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Atypical beta-Catenin Activated Child Hepatocellular Tumor.

Aynur TURAN ; Havva Akmaz UNLU ; Esra KARAKUS ; Arzu YAZAL ERDEM ; Zeynep Ilerisoy YAKUT

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):144-148. doi:10.5223/pghn.2015.18.2.144

Hepatocellular adenomas are a benign, focal, hepatic neoplasm that have been divided into four subtypes according to the genetic and pathological features. The beta-catenin activated subtype accounts for 10-15% of all hepatocellular adenomas and specific magnetic resonance imaging features have been defined for different hepatocellular adenomas subtypes. The current study aimed to report the magnetic resonance imaging features of a well differentiated hepatocellular carcinoma that developed on the basis of beta-catenin activated hepatocellular adenomas in a child. In this case, atypical diffuse steatosis was determined in the lesion. In the literature, diffuse steatosis, which is defined as a feature of the hepatocyte nuclear factor-1alpha-inactivated hepatocellular adenomas subtype, has not been previously reported in any beta-catenin activated hepatocellular adenomas case. Interlacing magnetic resonance imaging findings between subtypes show that there are still many mysteries about this topic and larger studies are warranted.
Adenoma ; Adenoma, Liver Cell ; beta Catenin* ; Carcinoma, Hepatocellular ; Child* ; Hepatocytes ; Humans ; Liver ; Liver Neoplasms ; Magnetic Resonance Imaging

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Clinical, Biochemical, and Genetic Characterization of Glycogen Storage Type IX in a Child with Asymptomatic Hepatomegaly.

Jung Ah KIM ; Ja Hye KIM ; Beom Hee LEE ; Gu Hwan KIM ; Yoon S SHIN ; Han Wook YOO ; Kyung Mo KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):138-143. doi:10.5223/pghn.2015.18.2.138

Glycogen storage disease type IX (GSD IX) is caused by a defect in phosphorylase b kinase (PhK) that results from mutations in the PHKA2, PHKB, and PHKG2 genes. Patients usually manifest recurrent ketotic hypoglycemia with growth delay, but some may present simple hepatomegaly. Although GSD IX is one of the most common causes of GSDs, its biochemical and genetic diagnosis has been problematic due to its rarity, phenotypic overlap with other types of GSDs, and genetic heterogeneities. In our report, a 22-month-old boy with GSD IX is described. No other manifestations were evident except for hepatomegaly. His growth and development also have been proceeding normally. Diagnosed was made by histologic examination, an enzyme assay, and genetic testing with known c.3210_3212del (p.Arg1070del) mutation in PHKA2 gene.
Child* ; Diagnosis ; Enzyme Assays ; Genetic Heterogeneity ; Genetic Testing ; Glycogen Storage Disease ; Glycogen* ; Growth and Development ; Hepatomegaly* ; Humans ; Hypoglycemia ; Infant ; Male ; Phosphorylase Kinase

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A Case of Intussusception with Acute Appendicitis.

Hyung Min KEE ; Ji Young PARK ; Dae Yong YI ; In Seok LIM

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):134-137. doi:10.5223/pghn.2015.18.2.134

In children presenting to hospital with gastrointestinal symptoms, diseases such as intussusception and acute appendicitis require particular attention and careful examination. Early diagnosis and proper treatment are important because of possible severe complications such as peritonitis and death. Intussusception and appendicitis share similar clinical manifestations. More importantly, the presence of acute appendicitis together with intussusception in children is very rare. We describe an interesting case of a 38-month-old boy who presented with abdominal pain in the right lower quadrant. His vital signs were stable and laboratory test findings showed no specific alterations. We detected tenderness in the right lower quadrant. A computed tomography scan showed an ileocolic intussusception with no strangulation and diffuse wall thickening of the appendix trapped within the intussusception. The patient underwent an appendectomy and manual reduction.
Abdominal Pain ; Appendectomy ; Appendicitis* ; Appendix ; Child ; Child, Preschool ; Early Diagnosis ; Humans ; Intussusception* ; Male ; Peritonitis ; Vital Signs

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A Case of Visceral Autonomic Neuropathy Complicated by Guillain-Barre Syndrome Accompanied with Cyclic Vomiting Syndrome-like Disorder in a Child.

Suk Jin HONG ; Byung Ho CHOE

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):128-133. doi:10.5223/pghn.2015.18.2.128

We present a case of an 8-year-old boy with visceral autonomic neuropathy complicated by Guillain-Barre syndrome. In this pediatric patient, gastroparesis was the major symptom among the autonomic symptoms. Due to the gastroparesis, there was no progress with the oral diet, and nutrition was therefore supplied through a nasojejunal tube and gastrojejunal tube via Percutaneous endoscopic gastrostomy (PEG). After tube feeding for 9 months, the patient's gastrointestinal symptoms improved and his oral ingestion increased. The pediatric patient was maintained well without gastrointestinal symptoms for 3 months after removal of the PEG, had repeated vomiting episodes which lead to the suspicion of cyclic vomiting syndrome. Then he started treatment with low-dose amitriptyline, which resulted in improvement. Currently, the patient has been maintained well for 6 months without recurrence, and his present growth status is normal.
Amitriptyline ; Child* ; Diet ; Eating ; Enteral Nutrition ; Gastroparesis ; Gastrostomy ; Guillain-Barre Syndrome* ; Humans ; Male ; Primary Dysautonomias ; Recurrence ; Vomiting*

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Wilson's Disease in Bangladeshi Children: Analysis of 100 Cases.

Md RUKUNUZZAMAN

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):121-127. doi:10.5223/pghn.2015.18.2.121

PURPOSE: To evaluate clinical and laboratory profile of Wilson's disease (WD) in children. METHODS: This cross sectional study was conducted at Bangabandhu Sheikh Mujib Medical University Hospital. Bangladesh, over a period of 3 years. One hundred consecutive children of WD between 3 to 18 years of age were evaluated. RESULTS: Mean age was 8.5+/-1.5 years. Male female ratio was 2:1. Ninety-one percent of patients were Muslim and 9% Hindu. A total of 53% cases of hepatic WD presented between 5 to 10 years of age and most of the neurologic WD manifested in 10-15 years age group. Sixty-nine children presented only with hepatic manifestations, 6 only with neurological manifestations, 14 with both hepatic and neurological manifestation, 10 children was asymptomatic and 1 patient presented with psychiatric features. WD presented as chronic liver disease (CLD) in 42%, CLD with portal hypertension in 34%, acute hepatitis in 20% and fulminant hepatic failure in 4% cases. Stigmata of CLD were found in 18% patients. Keiser-Fleischser ring was found in 76% total patients. Elevated serum transaminase was found in 85% cases, prolonged prothrombin time in 59% cases and hypoalbuminaemia in 53% cases. A total of 73% patients had low serum ceruloplasmin, basal urinary copper of >100 microg/day was found in 81% cases and urinary copper following penicillamine challenge of >1,200 microg/day was found in 92% cases. CONCLUSION: Majority of studied WD children presented with hepatic manifestation of which 76% presented with CLD. Any child presented with jaundice after the age of 3 years should be investigated for WD.
Bangladesh ; Ceruloplasmin ; Child* ; Christianity ; Copper ; Female ; Hepatitis ; Hepatolenticular Degeneration* ; Humans ; Hypertension, Portal ; Islam ; Jaundice ; Liver Diseases ; Liver Failure, Acute ; Male ; Neurologic Manifestations ; Penicillamine ; Prothrombin Time

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Pediatric Cholecystectomy: Clinical Significance of Cases Unrelated to Hematologic Disorders.

Hae Young KIM ; Soo Hong KIM ; Yong Hoon CHO

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):115-120. doi:10.5223/pghn.2015.18.2.115

PURPOSE: Cholecystectomy is rarely performed in the child and adolescent. However, it is associated with several conditions. This study was conducted to describe the characteristics of pediatric patient who underwent cholecystectomy unrelated to hematologic disorders, and then to suggest its clinical significance in management by comparing a simple and complicated gallbladder disease. METHODS: We reviewed cases of cholecystectomy in pediatric patients (under 18 years old) at a single institution between January 2003 and October 2014. There were 143 cases during the study period and 24 were selected as the subject group. RESULTS: There were 7 male (29.2%) and 17 female (70.8%) patients. The mean age was 13.1 years old, and 66.6% of patients were older than 12 years. Mean body weight was 52.7 kg, and body mass index was 21.7 kg/m2, with 41.7% of patients being overweight or obese. We could identify a female predominance and high proportion of overweight or obesity in a complicated disease. There were also significantly increased levels of aspartate transaminase (AST), alanine transaminase (ALT), alkaline phosphatase (ALP) and bilirubin in this group. Most patients (87.5%) underwent laparoscopic cholecystectomy. CONCLUSION: Cholecystectomy for diseases unrelated to hematologic disorders is rarely performed in the child and adolescent. In general, female patients who are overweight or obese, and those older than 12 years old, require laparoscopic cholecystectomy owing to multiple gallstones. This condition has a tendency to show a complicated gallbladder disease and significantly increased levels of AST, ALT, ALP, and bilirubin.
Adolescent ; Alanine Transaminase ; Alkaline Phosphatase ; Aspartate Aminotransferases ; Bilirubin ; Body Mass Index ; Body Weight ; Child ; Cholecystectomy* ; Cholecystectomy, Laparoscopic ; Female ; Gallbladder Diseases ; Gallstones ; Humans ; Male ; Obesity ; Overweight

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Abdominal Subcutaneous Fat Thickness Measured by Ultrasonography Correlates with Hyperlipidemia and Steatohepatitis in Obese Children.

Sung Hyun LEE ; Dongwan KIM ; Min Young BAEK ; Hann TCHAH ; Yeon Sun KIM ; Eell RYOO ; Yun Mi KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):108-114. doi:10.5223/pghn.2015.18.2.108

PURPOSE: The aim of this study is to evaluate the relationship between abdominal subcutaneous fat thickness measured by ultrasonography (US) and serum lipid profile and liver transaminases in obese children. METHODS: One hundred and sixty-six children diagnosed with obesity from May 2001 to December 2013 were included in this study. Data on serum lipid profile and liver transaminases were collected from clinical records. Abdominal subcutaneous fat thickness and grade of hepatic steatosis were evaluated by US. RESULTS: Of the 166 children, 107 were diagnosed with hepatic steatosis by US, 46 with grade I, 56 with grade II, and five children with grade III. According to the grade of hepatic steasosis, the average values of midline abdominal subcutaneous fat thickness and right flank abdominal subcutaneous fat thickness measured 2.9+/-0.8 cm and 1.9+/-0.7 cm in the normal group, 3.3+/-0.8 cm and 2.0+/-0.7 cm in grade I, 3.8+/-0.8 cm and 2.3+/-0.8 cm in grade II, and 4.1+/-0.8 cm and 2.8+/-1.4 cm in grade III, respectively. Abdominal subcutaneous fat thickness correlated with grade of hepatic steatosis (p<0.01). In addition, abdominal subcutaneous fat thickness correlated with concentration of serum lipids and liver transaminases in the age group of 12-14 years (p<0.01). CONCLUSION: Abdominal subcutaneous fat thickness measured by US can be used as a reliable predictor of possible hyperlipidemia and steatohepatitis in children, especially during the adolescent stage.
Adolescent ; Child* ; Fatty Liver* ; Humans ; Hyperlipidemias* ; Liver ; Obesity ; Subcutaneous Fat, Abdominal* ; Transaminases ; Ultrasonography*

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The Iron Status of Very Low Birth Weight Infants Receiving Multiple Erythrocyte Transfusions during Hospitalization in the Neonatal Intensive Care Unit.

Sook Hyun PARK ; Heng Mi KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):100-107. doi:10.5223/pghn.2015.18.2.100

PURPOSE: We investigated the iron status of very low birth weight infants receiving multiple erythrocyte transfusions during hospitalization in the neonatal intensive care unit (NICU). METHODS: We enrolled 46 very low birth weight infants who were admitted to the Kyungpook National University Hospital between January 2012 and December 2013. Serum ferritin was measured on their first day of life and weekly thereafter. We collected individual data of the frequency and volume of erythrocyte transfusion and the amount of iron intake. RESULTS: A total of 38 (82.6%) of very low birth weight infants received a mean volume of 99.3+/-93.5 mL of erythrocyte transfusions in NICU. The minimum and maximum serum ferritin levels during hospitalization were 146.2+/-114.9 ng/mL and 456.7+/-361.9 ng/mL, respectively. The total volume of erythrocyte transfusion was not correlated to maximum serum ferritin concentrations after controlling for the amount of iron intake (r=0.012, p=0.945). Non-transfused infants took significantly higher iron intake compared to infants receiving > or =100 mL/kg erythrocyte transfusion (p<0.001). Minimum and maximum serum ferritin levels of non-transfused infants were higher than those of infants receiving <100 mL/kg erythrocyte transfusions (p=0.026 and p=0.022, respectively). Infants with morbidity including bronchopulmonary dysplasia or retinopathy of prematurity received a significantly higher volume of erythrocyte transfusions compared to infants without morbidity (p<0.001). CONCLUSION: Very low birth weight infants undergoing multiply erythrocyte transfusions had excessive iron stores and non-transfused infants also might had a risk of iron overload during hospitalization in the NICU.
Bronchopulmonary Dysplasia ; Erythrocyte Transfusion* ; Ferritins ; Gyeongsangbuk-do ; Hospitalization* ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal* ; Iron Overload ; Iron* ; Retinopathy of Prematurity

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Benefits of a Dedicated Breastfeeding Facility and Support Program for Exclusive Breastfeeding among Workers in Indonesia.

Ray W BASROWI ; Astrid B SULISTOMO ; Nuri Purwito ADI ; Yvan VANDENPLAS

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(2):94-99. doi:10.5223/pghn.2015.18.2.94

PURPOSE: A mother's working environment is believed to be a major determinant of exclusive breastfeeding (EBF) practice. We aimed to define the influence of a facility dedicated to breastfeeding and a breastfeeding support program at the workplace on breastfeeding practice. METHODS: A cross-sectional study was performed in five workplaces. The inclusion criteria were female workers whose last child was between 6 and 36 months old. Observational data were obtained and a questionnaire was filled out. The World Health Organization definition for EBF was used. RESULTS: Data from 186 subjects (74 office workers and 112 factory workers) were collected. Just over half (52%) of the mothers were between 20 and 46 years old, 75.3% had graduated from high school and university, 12.9% had more than two children and 36.0% owned a house. The prevalence of EBF during the last 6 months was 32.3%. A proper dedicated breastfeeding facility was available for 21.5% of the mothers, but only 7.5% had been in contact with a breastfeeding support program. The presence of a dedicated breastfeeding facility increased EBF practice almost threefold, by an odds ratio (OR) of 2.74 and a 95% confidence interval (CI) of 1.34-5.64 (p<0.05). Knowledge of the breastfeeding support program increased EBF practice by almost six times (OR, 5.93; 95% CI, 1.78-19.79) (p<0.05). CONCLUSION: Our findings suggest that Governments should make it obligatory for employers to offer a breastfeeding support program and a dedicated breastfeeding facility at the workplace as these simple measures significantly increase EBF.
Breast Feeding* ; Child ; Cross-Sectional Studies ; Female ; Humans ; Indonesia* ; Mothers ; Odds Ratio ; Prevalence ; Surveys and Questionnaires ; World Health Organization

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Middle East Consensus Statement on the Diagnosis and Management of Functional Gastrointestinal Disorders in <12 Months Old Infants.

Yvan VANDENPLAS ; Muath Abdurrahman ALTURAIKI ; Wafaa AL-QABANDI ; Fawaz ALREFAEE ; Ziad BASSIL ; Bassam EID ; Ahmed EL BELEIDY ; Ali Ibrahim ALMEHAIDIB ; Pierre MOUAWAD ; Maroun SOKHN

Pediatric Gastroenterology, Hepatology & Nutrition.2016;19(3):153-161. doi:10.5223/pghn.2016.19.3.153

This paper covers algorithms for the management of regurgitation, constipation and infantile colic in infants. Anti-regurgitation formula may be considered in infants with troublesome regurgitation, while diagnostic investigations or drug therapy are not indicated in the absence of warning signs. Although probiotics have shown some positive evidence for the management of functional gastrointestinal disorders (FGIDs), the evidence is not strong enough to make a recommendation. A partially hydrolyzed infant formula with prebiotics and β-palmitate may be considered as a dietary intervention for functional constipation in formula fed infants. Lactulose has been shown to be effective and safe in infants younger than 6 months that are constipated. Macrogol (polyethylene glycol, PEG) is not approved for use in infants less than 6 months of age. However, PEG is preferred over lactulose in infants >6 months of age. Limited data suggests that infant formula with a partial hydrolysate, galacto-oligosaccharides/fructo-oligosaccharides, added β-palmitate may be of benefit in reducing infantile colic in formula fed infants in cases where cow's milk protein allergy (CMPA) is not suspected. Evidence suggests that the use of extensively hydrolyzed infant formula for a formula-fed baby and a cow's milk free diet for a breastfeeding mother may be beneficial to decrease infantile colic if CMPA is suspected. None of the FGIDs is a reason to stop breastfeeding.
Breast Feeding ; Colic ; Consensus* ; Constipation ; Diagnosis* ; Diarrhea ; Diet ; Drug Therapy ; Gastrointestinal Diseases* ; Humans ; Hypersensitivity ; Infant Formula ; Infant* ; Lactulose ; Middle East* ; Milk ; Milk Proteins ; Mothers ; Polyethylene Glycols ; Prebiotics ; Probiotics

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