• Clinical and Experimental Pediatrics
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• v.53 no.9
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• pp.824-829
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• 2010

The safety and effectiveness of colonoscopy in the investigation of lower gastrointestinal tract pathology in children has been established for more than 2 decades in Korea. The skill and experience have since advanced to the point that both diagnostic and therapeutic colonoscopy are now routinely performed by most pediatric gastroenterologists. Pediatric colonoscopy differs significantly from its adult parallels in nearly every aspect including patient and parent management and preparation, selection criteria for sedation and general anesthetic, bowel preparation, expected diagnoses, instrument selection, imperative for terminal ileal intubation, and requirement for biopsies from macroscopically normal mucosa. Investigation of inflammatory bowel disease, whether for diagnosis or follow-up evaluation, and suspected colonic polyps are the most common indication for pediatric colonoscopy. The child who presents with signs and symptoms of lower gastrointestinal disorder should undergo colonoscopy with biopsy to make the diagnosis, as well as to help determine the appropriate therapy. This review introduces practical information on pediatric colonoscopy, the author's experiences, and the role of colonoscopic examination in the diagnosis and treatment of pediatric lower gastrointestinal disorders.

• Advances in pediatric surgery
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• v.4 no.1
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• pp.74-78
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• 1998

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. If there are intervening ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the disease. There were aganglionic segments from the anal margin to the terminal ileum 3.7cm proximal to the ileocecal valve. The entire transverse colon and appendix were normally ganglionated.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.1
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• pp.72-79
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• 2019

Purpose: The efficacy of exclusive enteral nutrition (EEN) with a polymeric diet has not been confirmed in Korean pediatric patients with Crohn's disease (CD). This study aimed to compare the effectiveness of EEN with a specific polymeric diet ($Encover^{(R)}$) and corticosteroids (CSs) for the induction of remission in Korean pediatric CD patients. Methods: We retrospectively compared data from 51 pediatric CD patients who underwent induction therapy with EEN (n=19) or CSs (n=32) at Severance Children's Hospital or Incheon St. Mary's Hospital in Korea. The primary endpoint of this study was the rate of clinical remission, defined as a Pediatric Crohn's Disease Activity Index (PCDAI) score <10, after 8 weeks of induction treatment. Clinical, laboratory, and growth data at post-induction as well as their changes from baseline were also compared between groups. Results: After 8 weeks of induction therapy, clinical remission rates were 78.9% (15/19) and 65.6% (21/32) in the EEN and CS groups, respectively (p=0.313). No significant differences in PCDAI scores, laboratory variables, and growth parameters were noted between the two groups at post-induction. However, significant changes in albumin levels at post-induction were observed in the EEN group compared to the CS group (p=0.038). Conclusion: Our results suggest that the effectiveness of EEN with a polymeric diet and CSs for induction therapy did not differ in Korean pediatric CD patients. EEN with a polymeric diet is a good first-line treatment option for the induction of remission in these patients.

• Clinical and Experimental Pediatrics
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• v.57 no.11
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• pp.465-471
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• 2014

Inflammatory bowel disease (IBD) is a chronic relapsing disorder of unknown etiology, which is believed to be multifactorial. Recently, the incidence of pediatric IBD has steeply increased in Korea since 2000. Poorly controlled disease activity can result in complications such as intestinal fistulae, abscess, and stricture, as well as growth retardation and delayed puberty in children. Because of a lack of confirmative tests, various diagnostic modalities must be used to diagnose IBD. Onset age, location, behavior, and activity are important in selecting treatments. Monogenic IBD must be excluded among infantile and refractory very-early-onset IBD. Early aggressive therapy using biologics has recently been proposed for peripubertal children to prevent growth failure and malnutrition.

• Childhood Kidney Diseases
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• v.22 no.2
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• pp.81-85
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• 2018

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.436-439
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• 2015

The majority of clinical studies on moyamoya disease (MMD) have focused on anterior circulation. The disease involvement of posterior circulation in MMD, mainly in the posterior cerebral artery (PCA), has been mentioned since the early 1980s, and it has been repeatedly emphasized as one of the most important factors related to poor prognosis in MMD. However, its clinical features and outcome have only been elucidated during the last few years. In this review, the angiographic definition of PCA stenosis is summarized. The clinical features are elucidated as being either early-onset or delayed-onset, according to the time of PCA stenosis diagnosis in reference to the anterior circulation revascularization surgeries. The surgical strategy and hypothesis on the mechanism of PCA stenosis is also briefly mentioned. It appears that some MMD patients may show PCA stenosis during the early or late course of the disease and that the presenting symptoms may vary. Because the hemodynamic compromise caused by PCA stenosis may respond well to surgical treatment, clinicians should be aware of the condition, especially during follow-up of MMD patients.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.408-414
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• 2015

Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

• Advances in pediatric surgery
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• v.7 no.2
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• pp.130-136
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• 2001

The aim of this study was to evaluate the short-term outcomes of the trans anal one-stage pull-through procedure(TOP) in Hirschsprung's disease. Eight patients aged 3 weeks to 8 months with Hirschsprung's disease underwent this procedure. A rectal mucosectomy was performed from just proximal to dentate line to the level of peritoneal reflexion, where muscle layer was incised circumferentially. Rectosigmoid was mobilized out through the anus, and full-thickness frozen biopsy was taken for confirmation of ganglionic cells. After the rectal muscular cuff was divided longitudinally in the posterior aspect, aganglionic bowel was removed and ganglionic colon was anastomozed to the anus. The mean operating time was 161 minutes, and the mean hospital stay after operation was 3.8 days. Five patients had three to four bowel movement per day without other therapy at mean postoperative 39.2 days. Although long-term follow-up will be required, the TOP might be the new alternative surgical procedure for Hirschsprung's disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.1
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• pp.53-60
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• 2013

In Crohn's disease, mild gastrointestinal bleeding often occurs; however massive gastrointestinal hemorrhage, which can have a dramatic effect on a patient's vital sign, is rare. This could result in potentially life-threatening complications, which can lead to death. Massive hemorrhagic Crohn's disease is not well known and for this reason, they are a diagnostic and therapeutic challenge. Various diagnostic and therapeutic methods are currently being developed and used. The surgical method is often used only as a last measure since this approach has the risk of serious complications that may endanger patients. However, if massive bleeding continues even after all therapeutic methods are used, the surgical method must be implemented. In this case, all therapeutic methods were found to be ineffective; therefore, surgery was used as a last option. Ultimately, the surgical method was found to be successfully used to treat life-threatening hemorrhagic Crohn's disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.6
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• pp.564-570
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• 2021

Perianal fistulizing diseases, namely perianal fistulas and/or abscesses, are well-known complications of Crohn's disease (CD). These are known to develop more frequently in Asian children with CD, especially in the early stages of the disease course. Approximately half of the pediatric CD cases in Korea present with perianal fistulizing diseases at diagnosis. We report a rare case of a 12-year-old boy with CD with an incidental discovery of a perianal abscess on pelvic magnetic resonance imaging during CD diagnosis. No symptoms or signs of perianal fistulizing disease were identified. The early diagnosis of the perianal abscess enabled timely and effective treatment. Considering the high incidence of concomitant perianal CD in Korean children at diagnosis, perianal imaging may be useful and should be considered during diagnostic evaluation, even in patients with no subjective or objective findings indicating perianal CD.