Management of post-hematopoietic stem cell transplant (HSCT) patient is very important and determines the success of the procedure. Infections represent a serious hazard for HSCT patients both during the early and late post-transplant period. Vaccinations are an important approach to prevent many infections. Guidelines on revaccination after HSCT require continuous updates. Recommendations of Polish Society of Pediatric Oncology and Hematology for the vaccination after auto- and allo- HSCT based on the European Conference on Infections in Leukemia 2017 (ECIL7) are presented in this report. 

In this paper we present guidelines for enteral nutrition in pediatric patients. The aim of the paper is to adapt to Polish conditions the current guidelines of the international medical societies. The document is the result of a panel discussion of the Working Group of the Polish Society for Pediatric Gastroenterology, Hepatology and Nutrition.

Congenital toxoplasmosis is a parasitic disease caused by Toxoplasma gondii (T. gondii). It may lead to a severe fetal harm or a latent disease, which when untreated may manifest as late complications: blindness, neurological disorders or delayed motor development. Coordinated efforts of gynaecologists, infectious diseases specialists and neonatologists/paediatricians should aim to early detection of fetal risk, prenatal treatment, then early diagnosis and treatment of a newborn. The article presents the current knowledge about congenital T. gondii infection and practical guidelines regarding diagnosis and treatment of this serious disease in the context of Poland. 

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The article presents a summary of the new EAACI/GA²LEN/EDF/WAO guidelines for the definition, classification, diagnosis and management of urticaria published in 2018 in Allergy. 

The article discusses news regarding celiac disease diagnostics presented during the 51st Annual Meeting of European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN), which took place in Geneva from 9 to 12 May 2018. 

Proper nutrition in the first 1000 days of life is crucial for child’s growth and development. In spite of numerous information about nutrition of infants and children included in the guides and in the internet, the difficulties in the implementation of detailed nutritional recommendations are still observed among parents. The calendar of child nutrition in the first 1000 days is the first tool containing the whole period of the first years of the child’s life, that transmits reliable knowledge based on the latest recommendations in a simple manner that is clear to parents. 

The gastrointestinal tract and intestinal microbiota connected with it are the largest center of the human immune system. Their proper functioning 
 guarantees an effective response against microbes, both locally, in the intestines and other organs, eg. bronchi and the development of tolerance to many antigens and thus counteracting the development of allergic or autoimmune diseases. Together with the nervous system through a complex network of intestine-brain-microbiome, it also modulates many other physiological and pathophysiological processes. The effect on the immunological activity of the digestive tract and the composition of intestinal microbiota is attributed to many diet supplements. However, it does not always have a scientific basis. The following article analyzes the scientific data for four of them: vitamin D3, Lactobacillus rhamnosus GG ATCC53103, lactoferrin and inulin. 

Eating disorders are widely considered as culturally conditioned syndromes. They are an important clinical problem in the countries belonging to the Western cultural circle. Their significant increase may be observed in countries, such as Poland, that undergo the process of adoption of standards and values of the Western world. Eating disorders were first found among young women. To this day its highest prevalence occurs among women during their peripubescent period, however they can occur during early childhood or later decades of life. More and more often eating disorders affect men. Changes also occur in psychiatric classifications − currently valid diagnostic criteria are undergoing modifications and new clinical syndromes are considered. The knowledge of this subject is, for different reasons, important for pediatricians. This article will discuss several issues related to eating disorders. In the first part of the article diagnoses and diagnostic criteria for disorders belonging to a group of eating disorders. They will be complemented with a discussion of the phenomena known as sub-clinical eating disorders. Then, issues related to the impact of how eating disorders may have on pregnancy, child health and early childhood relationships, are discussed. 

In 2017 a paper was published, titled Calcium preparations do not inhibit allergic reactions. A randomized controlled trial (K. Matysiak et al. 2017)1, that reverberated across medical world in Poland. Authors of that paper stated, that the results obtained in their study allow to ultimately question applicability of calcium preparations in the treatment of allergy. In this article, we want to suggest, that they drew their conclusions too hastily and above all they based them on the results from a partially improperly designed study. We will also try to provide a background to allow sober perspective on the subject of using calcium preparations in the treatment of allergy. 

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Disorders of sex development (DSD) are a huge diagnostic and therapeutic challenge. There are many controversies regarding therapeutic methods, timing and terminology among patients and their families, but also among doctors. Proper diagnosis of clinical symptoms facilitates effective treatment. Proceedings in DSD are a diagnostic challenge due to the lack of clear guidelines and limited availability to specialists in the above field. There are only few medical centers in Poland which undertake diagnostics and therapy in these complicated cases. Recently more publications appear and Polish specialists are taking part in international projects aimed at improving the quality of care for people with this rare disorder. The aim of this work is to present the current state of knowledge on diagnostics and therapy and to indicate a model of conduct in disorders of sex development. 

Gut microbiota is a set of microorganisms inhabitating digestive tract which plays a crucial role in body homeostasis. It has several important roles, such as: regulation of the digestion, maturation of the intestinal epithelium, peristalsis, vitamin production, absorption of nutritional substances, metabolism of bile salts, fiber fermentation to short chain fatty acids (SCFA). Function of intestinal microbiota is not limited only to its’ location. It is now well proven that gut microbiota modulates immune system and central nervous system via gut-brain axis. Intestinal bacteria coordinate immune system function and first of all are responsible for immune maturation due to cytokine balance between Th1/Th2/Th17 and induction of nonspecific responses such as production of sIgA or defensins. Suboptimal microbiota profile can be responsible for various diseases: necrotizing enterocolitis, celiac disease, obesity, inflammatory bowel disease (IBD), irritable bowel syndrome (IBS), infantile colic, atopic dermatitis, autism, depression and autoimmune diseases. Thus, fetal and early postnatal period plays an important role as early gut colonization will program function of immune and nervous system determining long term outcomes. Due to these facts strong emphasis is put on first 1000 days from conception, along pregnancy and early childhood. Original, early neonatal microbiota modulates programming and function of the child gut maturation and immune system. Thus maternal diet and other environmental factors such as drugs, life style and stress during pregnancy and lactation period should be well controlled. Factors influencing maternal and neonatal microbiota can be divided into: prenatal, perinatal and postnatal factors.

Growth and weight faltering in neonates and infants occurs if their high demand for energy and nutrients is not provided. This is an alarming symptom that requires proper diagnostics and parallel nutritional intervention. If, despite the optimization of breastfeeding, abnormal growth of the child is still observed, the use of enriched formula should be considered. In premature newborns, with low body weight or with body weight too small to gestational age, it is necessary to enrich the human milk with a multi-ingredient preparation i.e. human milk fortifier. In non-breastfed preterm neonates special modified milk, for premature neonates, should be used in the hospital, and then, after discharge home, post-discharge formula, which composition had been adapted to the specific nutritional needs of this group of newborns. 

Caries is a pathological process leading to the decalcification of hard dental tissues associated with their inevitable loss. The problem of tooth decay affects more and more children with deciduous and permanent dentition. Proper prophylaxis and treatment of caries in deciduous teeth will reduce the incidence of caries in permanent teeth. The carious process has general health consequences. The severity of caries increases with age in Poland, reaching 90% between 6-7 years of age. The first years of the child are the most important period in its development, why it is so important to develop appropriate eating and hygiene habits that in the future will reflect the health of the whole child’s body. The aim of the work is to present the role of a pediatrician in the treatment of this disease entity. The possibilities of preventing the occurrence of caries were also presented. 

In recent years, the approach to the Pervasive Developmental Disorders (PDD), which include, among others, infantile autism and Asperger syndrome, has been changing. Currently this problem is considered as the Autism Spectrum Disorders (ASD). It enables more credible and reliable diagnosis, corresponding to the changing level of severity of symptoms during life. ASD is a group of neurodevelopmental disorders characterized by deficits in the field of social functioning, communication and repetitive patterns of behavior. The disorder is accompanied by numerous problems associated with the child development and his or her behavior, which often occur since early childhood and require a multidisciplinary care as well as coordinated assistance provided both to the patient and to his family. 

A cough is a physiological protective reflex, which helps to clear the airways from fluids, irritants, foreign particles and excess of secretions. The most common cause of cough are respiratory tract infections. Initially dry cough, in the second phase of the infection evolves into a productive one, accompanied by the evacuation of thick, viscous sputum. Interventions aiming to reduce the mucus viscosity, such as patients’ proper rehydration and air humidification in surrounding area, are one of the most effective strategies in respiratory infection treatment. An additional factor supporting the evacuation of the mucus from the respiratory tract are expectorants, with mucolytics among them. N-acetylcysteine   (NACC) is one of the most commonly used mucolytic. In addition to its’ mucolytic activity, acetylcysteine   also has a strong antioxidant and detoxicant functions. In this article the pathogenesis of productive cough, types of expectorants and current literature on the effectiveness and safety of NACC in respiratory infections are discussed. Although some studies have demonstrated the efficacy of NACC in the treatment of acute infections, we still need more data on its’ effectiveness and safety, to create a clear recommendation on its’ use. It is generally recommended to use NACC in chronic respiratory diseases to prevent their exacerbations. 

Diverse functions of vitamin C in human body imply that its concentration is an important homeostatic parameter. Local concentrations of vitamin C range from 50 µM (serum) up to 10 mM (neurons). The complex distribution of vitamin C in the organism is a result of its availability from diet, redistribution, facilitated by workings of vitamin C specific transporters (SVCT1 and SVCT2), and elimination due to metabolism and excretion by kidneys. The lack of an endogenous source of vitamin C and unreliable dietary intake may result with temporal or permanent deficiency. It has been demonstrated that even small, but extended in time, deficiency may cause irreversible alterations and/or degradation of an organism as demonstrated by elevated propensity to develop cancer or accelerated neurodegeneration. Changing metabolic requirements for vitamin C along with its complex pharmacokinetics require that it need to be provided in excess so even large temporal fluctuation in its metabolism will be satisfy. In the presented review, facts regarding metabolism, physiology and pharmacokinetics of vitamin C are presented. Next, based on literature data, medical consequences of vitamin C deficiency are listed along with circumstances, when vitamin C should be supplemented. 

In assessing of efficiency of children with congenital heart defects (CHD) we observe an increasing role of the ergospirometry (cardiopulmonary exercise test, CPET). The useful parameters evaluated during the test are: peak oxygen uptake (on top exercise and on the anaerobic threshold), ventilation, RERpeak and maximum heart rate (HRmax).

The aim of the study was to evaluate the cardio-pulmonary efficiency in patients with postoperative CHD in relation to population norms of healthy children (in similar age) published in literature. The correlation between peak oxygen uptake and modified NYHA-scale score, the number of past cardiac surgeries, type of CHD and physical activity at school was also evaluated.

Patients and methods: The study included CPET results (treadmill protocol Bruce-RAMP) in 30 children with different types of postoperative CHDs. The group consisted of 13 girls and 17 boys with various CHDs after repair and palliative surgeries. More than half of children participated in physical education classes (16/30).

Results: On mean RER values at the top of the exercise 0,99 for girls with CHD, and 1,04 for the boys with CHD, heart activity for girls was 173 per minute boys and 159,9 per minute for boys. Oxygen absorption at the top of the exercise (VO2peak) was 29,5 ml/kg/min for girls and 30,5 ml/kg/min for boys, and at an anaerobic threshold 19,65 ml/kg/min for girls and 20,57 ml/kg/min for boys. In all material the mean values   of VO2peak represent 60% of mean values for healthy children. Coefficient of correlation R between VO2peak and patient score on modified NYHA scale was - 0.8571 (p < 0.05). Conclusions: Children with complex CHD after repair or palliative cardiac surgery had diminished values of oxygen absorption at the top of the exercise (VO2peak), with negative correlation with patient score on modified NYHA scale  but without statistical correlation with the number of operations performed in the past. Patients after repair surgery of CHD and children which participate in physical education classes had better results during cardio-pulmonary exercise test. 

Background: Methylmalonic aciduria (MMA) is caused by an enzyme defect (methylmalonyl-CoA mutase) on the isoleucine (ILE) and valine (VAL) catabolic pathway, leading to the accumulation of toxic intermediate metabolites (including methylmalonic acid) in the body. Abnormal functioning of the enzyme may be due to its lack, decreased activity or defects of the cofactor synthesis (adenosylokobolamine, AdoCbl). MMA may have an acute course (in the form of a neonatal intoxication syndrome) or chronic, in which there is a rich symptomatology of symptoms associated mainly with damage to the nervous system and the kidneys. In the chronic form, there may also be episodes of rapid deterioration of the general condition, i.e. metabolic decompensation, which if untreated can lead to coma and death.

Methods: The aim of the study was a retrospective analysis of the clinical picture and the effects of treatment of patients with methylmalonic aciduria, remaining under the care of the Department of Paediatrics, Nutrition and Metabolic Diseases, IPCZD in Warsaw.

Results: The study presents 5 patients diagnosed with MMA acyduria associated with abnormal cobalamin A synthesis (cblA phenotype). The diagnosis of all patients was made on the basis of an extended selective screening (evaluation of urinary methylmalonic acid excretion using GC/MS). At the time of diagnosis, the median age was 18.8 months and the predominant symptom was recurrent vomiting and delayed psychomotor development. All patients were diagnosed with metabolic acidosis with a significantly increased anion gap. In 4 patients, transaminases, thrombocytopenia and elevated lactic acid were found at the time of diagnosis. All patients, from the moment they are diagnosed, remain on a limited ILE and VAL diet and receive carnitine supplementation, and three of them additionally receive vitamin B12 (hydroxocobalamin). The median duration of therapy is 12.2 years. During the treatment, a good metabolic control was obtained in all patients. Three patients have confirmed renal failure and pharmacologically treated hypertension.

Conclusions: Patients with MMA present a wide range of non-specific clinical symptoms. Inclusion of a diet with limitated ILE and VAL and carnitine and vitamin B12 supplementation minimizes the risk of metabolic decompensation, but does not protect against the occurrence of chronic renal failure. 

The recommended dose of vitamin D3 in children aged 2-10 years is within the range 600-1000 IU/day. Despite the recommended prophylaxis, the problem of vitamin D3 deficiency increases with age.

Aim: To assess the effect of vitamin D3 supplementation in the winter-spring season.

Material and Method: 73 healthy children aged 4-10 years, supplemented with vitamin D3 at a dose of 1000 IU/day were included into the prospective 3 months intervention study. 25-hydroksyvitamin D3 [25(OH)D] was determined by the chemiluminescence method. Intake of vitamin D3, body composition (bioelectrical impedance), body weight, height and BMI were assessed.

Results: Serum 25(OH)D concentration increased from 24,81 ± 7,54 ng/ml to 35,19 ± 6, 11 ng/ml (p < 0.001) in 66 children after 3 months of supplementation. The prevalence of vitamin D3 deficiency [25(OH)D < 20 ng/ml] decreased from 22.7% to zero (p < 0.001), while the prevalence of optimal vitamin D3 status [25(OH)D ≥ 30 ng/ml] increased from 24.2% to 80.3%. Final 25(OH)D concentration corelated with baseline 25(OH)D concentration (R = 0.29; p < 0.05), vitamin D3 dose per kg body weight (R = 0,43; p < 0.05), height (R = 0.35; p < 0.05), BMI (R = -0.33; p < 0.05), body fat mass (R = -0.34; p < 0.05) and child’s age (R = 0.43; p < 0.05). In a multiple regression analysis (R2 = 0.49; p = 0.004), only an association between 25(OH)D concentration and child’s age remained significant (b = -2.1; p = 0.048). Post-supplementation 25(OH)D concentration was higher in pre-school children than school children (37,55 ± 5,79 vs 33,10 ± 5,68 ng/ml; p= 0.003).

Conclusions: Vitamin D3 supplementation at a dose of 1000 IU/day eliminates the problem of vitamin D3 deficiency and provides optimal vitamin D3 status for most children aged 4-10 years. Effect of vitamin D3 supplementation is dependent on child’s age. 

Hematocolpos due to imperforate hymen may cause acute urinary retention in adolescent girls. It is not frequent but clinical presentation is so characteristic that diagnosis may be based solely on the history and physical examination and only confirmed by ultrasonography. Typical triad consists of: primary amenorrhoea, lower abdominal pain (usually cyclic) and an interlabial bulge of bluish hymen. USG reveals hyperechogenic fluid accumulation in the vagina compressing the bladder and sometimes also distended uterus (hematometrocolpos) and obstructive uropathy. Treatment of urinary retention caused by hematocolpos starts with bladder catheterization followed by hymen incision (hymenotomy) or excision (hymenectomy). We present a case of a 14-year-old girl with diagnosis of acute urinary retention caused by hematocolpos established in pediatric emergency room, treated immediately and effectively with catheterization and hymenectomy. Standardy