The paper presents recommendations for the management of arterial hypertension in children and adolescents based on previously published recommendations
of the 4th Report of the National High Blood pressure Working Group, pediatric recommendations of European Society of Hypertension, European
Society of Cardiology, American Heart Association, American Academy of Pediatrics, recommendations of other scientific societies, literature review and expert
opinions. Presented recommendations are an extended version of previously presented by the authors, pediatric recommendations of Polish Society
of Hypertension published in 2015. At presented version authors added epidemiological information on the prevalence and incidence of hypertension in
the developmental age, modified sections on the diagnosis and assessment of target organ damage, arterial hypertension in children with diabetes, chronic
kidney disease, and the section discussing diagnostic and therapeutic difficulties in determining the proper blood pressure values. Modified definitions
of blood pressure status and interpretation of differences between pediatric and adult definitions of hypertension have been added.

Vitamin K supplementation regimens are present in many countries in order to prevent vitamin K deficiency bleedings (VKDB). However, optimal dosing
and administration route of vitamin K are still unclear. VKDB risk is especially high in breastfed infants with cholestasis. Danish and Dutch registries of patients
with biliary atresia allow a comparison of efficacy of the countries’ prophylactic regimens in this high-risk population.

Vitamin K supplementation regimens are present in many countries in order to prevent vitamin K deficiency bleedings (VKDB). However, optimal dosing
and administration route of vitamin K are still unclear.

Risk of allergic disease development may depend on the composition of intestinal microbiota. In 2016 World Allergy Organization published recommendations,
based on available evidence, about the use of prebiotics in the prevention of allergy.

Ciliopathies are a clinical heterogeneous group of diseases that arise as a result of ciliary dysfunction. There is an increasing in the topic of ciliopathies,
mainly due to the progress in the basic sciences, but the knowledge about them among clinicians is still not common. The authors present the general
characteristics of these diseases.
 

Infant regurgitations are the most common functional disorders of the gastrointestinal tract in the first 12 months of life. Symptoms are most
often mild and improve spontaneously by the end of first year of life. Medical history and physical examination are mandatory and sufficient
to exclude organic disease. If no red flag symptoms exist, reassurance of the caregivers of the natural course of infant regurgitation is the basic
management. There is evidence of positive effect of thickening milk and giving certain strains of probiotics. There are multiple randomized trials
showing a lack of benefit of pharmacological treatment in infant regurgitation.

Mammary glands enlargement in children always arouse anxiety in their parents. The term precocious puberty is defined as the appearance of secondary
sex characteristics before the age of 8 in girls. There are two major forms of premature sexual maturation: gonadotrophin-dependent (central precocious
puberty) and gonadotrophin-independent precocious puberty, which is characterized by increased production of sex steroids with the absence of reactivation
of the hypothalamic-pituitary axis. Girls with precocious thelarche without growth acceleration present the benign variant of puberty and need
clinical follow up only. We review the etiopathogenesis, clinical course, and diagnosis various disorders causing thelarche and gynecomastia.

Kangaroo Mother Care (KMC) is a method of care, which meets not only the needs of a newborn on a physiological, but also on an emotional level. During
KMC, the mother and her child form an inseparable mother-infant dyad. The child, lying on mother’s breast experiences an intimate, skin to skin contact,
which allows to form an unique bond between them. An important part of KMC is breastfeeding, by which a newborn receives all necessary nutrients, but
also other components which influence his future development. KMC was first described in countries with low-income, where access to incubators and other
specialized medical equipment remains limited, but nowadays is also gaining importance in civilized regions of the world. Publications from all over the
world point at the safety of this method, even for preterm infants. It’s possible to perform KMC even in the NICU (Neonatal Intensive Care Units), if child’s parameters
are being monitored. This part describes the method itself, and indications for applying it basing on the WHO guidelines and the world literature.

Current clinical guidelines issued by European neonatal societies as well as by American Academy of Pediatrics strongly suggest use of noninvasive modes
of ventilation support from the first minutes of life in all premature infants with signs of spontaneous respiration. Effectiveness of noninvasive ventilation
depends on many prenatal and postnatal factors. When respiratory failure progresses to more severe forms of the disease, exogenous surfactant instillation
is the next step of treatment algorithm. Nevertheless, this is an invasive procedure which requires use of laryngoscope, endotracheal tube or other catheter
which can be inserted below vocal cords. Presence of severe adverse events as well as need for use of certain medication cause many clinicians questioning
when this procedure should be performed and which method of surfactant administration should be utilized in order to increase chances of avoiding
re-intubations and mechanical ventilation. The crucial question remains: how to provide non-invasive breathing support in order to avoid intubation as
well as other severe long and short term complications among premature newborns?

Primary ciliary dyskinesia is a rare genetic disease. Symptoms usually appear during early childhood. The clinical manifestation of primary ciliary dyskinesia
involves recurrent infections of the upper and lower respiratory tract, infertility and abnormal lateralization.
In this paper authors shortly discuss the clinical picture and current diagnostic approaches.

Inborn errors of metabolism (IEM) remain a significant challenge for paediatricians, due to their rarity, diversity of the clinical picture and difficulty of proper
diagnostics and therapy. Thanks to worldwide progress in diagnostics and therapy of IEM, there is an increased need for continuous training of Polish
physicians just to offer patients with IEM the most up-to-date form of therapy. Nowadays in selected IEM (MPS I, MPS II, MPS IV, MPS VI, Gaucher Disease,
Fabry Disease and Pompe Disease) we have the possibility to implement enzyme replacement therapy (ERT) which improves prognosis and quality of life
of the patients. However, the knowledge about the possibilities of hematopoietic stem cell transplantation (HSCT) in IEM, which in many cases can stabilize
the disease progression and improve patient’s condition still remains unsatisfactory. We present a case of an 8,5 month-old boy (at the time of HSCT)
with Hurler syndrome (mucopolysaccharidosis type 1, MPS IH) in whom we have performed the first HSCT in this disease in Poland. The boy underwent
2 transplantations in the interval of one month – the first form umbilical cord blood (UCB) but due to the absence of haematological recovery within 28
days after HSCT he was qualified for the second transplantation from matched unrelated donor (MUD). Since the age of 4 months the patient has been on
ERT (laronidase – Aldurazyme®), which was discontinued on month 6 after the second HSCT due to the normal levels of the enzyme, L-α-iduronidase, in his
peripheral blood (PB). Currently, more than one year after HSCT, tests confirm 100% allogenic chimerism in patient’s PB. The boy is in very good general
condition. His organomegaly and dilated cardiomyopathy resolved, joint contractures are giving away and he makes up the for the delay in reaching milestones
in his psychophysical development.

Introduction: An intracranial haemorrhage (ICH) in full-term neonates may be associated with the risk of the health and life-threatening complications as
well as with negative impact on their further development.
Objective: The aim of this study is to draw attention to the cause, clinical manifestation, and complications occurring in full-term newborns that have gone
ICH. An attempt was made to develop an algorithm for dealing with suspected intracranial haemorrhage in the full-term newborn, taking into account our
own experience and data from the literature.
Methods: Retrospective analysis was performed toward 86 full-term newborns who were hospitalized in clinic in year-range-2007-2013 due to bleeding
into the CNS (> Second Grade scale Papille’a).
Results: An intracerebral haemorrhage was diagnosed in 77 patients (89,55%). There were only 13 cases of isolated intracerebral haemorrhage while remaining
64 cases were associated by intraventricular or extracerebral haemorrhage. An isolated intraventricular haemorrhage was diagnosed in 7 cases, while
an isolated subdural or subarachnoid haemorrhage in only single cases.
In all patients first diagnostic test was US. In 75 cases extension of the diagnosis of CT and/or MRI was required. In most cases conservative treatment was
used, but in 16 patients neurosurgical treatment was required because of the need for evacuation of hematoma or development of hydrocephalus.

Knowledge and awareness of nutritional recommendations may determine compliance with the principles of proper nutrition and translate into improved
quality of the diet of children in nurseries. The aim of this study was to evaluate the effect of nutrition education of nurseries’ personnel on the implementation
of the recommendations of the model daily food ration with special emphasis on the supply of fruit and vegetables, milk and milk products in menus.
The study was carried out in the years 2015-2016; included 35 nurseries (24 from Warsaw and 11 other cities). Menus and warehouse reports of 10 consecutive days (700 documents) acquired before (I phase) and after 3 months of nutrition education (second stage) were analyzed. Before education the model daily food ration was realized at a minimum of 70% in respect of potatoes, vegetables, fruit, cottage cheese, hard cheese and fish. Too low average intake was observed in the case of flour and pasta, cereal, rice and cereals, milk and dairy fermented beverages, and vegetable fats; too high MRP implementation was recorded in relation to bread, meat, poultry and meats, animal fat and sugar and sweets. Nutritional education of staff significantly contributed to increasing of the supply of vegetables and dairy fermented beverages on nurseries’ menus.

Hepatopulmonary syndrome (HPS) is a severe complication of liver disease. Liver metabolism disturbances causes an increase concentration of vasodilatation
substances, which leads to pulmonary vascular dilatation and development of intrapulmonary arteriovenous shunts. Pharmacotherapy and medical
treatment leading to decrease portal pressure can temporary improve the clinical condition of patients. Liver transplantation is the only definitive therapy.
We describe a case of 4.5-year-old boy with liver cirrhosis caused by biliary atresia complicated by development of HPS. HPS was confirmed by lung perfusion
scintigraphy. Spontaneous regression of HPS was noted after two years of observation.

Medical practice should be based on current and up-to-date medical knowledge, because of both, ethical and legal obligations. However, neither the term
“current medical knowledge” nor its reliable sources are precisely defined in the Polish law. The article discusses the aforementioned issues and describes
the role of guidelines and recommendations in this context.

Vitamin K constitutes collective name for a group of micronutrients – naphtoquinones of natural origin, which feature as a principal biological activity posttranslational carboxylation of glutamic acid residues in proteins acting as clotting factors. Lack of vitamin K leading to spontaneous hemorrhaging is rare,
because of ubiquitous presence of phylloquinone in plants, except for newborn and breast-fed infants, for which vitamin K deficiency bleeding is potentially
life treating. This paper explains biogenesis of naphtoquinones and discuss their biological roles in humans. Finally, sources of vitamin K active substance(
s) for supplementation, with focus on chemical synthesis, are presented.

Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome complicated both phenotypically and genetically. Analysis of prenatal period can provide
the first evidence, and allows the team to prepare for the birth of a sick child. Neonatologist being the first doctor taking care of the baby after birth,
has a major role in suggesting the diagnosis of BWS but molecular confirmation is necessary. According to an increased risk for embryonal tumor development,
the prophylaxis need to be taken. Children with BWS need a multi-specialty care.