Changes in NT-proBNP in young children with congenital heart malformations.

OBJECTIVE: We measured serum NT-proBNP levels in 2 groups of young patients (0-36 months) with congestive heart failure (CHF) secondary to congenital heart malformations (CHM). METHODS: The first group included 23 patients prior to surgery for heart malformations. The second group included 12 patients with CHM within 24 hours post-operation. NT-proBNP levels were measured by an enzyme-linked immunosorbent immunoassay (ELISA) using the Chem Well 2910 (Awareness Technology Inc.) automated analyzer. RESULTS: NT-proBNP values were 4 times higher in patients with unoperated CHM compared to healthy controls (P < 0.001), and 12 times higher in patients with CHM 24 hours after surgery compared to controls (P = 0.0001). CONCLUSIONS: These results demonstrated significant changes in NT-proBNP concentrations before and after surgery to correct congenital heart malformations.

IgA anti-tissue transglutaminase antibodies, first line in the diagnosis of celiac disease.

BACKGROUND: According to the 2008 celiac disease working group run by Dr. A. Fassano under the auspices of the Federation of International Societies of Pediatric Gastroenterology, Hepatology and Nutrition, celiac disease is a chronic immune-mediated enteropathy characterized by gluten sensitivity, which can affect any organ or system, having a wide range of clinical manifestations of variable severity. The serological diagnosis of celiac disease is based on high sensitivity and specificity tests. The measurement of IgA anti-tissue transglutaminase antibodies by ELISA is universally accepted in the screening of celiac disease. METHODS: Using the gold standard represented by IgA anti-endomysium antibodies in a group of 890 children investigated during 2008-2009, we aimed to evaluate IgA anti-tissue transglutaminase antibodies (tTG IgA), as well as to establish their prevalence in associated diseases. RESULTS: Following the measurement of tTG IgA in the entire group, we obtained: sensitivity 773%, positive predictive value 55.2%, specificity 93.1%, negative predictive value 973%, p = 0.000, and in tTG IgA associations we obtained the value 0.51 for the ROC curve area. We found associations of tTG IgA with type 1 diabetes mellitus (235% prevalence), protein-calorie malnutrition (0.89% prevalence), and intestinal malabsorption (0.56% prevalence). CONCLUSIONS: Our results have a high specificity and sensitivity in the screening of celiac disease, while requiring a second method of confirmation.

Romanian experience in child celiac disease diagnosis.

Last consensus in celiac disease in 2008 conducted under the aegis of the European Society of Pediatric Gastroenterology, Hepatology and Nutrition jointly with North American Society of Pediatric Gastroenterology, Hepatology and Nutrition reveals the following: "celiac disease is a chronic immune-mediated enteropathy characterized by sensitization to gluten. That can affect any organ or system, with a wide range of clinical manifestations of variable severity". Thus, in recent years, clinical picture of celiac disease has changed the old paradigm--bowel disease with villous atrophy and malnutrition, being replaced with the new paradigm--multi-organ autoimmune disease, affecting many organs and systems throughout but with more less specific symptoms, which undiagnosed leads to delayed diagnosis, at a late-onset disease and long-term major complications as the risk of cancer. According to this consensus "the serological diagnosis of celiac disease is based on high sensitivity and specificity tests", but in line with changing clinical features of celiac disease, its diagnosis has undergone significant changes in recent years. These changes in the diagnosis of celiac disease, we have decided to analyze them.

Controversies in the laboratory diagnosis of celiac disease in children; new haplotypes discovered.

The latest consensus on celiac disease in 2008, under the auspices of the International Societies of Pediatric Gastroenterology, Hepatology and Nutrition, shows that HLA DQ2/DQ8 typing indicates the highest negative predictive value for celiac disease, which would exclude the diagnosis of celiac disease. In Romania, there are no studies on the implication of HLA-DQ2/DQ8 in celiac disease in children. The aim of our study was to analyze the significance of genetic tests, with a focus on negative HLA-DQ2/DQ8 cases, as well as to determine the main haplotypes involved in celiac disease in children. We tested in 37 children with old celiac disease, confirmed based on the presence of intestinal villi changes on duodenal biopsy, the IgA anti-tissue transglutaminase antibodies (TgA-IgA) by ELISA and the IgA anti-endomysium antibodies (EmA-IgA) by indirect immunofluorescence, compared to HLA-DQ2/DQ8 typing by polymerase chain reaction (PCR). In 25 children, the determined HLA haplotypes predominantly belonged to DQ2, and in 3 children we report the presence of a new haplotype, DR3-DQ2/DR4-DQ8, formed by pattern 1, DR3-DQ2-the DQA1*0501 and DQB1*0201 alleles, and pattern 5, DR4-DQ8-the DQA1*0301 and DQB1*0302 alleles. In 9 children, genetic tests were negative for celiac disease. The identification of HLA-DQ2/DQ8 provides additional data in the diagnosis of celiac disease, but a rigid algorithm in the diagnosis of celiac disease has no practical applicability.

Evaluation of NT-proBNP in children with heart failure younger than 3 years old.

INTRODUCTION: Heart failure (HF) is characterized by neuroendocrine activation. The cardiac natriuretic hormones, including atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP), together with their related pro-peptides (proANP and proBNP) represent a group of peptide hormones produced by the heart. A normal NT-proBNP level has a high negative predictive value for heart failure. The use of NT-proBNP testing is helpful in diagnosing acute HF in the emergency care setting, allowing an early and optimal treatment. The purpose of this study is to assess the prognostic value of NT-proBNP in heart failure in children younger than 3 years old and to establish whether it correlates with the NYHA/Ross functional class and left ventricle systolic function. METHODS: We enrolled 24 consecutive children with HF due to congenital heart diseases and dilated cardiomyopathy. The serum levels of NT-proBNP were measured, all patients underwent echocardiography and left ventricle ejection fraction was calculated. RESULTS: The highest median value of NT-proBNP was recorded in patients with cyanotic heart diseases (248.0 fmol/mL), p = 0.610. NT-proBNP had a negative correlation with the ejection fraction of the left ventricle: Spearman's rank correlation coefficient was -0.165. CONCLUSIONS: NT-proBNP levels correlate with the severity of HF in infants and small children younger than 3 years old with heart failure due to congenital heart diseases and dilated cardiomyopathy.

Systolic and Diastolic Function in Congestive Heart Failure Pediatric Patients.

BACKGROUND AND AIMS: Two-dimensional (2D) and Doppler echocardiography are the main methods for the non-invasive evaluation of ventricular function in children. Our study monitored the evaluation of systolic and diastolic function in pediatric patients, using classical echocardiographic parameters and pulsed tissue Doppler parameters, as well as the correlation between these. METHODS: The study included 18 healthy children and 9 children diagnosed with congestive heart failure secondary to congenital heart malformations. The parameters of systolic and diastolic function were measured by 2D echocardiography, 2D guided M mode, color and pulsed Doppler, as well as by pulsed tissue Doppler at the level of the mitral and tricuspid annulus. RESULTS: A relaxation alteration pattern or a pseudonormal pattern of E diastolic velocity compared to the A wave was found (E = A; E > A) in the group of subjects with heart failure. E wave deceleration time had significantly increased values in the case of patients with CHF, being correlated with diastolic dysfunction. Left ventricular flow propagation velocity Vp was decreased in patients with heart failure, the E/Vp ratio being maintained relatively constant in subjects with congestive heart failure and healthy subjects, most probably on account of the concomitant change in the E wave. Associations between the severity of systolic dysfunction and the diastolic dysfunction were found in pediatric patients diagnosed with congestive heart failure (Student test, p < 0.05). CONCLUSIONS: Tissue Doppler measurements proved to be useful for the evaluation of pediatric patients with altered ventricular geometry secondary to congenital heart disease, systolic-diastolic dysfunction and heart failure.

Etiological Peculiarities in Pediatric Heart Failure.

Heart failure in children presents important characteristic features different from adult congestive failure, from a pathophysiological and mostly from an etiopathogenic point of view. Heart failure in children is, in most cases, a consequence of congenital structural cardiac abnormalities that remained unoperated, underwent a palliative operation or presented post-surgery complications, or of cardiomyopathy. Based on the nature of the clinical presentation, new onset heart failure can be differentiated from transient heart failure and chronic heart failure. Chronic heart failure may occur in children with biventricular circulation (systolic or diastolic dysfunction), in cardiac structural abnormalities with a right systemic ventricle and in the so-called univentricular heart. Acute heart failure can appear as acute heart failure at onset or as an aggravation of heart failure on the background of acute decompensated chronic heart failure.

Difficulties in Celiac Disease Diagnosis in Children - A case report.

Diagnosis of celiac disease in a patient with lactose intolerance has special importance having implications for the treatment of both diseases. The authors present the case of a 2 years old girl, first diagnosed with enterocolitis, but her clinical evolution revealed a complex situation: both celiac disease and secondary lactose intolerance. We present the case as a special situation in clinical pediatric practice that must be taken into account more often.

[Clinical pictures of bronchial asthma in children]. / Fenotipuri clinice de astm bronsic la copil.

In the tip-to-date medical literature a topic for discussion is the variety of asthma clinical phenotypes. The knowledge of the various clinical asthma phenotypes is very important as regards the appropriate treatment approach, and the prognostic assessment of the patients with asthma. This allows a better understanding of pathophysiologic mechanisms, and of the phenotype-genotype correlation. A diagrammatic classification of clinical asthma phenotypes includes following criteria: onset age, inflammatory profile, severity pattern. According to the onset age, asthma clinical phenotypes specific for children and clinical phenotypes more frequent in adulthood are known. The main childhood asthma phenotypes are: transient infant wheezing, non atopic wheezing of the toddler and early school years, persistent IgE-mediated wheezing/asthma, late-onset childhood asthma. Other asthma phenotypes like aspirin-induced asthma, asthma related to chronic, persistent respiratory infection, are rare in childhood. It is reported a prevalence of 2-5% for the aspirin-induced asthma among pediatric patients. According to the airway inflammation pattern there are two phenotypes to distinguish: eosinophilic and non-eosinophilic asthma. The majority of the patients have an eosinophilic inflammation, but there is also a meaningful percentage of patients with noneosinophilic asthma which is neutrophil mediated. Clinical phenotypes of severe asthma are rarely described in childhood: near-fatal asthma, asthma with fixed airflow obstruction, and corticosteroid-resistant asthma.

[Correlation between fever and the clinical severity of acute bronchiolitis]. / Corelatia dintre febra si severitatea clinica a bronsiolitei acute.

INTRODUCTION: The relationship between fever and the severity of acute infant bronchiolitis has been little studied, and the results of the few published studies are not concordant. AIM: To assess the significance of fever in children aged under 1 year with acute bronchiolitis. MATERIAL: 83 children aged between 0-1 year; hospitalized with the diagnosis of acute bronchiolitis during the winter of 2004-2005, assigned to two groups, with febrile bronchiolitis and afebrile bronchiolitis, respectively. METHOD: The evaluation of patients using a clinical score including respiratory effort, O2 saturation, respiratory rate, and the statistical processing of results comparatively in the two study groups. RESULTS: The mean duration of hospitalization was 5.8 days for febrile patients compared to 4.4 days for afebrile patients (p = 0.003). Infants with febrile bronchiolitis had a more severe clinical picture, evaluated by the clinical score, compared to afebrile patients (p = 0.03). CONCLUSION: Fever associated with acute bronchiolitis in children aged 0-1 year has the significance of a higher clinical severity and prolonged evolution of the disease.