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Coexistence of Gilbert Syndrome and Hereditary Spherocytosis in a Child Presenting with Extreme Jaundice.

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):266-269. doi:10.5223/pghn.2014.17.4.266

Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyperbilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings.
Anemia ; Anemia, Hemolytic ; Bilirubin ; Child* ; Diagnosis ; Erythrocytes ; Gilbert Disease* ; Hemolysis ; Humans ; Hyperbilirubinemia ; Jaundice* ; Male ; Nausea ; Osmotic Fragility ; Spleen ; Splenomegaly ; Thalassemia

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A Case of Epiploic Appendagitis with Acute Gastroenteritis.

Min Sun CHO ; Seok HWANG-BO ; Ui Yoon CHOI ; Hwan Soo KIM ; Seung Hoon HAHN

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):263-265. doi:10.5223/pghn.2014.17.4.263

Epiploic appendagitis is an inflammation of the epiploic appendage in which the small sacs projecting from the serosal layer of the colon are positioned longitudinally from the caecum to the rectosigmoid area. Epiploic appendagitis is rare and self-limiting; however, it can cause sudden abdominal pain in children. Epiploic appendagitis does not typically accompany other gastrointestinal diseases. Here, we report on a healthy eight-year-old girl who presented with abdominal pain, fever, vomiting, and diarrhea. Based on these symptoms, she was diagnosed with acute gastroenteritis, but epiploic appendagitis in the ascending colon was revealed in contrast computed tomography (CT). The patient was treated successfully with conservative management. CT is beneficial in diagnosis and further assessment of epiploic appendagitis. Pediatricians need to be aware of this self-limiting disease and consider it as a possible alternate diagnosis in cases of acute abdominal pain.
Abdominal Pain ; Child ; Colon ; Colon, Ascending ; Diagnosis ; Diarrhea ; Female ; Fever ; Gastroenteritis* ; Gastrointestinal Diseases ; Humans ; Inflammation ; Vomiting

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The Complex Surgical Management of the First Case of Severe Combined Immunodeficiency and Multiple Intestinal Atresias Surviving after the Fourth Year of Life.

Riccardo GUANA ; Salvatore GAROFANO ; Elisabetta TERUZZI ; Simona VINARDI ; Giulia CARBONARO ; Alessia CERRINA ; Isabella MORRA ; Davide MONTIN ; Alessandro MUSSA ; Jurgen SCHLEEF

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):257-262. doi:10.5223/pghn.2014.17.4.257

Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastrointestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before the age of 3 months and without intervention SCID usually results in severe infections and death by the age of 2 years. Its association with intestinal anomalies as multiple intestinal atresias (MIA) is rare and worsens the prognosis, resulting lethal. We describe the case of a four year-old boy with SCID-MIA. He presented at birth with meconium peritonitis, multiple ileal atresias and underwent several intestinal resections. A targeted Sanger sequencing revealed a homozygous 4-bp deletion (c.313DeltaTATC; p.Y105fs) in tetratricopeptide repeat domain 7A (TTC7A). He experienced surgical procedures including resection and stricturoplasty. Despite parenteral nutrition-associated liver disease, the patient is surviving at the time of writing the report. Precocious immune system assessment, scrutiny of TTC7A mutations and prompt surgical procedures are crucial in the management.
B-Lymphocytes ; Humans ; Immune System ; Intestinal Atresia* ; Liver Diseases ; Male ; Meconium ; Parturition ; Peritonitis ; Prognosis ; Severe Combined Immunodeficiency* ; T-Lymphocytes ; Writing

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The Clinical Significance of Serum Ferritin in Pediatric Non-Alcoholic Fatty Liver Disease.

Ji Hoon NA ; So Won PARK ; Yunkoo KANG ; Hong KOH ; Seung KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):248-256. doi:10.5223/pghn.2014.17.4.248

PURPOSE: Non-alcoholic fatty liver disease (NAFLD) in children has become an important public health issue because of its high prevalence and severity. Several noninvasive methods for estimating NAFLD are under investigation. We aimed to evaluate the usefulness of serum ferritin as a biomarker of severity of pediatric NAFLD patients. METHODS: A total of 64 NAFLD patient were enrolled from Severance Children's Hospital from March 2010 to February 2013. Serum ferritin levels, liver related laboratory tests, liver magnetic resonance imaging (MRI) (2-dimensional [2D] proton density-fat fraction) and NAFLD severity markers were compared between obese group and overweight group. Correlation analyses were performed between serum ferritin and laboratory values including NAFLD severity markers. RESULTS: In obese group, serum ferritin, alanine aminotransferase (ALT), total bilirubin, international normalized ratio (INR), MRI 2D proton density-fat fraction, aspartate aminotransferase (AST) to platelet ratio index (APRI) and fibrosis-4 (FIB-4) (an index score calculated from platelet count, ALT, AST and age) were significantly higher than those of overweight group. NAFLD severity markers, APRI and FIB-4, and liver specific important laboratory values, AST, ALT, INR, cholesterol, triglyceride and low density lipoprotein show significant correlation with serum ferritin in NAFLD patients. CONCLUSION: Serum ferritin concentrations could be a candidate of useful severity marker in the pediatric NAFLD patients.
Alanine Transaminase ; Aspartate Aminotransferases ; Bilirubin ; Biomarkers ; Blood Platelets ; Child ; Cholesterol ; Fatty Liver* ; Ferritins* ; Humans ; International Normalized Ratio ; Lipoproteins ; Liver ; Magnetic Resonance Imaging ; Obesity ; Overweight ; Platelet Count ; Prevalence ; Protons ; Public Health ; Triglycerides

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Does Type I Truly Dominate Hepatic Glycogen Storage Diseases in Korea?: A Single Center Study.

Yu Ju JEONG ; Ben KANG ; So Yoon CHOI ; Chang Seok KI ; Soo Youn LEE ; Hyung Doo PARK ; Yon Ho CHOE

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):239-247. doi:10.5223/pghn.2014.17.4.239

PURPOSE: There are no studies of hepatic glycogen storage diseases (GSDs) other than type I and III in Korea. We aimed on investigating the characteristics of hepatic GSDs in Korea diagnosed and followed at a single center. METHODS: We retrospectively analyzed patients who were diagnosed as GSD and followed at Samsung Medical Center from January, 1997 to December, 2013. Clinical manifestations, laboratory results, treatment, and prognosis were investigated. RESULTS: Twenty-one patients were included in the study. The types of 17 patients were confirmed by enzyme activity tests and/or gene analysis. GSD Ia was diagnosed in 7 patients (33.3%), Ib in 1 patient (4.8%), III in 2 patients (9.5%), IV in 1 patient (4.8%), and IX in 6 patients (28.6%). Types other than GSD I constituted 52.9% (9/17) of the patients diagnosed with a specific type of hepatic GSD. The median age at presentation was 2 years. Hepatomegaly was observed in 95.2%, elevated liver transaminases in 90.5%, and hyperlactacidemia in 81.0% of the patients. The duration for follow-up was 77+/-62.0 months. Uncooked corn starch was initiated in all the patients. No mortality was observed during the follow-up period, and liver transplantation was performed in 14.3%. CONCLUSION: Types other than GSD I comprised more than half of the patients diagnosed with a specific type of hepatic GSD. Clinical suspicion and thorough evaluation of hepatic GSDs in Korea should be focused not only on GSD I, but also on other types.
Follow-Up Studies ; Glycogen Storage Disease ; Hepatomegaly ; Humans ; Korea ; Liver ; Liver Glycogen* ; Liver Transplantation ; Mortality ; Prognosis ; Retrospective Studies ; Starch ; Transaminases ; Zea mays

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Clinical Characteristics of Symptomatic Clostridium difficile Infection in Children: Conditions as Infection Risks and Whether Probiotics Is Effective.

Jae Yoon NA ; Jong Mo PARK ; Kyung Suk LEE ; Jung Oak KANG ; Sung Hee OH ; Yong Joo KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):232-238. doi:10.5223/pghn.2014.17.4.232

PURPOSE: This study investigated the clinical presentations of symptomatic Clostridium difficile infection (CDI) in children. METHODS: We reviewed the medical records of 43 children aged <20 years who showed either positive C. difficile culture or C. difficile toxin test results between June 2010 and April 2014. RESULTS: Of the 43 patients (mean age 6.7 years), 22 were boys. Sixteen patients (37.2%) showed both positive C. difficile culture and toxin test results. Seventeen out of 43 children (39.5%) had preexisting gastrointestinal diseases, and 26 children had other medical conditions that were risk factors for CDI. Twenty-eight children had a history of antibiotic treatment for >3 days, and the most frequently prescribed antibiotic was amoxicillin-clavulanate (35.7%). Twenty-eight patients were diagnosed with CDI despite taking probiotic supplements, most commonly Lactobacillus acidophilus (53.6%). The most common symptom was diarrhea (72.1%) at the time CDI was diagnosed. C. difficile was eradicated in 11 patients (25.6%) after treatment with oral metronidazole for 10-14 days, and in the two patients (4.6%) who required two courses of oral metronidazole. Sixteen patients (37.2%) showed clinical improvement without any treatment. CONCLUSION: This study showed the various clinical characteristics of CDI in children and that preexisting clinical conditions favored the development of CDI. In addition, CDI was found to occur in a number of patients even after probiotic prophylaxis given in conjunction with antibiotic therapy.
Child* ; Clostridium difficile* ; Diarrhea ; Gastrointestinal Diseases ; Humans ; Lactobacillus acidophilus ; Medical Records ; Metronidazole ; Probiotics* ; Risk Factors

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Systemic Classification for a New Diagnostic Approach to Acute Abdominal Pain in Children.

Ji Hoi KIM ; Hyun Sik KANG ; Kyung Hee HAN ; Seung Hyo KIM ; Kyung Sue SHIN ; Mu Suk LEE ; In Ho JEONG ; Young Sil KIM ; Ki Soo KANG

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):223-231. doi:10.5223/pghn.2014.17.4.223

PURPOSE: With previous methods based on only age and location, there are many difficulties in identifying the etiology of acute abdominal pain in children. We sought to develop a new systematic classification of acute abdominal pain and to give some helps to physicians encountering difficulties in diagnoses. METHODS: From March 2005 to May 2010, clinical data were collected retrospectively from 442 children hospitalized due to acute abdominal pain with no apparent underlying disease. According to the final diagnoses, diseases that caused acute abdominal pain were classified into nine groups. RESULTS: The nine groups were group I "catastrophic surgical abdomen" (7 patients, 1.6%), group II "acute appendicitis and mesenteric lymphadenitis" (56 patients, 12.7%), group III "intestinal obstruction" (57 patients, 12.9%), group IV "viral and bacterial acute gastroenteritis" (90 patients, 20.4%), group V "peptic ulcer and gastroduodenitis" (66 patients, 14.9%), group VI "hepatobiliary and pancreatic disease" (14 patients, 3.2%), group VII "febrile viral illness and extraintestinal infection" (69 patients, 15.6%), group VIII "functional gastrointestinal disorder (acute manifestation)" (20 patients, 4.5%), and group IX "unclassified acute abdominal pain" (63 patients, 14.3%). Four patients were enrolled in two disease groups each. CONCLUSION: Patients were distributed unevenly across the nine groups of acute abdominal pain. In particular, the "unclassified abdominal pain" only group was not uncommon. Considering a systemic classification for acute abdominal pain may be helpful in the diagnostic approach in children.
Abdominal Pain* ; Appendicitis ; Child* ; Classification* ; Diagnosis ; Humans ; Retrospective Studies ; Ulcer

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Short-Term Complications of Percutaneous Endoscopic Gastrostomy according to the Type of Technique.

Mi Hyeon GANG ; Jae Young KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):214-222. doi:10.5223/pghn.2014.17.4.214

PURPOSE: The method of percutaneous endoscopic gastrostomy (PEG) tube placement can be divided into the pull and introducer techniques. We compared short-term complications and prognosis between patients who underwent the pull technique and two other types of introducer techniques, the trocar introducer technique and T-fastener gastropexy technique. METHODS: Twenty-six patients who underwent PEG were enrolled in this study. We retrospectively investigated the age, sex, body weight, weight-for-age Z-score, underlying diseases, PEG indications, complications, duration of NPO (nil per os), pain control frequency, and duration of antibiotic therapy. The patients were classified into three groups according to the PEG technique. The occurrence of complications was monitored for 10 weeks after the procedure. RESULTS: The age, sex, body weight, and weight-for-age Z-score were not significantly between the three groups. Most patients had cerebral palsy and seizure disorders. Dysphagia was the most common indication for PEG. Major complications occurred in 5 (50%), 4 (66.7%), and 0 (0%) patients in group I, II, and III, respectively (p=0.005). Further, peristomal infection requiring systemic antibiotic therapy occurred in 2 (20%), 3 (50%), and 0 (0%) patients in group I, II, and III, respectively (p=0.04). There was no significant difference between the groups with respect to minor complications, duration of NPO, pain control frequency, and duration of antibiotic therapy. CONCLUSION: The results indicate that the T-fastener gastropexy technique was associated with the lowest rate of major complications.
Body Weight ; Cerebral Palsy ; Deglutition Disorders ; Epilepsy ; Gastropexy ; Gastrostomy* ; Humans ; Prognosis ; Retrospective Studies ; Surgical Instruments

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Clonazepam Treatment of Pathologic Aerophagia in Children with Mental Retardation.

Ga Hyun LEE ; Hyo Jeong JANG ; Jin Bok HWANG

Pediatric Gastroenterology, Hepatology & Nutrition.2014;17(4):209-213. doi:10.5223/pghn.2014.17.4.209

PURPOSE: Pathologic aerophagia (PA) may lead to bowel perforation or volvulus in mentally retarded patients. The authors investigated the effects of clonazepam on the management of PA in children with severe to profound mental retardation (MR). METHODS: This study was undertaken as a retrospective case analysis of 21 PA patients with MR who were followed for over 12 months and diagnosed as having PA. Patients were assigned to two management groups, that is, to a clonazepam randomized open-labeled, treatment group or a reassurance group. The following were recorded and analyzed; age, response, remission rate to clonazepam treatment, and the side effect of clonazepam. It was defined positive response (response+) as being symptom-free for a whole week within 1 month of commencing treatment and remission(+) as being symptom-free for a whole month within 6 months of treatment. RESULTS: The average age of the 21 PA children with MR was 10 years and 13 patients were female. Symptom duration before diagnosis of PA was 7 months. Clinical features of the clonazepam-trial group (n=11) and the reassurance group (n=10) were non-significantly different. Response(+) was achieved by 2 patients (18.2%) in the clonazepam-trial group and by no patient in the reassurance group. Remission(+) was achieved by 6 patients (54.5%) in the clonazepam-trial group and by one patient (10%) in the reassurance group (p=0.040). CONCLUSION: When PA children with MR with severe bowel distention are considered for surgical treatment to prevent acute abdomen, a trial of clonazepam could be recommended.
Abdomen, Acute ; Aerophagy ; Child* ; Clonazepam* ; Diagnosis ; Female ; Humans ; Intellectual Disability* ; Intestinal Volvulus ; Mentally Disabled Persons ; Retrospective Studies

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Failure of Fecal Microbiota Transplantation in a Three-Year-Old Child with Severe Refractory Ulcerative Colitis.

Hideki KUMAGAI ; Koji YOKOYAMA ; Tomoyuki IMAGAWA ; Shun INOUE ; Janyerkye TULYEU ; Mamoru TANAKA ; Takanori YAMAGATA

Pediatric Gastroenterology, Hepatology & Nutrition.2016;19(3):214-220. doi:10.5223/pghn.2016.19.3.214

Fecal microbiota transplantation (FMT) is a treatment designed to correct gut dysbiosis by administration of feces from a healthy volunteer. It is still unclear whether FMT for children with ulcerative colitis (UC) is effective or hazardous. Here we describe a young patient to have received FMT for UC. A three-year-old girl was admitted to our hospital with severe active UC, and treated with aminosalicylates and various immunosuppressive drugs. As remission was not achieved, we decided to try FMT before colectomy. We administered donor fecal material a total of six times by retention enema (×2) and via a nasoduodenal tube (×4) within 10 days. The patient developed abdominal pain and pyrexia after each FMT session. Analyses revealed the transferred donor fecal microbiota had not been retained by the patient, who ultimately underwent colectomy. The severity of the UC and/or timing of FMT may have partly accounted for the poor outcome.
Abdominal Pain ; Child* ; Colectomy ; Colitis, Ulcerative* ; Dysbiosis ; Enema ; Fecal Microbiota Transplantation* ; Feces ; Female ; Fever ; Gastrointestinal Microbiome ; Healthy Volunteers ; Humans ; Inflammatory Bowel Diseases ; Microbiota ; Tissue Donors ; Ulcer*

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