Evaluation of Arsenic and Cobalt Levels in Pediatric Patients Receiving Long-Term Parenteral Nutrition.

This study continues the research in which we determined the concentration of aluminum in children receiving long-term parenteral nutrition (LPN). Since our results were interesting, we decided to assay arsenic (As) and cobalt (Co) in the collected material, which, like aluminum, constitute contamination in the mixtures used in parenteral nutrition. Excesses of these trace elements in the human body are highly toxic, and deficiencies, particularly in the case of Co, can lead to various complications. The aim of this study was to determine the impact of LPN in children on their serum levels of As and Co, as well as the excretion of these elements in urine, and to compare them with a control group of healthy children. The study group consisted of 83 children receiving home parenteral nutrition from two Polish centers, while the control group included 121 healthy children. In both groups, the levels of As and Co in serum and urine were measured. The elemental compositions of the samples were determined using inductively coupled plasma mass spectrometry (ICP-MS). It was demonstrated that the children receiving LPN did not have increased As exposure compared to the controls. Greater exposure compared to the control group was shown for Co. In conclusion, children receiving LPN are not exposed to As, and even though the concentrations of Co in serum and urine were higher in the LPN group than in the healthy controls, neither trace element poses a health threat to children requiring LPN.

Unexpected Serum and Urine Aluminum Concentrations in Pediatric Patients on Home Parenteral Nutrition.

The intravenous supply of aluminum (Al) present in parenteral nutrition solutions poses a high risk of the absorption of this element, which can result in metabolic bone disease, anemia, and neurological complications. The aim of this study is to determine the impact of long-term parenteral nutrition (PN) in children on serum Al concentration and its urinary excretion compared to healthy children. We evaluated serum Al concentrations and its urinary excretion in patients enrolled in the Polish home parenteral nutrition (HPN) program between 2004 and 2022. The study group included 83 patients and the control group consisted of 121 healthy children. In children whose PN was started in the neonatal period, we found higher serum Al concentrations and higher urinary Al excretion than in other subjects whose PN was started later. Only 12% of the children on chronic parenteral nutrition had serum Al concentrations of less than 5 µg/L. Healthy children in the control group had higher serum Al concentrations than those in the parenteral nutrition group, which may indicate the influence of one's environment and diet on Al serum levels.

Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome (MMIHS): Series of 4 Cases Caused by Mutation of ACTG2 (Actin Gamma 2, Smooth Muscle) Gene.

MMIHS, also known as Berdon's syndrome, is a rare disease that belongs to primary causes of CIPOS (chronic intestinal pseudoobstruction syndrome). Clinical characteristics of MMIHS are differential, but we come across the following classic symptoms: disorders of intestinal peristalsis, microcolon, and megacystis. In this article, we present a series of 4 patients with Berdon's syndrome, in whom we managed to identify the genetic causes of MMIHS. All infants showed clinical features of bowel obstruction and dysfunction of the urinary system after birth. Two of them also manifested disorders from other systems. The prognosis for these patients is poor, but a constant betterment of management in MMIHS, in which the leading role plays TPN (total parental nutrition), causes improvement of patients' survival.

Porto-systemic shunt - a rare cause of hyperandrogenism in children. Two case reports and review of literature.

Objectives The main cause of hyperandrogenism in children is congenital adrenal hyperplasia, adrenal and gonadal tumors, polycystic ovary syndrome (PCOs) and Cushing's disease. In the last 20 years several descriptions of girls with hyperandrogenism and venous porto-systemic shunts appeared in literature. Case presentation First case is an eleven and a half-year-old girl, was admitted to Department of Endocrinology because of symptoms of hyperandrogenism. Laboratory tests revealed high serum testosterone, androstenedione, and dehydroepiandrosterone sulfate (DHEAS). The ammonia concentration was also increased. In the abdominal angio-CT scans persistent umbilical vein which connected portal and femoral vein was found. The second case was a seven-year-old boy with symptoms of precocious puberty. Blood tests also revealed high concentration of testosterone, androstenedione, DHEAS and ammonia. Imaging studies showed persistent ductus venosus. Conclusion Although pathophysiological relation is not clear, porto-systemic shunts should be considered as a cause of hyperandrogenism of unknown origin in children.

A Retrospective Analysis of the Effect of Combination of Pure Fish Oil with Third Generation Lipid Emulsion on Liver Function in Children on Long-Term Parenteral Nutrition.

BACKGROUND: Deterioration of liver function, or intestinal failure-associated liver disease, is often observed in long-term parenterally fed children. Fish oil-based intravenous lipids have been reported to play a role in the prevention and treatment of intestinal failure associated liver disease. METHODS: This retrospective analysis included 40 pediatric patients, (20 male and 20 female), median age 38 months (range 1.5-200 months) on long-term (≥1 month) parenteral nutrition who received the parenteral mixtures containing a combination of a third-generation lipid emulsion and pure fish oil because of laboratory liver function abnormalities. The total dose of fish oil from both emulsions for each patient exceeded 0.5 g/kg/day. Data from visits in an outpatient clinic were retrospectively analyzed using the Wilcoxon test, Mann-Whitney test, and Spearman correlation test. RESULTS: The median time of therapy was 149 days (range 28-418 days). There was a decrease of median total and direct (conjugated) bilirubin concentration from 22.23 µmol/L (range 3.42-243 µmol/L) to 10.26 µmol/L (range 3.42-180.58 µmol/L; p < 0.005) and 8.55 (range 1.71-212.04 µmol/L) to 6.84 µmol/L (range 1.71-150.48 µmol/L; p < 0.007) respectively. A significant decrease in median alanine aminotransferase, aspartate aminotransferase and gamma-glutamyl transferase was also observed. In 11 patients bilirubin concentrations increased or remained unchanged. When compared to the patients who responded to the combination therapy, the patients who did not respond received parenteral nutrition for a longer time prior to the start of the therapy (51 vs. 30 months; p < 0.05). CONCLUSIONS: The mixture of an intravenous lipid emulsion containing soybean oil, medium-chain triglycerides, olive oil, and fish oil with the addition of pure fish oil emulsion may be helpful in the treatment of liver complications in children on long-term parenteral nutrition.

Nutritional therapy in paediatric intensive care units: a consensus statement of the Section of Paediatric Anaesthesia and Intensive Therapy the Polish Society of Anaesthesiology and Intensive Therapy, Polish Society of Neonatology and Polish Society for.

Providing nutritional therapy via the gastrointestinal tract in patients in paediatric intensive care units (PICUs) is an effective method for delivering energy and other nutrients. In the event of contraindications to using this method, it is necessary to commence parenteral nutrition. In the present study, methods for nutritional treatments in critically ill children are presented, depending on the clinical situation.

Does long term parenteral nutrition in children have an impact on malocclusion? Preliminary report.

AIM: To evaluate the impact of long-term partial parenteral nutrition and its complications on malocclusion in children and adolescents. MATERIAL AND METHODS: The assessment involved 61 patients (2.25 to16 years of age) without a masticatory parafunction - i.e. 31 subjects receiving parenteral nutrition for a mean period of 5.71±2.87 years, and 30 healthy control subjects. The medical records provided information on the delivery (full-term, preterm), birth body mass, Apgar score, weight deficiency at the age of 1 year; the patient assessment included the current body mass, the number of enteral meals per day and parenteral meals per week, occlusion (acc.to Orlik-Grzybowska's parameters). The statistical analysis was performed by using the chi-square test, Spearman's correlation analysis; the statistical significance was p<0.05. RESULTS: Premature infants with low birth body mass (38.7%), Apgar score below 7 (25.8%), underweight in the first year of life (74.2%) and on examination day (58.1%) were only part of the test group. Mean number of eaten meals: 4.63±1.88 in parenteral nutrition patients, 6.26±1.39 in healthy individuals in the control group. Malocclusions were significantly more frequent in the children receiving parenteral nutrition (38.71%: the most frequent defects included crossbite (19.31%), open bite malocclusion (12.9%), crowding of teeth (9.67%), than in the control group (13.3%: crossbite (3.3%), open bite malocclusion (3.3), crowding of teeth (3.3%). A correlation was statistically proved between the malocclusion and parenteral nutrition, the number of parenteral feeding sessions per week, the current low body mass. CONCLUSION: Long-term parenteral nutrition, a decreased number of oral meals and a coexistent low body mass at the developmental age may contribute to the development of malocclusion.