Anomalous origin of the right coronary artery from the main pulmonary artery treated surgically in a 6-week-old infant. A case report and review of the literature.

Background: In the present paper, the authors describe a case of a rare congenital defect - anomalous origin of the right coronary artery from the main pulmonary artery diagnosed in a 5-week-old infant who was deemed eligible for surgical treatment based solely on echocardiography. Such anatomical abnormalities of the coronary arteries are subtle and thus extremely difficult to visualize, especially in patients in whom permanent extensive damage of the cardiac muscle has not yet occurred. For this reason, the diagnosis is usually established when the disease is highly advanced, oftentimes only postmortem.

Physical activity, BMI and body weight perception among 15-year-old boys and girls in Poland in the light of international comparisons.

Background: The wrong subjective assessment of body mass may have consequences for the health, quality of life and proper functioning of adolescents in society.

Aim: The purpose of the study is to provide a detailed analysis of the discrepancies between the objective and subjective assessment of body mass by 15-year-old boys and girls in Poland against the background of international statistics and to assess the effect of physical activity on the level and directions of these discrepancies.

Material And Methods: The data concerned 72037 schoolchildren from 42 countries, including 1484 in Poland.

Impact of incorrect oral habits on mastication anomalies in children and adolescents - literature review and own observations.

Incorrect oral habits, such as dysfunctions and parafunctions, may lead to the development of mastication anomalies. The present analysis covered 15 publications from the years 2005-2015, examining the impact of harmful oral habits and dysfunctions on the development of mastication anomalies. Based on the literature review, it can be stated that malocclusion is common in populations worldwide, irrespective of the racial background.

Factors related to compliance with palivizumab prophylaxis for respiratory syncytial virus (RSV) infection - data from Poland.

Aim: To evaluate compliance and health outcomes in children receiving palivizumab prophylaxis and to identify factors that could impact parental compliance with the recommended regimen of palivizumab immunoprophylaxis.

Material And Methods: A retrospective, multicentre, non-interventional study of children enrolled in the Polish National Programme for Respiratory Syncytial Virus (RSV) Immunoprophylaxis who received ≥1 dose of palivizumab during two consecutive RSV seasons (I: 2008-2009, II: 2009-2010). For each child qualified to receive palivizumab, the following data were collected: sociodemographic factors, clinical characteristics at enrolment, and in the course of palivizumab prophylaxis.

Phenotypic variability in gap junction syndromic skin disorders: experience from KID and Clouston syndromes' clinical diagnostics.

Connexins belong to the family of gap junction proteins which enable direct cell-to-cell communication by forming channels in adjacent cells. Mutations in connexin genes cause a variety of human diseases and, in a few cases, result in skin disorders. There are significant differences in the clinical picture of two rare autosomal dominant syndromes: keratitis-ichthyosis-deafness (KID) syndrome and hidrotic ectodermal dysplasia (Clouston syndrome), which are caused by GJB2 and GJB6 mutations, respectively.

The effect of bolus and food calculator Diabetics on glucose variability in children with type 1 diabetes treated with insulin pump: the results of RCT.

Background: The calculation of prandial insulin dose is a complex process in which many factors should be considered. High glucose variability during the day, arising from difficulties which include errors made in food counting and inappropriate insulin adjustments, influence hemoglobin A1c levels. During this study, in children using insulin pumps to manage type 1 diabetes, we compared 2-h postprandial blood glucose levels (BGL) and glucose variability when calorie tables and mental calculation were used, to when Diabetics software was used.

A novel CLCN5 mutation in a boy with Bartter-like syndrome and partial growth hormone deficiency.

Dent disease is an X-linked recessive disorder affecting the proximal tubule and is characterized by low-molecular-weight proteinuria (LMWP), hypercalciuria, nephrocalcinosis/nephrolithiasis with a variable number of features of Fanconi syndrome. It is most often associated with mutations in CLCN5, which encodes the endosomal electrogenic chloride/proton exchanger ClC-5. Renal acidification abnormalities are only rarely seen in Dent disease, whereas the hypokalemic metabolic alkalosis associated with hyperreninemic hyperaldosteronism (Bartter-like syndrome) has been reported in only one patient so far.

Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment.

Aim: To identify risk factors for a child with Henoch-Schönlein purpura (HSP) either to develop nephritis (HSPN) or to contract progressive course and to obtain the currently available evidence on the efficacy of treatment options in both preventing and treating the established renal disease.

Method: Review of the literature published over the last two decades.

Results: Persistent or recurrent purpura, severe abdominal symptoms and an older age proved as the most significant risk factors for later HSPN.

Diabetic nephropathy in children and adolescents.

Type 1 diabetes mellitus (T1DM) commonly occurs in childhood or adolescence, although the rising prevalence of type 2 diabetes mellitus (T2DM) in these age groups is now being seen worldwide. Diabetic nephropathy (DN) develops in 15-20% of subjects with T1DM and in similar or higher percentage of T2DM patients, causing increased morbidity and premature mortality. Although overt DN or kidney failure caused by either type of diabetes are very uncommon during childhood or adolescence, diabetic kidney disease in susceptible patients almost certainly begins soon after disease onset and may accelerate during adolescence, leading to microalbuminuria or incipient DN.

The use of human papillomavirus typing in detection of cervical neoplasia in Recife (Brazil).

High risk types of human papillomavirus (HR-HPV) play a major role in cervical cancer oncogenesis. This study aims to evaluate the efficacy of HPV detection and typing as a means of identifying cervical neoplasia in a high risk population. A management algorithm for implementation of HPV detection in clinical practice is also proposed.