• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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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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• v.20 no.1
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• pp.55-60
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• 2017

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to $Omegaven^{TM}$, a fat emulsion comprised of omega-3 fatty acids. $Omegaven^{TM}$ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with $Omegaven^{TM}$ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of $Omegaven^{TM}$. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of $Omegaven^{TM}$.

• Journal of Clinical Nutrition
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• v.10 no.2
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• pp.45-50
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• 2018

Purpose: Intense multidisciplinary team effort is required for the intestinal rehabilitation of patients afflicted with the short bowel syndrome (SBS). These include enteral and parenteral nutrition (PN) support, monitoring of complications related to treatment, and considering further medical or surgical options for intestinal adaptation. Methods: In the Intestinal Rehabilitation Team (IRT) at the Samsung Medical Center, we have experienced 20 cases of adult SBS requiring multidisciplinary intestinal rehabilitation. This study is a retrospective review of the collected medical records. Results: Of the 20 subjects treated, 12 patients were male and 8 patients were female. At the time of referral to the IRT, the mean age was 51.5 years, and the mean body weight was 50.1 kg, which was 90% of the usual body weight. The diseases or operative managements preceding massive bowel resection were malignancy in 11 cases, cardiac surgery in 2 cases, trauma in 2 cases and one case, each of tuberculosis, corrosive esophagitis, atrial fibrillation, simultaneous pancreas and kidney transplantation, and perforated appendicitis. Of these, there were 14 survivals and 6 mortalities. The fatalities were attributed to progression of disease, intestinal failure-associated liver disease, and sepsis (unrelated to intestinal failure) (2 cases each). Among the 14 surviving patients, 8 patients have been weaned off PN, whereas 6 are still dependent on PN (mean PN dependence 36%). Conclusion: This paper reports the results of multidisciplinary intestinal rehabilitation of adult short bowel patients treated at the Samsung Medical Center. Further studies are required to improve survival and enteral tolerance of these patients.

• Gut and Liver
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• v.12 no.6
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• pp.623-632
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• 2018

Intestinal Behçet's disease is a rare, immune-mediated chronic intestinal inflammatory disease; therefore, clinical trials to optimize the management and treatment of patients are scarce. Moreover, intestinal Behçet's disease is difficult to treat and often requires surgery because of the failure of conventional medical treatment. Administration of anti-tumor necrosis factor-${\alpha}$, a potential therapeutic strategy, is currently under active clinical investigation, and evidence of its effectiveness for both intestinal Behçet's disease and inflammatory bowel diseases has been accumulating. Here, we review updated data on current experiences and outcomes after the administration of anti-tumor necrosis factor-${\alpha}$ for the treatment of intestinal Behçet's disease. In addition to infliximab and adalimumab, which are the most commonly used agents, we describe agents such as golimumab, etanercept, and certolizumab pegol, which have recently been shown to be effective in refractory intestinal Behçet's disease. This review also discusses safety issues associated with anti-tumor necrosis factor-${\alpha}$, including vulnerability to infections and malignancy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.5
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• pp.413-422
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• 2021

Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy. Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia. Determining the extent of the lesion provides direction for treatment options. Focal short-segment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.1
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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.

• Neonatal Medicine
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• v.28 no.1
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• pp.53-58
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• 2021

Intestinal malrotation with midgut volvulus (MV) is a life-threatening surgical emergency. Most events of MV occur in the neonatal period with bilious vomiting, abdominal distension, feeding intolerance, and bloody stools. Neonatal gastric perforation (GP) is a rare and life-threatening condition associated with high mortality. It occurs either in an idiopathic form or in association with gastrointestinal anomalies such as duodenal atresia and MV. The pathogenesis of both MV and GP is related to ischemic change and inflammatory response. MV and GP can lead to morbidities such as sepsis, intestinal ischemia, and organ failure, but not neurologic problems. We herein report the case of a term infant at 5 days after birth, with MV accompanied by GP, who developed periventricular leukomalacia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.99-103
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• 1999

Duodenal web is an uncommon lesion that infrequently cause partial or complete intestinal obstruction. If the duonenal web is incomplete and only partially obstructs the duodenal lumen, the onset of symptoms may be delayed for years, and obstructive symptoms will be only minimal and intermittent. Congenital duodenal obstruction is occasionally associated with intestinal malrotation. We experienced a case of duodenal web associated with intestinal malrotation in a 9-year-old girl who had been suffered from intermittent projectile vomiting since birth without failure to thrive. The diagnosis was made by UGI series & abdominal CT. Patient remained asymptomatic after operation.

• Tuberculosis and Respiratory Diseases
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• v.68 no.1
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• pp.29-33
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• 2010

Strongyloides stercoralis is an intestinal nematode that is a parasite to humans. The infecting filariform larvae of S. stercoralis enters the host body via the bloodstream, passes through the lungs, penetrates the alveoli, and then ascends the airway to transit down the esophagus into the small bowel. The infection can persist for decades without causing major symptoms and can elicit eosinophilia of varying magnitudes. Of note, this infection can also develop into a disseminated, often fatal, disease (hyperinfection) in patients receiving immunosuppressive corticosteroids. A 65-year-old man who was receiving corticosteroid therapy for the treatment of spinal stenosis was admitted to the emergency room with complaints of abdominal pain and severe dyspnea. We detected many S. stercoralis larvae in the sputum and in the bronchoalveolar-lavage sample collected by bronchoscopy. Here, we report a fatal case of strongyloidiasis with acute respiratory failure and intestinal perforation. In addition, we provide a brief review of the relevant medical literature.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.79-82
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• 2002

Intestinal malrotation is a developmental anomaly resulting from embryologic failure of fixation and rotation of the gut and predisposed to midgut volvulus and small bowel obstruction. Acute midgut volvulus is most often encountered in the newborn period. But older children and adults may have a history of intermittent episodes of partial volvulus presenting with recurrent colicky abdominal pain accompanying bilious vomiting. We experienced a case of intestinal malrotation complicated by midgut volvulus presented with recurrent vomiting and abdominal pain in a 6-year-old boy.