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Archives of Pediatric Gastroenterology, Hepatology, and Nutrition
Published by Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition
ISSN : -     EISSN : 28305442     DOI : -
Core Subject : Health,
Medicine & Pharmacology
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition (APGHN) is the official journal issued by the Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition (Perhimpunan Gastroenterologi, Hepatologi, dan Nutrisi Anak Indonesia). APGHN is issued four times in a year and published in English. Previously published in print form as Jurnal Gastrohepatologi Anak Indonesia (JGAI), APGHN is committed to promote scientific development in child’s health through high-quality publication and provides recent updates on pediatric gastroenterology, hepatology, and nutrition for health practitioners and scholars. APGHN accepts original articles, case reports, review articles, medical illustrations and clinical practice guidelines, all of which have been peer-reviewed carefully by our selected experts.
Arjuna Subject : Kedokteran - Pediatrics, Perinatology, and Child Health
Articles 74 Documents
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Infantile Hyperchylomicronemia Due to A Novel GPIHBP1 Disease-Causing Variant Presenting with Milky Blood: A Rare Case Report Iman Marzouq; Basant Elbanna; Sabine Schröder; Kornelia Tripolszki; Aida M. Bertoli-Avella; Aml Mahfouz
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 2 (2023): APGHN Vol. 2 No. 2 May 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.2.2023.25-31

Abstract

Background: Familial hyperchylomicronemia is a very rare autosomal recessive disorder and the most severe type of pediatric hyperlipidemia. The purpose of this case report is to enhance clinician's insight on the diagnosis and management plan in the case of infantile hyperchylomicronemia presenting with milky blood. Case: We reported a 2-month-old infant with familial chylomicronemia syndrome. The patient was ‘accidentally’ diagnosed by the observation of milky blood. Exome sequencing revealed a homozygous likely pathogenic GPIHBP1 variant (NM_178172.5:c.193T>C p.(Cys65Arg)) confirming the diagnosis. He was treated with low-fat diet, a formula rich in medium-chain triglycerides and fenofibrates. After 4 days, his serum triglycerides decreased markedly. Fenofibrates were stopped at the age of one year and his serum triglycerides were maintained at low level with dietary measures. No complications occurred during two years follow-up period. Discussion: Clinical manifestations of familial chylomicronemia syndrome start in early life with a very high level of hypertriglyceridemia and with monogenetic etiology, in contrast to multifactorial chylomicronemia syndrome that starts in adulthood, with proposed polygenic etiology. The main treatment of familial chylomicronemia syndrome is dietary fat restriction to less than 15% of the total caloric intake and medium-chain triglycerides which can bypass the chylomicron pathway of fat metabolism. Conclusion: The main challenge in this case was the early diagnosis to protect the patient against serious complications. The mainstay of therapy is low-fat diet and medium-chain triglycerides. This case illustrates the relevance of establishing a timely genetic diagnosis and treatment.
Crohn's Disease in Children: A Case Report Nicodemus Nicodemus; Nuraini Irma Susanti
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 2 (2023): APGHN Vol. 2 No. 2 May 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.2.2023.32-45

Abstract

Background: Crohn's disease in children is a chronic inflammatory bowel disease (IBD). The incidence of this disease has tended to increase in recent decades. This case report aimed to increase clinician insight into Crohn's disease. Case: We reported a case of Crohn's disease, one of the inflammatory bowel disease (IBD) type in a 16-year-old boy. The patient came with complaints of loose stools without mucus and blood, accompanied by heartburn, nausea, vomiting, and decreased appetite. The patient had a history of changes in defecation patterns in the last 4 months and decreased appetite and weight loss in the last 1 month. There was epigastric tenderness on physical examination. Inflammatory markers and fecal calprotectin values were increased. Gastrointestinal endoscopy results found pangastritis and pancolitis with histopathological examination showing results appropriate to IBD. The patient received corticosteroid methylprednisolone 1 mg/kg/day as induction therapy and experienced improvement in symptoms and laboratory results after 7 days of therapy. Discussion: There are characteristic differences between Crohn's disease and ulcerative colitis. A definite diagnosis is made by endoscopy and histopathological examination. The current goal of Crohn's disease therapy is no longer limited to improving symptoms or optimizing growth and development, but also targeting the improvement of the gastrointestinal mucosa. Remission induction therapy can be carried out with exclusive enteral nutrition or corticosteroids which are gradually reduced. Conclusion: This case report increases clinician insight into the characteristics, approaches to IBD diagnosis, and remission induction therapy in Crohn's disease in children.
Comparison of PUCAI Score in Mesalazine-Treated Children with Ulcerative Colitis Nadia Chairunnisa; Juwita Pratiwi; Ninung Rose Diana Kusumawati
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 2 (2023): APGHN Vol. 2 No. 2 May 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.2.2023.18-24

Abstract

Background: Ulcerative colitis is a chronic idiopathic inflammatory bowel disease (IBD) characterized by intestinal inflammation confined to the superficial mucosal layer. Mesalazine, a 5-aminosalicylic acid (5-aminosalicylic, or 5-ASA) compound, is most often used as first-line therapy for mild to moderate ulcerative colitis. The Pediatric Ulcerative Colitis Activity Index (PUCAI) is a non-invasive multi-item measure that has been shown to be valid, reliable, and responsive to short-term changes in several clinical trials and cohort studies. Therefore, this study aims to compare the PUCAI scores in children with ulcerative colitis who received mesalazine therapy to those who did not. Methods: We performed a retrospective database analysis of 12 patients, who were diagnosed with ulcerative colitis at Dr. Kariadi General Hospital, Semarang, Indonesia in a span of 1 year. We included all cases of pediatric patients with ulcerative colitis, then we divided them into 2 groups, the group receiving mesalazine therapy and the group who did not. We monitored the development of PUCAI scores before and after treatment. Results: The number of samples in this study was 12 samples. All sample data were taken based on data from pediatric gastroenterohepatology patients diagnosed with colitis ulcerative based on pathology anatomy results, who were treated in the pediatric ward of RSUP Dr. Kariadi Semarang. From the result of the paired t-test, there was a significant decrease in PUCAI score in patients who received Mesalazine, (p = 0.007), while those who did not receive mesalazine, did not show any significant decrease in PUCAI score. Conclusion: Ulcerative colitis (UC) is a chronic relapsing inflammatory condition. UC is often treated with mesalazine as the first-line treatment. The use of the PUCAI score is an appropriate tool to determine the progression of this disease. Based on the data obtained, the administration of mesalazine therapy in children with ulcerative colitis can improve PUCAI scores compared to children who do not receive mesalazine therapy.
Pediatric Gastroesophageal Reflux Disease (GERD): A Literature Review Farahdina Shahnaz; Yuda Satrio Wicaksono; Himawan Aulia Rahman
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 2 (2023): APGHN Vol. 2 No. 2 May 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.2.2023.46-60

Abstract

Background: Gastroesophageal reflux disease (GERD) is a condition where stomach contents reflux into the esophagus, causing discomfort and complications. It is most prevalent in infants (26.9%) and lesser in children under 10 (3.2%) and over 10 years old (10.1%). Discussion: GERD is caused by frequent relaxations of the lower esophageal sphincter (LES), allowing stomach contents to escape into the esophagus. Symptoms vary with age, with infants experiencing regurgitation and irritability, while older children may have heartburn and nausea. Diagnosis requires differentiating GERD from similar conditions and may involve various tests, though their primary use lacks sufficient evidence. Nonpharmacological treatments include thickened feeding, reducing feeding volume but increasing frequency and possibly eliminating cow's milk protein. Pharmacological treatments include Proton Pump Inhibitors (PPIs), and Histamine Receptor Antagonists (H2RAs), though their efficacy varies. Prokinetics are generally not recommended due to lack of evidence. If all these treatments fail, anti-reflux surgery such as fundoplication can be considered. Conclusion: The hallmark of GERD is the presence of esophagitis during endoscopy. However, Barrett’s esophagus is rare in pediatric GERD patients. Factors indicating a worse prognosis include early onset age, an initial GERD diagnosis, and the need for PPI or combination of H2RA and PPI treatment.
Fundoplication in Pediatric Achalasia Patients Undergoing Heller’s Myotomy: A Systematic Review Sarah Susanto; Brendan O’Connor; Warwick J. Teague
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 2 (2023): APGHN Vol. 2 No. 2 May 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.2.2023.1-17

Abstract

Background: Achalasia is a rare motility disorder, with the incidence being the lowest among children. The disease is caused by the inability of the LOS to relax and the absence of normal peristalsis of esophagus. Heller’s myotomy has been known to be the gold standard management of achalasia, however, reports on postoperative GERD were established in the past. To control the reflux symptoms, fundoplication has been used as an addition to Heller’s myotomy. Unfortunately, there has been an inconclusive finding from available studies regarding the need for performing fundoplication. Hence, we aim to determine whether or not the addition of fundoplication to Heller myotomy in achalasia children resulted in better postoperative outcomes and fewer complications of GERD. Methods: A literature search was carried out in four databases: Medline, EMBASE, Pubmed, and Cochrane Library. The search was limited to publications from 2006 to 2019, English studies, and achalasia patients age 0-18 years old that underwent Heller’s myotomy. The exclusion of studies from the primary screening according to title and abstracts and secondary screening on the full text were done according to a priori protocol. Duplicate studies were also eliminated by using reference management software and manually. Results: A total of 446 studies were retrieved from the search. Preliminary screening based on the eligibility criteria resulted in 21 articles to be included in this review. A total of 410 patients were included in this study, in which 80 underwent HM alone and 330 experienced fundoplication as an adjunct to HM (HMF). There was a higher proportion of asymptomatic patients in the HM group (56.3%) compared to HMF (48.8%). Both groups had a similar rate of complications (HMF 12.1%, HM 10.0%). However, in terms of postoperative GERD, slightly better results were seen among HMF patients (9.7%) than HM (15%). Conclusion: Fundoplication did not result in better resolution of symptoms, as seen from its percentage of asymptomatic patients. Improvements in postoperative GERD were seen in HMF patients, however, it was deemed as insignificant. The findings suggest that there was a limited benefit in using fundoplication.
Esophagoduodenal Varices in Non-cirrhotic Portal Hypertension with Myelodysplastic Syndrome: A Case Report Ina Rosalina; Reza Latumahina; Yudith Setiati Ermaya; Dwi Prasetyo
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 3 (2023): APGHN Vol. 2 No. 3 August 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.3.2023.25-32

Abstract

Background: Esophagogastroduodenal varices are dilated submucosal of distal esophageal, gastric, and duodenal veins connecting the portal and systemic circulation. This case report aims to describe a unique case of a child with esophagoduodenal varices due to myelodysplastic syndrome. Case: We reported a case of 3-year-old girl who came to Hasan Sadikin General Hospital on April 3 2022, complaining of black stools 1 time per day for two days before admission. She had previously been diagnosed with esophagogastroduodenal varices since 2019. On initial examination, the patient was fully conscious and appeared pale. The patient's clinical condition improved after adequate treatment of blood transfusion, octreotide, omeprazole and propanolol. However, patient later developed pancytopenia and underwent bone marrow puncture examination which revealed a myelodysplastic syndrome. Discussion: Myelodysplastic syndrome is a condition where ineffective hematopoiesis occurs and can lead to blood malignancy, especially acute myeloblastic leukemia. In this patient, she presented with unequivocal hypertensive gastroesophageal varices, splenomegaly, absence of fibrosis and thrombocytosis supporting subsequent diagnosis of idiopathic non cirrhosis portal hypertension. On the other hand, non-cirrhotic portal hypertension can also be caused by myelodysplastic syndrome as described in this case report. Conclusion: Myeloproliferative malignancies can be a cause of idiopathic non cirrhosis portal hypertension. Pancytopenia often occurs in patients with portal hypertension due to splenomegaly or myelodysplastic syndrome, which can lead to acute myeloblastic leukemia, an example of a myeloproliferative malignancy.
Nutritional Status in Children with End-Stage Kidney Disease Undergoing Hemodialysis and Other Related Factors Namira Metasyah; Eka Laksmi Hidayati
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 3 (2023): APGHN Vol. 2 No. 3 August 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.3.2023.13-24

Abstract

Background: Chronic kidney disease causes several changes in the body's function in metabolizing nutrients. This has led to the discovery of cases of malnutrition in chronic kidney disease patients, especially in ESRD patients undergoing hemodialysis. This certainly needs to be a concern because nutrition is very important for children's growth. Therefore, this study was conducted to find out the effect of hemodialysis and other influencing factors on the nutritional status of children. Methods: The study was conducted with a cross- sectional design by taking secondary data in the form of disease stage, duration of disease, primary etiologic factors, and comorbidities from medical records. Data on the nutritional status of children was obtained by measuring weight and height, and upper arm circumference and then entered into the WHO Anthro application. Demographic data, such as the education level of the father & mother, family economic status, age, and gender were obtained by filling out the Case Report Form (CRF). Twenty respondents met the inclusion and exclusion criteria of this study. Results: The average nutritional status assessment seen from the body mass index according to age showed results of -2 SD < x < 1 SD with good nutrition interpretation and x <-2 SD (short stature) in terms of height according to age. Based on bivariate analysis, there was no significant effect between duration of hemodialysis, frequency of hemodialysis, etiology, age, sex, and comorbidities (p>0.05) in children with chronic kidney failure who were undergoing hemodialysis on their nutritional status. Conclusion: The nutritional status of CKD children undergoing hemodialysis was assessed based on body mass index and height according to age. The average results were good nutrition but with short stature. There was no effect of duration, frequency, etiology, age, gender, and comorbidities in children with chronic kidney failure undergoing hemodialysis on their nutritional status.
Nutritional Approach of Neonatal with High Output Stoma Due to Long Segment Hirschsprung Disease: A Case Report Aris Primadi; Filla Reviyani Suryaningrat
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 3 (2023): APGHN Vol. 2 No. 3 August 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.3.2023.33-8

Abstract

Background: High Output Stoma (HOS) continues to be one of the most challenging problems for pediatrician especially in neonates. One of the most common causes in neonatal HOS is post resection long segment Hirschsprung disease. Case: We reported a case of three-day-old baby boy diagnosed as Hirschsprung diseases with peritonitis possibility and did laparotomy with ileal resection, double barrel ileostomy and biopsy. Nutritional management is a major subject on taking care of this type of neonatal patient. We share our experience in limited facilities with all the patient uniqueness Discussion: Loss of a significant length of the small bowel results in interrelated physiologic events as a result of decreased small intestinal mucosal absorptive cell. This leads to a lesser fraction of ingested food and intestinal secretion thus causing an excessive volume loss. The introduction of early enteral feeds promotes intestinal adaptation, with subsequent weaning off parenteral nutrition. Most off patient with high output stoma will require parenteral nutrition which is associated with acute and long-term complications. In our case, we did early nutritional intervention using parenteral and enteral nutrition, counting ongoing fluid loss trough stoma and adjust it to total daily fluid requirement. We found weight loss during hospitalized due to HOS, and we do catch up at the end. We found difficulties to adjust comparation between enteral and parenteral intake to maintain the weight gain. Conclusion: Although parenteral nutrition is often necessary, at least initially, the therapeutic goal should be to enhance intestinal adaptation and enteral nutrient assimilation, and thereby reduce parenteral nutrition requirements. Daily monitoring for ongoing fluid loss very crucial for adjusting nutrition.
The Role of Mesenchymal Stem Cells in Liver Regeneration Hardian Gunardi
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 3 (2023): APGHN Vol. 2 No. 3 August 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.3.2023.39-51

Abstract

Background: Inflammation of the liver caused by cholestasis, viral infection, alcohol, autoimmune reactions, toxins, or metabolism will result in a prolonged immune response. As a result, simultaneous inflammation and tissue remodelling occur, resulting in fibrosis and eventually leading to cirrhosis. The main treatment for end-stage liver cirrhosis is liver transplantation. However, it is often not possible for patients to undergo this life-saving procedure. On the other hand, stem cell transplantation may be a potential strategy to prevent disease progression and improve the degree of fibrosis. Discussion: Inflammation of the liver activates hepatic stellate cells, which are perisinusoidal cells in the Disse cavity that contain vitamin A. Hepatic stellate cells activation results in retinoid storage loss and transformation into myofibroblast-like cells that express α-smooth muscle action (α-SMA) and produce collagen which plays a major role in fibrosis. Liver regeneration due to chronic liver damage is played by mesenchymal cells through the mesenchymal-epithelial or epithelial-mesenchymal transition (MET/EMT) process. Administration by the intrahepatic route is thought to be the ideal route because fewer cells are lost in the circulation and more mesenchymal stem cells differentiates into hepatocytes in the damaged liver area. However, intrasplenic route maybe an alternative with easier administration technique. There are special considerations regarding the risks, including the risk of carcinogenesis and viral transmission. Conclusion: Mesenchymal stem cells transplantation may be a potential therapeutic strategy for patients with end stage liver disease in the future. However, future research is needed regarding the risk of carcinogenesis and viral transmission following the procedure.
Endoscopic Retrograde Cholangiopancreatography with an Ultra-Slim Forward-Viewing Gastroscope in a 3.8 kg Infant: A Case-Report Maria Inês Canha; Rita Prata; Pedro Martins; Gonçalo Ramos; Rui Alves; Isabel Afonso; António Mateus Dias; João Coimbra
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 2 No. 4 (2023): APGHN Vol. 2 No. 4 November 2023
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.2.4.2023.24-9

Abstract

Background: Experience with pediatric endoscopic retrograde cholangiopancreatography (ERCP), particularly in infants, is limited. The lower incidence of biliopancreatic disorders in infants and the discontinuation of pediatric duodenoscopes raise concerns regarding ERCP’s technical efficacy and safety in this vulnerable population, for whom conventional duodenoscopes are not recommended. Short case description: We report the case of a low-weight infant with short bowel syndrome under parenteral nutrition, who was referred for ERCP due to choledocholithiasis with progressive jaundice. We opted for an ultra-slim forward viewing gastroscope (5.4mm distal end outer diameter and 2.2mm working channel) with a needle knife sphincterotome (1.8mm outer sheath diameter). This allowed sphincterotomy with adequate gallstone removal and complete normalization of cholestasis parameters without associated complications. Discussion and conclusion: With this case we aimed to describe a previously unreported approach to a low-weight infant with gallstone disease requiring ERCP, which proved to be safe and effective.

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