Department Of Pediatric Gastroenterology Research Articles

Next-generation sequencing in diagnosis of monogenic cholestatic liver disorders: A single-center experience

Introduction Cholestasis in childhood is a rare clinical condition, yet a definitive diagnosis is crucial for initiating treatment of these curable diseases and preventing related morbidity and mortality. The most common cause of infant cholestasis is biliary atresia (25-40%), followed by monogenic cholestatic diseases (25%), metabolic diseases (20%), and cryptogenic cholestasis. This study focuses on assessing the clinical utility of next-generation sequencing (NGS) panels, including clinical exome sequencing and whole exome sequencing, in diagnosing cholestatic diseases when the etiology cannot be elucidated through conventional methods. Materials and Methods We conducted a retrospective examination of pediatric patients who sought care at a single-center pediatric gastroenterology department between August 2020 and March 2022, and were diagnosed with cholestasis. A total of 36 patients underwent a thorough investigation to rule out infectious, toxic, metabolic, structural, chromosomal, and endocrine causes. Patients whose diagnoses were established through traditional investigations were excluded from the study. The remaining 14 patients underwent either whole exome sequencing or targeted next-generation sequencing methods. Results A definitive diagnosis was achieved for 12 patients, while two patients remained undiagnosed despite comprehensive genetic examinations. The most commonly encountered diseases in this cohort were progressive familial intrahepatic cholestasis, linked to mutations in the ABCB11, ATP8B1, and TJP2 genes, as well as Dubin-Johnson syndrome associated with ABCC2 mutations. Next-generation sequencing demonstrated a diagnostic accuracy of 85.7% in patients for whom a diagnosis could not be established through extensive traditional workup. Conclusion Next-generation sequencing emerges as a valuable diagnostic tool in cases of cholestasis where traditional methods fall short in providing a definitive diagnosis. Moreover, our study unveiled three previously undocumented variants in the ABCB11 [c.1165G>C; p.(Ala389Pro) and c.783+1G>A] and ABCC2 [c.4246_4247del; p.(Lys1416ValfsTer46)] genes.

Growth Status of Bangladeshi Children in Functional Constipation: Experience From A Tertiary Care Hospital

Background: Constipation is one of the common complaints of children with long-term hazardous consequences. It is a frequent cause of hospital visit in both primary and specialized care which needs proper evaluation and management. Recently in few studies, impairment of growth status has been reported as a result of functional constipation. But the relation is not well evaluated, especially in the pediatric population of our country. Aim: To assess the growth status of Bangladeshi children in functional constipation. Methods: This analytical study was conducted at the department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka. A total 150 children aged 2-18 years were enrolled in this study. Children were divided into two groups, 75 having functional constipation (FC) and 75 healthy children with no constipation. Samples were collected randomly from pediatric out-patient department of BSMMU with consent of parents and child’s approval. Diagnosis of functional constipation was made by Rome IV criteria. Children with any red flag sign, chronic disease and on treatment of constipation were excluded from the study. Growth status (weight, height & BMI) was evaluated by using growth charts. Data collected in semi-structured questionnaire and analyzed by SPSS 23.0. Results: We evaluated 75 constipated children with the mean age of 7.61±3.50 years and 75 healthy children with the mean age of 7.40±3.88 years. The mean weight of children with functional constipation was 22.8±10.01 kg, mean height 117.7 ±18.4 cm and BMI 15.6±2.3 kg/m2. The mean weight of children with no constipation was 28.6± 13.72 kg, mean height 124.49±23.3 cm and BMI 18.11±2.9 kg/m2,demonstrated significant difference between two sets. In functional constipation group, on weight for age and height for age chart, 28% and 24% children were below the 3rd centile respectively and 18.6% children had BMI below 5th centile. All these parameters (weight, height, BMI) were statistically significant in compare to children without constipation and p value is <.05. Presenting features of functional constipation were anorexia (64%), abdominal pain (73.3%), hard stool (84%), blood mixed hard stool (13.3%) and nausea (34%). Conclusion: Growth impairment occurs in children with functional constipation in comparison to children without constipation. Children aged 2 to 18 years with functional constipation may have decelerated weight, height and BMI growth. And this observation needs further large-scale multicenter study for ensuring optimal growth of children with constipation. J Shaheed Suhrawardy Med Coll 2023; 15(1): 73-77

Comparison of different diagnostic methods in infants for differentiating idiopathic neonatal hepatitis from biliary atresia

Background: Biliary atresia (BA) and idiopathic neonatal hepatitis (INH) are two most common etiologies of neonatal cholestatic jaundice. It is important to distinguish INH from BA biliary atresia in an infant. The study was conducted to compare of different diagnostic methods in infants in differentiating INH and BA. Methods: This cross-sectional study was conducted at the Department of Pediatric Gastroenterology & Nutrition, BSMMU during the period from July 2018 to June 2019 among infants with neonatal cholestasis. Total 50 cholestatitic cases were selected through purposive sampling. A thorough history and physical examination were done and liver enzymes were studied. Abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy were done in all cases. Clinical parameter and investigational sensitivity, specificity, and diagnostic precision were calculated. To diagnose BA and INH, liver biopsy was considered the gold standard. Results: Among the participants, 72% were diagnosed as BA and 28% were INH. More than half of patients were male. There exists significant difference of birth weight and term baby between BA and INH. Presence of persistent pale coloured stool was more commonly seen in patients with BA. Diagnostic accuracy of persistent pale coloured stool, birth weight (normal) and term baby were 94%, 92% & 88% respectively. Sensitivity and specificity of Gammaglutamyl transpeptidase (GGT) in differentiating cases with BA and INH with cut point of ≥430.0 U/L were 86.1% and 85.7%, respectively. Hepatobiliary scintigraphy is found to have a better sensitivity than ultrasonography. GGT had diagnostic accuracy 86%, hepatobiliary scintigraphy had diagnostic accuracy 88% & USG had diagnostic accuracy 80% for BA and INH. Conclusion: Among the studied clinical parameters persistent pale coloured stool was most accurate to suggest the presence of BA. Serum level of GGT at cut-off value ≥ 430 U/L may be used as a reliable laboratory parameter to differentiate INH and BA. Hepatobiliary scintigraphy found to have a good sensitivity for BA. Positive result of hepatobiliary scintigraphy may be used as BA in infants with cholestasis. JOPSOM 2023; 42(1):14-20

Validity of Serum Ammonia Level for Diagnosis of Severity of Hepatic Encephalopathy in Children

Hepatic encephalopathy is a broad spectrum neuropsychiatric abnormalities of liver dysfunction. Ammonia level may correlate with the severity of liver failure. The brain is very sensitive to the toxic effects of ammonia. As a result patient may manifests with irritability, slurring of speech, reversal of sleep-awake cycle, flapping tremor, confusion, stupor or even deep coma. This study was aimed to validate the ammonia level in children with liver failure for the assessment of its severity considering hepatic encephalopathy. This cross-sectional comparative study was conducted among 64 children aged 1-15 years of both sexes (study subjects) diagnosed as acute or acute on chronic liver failure in the Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh during the period of November 2017 to September 2019. The subject were divided into two groups for the comparison of ammonia level to assess the severity in contrast to hepatic encephalopathy. In the first group 32 were liver failure with encephalopathy and in the second group 32 were liver failure without encephalopathy. Hepatic encephalopathy was diagnosed on the basis of West Haven Criteria. The analysis was done by the Receiver Operating Characteristic Curve with SPSS-20. Among the 64 patients female were 45% whereas male patients were 55%, male female ratio was 1.2: 1. Regarding etiology, Wilson disease was the most common cause and it was nearly two-third (65.6%) of children, cryptogenic cirrhosis was 10%, Hepatitis A was 9.4%, Autoimmune Hepatitis (AIH) was 3.10%, Hepatitis E, Hepatitis B, Hepatitis C, biliary atresia and lipid storage were 1.60% respectively. This study showed that, ammonia of ≥71 µmol/L is an indicator for presence of hepatic encephalopathy in children. The analysis by the Receiver Operating Characteristic Curve showed area under the curve (AUC) is 0.86 with upper bound 0.96 and lower bound is 0.77. It was observed that about half (48.4%) of the children had positive blood ammonia level (≥71.0 umol/L) and among the children of positive blood ammonia level most of them (80.65%) had hepatic encephalopathy and 19.35% had no encephalopathy. More than half (51.6%) children had negative (<71.0 umol/L) blood ammonia level, among them 21.21% children had encephalopathy and 78.79% patients had no encephalopathy. Sensitivity of blood ammonia was found 78.1%, specificity 81.2%, positive predictive value 80.6%, negative predictive value 78.8% and accuracy 79.7%. In conclusion, high level of ammonia is found with higher grade of encephalopathy and hyperammonia is also found in liver failure without encephalopathy. Bangladesh Med J. 2023 May; 52(2): 1-5

Clinical Findings, Bacterial Agents, and Antibiotic Resistance in Children with Spontaneous Peritonitis in Southern Iran: An Academic Tertiary Referral Center's Experience.

Spontaneous bacterial peritonitis (SBP) is a fatal complication of ascites fluid infection. The causes of SBP in children differ from those in adults, and these bacteria are frequently resistant to antibiotics. Therefore, this study investigated the clinical findings, bacterial etiology, and antimicrobial resistance in children with SBP. This study was conducted on all new pediatric ascites patients, who were admitted to the Department of Pediatric Gastroenterology, Namazi Hospital, affiliated with Shiraz University of Medical Sciences (Shiraz, Iran) from 2021 to 2022. Required data such as demographic information, and clinical information such as complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Gram staining, blood culture by Automated Blood Culture System (BACTEC), and antibiogram of ascites fluids by disc diffusion method were all collected. Finally, the data were statistically analyzed using SPSS Software (version 26). Besides, the t test, Fisher's exact, Mann-Whitney, and Chi square tests were used for data analysis. In all tests, P≤0.05 was considered statistically significant. The present study examined 62 children with ascites of which 18 (29%) had SBP. The median (IQR) age was 2.5 (8.1) years. Thirty-four (54.8%) of the participants were girls. Abdominal pain was the most common clinical manifestation in patients (54%), and there was a significant association between abdominal pain and SBP (P=0.02). In 12 positive ascites fluid cultures, coagulase-negative staphylococci had the highest frequency (25%), followed by Escherichia coli (16.7%). Third-generation cephalosporins had a 25% sensitivity in the total positive cultures. This sensitivity was 33.3% for Gram-negative cultures and 16.6% for Gram-positive cultures. Although third-generation cephalosporins are recommended as the primary antibiotic for the empirical treatment of SBP, the present study found high bacterial resistance. Finally, empirical therapy should be tailored to each region's bacterial resistance features.

Evaluation of cardiac function in paediatric Wilson's disease patients with advanced echocardiographic modalities (strain and strain rate echocardiography).

In Wilson's disease (WD), copper accumulation in the organs and/or damage caused by oxygen free radicals occurs due to disturbances in copper excretion. In our study, we aimed to evaluate cardiac involvement with advanced echocardiographic modalities (tissue Doppler echocardiography, strain and strain-rate echocardiography). Twenty WD patients and 20 healthy children from the Pediatric Gastroenterology Department of Diyarbakır Children's Hospital were included in the study between 2022 and 2023. The mean age of the WD patients was 12.89 ± 3.79 years. Left ventricular wall thicknesses and diameters (diastolic interventricular septum thickness, diastolic left ventricular posterior wall thickness, left ventricular end-diastolic diameter), left ventricular diastolic function parameters (E, A, E/A, deceleration time) and left ventricular ejection fraction and tricuspid annular plane systolic excursion were similar and not statistically significantly different in the WD and control groups. Mitral lateral e', mitral septal e' and tricuspid lateral e' velocities were lower in the WD patients and statistically significantly different from the controls (p = 0.02, 0.04 and 0.005, respectively), as assessed by tissue Doppler echocardiography. Global longitudinal systolic strain was similar in the WD and control groups and no statistically significant difference was detected. Longitudinal early diastolic strain rate was lower in the WD patients and statistically significantly different (p = 0.002). Subclinical early diastolic dysfunction and segmental systolic dysfunction were detected in WD patients with advanced echocardiographic modalities, in addition to normal cardiac function as assessed by conventional echocardiography. Advanced echocardiographic modalities can be used in the follow up of WD patients.

Understanding the living-related pediatric liver transplantation donor's decision process: A questionnaire-based retrospective study.

To analyse the donor characteristics and understand the sociocultural and familial aspects of the decision to donate for living donor liver transplant. This is a retrospective study done in the Department of Pediatric Gastroenterology and Hepatology of a liver transplant centre. We enrolled the consenting donors of pediatric liver transplantation between January 2020 and January 2022. The study was conducted through an anonymized questionnaire which was drafted in a multiple-choice format. The questionnaire had three domains having questions pertaining to demographic details, donor characteristicsand sociofamilial aspects of the decision-making process. The study cohort consisted of 50 donors, 23 males (46%) and 27 females (54%), with the mean age being 31.8 ± 5.6 years. Parent-to-child was the most frequent relationship (n = 39, 78%). More than half of our donors had annual income <2.5lakh/annum or <3500 USD(n = 27, 54%). Twenty-six (52%) responders had >4 members in their family; 62% were residing in urban areas and 52% of the donors were graduates. The main source of information regarding the feasibility of living donor liver transplantation wasprimary physicians in 46%. Financial management was the main hurdle in 72%. Majority (74%) denied any effect on their marital relationship and 6% of donors thought that the degree of information provided to them before the transplantation regarding donation was insufficient. Our study serves as an essential tool for health professionals to provide sufficient support to the donor. Clinical outcomes and cost must be part of the discussion between caregivers, health professionalsand fund contributors.

Comparison of Different Diagnostic Methods of Helicobacter Pylori Infection in Children

Background: Helicobacter Pylori Infection (HPI) is usually acquired during childhood, without specific treatment this infection rarely resolves spontaneously. Therefore, correct diagnosis is necessary for the effective eradication and there is no single test can be regarded as the gold standard for the identification of H. pylori.Objective: To compare the sensitivity, specificity, and accuracy of the stool antigen test, serologic test, rapid urease test, and histology for the diagnosis of H. pylori infection.Materials and Methods: This cross-sectional descriptive study was conducted at the Department of Pediatric Gastroenterology &amp; Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka from April 2019 to October 2021 on 54 children aged less than 18 years suspected to be H. pylori infection, who attended the outpatient as well as admitted. In a typical data sheet, a complete clinical history, exam results, and investigation reports were documented. Results were expressed as sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy.Results: The gastro-duodenal biopsy and Rapid Urease Test (RUT) (100%) have the highest sensitivity, followed by serology (95%), and Saturation (SAT) (70%). Highest specificity was found in gastro-duodenal biopsy 88.2% followed by RUT 67.6% &amp; SAT 41.2% and only 32.4% by serology. The gastro-duodenal biopsy had the highest diagnosis accuracy (92.6%), followed by RUT (79.6%), serology (55.6%) and SAT (51.9%).Conclusion: In the study, the gastro-duodenal biopsy and rapid urease test both showed high sensitivity, specificity, and negative predictive value; as a result, the combination of these tests constituted the best alternative for making the diagnosis. Because of their high sensitivity, serological tests may be useful non-invasive screening methods. KYAMC Journal Volume: 14, No: 03, October 2023: 130-137.

Computed Tomography Assessment of Severity of Acute Pancreatitis in Bangladeshi Children.

Acute pancreatitis (AP) is common among children in Bangladesh. Its management depends mainly on risk stratification. This study aimed to assess the severity of pediatric AP using computed tomography (CT). This cross-sectional, descriptive study was conducted in pediatric patients with AP at the Department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka, Bangladesh. Altogether, 25 patients with AP were included, of whom 18 (mean age, 10.27±4.0 years) were diagnosed with mild AP, and 7 (mean age, 10.54±4.0 years) with severe AP. Abdominal pain was present in all the patients, and vomiting was present in 88% of the patients. Etiology was not determined. No significant differences in serum lipase, serum amylase, BUN, and CRP levels were observed between the mild and severe AP groups. Total and platelet counts as well as hemoglobin, hematocrit, serum creatinine, random blood sugar, and serum alanine aminotransferase levels (p>0.05) were significantly higher in the mild AP group than in the severe AP group (p=0.001). The sensitivity, specificity, positive predictive value, and negative predictive value of CT severity index (CTSI) were 71.4%, 72.2%, 50%, and 86.7%, respectively. In addition, significant differences in pancreatic appearance and necrosis were observed between the two groups on CT. CT can be used to assess the severity of AP. In the present study, the CTSI effectively assessed the severity of AP in pediatric patients.

Caroli’s Disease vs Caroli Syndrome: Clinical and Radiological Spectrum, with disease Outcome at a Teaching Hospital in Lahore

Background: Caroli disease and Caroli syndrome are part of the clinical spectrum of ductal plate (DP) malformations, also referred to, as the fibrocystic liver diseases. This article aimed to study both these conditions, in terms of clinical features and disease outcome at a teaching hospital. Objective: To describe the clinical spectrum, laboratory and imaging findings, management course and outcome of fibrocystic liver diseases in children, at the University of Child Health Sciences, Lahore, Pakistan. Study Design: Cross-sectional Study. Settings: Department of Pediatric Gastroenterology &amp; Hepatology, University of Child Health Sciences, Lahore Pakistan. Duration: January 2014 to July 2017. Methods: The study included patients with Caroli disease and Caroli Syndrome, confirmed on the basis of characteristic findings on magnetic resonance cholangiopancreatography. SPSS v.20 was used for statistical analysis. Results: The mean age of 15 patients included in the study was 6.54 ± 4.18 years. Among them, 7/15 (46.7%) had Caroli’s disease (CD), whereas 8/15 (53.3%) had Caroli’s syndrome (CS). Male to female ratio was 4:1. CS pre-dominantly affected male children (8:0), while CD affected both genders almost equally (4:3). CD commonly presented with recurrent cholangitis (6/7, 85%), while CS as portal hypertension (7/8, 87.5%). Renal involvement was found in 5/15(33.33%) patients; mostly in CS (4/5,80%) and one (6.66%) patient progressed to end stage renal disease. One patient of CD underwent lobectomy and one died of end stage liver disease. Two patients of CS were treated with shunt surgery with partial splenectomy. Six patients are doing well after endoscopic variceal band ligation and propranolol prophylaxis. Conclusion: Caroli disease and Caroli syndrome are two sides of the same coin; it is important to differentiate the two disorders, especially from management point of view. CS is more common in our set up, leading to portal hypertension, which can be treated with sclerotherapy, endoscopic band ligation or shunt surgery. CD leads to cholangitis, which is managed with antibiotics.

Etiology and Outcome of Hepatic Encephalopathy in Bangladeshi Children

Hepatic encephalopathy is one of the complications of liver failure which causes increase mortality and morbidity in children. This cross sectional type of observation study was conducted in the department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University of Bangladesh to determine the etiology and outcome of hepatic encephalopathy in Bangladeshi children. Patients between 1 to 18 years were included who fulfilled the West Heaven clinical criteria of hepatic encephalopathy. The data were collected and recorded questionnaire after IRB approval and was analyzed accordingly. Total 32 patients were included in this study; among which 20 were males and 12 were female with a mean age was 8.1±3.5 years. The most common etiologies were Wilson disease (47%), followed by cryptogenic (25%) and Hepatitis A virus (18.8%). Total 9 patients out of 32 were expired, another 8 got discharge with advice, 1 patient recovered after liver transplantation and others were improved with advice of follow up. All the patients were expired who developed hepatic encephalopathy due to Hepatitis A virus except one patient who received liver transplantation from abroad. This study highlights that hepatic encephalopathy from infective diseases as hepatitis A virus leads higher mortality rate in children. Though liver transplantation facility is not available in the developing countries, maintenance of hygiene and vaccination is the main preventive measures to reduce the mortality in children. Bangabandhu Sheikh Mujib Med. Coll. J. 2023;2(2):88-91.

Akut pankreatitli çocuklarda klinik özellikler, tanı, tedavi ve komplikasyonlar yönünden retrospektif analiz: tek merkez sonuçları

Aim: In the present study, the purpose was to evaluate the demographic and clinical data of pediatric patients, who had pancreatitis, who were followed up as outpatients or in the wards, and to evaluate the treatment modalities used along with nutritional status, length of hospital stays, and complications.&#x0D; Methods: This study was carried out by retrospectively evaluating the files of 166 patients identified by INSPPIRE Criteria who were followed up at the Department of Pediatric Gastroenterology, Faculty of Medicine, at xx University between 2011 and 2021. The age, gender, height, weight, known disease, drugs used, follow-up periods by us, complaints on admission, the etiological reason for the diagnosis, type of pancreatitis, length of hospital stay, treatment modalities applied, and complications of the patients were recorded. The PAPPS Scores, Ranson Admission Scores, and Ranson 48th hour Scores of the patients were analyzed.&#x0D; Results: A total of 120 patients (72.3%) had AP, 25 patients (15.1%) had CP, and 21 patient (12.7%) had ARP. According to the Atlanta Criteria, 82.5% were mild and 17.5% were moderate. The most common complaint was abdominal pain and the most common cause was found to be idiopathic. There was a weak and positive correlation between the PAPPS Score, Ranson Admission Score, and length of hospital stay.&#x0D; Conclusion: In the diagnosis and follow-up of pancreatitis, it is important to determine the severity of the disease and to reveal the etiology. Establishing and applying standard approaches for early diagnosis and treatment of patients will lead to prognostic improvement and prevent related complications.

The Importance of Biomarkers in the Diagnosis and Follow-Up of the Celiac Disease

Aim: In the present study, the purpose was to investigate the clinical significance, diagnostic capabilities, relationships, and correlations of inflammation-based biomarkers before and after treatment in children with Celiac Disease. Methods: The study was conducted by retrospectively evaluating the files of patients who were diagnosed and followed up in the Department of Pediatric Gastroenterology of Selçuk University between January 2011 and January 2023. The study was completed with 202 Celiac Disease patients who were diagnosed according to the criteria of the European Pediatric Gastroenterology, Hepatology, and Nutrition Community and 160 healthy follow-ups. In case of clinical or laboratory suspicion, serological tests such as positive Tissue Transglutaminase Antibody and Anti-Endomysium values and the histopathological examination of the endoscopic tissue sample findings consistent with Celiac Disease were evaluated. Results: There were 122 (56.7%) girls and 80 (54.4%) boys in the patient group and 93 (43.3%) girls and 67 (45.6%) boys in the control group. The most common intestinal complaint was abdominal pain in 49 (22.3%) patients. Gluten-free diet treatment was started after the diagnosis. The values of the patients at the time of diagnosis, in the 6th month after the diet, and the values of healthy control patients were compared. A weak and positive correlation was found between NLR, RPO, RLO levels, and age, body weight, and height in the correlation analysis. The ROC curve for hematological biomarkers was used to evaluate the level of additional diagnostic support in Celiac Disease patients. Conclusion: After a gluten-free diet, symptoms regress, and the development of more serious celiac-related damage can be prevented. This suggests that NLR, RLR, PLO, SII, HRO, and PNI formulas, which are used in many inflammatory conditions and obtained from routine hemogram parameters, can be used to determine dietary compliance in Celiac Disease patients during their follow-ups.

Causal Evaluation of Pancreatitis in Children

Pancreatitis in childhood is not common. It can be associated with severe morbidity and mortality. The role of clinicalevaluation is vital as it can be misdiagnosed easily. This study was aimed to review the prevalence, etiology,presentation and outcome of pancreatitis in children. This cross-sectional study was conducted at the Department ofPediatric Gastroenterology &amp; Nutrition of Bangabandhu Sheikh Mujib Medical University, Dhaka from January,2017 through June, 2018. A total 43 cases of pancreatitis were included in this study. Pancreatitis was classified asacute pancreatitis(AP), acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) as per diagnostic criteriamade by INSPPIRE (International Study Group of Pediatric Pancreatitis: In Search for a Cure) group. Among 43children (age 10.1 mean ± 2.55,18 males); AP,14 (32.6%); ARP, 14(32.6%) and CP,15(34.8 %). Half of AP wasidiopathic. Majority of ARP (28.6%) were idiopathic and CP (40%) had calcific pancreatitis. Majority of patients hadvomiting followed by moderate to severe abdominal pain. In CP 4(26.6%) patient developed diabetes mellitus.Majority of pancreatitis in children is idiopathic. A sub-set of AP goes on to develop ARP and CP on follow-up andbeing in the idiopathic sub-set is a risk factor. CP is progress to endocrine and exocrine insufficiency. Early diagnosis,close monitoring and proper intervention are mandatory to reduce the potential morbidity and mortality. Bangabandhu Sheikh Mujib Med. Coll. J. 2022;1(2):76-79

Celiac Disease in Uzbek Children: Insights into Disease Prevalence and Clinical Characteristics in Symptomatic Pediatric Patients.

A few studies on pediatric Celiac Disease (CD) are available from Central Asia. Recent immunogenetic research has highlighted that the HLA-DQ2/8 genetic predisposition to CD as well as the dietary intake of gluten in this geographical area, are comparable to other regions of the world where CD prevalence is known to be 1% or higher. This is a prospective and cross-sectional study investigating the prevalence and clinical characteristics of CD in symptomatic children referred to the pediatric gastroenterology department of a tertiary hospital in Uzbekistan from 1 September 2021, until 31 July 2022. In addition to collecting the relevant information related to clinical manifestations and laboratory analyses from the clinical files, a specific survey was also administered to patients' guardians. Serological, histopathological, and immunogenetic parameters specific to CD, fecal zonulin, and pancreatic elastases were assessed in CD patients. The study population consisted of 206 children. Overall, almost all of them (n = 192; 93.2%) were referred because of gastrointestinal manifestations, which were associated with extra-gastrointestinal manifestations in most cases (n = 153; 74.3%); a minority (n = 14; 6.8%) was mainly referred due short stature and/or growth failure only. Among all of these study participants, CD was diagnosed in 11 children (5.3%). Notably, although diarrhea was similarly reported in CD and non-CD patients, watery diarrhea (type 7 according to the Bristol stool scale) was much more frequently and significantly observed in the former group. All of these CD patients showed anti-tTG IgA 10 times higher than the upper normal limit, except one child with lower serum levels of total IgA; however, all of them received a diagnostic confirmation by histopathological analysis due to the lack of EMA testing in the country. Notably, most CD children (82%) showed a Marsh III histological grading. Around half patients (54.5%) showed zonulin values above the reference range, whereas none showed insufficient levels of pancreatic elastase. However, no correlation or association between zonulin and clinical, laboratory, histopathological, and immunogenetic parameters was found. This study may further suggest a relevant prevalence of CD in Uzbek children, based on this partial picture emerging from symptomatic patients only. Additionally, we highlighted the prevalence of typical CD forms with watery diarrhea, which should strongly support a full diagnostic work-up for CD in the local clinical setting. The high levels of anti-tTG IgA and high Marsh grade might also lead us to speculate a significant diagnostic delay despite the classical clinical expression of CD.

Portal Hypertension in Children at the Department of Pediatric Gastroenterology &amp; Nutrition, BSMMU, Dhaka, Bangladesh

Background: Portal hypertension is the hemodynamic abnormality frequently associated with serious liver disease, although it is recognized in a variety of extrahepatic diseases also. Portal hypertension is an important cause of morbidity and mortality in Bangladeshi children. Development of esophageal varices and bleeding is one of the major complications of CLD. The mortality from each episode of variceal bleeding is 30-50% depending on the clinical status of the patient. All conditions that interfere with blood flow at any level within the portal system can lead to portal hypertension. For better management of this disorder, it is important to determine the underlying cause. Objective: To assess the portal hypertension in children at the Department of Pediatric Gastroenterology &amp; Nutrition, BSMMU, Dhaka, Bangladesh. Methods: This cross-sectional descriptive study was conducted at the Department of Pediatric Gastroenterology &amp; Nutrition, BSMMU during the period Jan 2018 to July 2019. 50 patients who were diagnosed as portal hypertension were determined by Upper GI Endoscopy. Doppler USG was also done for supporting the diagnosis of Portal HTN as well as to differentiate between extrahepatic Portal HTN and CLD with portal HTN. Demographic data and other related information regarding etiology and complications were recorded in a standard datasheet. Results: Results: A total of 50 cases were included in this study. Their age range was 1.5-16 years. It was observed that 21 (42.0%) patients belonged to age group 6-10 years. The mean age was 9.22±9.85 years with ranged from 2.5 to 16 years. It was observed that almost two third 31 (62.0%) patients were male and 19 (38.0%) were female. Among 50 patients 29 were diagnosed as extrahepatic portal hypertension and 21 were diagnosed as CLD with portal HTN. Shows the etiology of portal hypertension of studied patients. Extrahepatic portal hypertension was the most common etiology (58.0%). Among CLD patients Wilson disease was the most common (13; 26.0%). Two (4.0%) patients were cryptogenic CLD and two (4.0%) were Budd Chiari Syndrome. One patient was Biliary cirrhosis and one patient had Auto immune hepatitis. Conclusion: We concluded that Extrahepatic Portal HTN is the most common cause of portal hypertension in children in this center. Among the intrahepatic causes of portal HTN Wilson disease was the

Evaluation of Gastrointestinal System Complaints and Comorbidities in Pediatric Familial Mediterranean Fever Patients.

Familial Mediterranean fever (FMF) is the most prevalent hereditary autoinflammatory disease among children. Abdominal pain and various gastrointestinal system (GIS) manifestations may arise directly from FMF or concomitantly with FMF. This study aimed to evaluate GIS complaints and findings other than classic peritonitis attacks in patients with FMF and to interpret concomitant GIS and hepatic disorders in these patients. The medical and genetic findings of patients with FMF who attended our clinic between December 2011 and December 2021 were reviewed. Gastrointestinal system symptoms, liver function tests, abdominal images, and endoscopic and histopathological data were extracted from medical records. A total of 576 pediatric patients (female, 52.3%) diagnosed with FMF were included. Among them, almost one-fifth displayed GIS complaints, such as abdominal pain, defecation problems, and dyspepsia, distinct from typical FMF attacks. High serum aminotransferase levels were detected in 18.4% of the patients, with viral infections being the most common cause of moderate/severe hypertransaminasemia. In addition, during follow-up, 26.9% of them were referred to the pediatric gastroenterology department. At least 1 gastroenterological and hepatobiliary disorder was detected in 17.5% of the patients because of organic and functional GIS disorders or hepatobiliary disorders, such as gastroesophageal reflux disease, esophagitis, functional dyspepsia, and inflammatory bowel diseases. Various GIS and hepatic disorders can be encountered in children with FMF. The spectrum of these complaints and pathologies can range from frequently observed health problems to more severe diseases.

Risk factors of persistent diarrhea in children below 5 years of age

Background: Persistent diarrhea is a known cause of mortality, morbidity, and malnutrition in developing countries. With recent improvement of rehydration therapy death due to acute diarrhea has been reduced. Though persistent diarrhea accounts for 2-20% of total diarrhea cases, it accounts for 23-62% of all diarrhea related deaths. Objective: The study was done to identify the risk factors associated with persistent diarrhea in children below 5 years of age.&#x0D; Methods: This prospective observational study was conducted in the department of Pediatric Gastroenterology, Hepatology and Nutrition of Dhaka Shishu hospital from July 2019 to June 2020. It included 50 children with persistent diarrhea age between 1 month to &lt;5 years. Detailed history, examination and appropriate investigations were done for all children. Crude odd ratio was calculated for each risk factor by univariate analysis and adjusted odds ratio was calculated by multivariate logistic regression.&#x0D; Results: Most of the children (52%) were under the age of 6 months to 1 year. Male (84%) were more affected than female (16%). Acute malnutrition was observed in 44% cases; among them severe acute malnutrition was in 40% cases. Unsafe drinking water, severe acute malnutrition, diarrhea within past 2 months, bottle feeding, persistent of dehydration more than 24 hours, feeding of cow’s milk, lack of exclusive breast feeding, prior antibiotic used, UTI, LRTI were statistically significant risk factors by univariate analysis. Unsafe drinking water (OR-1.6; 95% CI- 0.27- 2.78), severe acute malnutrition (OR-1.92; 95% CI-0.64 - 5.72), Feeding of cow’s milk (OR-3.90; 95% CI- 1.21- 12.49), lack of exclusive breastfeeding (OR-5.77; 95% CI- 1.44- 23.10), irrational use of antibiotics (OR-3.37; 95% CI- 1.01- 11.38), LRTI (OR-1.16; 95% CI- 0.49- 1.53) were found to be independent risk factors by multivariate logistic regression analysis.&#x0D; Conclusions: The results of this study concluded that use of unsafe drinking water, severe acute malnutrition, feeding of cow’s milk, lack of exclusive breastfeeding, irrational use of antibiotics are significant risk factors for the incidence of diarrhea in the present study population.&#x0D; Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 539-543

Safety and Effectiveness of Percutaneous Endoscopic Gastrostomy in Children

Objective: To determine the safety and effectiveness of percutaneous endoscopic gastrostomy in Paediatrics population. Study Design: Prospective longitudinal study. Place and Duration of Study: Department of Pediatric Gastroenterology, Pak Emirates Military hospital, Rawalpindi Pakistan, from Mar 2014 till Dec 2017. Methodology: Total 40 children who underwent percutaneous gastrostomy at the Paediatric gastroenterology department were included in the study. Patients were followed up for a period for 6 months. Demographic data, indications, weight gainand complications were noted for 6 months after the procedure. Results: Forty patients were enrolled and there were 23(57.5%) males with mean age of 39.8±20.2 months. Swallowing difficulty 23(47.5%) was the major indication followed by poor weight gain 10(25%), prolonged nasogastric tube feeding8(20%) and frequent aspirations 3(7.5%). Majority 32(80%) had underlying diagnosis of cerebral palsy. In six months follow up, 38(95%) children had an increment in weight (mean weight gain 1.89±1.0Kg). Early feed at 6 hours after procedure was tolerated by 38(95%) of patients. The majority of children did not have any complications and only few were observed to havewound infection, vomiting, and irritability in 3(7.50%), 1(2.50%) and 3(7.50%) respectively. Conclusion: Percutaneous endoscopic gastrostomy placement is a minimally invasive, safe and effective feeding technique for neurologically impaired children, especially cerebral palsy patients requiring long term assisted feeding.

Retracted: Data Analysis of Nursing Effects in Pediatric Gastroenterology Department under High Content Image Analysis Technology.

[This retracts the article DOI: 10.1155/2022/4302331.].