Dyssynergic Defecation in Chronically Constipated Children in Korea / 대한소아소화기영양학회지

Purpose@#Dyssynergic defecation (DSD) is one of the important causes of chronic constipation in children. We aimed to analyze the clinical features, diagnostic test results, and treatments for DSD in children. @*Methods@#Children diagnosed with DSD using fluoroscopic defecography were enrolled in this study. Clinical data, including the results of colon transit time (CTT) test and biofeedback (BF) therapy, were collected from medical records retrospectively. @*Results@#Nineteen children were enrolled. The median age was 9 years (6–18 years), the median frequency of bowel movement was 1/7 days (1–10 days), the median duration of constipation was 7.0 years (2–18 years), the median age of onset of constipation was 2.5 years (1–11 years). In the CTT test, outlet obstruction type was noted in 10/18 (55.6%), slow transit type in 5/18 (27.8%), and normal transit in 1/18 (5.6%). The median CTT was 52 hours (40–142 hours). Initial medical therapy was performed with the polyethylene glycol 4000, and the response was good in 9/19 (47.4%), fair in 9/19 (47.4%), and poor in 1/19 (5.0%). BF was performed in 8/19, with good results in 6/8 (75.0%) children and failure in 2/8 (25.0%) children. After long-term medical therapy (11/19), 3/5 showed good response with medication alone, 6/8 showed good response with BF and medication combined. @*Conclusion@#DSD should be considered as a cause of chronic constipation in children, especially in those with abnormal CTT test results. BF combined with medical therapy is effective even with age-limited cooperation.

Incidence of Postnatal CMV Infection among Breastfed Preterm Infants:a Systematic Review and Meta-analysis

Background@#We performed a systematic review and meta-analysis to evaluate the incidence of breast milk-acquired cytomegalovirus (CMV) infection in preterm infants born to CMVseropositive mothers. @*Methods@#PubMed, Embase, and Cochrane Library databases were searched using the terms: (“breast feeding” or “breast milk” or “human milk” or “breast”) and (“HCMV” or “cytomegalovirus”) and (“infant, extremely premature” or “premature birth” or “newborn” or “neonate” or “low birth weight” or “very low birth weight” or “premature” or “preterm infant”). Studies that had information on CMV status and breast feeding were included in the meta-analysis. @*Results@#A total of 2,502 newborns from 19 studies were included in this meta-analysis. The rate of postnatally acquired CMV infection among breastfed infants with CMV-seropositive mothers was 16.5% (95% confidence interval [CI], 0.10–0.26; P < 0.001). The infection rate was 26% with fresh breast milk, 8% with a combined diet of fresh and freeze–thawed breast milk, and 11% with freeze–thawed breast milk. Among cases where the CMV status of breast milk was determined, CMV shedding into breast milk occurred in 80.5% (95% CI, 0.71–0.87; P < 0.001) of CMV seropositive mothers. The breast milk-acquired CMV infection rate among infants fed CMV-positive breast milk was 20.7% (95% CI, 0.14–0.30; P < 0.001). @*Conclusion@#This meta-analysis examined the rate of breast milk-acquired CMV infections in preterm infants with CMV-seropositive mothers; the CMV infection rate was higher in preterm infants fed fresh breast milk. Until further data are available, we cautiously suggest the use of freeze–thawed breast milk, rather than fresh breast milk, for preterm infants or very low birth weight infants.

Incidence of Postnatal CMV Infection among Breastfed Preterm Infants:a Systematic Review and Meta-analysis

Background@#We performed a systematic review and meta-analysis to evaluate the incidence of breast milk-acquired cytomegalovirus (CMV) infection in preterm infants born to CMVseropositive mothers. @*Methods@#PubMed, Embase, and Cochrane Library databases were searched using the terms: (“breast feeding” or “breast milk” or “human milk” or “breast”) and (“HCMV” or “cytomegalovirus”) and (“infant, extremely premature” or “premature birth” or “newborn” or “neonate” or “low birth weight” or “very low birth weight” or “premature” or “preterm infant”). Studies that had information on CMV status and breast feeding were included in the meta-analysis. @*Results@#A total of 2,502 newborns from 19 studies were included in this meta-analysis. The rate of postnatally acquired CMV infection among breastfed infants with CMV-seropositive mothers was 16.5% (95% confidence interval [CI], 0.10–0.26; P < 0.001). The infection rate was 26% with fresh breast milk, 8% with a combined diet of fresh and freeze–thawed breast milk, and 11% with freeze–thawed breast milk. Among cases where the CMV status of breast milk was determined, CMV shedding into breast milk occurred in 80.5% (95% CI, 0.71–0.87; P < 0.001) of CMV seropositive mothers. The breast milk-acquired CMV infection rate among infants fed CMV-positive breast milk was 20.7% (95% CI, 0.14–0.30; P < 0.001). @*Conclusion@#This meta-analysis examined the rate of breast milk-acquired CMV infections in preterm infants with CMV-seropositive mothers; the CMV infection rate was higher in preterm infants fed fresh breast milk. Until further data are available, we cautiously suggest the use of freeze–thawed breast milk, rather than fresh breast milk, for preterm infants or very low birth weight infants.

Clinical Features of Severely Constipated Children: Comparison of Infrequent Bowel Movement and Fecal Soiling Groups / 대한소아소화기영양학회지

Value of Fluoroscopic Defecography in Constipated Children With Abnormal Colon Transit Time Test Results

Background/Aims@#Colon transit time (CTT) test is regarded as the gold standard for evaluating colon transit function. Fluoroscopic defecography (FD) is a dynamic radiologic test to assess anorectal function. The aim is to evaluate the value of FD in constipated children with abnormal CTT test results. @*Methods@#Fifty-one children (27 girls) with a mean age of 9.8 ± 3.2 years who met Rome III criteria for constipation and older than 5 years with abnormal CTT test results underwent FD. @*Results@#Of 51 children, 27 (52.9%) showed positive findings on FD, including pelvic floor dyssynergia (PFD) (10/27, 37.0%), structural abnormality (15/27, 55.6%) (rectocele 53.3%, intussusception 33.3%, and both 13.4%), and both PFD and rectocele (2/27, 7.4%). In terms of CTT test subtype, of 35 children who had outlet obstruction type in CTT test, 19 (54.2%) had positive findings, including PFD (8/19, 42.1%), structural abnormality (9/19, 47.4%) (rectocele 55.6%, intussusception 22.2%, and both 22.2%), and both PFD and rectocele (2/19, 10.5%). Of the 16 children who had slow transit type of CTT test, 8 (50.0%) had positive findings, including PFD (2/8, 25.0%) and structural abnormality (6/8, 75.0%). Of the 6 children who had structural abnormality, 3 (50.0%) had rectocele and 3 (50.0%) had intussusception. For the 2 children (2/16, 12.5%) who had PFD, puborectalis muscle relax failure was found on FD. Puborectalis muscle relax failure was treated with biofeedback and medication. In the minor abnormalities, medication continued without additional therapeutic modalities. @*Conclusions@#FD was valuable for both diagnoses of underlying causes and interpretation of CTT test results in children with abnormal CTT test results. Therefore, this study suggests that FD and CTT tests should be incorporated into logical thinking for constipation in children.

Application of body composition zones in boys with nonalcoholic fatty liver disease

PURPOSE: Screening nonalcoholic fatty liver disease (NAFLD) by body mass index (BMI) as a single surrogate measure for obesity has limitations. We suggest considering body composition zones by drawing a body composition chart composed of body composition indices, including BMI and percent body fat (PBF), to visualize the risk of NAFLD in obese children and adolescents.METHODS: Thirty-eight boys diagnosed with NAFLD were selected retrospectively from patients who visited Konkuk University Medical Center from 2006 to 2015. They had gone through body composition analysis by bioelectrical impedance analysis (BIA), and biochemical analyses, including a liver function test (LFT) and lipid panel, were performed. Fat-free mass index (FFMI) and fat mass index (FMI) were calculated from body composition analysis and height. We plotted FFMI and FMI of patients on a body composition chart and classified the patients into zones A to D. In addition, we analyzed the correlations between LFT, lipid panel, and body composition indices.RESULTS: Thirty-three of 38 boys (86.8%) were located in zone C, corresponding to high BMI and PBF. Four boys (10.5%) were located in zone D, which correlates with sarcopenic obesity. One boy located in zone B was a muscular adolescent. Alanine aminotransferase level was positively correlated with PBF, FMI, and BMI z-score.CONCLUSION: Body composition zones on a body composition chart might be useful in risk assessment in obesity-related diseases such as NAFLD. Zones on a body composition chart could have practical applications, especially in sarcopenic obese children and adolescents.

Kawasaki Disease with Fever and Cervical Lymphadenopathy as the Sole Initial Presentation

BACKGROUND AND OBJECTIVES: Some patients with Kawasaki disease (KD) present with fever and cervical lymphadenopathy alone. The purpose of this study was to characterize the clinical features of these unusual KD patients and determine whether this is a severe form of KD associated with increased risks of intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs). SUBJECTS AND METHODS: A total of 146 children with KD were reviewed retrospectively, and classified into two groups according to initial clinical features. Those presenting with only fever and cervical lymphadenopathy (LKD) were classified as LKD patients. Other-KD patients included all except the LKD patients. RESULTS: Among 146 KD patients, 13 (8.9%) were classified as LKD patients. The LKD patients were significantly older and admitted earlier. The duration between fever onset and KD diagnosis was significantly longer in the LKD patients (5.9 days vs. 4.9 days, p=0.023). The frequency of IVIG resistance was not different between the two groups., In the LKD patients, the incidence of CALs was significantly higher in the acute phase, and without significant difference in the convalescent phase. The percentage of neutrophils and C-reactive protein, albumin, and total bilirubin levels were significantly higher in LKD patients. CONCLUSION: Even though LKD patients were older, admitted earlier, and had higher inflammatory marker levels, they did not have a greater risk of CALs or IVIG resistance. However, echocardiography may be helpful in the acute stage if patients have only fever and cervical lymphadenopathy and are unresponsive to empirical antibiotics.

Efficacy and Safety of Combined Oral and Enema Therapy Using Polyethylene Glycol 3350-Electrolyte for Disimpaction in Pediatric Constipation / 대한소아소화기영양학회지

PURPOSE: We evaluated the efficacy and safety of combined oral and enema therapy using polyethylene glycol (PEG) 3350 with electrolyte solution for disimpaction in hospitalized children. METHODS: We retrospectively studied 28 children having functional constipation who received inpatient treatment between 2008 and 2016. The amount of oral PEG 3350 electrolyte solution administered was 50–70 mL/kg/d (PEG 3350, 3–4.1 g/kg/d), and an enema solution was administered 1–2 times a day as a single dose of 15–25 mL/kg (PEG 3350, 0.975–1.625 g/kg/d). A colon transit time (CTT) test based on the Metcalf protocol was performed in some patients. RESULTS: Administration of oral and enema doses of PEG 3350 electrolyte solution showed 2.1±0.3 times and 2.9±0.4 times, respectively. After disimpaction, the frequency of defecation increased from 2.2±0.3 per week to once a day (1.1±0.1 per day). The number of patients who complained of abdominal pain was reduced from 15 (53.6%) to 4 (14.3%). Before hospitalization, nine patients underwent a CTT test, and 5 of 9 patients (55.6%) were classified as belonging to a group showing abnormalities. And in some patients, mild adverse effects were noted. We examined electrolytes and osmolality before and after disimpaction in 16 of 28 patients, and no abnormalities were noted. CONCLUSION: In terms of therapeutic efficacy and safety, combined oral and enema therapy using high-dose PEG 3350 with electrolytes is considered superior to conventional oral monotherapy or combined oral and enema therapy on an outpatient basis.

Efficacy and Safety of Combined Oral and Enema Therapy Using Polyethylene Glycol 3350-Electrolyte for Disimpaction in Pediatric Constipation / 대한소아소화기영양학회지

PURPOSE: We evaluated the efficacy and safety of combined oral and enema therapy using polyethylene glycol (PEG) 3350 with electrolyte solution for disimpaction in hospitalized children. METHODS: We retrospectively studied 28 children having functional constipation who received inpatient treatment between 2008 and 2016. The amount of oral PEG 3350 electrolyte solution administered was 50–70 mL/kg/d (PEG 3350, 3–4.1 g/kg/d), and an enema solution was administered 1–2 times a day as a single dose of 15–25 mL/kg (PEG 3350, 0.975–1.625 g/kg/d). A colon transit time (CTT) test based on the Metcalf protocol was performed in some patients. RESULTS: Administration of oral and enema doses of PEG 3350 electrolyte solution showed 2.1±0.3 times and 2.9±0.4 times, respectively. After disimpaction, the frequency of defecation increased from 2.2±0.3 per week to once a day (1.1±0.1 per day). The number of patients who complained of abdominal pain was reduced from 15 (53.6%) to 4 (14.3%). Before hospitalization, nine patients underwent a CTT test, and 5 of 9 patients (55.6%) were classified as belonging to a group showing abnormalities. And in some patients, mild adverse effects were noted. We examined electrolytes and osmolality before and after disimpaction in 16 of 28 patients, and no abnormalities were noted. CONCLUSION: In terms of therapeutic efficacy and safety, combined oral and enema therapy using high-dose PEG 3350 with electrolytes is considered superior to conventional oral monotherapy or combined oral and enema therapy on an outpatient basis.

Mesenteric Panniculitis in a Thirteen-Year-Old Korean Boy Treated with Prednisolone: A Case Report / 대한소아소화기영양학회지

Pediatric mesenteric panniculitis is an extremely rare disease of unknown etiology characterized by chronic inflammation, fat necrosis, and fibrosis in the mesenteric adipose tissue. A previously healthy 13-year-old boy was admitted because of right upper abdominal pain. An abdominal computed tomography scan revealed increased attenuation and enhancement in the left upper abdominal omental fat and anterior peritoneal wall thickening. A laparoscopic biopsy showed mesenteric panniculitis with chronic inflammation, adiponecrosis, and septal fibrosis. Serological tests for autoimmune diseases, nested polymerase chain reaction for Mycobacterium tuberculosis, and special immunohistochemical stains for malignancy were all negative. Symptomatic improvement and improved abnormal findings were achieved after an 8-month treatment with prednisolone according to a follow-up abdominal computed tomography scan. Here, we report a case of pediatric mesenteric panniculitis treated with prednisolone.

Colon Transit Time Test in Korean Children with Chronic Functional Constipation / 대한소아소화기영양학회지

PURPOSE: Each ethnic group has a unique life style, including diets. Life style affects bowel movement. The aim of this study is to describe the results of colon transit time (CTT) tests in Korean children who had chronic functional constipation based on highly refined data. METHODS: One hundred ninety (86 males) out of 415 children who performed a CTT test under the diagnosis of chronic constipation according to Rome III criteria at Konkuk University Medical Center from January 2006 through March 2015 were enrolled in this study. Two hundreds twenty-five children were excluded on the basis of CTT test result, defecation diary, and clinical setting. Shapiro-Wilk and Mann-Whitney U, and chi-square tests were used for statistical analysis. RESULTS: The median value and interquartile range (IQR) of CTT was 54 (37.5) hours in Encopresis group, and those in non-encopresis group was 40.2 (27.9) hours (p<0.001). The frequency of subtype between non-encopresis group and encopresis was statistically significant (p=0.002). The non-encopresis group (n=154, 81.1%) was divided into normal transit subgroup (n=84, 54.5%; median value and IQR of CTT=26.4 [9.6] hours), outlet obstruction subgroup (n=18, 11.7%; 62.4 [15.6] hours), and slow transit subgroup (n=52, 33.8%; 54.6 [21.0] hours]. The encopresis group (n=36, 18.9%) was divided into normal transit subgroup (n=8, 22.2%; median value and IQR of CTT=32.4 [9.9] hours), outlet obstruction subgroup (n=8, 22.2%; 67.8 [34.8] hours), and slow transit subgroup (n=20, 55.6%; 59.4 [62.7]hours). CONCLUSION: This study provided the basic pattern and value of the CTT test in Korean children with chronic constipation.

Value of 24-hour Delayed Film of Barium Enema for Evaluation of Colon Transit Function in Young Children with Constipation

BACKGROUND/AIMS: A colon transit time test using radio-opaque markers (CTTRM) is considered the gold standard for evaluating colon transit function. A 24-hour delayed film of barium enema (BE) has been used as a supplementary method in structural evaluations. The aim of this study was to evaluate the utility of a 24-hour delayed BE film for assessing colon transit function in young children with constipation. METHODS: In total, 93 children with constipation who performed both single-contrast BE and CTTRM were enrolled in this study. Of these, the data from 70 children were analyzed (males 33, females 37; mean age [range], 5.63 ± 2.94 [2-14] years). The basic principle of the study is "velocity = distance/time". Time values were identified in both studies, and the colon length and distance of barium movement were measured on the 24-hour delayed BE film. Thus, colon transit velocity values could be calculated using both methods. The correlation between colon transit velocity using a 24-hour delayed BE film versus CTTRM was analyzed statistically. RESULTS: Median value (interquartile range) of colon transit velocity using CTTRM was 1.57 (1.07-2.89) cm/hr, and that using BE of that was 1.58 (0.94-2.07) cm/hr. The Spearman correlation coefficient was 0.438 (P < 0.001) for the overall group. The correlation was strongest in children younger than 4 years (r = 0.537, P = 0.032). CONCLUSIONS: Although the correlation between BE and CTTRM was not very strong, the 24-hour delayed BE film could provide broad information about colon transit function in young children, especially those under 4 years who usually cannot undergo CTTRM.

Use of Omega-3 Polyunsaturated Fatty Acids to Treat Inspissated Bile Syndrome: A Case Report / 대한소아소화기영양학회지

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.

Correlation between Colon Transit Time Test Value and Initial Maintenance Dose of Laxative in Children with Chronic Functional Constipation / 대한소아소화기영양학회지

PURPOSE: To evaluate the correlation between colon transit time (CTT) test value and initial maintenance dose of polyethylene glycol (PEG) 4000 or lactulose. METHODS: Of 415 children with chronic functional constipation, 190 were enrolled based on exclusion criteria using the CTT test, defecation diary, and clinical chart. The CTT test was performed with prior disimpaction. The laxative dose for maintenance was determined on the basis of the defecation diary and clinical chart. The Shapiro-Wilk test and Pearson's and Spearman's correlations were used for statistical analysis. RESULTS: The overall group median value and interquartile range of the CTT test was 43.8 (31.8) hours. The average PEG 4000 dose for maintenance in the overall group was 0.68±0.18 g/kg/d; according to age, the dose was 0.73±0.16 g/kg/d (<8 years), 0.53±0.12 g/kg/d (8 to <12 years), and 0.36±0.05 g/kg/d (12 to 15 years). The dose of lactulose was 1.99±0.43 mL/kg/d (<8 years) or 1.26±0.25 mL/kg/d (8 to <12 years). There was no significant correlation between CTT test value and initial dose of laxative, irrespective of the subgroup (encopresis, abnormal CTT test subtype) for either laxative. Even in the largest group (overall, n=109, younger than 8 years and on PEG 4000), the correlation was weak (Pearson's correlation coefficient [R]=0.268, p=0.005). Within the abnormal transit group, subgroup (n=73, younger than 8 years and on PEG 4000) correlation was weak (R=0.267, p=0.022). CONCLUSION: CTT test value cannot predict the initial maintenance dose of PEG 4000 or lactulose with linear correlation.

Identification of a Novel De Novo Mutation of the TAZ Gene in a Korean Patient with Barth Syndrome

Barth syndrome (BTHS) is a rare genetic disorder characterized by various types of cardiomyopathy, neutropenia, failure to thrive, skeletal myopathy, and 3-methylglutaconic aciduria. BTHS is caused by loss-of-function mutations in the tafazzin (TAZ) gene located on chromosome Xq28, leading to cardiolipin deficiency. We report a 13-month-old boy with BTHS who had a novel de novo mutation in the TAZ gene. To the best of our knowledge, this is the first reported case of a BTHS patient with a de novo mutation in Korea. This report will contribute towards expanding the knowledge on the mutation spectrum of the TAZ gene in BTHS.

Ileal Fecaloma Presenting with Small Bowel Obstruction / 대한소아소화기영양학회지

A fecaloma refers to a mass of accumulated feces that is much harder than a mass associated with fecal impaction. Fecalomas are usually found in the rectosigmoid area. A 10-year-old male with chronic constipation was admitted because of increasing abdominal pain. An abdominal computed tomography scan and a simple abdominal x-ray revealed rapidly evolving mechanical obstruction in the small intestine. Most of the fecalomas are successfully treated by conservative methods such as laxatives, enemas and rectal evacuation. When conservative treatments have failed, surgical intervention may be needed. In this case, an emergency operation was performed and a 4x3x2.5 cm fecaloma was found in the distal ileum. We thus report a case of ileal fecaloma inducing small bowel obstruction in a patient with chronic constipation, who required surgical intervention. When symptoms of acute small intestinal obstruction develop in a patient with chronic constipation, a fecaloma should be considered in differential diagnosis.

Diets for Constipation / 대한소아소화기영양학회지

Chronic constipation is a very common disease in children. Successful treatment of constipation can be achieved not only with medication but also with lifestyle changes, including a proper diet. Diets including fruits, fluids, and probiotics are good for constipation. Some dietary components are helpful for constipation, and some are harmful. In this study, we present diets related to constipation from the literature, and propose some perspectives regarding diets related to constipation.

Omega-3 Polyunsaturated Fatty Acid for Cholestasis due to Bile Duct Paucity / 대한소아소화기영양학회지

Omega (omega)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nutrition-associated liver disease, and several mechanisms were proposed for this observation. An 8-week-old male infant with cholestasis and acholic stool was diagnosed non-syndromic intrahepatic interlobular bile duct paucity by open-wedge liver biopsy. Initially he was treated with usual supportive medical therapy, including ursodeoxycholic acid. However, the clinical status and laboratory tests did not improve. Omega (omega)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including aminotransferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effectiveness of omega-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease.

Recent Achievements in Stem Cell Therapy for Pediatric Gastrointestinal Tract Disease / 대한소아소화기영양학회지

The field of stem cell research has been rapidly expanding. Although the clinical usefulness of research remains to be ascertained through human trials, the use of stem cells as a therapeutic option for currently disabling diseases holds fascinating potential. Many pediatric gastrointestinal tract diseases have defect in enterocytes, enteric nervous system cells, smooth muscles, and interstitial cells of Cajal. Various kinds of therapeutic trials using stem cells could be applied to these diseases. This review article focuses on the recent achievements in stem cell applications for pediatric gastrointestinal tract diseases.

Identification of a Novel Mutation in the ATP7A Gene in a Korean Patient with Menkes Disease

Menkes disease is an infantile-onset X-linked recessive neurodegenerative disorder caused by diverse mutations in a copper-transport gene, ATP7A. Affected patients are characterized by progressive hypotonia, seizures, failure to thrive and death in early childhood. Here, we report a case of Menkes disease presented by intractable seizures and infantile spasms. A 3-month-old male infant had visited our pediatric clinic for lethargy, floppy muscle tone, poor oral intake and partial seizures. His hair was kinky, brown colored and fragile. Partial seizures became more frequent, generalized and intractable to antiseizure medications. An EEG showed frequent posteriorly dominant generalized spikes that were consistent with a generalized seizure. From a genetic analysis, a c.2743C>T (p.Gln915X) mutation was detected and diagnosed as Menkes disease. The mutation is a novel one that has not been previously reported as a cause of Menkes disease.