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The last two decades have seen an increase in the incidence of inflammatory bowel disease (IBD) in many regions of the world, which has had a significant impact on both the social and economic burden of governments and healthcare systems. The aim of this study was to determine the level of hospitalization and outpatient treatment costs for children and adolescents with Crohn's disease and ulcerative colitis, depending on age, location, and activity of the disease. Methods were a retrospective analysis of the medical documentation of 240 children with IBD, hospitalized in the Gastroenterology Ward, Department of Pediatrics Medical University of Silesia (Katowice, Poland), along the three years follow up. The costs of treatment consisted of calculations of the supply of oral and intravenous drugs, calculations of the costs of laboratory tests, imaging, and consultations, as well as person-day costs. The most important results, determined with high costs of IBD treatment, are associated with younger age, high disease activity, localization in the small intestine in Crohn's disease (CD), and inflammatory changes in the entire colon in Ulcerative Colitis (UC). During the observation, it was noticed that the shortening of the hospitalization time did not significantly affect the total costs, which remained at a stable level.

Background: Pregnancy is a condition in which new cardiac arrhythmias can occur or prior undiagnosed arrhythmias may provide symptoms. The occurrence of severe ventricular arrhythmias and polymorphic ventricular tachycardia that may lead to fainting or sudden cardiac death is promoted by the prolongation of the QTc interval. The post-partum adaptation period is the most arrhythmogenic. TpTe (Tpeak-Tend interval) is a novel marker of arrhythmogenesis by many considered a more sensitive marker than QTc., Objective: The aim of our work was to determine the TpTe interval (Tpeak-Tend) in women in the first, second and third trimester of pregnancy and the post-partum period., Materials and Methods: The study group consisted of 128 women in pregnancy or postpartum and a control group of 32 non-pregnant women. A standard 12-lead ECG (electrocardiograph) recording with evaluation of the duration of TpTe and QTc was performed in all patients., Results: In comparison to the non-pregnant women, higher values of QTc and TpTe were observed starting in the first trimester with highest values observed in the postpartum period. Mean duration of TpTe interval during pregnancy (81.59 ± 5.92 ms) and in the whole study group (pregnancy + postpartum) (85.46 ± 6.45 ms) was significantly longer ( p < 0.001) compared to the TpTe interval in the control group (74.06 ± 6.14 ms). During pregnancy and postpartum, the increase in the TpTe interval in comparison to the increase in the QTc parameter (31.10% vs. 4.18%) was significantly higher ( p < 0.001)., Conclusions: The study showed an increase in the duration of the TpTe interval and QTc parameter during pregnancy and postpartum with the highest values in the postpartum period. TpTe interval increase was significantly higher compared to QTc increase during pregnancy and postpartum. Changes of TpTe interval were not associated with any clinical outcome or measure of arrythmia burden. Further studies are needed in order to see the clinical significance of these ECG findings, in particular for larger groups of patients with automatic measurement in correlation with echocardiography.

Background: The changes in the period of ventricular repolarization, i.e., QT interval, QTp (Q-Tpeak) and TpTe interval (Tpeak-Tend), make it possible to assess the electrical instability of the heart muscle, which may lead to the development of life-threatening ventricular arrhythmia. The aim of the study was to determine and evaluate the use of differences in T-wave morphology and durations of repolarization period parameters (QT, TpTe) in resting ECGs for children with ventricular arrhythmias., Methods: The retrospective analysis was made of the disease histories of 80 examined children with resting ECGs, which were admitted to the Children's Cardiology Department. The study group consisted of 46 children aged 4 to 18 with ventricular arrhythmias and the control group consisted of 34 healthy children between 4 and 18 years of age, with no arrhythmias., Results: The duration of the TpTe interval was significantly ( p < 0.001) longer in the group of children with ventricular arrhythmia with abnormal T-wave (bactrian/bifid, humid/biphasic) compared to the TpTe interval in children with ventricular arrhythmia with the normal repolarization period. The duration of the TpTe ( p < 0.001), QTcB ( p < 0.001) and QTcF ( p < 0.001) intervals were significantly longer in the group of children with ventricular arrhythmias and with abnormal T-wave compared to the values of the TpTe, QTcB, and QTcF intervals of the control group with normal morphology of the repolarization period. Only the duration of the TpTe interval was significantly ( p = 0.020) longer in the group of children with ventricular arrhythmia without clinical symptoms., Conclusions: Children with benign ventricular arrhythmias recorded on a standard ECG with prolonged TpTe and QT intervals and abnormal T-wave morphology require systematic and frequent cardiac check up with long term ECG recordings due to the possibility of future more severe ventricular arrhythmias. Further follow-up studies in even larger groups of patients are necessary to confirm the values of these repolarization parameters in clinical practice.

The head-up tilt table test (HUTT) with the upright phase is used to help determine an imbalance of the sympathetic nervous system that is related to abnormal electrocardiographic repolarization in children with vasovagal syncope (VVS) and also in patients with the long QT syndrome (LQTS). The study attempted to evaluate T wave morphology and QT and TpTe (Tpeak-Tend) intervals recorded in ECG during the HUTT for a more accurate diagnosis of children with VVS. The group investigated 70 children with a negative HUTT result: 40 patients with VVS and 30 healthy volunteers without syncope. The RR interval as well as TpTe, and QTc intervals were measured in lead V5 of electrocardiogram (ECG) on admission to the hospital and during three phases of the HUTT. In syncopal children, which included 23 children with bifid or flat T waves and 17 patients with normal T waves in the upright phase, the QTc and TpTe were longer ( p < 0.001) compared to the other test phases and longer ( p < 0.001) than in the control group, respectively, with the risk of arrhythmias. Only in the control group, the TpTe was shorter ( p < 0.001) in the upright phase than in the other tilt phases. The TpTe in the upright phase (>70 ms) was a good discriminator, and was better than the QTc (>427 ms). Prolongation of electrocardiographic TpTe and QT intervals, in addition to the (abnormal T wave morphology recorded during the HUTT, are helpful for identifying VVS children more predisposed to ventricular arrhythmias with a latent risk of LQTS. Further studies are required to assess the value of these repolarization parameters in clinical practice.

(1) Background: The features characterizing vasovagal syncope (VVS) are an important factor in the correct evaluation of diagnostic risk stratification in children and adolescents. The aim of the study was to determine the value of identifying the clinical characteristics in children with VVS. (2) Methods: We made a retrospective analysis of the medical records of 109 children with diagnosed VVS. We investigated the specific characteristics of syncope in children with VVS including the positive VVS (+) and negative VVS (-) result of the Head-Up Tilt Table Test (HUTT). (3) Results: We did not observe significant differences in the prodromal symptoms of VVS with HUTT response. In addition to typical prodromal symptoms, no difference in statistically reported palpitations (35/109 or 32.1%) and chest discomfort (27/109 or 27.7%) were recorded. Fear-pain-stress emotions as circumstances of syncope were more often reported by children with a negative HUTT ( p = 0.02). Cramps-contractures ( p = 0.016) and speech disorders ( p = 0.038) were significantly higher in the group with negative HUTT. (4) Conclusions: There is a close relationship in the diagnostic profile between the negative and positive results of head-up tilt table test in children with vasovagal syncope.

Background: Electrocardiography (ECG) and the head-up tilt test (HUTT) are vital in clinical work-up in children with vasovagal syncope (VVS). Ventricular repolarization parameters (QT) measured during the HUTT can be indicative of electrical instability; however, these parameters are not frequently assessed. This study aimed to investigate if ventricular repolarization parameters measured during the HUTT could be indicative of future ventricular arrhythmias in children with syncope., Methods: The shape and amplitude of the T-wave and parameters of the repolarization period (QT, QTpeak, Tpeak-Tend) were evaluated in a resting ECG performed on the first day of hospitalization and in ECGs performed during three phases of the HUTT., Results: In the after-tilt phase of the HUTT, 19/30 children displayed a change in T-wave morphology. QTc was significantly longer in VVS I compared to that in VVS II patients, but not in the controls ( p = 0.092)., Conclusions: We need further follow-up studies to establish the clinical importance of abnormal dynamics of the repolarization period in children with VVS and negative HUTT. Therefore, children with abnormal T-wave refraction and prolonged duration of the TpTe should remain under the care of a cardiological outpatient.

Background: T-wave parameters, especially the Tpeak-Tend interval (TpTe), reflect the total dispersion of repolarization, whose amplification may lead to the development of life-threatening ventricular arrhythmias observed in the long QT syndrome (LQTS)., Objectives: The study attempted to evaluate QT, QTp (Q-Tpeak) and TpTe (Tpeak-Tend) intervals in unaffected and affected blood relatives of children with clinically confirmed LQTS as well as to determine whether the values of these repolarization parameters may be used in clinical practice., Material and Methods: The study group included 47 affected blood relatives (27 LQTS1 and 20 LQTS2) and 68 unaffected family members without clinically confirmed LQTS symptoms. The TpTe, QT and QTp intervals were measured manually in the lead V5 of standard ECGs and corrected using Bazett's and Fridericia's formulas., Results: The RR, QT, QTp and TpTe intervals with their corrected values were significantly longer (p < 0.0001) in the affected subjects than in the unaffected subjects and, similarly, in LQTS1 and LQTS2 patients compared with the unaffected family members. The TpTe interval in LQTS2 showed only a tendency to be longer compared to LQTS1, but did not reach statistical significance (p = 0.0933). For affected blood relatives, only the TpTe interval (p < 0.0409) and QT interval, corrected with Bazett's (p < 0.0393) and Fridericia's (p < 0.0495) formulas, enabled differentiation between LQTS1 (mean TpTe = 103 ±15) and LQTS2 women (mean TpTe = 106 ±17). Moreover, there were statistically significant differences (p < 0.05) in the TpTe interval between the 6 sex subgroups: unaffected women and men as well as women and men with LQTS1 and LQTS2., Conclusions: The electrocardiographic Tpeak-Tend parameter, in addition to the QT interval, is helpful in identifying affected blood relatives of children with LQTS, particularly for the group of LQTS1 and LQTS2 women. Further studies are required to assess the clinical importance of the TpTe interval in families with long QT syndrome.

Introduction: The main goal of this study was to examine the patient age and sex dependent expression of KCNQ1 and HERG genes that encode potassium channels responsible for the occurrence of long QT syndrome (LQTS)., Material and Methods: The study enrolled 43 families whose members suffered from LQTS type 1 (LQTS1) or 2 (LQTS2) or were healthy. The study attempted to prove that β-actin is a good endogenous control when determining the expression of the studied genes. Examination of gene expression was achieved with quantitative real-time PCR (QRT-PCR). Expression of the investigated genes was inferred from the analysis of the number of mRNA copies per 1 μg total RNA isolated from whole blood., Results: Significantly lower KCNQ1 and KCNH2 mRNA levels in healthy females than healthy males were observed (p = 0.032; p = 0.02). In male patients both transcripts were expressed at a lower level (p = 0.0084; p = 0.035). The comparison of transcriptional activity of KCNQ1 and KCNH2 in healthy adults and children revealed higher KCNQ1 and lower KCNH2 mRNA levels in healthy adults (p = 0.033; p = 0.04), higher KCNQ1 and lower KCNH2 mRNA levels in adult patients below 55 years old than in adults over 55 years old (p=0.036; p = 0.044), and significantly higher KCNQ1 and lower KCNH2 mRNA levels in adult patients (over 55 years) than in paediatric patients (below 15 years) (p=0.047; p = 0.08)., Conclusions: The results support the hypothesis that KCNQ1 and HERG gene expression is influenced by age and gender in human patients with long QT syndrome and in healthy subjects.

Background: The KCNQ1 and HERG genes mutations are responsible for specific types of congenital long QT syndrome (LQT)., Aim: To examine the expression of KCNQ1 and HERG genes that encode potassium channels (rapid and slow) responsible for the occurrence of particular types of LQT syndrome. The study also attempted to prove that beta-actin is a good endogenous control when determining the expression of the studied genes., Methods: The study enrolled six families whose members suffered from either LQT1 or LQT2, or were healthy. Examination of gene expression was achieved with quantitative PCR (QRT-PCR). Expression of the investigated genes was inferred from the analysis of the number of mRNA copies per 1 mg total RNA isolated from whole blood. On the basis of KCNQ1 gene expression profile, the presence of, or absence of, LQT1 could be confirmed., Results and Conclusions: The study revealed a statistically significant difference (p = 0.031) between the number of KCNQ1 gene copies in patients and healthy controls. On the basis of HERG (KCNH2) gene expression profile, patients with LQT2 cannot be unequivocally differentiated from healthy subjects (p = 0.37).

Background: Ventricular arrhythmias are the most common consequences of structural and functional heart diseases, but cases with no evident pathology are also observed. A parameter indicating asymptomatic circulatory failure could support decisions related to possible treatment of ventricular arrhythmias., Hypothesis: The study objective was the evaluation of N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels in children with ventricular arrhythmias and an attempt to determine if this parameter may be used for diagnosis and prognosis of ventricular arrhythmias., Material and Methods: The study population was comprised of 36 children age 5 to 17.5 years old with idiopathic ventricular arrhythmias (Group B) graded mild or potentially malignant; 29 patients with mild ventricular arrhythmias were included into Group B1; and 7 patients with potentially malignant cases into Group B2. In all the patients, NT-proBNP assays were performed., Results: The NT-proBNP levels in Groups B, B1, B2 and the control group (Group K) were as follows: 41.5 +/- 15.1 pg/mL, 35.5 +/- 18.5 pg/mL, 66.3 +/- 24.9 pg/mL and 31.5 +/- 15.1 pg/mL, respectively. Between the groups with and without arrhythmias (Group B vs Group K), no statistically significant differences in NT-proBNP levels were found. However, markedly higher NT-proBNP levels were shown in the children with potentially malignant arrhythmias (Group B2) compared to the patients with mild arrhythmias (B1) and the control group (Group K)., Conclusions: The level of NT-proBNP increases with the severity of ventricular arrhythmia. NT-proBNP assays can be helpful for diagnosing and grading the severity of ventricular arrhythmias.

Background: Mitral valve prolapse (MVP) is the most common primary valvular abnormality in a young population. In some individuals, MVP is silent or associated with palpitations, dizziness, chest pain, and abnormal electrocardiogram (ECG) repolarization with or without ventricular arrhythmias., Hypothesis: The aim of the present study was to assess the occurrence of the clinical and electrocardiographic abnormalities in young athletes with silent MVP., Methods: A group of 10 children, who have been sport training intensively, with preparticipation silent MVP were examined for symptoms and/or ECG abnormalities. The diagnosis of MVP was made by echocardiography., Results: Three athletes were asymptomatic at initial presentation. The other 7 athletes presented with symptoms. The QTc intervals > 440 msec were recorded in 2 athletes (1 with syncope). Abnormal ECG repolarization was found in 7 athletes (4 athletes were symptomatic and 3 were asymptomatic). A large variety of T-waves was registered in athletes who presented with symptoms. In asymptomatic athletes, the tall and flat T-waves were recorded., Conclusions: Young athletes with MVP are often predisposed to electrocardiographic abnormalities of ventricular repolarization, which requires annual cardiologic evaluation.

This review discusses the risk of cardiac events and genotype-based management of LQTS. We describe here the genetic background of long QT syndrome and the eleven different genes for ion-channels and a structural anchoring protein associated with that disorder. Clinical Background section discusses the risk of cardiac events associated with different LQTS types. Management and Prevention section describes in turn gene-specific therapy, which was based on the identification of the gene defect and the dysfunction of the associated transmembrane ion channel. In patients affected by LQTS, genetic analysis is useful for risk stratification and for making therapeutic decisions. A recent study reported a quite novel pathogenic mechanism for LQTS and suggested that treatments aimed at scaffolding proteins rather than specific ion channels may be an alternative to antiarrhythmic strategy in the future.

Isolated noncompaction of the ventricular myocardium (INVM) sometimes referred to as spongy myocardium is a rare, congenital and also acquired cardiomyopathy. It appears to divide the presentation into neonatal, childhood and adult forms of which spongy myocardium and systolic dysfunction is the commonality. The disorder is characterized by a left ventricular hypertrophy with deep trabeculations, and with diminished systolic function, with or without associated left ventricular dilation. In half or more of the cases, the right ventricle is also affected. The sporadic type, however, in some patients, may be due to chromosomal abnormalities and the occurrence of familial incidence. Isolated noncompaction of the left ventricular myocardium in the majority of adult patients is an autosomal dominant disorder. The familial and X-linked disorders have been described by various authors. We here describe the genetic background of this disorder: some of the most mutated genes that are responsible for the disease are (G4.5 (tafazzin gene): alpha-dystrobrevin gene (DTNA); FKBP-12 gene; lamin A/C gene; Cypher/ZASP (LIM, LDB3) gene); and some genotype-phenotype correlations (Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy or Barth syndrome) based on the literature review., ((Copyright) 2008 Wiley Periodicals, Inc.)

We describe the clinical and genetic characteristics of the family, in which the diagnosis of LQT1 had been made. The electrocardiogram (ECG) characteristics of this patient indicated the likelihood of LQTS1. Polymorphic ventricular extrasystolies and episodes of polymorphic non-sustained ventricular tachycardia were confirmed by Holter ECG monitoring. On the exertional electrocardiogram polymorphic ventricular tachycardia (torsade de pointes) was recorded. Direct sequencing of both DNA strands revealed the absence of mutations or polymorphisms in the KCNQ1, HERG, and SCN5A genes.

Arrhythmogenic right ventricular dysplasia (ARVD) is a complex arrhythmogenic cardiomyopathy, characterized by a partial or total replacement of the right ventricular myocytes by fatty and fibrous tissue. In this study, we present a case of ARVD in 17 year old girl, who was admitted to the hospital after syncope with ventricular arrhythmia. The echocardiography did not demonstrate structural cardiac abnormalities but the magnetic resonance recently showed thinning of the right ventricular wall. The girl was treated with the lidocaine, amiodarone and next, after radiofrequency catheter ablation she was receiving metoprolol. The girl has remained asymptomatic for four years of follow-up.

Background: About 5-15% of children suffer from hypertension. The influence of maturity and the physical development of newborns on primary hypertension has been recently underlined., Aim: The aim of the study was to estimate the importance of low birth weight (< 10 percentiles--2500 g), low birth length (< 10 percentiles--50 cm), low Apgar score (< 4 points), prematurity (< 37 Hbd) in the developmental of hypertension in children., Methods: The project was conducted retrospectively in a group of 197 children with primary hypertension (aged 15.4 +/- 1.9) and 119 healthy children (aged 12.9 +/- 3.1) as the control group. The statistical analysis was performed with Epilnfo 6.0 procedures., Results: Male sex, overweight and family history of hypertension increased the risk of hypertension in a statistically significant way and the adequate estimated odds ratios were: OR = 3.69 (95% CI: 2.20-6.19), OR = 22.76 (95% CI: 6.61-94.12), OR = 21.87 (95% CI: 6.27-91.1). No influence of low birth weight, low birth length, low Apgar score and prematurity on primary hypertension was observed., Conclusion: The results suggest that the role of low birth weight, low birth length, low Apgar score and prematurity in the development of hypertension in children is arguable. It also suggests the necessity to conduct epidemiologic surveys in more frequent groups of children.

There is some evidence that deaf children are more threatened than the general population by dangerous heart arrhythmias. An example is Jervell-Lange-Nielsen syndrome (one of the forms of long QT syndrome) which is characterised primarily by congenital deafness and prolongation of the QT interval. The aim of this study was to perform preliminary cardiological examinations on 162 deaf children (76 girls and 86 boys, 3-15 years old, mean age 10.5 +/- 2.8 years) who attend the Regional School Centre for Deaf Children in Katowice. The data in our analysis was obtained from case histories (school records and special questionnaires sent to parents), physical examinations with special regard to the cardiovascular system, double blood pressure measurement and 12 lead surface electrocardiogram. In the studied group, 90 children (55.5%) were congenitally deaf. Within this group, 24 children manifested tachycardia while 4 children manifested-bradycardia. A-V block of I degree was found in 3 children. Incomplete right bundle branch block (RBBB) was observed in 44 children while a complete RBBB was noticed in 1 child. QTc interval was prolonged (> 0.44s) in 12 children suffering from congenital form of deafness and in 16 children suffering from all causes of deafness. On the basis of case history, physical examination, and standard 12-lead electrocardiogram, we established the occurrence of risk factors according to the new diagnostic criteria of Schwartz et al. with relative points assigned to the electrocardiographical, clinical and familial findings. These points when summed up represent the risk of sudden death caused by ventricular arrhythmia due to long QT syndrome. In the studied group (according to the Schwartz criteria), 2 (1.2%) children had high probability (> or = 4 points) of long QT syndrome (LQTS), 25 (15.4%) children had intermediate probability of LQTS (2-3 points) and 135 children had low probability of LQTS (< or = 1 point).