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      Fisiopatología, diagnóstico diferencial y tratamiento de la apnea emotiva grave: A propósito de un caso clínico Translated title: Pathophysiology, differential diagnosis and treatment of severe emotional apnea: based on report case

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          Abstract

          Introducción: Las apneas emotivas (AE) son eventos paroxísticos no epilépticos que afectan al 5% de niños sanos. El diagnóstico se basa en una secuencia estereotipada de eventos clínicos iniciado con llanto provocado por un estimulo emocional que desencadena una alteración refleja del sistema nervioso autonómico con cambio de color, pálido o cianótico. En el 15% se asocia a pérdida de conciencia, cambios del tono o movimientos tónico-clónicos secundarios a hipoxia. Objetivo: Presentar un caso clínico de AE grave, revisar el diagnóstico diferencial y tratamientos preventivos. Caso clínico: Lactante de 15 meses con cianóticas a partir de los 8 meses de edad, desencadenados por dolor, disgusto o miedo que aumentaron en frecuencia (3-4 por día) e intensidad con alteración de conciencia e hipotonía. A los 12 meses, se agregó además una crisis tónico clónica de 3 min de duración, por la cual ingresó a Servicio de Urgencia. Se constató un desarrollo psicomotor normal, examen físico, neurológico y parámetros de laboratorio normales (sin anemia), al igual que la electroencefalografía y resonancia magnética cerebral. Se inició terapia preventiva con piracetam con el propósito de disminuir las crisis, lo que se produjo desde el primer mes de tratamiento. Conclusiones: En la mayoría de los casos, la naturaleza benigna e historia natural de las AE permiten que una entrega de información oportuna a los padres sea suficiente. Sin embargo, cuando la frecuencia y severidad de las AE impactan al niño y su familia, se requiere descartar patología cardíaca o epilepsia y buscar opciones de tratamiento preventivo.

          Translated abstract

          Introduction: Emotional apneas (EA) are non-epileptic paroxysmal events affecting 5% of healthy children. The diagnosis is based on a stereotyped sequence of clinical events that start with tears caused by emotional stimulus, resulting in an autonomic nervous system alteration with transient color change, pale or cyanotic. 15% of the cases are associated with loss of consciousness, changes in tone or tonic-clonic movements secondary to hypoxia. Objective: To report a case of severe EA and to review the differential diagnosis and preventive treatments. Case report: A 15-month old infant with cyanotic emotional apnea since 8 months of age, triggered by pain, disgust or fear, increasing in frequency (3-4 per day) and intensity with altered consciousness and hypotonia. At 12 months, the patient also presented generalized tonic-clonic seizures of 3 minutes long, reason why the infant was admitted to the emergency service. Normal psychomotor development as well as normal physical, neurological and laboratory test results (without anemia) were found. Electroencephalography and brain MRI presented no abnormalities. Preventive therapy using Piracetam was performed in order to reduce crisis, which occurred in the first month of treatment. Conclusions: In most cases, a timely information delivery to parents is enough due to the benign nature and natural history of EA. However, when the frequency and severity of EA impact the child and family, to rule out heart disease or epilepsy and to seek preventive treatment options are required.

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          Most cited references31

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          Systematic review: worldwide variation in the frequency of coeliac disease and changes over time.

          Coeliac disease (CD), originally thought to be largely confined to Northern Europe and Australasia and uncommon in North America and the Middle East, is now recognised to be equally common in all these countries. It is still thought to be rare in the Orient and Sub-Saharan Africa. To assess geographical differences and time trends in the frequency of CD. Medline and Embase searches were conducted on 10 November 2012, from 1946 and 1980 respectively, using the key words: coeliac disease or celiac disease + prevalence or incidence or frequency. There were significant intra- and inter-country differences in the prevalence and incidence of CD. Only 24 ethnic Chinese and Japanese patients have been reported in the English literature. Of CD-associated HLA DQ antigens, DQ2 occurs in 5-10% of Chinese and sub-Saharan Africans, compared to 5-20% in Western Europe. DQ8 occurs in 5-10% of English, Tunisians and Iranians, but in <5% of Eastern Europeans, Americans and Asians. The prevalence and incidence of both clinically and serologically diagnosed CD increased in recent years. These geographical and temporal differences seem genuine, although variable indices of suspicion and availability of diagnostic facilities are confounding factors. Coeliac disease is increasing in frequency, with significant geographical differences. Although few cases have been described to date in the Orient and Sub-Saharan Africa, there is a significant prevalence of HLA DQ2 and wheat consumption is of the same order as that in Western Europe. CD may therefore become more common in the future in these countries. © 2013 John Wiley & Sons Ltd.
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            Prospective study of children with cyanotic and pallid breath-holding spells.

            F DiMario (2001)
            In this investigation, we sought to prospectively document the natural history of severe breath-holding spells (BHS) among children with both cyanotic and pallid BHS who were referred for neurologic consultation. Prospective cohort study. A structured interview was undertaken at the time of initial consultation and at subsequent 1-year intervals regarding type of BHS, frequency of spells, associated phenomenon, sequelae, family history, and age at termination of spells. A total of 95 children (48 boys, 47 girls) with BHS were identified and followed over a 9-year interval. There were no significant differences between genders. Median onset age was between 6 and 12 months old with 15% presenting younger than 6 months. A median frequency of spells was weekly with 30% experiencing 1 or more spells per day. The median age at peak frequency was between 12 and 18 months old with a range extending from 6 months to 4 years of age. Of the patients whose BHS had remitted for >12 months' time (n = 67), the last spell occurred at a median age of 37 to 42 months. Of those children whose BHS were still occurring, the oldest age at time of latest spell was at 7 years old. Hypoxic convulsions were associated with BHS in
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              Breathholding spells (cyanotic and pallid infantile syncope).

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                Author and article information

                Journal
                rcp
                Revista chilena de pediatría
                Rev. chil. pediatr.
                Sociedad Chilena de Pediatría (Santiago, , Chile )
                0370-4106
                July 2014
                : 85
                : 4
                : 481-485
                Affiliations
                [02] orgnameUniversidad de Antofagasta orgdiv1Pediatría Chile
                [01] orgnamePontificia Universidad Católica de Chile orgdiv1Facultad de Medicina orgdiv2Unidad Neuropediatría Chile mhernand@ 123456med.puc.cl
                [03] orgnameHospital Clínico Regional de Antofagasta orgdiv1Servicio de Pediatría Chile
                Article
                S0370-41062014000400012 S0370-4106(14)08500400012
                10.4067/S0370-41062014000400012
                f3c4c716-9ef0-4f56-807a-96e3c3b22c55

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 16 April 2014
                : 25 June 2014
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 29, Pages: 5
                Product

                SciELO Chile

                Self URI: Texto completo solamente en formato PDF (ES)
                Categories
                CASOS CLINICOS

                expiratory apnea,Apnea emotiva,sincope autonómico,evento paroxístico cerebral,apnea espiratoria,disfunción autonómica,Emotional apnea,autonomic syncope,paroxysmal event,autonomic dysfunction

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