Functional gastrointestinal disorders are symptoms, that are not explained by structural or biochemical abnormalities. Definitions and diagnostic criteria
were collected in publication of Rome criteria. The current edition, Rome IV, was published in 2016. The paper presents the new classification of pediatric functional gastrointestinal disorders. Diagnostic criteria and treatment of the most frequent syndromes were discussed. The differences and most important changes made in comparison to previous Rome III criteria were underlined.

Besides inappropriate diet and insufficient physical activity, also genetic factors have a significant impact on the risk of obesity. TMEM18, TNNI3K, POMC and FTO are some of the identified genes that affect body weight in humans. The fat mass and obesity-associated gene (FTO), located on the long arm of chromosome 16, has the strongest known association with higher BMI in the human genome. Study conducted by Claussnitzer et al. indicates that some variants of the gene lead to an increased expression of the IRX3 and IRX5 genes in fat tissue. As a result thermogenesis and metabolism are decreased and lipid storage is promoted in adipocytes. The effect on the body weight may also be caused by an inhibition of adipocytes browning due to the increased expression of the IRX3 and IRX5 genes.

The authors present the summary of latest (February 2016) polish consensus guidelines on the use of acyclovir in the treatment and prevention
of VZV and HSV infections.

Acyclovir.

The authors discussed a selection of reports on liver problems discussed at the 49th Annual Meeting of European Society for Paediatric Gastroenterology,
Hepatology and Nutrition, 25-28 May in Athens.

The author discussed a selection of reports regarding pediatric gastroenterology and nutrition discussed during the 49th Annual Meeting of the European
Society for Paediatric Gastroenterology, Hepatology and Nutrition, 25-28 May in Athens.

Febrile convulsions in children under five are the most common neurological disorder. Diagnostic criteria of febrile seizures include: seizure
associated with fever over 38°C, the child’s age between 6 months and 5 years and exclusion of infection of the central nervous system.
During an episode of febrile seizures it is recommended to lay a child in the recovery position on the side, loose clothes, observe the course and duration of seizures and maintenance and monitoring of vital functions. Emergency treatment of febrile convulsions in a fit involves rectal administration of diazepam 0.5 mg per kg of body weight or by the intravenous at a dose of 0.2-0.3 mg/kg. The use of antipyretics in a fit of febrile seizures has only an impact on the alleviation of symptoms secondary to elevated body temperature. Lowering the body temperature can be achieved by the use of both physical methods and pharmacological treatment. Nowadays there are two safe and effective antipyretic drugs: paracetamol and ibuprofen. Patients with a very high and life – threatening fever should be treated with a second – line drug – metamizol, at a dose of 1-2 mg/kg. The dose of paracetamol administered orally is 10-15 mg/kg (not to exceed 90 mg/kg/day), whereas rectally in a dose of 25-30 mg/kg up to 150 mg/kg/day. Ibuprofen in children is used at a dose of 7-10 mg/kg, without exceeding the maximum daily dose of 30 mg/kg/day. The most important objective diagnosis of febrile seizures is to determine their nature, because simple febrile seizures do not require enhanced diagnostics, and only exclude infection of the central nervous system.

Atopic dermatitis is a chronic inflammatory dermatosis characterized by alternate periods of remissions and exacerbations with typical morphology and distribution of skin lesion. The basic feature of the disease is the extreme dryness of the skin and intensive pruritus. Atopic dermatitis is one of the most frequent skin diseases of the childhood and in most cases it begins in the infantile period or before the fifth year of life. Its pathophysiology is complex, thus the therapeutic strategy should be multidirectional primarily targeted on patient’s quality of life improvement and prevention of other atopic allergy symptoms. This paper reviews pathogenic issues and recent recommendations as well as practical approach to skin care among atopic children.

Pertussis is a highly contagious respiratory infection. Its incidence has increased over the last years in Poland and worldwide. It may present as a fulminant
process, mostly in unvaccinated young infants, with high mortality rate. Risk factors of mortality include hyperleukocytosis resulting in pulmonary hypertension, cardiac and respiratory failure and late onset of treatment with macrolide. Suspicion of this diagnosis should prompt diagnostic tests (PCR and
culture). Macrolide should be started without waiting for laboratory confirmation and white blood cells (WBC) count should be checked. All the infants
< 4 months as well as those > 4 months with WBC > 30 000/mm3 and/or respiratory or cardiovascular disturbances should be hospitalised. Indications for
PICU (paediatric intensive care unit) admission include: apnea with cyanosis or bradycardia, significant dyspnea, cardiac failure, tachycardia >180/min, altered
mental status, seizures and/or rapidly increasing leucocytosis. Frequent echocardiographic examinations are mandatory to diagnose pulmonary hypertension.
Double-volume exchange blood transfusion may be life-saving as it is effective in rapid reduction of WBC count, hence it decreases symptoms of leucostasis and pulmonary hypertension. In the most severe cases extracorporeal membrane oxygenation (ECMO) may be necessary. Polish management algorithm has been proposed.

Juvenile dermatomyositis is the most common idiopathic inflammatory myopathy of the developmental age. The basis of the disease are the inflammatory changes of small blood vessels. Genetic factors and autoimmune processes play a role in the etiology of juvenile dermatomyositis. Dermatitis is manifested by heliotropic upper eyelids erythema and changes of the erythematous follicular hyperkeratosis of the erect surfaces arthritic hands (Gottron’s sign). Myositis manifests itself as symmetric occupation of proximal muscles of the upper limbs, lower limbs, neck muscles and back. Work is underway to develop the criteria for juvenile dermatomyositis. The choice of juvenile dermatomyositis treatment depends on the level of disease activity and the extent of muscle inflammation. The drug of choice for the juvenile dermatomyositis treatment are the glucocorticoids, in parallel is run immunosuppressive therapy.

Interstitial lung diseases in children, also called diffuse parenchymal lung diseases, are heterogenous group of rare pulmonary disorders. Numerous possible underlying conditions, as well as the lack of clear therapeutic guidelines pose difficulties in the management of affected patients. The aim of this publication is to present how a clinician should approach to a child suspected of interstitial lung disease.

Adrenal insufficiency can result from disorder occurring on each level of the hypothalamic-pituitary-adrenal axis. In primary adrenal insufficiency the cause is intrinsic to the adrenal cortex. Secondary adrenal insufficiency is a result of impaired function of the hypothalamus and/or pituitary gland and insufficient production of CRH and/or ACTH. Symptoms are non-specific and include weakness, fatigue, lack of appetite, vomiting, weight loss and depression. The characteristic of primary adrenal insufficiency is increased pigmentation of skin resulting from excessive secretion of ACTH. Often the first manifestation of primary adrenal insufficiency is adrenal crisis, which is a medical emergency and requires immediate administration of intravenous hydrocortisone. Chronic treatment in adrenal insufficiency consist of replacement of hormonal deficiencies, prevention of adrenal crisis and, in children with congenital adrenal hyperplasia, reduce secretion of androgens. Despite the conventional replacement therapy, the quality of life of patients with adrenal insufficiency is reduced and the mortality increased. They require lifelong endocrine specialist care.

The transition between childhood and adulthood also leads to a change in incidence of various glomerulonephrits. In younger children (< 12 y.) minimal change disease (MCD) predominates. In contrast, the spectrum of adolescent glomerulopathies (> 13 y. and < 18 y.) differs from childhood and is similar to adult population. Corticosteroid-sensitive MCD remains to appear in adolescents, however IgA nephropathy and focal segmental glomerulosclerosis (FSGS) as well as nephrotic syndrome in course of membranous nephropathy (MN) and the membranoproliferative glomerulonephritis (MPGN) are more frequent in adolescents than in younger children. Therefore the final diagnosis of glomerulonephritis in adolescents should be based on the results of renal biopsy as in adult patients. Treatment in adolescents is similar to that in adults and the same drugs are used.

Necrotizing enterocolitis affects mainly premature very low weight infants. The pathogenesis of this disease is multifactorial and one of them is intestinal dysbiosis. Abberant bacterial colonization of the intestine plays an important role in the course of NEC. Now, we know, that intestinal microbiota
is important to human health. Establishing a core microbiota begins at birth, and intestinal bacteria are the first antigens faced by the immune system
of the newborn. This process may be named as microbial programming of our future health. Modulation of the intestinal microbiota of the preterm newborns by probiotics is one of the proposed methods of preventing NEC. Several studies have demonstrated the efficacy of this therapeutic strategy for the prevention of NEC. Nevertheless still there is lack of clinical guidelines advising its’ routine use in this population.

Pathological changes in the bones occur often in children and adolescents and their presence often disturb parents. Their presence not infrequently disturbs
parents. Fortunately, most of them are of a benign nature, but every year in Poland about 60 children are diagnosed with primarly malignant bone tumor. Althouh specific attention has been paid to early diagnostic of neoplasm diseases for last years, still too many patients are admitted to specialistic treatment centers with an advanced stage of disease and that influences treatment results. In this paper we would like to bring forward to the readers a group of diseases of the osseous system emphasizing those symptoms that suggest a possible malignant process, which should induce further quick diagnostic assuring, in case of confirmation of the diagnosis, early introduction of specialistic oncological treatment. Additionally, we would like to point out to the first symptoms which could suggest the development of a malignant neoplasm disease in the background of a benign change, keeping in mind that early diagnosis is one of the main prognostic factors influencing also to a large extent on the quality of life of the patient after completion of therapy.

Kangaroo Mother Care (KMC) is not only a method of care, but it has also a therapeutic value. It helps to stabilize life parameters: temperature, breathing
and heart rate, it is analgesic, and it allows a very important close “skin-to-skin” contact with mother. Possibly, KMC shows some long-term benefits for child’s development. Mother during KMC session learns to recognize, and react on her child’s needs, which is enhanced by “bonding”. KMC also has a positive influence on duration of breastfeeding. A number of hospitalizations and their duration is proved to be lower in KMC children, therefore it is beneficial for the healthcare system. However, it requires optimally educated medical professionals, ready to cooperate with the mother, and experienced enough to use this method of care even for very preterm infants. The second part of article focuses on the practical aspects of this method, according to WHO guidelines, and the benefits of KMC to mother, newborn infant and family.

Liver abscesses are rare in healthy, immunocompetent children. Clinical manifestation is non specific that is why imaging procedures play an important
role in the diagnostic management. Antibiotic therapy is a basic procedure but sometimes percutaneous drainage or other surgical procedures are possible
and curative.
The authors present a review summarizing current approach to liver abscesses in children according to literature review and own experience.

Liver poses a central role in iron metabolism. It synthesizes proteins involved in iron metabolism and serves as a magazine to it. The clue of hemolytic anemia is shortened erythrocyte life span and their premature degradation. It leads to release of iron and hem. Patients with severe disease receive frequently blood transfusions due to low red blood cell count. As a complication iron overload syndrome may develop since human body has no active way to excrete iron excess. In the state of iron overload it accumulates predominantly in liver, pancreas, heart and pituitary gland. Hem released from erythrocytes is
metabolized to bilirubin. Bile oversaturation with bilirubin poses a threat of cholelythiasis, choledocholithiasis and their complications. The authors present
the most frequently encountered hepatic and bile duct complications of hemolytic anaemias.

Mitochondrial hepatopathies are genetic inherited disorders in which liver injury is sometimes the only sign of the disease, although multiple organ manifestation is more common. Clinical symptoms can occur at any age and include different stages of liver impairment (from slightly elevated aminotransferase levels with lactic acidosis, through hepatic steatosis, portal hypertension and cirrhosis, to acute or recurrent liver failure, which is very often accompanied by neurological symptoms). Long-term outcomes of patients with primary mitochondrial hepatopathies are usually very poor and unfortunately no effective therapy for mitochondrial hepatopathies is now available. The role of liver transplantation in treating mitochondrial hepatopathies remains controversial. In the article, authors present current diagnostic and therapeutic strategies in children with suspected mitochondrial hepatopathy, considering available data about the role of liver transplantation performed in these children.

The current standard of perinatal care requires all maternity hospitals to take actions for the protection, promotion and support of breastfeeding.

Aim of the work: Analysis of the lactation procedures and the infants’ method of feeding during hospitalization and at discharge from the hospital,
based on a survey conducted among mothers of healthy babies born at term.

Material and methods: The questionnaire (using PAPI method) was conducted among 1,679 women in 42 randomly selected hospitals in Poland in
2014. Statistical analysis was carried out by TNS Poland.

Results: 72% of respondents were 25-35 years old, 70% − lived in towns and 90% had secondary or higher education. 57% of women delivered vaginally,
more than 90% newborns weighing more than 2500 g, most of them (53%) were the second and next child of the women participating in the
study. “Skin to skin” contact immediately after birth applied to 64% of mother – child pairs, but only 29% − for 2 hours and longer. Continuous “rooming-
in” from birth to discharge was allowed to 74% of women, cesarean section being the most common reason for separation (70%). 97% of women
started breastfeeding immediately after childbirth, but as many as 55% of newborns during hospitalization was supplemented, mainly by formula.
On the day of discharge from hospital, only 75% of infants were exclusively breastfed.

Conclusions: The results show a low level of implementation of procedures promoting breastfeeding, which are a current standard of perinatal care
in Polish hospitals. 97% of women initiates breastfeeding, but only 75% of infants were exclusively breastfed at discharge. Education of mothers in
hospitals should be preceded by education of medical staff. It is necessary to take steps that will lead to the implementation of existing law standards.

Gruczolakorak jelita grubego najczęściej dotyczy osób po 40. r.ż. Rzadko występuje u młodzieży i zwykle jest wówczas konsekwencją zespołu genetycznego. Celem artykułu jest zwrócenie uwagi na ważny problem kliniczny, jakim jest zachorowalność w młodym wieku na raka jelita grubego chorych na mukowiscydozę.
Autorzy chcieliby podkreślić potrzebę wykonywania badań kolonoskopowych w tej grupie pacjentów.