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Gastric Hemangioma Treated with Argon Plasma Coagulation in a Newborn Infant.

Young Ah LEE ; Peter CHUN ; Eun Ha HWANG ; Yeoun Joo LEE ; Chang Won KIM ; Jae Hong PARK

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(2):134-137. doi:10.5223/pghn.2017.20.2.134

Gastric hemangioma in the neonatal period is a very rare cause of upper gastrointestinal bleeding. We present a case of hemangioma limited to the gastric cavity in a 10-day-old infant. A huge, erythematous mass with bleeding was observed on the lesser curvature side of the upper part of the stomach. Surgical resection was ruled out because the location of the lesion was too close to the gastroesophageal junction. Medical treatment with intravenous H₂ blockers, octreotide, packed red blood cell infusions, local epinephrine injection at the lesion site, application of hemoclip, and gel-form embolization of the left gastric artery did not significantly alter the transfusion requirement. Hemostasis was achieved with endoscopic argon plasma coagulation (APC). After two sessions of APC, complete removal of the lesion was achieved. APC was a simple, safe and effective tool for hemostasis and the ablation of gastric hemangioma without significant complications.
Argon Plasma Coagulation* ; Argon* ; Arteries ; Epinephrine ; Erythrocytes ; Esophagogastric Junction ; Hemangioma* ; Hemorrhage ; Hemostasis ; Humans ; Infant ; Infant, Newborn* ; Octreotide ; Stomach

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Ileal Perforation with Norovirus Gastroenteritis in a 3-Month-Old Infant.

Seol Woo WI ; Su Jin LEE ; Eun Kyeong KANG ; Sung Min CHO

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(2):130-133. doi:10.5223/pghn.2017.20.2.130

Noroviruses have been recognized as the leading cause of epidemic and sporadic gastroenteritis since the advent of molecular diagnostic technique. They have been documented in 5-31% of pediatric patients hospitalized with gastroenteritis. Although norovirus gastroenteritis is typically mild and self-limited, it causes severe, but sometimes fatal, conditions in the vulnerable population such as immunocompromised patients, young children, and the elderly. Bowel perforation due to norovirus infection is rare. We report a case of small bowel perforation with norovirus gastroenteritis in the infant with Down syndrome during the hospitalization with pneumonia. Severe dehydration may cause bowel ischemia and could have triggered bowel perforation in this case. Physicians should be alert to the potential surgical complications followed by severe acute diarrhea, especially in high risk groups.
Aged ; Child ; Dehydration ; Diarrhea ; Down Syndrome ; Gastroenteritis* ; Hospitalization ; Humans ; Immunocompromised Host ; Infant* ; Ischemia ; Molecular Diagnostic Techniques ; Norovirus* ; Pneumonia ; Vulnerable Populations

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Pediatric Mycoplasma pneumoniae Infection Presenting with Acute Cholestatic Hepatitis and Other Extrapulmonary Manifestations in the Absence of Pneumonia.

Won Jae SONG ; Ben KANG ; Hwa Pyung LEE ; Joongbum CHO ; Hae Jeong LEE ; Yon Ho CHOE

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(2):124-129. doi:10.5223/pghn.2017.20.2.124

Mycoplasma pneumoniae infections mainly involve respiratory tract; however, also can manifestate other symptoms by site involved. Extrapulmonary manifestations of M. pneumoniae infection are rarely known to occur without pneumonia. Herein we report a case of a 9-year-old boy who presented with acute cholestatic hepatitis in the absence of pneumonia. Rhabdomyolysis, skin rash, and initial laboratory results suspicious of disseminated intravascular coagulopathy were also observed in this patient. M. pneumoniae infection was identified by a 4-fold increase in immunoglobulin G antibodies to M. pneumoniae between acute and convalescent sera by enzyme-linked immunosorbent assay. This is the first pediatric case in Korea of M. pneumoniae infection presenting with acute cholestatic hepatitis in the absence of pneumonia.
Antibodies ; Child ; Enzyme-Linked Immunosorbent Assay ; Exanthema ; Hepatitis* ; Humans ; Immunoglobulin G ; Korea ; Male ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pneumonia* ; Pneumonia, Mycoplasma* ; Respiratory System ; Rhabdomyolysis

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Early Diagnosis of ABCB11 Spectrum Liver Disorders by Next Generation Sequencing.

Su Jeong LEE ; Jung Eun KIM ; Byung Ho CHOE ; An Na SEO ; Han Ik BAE ; Su Kyeong HWANG

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(2):114-123. doi:10.5223/pghn.2017.20.2.114

PURPOSE: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. METHODS: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. RESULTS: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. CONCLUSION: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.
Biliary Atresia ; Choledochal Cyst ; Cholestasis ; Cholestasis, Intrahepatic ; Diagnosis ; Early Diagnosis* ; Exome ; Genetic Counseling ; Hepatitis ; High-Throughput Nucleotide Sequencing ; Humans ; Hyperbilirubinemia ; Jaundice ; Liver Transplantation ; Liver* ; Metabolic Diseases ; Parenteral Nutrition, Total ; Syphilis, Congenital

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Bannayan-Riley-Ruvalcaba Syndrome in a Patient with a PTEN Mutation Identified by Chromosomal Microarray Analysis: A Case Report.

Sun Hwa LEE ; Eell RYOO ; Hann TCHAH

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(1):65-70. doi:10.5223/pghn.2017.20.1.65

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is one of the phosphatase and tensin homolog hamartoma tumor syndrome with a PTEN gene mutation. It is a rare dominant autosomal disorder characterized by cutaneous lipomas, macrocephaly, intestinal polyps, and developmental delay. Diagnosing this syndrome is important, because it may represent the pediatric phenotype of Cowden syndrome, in which there is an increased risk for malignant tumors in children. Until now, the prevalence of BRRS is unknown. Several dozen cases have been reported in the medical literature, but no case has been reported in Korea. Here we report a case of a 19-year-old girl who was diagnosed with BRRS because of macrocephaly, intellectual disability, and intestinal polyps. Her mother had similar findings and a PTEN mutation. Neither patient had mutations detected by conventional mutation-detection techniques, but a PTEN gene deletion was demonstrated by chromosomal microarray analysis.
Child ; Female ; Gene Deletion ; Hamartoma ; Hamartoma Syndrome, Multiple* ; Humans ; Intellectual Disability ; Intestinal Polyps ; Korea ; Lipoma ; Megalencephaly ; Microarray Analysis* ; Mothers ; Phenotype ; Prevalence ; Young Adult

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A Rare Cause of Recurrent Acute Pancreatitis in a Child: Isovaleric Acidemia with Novel Mutation.

Elif SAG ; Alper Han CEBI ; Gulay KAYA ; Gulay KARAGUZEL ; Murat CAKIR

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(1):61-64. doi:10.5223/pghn.2017.20.1.61

Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, “the odor of sweaty feet,” abdominal pain, vomiting, feeding intolerance, shock and coma. Recurrent acute pancreatitis associated with IVA have been rarely reported. Herein; we report a child who admitted with recurrent acute pancreatic attacks and had the final diagnosis of IVA. Mutation analysis revealed a novel homozygous mutation of (p.E117K [c.349G>A]) in the IVA gene. Organic acidemias must kept in mind in the differential diagnosis of recurrent acute pancreatic attacks in children.
Abdominal Pain ; Acid-Base Equilibrium ; Acidosis ; Child* ; Coma ; Diagnosis ; Diagnosis, Differential ; Humans ; Hyperammonemia ; Hypoglycemia ; Isovaleryl-CoA Dehydrogenase ; Ketosis ; Metabolic Diseases ; Metabolism ; Odors ; Pancreatitis* ; Shock ; Vomiting

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Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™.

Racha KHALAF ; Sara KARJOO ; Paul DANIELSON ; Michael WILSEY ; Fauzia SHAKEEL

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(1):55-60. doi:10.5223/pghn.2017.20.1.55

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of Omegaven™. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of Omegaven™.
Alanine Transaminase ; Alkaline Phosphatase ; Aspartate Aminotransferases ; Bilirubin ; Cholestasis ; Compassionate Use Trials* ; Diagnosis ; Empathy* ; Emulsions ; Fatty Acids, Omega-3 ; Hirschsprung Disease ; Humans ; Infant, Newborn ; Liver ; Liver Diseases ; Parenteral Nutrition, Total ; United States Food and Drug Administration

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Effect of Metronidazole in Infants with Bowel Habit Change: Irrelative to the Clostridium difficile Colonization.

Eun Jin KIM ; Sung Hyun LEE ; Hann TCHAH ; Eell RYOO

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(1):47-54. doi:10.5223/pghn.2017.20.1.47

PURPOSE: Clinical symptoms associated with Clostridium difficile infection (CDI) can vary widely. Carrier state without apparent symptoms is relatively common during infancy. The objective of this study was to determine the association of C. difficile colonization with bowel habit change and the effect of C. difficile colonization treatment on restoration of normal bowel habit. METHODS: Between 2006 and 2014, infants at 1 to 12 months of age with diarrhea for more than 2 weeks who did not improve with conservative care were recruited from Gachon University Gil Medical Center. Infants who were followed up for at least 7 days were included. The presence or absence of C. difficile colonization, effect of metronidazole, and other medical records were reviewed. To determine the association between CDI and bowel habit change, logistic regression analysis was used. RESULTS: Of a total of 126 infants, 74 (58.7%) were male patients. Of the 126 patients, 27 (21.4%) had C. difficile colonization. Significant (p<0.05) risk factors for C. difficile colonization included artificial milk feeding (odds ratio [OR], 4.310; 95% confidence interval [CI], 1.564-11.878), prior rotavirus vaccination (OR, 4.322; 95% CI, 1.018-18.349), and antibiotic use (OR, 4.798; 95% CI, 1.430-16.101). There was improvement in bowel habit after metronidazole therapy (OR, 0.34; 95% CI, 0.15-0.79; p<0.05), regardless of the presence or absence of C. difficile colonization, CONCLUSION: There was no significant correlation between bowel habit change and C. difficile colonization during infancy. However, metronidazole can be used as an optional method to manage functional gastrointestinal disorders.
Carrier State ; Clostridium difficile* ; Clostridium* ; Colon* ; Diarrhea ; Gastrointestinal Diseases ; Humans ; Infant* ; Logistic Models ; Male ; Medical Records ; Methods ; Metronidazole* ; Milk ; Risk Factors ; Rotavirus ; Vaccination

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Effect of the Baseline Vitamin D Level on Growth Outcome in Pediatric Crohn Disease.

Eun Joo LEE ; Jin Soo MOON ; Jae Sung KO ; Hye Ran YANG ; Ju Young JANG ; Ju Whi KIM ; Kyung Jae LEE

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(1):41-46. doi:10.5223/pghn.2017.20.1.41

PURPOSE: Vitamin D deficiency is common in Crohn disease (CD). The aim of the study was to examine the prevalence of vitamin D deficiency and evaluate the association between vitamin D status and growth outcome in Korean pediatric CD patients. METHODS: In this retrospective study, 17 children younger than 18 years old diagnosed with CD were enrolled and their serum 25-hydroxy vitamin D (25[OH]D) was checked between 2011 and 2015. We categorized the patients into two groups, Group 1 and Group 2. Group 1 included patients with serum 25(OH)D levels below 10 ng/mL, and Group 2 was for patients with a 25(OH)D serum levels between 10 ng/mL and 30 ng/mL. The z-scores for height (Htz), weight (Wtz), and body mass index (BMIz) were measured at baseline, 6 months, and 12 months. RESULTS: The mean serum 25(OH)D levels of the total 65 CD patients and 17 enrolled patients were 15.64±6.9 ng/mL and 13.1±5.1 ng/mL, respectively. There was no correlation at the beginning of the study between vitamin D level and growth parameters (Htz, Wtz, BMIz) or other variables including laboratory data and Pediatric Crohn Disease Activity Index. The Htz, Wtz, and BMIz in Group 1 showed no significant improvement at 6 months and 12 months follow-up. In Group 2, Wtz and BMIz showed significant improvements sustained until 12 months of follow-up. Htz showed no significant improvement at 6 months but there was significant improvement at 12 months. CONCLUSION: It seems that baseline vitamin D status affects growth outcome in pediatric CD.
Body Mass Index ; Child ; Crohn Disease* ; Follow-Up Studies ; Humans ; Prevalence ; Retrospective Studies ; Vitamin D Deficiency ; Vitamin D* ; Vitamins*

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Single Center Experience with Gastrostomy Insertion in Pediatric Patients: A 10-Year Review.

Jiyoung KIM ; Hong KOH ; Eun Young CHANG ; Sun Yeong PARK ; Seung KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2017;20(1):34-40. doi:10.5223/pghn.2017.20.1.34

PURPOSE: This study was performed to review the outcomes of gastrostomy insertion in children at our institute during 10 years. METHODS: A retrospective chart review was performed on 236 patients who underwent gastrostomy insertion from October 2005 to March 2015. We used our algorithm to select the least invasive method for gastrostomy insertion for each patient. Long-term follow-up was performed to analyze complications related to the method of gastrostomy insertion. RESULTS: Out of 236 patients, 120 underwent endoscopic gastrostomy, 79 had laparoscopic gastrostomy, and 37 had open gastrostomy procedures. The total major complication rates for endoscopic gastrostomy insertion, laparoscopic gastrostomy insertion, and open gastrostomy were 9.2%, 8.9%, and 8.1%, respectively. The most common major complication was gastroesophageal reflux requiring Nissen fundoplication (3.8%), and other complications included peritonitis (1.3%), hiatal hernia (1.3%), and bowel perforation (0.8%). Gastrostomy removal was successful in 8.6% and 5.0% of patients in the endoscopic and surgical gastrostomy groups, respectively. Gastrocutaneous fistula occurred in 60% of surgically inserted cases, requiring a second operation. CONCLUSION: This retrospective study was performed to review the outcome of gastrostomy insertion, as well as to introduce an algorithm that can be used for future cases. Further studies should be conducted to make a consensus on choosing the most appropriate method for gastrostomy insertion.
Child ; Consensus ; Fistula ; Follow-Up Studies ; Fundoplication ; Gastroesophageal Reflux ; Gastrostomy* ; Hernia, Hiatal ; Humans ; Intestinal Fistula ; Methods ; Peritonitis ; Retrospective Studies

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