• 한국임상수의학회지
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• 제33권3호
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• pp.179-182
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• 2016

An 11-year-old castrated male Maltese had vomiting, diarrhea, and abdominal distension for over two weeks and weight loss for several months. Clinical laboratory studies were not remarkable. Abdominal radiographs showed severe dilated intestine with a gravel sign. Colon was empty with normal diameter in the pneumocolon study. On ultrasonographs, most small bowel loops were dilated without normal peristalsis and showed abnormal thin wall. Barium contrast study revealed remarkably delayed gastric emptying and transit time up to $6^{th}$ day. On exploratory laparotomy, there were no mechanical obstruction and extra-intestinal abnormalities except severe dilated small intestine. Chronic fibrosing lymphohistiocytic leiomyositis with atrophy of tunica muscularis in the small intestines and colon was identified through full thickness biopsy and histopathology. Therefore, primary myopathic chronic intestinal pseudo-obstruction was diagnosed. This dog is survival with symptomatic treatments for eight months.

• 한국임상수의학회지
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• 제33권4호
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• pp.210-213
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• 2016

A 9-year-old, mixed-breed, castrated male dog presented with a one-week history of severe vomiting. Abdominal radiography showed a dilated small intestine loop. Abdominal ultrasonography showed small-intestine distension up to 5 cm in diameter without mechanical obstruction. During exploratory laparotomy, an aperistaltic ileal segment was resected. There was no narrowed cavity in the resected plane. Histopathologic and immunohistochemical findings confirmed visceral myopathy due to intestinal adenocarcinoma in the ileum and revealed partial destruction of the longitudinal and circular muscles with fibrosis. Chronic intestinal pseudo-obstruction caused by adenocarcinoma was diagnosed after considering the above investigative results.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제27권4호
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• pp.197-205
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• 2024

T-lymphocytic intestinal leiomyositis is a rare cause of "pediatric intestinal pseudo-obstructions." Diagnosis may be difficult and requires full-thickness bowel biopsies during laparotomy or laparoscopy with possible enterostomy. Currently, immunosuppressive therapy is the only available treatment. A delay in diagnosis and therapy may negatively affect the prognosis because of ongoing fibrotic alterations; therefore, early diagnosis and consequent treatment are crucial. This review summarizes the available information on the nosology, diagnostic steps, and treatment modalities. Here, we report the youngest case of enteric leiomyositis reported in the last two decades and analyze its management by reviewing previous cases.

• Neonatal Medicine
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• 제15권2호
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• pp.196-199
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• 2008

저자들은 Hirschsprung 병을 의심한 환아에서 Cajal세포 감소에 의한 미숙아 가성 장 폐쇄를 경험하였기에 보고하는 바이다. 심한 복부 팽만과 장 폐쇄 증상으로 결장 절개술이 필요한 미숙아에서 ganglion cell의 존재 유무 뿐만 아니라 Cajal 세포의 결핍도 확인할 필요가 있을 것으로 생각된다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권5호
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• pp.487-492
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• 2019

Waardenburg syndrome (WS) type IV is characterized by pigmentary abnormalities, deafness and Hirschsprung's disease. This syndrome can be triggered by dysregulation of the SOX10 gene, which belongs to the SOX (SRY-related high-mobility group-box) family of genes. We discuss the first known case of a SOX10 frameshift mutation variant defined as c.895delC causing WS type IV without Hirschsprung's disease. This female patient of unrelated Kuwaiti parents, who tested negative for cystic fibrosis and Hirschsprung's disease, was born with meconium ileus and malrotation and had multiple surgical complications likely due to chronic intestinal pseudo-obstruction. These complications included small intestinal necrosis requiring resection, development of a spontaneous fistula between the duodenum and jejunum after being left in discontinuity, and short gut syndrome. This case and previously reported cases demonstrate that SOX10 gene sequencing is a consideration in WS patients without aganglionosis but with intestinal dysfunction.

• 대한한방내과학회지
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• 제42권5호
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• pp.1094-1101
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• 2021

Objectives: This study examined a case of chronic intestinal pseudo-obstruction (CIPO) in an adult with unspecified abdominal pain.Methods: The patient was treated with herbal medicine, acupuncture, and moxa. Treatment progress was evaluated by follow-up monitoring of the intensity, frequency, and duration (in hours) of abdominal pain and body weight. Results: The average abdominal pain level on the Numerical Rating Scale (NRS) and its incidence decreased. The duration of pain was 15 hours maximum and 2.5 hours minimum. The body weight repeatedly increased and decreased but increased overall. Despite no remarkable findings from X-rays, air-fluid levels and coil-shaped folds of the small bowel were observed. Conclusions: Korean medical treatment based on a dialectic effectively relieved chronic abdominal pain and suspected CIPO-a disease that requires continuous management and therapeutic intervention.

• Clinical and Experimental Pediatrics
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• 제51권6호
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• pp.655-659
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• 2008

전신 홍반 루푸스는 다양한 증상으로 발현되는 자가면역질환이다. 위장관 증상도 질환의 경과 중에 나타날 수 있으나, 장 가성 폐쇄로 처음 진단되는 경우는 소아에서 매우 드물다. 장 가성 폐쇄는 원발성 또는 속발성으로 장의 평활근이나 신경계에 이상이 있어 해부학적 원인 없이 장폐쇄의 증상과 징후가 나타나는 것이며, 장폐쇄로 인하여 수술을 하였다는 보고도 있다. 그러나 장 가성 폐쇄가 전신 홍반 루푸스에 속발한 경우 장간막 혈관의 폐쇄와 장괴사로 진행하기 전에 조기에 진단하고 치료하면 합병증을 예방하고 수술을 피할 수 있다. 저자들은 장 가성 폐쇄의 증상으로 내원한 13세 여아에서 전신 홍반 루푸스를 진단하여, 불필요한 수술을 피하고 조기에 치료한 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권4호
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• pp.303-329
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• 2019

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제19권1호
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• pp.44-53
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• 2016

Purpose: Parenteral nutrition (PN) not only provides nutritional support but also plays a crucial role in the treatment of children with intestinal failure. The aim of this study was to evaluate the clinical significance and clinical outcomes of long-term PN. Methods: Retrospective cohort study was conducted using the medical records of patients treated at Seoul National University Children's Hospital. This study included 19 patients who received PN for over six months. Most patients received home PN. Results: The indications for PN included short bowel syndrome, chronic intestinal pseudo-obstruction, and intractable diarrhea of infancy. The median age of PN initiation was 1.3 years, and the median treatment duration was 2.9 years. Two patients were weaned from PN; 14 continued to receive PN with enteral feedings; and 3 patients died. The overall survival rates at 2 and 5 years were 93.3% and 84.0%, respectively. The incidence of catheter-related bloodstream infections was 2.7/1,000 catheter-days and was associated with younger age at PN initiation and lower initial height Z-score. Six patients developed catheter-related central vein thrombosis, with an incidence of 0.25/1,000 catheter-days. Eleven patients experienced PN-associated liver disease (PNALD), and one patient underwent multi-visceral transplant. The patients with PNALD exhibited lower final heights and body weight Z-scores. All patients experienced micronutrient deficiencies transiently while receiving PN. Conclusion: PN is an important and safe treatment for pediatric intestinal failure. PNALD was linked to final anthropometric poor outcomes. Micronutrient deficiencies were common. Anthropometric measurements and micronutrient levels must be monitored for successful PN completion.