• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.2
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• pp.152-161
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• 2019

Purpose: We examined the prevalence of anemia, annual screening for anemia, and treatment of anemia with iron among children with inflammatory bowel disease (IBD). Methods: A retrospective study of U.S. pediatric patients with IBD was performed in the MarketScan commercial claims database from 2010-2014. Children (ages 1-21) with at least two inpatient or outpatient encounters for IBD who had available lab and pharmacy data were included in the cohort. Anemia was defined using World Health Organization criteria. We used logistic regression to determine differences in screening, incident anemia, and treatment based on age at first IBD encounter and sex. Results: The cohort (n=2,446) included 1,560 Crohn's disease (CD) and 886 ulcerative colitis (UC). Approximately, 85% of CD and 81% of UC were screened for anemia. Among those screened, 51% with CD and 43% with UC had anemia. Only 24% of anemia patients with CD and 20% with UC were tested for iron deficiency; 85% were iron deficient. Intravenous (IV) iron was used to treat 4% of CD and 4% UC patients overall and 8% of those with anemia. Conclusion: At least 80% of children with IBD were screened for anemia, although most did not receive follow-up tests for iron deficiency. The 43%-50% prevalence of anemia was consistent with prior studies. Under-treatment with IV iron points to a potential target for quality improvement.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.2
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• pp.154-163
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• 2021

Purpose: In children overlap of autoimmune hepatitis (AIH) and primary sclerosing cholangitis is labelled as autoimmune sclerosing cholangitis (ASC). The only prospective pediatric study showed a high prevalence of ASC by using endoscopic retrograde cholangiopancreatography. Aims of our study were to find the prevalence of ASC by using magnetic resonance cholangiography (MRC) in AIH and in non-AIH cirrhosis and to compare clinical presentation and outcome of AIH and ASC. Methods: Prospectively we did MRC in 38 children with AIH (cases) and 19 disease controls (Wilson disease). Multiple biliary strictures with proximal dilatation on MRC were taken as definitive changes of ASC. Detail clinical, laboratory parameters, liver histopathology and treatment outcome were recorded. Results: The median age of cases was 11.5 (3-18) years, 22 (57.9%) were girls and 28 (73.7%) were diagnosed as type 1 AIH. MRC was done in 11 children (28.9%) at the time of diagnosis and in 27 (71.1%) after a median follow-up of 2.5 (0.3-10) years. Abnormal MRC changes were seen in 14/38 (36.8%) of AIH and 8/19 (42.1%) of controls. However, definite changes of ASC were present in four (10.5%) children in AIH and none in controls. None of the clinical, laboratory, histological parameters and treatment response were significantly different between ASC and AIH groups. Conclusion: The prevalence of ASC in children with AIH was just 10.5%. We suggest MRC in select group with cholestatic features, inflammatory bowel disease and in those who showed poor response to immunosuppression instead of all children with AIH.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.4
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• pp.403-412
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• 2021

Purpose: This study aimed to determine the serum 25-hydroxy-vitamin D (25(OH)D) status of breastfed infants less than six months old and their mothers, and factors affecting the status. Methods: This cross-sectional study was done on breastfed, term, Filipino infants less than six months old who were seen at local health centers and clinics in an urban area. The serum 25(OH)D levels of these infants and their mothers were determined, and their demographic data, nutritional status, sun exposure behavior, and maternal vitamin D intake were analyzed for correlation using regression models. Results: Among the 131 infants, 101 (77%) had vitamin D deficiency (VDD), which was defined as having 25(OH)D levels <37.5 nmol/L, and 13 (10%) had vitamin D insufficiency (VDI), with levels >37.5-50 nmol/L. Conversely, maternal VDD with levels <50 nmol/L was seen in 31 (24%) mothers and maternal VDI with levels 50-75 nmol/L, in 63 (48%) mothers. Infant age and maternal 25(OH)D status were independent predictors of infant VDD. Infants less than three months old were found to have a six-time increased risk of infant VDD (p=0.004). Infants who had mothers with VDD had a six-time increased risk, whereas those with maternal VDI had a four-time increased risk of infant VDD (p=0.049 and p=0.020, respectively). Conclusion: Both infant and maternal VDD and VDI were seen to be highly prevalent in this tropical, urban community. Young infants and maternal VDD/VDI independently increased the risk of infant VDD, whereas lack of sun exposure of the mothers increased the risk for maternal VDI.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.3
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• pp.180-193
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• 2022

Pediatric inflammatory bowel disease (PIBD) is a multisystem disorder characterized by intestinal and extraintestinal manifestations and complications. Cerebrovascular events (CVE) are rare extraintestinal complications in patients with PIBD. Statistics show that 3.3% patients with PIBD and 1.3-6.4% adult patients with inflammatory bowel disease (IBD) experience CVE during the course of the disease. Therefore, this study aimed to review the records of children with IBD who developed CVE during the course of the disease. We retrospectively reviewed 62 cases of PIBD complicated by CVE. The mean patient age at the time of thrombotic events was 12.48±4.13 years. The incidence of ulcerative colitis was significantly higher than that of Crohn's disease (43 [70.5%] vs. 13 [21.3%] patients). Most patients (87.93%) were in the active phase of IBD at the time of CVE. The mean time interval between the onset of IBD and CVE was 20.84 weeks. Overall, 11 (26.83%) patients showed neurological symptoms of CVE at disease onset. The most frequent symptom on admission was persistent and severe headaches (67.85%). The most common site of cerebral venous thrombosis was the transverse sinuses (n=23, 53.48%). The right middle cerebral artery (n=3, 33.34%) was the predominant site of cerebral arterial infarction. Overall, 41 (69.49%) patients who were mostly administered unfractionated heparin or low-molecular-weight heparin (56.09%) recovered completely. Patients with IBD are at a risk of thromboembolism. CVE may be the most common type of thromboembolism. Based on these findings, the most common risk factor for CVE is IBD flares. In patients with CVE, anticoagulant therapy with heparin, followed by warfarin, is necessary.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.1
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• pp.23-33
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• 2023

Purpose: The incidence and prevalence of inflammatory bowel disease (IBD) are increasing along with an increasing number of patients with comorbid conditions like psychiatric and behavioral disorders, which are independent predictors of quality of life. Methods: Non-overlapping years (2003-2016) of National Inpatient Sample and Kids Inpatient Database were analyzed to include all IBD-related hospitalizations of patients less than 21 years of age. Patients were analyzed for a concomitant diagnosis of psychiatric/ behavioral disorders and were compared with IBD patients without psychiatric/behavioral disorder diagnoses for outcome variables: IBD severity, length of stay and inflation-adjusted hospitalization charges. Results: Total of 161,294 IBD-related hospitalizations were analyzed and the overall prevalence rate of any psychiatric and behavioral disorders was 15.7%. Prevalence rate increased from 11.3% (2003) to 20.6% (2016), p<0.001. Depression, substance use, and anxiety were the predominant psychiatric disorders. Regression analysis showed patients with severe IBD (odds ratio [OR], 1.57; confidence interval [CI], 1.47-1.67; p<0.001) and intermediate IBD (OR, 1.14; CI, 1.10-1.28, p<0.001) had increased risk of associated psychiatric and behavioral disorders than patients with a low severity IBD. Multivariate analysis showed that psychiatric and behavioral disorders had 1.17 (CI, 1.07-1.28; p<0.001) mean additional days of hospitalization and incurred additional $8473 (CI, 7,520-9,425; p<0.001) of mean hospitalization charges, independent of IBD severity. Conclusion: Prevalence of psychiatric and behavioral disorders in hospitalized pediatric IBD patients has been significantly increasing over the last two decades, and these disorders were independently associated with prolonged hospital stay, and higher total hospitalization charges.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.1
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• pp.50-57
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• 2023

Purpose: The purpose of this study was to determine the pattern of human leukocyte antigen (HLA)-DQ genotype in children diagnosed with celiac disease (CD) (biopsy proven), and to compare this with a control group; and secondarily, to correlate HLA genotypes with clinical profiles of CD. Methods: This cross-sectional comparative observational study included 26 controls and 52 patients diagnosed with CD who presented at Sir Padampat Mother and Child Health Institute, Jaipur, from May, 2017 to October, 2018. HLA DQ genotype was assessed for each patients and correlated with clinical profiles. Results: HLA DQ2/DQ8 genotypes were significantly more common in CD (present in 100.0% cases) than in controls (23.1%) in Northern India (Rajasthan). When HLA DQ2.5 and DQ8 were present together, individuals had significantly more atypical presentations and severe findings on duodenal biopsy. Similarly, patients with the HLA DQ 2.5 genotype were also predisposed to more severe endoscopic findings, while HLA DQ2.2 predisposed them to less severe biopsy findings. HLA DQ8 was significantly associated with later age at diagnosis (>5 years) and shorter stature. The highest HLA DQ relative risk (RR) for CD development was associated with HLA DQ2.5 and DQ2.2 in combination, followed by HLA DQ2.5 and DQ8 in combination, while HLA DQx.5 and HLA DQ2.2 together had the lowest risk. Conclusion: HLA DQ2/DQ8 genotypes are strongly associated with pediatric CD patients in northern India. These genotypes and their combinations may be associated with different clinical presentations of CD, and may help predict severity of CD.

• Journal of Chest Surgery
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• v.57 no.1
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• pp.79-86
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• 2024

Background: This study investigated the surgical outcomes associated with coronary artery fistulas (CAFs) in children. Methods: We retrospectively reviewed the medical records of 23 pediatric patients who underwent surgical closure of CAFs between 1995 and 2021. At presentation, 7 patients (30.4%) exhibited symptoms. Associated cardiac anomalies were present in 8 patients. Fourteen fistulas originated from the right coronary artery and 9 from the left. The most common drainage site was the right ventricle, followed by the right atrium and the left ventricle. The median follow-up duration was 9.3 years (range, 0.1-25.6 years) Results: The median age and body weight at repair were 3.1 years (range, 0-13.4 years) and 14.4 kg (range, 3.1-42.2 kg), respectively. Cardiopulmonary bypass was used in 17 cases (73.9%), while cardioplegic arrest was employed in 14 (60.9%). Epicardial CAF ligation was utilized in 10 patients (43.5%), the transcoronary approach in 9 (39.1%), the endocardial approach in 2 (8.7%), and other methods in 2 patients (8.7%). The application of cardioplegic arrest during repair did not significantly impact the duration of postoperative intensive care unit stay or overall hospital stay. One in-hospital death and 1 late death were recorded. The overall survival rate was 95.7% at 10 years and 83.7% at 15 years. A residual fistula was detected in 1 patient. During the follow-up period, no surviving patient experienced cardiovascular symptoms or coronary events. Conclusion: Surgical repair of CAF can be performed safely with or without cardioplegic arrest, and it is associated with a favorable prognosis in children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.27 no.1
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• pp.37-42
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• 2024

Purpose: Limited data exist regarding outcome and morbidity associated with portosystemic shunts in the pediatric transplant population. Our study assesses the outcomes of pediatric patients who underwent a portosystemic shunt procedure, both with and without liver transplantation (LT). Methods: This study retrospectively reviewed the medical records of pediatric patients aged 0-19 years who underwent shunt placement between 2003 and 2017 at a tertiary care center. The analysis included cases of shunt placement with or without LT. Results: A total of 13 pediatric patients were included in the study with median age of 8.8 years. Among the cases, 11 out of 13 (84.6%) underwent splenorenal shunt, 1 (7.7%) underwent a mesocaval shunt, and another 1 (7.7%) underwent a Modified Rex (mesoportal) shunt. Additionally, 5 out of 13 (38.5%) patients had LT, with 4 out of 5 (80.0%) receiving the transplant before shunt placement, and 1 out of 5 (20.0%) receiving it after shunt placement. Gastrointestinal bleeding resulting from portal hypertension was the indication in all cases. A total of 10 complications were reported in 5 patients; the most common complication was anemia in 3 (23.1%) patients. At the most recent follow-up visit, the shunts were functional without encephalopathy, and no deaths were reported. Conclusion: Shunt placement plays a crucial role in the management of patients with portal hypertension. Our study demonstrates favorable long-term outcomes in pediatric patients who underwent shunt placement. Long term shunt outcomes were similar and unremarkable in patients with LT and without LT.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.4
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• pp.357-365
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• 2021

Purpose: A change in diagnosis from ulcerative colitis (UC) to Crohn's disease (CD) has been reported in pediatric inflammatory bowel disease; however, only a few clinical characteristics and predictors of this diagnostic change have been reported. We aimed to describe the clinical characteristics of patients with UC who underwent a change in diagnosis to CD and identify variables associated with the change. Methods: The medical records of pediatric patients with UC who were followed up at the National Center for Child Health and Development between 2006 and 2019 were retrospectively reviewed. Clinical data on disease phenotype, laboratory parameters, endoscopic findings, and treatment of patients whose diagnosis changed to CD (cCD) were compared to those of patients whose diagnosis remained UC (rUC). Results: Among the 111 patients initially diagnosed with UC, 11 (9.9%) patients were subsequently diagnosed with CD during follow-up. There was no significant difference between the cCD and rUC groups in terms of sex, age at initial diagnosis, and the extent and severity of disease at initial diagnosis. Albumin and hemoglobin levels were significantly lower in the cCD group than in the rUC group. The proportion of patients who required biologics was significantly higher in the cCD group than in the rUC group (p<0.05). Conclusion: Approximately 10% children initially diagnosed with UC were subsequently diagnosed with CD. Hypoalbuminemia and anemia at initial diagnosis and use of biologics could be predictors of this diagnostic change.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.1
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• pp.52-60
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• 2022

Purpose: Colonoscopy is considered the most reliable method for the diagnosis of juvenile polyps. However, colonoscopic screening is an invasive and expensive procedure. Fecal calprotectin (FCP), a marker of intestinal inflammation, has been shown to be elevated in patients with polyps. Therefore, this study aimed to evaluate FCP as a screening biomarker for the diagnosis of juvenile polyps. Methods: This cross-sectional, observational study was conducted at the Pediatric Gastroenterology and Nutrition Department, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. For children with polyps, colonoscopic polypectomy and histopathology were performed. FCP levels were analyzed before and 4 weeks after polypectomy in all patients. Information was recorded in a datasheet and analyzed using the computer-based program SPSS. Results: The age of the children was between 2.5 and 12 years. Approximately 93% of the polyps were found in the rectosigmoid region. Children with juvenile polyps had elevated levels of FCP before polypectomy that subsequently normalized after polypectomy. The mean FCP levels before and after polypectomy were 277±247 ㎍/g (range, 80-1,000 ㎍/g) and 48.57±38.23 ㎍/g (range, 29-140 ㎍/g) (p<0.001), respectively. The FCP levels were significantly higher in patients with multiple polyps than in those with single polyps. Moreover, mean FCP levels in patients with single and multiple polyps were 207.6±172.4 ㎍/g and 515.4±320.5 ㎍/g (p<0.001), respectively. Conclusion: Colonic juvenile polyps were found to be associated with elevated levels of FCP that normalized after polypectomy. Therefore, FCP may be recommended as a noninvasive screening biomarker for diagnosis of colonic juvenile polyps.