Purpose: Low bone mineral density (BMD) is a complication in children with inflammatory bowel disease (IBD). There are limited data evaluating dual-energy x-ray absorptiometry (DXA) as a screening tool for low BMD in children with IBD. We performed a single site retrospective analysis of DXA use.
Methods: Children aged 5-18 years with IBD diagnosed between 2013 to 2017 at the Royal Children's Hospital, Australia, were included. Patient demographics, measures of disease activity, DXA scores, and factors related to BMD were collected.
Results: Over a median follow up of 5.1 (4-6.4) years, 72/239 (30.1%) children underwent DXA, and 28/239 (11.7%) children had a second DXA. Our DXA practice differed to consensus guidelines regarding initial screening based on height and/or body mass index (BMI) z-score (8/17 [47.1%]), and repeat surveillance (13/42 [31.0%]). Children had a median lumbar spine (LS) z-score -0.80 (-1.65-0.075). Children with LS z-score≤-2.0 (n=14) had lower weight (6.57 [1.78-23.7] vs. 51.1 [26.5-68.7], p=0.0002) and height centiles (3.62 [1.17-17.1] vs. 42 [16.9-67.1], p=0.0001), and higher faecal calprotectin (FCP) (3041 [1182-4192] vs. 585 [139-2419], p=0.009) compared to children with LS z-score>-2.0. No fractures were reported. Of 28 children who underwent a second DXA 1.6 (1.1-2.2) years following initial DXA, no significant change in z-scores occurred.
Conclusion: Children with IBD had low BMD. In addition to height centile and weight centile, FCP was associated with lower BMD, and should be considered in DXA screening guidelines. Greater clinician awareness of DXA consensus guidelines is required. Future prospective studies are required.
Purpose: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a congenital anomaly that can cause frequent digestive and nutritional problems, even after repair. The most common complication is anastomotic stricture, for which reoperation or balloon dilatation is performed. This study aimed to evaluate the postoperative complications of EA and the role of endoscopic balloon dilatation (EBD) in cases of anastomotic stricture.
Methods: We retrospectively analyzed patients diagnosed with EA with or without TEF between January 2000 and February 2021. Patients' baseline characteristics, associated anomalies, and postoperative complications were reviewed.
Results: Among 26 patients, 14 (53.8%) were male, 12 (46.2%) had coexisting anomalies, and the median follow-up was 6.1 years (range, 1.2-15.7 years). In univariate analysis, prematurity, low birth weight, and long-gap EA were associated with postoperative complications in 12 (46.2%) patients. Among the 10 (38.5%) patients with anastomotic stricture, nine (90.0%) required EBD. Regarding the first EBD, it was performed at a median of 3.3 months (range, 1.2-7.6 months) post-repair, while the average patient weight was 4.6 kg. The mean diameter ranged from 3.3 to 9.1 mm without major complications. In univariate analysis, long-gap EA alone was significantly associated with EBD.
Conclusion: Approximately half of the patients experienced complications after EA repair. In particular, patients with a long-gap EA had a significantly increased risk of complications, such as anastomotic strictures. EBD can be safely used, even in infants.
Purpose: Liver transplantation (LT) is the only curative treatment for acute liver failure (ALF) and acute-on-chronic liver failure (ACLF). In high-volume therapeutic plasma exchange (HV-TPE), extracorporeal liver support filters accumulate toxins and improve the coagulation factor by replacing them. In this study, we aimed to evaluate the effectiveness of HV-TPE in pediatric patients with ALF and ACLF.
Methods: We reviewed the records of children waiting for LT at Severance Hospital who underwent HV-TPE between 2017 and 2021. Aspartate aminotransferase (AST), alanine aminotransferase (ALT), total and direct bilirubin (TB and DB), gamma-glutamyl transferase (GGT), ammonia, and coagulation parameter-international normalized ratio (INR) were all measured before and after HV-TPE to analyze the liver function. The statistical analysis was performed using IBM SPSS Statistics for Windows, version 26.0 (IBM Co., Armonk, NY, USA).
Results: Nine patients underwent HV-TPE with standard medical therapy while waiting for LT. One had neonatal hemochromatosis, four had biliary atresia, and the other four had ALF of unknown etiology. Significant decreases in AST, ALT, TB, DB, GGT, and INR were noted after performing HV-TPE (930.38-331.75 IU/L, 282.62-63.00 IU/L, 11.75-5.59 mg/dL, 8.10-3.66 mg/dL, 205.62-51.75 IU/L, and 3.57-1.50, respectively, p<0.05). All patients underwent LT, and two expired due to acute complications.
Conclusion: HV-TPE could remove accumulated toxins and improve coagulation. Therefore, we conclude that HV-TPE can be regarded as a representative bridging therapy before LT.
Purpose: We compared the health-related quality of life (HRQOL) of children and adolescents with functional abdominal pain disorders (FAPDs) and organic abdominal pain disorders (ORGDs).
Methods: This was a single-center, cross-sectional, observational study. The PedsQL 4.0 generic cores scales parent proxy-report was administered to parents/caregivers of 130 and 56 pediatric patients with FAPDs and ORGDs respectively on their first visit. The self-reported pain intensity in the patients was assessed using a visual analog scale (VAS) and facial affective scale (FAS).
Results: Irritable bowel syndrome was the most prevalent FAPDs, and the most prevalent ORGDs were reflux esophagitis (41.1%) and gastritis associated with Helicobacter pylori (21.4%). There was no difference in HRQOL among patients diagnosed with ORGDs and FAPDs (p>0.05). Patients with ORGDs and FAPDs had lower HRQOL Scale scores than healthy Brazilian and American children's references, with a high proportion of children at risk for impaired HRQOL (p<0.0001). There was no difference in the VAS and the FAS scores between the ORGDs and the FAPDs. FAPDs had a higher prevalence of girls' and couples' disagreement (p<0.02), although poor school performance (p<0.0007) and bullying (p<0.01) were higher in patients with ORGD.
Conclusion: This study revealed that there was a difference in impaired HRQOL between patients with ORGDs and FAPDs. Thus, considering the high prevalence of chronic abdominal pain in children, a well-founded treatment plan is necessary for a multidisciplinary cognitive-behavioral Pain management program.
Purpose: The survival rate of pediatric patients undergoing liver transplantation has increased considerably. Despite this, the period after transplantation is still complex and poses several challenges to the recipient's family, which is responsible for care management. Recently, more attention has been paid to the impact of this complex procedure on the quality of life of caregivers. Hence, this study is aimed at assessing the quality of life of caregivers of patients who have undergone liver transplantation and the aspects that influence it.
Methods: This was an observational and cross-sectional study. From November 2020 to January 2021, short-form-36 questionnaires and additional questions were given to the main caregivers of children and adolescents who underwent pediatric liver transplantation.
Results: Thirty-eight questionnaires were completed and the results revealed a lower quality of life in comparison to Brazilian standards, primarily in the mental domains (41.8±14.1 vs. 51.1±2.8; p<0.001). It did not show a significant association with socioeconomic or transplant-related factors, but it did show a negative impact on parents' perception of the child's health. Parents who reported worse health status for their children had a lower mental quality of life (44.1±13.8 vs. 33.3±12.6; p<0.05).
Conclusion: The caregivers of transplanted children have a lower quality of life than those of the local population. Psychological assistance should be routinely provided to parents for long-term follow-up to mitigate potential negative effects on the transplanted child's care.
Purpose: Golimumab (GLM) is an anti-tumor necrosis factor (TNF)-α antibody preparation known to be less immunogenic than infliximab (IFX) or adalimumab. Few reports on GLM in pediatric patients with ulcerative colitis (UC) are available. This study aimed to review the long-term durability and safety of GLM in a pediatric center.
Methods: The medical records of 17 pediatric patients (eight boys and nine girls) who received GLM at the National Center for Child Health and Development were retrospectively reviewed.
Results: The median age at GLM initiation was 13.9 (interquartile range 12.0-16.3) years. Fourteen patients had pancolitis, and 11 had severe disease (pediatric ulcerative colitis activity index ≥65). Ten patients were biologic-naïve, and 50% achieved corticosteroid-free remission at week 54. Two patients discontinued prior anti-TNF-α agents because of adverse events during remission. Both showed responses to GLM without unfavorable events through week 54. However, the efficacy of GLM in patients who showed primary nonresponse or loss of response to IFX was limited. Four of the five patients showed non-response at week 54. Patients with severe disease had significantly lower corticosteroid-free remission rate at week 54 than those without severe disease. No severe adverse events were observed during the study period.
Conclusion: GLM appears to be safe and useful for pediatric patients with UC. Patients with mild to moderate disease who responded to but had some adverse events with prior biologics may be good candidates for GLM. Its safety and low immunogenicity profile serve as favorable options for selected children with UC.
Congenital diarrheal disorders (CDDs) with genetic etiology are uncommon hereditary intestinal diseases characterized by chronic, life-threatening, intractable watery diarrhea that starts in infancy. CDDs can be mechanistically divided into osmotic and secretory diarrhea. Congenital tufting enteropathy (CTE), also known as intestinal epithelial dysplasia, is a type of secretory CDD. CTE is a rare autosomal recessive enteropathy that presents with intractable neonatal-onset diarrhea, intestinal failure, severe malnutrition, and parenteral nutrition dependence. Villous atrophy of the intestinal epithelium, crypt hyperplasia, and irregularity of surface enterocytes are the specific pathological findings of CTE. The small intestine and occasionally the colonic mucosa include focal epithelial tufts. In 2008, Sivagnanam et al. discovered that mutations in the epithelial cell adhesion molecule (EpCAM, MIM# 185535) were the genetic cause of CTE (MIM# 613217). More than a hundred mutations have been reported to date. Furthermore, mutations in the serine peptidase inhibitor Kunitz type 2 (SPINT2, MIM# 605124) have been linked to syndromic CTE. In this study, we report the case of a 17-month-old male infant with congenital diarrhea. Despite extensive etiological workup, no etiology could be established before admission to our center. The patient died 15 hours after being admitted to our center in a metabolically decompensated state, probably due to a delay in admission and diagnosis. Molecular autopsy with exome sequencing revealed a previously reported homozygous missense variant, c.757G>A, in EpCAM, which was confirmed by histopathological examination.
Purpose: Recently, great interest has been focused on dietary fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAPs) for the treatment of functional abdominal pain (FAP). Several meta-analyses, including those on the adult population, have been published, potentiating its role. However, pediatric studies are scarce. We aimed to evaluate the effect of a low-FODMAP diet on the severity of FAP in children.
Methods: This clinical trial included 50 patients aged 3-18 years with irritable bowel syndrome and FAP that were not otherwise specified. The patients were instructed to receive a low FODMAP diet guided by a dietitian. The primary outcome was the percentage of responders after 2 months of dietary intervention compared with baseline. Other outcomes included changes in stool consistency and quality of life (QoL) scores using the KIDSCREEN-10 questionnaire, and weight-for-age z-scores.
Results: After the dietary intervention, 74% of patients showed more than 30% lower pain intensity, as examined using the Wong-Baker Faces pain rating scale. Their QoL significantly improved, and patients have gained weight.
Conclusion: A low FODMAP diet can improve pain intensity and QoL among children with functional abdominal pain, with no detrimental effects on body weight.
Purpose: Eating disorders often result in somatic complications, including cardiac abnormalities. Cardiac abnormalities may involve any part of the heart, including the cardiac conduction system, and can lead to sudden cardiac death. The current study aimed to evaluate the incidence of cardiac complications in pediatric patients with eating disorders and their associated factors.
Methods: We retrospectively analyzed patients aged 10-18 years who were diagnosed with DSM-V (Diagnostic and Statistical Manual of Mental Disorder-V) eating disorders and underwent electrocardiography (ECG) and/or echocardiography between January 2015 and May 2020.
Results: In total, 127 patients were included, of whom 113 (89.0%) were female. The median body mass index (BMI) was 15.05±3.69 kg/m2. Overall, 74 patients (58.3%) had ECG abnormalities, with sinus bradycardia being the most common abnormality (91.9%). Patients with ECG abnormalities had significantly lower BMI (14.35±2.78 kg/m2 vs. 16.06± 4.55 kg/m2, p<0.001) than patients without ECG abnormalities, as well as lower phosphorus and higher cholesterol levels. Among the 46 patients who underwent echocardiographic evaluation, 23 (50.0%) had echocardiographic abnormalities, with pericardial effusion being the most common (60.9%). The median left ventricular mass (LVM) and ejection fraction were 67.97±21.25 g and 66.91±28.76%, respectively. LVM and BMI showed a positive correlation (r=0.604, p<0.001). After weight gain, the amount of pericardial effusion was reduced in 3 patients, and 30 patients presented with normal ECG findings.
Conclusion: Cardiac abnormalities are relatively frequent in patients with eating disorders. Physicians should focus on this somatic complication and careful monitoring is required.
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