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.
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.
In 2017, in the Polish-German transborder area of West Pomerania, Mecklenburg-Western Pomerania, and Brandenburg, in collaboration with two centers in Warsaw, a partnership in the field of newborn ...screening (NBS) for severe primary immunodeficiency diseases (PID), mainly severe combined immunodeficiency (SCID), was initiated. SCID, but also some other severe PID, is a group of disorders characterized by the absence of T and/or B and NK cells. Affected infants are susceptible to life-threatening infections, but early detection gives a chance for effective treatment. The prevalence of SCID in the Polish and German populations is unknown but can be comparable to other countries (1:50,000-100,000). SCID NBS tests are based on real-time polymerase chain reaction (qPCR) and the measurement of a number of T cell receptor excision circles (TREC), kappa-deleting recombination excision circles (KREC), and beta-actin (ACTB) as a quality marker of DNA. This method can also be effective in NBS for other severe PID with T- and/or B-cell lymphopenia, including combined immunodeficiency (CID) or agammaglobulinemia. During the 14 months of collaboration, 44,287 newborns were screened according to the ImmunoIVD protocol. Within 65 positive samples, seven were classified to immediate recall and 58 requested a second sample. Examination of the 58 second samples resulted in recalling one newborn. Confirmatory tests included immunophenotyping of lymphocyte subsets with extension to TCR repertoire, lymphoproliferation tests, radiosensitivity tests, maternal engraftment assays, and molecular tests. Final diagnosis included: one case of T-B
NK+ SCID, one case of atypical T
B
NK+ CID, one case of autosomal recessive agammaglobulinemia, and one case of Nijmegen breakage syndrome. Among four other positive results, three infants presented with T- and/or B-cell lymphopenia due to either the mother's immunosuppression, prematurity, or unknown reasons, which resolved or almost normalized in the first months of life. One newborn was classified as truly false positive. The overall positive predictive value (PPV) for the diagnosis of severe PID was 50.0%. This is the first population screening study that allowed identification of newborns with T and/or B immunodeficiency in Central and Eastern Europe.
"Managing Undernutrition in Pediatric Oncology" is a collaborative consensus statement of the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and ...Hematology. The early identification and accurate management of malnutrition in children receiving anticancer treatment are crucial components to integrate into comprehensive medical care. Given the scarcity of high-quality literature on this topic, a consensus statement process was chosen over other approaches, such as guidelines, to provide comprehensive recommendations. Nevertheless, an extensive literature review using the PubMed database was conducted. The following terms, namely pediatric, childhood, cancer, pediatric oncology, malnutrition, undernutrition, refeeding syndrome, nutritional support, and nutrition, were used. The consensus was reached through the Delphi method. Comprehensive recommendations aim to identify malnutrition early in children with cancer and optimize nutritional interventions in this group. The statement underscores the importance of baseline and ongoing assessments of nutritional status and the identification of the risk factors for malnutrition development, and it presents tools that can be used to achieve these goals. This consensus statement establishes a standardized approach to nutritional support, aiming to optimize outcomes in pediatric cancer patients.
Home artificial nutrition (HAN) is a developing method of treatment that reduces the need for hospitalizations. The epidemiology of pediatric HAN in Poland has not yet been covered in detail. This ...study is a longitudinal nationwide analysis of incidence, prevalence, and patients' profile for HAN in Polish children.
Assessment of National Health Fund (NFZ) data covering all pediatric patients treated with HAN in Poland between 2010 and 2018.
HAN was received by 4426 children, 65 patients were on home enteral nutrition (HEN) or home parenteral nutrition (HPN) at different times (HEN
= 3865, HPN
= 626). HAN was most frequently started before the child was 3 years old and long-term HAN programs (5-9 years) were reported. The most common principal diagnosis in HEN was food-related symptoms and signs. In HPN, it was postoperative gastrointestinal disorders. A regionally differentiated prevalence of HAN patients and centers was demonstrated. Mortality among patients was 24.9% for HEN, and 9.6% for HPN, and the main in-hospital cause of death was cardiac arrest.
HAN's use is increasing and evolving in Poland. Uneven distribution of patients and centers results in difficult access to the nutritional procedure which, together with the increasing number of patients, highlights the need for data analysis and development of nutrition centers.
The aim of the study was to assess both patients' and their parents' knowledge of phenylketonuria (PKU) treatment and compliance with PKU diet.
The study included 173 PKU patients aged 10-19 and 110 ...parents of PKU children who were enrolled in the study on the basis of questionnaire data. The study also included 45 patients aged ≥20.
Our study demonstrated that only 45% (
= 74) of PKU patients knew daily Phe intake recommendations, 27% of patients (
= 41) knew the Phe content in a minimum of three out of four researched food products. Patients' knowledge concerning Phe intake (
= 0.0181) and the knowledge of selected food products (
= 0.041819) improved with age. We did not establish such a correlation in the group of PKU children's parents. Approximately 31% of patients and 22% of parents reported helplessness, which increased with the child's age, associated with the necessity to adhere to the diet; 30% of patients reported feeling ashamed of the fact that they could not eat all food products. Regardless of age, children were more likely than parents to report helplessness (
= 0.032005). Among patients, 41.40% declared that they would wish to select products unassisted but their parents did not permit them to do so. The question of whether parents teach children self-reliance in meal preparation was answered affirmatively by 98% of parents and only 81% of children (
= 0.0001).
Our data demonstrated that parents' and children's knowledge concerning treatment recommendations and food products does not have a direct impact on attitude to the PKU diet. Limiting children's independence in meal selection, growing helplessness in the face of dietary adherence and shame resulting from the necessity to follow a different diet observed in PKU families are responsible for shaping and perpetuating a consistently negative attitude to the diet. The care of PKU paediatric patients requires consistent, long-term family and individual therapy which may counteract the effects of learned helplessness. In regard to the educational effort, a good parent-child relationship as well as the teaching of behaviours motivating patients to comply with the diet are of great importance.
Vitamin D deficiency is an important public health problem worldwide. Vitamin D deficiency confers a significant risk for both skeletal and non-skeletal disorders and a number of lifelong negative ...health outcomes. The objectives of this evidence-based guidelines document are to provide health care professionals in Poland, an updated recommendation for the prevention, diagnosis and treatment of vitamin D deficiency.
A systematic literature search examining the prevention and treatment strategies for vitamin D deficiency was conducted. Updated recommendations were developed using the Grading of Recommendations, Assessment, Development and Evaluation system describing the strength of the recommendation and the quality of supporting evidence. Twenty-seven contributors representing different areas of expertise and medical specialties, including pediatricians, geriatricians, endocrinologists, epidemiologists, nephrologists, gynecologists and obstetricians evaluated the available published evidence related to vitamin D, formulated the goals of this document and developed a common consolidated position. The consensus group, representing six national specialist consultants and eight Polish and international scientific organizations/societies, participated in the process of grading evidence and drawing up the general and specific recommendations.
The updated recommendations define the diagnostic criteria for the evaluation of vitamin D status and describe the prevention and treatment strategies of vitamin D deficiency in the general population and in groups at increased risk of the deficiency. Age- and weight-specific recommendations for prevention, supplementation and treatment of vitamin D deficiency are presented, and detailed practice guidance is discussed regarding the management in primary and specialized health care.
Vitamin D deficiency remains still highly prevalent in Poland, in all age groups. Currently, there is a great necessity to implement a regular supplementation with recommended doses and to develop an effective strategy to alleviate vitamin D deficiency in the population. These updated recommendations are addressed to health professionals and the authorities pursuing comprehensive health policies and should also be included in public health programs aimed at preventing a broad spectrum of chronic diseases.
Existing research into the effects of teat application has mainly focused on its negative and positive influence on the development of the oral cavity. Our work demonstrates that apart from changing ...the setting of the articulatory organs, the teat can also affect the quality of breathing, eating and sleeping.
We described the cases of 2 children: a 19-month-old girl and a 2.5-month-old boy, who had breathing disorders due to withdrawal of the tongue and impaired food intake.
The babies were bottled fed with a special teat for cleft lip patients to observe the influence of the teat on the setting of the articulatory organs and breathing.
We suspected that the specific construction of the teat-the wide outer part and the short internal part-would affect children's reflexes and articulatory organs so as to force the frontal position of the tongue, which was meant to facilitate breathing and eating.
It was found that feeding with the cleft lip teat stimulates the gyro-linguistic muscle, which results in the proper position of the tongue and consequently better breathing and improved quality of sleep.
A specialist bottle teat designed for babies with cleft lips can constitute an effective tool in the therapy of nonspecific respiratory disorders resulting from improper position of the tongue and other articulatory organs.
The authors present a case report of a boy with a classical form of phenylketonuria and Alazami syndrome. The inborn error of phenylalanine metabolism was diagnosed in the neonatal period based on ...population new-born screening. Despite early implementation of a low-phenylalanine diet and good biochemical control, the patient developed behavioural disorders and intellectual disability. He also presented with dysmorphic features. After long and extensive attempts to establish the genetic cause of this unusual phenotype, finally, at the age of 16 the boy was diagnosed with Alazami syndrome based on whole exome sequencing. The authors discussed the problem of neuropsychological disorders in patients with phenylketonuria and described typical clinical symptoms of Alazami syndrome. It was emphasized that the presence of one monogenic disease does not exclude the coexistence of another one.
The etiology of type 1 diabetes mellitus (DM1) is not fully understood. Some studies indicate an excess or deficiency of certain trace elements may affect glucose and insulin metabolism. This study ...aimed to assess the concentrations of trace elements in children with newly diagnosed DM1. The study group comprised 35 children aged 3-17 years (mean, 8.83±3.55 years). Serum concentrations of selenium, zinc, copper, and arsenic were determined at the time of diagnosis, after ~2 weeks (during insulin treatment), and after 6 months. No trace element deficiency was observed. Selenium levels were increased at all time points (77.61±14.03 µg/l; 70.42±11.04 µg/l; 75.79±12.89 µg/l). Arsenic levels were increased at the time of discharge (0.30±0.24 µg/l) and upon 6 months control visit (0.67±1.98 µg/l) for DM1. Copper levels were elevated at the time of diagnosis (1333±244 µg/l). No significant differences were observed in zinc concentrations between study and control group or between time points. Trace elements in the environment, especially selenium, may increase the incidence of DM1, although further research is required to confirm this association.
PDF
