OBJECTIVES: To study the long-term effect of active parenteral nutrition support regimen in preterm infants with a gestational age of <34 weeks. METHODS: According to the different doses of fat emulsion and amino acids used in the early stage, the preterm infants with a gestational age of <34 weeks, who were admitted to the hospital within 24 hours after birth from May to December 2019, were divided into an active parenteral nutrition group and a conventional parenteral nutrition group ( RESULTS: At the age of 6 months, the active parenteral nutrition group ( CONCLUSIONS For preterm infants with a gestational age of <34 weeks, an active parenteral nutrition support strategy with high doses of fat emulsion and amino acids within 24 hours after birth can improve their long-term neurodevelopment.
Amino Acids ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Parenteral Nutrition ; Parenteral Nutrition, Total

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) patients need parenteral nutrition because of nausea, vomiting, and mucositis caused by conditioning regimens. The demand for glutamine increases during the HSCT period. We evaluated the effects of glutamine-containing parenteral nutrition on the clinical outcomes of HSCT patients. METHODS: In this retrospective analysis, we reviewed HSCT patients from Seoul National University from August 2013 to July 2017. Depending on their glutamine supplementation status, 91 patients were divided into 2 groups: glutamine group (N=44) and non-glutamine group (N=47). We analyzed the rate of weight change, infection (clinically/microbiologically documented), complications (duration of mucositis and neutropenia, acute graft versus host disease), and 100-days mortality in each group. RESULTS: Regarding the clinical characteristics of the patients, there were no significant differences between the 2 groups except that there was a larger proportion of myeloablative conditioning regimen in the glutamine group (P=0.005). In the glutamine group, the average number of days of glutamine use, parenteral nutrition, and mucositis was 7.6±1.4, 14.6±9.9, and 13.3±9.5, respectively. Furthermore, multivariate analysis revealed odds ratios of 0.37 (95% CI, 0.14–0.96; P=0.042) and 0.08 (95% CI, 0.01–0.98; P=0.048) for clinically documented infection and 100-days mortality, respectively, in the glutamine group. CONCLUSION: Results showed that the glutamine group had less clinically documented infection and 100-days mortality than the non-glutamine group, but the other outcomes did not show significant differences. The extended duration of glutamine supplementation according to the period of total parenteral nutrition and mucositis should be considered.
Adult ; Glutamine ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Mortality ; Mucositis ; Multivariate Analysis ; Nausea ; Neutropenia ; Odds Ratio ; Parenteral Nutrition ; Parenteral Nutrition, Total ; Retrospective Studies ; Seoul ; Transplants ; Vomiting

PURPOSE: Newborn screening (NBS) programs are important for appropriate management of susceptible neonates to prevent serious clinical problems. Neonates admitted to neonatal intensive care units (NICU) are at a potentially high risk of false-positive results, and repetitive NBS after total parenteral nutrition is completely off results in delayed diagnosis. Here, we present the usefulness of a targeted next-generation sequencing (TNGS) panel to complement NBS for early diagnosis in high-risk neonates. MATERIALS AND METHODS: The TNGS panel covered 198 genes associated with actionable genetic and metabolic diseases that are typically included in NBS programs in Korea using tandem mass spectrometry. The panel was applied to 48 infants admitted to the NICU of Severance Children's Hospital between May 2017 and September 2017. The infants were not selected for suspected metabolic disorders. RESULTS: A total of 13 variants classified as likely pathogenic or pathogenic were detected in 11 (22.9%) neonates, including six genes (DHCR7, PCBD1, GAA, ALDOB, ATP7B, and GBA) associated with metabolic diseases not covered in NBS. One of the 48 infants was diagnosed with an isobutyl-CoA dehydrogenase deficiency, and false positive results of tandem mass screening were confirmed in two infants using the TNGS panel. CONCLUSION: The implementation of TNGS in conjunction with conventional NBS can allow for better management of and earlier diagnosis in susceptible infants, thus preventing the development of critical conditions in these sick infants.
Complement System Proteins ; Delayed Diagnosis ; Diagnosis ; Early Diagnosis ; Humans ; Infant ; Infant, Newborn ; Intensive Care Units, Neonatal ; Korea ; Mass Screening ; Metabolic Diseases ; Metabolism, Inborn Errors ; Oxidoreductases ; Parenteral Nutrition, Total ; Tandem Mass Spectrometry

OBJECTIVE: To study the clinical effect of alanyl-glutamine-enriched nutritional support in the treatment of children with abdominal Henoch-Schönlein purpura. METHODS: Children with abdominal Henoch-Schönlein purpura who needed nutritional support were enrolled and stratified according to age, sex and the severity of disease, and were randomly divided into a control group (n=118) and an enriched nutritional support group (n=107). The control group was given nutritional support without using alanyl-glutamine, while the enriched nutritional support group was given alanyl-glutamine-enriched nutritional support. Intravenous steroids were used according to the severity of disease in both groups. Other therapies were the same in the two groups. The two groups were compared in terms of the length of hospital stay, the rate and duration of use of intravenous steroids, the recurrence rate of symptoms during hospitalization, the rate of total parenteral nutrition (TPN), the rate of weight loss and the rate of fasting for more than 5 days. All patients were followed up for 3 months after discharge to monitor the recurrence of symptoms. RESULTS: There were no significant differences in the length of hospital stay, the rate of TPN and the rate of fasting for more than 5 days between the two groups (P>0.05). Compared with the enriched nutritional support group, the control group showed significant increases in the rate and duration of use of intravenous steroids, the recurrence rate of symptoms and the rate of weight loss (P<0.05). After the 3-month follow-up, all the children resumed normal diet, and the recurrence rate of digestive symptoms was less than 20% in each group. Abdominal pain was the most common symptom (83.33%, 30/36), followed by vomiting and abdominal distention. No digestive hemorrhage was observed. All the symptoms were relieved after symptomatic treatment. No significant difference was found between the two groups in the recurrence rate of digestive symptoms (P=0.693). CONCLUSIONS Alanyl-glutamine-enriched nutritional support in the treatment of children with abdominal Henoch-Schönlein purpura can reduce the use of intravenous steroids and weight loss, but without impact on the length of hospital stay and post-discharge recurrence.
Child ; Dipeptides ; Humans ; Parenteral Nutrition, Total ; Purpura, Schoenlein-Henoch ; Recurrence

total parenteral nutrition. He was diagnosed to have PA after a metabolic work-up and confirmed to have novel mutation by molecular genetic analysis. Because children with PA may have acute pancreatitis, although rare, vomiting and abdominal pain should raise a suspicion of acute pancreatitis. On the contrary, even among children who have never been diagnosed with a metabolic disease, if a child has recurrent pancreatitis, metabolic pancreatitis caused by organic acidemia should be considered.]]>
Abdominal Pain ; Acidosis ; Child ; Child, Preschool ; Diagnosis ; Diethylpropion ; Humans ; Hyperammonemia ; Inpatients ; Metabolic Diseases ; Methylmalonyl-CoA Decarboxylase ; Molecular Biology ; Muscle Hypotonia ; Pancreatitis ; Parenteral Nutrition, Total ; Propionic Acidemia ; Seizures ; Shock ; Vomiting

PURPOSE: We investigated the effect of delayed elevation of thyrotropin (TSH) (deTSH) on gastrointestinal motility in very low birth weight infants (VLBWI). METHODS: This study retrospectively investigated 228 premature VLBWI aged ≥4 weeks with normal neonatal TSH screening test results and free serum thyroxine levels. Infants with serum TSH levels ranging from 5 to 10 µIU/mL were categorized as the deTSH group (n=76), when TSH was measured at 4 (n=53), 8 (n=20), or 12 (n=3) weeks of age. Serum TSH levels in the control group (n=152) were <5 µIU/mL. Multivariate logistic regression analysis was used to determine the risk factors for the development of deTSH. Covariance analysis was used to analyze the relationship between deTSH and gastrointestinal motility. RESULTS: The mean gestational age and birth weight were 29.11±2.25 weeks and 1,157.4±218.0 g, respectively. Risk factors affecting deTSH were dopamine administration (odds ratio [OR], 8.71; 95% confidence interval [CI], 1.80 to 42.05; P=0.007) and operation time (OR, 6.95; 95% CI, 1.43 to 33.75; P=0.016) when the cumulative operating time was ≥1 hour. The mean±standard deviation (SD) duration of a nil per os (NPO) status was significantly higher in the deTSH (99.57±134.99 hours) than in the control group (37.25±59.02 hours) (P from analysis of covariance [ANCOVA]=0.001). The mean±SD duration (33.84±22.34 days) of total parenteral nutrition (TPN) was considerably longer in the deTSH group than in the control group (27.68±13.08 days) (P from ANCOVA=0.003). CONCLUSION: Clinicians must consider deTSH in VLBWI showing feeding intolerance with a prolonged NPO and TPN status.
Birth Weight ; Dopamine ; Gastrointestinal Motility ; Gestational Age ; Humans ; Infant ; Infant, Very Low Birth Weight ; Logistic Models ; Mass Screening ; Parenteral Nutrition ; Parenteral Nutrition, Total ; Retrospective Studies ; Risk Factors ; Thyrotropin ; Thyroxine

Thiamine (vitamin B₁) is a water-soluble vitamin that is not endogenously synthesized in humans. It is absorbed by the small intestine, where it is activated. Its active form acts as a coenzyme in many energy pathways. We report a rare case of thiamine deficiency in a 3.5-year old boy with short bowel syndrome secondary to extensive bowel resection due to necrotizing enterocolitis during his neonatal age. The patient was parenteral nutrition-dependent since birth and had suffered from recurrent central catheter-related bloodstream infections. He developed confusion with disorientation and unsteady gait as well as profound strabismus due to bilateral paresis of the abductor muscle. Based on these and a very low thiamine level he was diagnosed and treated for Wernicke encephalopathy due to incomplete thiamine acquisition despite adequate administration. He fully recovered after thiamine administration. After 1999 eight more cases have been reported in the PubMed mostly of iatrogenic origin.
Child ; Enterocolitis, Necrotizing ; Gait Disorders, Neurologic ; Humans ; Intestine, Small ; Male ; Parenteral Nutrition, Total ; Paresis ; Parturition ; Short Bowel Syndrome ; Strabismus ; Thiamine Deficiency ; Thiamine ; Vitamins ; Wernicke Encephalopathy

Malnutrition is observed more frequently in patients with inflammatory bowel disease (IBD) than in the general population and associated with adverse clinical outcomes. This study aimed to review the current knowledge regarding the efficacy of dietary and nutritional intervention in IBD patients. Exclusive enteral nutrition might be inferior to corticosteroid treatment in adults with active Crohn’s disease (CD) but might even be superior considering the adverse effects of corticosteroid treatment in children. Total parenteral nutrition has no advantage over enteral nutrition, which is considered a more physiologic modality in organ function. Current guidelines do not yet recommend ω3-polyunsaturated fatty acid supplementation for the prevention and maintenance of remission in IBD patients. Dietary fiber supplementation could be effective in the relief of symptoms and maintenance of remission in ulcerative colitis (UC). Although vitamin D may be favorable to clinical course of IBD and bone density. Probiotic supplementation has proven to be effective in preventing and treating pouchitis for UC but is less effective in treating CD. Nutritional interventions not only correct nutritional deficiencies but also improve symptoms and clinical courses of the disease. Hence, nutritional approaches need to be developed to significantly evaluate the effectiveness of dietary interventions used to treat IBD.
Adult ; Bone Density ; Child ; Colitis, Ulcerative ; Crohn Disease ; Dietary Fiber ; Enteral Nutrition ; Humans ; Inflammatory Bowel Diseases ; Malnutrition ; Parenteral Nutrition, Total ; Pouchitis ; Probiotics ; Vitamin D

Early diagnosis followed by primary repair is the best treatment for spontaneous esophageal perforation. However, the appropriate management of esophageal leakage after surgical repair is still controversial. Recently, the successful adaptation of vacuum-assisted closure therapy, which is well established for the treatment of chronic surface wounds, has been demonstrated for esophageal perforation or leakage. Conservative treatment methods require long-term fasting with total parenteral nutrition or enteral feeding through invasive procedures, such as percutaneous endoscopic gastrostomy or a feeding jejunostomy. We report 2 cases of esophageal leakage after primary repair treated by endoscopic vacuum therapy with continuous enteral feeding using a Sengstaken-Blakemore tube.
Early Diagnosis ; Endoscopy ; Enteral Nutrition ; Esophageal Perforation ; Fasting ; Gastrostomy ; Jejunostomy ; Negative-Pressure Wound Therapy ; Parenteral Nutrition, Total ; Vacuum ; Wounds and Injuries

Laparoscopic sleeve gastrectomy has become a standard procedure in bariatric surgery owing to its efficacy and simplicity. However, this procedure can cause life-threatening complications such as a gastric staple-line leak. A 24-year-old woman was transferred to the emergency department for evaluation of epigastric pain. Nine days prior to transfer, she underwent laparoscopic sleeve gastrectomy at another institution. Abdominal computed tomography (CT) revealed fluid collection with air density along the left subphrenic space and gastrosplenic ligament area. Intravenous antibiotics and total parenteral nutrition were initiated. She underwent percutaneous catheter drainage. On postoperative day 18, an esophagogastroduodenoscopy was performed to assess the site and size of the leak, and revealed a leak at the proximal staple line just below the gastroesophageal junction. A newly designed, fully covered antimigratory esophageal stent was placed to cover the leak from the distal esophagus to gastric midbody. Follow-up abdominal CT demonstrated improvement of the fluid collection at the location of the previous gastric leak. The stent was removed 3 weeks after insertion, and a barium study confirmed no more leakage. In this case, we experienced that the newly designed esophageal stent was safe and effective for preventing migration in the management of leak after laparoscopic sleeve gastrectomy.
Anastomotic Leak ; Anti-Bacterial Agents ; Bariatric Surgery ; Barium ; Catheters ; Drainage ; Emergency Service, Hospital ; Endoscopy, Digestive System ; Esophagogastric Junction ; Esophagus ; Female ; Follow-Up Studies ; Gastrectomy ; Humans ; Ligaments ; Obesity ; Parenteral Nutrition, Total ; Stents ; Tomography, X-Ray Computed ; Young Adult