequency was higher in patients with less severe liver disease(26.92%)than those with ALF and hemolytic anemia(14.28%).CONCLUSION It remains challenging to prove a genotype-phenotype correlation in WD patients.In children with ALF and hemolytic anemia,the missense variants other than p.His1069Gln(c.3207A>G)and frame-shift variants were the most frequently present in homozygous status or compound heterozygous status with site splice variants.As genetic analysis is usually time-consuming and the results are late,the importance at the onset of the ALF is questionable.If variants proved to be associated with severe forms are found in the pre-symptomatic phase of the disease,this could be essential to predict a possible severe evolution.

Inflammatory bowel disease incidence in Czech children:A regional prospective study,2000-2015

AIM To examine the incidence and trends in pediatric inflammatory bowel diseases(IBDs) over 2000-2015 and project the incidence to 2018. METHODS A 16-year prospective study of IBD patients < 19 years of age was conducted in the Czech Republic(the Pilsen region). All incident IBD cases within a well-defined geographical area were retrieved from a prospectively collected computerized clinical database. Historical Czech data were used for comparison(1990-2001). Our catchment population was determined from the census data. We calculated the incidence by relating the number of newly diagnosed cases to the size of thepediatric population-at-risk in each calendar year. Age/sex, disease type, place of residence, and race/ethnicity were identified. RESULTS In total, 170 new IBD cases [105 Crohn's disease(CD), 48 ulcerative colitis(UC), and 17 IBD-unclassified(IBD-U)] were identified. The median age at IBD diagnosis was 14.2 years, 59.4% were males, and 97.1% were Caucasians. A male preponderance of IBD(P = 0.026) and CD(P = 0.016) was observed. With 109209 person-years in the catchment area, the average incidence of IBD per 100000 person-years was 10.0(6.2 for CD, 2.8 for UC, and 1.0 for IBD-U) for children aged 0 to 19 years; for those aged 0 to 15 years, the incidence rate was 7.3(4.6 for CD, 2.0 for UC, and 0.7 for IBD-U). An increase in incidence with age was observed(P = 0.0003). Over the 16-year period, the incidence increased for IBD patients(P = 0.01) and CD in particular(P < 0.0001), whereas the incidence for UC(P = 0.09) and IBD-U(P = 0.339) remained unchanged. IBD-projected data from 2016 to 2018 were 12.1, 12.3 and 12.6 per 100000 personyears, respectively.CONCLUSION Pediatric-onset IBD incidence is around its highest point. The increase, which is particularly pronounced for CD, may be challenging to relate to causes of pediatric disease.

Factors Affecting Children’s Cholesterol Levels with a View to Prevent Cardiovascular Disease—The Unique Benefits of Health Screening at the Elementary School

Background: The prevention of cardiovascular disease (CVD) can substantially contribute to reducing the mortality rate across countries. The level of interest in cholesterol among experts increases when the subjects are elderly and obese individuals. However, specialists do not recommend that children should receive the cholesterol test. The objective of this study was to investigate the distribution of cholesterol levels among the children of public school and their parents’ lifestyles that are associated with cholesterol levels, and to assess the need for and utility of cholesterol testing in school settings. Methods: The study investigated a group of 226 fourth-grade public school children aged between 9 and 10 years and guardians in Akitakatacity of Hiroshima Prefecture, Japan. Multiple regression analyses were performed with the logarithmic value of cholesterol levels as a response variable, awareness about lifestyles and health of children as an explanatory variable, and child’s sex and body mass index (BMI) as moderating variables. Using questionnaires about lifestyles, the step-down procedure was applied in multiple regression analyses to narrow down relevant lifestyle variables. Results: The percentage of children with the high total cholesterol (TC) value was about 15 and with low-density lipoprotein (LDL) cholesterol value was about 10. Children with low high-density lipoprotein (HDL) cholesterol value were 5%. Treatment was not required according to the comprehensive assessment. Cholesterol levels were associated with the situation of the children and their guardians as follows, guardian need to consider the food, and child has the correct knowledge about food like how to eat snacks. Conclusions: Children had poor cholesterol levels. From childhood, along with the parents, there is a need to learn about appropriate level of cholesterol for CVD prevention.

High-sensitivity C-reactive protein in paediatric inflammatory bowel disease

AIM:To study whether high-sensitivity C-reactive protein(hs-CRP) measurement can aid the assessment of disease activity and glucocorticoid treatment in paediatric inflammatory bowel disease(IBD).METHODS:CRP levels were measured in 39 children with IBD undergoing colonoscopy [median age 12.8 years,Crohn's disease(CD) n=20],in 22 other children with IBD followed for acute response to glucocorticoids,and in 33 paediatric non-IBD patients.When standard CRP level was below detection limit(<5mg/L),hs-CRP was analyzed.RESULTS:Sixty-four percent(25/39) of the children with IBD undergoing colonoscopy displayed undetectable(<5mg/L) standard CRP levels.Of these,the hs-CRP measurement could not differentiate between active(median,0.2 mg/L,range,0.007-1.37,n=17) or quiescent(0.1 mg/L,0.01-1.89,n=8,P=NS) disease.Patients with ileocolonic CD had higher CRP levels(14mg/L,0.06-45,n=13) than patients with no ileal involvement(0.18 mg/L,0.01-9,n=7,P<0.01) or ulcerative colitis(UC)(0.13 mg/L,0.007-23,P<0.05).In children with active IBD treated with systemic glucocorticoids,the standard CRP was undetectable in 59% of the patients.The hs-CRP levels did not differ between patients that responded to steroid therapy and in non-responders.CONCLUSION:The measurement of hs-CRP did not prove useful in the assessment of disease activity or glucocorticoid treatment in paediatric IBD patients that had undetectable standard CRP.

Consecutive fecal calprotectin measurements for predicting relapse in pediatric Crohn's disease patients

BACKGROUND Asymptomatic children with Crohn's disease(CD) require ongoing monitoring to ensure early recognition of a disease exacerbation.AIM In a cohort of pediatric CD patients, we aimed to assess the utility of serial fecal calprotectin measurements to detect intestinal inflammatory activity and predict disease relapse.METHODS In this prospective longitudinal cohort study, children with CD on infliximab therapy in clinical remission were included. Fecal calprotectin levels were assessed at baseline and at subsequent 2-5 visits. Clinical and biochemical disease activity were assessed using the Pediatric Crohn's Disease Activity Index, Creactive protein and erythrocyte sedimentation rate at baseline and at visits over the following 18 mo.RESULTS 53 children were included and eighteen patients(34%) had a clinical disease relapse during the study. Baseline fecal calprotectin levels were higher in patients that developed symptomatic relapse [median(interquartile range), relapse 723μg/g(283-1758) vs 244 μg/g(61-627), P = 0.02]. Fecal calprotectin levels > 250μg/g demonstrated good predictive accuracy of a clinical flare within 3 mo(area under the receiver operator curve was 0.86, 95% confidence limits 0.781 to 0.937).CONCLUSION Routine fecal calprotectin testing in children with CD in clinical remission is useful to predict relapse. Levels > 250 μg/g are a good predictor of relapse in the following 3 mo. This information is important to guide monitoring standards used in this population.

Crohn’s and colitis in children and adolescents

Crohn＇s disease and ulcerative colitis can be grouped as the inflammatory bowel diseases （IBD）. These conditions have become increasingly common in recent years, including in children and young people. Although much is known about aspects of the pathogenesis of these diseases, the precise aetiology is not yet understood, and there remains no cure. Recent data has illustrated the importance of a number of genes-several of these are important in the onset of IBD in early life, including in infancy. Pain, diarrhoea and weight loss are typical symptoms of paediatric Crohn＇s disease whereas bloody diarrhoea is more typical of colitis in children. However, atypical symptoms may occur in both conditions： these include isolated impairment of linear growth or presentation with extra-intestinal manifestations such as erythma nodosum. Growth and nutrition are commonly compromised at diagnosis in both Crohn＇s disease and colitis. Consideration of possible IBD and completion of appropriate investigations are essential to ensure prompt diagnosis, the- reby avoiding the consequences of diagnostic delay. Patterns of disease including location and progression of IBD in childhood differ substantially from adult- onset disease. Various treatment options are available for children and adolescents with IBD. Exclusive enteral nutrition plays a central role in the induction of remission of active Crohn＇s disease. Medical and surgical therapies need to considered within the context of a growing and developing child. The overall management of these chronic conditions in children should include multi-disciplinary expertise, with focus upon maintaining control of gut inflammation, optimising nutrition, growth and quality of life, whilst preventing disease or treatment-related complications.

Crohn's disease and Takayasu's arteritis: An uncommon association

Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.

A pediatric non-protein losing Menetrier's disease successfully treated with octreotide long acting release

Pediatric Menetrier's disease (MD) is an uncommon, acute, self-limited hypertrophic gastropathy characterized by enlarged gastric folds associated with epithelial hyperplasia and usually accompanied by protein losing gastropathy. Gastric cytomegalovirus infection is found in one third of MD children and its treatment is often associated with remission. Diagnosis often requires fullthickness biopsy due to inability to detect typical histological findings with conventional endoscopic biopsy. We report an uncommon case of non self-limited pediatric MD needing endoscopic mucosal resection for diagnosis which was then successfully treated with octreotide long-acting release (LAR). To the best of our knowledge, this is the first pediatric MD case successfully treated with octreotide LAR. Our experience suggests octreotide LAR as treatment for refractory MD before gastrectomy.

Fungal dysbiosis predicts the diagnosis of pediatric Crohn's disease

AIM To investigate the accuracy of fungal dysbiosis inmucosa and stool for predicting the diagnosis of Crohn's disease(CD). METHODS Children were prospectively enrolled in two medical centers: one university hospital and one private gastroenterology clinic in the city of Riyadh, Kingdom of Saudi Arabia. The children with confirmed diagnosis of CD by standard guidelines were considered cases, and the others were considered non-inflammatory bowel disease controls. Mucosal and stool samples were sequenced utilizing Illumina MiSeq chemistry following the manufacturer's protocols, and abundance and diversity of fungal taxa in mucosa and stool were analyzed. Sparse logistic regression was used to predict the diagnosis of CD. The accuracy of the classifier was tested by computing the receiver operating characteristic curves with 5-fold stratified cross-validation under 100 permutations of the training data partition and the mean area under the curve(AUC) was calculated. RESULTS All the children were Saudi nationals. There were 15 children with CD and 20 controls. The mean age was 13.9(range: 6.7-17.8) years for CD children and 13.9(3.25-18.6) years for controls, and 10/15(67%) of the CD and 13/20(65%) of the control subjects were boys. CD locations at diagnosis were ileal(L1) in 4 and colonic(L3) in 11 children, while CD behavior was non-stricturing and non-penetrating(B1) in 12 and stricturing(B2) in 3 children. The mean AUC for the fungal dysbiosis classifier was significantly higher in stools(AUC = 0.85 ± 0.057) than in mucosa(AUC = 0.71 ± 0.067)(P < 0.001). Most fungal species were significantly more depleted in stools than mucosal samples, except for Saccharomyces cerevisiae and S. bayanus, which were significantly more abundant. Diversity was significantly more reduced in stools than in mucosa. CONCLUSION We found high AUC of fungal dysbiosis in fecal samples of children with CD, suggesting high accuracy in predicting diagnosis of CD.

Regional variation of pediatric inflammatory bowel disease in Saudi Arabia: Results from a multicenter study

BACKGROUND Incidence and severity variations of inflammatory bowel disease(IBD)have been reported from Western populations between continents and regions of the same countries.However,no data were available from other countries.AIM To investigate the regional differences in the IBD profiles of pediatric patients from the Kingdom of Saudi Arabia.METHODS Data from a national multicenter IBD study were used.The incidence,time trend,and clinical presentation of Crohn’s disease(CD)and ulcerative colitis(UC)in the Central region(CR),Western region(WR),and Eastern region(ER)were analyzed and compared.Statistical analysis included Poisson regression analysis for incidence variation and Chi-square test for demographic and clinical parameters.A P<0.05 was considered significant.RESULTS The prevalence of positive family history was lower in children with CD from the ER than the CR or the WR.Consanguinity rate was higher in children with CD and UC from the CR and the ER,respectively.The incidences and time trends of CD and UC were not significantly different between regions.In the ER,a significantly higher percentage of children with CD presented with abdominal pain(P<0.001),blood in stools(P=0.048),stricturing or penetrating disease(P=0.029),higher erythrocyte sedimentation rate(P<0.001),higher C-reactive protein(P<0.001),higher anemia(P=0.017),and lower albumin level(P=0.014).For children with UC from the ER,a significantly higher percentage presented with anemia(P=0.006)and a lower percentage with pancolitis(P<0.001).CONCLUSION The most important finding is the identification of significantly more severe presentation of CD in the ER of the Kingdom of Saudi Arabia.Prospective studies are needed to explain such variations.

100例后天因素所致儿童OSAHS变应原检测结果分析

目的 通过统计解放军总医院第四医学中心耳鼻咽喉头颈外科病区收治后天因素所致儿童阻塞性睡眠呼吸暂停低通气综合症(obstructive sleep apnea hypopnea syndrome, OSAHS)变应原及IgE检测结果,探究OSAHS儿童过敏情况。方法 选取2021年2月至2021年11月在解放军总医院第四医学中心耳鼻咽喉头颈外科因扁桃体伴腺样体肥大导致3~12岁儿童OSAHS而行扁桃体伴腺样体切除手术的100例完整临床资料。采用免疫印迹法,共检测吸入性变应原10种,食入性变应原9种,血清总IgE 1组。分析OSAHS儿童变应原和过敏情况。结果 吸入性变应原阳性率最高的种类依次为:真菌、猫毛、屋尘、夏秋花粉、户尘螨、早晚春花粉。食入性变应原阳性率种类依次为:牛奶、腰果、鸡蛋白、苋及虾。过敏原种类阳性1项有37人次,同时合并2项有20人次,同时合并3项及以上有11人次,总过敏原阳性人数为68人次,总阳性率为68%。吸入性及食入性过敏原阳性率由高到低前六位种类依次为:牛奶、猫毛皮屑、真菌、腰果、夏秋花粉组、屋尘。不同年龄组及不同睡眠呼吸暂停阻塞程度患儿其变应原阳性率统计中3~6岁年龄组儿童过敏源阳性率最高,其次为6~9岁年龄组,9~12岁年龄组阳性率最低。不同睡眠呼吸暂停阻塞程度患儿过敏原阳性率与其阻塞程度呈正相关。结论 后天因素导致的儿童OSAHS与过敏原刺激相关,且患儿发病年龄越小,过敏易感程度越高,3~6岁年龄组儿童过敏源阳性率最高,儿童OSAHS睡眠呼吸阻塞程度与过敏仍需进一步大样本统计分析。

英夫利西单抗治疗儿童肠白塞病的疗效分析

目的分析英夫利西单抗治疗儿童肠白塞病的疗效。方法回顾性分析首都医科大学附属北京儿童医院风湿科2017年1月至2020年12月收治的10例肠白塞病患儿的临床资料,均接受英夫利西单抗治疗>3次。探讨肠白塞病患儿消化道及其他系统受累情况、激素及免疫抑制剂治疗情况、英夫利西单抗应用情况以及随访情况等。结果男性7例,女性3例,起病年龄为(8.18±3.66)岁,病程中位时间为3(1~72)个月。10例患儿均有消化道症状,其中腹泻6例、腹痛5例、血便3例、呕吐1例、腹胀1例,3例肠穿孔。消化道超声和内镜检查提示回盲部溃疡最常见。3例合并血管受累。英夫利西单抗的剂量3~5 mg/(kg·次)。英夫利西单抗治疗时间为(20.9±9.5)个月。10例患儿随访中位时间为17.5(14~54)个月。9例患儿予甲泼尼龙冲击治疗。泼尼松初始剂量为1.6~2.0 mg/(kg·d)。8例使用甲氨蝶呤,5例使用环孢素A,5例使用沙利度胺,1例使用吗替麦考酚酯。治疗后所有患儿均无临床症状,炎症指标及消化道超声无异常,病情稳定。6例停用英夫利西单抗。英夫利西单抗输注过程中无过敏反应及活动性感染。结论英夫利西单抗治疗对儿童肠白塞病是一种耐受性良好且有效的治疗方法。

B细胞活化因子与小儿自身免疫性脑炎严重程度的相关性分析

目的分析B细胞活化因子(BAFF)与小儿自身免疫性脑炎严重程度的相关性,为临床诊治小儿自身免疫性脑炎提供思路。方法该研究采用病例-对照的研究方法,将该院2020年1月至2022年6月收治的45例自身免疫性脑炎患儿作为病例组,另将该院同期收治的45例病毒性脑炎患儿作为对照组;查阅两组患儿电子病历档案,收集性别、年龄、临床特征、脑脊液BAFF、血清BAFF等临床资料;应用改良Rankin量表(mRS)评估自身免疫性脑炎患儿的病情严重程度,比较轻度组、中重度组患儿实验室资料及影像学资料,分析BAFF与小儿自身免疫性脑炎严重程度的关系。结果病例组临床特征为精神行为异常、认知障碍、癫痫发作、语言障碍、意识障碍占比高于对照组,临床特征为发热、恶心占比低于对照组,差异有统计学意义(P<0.05);病例组脑脊液BAFF及血清BAFF水平低于对照组,差异有统计学意义(P<0.05);病例组与对照组其他资料对比,差异无统计学意义(P>0.05);45例自身免疫性脑炎患儿经评估,病情程度为轻度28例,中重度17例;轻度组白细胞计数、中性粒细胞计数、mRS评分低于中重度组,白蛋白、脑脊液BAFF及血清BAFF水平高于中重度组,差异有统计学意义(P<0.05);其他不同病情程度患儿实验室资料及影像学资料对比,差异无统计学意义(P>0.05);经Point-biserial相关性分析显示,脑脊液BAFF及血清BAFF与小儿自身免疫性脑炎严重程度均呈负相关(r=-0.524、-0.562,P<0.05);经Pearson相关性分析显示,自身免疫性脑炎患儿脑脊液BAFF与血清BAFF呈正相关(r=0.437,P<0.05);绘制受试者工作特征曲线,结果显示,脑脊液BAFF、血清BAFF预测小儿自身免疫性脑炎严重程度的曲线下面积均>0.7,均具有良好的预测价值。结论BAFF与小儿自身免疫性脑炎严重程度密切相关,且对预测小儿自身免疫性脑炎严重程度具有良好的价值。