• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.22 no.4
• /
• pp.303-329
• /
• 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.

• Gut and Liver
• /
• v.12 no.6
• /
• pp.623-632
• /
• 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
• /
• v.20 no.1
• /
• pp.55-60
• /
• 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
• /
• v.10 no.2
• /
• pp.45-50
• /
• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.19 no.1
• /
• pp.44-53
• /
• 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.

• Journal of Preventive Medicine and Public Health
• /
• v.51 no.6
• /
• pp.298-309
• /
• 2018

Objectives: The objective of this study was to assess the relationship between systolic and diastolic blood pressure (SBP, DBP) and the risk of death from specific causes other than cardiovascular diseases. Methods: We calculated the risk of specific death by SBP and DBP categories for 506 508 health examinees in 2002-2003 using hazard ratios (HRs) and 95% confidence intervals (CIs) in a Cox proportional hazards model. Results: Compared to normal levels (SBP <120 or DBP <90 mmHg), stage I systolic and diastolic hypertension (SBP 140-159, DBP 85-89 mmHg, respectively) were associated with an increased risk of death from diabetes mellitus, alcoholic liver disease, and renal failure (HR, 1.83; 95% CI, 1.51 to 2.22; HR, 1.24; 95% CI, 1.06 to 1.46; HR, 2.30; 95% CI, 1.64 to 3.21; HR, 1.67; 95% CI, 1.27 to 2.20; HR, 1.99; 95% CI, 1.41 to 2.81; HR, 1.31; 95% CI, 0.99 to 1.73, respectively), but a decreased risk of death from intestinal pneumonia (HR, 0.64; 95% CI, 0.42 to 0.98; HR, 0.59; 95% CI, 0.39 to 0.91). Only stage II systolic hypertension (SBP ${\geq}160mmHg$) was associated with an increased risk of death from pneumonia, liver cirrhosis, and intestinal ischemia (HR, 1.54; 95% CI, 1.19 to 1.98; HR, 1.46; 95% CI, 1.00 to 2.15; HR, 3.77; 95% CI, 1.24 to 11.40, respectively), and stage I and II diastolic hypertension (SBP 140-159 and ${\geq}160mmHg$) were associated with an increased risk of death from intestinal ischemia (HR, 3.07; 95% CI, 1.27 to 7.38; HR, 4.39; 95% CI, 1.62 to 11.88, respectively). Conclusions: An increase in blood pressure levels may alter the risk of death from certain causes other than cardiovascular diseases, a well-known outcome of hypertension, although the mechanism of these associations is not well documented.

• Experimental and Molecular Medicine
• /
• v.50 no.11
• /
• pp.14.1-14.14
• /
• 2018

The link between antibiotic treatment and IF-associated liver disease (IFALD) is unclear. Here, we study the effect of antibiotic treatment on bile acid (BA) metabolism and investigate the involved mechanisms. The results showed that pediatric IF patients with cholestasis had a significantly lower abundance of BA-biotransforming bacteria than patients without cholestasis. In addition, the BA composition was altered in the serum, feces, and liver of pediatric IF patients with cholestasis, as reflected by the increased proportion of primary BAs. In the ileum, farnesoid X receptor (FXR) expression was reduced in patients with cholestasis. Correspondingly, the serum FGF19 levels decreased significantly in patients with cholestasis. In the liver, the expression of the rate-limiting enzyme in bile salt synthesis, cytochrome P450 7a1 (CYP7A1), increased noticeably in IF patients with cholestasis. In mice, we showed that oral antibiotics (gentamicin, GM or vancomycin, VCM) reduced colonic microbial diversity, with a decrease in both Gram-negative bacteria (GM affected Eubacterium and Bacteroides) and Gram-positive bacteria (VCM affected Clostridium, Bifidobacterium and Lactobacillus). Concomitantly, treatment with GM or VCM decreased secondary BAs in the colonic contents, with a simultaneous increase in primary BAs in plasma. Moreover, the changes in the colonic BA profile especially that of tauro-beta-muricholic acid ($T{\beta}MCA$), were predominantly associated with the inhibition of the FXR and further altered BA synthesis and transport. In conclusion, the administration of antibiotics significantly decreased the intestinal microbiota diversity and subsequently altered the BA composition. The alterations in BA composition contributed to cholestasis in IF patients by regulating FXR signaling.

• Radiation Oncology Journal
• /
• v.8 no.2
• /
• pp.241-253
• /
• 1990

331 patients of stage IIb uterine cervix cancer trated by radiation alone at Kosin Medical Center between June 1980 and Dec. 1985 were analysed to determine parameters of radiotherapy associated to disease states. Survival rate was highest among the reported ($82.8{\%}$ for crude and $82.4{\%}$ for disease free survival). Pelvic control rate in 6 weeks after the end of radiotherapy was $93.6{\%}$ in the patients treated with ICR following total pelvic radiation and $71.6{\%}$ with small field additional external irradiation. 5 year survival rate in those who achieved pelvic control was $98.9{\%}$ and $12.9{\%}$ in those who had pelvic failure and/or metastasis after radiation. The survival rate figured maximal $88.5{\%}$ with dosage of $7500{\~}8500$ cGy to point A with acceptable incidence of complications ($4.9{\%}$) but without increasing survival above it and minimal $74.1{\%}$ with dosage of less than 6500 cGy. The treatment failure was counted $18.7{\%}$ (62 of 331 patients): Local failure $72.6{\%}$ (45 of 62 patients), locoregional failure $3.2{\%}$ (2 of 62 patients) and distant failure $24{\%}$ (15 of 62 patients). Late complications were found in 50 patients ($15.1{\%}$) and $42{\%}$ of them was rectal bleeding and stenosis. The dose of 8500 cGy to point A was found to be critical for complication and $70{\%}$ of complications occurred above it and was more serious one such as fistula. Rectal complications were developed above rectal dose 6500 cGy and bladder complication above bladder dose 7500 cGy. Major cause of death was cachexia due to locoregional failure ($73.7{\%}$ of death), next was due to metastasis to lung, liver and bone, and only 3 patients died of complication of intestinal perforations and obstruction. In conclusion higher external radiation dose for a bulky uterine cervix and barrel shaped uterus was essential for local control.

• Tuberculosis and Respiratory Diseases
• /
• v.42 no.3
• /
• pp.277-294
• /
• 1995

Background: The prevalence of tuberculosis in Korea decreased remarkably for the past 30 years, while the incidence of disease caused by mycobacteria other than tuberculosis is unknown. Korean Academy of Tuberculosis and Respiratory Diseases performed national survey to estimate the incidence of mycobacterial diseases other than tuberculosis in Korea. We analyzed the clinical data of confirmed cases for the practice of primary care physicians and pulmonary specialists. Methods: The period of study was from January 1981 to October 1994. We collected the data retrospectively by correspondence with physicians in the hospitals that referred the specimens to Korean Institute of Tuberculosis, The Korean National Tuberculosis Association for the detection of mycobacteria other than tuberculosis. In confirmed cases, we obtained the records for clinical, laboratory and radiological findings in detail using protocols. Results: 1) Mycobacterial diseases other than tuberculosis were confirmed that 1 case was in 1981, 2 cases in 1982, 4 cases in 1983, 2 cases in 1984, 5 cases in 1985, 1 case in 1986, 3 cases in 1987, 1 case in 1988, 6 cases in 1989, 9 cases in 1990, 14 cases in 1990, 10 cases in 1992, 4 cases in 1993, and 96 cases in 1994. Cases since 1990 were 133 cases(84.2%) of a total. 2) Fifty seven percent of patients were in the age group of over 60 years. The ratio of male to female patients was 2.6:1. 3) The distribution of hospitals in Korea showed that 61 cases(38.6%) were referred from Double Cross Clinic, 42 cases(26.6%) from health centers, 21 cases(13.3%) from tertiary referral hospitals, 15 cases(9.5%) from secondary referral hospitals, and 10 cases(6.3%) from primary care hospitals. The area distribution in Korea revealed that 98 cases(62%) were in Seoul, 17 cases(10.8%) in Gyeongsangbuk-do, 12 cases(7.6%) in Kyongki-do, 8 cases(5.1%) in Chungchongnam-do, each 5 cases(3.2%) in Gyeongsangnam-do and Chungchongbuk-do, 6 cases(3.8%) in other areas. 4) In the species of isolated mycobacteria other than tuberculosis, M. avium-intracellulare was found in 104 cases(65.2%), M. fortuitum in 20 cases(12.7%), M. chelonae in 15 cases(9.5%), M. gordonae in 7 cases(4.4%), M. terrae in 5 cases(3.2%), M. scrofulaceum in 3 cases(1.9%), M. kansasii and M. szulgai in each 2 cases(1.3%), and M. avium-intracellulare coexisting with M. terrae in 1 case(0.6%). 5) In pre-existing pulmonary diseases, pulmonary tuberculosis was 113 cases(71.5%), bronchiectasis 6 cases(3.8%), chronic bronchitis 10 cases(6.3%), and pulmonary fibrosis 6 cases(3.8%). The timing of diagnosis as having pulmonary tuberculosis was within 1 year in 7 cases(6.2%), 2~5 years ago in 32 cases(28.3%), 6~10 years ago in 29 cases(25.7%), 11~15 years ago in 16 cases(14.2%), 16~20 years ago in 15 cases (13.3%), and 20 years ago in 14 cases(12.4%). Duration of anti-tuberculous treatment was within 3 months in 6 cases(5.3%), 4~6 months in 17 cases(15%), 7~9 months in 16 cases(14.2%), 10~12 months in 11 cases(9.7%), 1~2 years in 21 cases(18.6%), and over 2 years in 8 cases(7.1%). The results of treatment were cure in 44 cases(27.9%) and failure in 25 cases(15.8%). 6) Associated extra-pulmonary diseases were chronic liver disease coexisting with chronic renal failure in 1 case(0.6%), diabetes mellitus in 9 cases(5.7%), cardiovascular diseases in 2 cases(1.3%), long-term therapy with steroid in 2 cases(1.3%) and chronic liver disease, chronic renal failure, colitis and pneumoconiosis in each 1 case(0.6%). 7) The clinical presentations of mycobacterial diseases other than tuberculosis were 86 cases (54.4%) of chronic pulmonary infections, 1 case(0.6%) of cervical or other site lymphadenitis, 3 cases(1.9%) of endobronchial tuberculosis, and 1 case(0.6%) of intestinal tuberculosis. 8) The symptoms of patients were cough(62%), sputum(61.4%), dyspnea(30.4%), hemoptysis or blood-tinged sputum(20.9%), weight loss(13.3%), fever(6.3%), and others(4.4%). 9) Smear negative with culture negative cases were 24 cases(15.2%) in first examination, 27 cases(17.1%) in second one, 22 cases(13.9%) in third one, and 17 cases(10.8%) in fourth one. Smear negative with culture positive cases were 59 cases(37.3%) in first examination, 36 cases (22.8%) in second one, 24 cases(15.2%) in third one, and 23 cases(14.6%) in fourth one. Smear positive with culture negative cases were 1 case(0.6%) in first examination, 4 cases(2.5%) in second one, 1 case (0.6%) in third one, and 2 cases(1.3%) in fourth one. Smear positive with culture positive cases were 48 cases(30.4%) in first examination, 34 cases(21.5%) in second one, 34 cases(21.5%) in third one, and 22 cases(13.9%) in fourth one. 10) The specimens isolated mycobacteria other than tuberculosis were sputum in 143 cases (90.5%), sputum and bronchial washing in 4 cases(2.5%), bronchial washing in 1 case(0.6%). 11) Drug resistance against all species of mycobacteria other than tuberculosis were that INH was 62%, EMB 55.7%, RMP 52.5%, PZA 34.8%, OFX 29.1%, SM 36.7%, KM 27.2%, TUM 24.1%, CS 23.4%, TH 34.2%, and PAS 44.9%. Drug resistance against M. avium-intracellulare were that INH was 62.5%, EMB 59.6%, RMP 51.9%, PZA 29.8%, OFX 33.7%, SM 30.8%, KM 20.2%, TUM 17.3%, CS 14.4%, TH 31.7%, and PAS 38.5%. Drug resistance against M. chelonae were that INH was 66.7%, EMB 66.7%, RMP 66.7%, PZA 40%, OFX 26.7%, SM 66.7%, KM 53.3%, TUM 53.3%, CS 60%, TH 53.3%, and PAS 66.7%. Drug resistance against M. fortuitum were that INH was 65%, EMB 55%, RMP 65%, PZA 50%, OFX 25%, SM 55%, KM 45%, TUM 55%, CS 65%, TH 45%, and PAS 60%. 12) The activities of disease on chest roentgenogram showed that no active disease was 7 cases(4.4%), mild 20 cases(12.7%), moderate 67 cases(42.4%), and severe 47 cases(29.8%). Cavities were found in 43 cases(27.2%) and pleurisy in 18 cases(11.4%). 13) Treatment of mycobacterial diseases other than tuberculosis was done in 129 cases(81.7%). In cases treated with the first line anti-tuberculous drugs, combination chemotherapy including INH and RMP was done in 86 cases(66.7%), INH or RMP in 30 cases(23.3%), and not including INH and RMP in 9 cases(7%). In 65 cases treated with the second line anti-tuberculous drugs, combination chemotherapy including below 2 drugs were in 2 cases(3.1%), 3 drugs in 15 cases(23.1%), 4 drugs in 20 cases(30.8%), 5 drugs in 9 cases(13.8%), and over 6 drugs in 19 cases (29.2%). The results of treatment were improvement in 36 cases(27.9%), no interval changes in 65 cases(50.4%), aggravation in 4 cases(3.1%), and death in 4 cases(3.1%). In improved 36 cases, 34 cases(94.4%) attained negative conversion of mycobacteria other than tuberculosis on cultures. The timing in attaining negative conversion on cultures was within 1 month in 2 cases(1.3%), within 3 months in 11 cases(7%), within 6 months in 14 eases(8.9%), within 1 year in 2 cases(1.3%) and over 1 year in 1 case(0.6%). Conclusion: Clinical, laboratory and radiological findings of mycobacterial diseases other than tuberculosis were summarized. This collected datas will assist in the more detection of mycobacterial diseases other than tuberculosis in Korea in near future.