[Non-compaction and restrictive cardiomyopathy in pediatrics: Two types of myocardial diseases that are important to recognize]. / Miocardiopatía no compactada y restrictiva en pediatría: Dos tipos de enfermedades del miocardio que son importantes saber reconocer.

Left ventricular non-compaction (LVNC) and restrictive cardiomyopathies (RCM) are rare diseases with high morbidity and mortality in the pediatric age group, particularly the restrictive. They can be diagnosed at any age even in fetal life, in isolation or association with other cardiomyopathies or congenital heart disease. The causes may be genetic, neuromuscular, metabolic, storage, or idiopathic disorders. The main morphological characteristic of LVNC is the presence of a non-compact myocar dium with numerous prominent trabeculations and deep recesses, which may results in myocardial dysfunction, malignant arrhythmias and thromboembolism. On the other hand, in RCM there is an abnormal myocardial stiffness, which generates a restrictive ventricular filling and atrial dilatation secondary. Clinically it manifested by severe diastolic dysfunction, pulmonary hypertension, arrhyth mias and sudden death. For both cardiomyopathies, the Doppler color echocardiography, electro cardiography and Holter monitoring of arrhythmias are the gold standard for diagnosis and follow up. Cardiac resonance adds information on functional assessment and quantification of myocardial fibrosis. The therapy is oriented to improve symptoms and quality of life. Patients with severe forms of LVNC and RCM may require extracorporeal ventricular support and cardiac transplantation, even in early stages of the disease. The pediatrician plays an important role in the early recognition of these pathologies for timing to referral as well as in the follow-up and screening for complications. The objective of this review is to update the clinical, genetic, diagnostic, therapeutic issues and prognostic of the LVNC and RCM.

[Adaptation and validation of the Spanish version of Child Perception Questionnaire CPQ-Spn11-14 in a Chilean community population]. / Adaptación y validación al español del cuestionario de percepción infantil CPQ-Esp11-14 en población comunitaria chilena.

BACKGROUND: In order to validly measure the oral health related quality of life in school age children it is necessary to adapt and validate the CPQ 11-14 for Spanish language. To confirm the four domains of CPQ-Esp 11-14 for the full and abbreviated version of 16 and 8 items. METHODS: The instrument was translated into Spanish and culturally adapted. It was administered to 288 12 year-old children attending public schools. Dental caries experience was measure with the DMFT index. The conceptual structure of the scales was assessed by the AFC. It was also evaluated: internal consistency with Cronbach 's alpha, test- retest temporal stability with intraclass correlation coeficient, and concurrent validity with correlation of score CPQ-Esp 11-14 with caries experience. RESULTS: The five measures used to confirm the structure of the factors on the version of 37 items showed values outside the range of the model fit. Version 16 and 8 items obtained indicators within values indicating the model fit. The internal consistency of full scale and versions 16 and 8 items were measured with Cronbach Alpha wich was higher than 0.6. All versions had intraclass correlation coefficient above 0.81, except for functional limitations of the subscale version a of 16 items. The Rho Spearman correlation was significant between CAOD and the score the questionnaire, except for oral symptoms and full version b version of 16 items. CONCLUSIONS: The hypothetical factor structure was confirmed by the CFA for 16 and 8 items versions. The information contained in abbreviated items allows measuring oral health related quality of life in Chilean children.