Chronic Granulomatous Disease Caused by a Novel Mutation in a 2-Month-Old Boy With Multifocal Splenic Abscesses

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Cardiac autonomic control in adolescents with primary hypertension.

BACKGROUND: Impairment in cardiovascular autonomic regulation participates in the onset and maintenance of primary hypertension. OBJECTIVE: The aim of the present study was to evaluate cardiac autonomic control using long-term heart rate variability (HRV) analysis in adolescents with primary hypertension. SUBJECTS AND METHODS: Twenty two adolescent patients with primary hypertension (5 girls/17 boys) aged 14-19 years and 22 healthy subjects matched for age and gender were enrolled. Two periods from 24-hour ECG recording were evaluated by HRV analysis: awake state and sleep. HRV analysis included spectral power in low frequency band (LF), in high frequency band (HF), and LF/HF ratio. - RESULTS: In awake state, adolescents with primary hypertension had lower HF and higher LF and LF/HF ratio. During sleep, HF was lower and LF/HF ratio was higher in patients with primary hypertension. CONCLUSIONS: A combination of sympathetic predominance and reduced vagal activity might represent a potential link between psychosocial factors and primary hypertension, associated with increased cardiovascular morbidity.

Cough reflex sensitivity in various phenotypes of childhood asthma.

Cough is a major symptom in some children with asthma, but the relationship between cough and the severity of asthma is defined insufficiently. As cough represents common problem of pediatrics, several objective methods for its assessment were developed. Cough reflex sensitivity (CRS) test with capsaicin is one of the most important tools for studying cough. In the present study, we aimed to study the CRS in various phenotypes of childhood asthma. We found that, in general, CRS was increased in asthmatic children compared with controls. The most evident increase of CRS was observed during acute asthma exacerbation, in children suffering from asthma with concomitant allergic rhinitis, and in atopic asthmatics. Interestingly, we noted a significant decline in lung function after capsaicin CRS. Various laboratory and clinical characteristics of asthmatic children influence cough sensitivity to a different extent. Cough reflex sensitivity measurement can add valuable information beside the commonly used spirometric and inflammometric methods in the management of asthmatic children.

Therapeutic approach to a child with acute respiratory distress syndrome: a report of two cases.

The course of a respiratory disorder in a child may end up in respiratory failure. There are also acute non-respiratory diseases which have a great influence on the respiratory functions and often lead to the acute lung injury and sometimes to the acute respiratory distress syndrome (ARDS). A feature of respiratory function deterioration is changed in the surfactant system. We often see inhibition of its synthesis or damage to its structure. Therapy of children suffering from ARDS should be complex and rapid. Despite many recently published studies explaining the principle of this disorder, the mortality of ARDS is still very high (30-50%). There are several studies documenting successful administration of exogenous surfactant as part of a complex combined therapy of patients with ARDS, which leads to decreased mortality, improved oxygenation, and decreased need for aggressive artificial pulmonary ventilation. The authors of this article present their own experience with administration of exogenous surfactant in therapy of children with ARDS.

Food allergens and respiratory symptoms.

Food allergy may be clinically expressed by a variety of respiratory symptoms, which can be provoked either by IgE- or cellular mediated reactions. Among the diagnostic procedures, newly introduced atopy patch test seems to be important for diagnosis of cellular, delayed immune reactions. We studied the prevalence of positive atopy patch tests with food and inhalant allergens and the correlation between the positivity of atopy patch tests and questionnaire derived atopic and nonatopic espiratory symptoms and diseases in an unselected children population. We found a correlation between the positive patch test result with wheat and cough after physical effort, allergic rhino-conjunctivitis, and bronchitis recidivans. The subjects with positive skin reaction to egg suffered from allergic rhino-conjunctivitis and bronchial asthma. Food and inhalant allergens play an important role in the induction and exacerbation of some respiratory allergic diseases. The positive correlation of positive results of skin tests and history of some respiratory diseases and symptoms also on the population level confirm the importance of these tests in the diagnostic work-up of these allergic diseases.

Heart rate variability changes in children after cardiac transplantation.

BACKGROUND: Heart rate variability analysis is a modern, non-invasive, precise and sensitive method of the assessment of cardiovascular systemic functions. OBJECTIVES: Find out changes in parameters of time-domain analysis of heart rate variability (HRV) in children after cardiac transplantation. METHODS: Twenty-two subjects were enrolled and examined in this study--2 subjects after cardiac transplantation (14 and 5 years of age) and 2 control groups of healthy subjects (10 subjects at age from 14 to 16 and 10 subjects at age from 5 to 7 y). Five parameters of HRV from 24-hour Holter monitoring have been analysed (NN intervals, SD, pNN50, rMSSD, triangular index). GiOtto ECG Holter monitor (Medatron) was used in each child. Time domain analysis parameters have been controlled and compared by using percentile range obtained from healthy individuals (Pl0-P90). RESULTS: rMSSD, pNN50, triangular index were below 10th percentile in a 14-year-old girl. rMSSD, SD were below the 10th percentile in a 5-year-old boy. CONCLUSION: Total heart rate variability (described by triangular index and SD parameter) and parameters describing parasympathetic activity (pNN50 and rMSSD) were decreased in both patients after cardiac transplantation. Considering the still unclear mechanism of heart innervation, it is necessary to continue in the research. Authors are planning to check the changes in the future also by means of frequency analysis of heart rate variability. (Tab. 3, Ref. 22.)