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Correction: 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 GAROFALO ; Elisabetta TERUZZI ; Simona VINARDI ; Giulia CARBONARO ; Alessia CERRINA ; Isabella MORRA ; Davide MONTIN ; Alessandro MUSSA ; Jurgen SCHLEEF

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):71-71. doi:10.5223/pghn.2015.18.1.71

The original version of this article, the second author name was erroneously misspelled.

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Suppurative Meckel Diiverticulum in a 3-Year-Old Girl Presenting with Periumbilical Cellulitis.

Ji Sook PARK ; Chun Woo LIM ; Taejin PARK ; Jae Min CHO ; Ji Hyun SEO ; Hee Shang YOUN

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):66-70. doi:10.5223/pghn.2015.18.1.66

Meckel diverticulum (MD) is one of the most common congenital gastrointestinal anomalies and occurs in 1.2-2% of the general population. MD usually presents with massive painless rectal bleeding, intestinal obstruction or inflammation in children and adults. Suppurative Meckel diverticulitis is uncommon in children. An experience is described of a 3-year-old girl with suppurative inflammation in a tip of MD. She complained of acute colicky abdominal pain, vomiting and periumbilical erythema. Laparoscopic surgery found a relatively long MD with necrotic and fluid-filled cystic end, which was attatched to abdominal wall caused by inflammation. Herein, we report an interesting and unusual case of a suppurative Meckel diverticulitis presenting as periumbilical cellulitis in a child. Because of its varied presentations, MD might always be considered as one of the differential diagonosis.
Abdominal Pain ; Abdominal Wall ; Adult ; Cellulitis* ; Child ; Child, Preschool* ; Diverticulitis ; Erythema ; Female ; Hemorrhage ; Humans ; Inflammation ; Intestinal Obstruction ; Laparoscopy ; Meckel Diverticulum ; Suppuration ; Vomiting

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Disseminated Cytomegalovirus Infection and Protein Losing Enteropathy as Presenting Feature of Pediatric Patient with Crohn's Disease.

Murat CAKIR ; Safak ERSOZ ; Ulas Emre AKBULUT

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):60-65. doi:10.5223/pghn.2015.18.1.60

We report a pediatric patient admitted with abdominal pain, diffuse lower extremity edema and watery diarrhea for two months. Laboratory findings including complete blood count, serum albumin, lipid and immunoglobulin levels were compatible with protein losing enteropathy. Colonoscopic examination revealed diffuse ulcers with smooth raised edge (like "punched out holes") in the colon and terminal ileum. Histopathological examination showed active colitis, ulcerations and inclusion bodies. Immunostaining for cytomegalovirus was positive. Despite supportive management, antiviral therapy, the clinical condition of the patient worsened and developed disseminated cytomegalovirus infection and the patient died. Protein losing enteropathy and disseminated cytomegalovirus infection a presenting of feature in steroid-naive patient with inflammatory bowel disease is very rare. Hypogammaglobulinemia associated with protein losing enteropathy in Crohn's disease may predispose the cytomegalovirus infection in previously healthy children.
Abdominal Pain ; Agammaglobulinemia ; Blood Cell Count ; Child ; Colitis ; Colon ; Crohn Disease* ; Cytomegalovirus ; Cytomegalovirus Infections* ; Diarrhea ; Edema ; Humans ; Ileum ; Immunoglobulins ; Inclusion Bodies ; Inflammatory Bowel Diseases ; Lower Extremity ; Protein-Losing Enteropathies* ; Serum Albumin ; Ulcer

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Achalasia Previously Diagnosed as Gastroesophageal Reflux Disease by Relying on Esophageal Impedance-pH Monitoring: Use of High-Resolution Esophageal Manometry in Children.

Jung Eun PYUN ; Da Min CHOI ; Jung Hwa LEE ; Kee Hwan YOO ; Jung Ok SHIM

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):55-59. doi:10.5223/pghn.2015.18.1.55

Gastroesophageal reflux disorder (GERD) is the most common esophageal disorder in children. Achalasia occurs less commonly but has similar symptoms to GERD. A nine-year old boy presented with vomiting, heartburn, and nocturnal cough. The esophageal impedance-pH monitor revealed nonacidic GERD (all-refluxate clearance percent time of 20.9%). His symptoms persisted despite medical treatment for GERD, and he was lost to follow up. Four years later, he presented with heartburn, solid-food dysphagia, daily post-prandial vomiting, and failure to thrive. Endoscopy showed a severely dilated esophagus with candidiasis. High-resolution manometry was performed, and he was diagnosed with classic achalasia (also known as type I). His symptoms resolved after two pneumatic dilatation procedures, and his weight and height began to catch up to his peers. Clinicians might consider using high-resolution manometry in children with atypical GERD even after evaluation with an impedance-pH monitor.
Candidiasis ; Child* ; Cough ; Deglutition Disorders ; Dilatation ; Electric Impedance ; Endoscopy ; Esophageal Achalasia* ; Esophageal pH Monitoring ; Esophagus ; Failure to Thrive ; Gastroesophageal Reflux* ; Heartburn ; Humans ; Lost to Follow-Up ; Male ; Manometry* ; Vomiting

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Liver Transplantation for Metabolic Liver Disease: Experience at a Living Donor Dominant Liver Transplantation Center.

Jun Suk KIM ; Kyung Mo KIM ; Seak Hee OH ; Hyun Jin KIM ; Jin Min CHO ; Han Wook YOO ; Jung Man NAMGOONG ; Dae Yeon KIM ; Ki Hun KIM ; Shin HWANG ; Sung Gyu LEE

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):48-54. doi:10.5223/pghn.2015.18.1.48

PURPOSE: Metabolic liver disease (MLD) often progresses to life-threatening conditions. This study intends to describe the outcomes of liver transplantation (LTx) for MLD at a living donor-dominant transplantation center where potentially heterozygous carrier grafts are employed. METHODS: We retrospectively evaluated the medical records of 54 patients with MLD who underwent LTx between November 1995 and February 2012 at Asan Medical Center in Seoul, Korea. The cumulative graft and patient survival rates were analyzed according to patient age, and living or deceased donor LTx. Recurrence of the original disease was also investigated. RESULTS: The post-transplant cumulative patient survival rates at one, five, and 10 years were 90.7%, 87.5% and 87.5%, and the graft survival rates were 88.8%, 85.5%, and 85.5%, respectively. There were no differences in the patient survival rates according to the recipient age, human leukocyte antigen matching, and living or deceased donor LTx. There were also no differences in the patient survival rates between the MLD and the non-MLD groups for children. Recurrence of the original metabolic disease was not observed in any patient during the follow-up period. CONCLUSION: Our results suggest that the living donor-dominant transplantation program is well-tolerated in MLD without recurrence of the original MLD using all types of transplantation.
Adult ; Child ; Chungcheongnam-do ; Follow-Up Studies ; Graft Survival ; Humans ; Korea ; Leukocytes ; Liver Diseases* ; Liver Transplantation* ; Living Donors* ; Medical Records ; Metabolic Diseases ; Recurrence ; Retrospective Studies ; Seoul ; Survival Rate ; Tissue Donors ; Transplants

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Laboratory Markers Indicating Gastrointestinal Involvement of Henoch-Schonlein Purpura in Children.

Jeana HONG ; Hye Ran YANG

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):39-47. doi:10.5223/pghn.2015.18.1.39

PURPOSE: To determine clinically useful biochemical markers reflecting disease activity and/or gastrointestinal (GI) tract involvement in Henoch-Schonlein purpura (HSP). METHODS: A total of 185 children with HSP and 130 controls were included. Laboratory data indicating inflammation, standard coagulation, and activated coagulation were analyzed for the HSP patients, including measurements of the hemoglobin level, white blood cell (WBC) count, absolute neutrophil count (ANC), platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, prothrombin time, activated partial thromboplastin time, and fibrinogen, D-dimer, and fibrin degradation product (FDP) levels. The clinical scores of the skin, joints, abdomen, and kidneys were assessed during the acute and convalescence phases of HSP. RESULTS: The WBC count, ANC, ESR, and CRP, fibrinogen, D-dimer, and FDP levels were significantly higher in the acute phase compared with the convalescent phase of HSP (p<0.05). The total clinical scores were more strongly correlated with the D-dimer (r=0.371, p<0.001) and FDP (r=0.369, p<0.001) levels than with inflammatory markers, such as the WBC count (r=0.241, p=0.001), ANC (r=0.261, p<0.001), and CRP (r=0.260, p<0.001) levels. The patients with GI symptoms had significantly higher ANC (median [interquartile range], 7,138.0 [4,446.4-9,470.0] vs. 5,534.1 [3,263.0-8,153.5], p<0.05) and CRP (0.49 [0.15-1.38] vs. 0.23 [0.01-0.67], p<0.05), D-dimer (2.63 [1.20-4.09] vs. 1.75 [0.62-3.39]), and FDP (7.10 [0.01-13.65] vs. 0.10 [0.01-7.90], p<0.05) levels than those without GI symptoms. CONCLUSION: D-dimer and FDPs are more strongly associated with disease activity and more consistently reflect GI involvement than inflammatory markers during the acute phase of HSP.
Abdomen ; Biomarkers* ; Blood Coagulation ; Blood Sedimentation ; C-Reactive Protein ; Child* ; Convalescence ; Fibrin ; Fibrin Fibrinogen Degradation Products ; Fibrinogen ; Humans ; Inflammation ; Joints ; Kidney ; Leukocytes ; Neutrophils ; Partial Thromboplastin Time ; Platelet Count ; Prothrombin Time ; Purpura ; Purpura, Schoenlein-Henoch* ; Skin

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Availability of Blood Urea Nitrogen/Creatinine Ratio in Gastrointestinal Bleeding with Melena in Children.

Kyu Seon KIM ; Chan Ho KANG ; Jae Young KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):30-38. doi:10.5223/pghn.2015.18.1.30

PURPOSE: The aims of our study were to evaluate the blood urea nitrogen to creatinine ratio (BUN/Cr ratio) for distinguishing between an upper and lower gastrointestinal bleeding (GIB), and differentiating between the two most common causes of upper gastrointestinal bleeding (UGIB) presenting with melena in children. METHODS: Retrospective data of patients with GIB presenting with melena were analyzed. The data from 60 cases were reviewed including demographics, laboratory findings, diagnostic modalities and results, treatments, and transfusions. RESULTS: Among the 60 cases, UGIB and lower gastrointestinal bleeding (LGIB) were found in 35 cases (58.3%) and 14 cases (23.3%), respectively. The two common causes of UGIB were varices (37.1%), and peptic ulcer diseases (PUD) (31.4%). The BUN/Cr ratio of 30 or greater was higher in UGIB than LGIB (odds ratio [OR], 6.9; 95% confidence interval [95% CI], 1.3-37.2). In UGIB, the BUN/Cr ratio of the varices group was higher than that of the PUD group (p=0.015). The OR for the BUN/Cr ratio appeared as 1.2 per unit increase in the varices group than the PUD group (95% CI, 1.03-1.3). There was no difference between the PUD group and Meckel's diverticulum group. CONCLUSION: The BUN/Cr ratio was not uneven in differentiating UGIB from LGIB of children with melena in our study. This suggests that BUN/Cr ratio should be interpreted carefully.
Blood Urea Nitrogen ; Child* ; Creatinine ; Demography ; Hemorrhage* ; Humans ; Meckel Diverticulum ; Melena* ; Peptic Ulcer ; Retrospective Studies ; Urea* ; Varicose Veins

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Clinical Characteristics of Celiac Disease and Dietary Adherence to Gluten-Free Diet among Saudi Children.

Ahmed Al SARKHY ; Mohammad I EL MOUZAN ; Elshazaly SAEED ; Aziz ALANAZI ; Sharifa ALGHAMDI ; Shirin ANIL ; Asaad ASSIRI

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):23-29. doi:10.5223/pghn.2015.18.1.23

PURPOSE: To describe the clinical characteristics of celiac disease (CD) among Saudi children and to determine the adherence rate to gluten free diet (GFD) and its determinant factors among them. METHODS: A cross-sectional study was conducted, in which all the families registered in the Saudi Celiac Patients Support Group were sent an online survey. Only families with children 18 years of age and younger with biopsy-confirmed CD were included. RESULTS: The median age of the 113 included children was 9.9 years, the median age at symptom onset was 5.5 years and the median age at diagnosis was 7 years, the median time between the presentation and the final diagnosis was 1 year. Sixty two of the involved children were females. Ninety two percent of the patients were symptomatic at the diagnosis while eight percent were asymptomatic. The commonest presenting symptoms included: chronic abdominal pain (59.3%), poor weight gain (54%), abdominal distention, gases, bloating (46.1%) and chronic diarrhea (41.6%). Sixty percent of the involved children were reported to be strictly adherent to GFD. Younger age at diagnosis and shorter duration since the diagnosis were associated with a better adherence rate. CONCLUSION: CD has similar clinical presentations among Saudi children compared to other parts of the ward; however, the adherence to GFD is relatively poor. Younger age at diagnosis and shorter duration since the diagnosis were associated with a better adherence rate.
Abdominal Pain ; Celiac Disease* ; Child* ; Cross-Sectional Studies ; Diagnosis ; Diarrhea ; Diet, Gluten-Free* ; Female ; Gases ; Humans ; Saudi Arabia ; Self-Help Groups ; Weight Gain

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The Efficacy of Saccharomyces boulardii CNCM I-745 in Addition to Standard Helicobacter pylori Eradication Treatment in Children.

Zhang BIN ; Xu YA-ZHENG ; Deng ZHAO-HUI ; Chu BO ; Jiang LI-RONG ; Yvan VANDENPLAS

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):17-22. doi:10.5223/pghn.2015.18.1.17

PURPOSE: This study aims to investigate Saccharomyces boulardii CNCM I-745 during Helicobacter pylori eradication in children. METHODS: One hundred ninety-four H. pylori positive children were randomized in two groups. Therapy (omeprazole+clarithromycin+amoxicillin or omeprazole+clarithromycin+metronidazole in case of penicillin allergy) was given to both groups during two weeks. In the treatment group (n: 102) S. boulardii was added to the triple therapy, while the control group (n: 92) only received triple therapy. The incidence, onset, duration and severity of diarrhea and compliance to the eradication treatment were compared. A 13C urea breath test was done 4 weeks after the end of eradication therapy in two groups of 21 patients aged 12 years and older to test the H. pylori eradication rate. RESULTS: In the treatment group, diarrhea occurred in 12 cases (11.76%), starting after 6.25+/-1.24 days, lasting 3.17+/-1.08 days, and compliance to eradication treatment was 100%. In the control group, diarrhea occurred in 26 cases (28.26%), starting after 4.05+/-1.11 days, lasting 4.02+/-0.87 days, and in six cases eradication treatment was stopped prematurely (p<0.05). The 13C urea breath test showed successful H. pylori eradication in 71.4% of the patients in the treatment and in 61.9 % in the control group (not significant). CONCLUSION: S. boulardii has a beneficial effect on the prevention and treatment of diarrhea during H. pylori eradication in children. Although S. boulardii did only slightly increase H. pylori eradication rate, compliance to eradication treatment was improved.
Breath Tests ; Child* ; Compliance ; Diarrhea ; Helicobacter pylori* ; Humans ; Incidence ; Penicillins ; Probiotics ; Saccharomyces* ; Urea ; Yeasts

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Changing Prevalence of Helicobacter pylori Infections in Korean Children with Recurrent Abdominal Pain.

Kyung Mi JANG ; Byung Ho CHOE ; Jae Young CHOE ; Suk Jin HONG ; Hyo Jung PARK ; Mi Ae CHU ; Seung Man CHO ; Jung Mi KIM

Pediatric Gastroenterology, Hepatology & Nutrition.2015;18(1):10-16. doi:10.5223/pghn.2015.18.1.10

PURPOSE: The aim of this study is to investigate the changing prevalence rate of Helicobacter pylori infection in children, of different age groups, with recurrent abdominal pain over a 10-year period. METHODS: Children with recurrent abdominal pain who visited the pediatric outpatient clinic at university hospital were screened for H. pylori. Children were divided into 3 age categories of 4-5, 6-11, and 12-16 years. To study the changes in the annual prevalence rates of H. pylori infection, the study period was divided into 3 time periods: 2004-2007, 2008-2010, and 2011-2014. Urea breath test was performed for all children aged 4-16 years, with a cut-off value of 4.0per thousand for children aged > or =6 years and 7per thousand for children aged <6 years. RESULTS: A total of 2,530 children (1,191 boys) with a mean age of 10.0+/-3.0 years (range, 4.0-16.9 years) were included in the study. The total prevalence of H. pylori infection was 7.4% (187/2,530). The prevalence rate of H. pylori infection in children with recurrent abdominal pain was 8.0% (70/873) in 2004-2007, 7.7% (51/666) in 2008-2010, and 6.7% (66/991) in the 2011-2014. Nevertheless, a significant difference was observed in the prevalence rate between children <12 years old and > or =12 years of age (p=0.018). CONCLUSION: The prevalence of H. pylori infection in Korean children with recurrent abdominal pain was 7.4%, showing no significant decrease in the last 11 years; however, the prevalence rate in children <12 years old was significantly lower than that in those > or =12 years old.
Abdominal Pain* ; Ambulatory Care Facilities ; Breath Tests ; Child* ; Endoscopy ; Gastrointestinal Diseases ; Helicobacter ; Helicobacter pylori* ; Humans ; Prevalence* ; Urea

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