• Pediatric Infection and Vaccine
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• v.28 no.2
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• pp.110-117
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• 2021

Toxic megacolon is a fatal complication of inflammatory or infectious bowel disease. Prognosis depends on the severity of the disease. In cases of poor prognosis, clinical outcomes range from intestinal resection to death, so early diagnosis and appropriate treatment are very important. However, the prevalence of toxic megacolon in children is very low, and in those without underlying diseases such as inflammatory bowel disease, early diagnosis may be delayed. A previously healthy 12-year-old boy presented to our hospital with lower abdomen pain, fever, and hematochezia. Despite antibiotic therapy, the symptoms worsened. On the third day, abdominal computed tomography revealed severe dilatation of the transverse colon, which indicated toxic megacolon. Stool culture was positive for Salmonella enteritidis group D, and rectal endoscopy showed no signs of inflammatory bowel disease. Ceftriaxone and intravenous methylprednisolone were administered, and the patient's condition improved without any complications. We report a case of toxic megacolon as a complication of infectious colitis caused by S. enteritidis group D, which was diagnosed using early imaging and successfully treated without surgical intervention.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.256-261
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• 1999

Ileus caused by impaction of hardened residual barium stagnated in the colon, a rare complication of barium-enema examination, constitutes a particularly serious problem when it occurs in congenital megacolon patients. The administration of gastrografin in such case has proven efficacy in dissolution of impacted barium refratory to routine means of evacuation. We experienced a case of barium-impaction occurred in 6-months old male who had congenital megacolon. Gastrografin enema was a safe and simple method used in the management of this case.

• Journal of Veterinary Clinics
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• v.31 no.5
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• pp.454-456
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• 2014

A 9-month-old intact male Persian cat (3.2 kg of body weight) was referred with primary complaint of constipation. Diagnostic studies found severe constipation and megacolon. After immediate medical treatment (e.g. enema), the physical examination was performed and revealed that the unusual urethral opening was found approximately 0.5 cm ventral to the tip of the penis where a 3.5 fr tomcat catheter was easily advanced into the bladder. The case was diagnosed as hypospadias. The cat was treated with castration and medical therapy for constipation. To our best knowledge, this case is the first case report for feline hypospadias complicated with chronic constipation in Korea.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.446-450
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• 2006

Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect in the migration of neuronal cells from the neural crest (neurocristopathy) during the intrauterine period. A diagnosis of CCHS is made by confirming the failure of adequate ventilation in response to hypercapnea and hypoxia during sleep and the exclusion of other diseases. Young infants frequently show atypical clinical courses, and their conditions are frequently complicated with the long-term sequela of hypoxemic episodes. Therefore, a high index of suspicion and active treatment with mechanical ventilation are important for reducing recurrent hypoxemic episodes in the neonatal period. This paper reports the follow up of a case of CCHS in a neonate who showed frequent intractable apnea and cyanosis and was given artificial mechanical ventilation during sleep.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.1
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• pp.80-84
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• 2014

Intractable ulcerating enterocolitis is an uncommon inflammatory bowel disease syndrome of neonatal onset first described in 1991. Intractable ulcerating enterocolitis usually presents in the neonate with a mouth ulceration and the subsequent development of perianal disease and colitis. In this case report, an infant, 18 days from birth, with ulcerative lesion on hard palate for systemic differential diagnosis about oral lesion is referred from the department depiatrics. At that time, there is no abnormality, except oral lesion-like Aphthous ulcer. The patient was discharged from pediatrics, but returned to the hospital 3weeks later with blood diarrhea. As a result of endoscopy, there were large ulcerating lesions and the patient was diagnosed intractable ulcerating enterocolitis. Early recognition of Intractable ulcerating enterocolitis appears to be beneficial because colectomy, as opposed to immunosuppression, appears to be effective in controlling disease symptoms and progression. Most of the infants who were affected intractable ulcerating enterocolitis were normal at birth and oral manifestation appeared earlier than others. So, it is very meaningful for dentists to know about Intractable ulcerating enterocolitis.

• Journal of Yeungnam Medical Science
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• v.4 no.1
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• pp.33-42
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• 1987

Congenital origin intestinal obstruction are important disease due to required emergency operation. So accurate and rapid diagnosis is needed for decreased mortality and morbidity. Radiologist must detect to accurate obstruction site and also associated other congenital anomalies. And also embriological bases are very important role to the diagnosis of theses diseases. We were analysed radiologically and clinically 25 cases with congenital origin intestinal obstruction with review of literature. 1. Hypertrophic pyloric stenosis 6 cases, midgut malrotation 4 cases, congenital megacolon 8 case, imperforated anus 5 cases, ileal atresia 1 case and duodenal atresia 1 case. 2. Male and female radio were 16:9. Especially on hypertrophic pyloric stenosis, 5 cases were male infants. 3. All cases of hypertrophic pyloric stenosis represented string sign and also pyloric beak sign shoulder sign on UGI. 4. I case duodenal atresia showed double bubble sign on simple abdomen x-ray and ileal atresia showed mechanical small bowel obstruction sign with microcolon. 5. On midgut malrotation, cecum was located in right upper abdomen on 4 cases. And 2 cases were associated with Ladd's band. I case with volvulus and 1 case with mesenteric defect. 6. Involved site of all congenital megacolon were localized to rectosigmoid colon. 7. On 5 cases imperforated anus, 3 cases were low type and 2 case high type. Rectoperineal and rectourogenital fistula were demonstrated on 4 cases.

• Advances in pediatric surgery
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• v.4 no.2
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• pp.172-175
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• 1998

With the recent advances in instruments and techniques, laparoscopic procedure have extended to neonates with congenital anomalies. The author reports a 6-day-old boy with Hirschsprung's disease, treated successfully by the laparoscopic endorectall pull-through procedure. The technique and its potential role in the treatment of Hirschsprung's disease are described. One camera port and three working ports were used for access to the peritoneal cavity. The descending and sigmoid colon were mobilized laparoscopically. The submucosal dissection was done transanally. The colon was then pulled down in continuity, divided above the transition zone, and secured to the anal mucosa about 10 mm above the pectinate line. Author concluded that endorectal pull-through can be performed safety with the laparoscope.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.202-207
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• 2003

Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.113-118
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• 2002

The etiology of several motility disorders, including persistent megacolon after definitive surgery for Hirschsprung's disease, meconium ileus which is not associated with cystic fibrosis and idiopathic megacolon, is still unclear. Interstitial cells of Cajal (ICC) are thought to modulate gut motility as gastrointestinal pace maker cells. The aim of this study was to evaluate the role of ICC in the bowel walls of the patients (n=15) who had variable motility disorders. The ICC were identified by immunohistochemical staining using an anti-C-Kit antibody and the results were compared with control specimens (n=2). The control group (G1) showed evenly distributed ICC in their bowel walls. The second group (G2, n=5) who had normal bowel movements after Duhamel procedures and the third group (G3, n=4) who had persistent megacolon after Duhamel procedures showed absent or scarcely distributed ICC in their aganglionic bowels. The ICC were immunohistochemical staining using an anti-C-Kit antibody and the results were compared with control specimens (n=2). The control group (G1) showed evenly distributed ICC in their bowel walls. The second group (G2, n=5) who had normal bowel movements after Duhamel procedures and the third group (G3, n=4) who had persistent megacolon after Duhamel procedures showed absent or scarcely distributed ICC in their aganglionic bowels. Whereas ICC were evenly distributed in the ganglionic bowels of G2, they were not seen or scarecely distributed in the ganglionic bowels of G3. Two patients (G4) who suffered from idiopathic megacolon showed absence or decrease of ICC in spite of presence of ganglion cells in their colons. Four neonates (G5) who underwent ileostomy because of meconium obstruction showed absent or markedly decreased ICC in the the colon at the time of ileostomy and the distribution of ICC was changed to a normal pattern at the time of ileostomy closure between 39-104 days of age and their bowel motility were restored after that. The results suggest that lack of ICC caused reduce motility in the ganglionic colons and it may be responsible for the development of various motility disorders. Delayed maturity of ICC may also playa role in the meconium obstruction of neinates.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.284-287
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• 2018

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutation of one of two genes, TSC1 (encoding hamartin, 9q34) and TSC2 (encoding tuberin, 16p13). It invades the central nervous system and various parts of the body, causing various symptoms. Crohn's disease (CD) is a chronic immune-mediated disease that has not been clearly elucidated. It is thought to be caused by an excessive immune response of the body to bacteria that normally exist in the digestive tract with genetic factors. No cases have been reported in which both of the above-mentioned diseases occurred simultaneously. We report a case of CD in a patient with TSC. A 12-year-old boy was brought to our hospital because of abdominal pain. Skin lesions were observed in the TSC. Fundus examination revealed a hamartoma in the right retina. Brain magnetic resonance imaging revealed a subendothelial giant cell astrocytoma (SEGA). On the basis of these findings, he was diagnosed as having TSC. Blood test results showed increased levels of inflammatory markers. On abdominal ultrasonography, his colon walls were observed to be thickened with increased vascularity of the proximal ascending colon, ileocecal valve, and terminal ileum. Colonoscopy revealed discontinuous ulcerations and inflammations of the ileum, IC valve, and cecum, similar to those found in CD. Everolimus was administered orally for the SEGA but was discontinued frequently owing to the exacerbation of CD. The possibility of CD should be kept in mind in patients with TSC considering to undergo treatment for SEGA.