Síndrome de QT largo congénito y bradicardia fetal: la importancia del diagnóstico / Congenital long QT syndrome and fetal bradycardia: the importance of the diagnosis

Antecedentes: El síndrome de QT largo es una canalopatía que afecta a la repolarización ventricular y aumenta el riesgo de sufrir arritmias ventriculares graves. Puede ser congénito o adquirido, y es una causa conocida de muerte súbita. Caso clínico: Gestante primigesta, de 28 años, sin antecedentes de interés. En ecografías prenatales se objetivó en el feto bradicardia sinusal mantenida desde la semana 28, sin repercusión hemodinámica, que persistió hasta la finalización de la gestación (semana 37+3). Al nacimiento se realizaron electrocardiogramas seriados que mostraron alteraciones en la repolarización con alargamiento del intervalo QT corregido. Se realizó estudio genético que confirmó síndrome de QT largo tipo 1 y se inició tratamiento oral con beta-bloqueantes, con buena respuesta. Conclusiones: El síndrome de QT largo suele diagnosticarse posnatalmente. Es importante conocer sus características clínicas prenatales para poder establecer un diagnóstico precoz y minimizar así el riesgo de muerte súbita de estos pacientes.

Background: Long QT syndrome is a channelopathy that affects ventricular repolarization and increases the risk of severe ventricular arrhythmias. It can be congenital or acquired, and is a known cause of sudden cardiac death. Case report: A 28-year-old primigravida with no significant medical history. Prenatal ultrasounds revealed sustained fetal sinus bradycardia from week 28, without hemodynamic repercussion, which persisted until the end of gestation (at 37+3 weeks). Serial electrocardiograms were performed after birth, showing repolarization abnormalities with prolonged corrected QT interval. A genetic study confirmed long QT syndrome type 1, and oral treatment with beta-blockers was initiated, showing a positive response. Conclusions: Long QT syndrome is often diagnosed postnatally. It is important to be aware of his prenatal clinical features in order to establish an early diagnosis and minimize the risk of sudden death in these patients.

Rupatadine oral solution in children with persistent allergic rhinitis: A randomized, double-blind, placebo-controlled study.

BACKGROUND: Allergic rhinitis (AR) is one of the most common chronic diseases in childhood. No large, multicentre clinical trials in children with persistent allergic rhinitis (PER) have previously been performed. Rupatadine, a newer second-generation antihistamine, effective and safe in adults, is a promising treatment for children with AR. The aim of the present study was to evaluate the efficacy and safety of a new rupatadine oral solution in children aged 6-11 yr with PER. METHODS: A multicenter, randomized, double-blind, placebo-controlled study was carried out worldwide. Patients between 6 and 11 yr with a diagnosis of PER according to ARIA criteria were randomized to receive either rupatadine oral solution (1 mg/ml) or placebo over 6 wk. The primary efficacy end-point was the change from baseline of the total nasal symptoms score (T4SS) after 4 wk of treatment. RESULTS: A total of 360 patients were randomized to rupatadine (n = 180) or placebo (n = 180) treatment. Rupatadine showed statistically significant differences vs. placebo for the T4SS reduction both at 4 (-2.5 ± 1.9 vs. -3.1 ± 2.1; p = 0.018) and 6 wk (-2.7 ± 1.9 vs. -3.3 ± 2.1; p = 0.048). Rupatadine also showed a statistically better improvement in the children's quality of life compared with placebo. Adverse reactions were rare and non-serious in both treatment groups. No QTc or laboratory test abnormalities were reported. CONCLUSIONS: Rupatadine oral solution (1 mg/ml) was significantly more effective than placebo in reducing nasal symptoms at 4 and 6 wk and was well tolerated overall. This is the first large clinical report on the efficacy of an H1 receptor antagonist in children with PER in both symptoms and quality of life.

Pediatric allergy and immunology in Spain.

The data of the ISAAC project in Spain show a prevalence of childhood asthma ranging from 7.1% to 15.3%, with regional differences; a higher prevalence, 22.6% to 35.8%, is described for rhinitis, and atopic dermatitis is found in 4.1% to 7.6% of children. The prevalence of food allergy is 3%. All children in Spain have the right to be visited in the National Health System. The medical care at the primary level is provided by pediatricians, who have obtained their titles through a 4-yr medical residency training program. The education on pediatric allergy during that period is not compulsory and thus very variable. There are currently 112 certified European pediatric allergists in Spain, who have obtained the accreditation of the European Union of Medical Specialist for proven skills and experience in pediatric allergy. Future specialists in pediatric allergy should obtain their titles through a specific education program to be developed in one of the four accredited training units on pediatric allergy, after obtaining the title on pediatrics. The Spanish Society of Pediatric Allergy and Clinical Immunology (SEICAP) gathers over 350 pediatric allergists and pediatricians working in this field. SEICAP has a growing activity including yearly congresses, continued education courses, elaboration of technical clinical documents and protocols, education of patients, and collaboration with other scientific societies and associations of patients. The official journal of SEICAP is Allergologia et Immunophatologia, published every 2 months since 1972. The web site of SEICAP, http://www.seicap.es, open since 2004, offers information for professionals and extensive information on pediatric allergic and immunologic disorders for the lay public; the web site is receiving 750 daily visits during 2011. The pediatric allergy units are very active in clinical work, procedures as immunotherapy or induction of oral tolerance in food allergy, contribution to scientific literature, and collaboration in international projects.

Calculating the prevalence of atopy in children.

BACKGROUND: Atopy is an important risk factor for asthma, rhinitis, atopic eczema and urticaria. For this reason, several studies have been done to determine the prevalence of atopy in the paediatric population. The important differences among these studies do not allow the extrapolating of results. In this study, we calculate the prevalence of atopy and atopy-related diseases in a paediatric population using a different methodology. METHODS: Retrospective study among children referred for drug allergy in which the latter was discarded. We evaluated the prevalence of atopy (measured by allergen sensitisation), asthma, rhinitis, urticaria, atopic eczema and their characteristics. RESULTS: Three hundred and forty-two patients were studied for adverse drug reaction. This was discarded in 325/342 patients. 20 % of the children in the sample were atopic. Atopy prevalence increased with age. Some atopy related disease was observed in 83/325 (25.5 %) children. Among these children allergen sensitisation increased from 42.3 % in the 0-3 years age group to 93.3 % in the 7-14 age group (p < 0.0001). Prevalence of asthma was 11.5 %, 10.2 % and 7 % in the 0-3, 4-6 and 7-14 age groups, respectively. Prevalence of rhinoconjunctivitis increased through age groups with a prevalence of 20 % among the 7 to 14-year old children. CONCLUSION: The use of this type of methodology seems to be correct to estimate the prevalence of atopy. Prevalence of allergen sensitisation is very high among 7 to 14-year old children with asthma and/or rhinoconjunctivitis.

Sublingual specific immunotherapy: state of the art.

Sublingual immunotherapy (SLIT) was first attempted more than a century ago. After a long parenthesis probably related to the lack of impressive clinical results, the advances on allergen quantification and characterization, together with the improvements in the recombination techniques have renewed the interest in this therapy during the past decade. There are currently enough high quality clinical trials on its efficacy in the management of respiratory allergies (asthma and rhinoconjunctivitis) to conclude that SLIT could be an effective tool for the management of those diseases. This effectiveness has been shown both in children and in adults. However, while there are some clues related to the mechanism of action of SLIT, there is still much to know about it. In addition, more studies comparing the effectiveness of SLIT vs the standard subcutaneous immunotherapy (SCIT) are needed to definitely establish the role of SLIT in the treatment of allergic diseases. SLIT has proven a very safe therapy as compared to SCIT, a fact which adds a very important advantage to the sublingual route.

Cow's milk protein allergy. A multi-centre study: clinical and epidemiological aspects.

BACKGROUND: Due to the age when it becomes apparent and the treatment needed, cow's milk proteins (CMP) allergy requires an accurate diagnosis to avoid labelling infants falsely as allergic and subjecting them to unnecessary diets. The objective of this multi-centre study carried out at the Allergy Units of 14 Children's Hospitals was to discover the epidemiological, clinical and evolutionary characteristics of cow's milk protein allergy (CMPA). METHODS AND RESULTS: Infants suspected of CMPA who attended allergy clinics at the hospitals taking part during the study period were studied and a detailed clinical history was collected on all of them. Prick tests were done with cow's milk and its proteins and specific IgE anti-bodies were determined by means of CAP with the same allergens as the Prick test. The challenge test with cow's milk was carried out unless contraindicated by the diagnostic protocol. Two different challenge regimens were used: one of them carried out in 3 days and the other in one day. 409 infants with suspected CMPA were included and the diagnostic challenge test was performed on 286 patients (70 %) and not carried out on 123, as it was not indicated according to the protocol. IgE-mediated allergy was confirmed in 234 infants (58 %) and in 15 (4 %) non-IgE-mediated hypersensitivity was diagnosed. The two challenge regimens were equally secure. The average age when the reaction to cow's milk formula took place was 3.5 months (10 days-10 months). The symptoms appeared in the first week of introduction in 95 % of cases and appeared in 60 % with the first feeding with the formula. The most frequent clinical signs were cutaneous in 94 % of cases and the majority of cases appeared within 30 minutes of the feed. 99 % had been breast fed and 44 % had received some cow's milk supplement during the lactation period. Sensitization to egg not given in the feed was noted in 30 % and to beef in 29 %, being well tolerated in all of these. CONCLUSIONS: Carrying out an appropriate diagnostic protocol in infants attending for suspected CMPA allows allergy to be ruled out in a high percentage of cases.

The use of spirometers and peak flow meters in the diagnosis and management of asthma among Spanish pediatricians. Results from the TRAP study.

The objective of this study was to determine the level of adherence of pediatricians in Spain to the Spanish National Guidelines for Asthma Treatment with regard to the use of a peak flow meter (PEFR) or a spirometer in the diagnosis and management of asthma in childhood and to analyze sources of variations in these practices. A prospective survey (consisting of demographic and asthma knowledge sections) was conducted over a 2-wk time interval of 3000 pediatricians throughout the country. At least one part of the questionnaire was completed and returned by 2773 individuals (92.4%), with 2347 (78.2%) answering both sections; results are for this population. Around 62% of the pediatricians reported having a peak flow meter or a spirometer in their office; however, only 33% and 48% of them used the devices for the diagnosis and treatment of asthma, respectively. There was a significant association between being older (36-55 yr old) and using PEFR or spirometry for the diagnosis (OR: 1.35, 95% CI 1.11-1.66) and the management (OR 1.47, 95% CI 1.22-1.77) of asthma. Males used a peak flow meter or a spirometer more often than females for the diagnosis (37.8% vs. 30.9%, p = 0.001) and management of asthma (52.0% vs. 45.6%, p = 0.008). Pediatricians with formal pediatric residence training used these devices more for the diagnosis (OR: 1.39, 95% CI 1.09-1.75) and management (OR: 1.58, 95% CI 1.27-1.96) than those without. Working in a hospital was also related with more peak flow meter or spirometer use than working in health centers (OR: 2.08, 95% CI 1.71-2.54 for diagnosis; OR: 1.83, 95% CI 1.50-2.22 for management). About one-third of the Spanish pediatricians surveyed use spirometers and/or peak flow meters for diagnosing asthma and about half use one of these devices occasionally for managing the disease. Independent factors favoring their use are: age 36-55 yr, male gender, working in a hospital setting, and having been trained in a formal pediatric residence program.