Apparent Life-threatening Events Research Articles

Consenso para o manejo e seguimento de pacientes com episódios de possível ameaça a vida (ALTE) e abordagem do diagnóstico diferencial de ALTE com primeira crise convulsiva

OBJETIVO: Este estudo teve como objetivo a elaboração de guia para manejo e seguimento de crianças com episódios de possível ameaça a vida (ALTE) com enfoque especial ao diagnóstico diferencial deste evento com primeiro episódio de crise convulsiva. MÉTODOS: Através de revisão da literatura foi elaborado um consenso, entre os membros do comitê de Síndrome da Morte Súbita do Lactente (SMSL) da Associação Latinoamericana de Pediatria (ALAPE), para orientação quanto ao manejo e investigação etiológica de pacientes com ALTE. RESULTADOS: A proposta de sistematização da investigação destes pacientes inicia definindo a gravidade do evento e estabelecendo a necessidade de internação ou seguimento ambulatorial. A pesquisa da etiologia deve ser realizada gradualmente sendo dividida em exames iniciais e exames específicos, que são aprofundados de acordo com as características clínicas do caso em questão. O manejo após alta hospitalar e a indicação de monitorização domiciliar devem ser individualizados e avaliados caso a caso. O ALTE pode ser a primeira manifestação de uma crise epiléptica ,entretanto, este diagnóstico algumas vezes é tardio, quando não é disponível EEG ictal. O EEG interictal, nestes casos, geralmente é normal e o refluxo gastroesofágico, distúrbio muito prevalente na infância, pode confundir o diagnóstico da manifestação epiléptica. CONCLUSÃO: O ALTE não deve ser considerado um diagnóstico etiológico, mas conjunto de sinais percebidos pelo observador que deve ser amplamente investigado. Apesar de pouco freqüente, a apnéia pode ser a única manifestação ictal de uma crise parcial. Esta possibilidade deve ser lembrada e excluída no diagnóstico diferencial da etiologia de ALTE. As orientações sugeridas neste artigo assim como o fluxograma de investigação apresentado podem auxiliar no manejo e seguimento dos pacientes com ALTE assim como resultar em redução do tempo e custo de internação destes pacientes.

The use of fundoplication for prevention of apparent life-threatening events

Objective Gastroesophageal reflux disease (GERD) is cited by many to be a common cause of apparent life-threatening events (ALTEs). However, there are few reports in the literature regarding the surgical treatment of GERD to prevent a recurrent ALTE. Methods A retrospective review of infants undergoing fundoplication between 2000 and 2005 for the prevention of another ALTE was undertaken. Preoperative, operative, and postoperative data as well as follow-up information were collected. Results During the study period, 81 patients underwent fundoplication after presenting with an ALTE. All but 3 patients (96.3%) had been treated with antireflux medication. Moreover, 71 infants (87.7%) were taking antireflux medication at the time of their ALTE. A significant number of infants (77.8%) were hospitalized with a second ALTE before referral for fundoplication. After fundoplication, only 3 patients (3.7%) experienced a recurrent ALTE during the follow-up period; 2 required a second fundoplication and 1 underwent pyloromyotomy. None of these 3 patients have experienced a recurrent ALTE after the second operation. The median follow-up has been 1738 days. Conclusion Our data suggest that among patients who had an ALTE and are found to have GERD, fundoplication appears to be an effective method for preventing recurrent ALTE.

An update on the approach to apparent life-threatening events

Apparent life-threatening events are an ongoing diagnostic dilemma for clinicians. Since most apparent life-threatening event episodes occur in infants under 6 months of age, they can generate considerable anxiety in parents and providers. This review will discuss issues to consider in the evaluation of infants after an apparent life-threatening event. To ensure proper management, a systematic approach should be taken to attempt to determine the cause of the event. More recent literature suggests that infants with apparent life-threatening events frequently present without signs or symptoms of illness. Obtaining a careful history and physical examination is essential in determining the cause of the event. In this article, we will review the most current literature and discuss the American Academy of Pediatrics new recommendations on sudden infant death syndrome prevention. After a careful review of the literature, prone sleeping is one of the biggest risk factors for sudden infant death syndrome. The association between apparent life-threatening events and sudden infant death syndrome remains to be explored further, but current evidence suggests minimal risk after an apparent life-threatening event episode. This article will help clinicians prepare for this difficult challenge by providing up-to-date information and identifying problems to be addressed in future research.

Apparent Life-threatening Events (ALTEs) and the Role of Home Monitors

Apparent Life-threatening Events (ALTEs) and the Role of Home Monitors

Sudden Infant Death Syndrome

1. Rachel Y. Moon, MD* 2. Linda Y. Fu, MD, MSc† 1. *Director, Academic Development, Goldberg Center for Community Pediatric Health, Children's National Medical Center, Washington, DC 2. †Assistant Professor of Pediatrics, Goldberg Center for Community Pediatric Health, Children's National Medical Center, Washington, DC After completing this article, readers should be able to: 1. Describe possible etiologic mechanisms for sudden infant death syndrome (SIDS). 2. Identify the risk factors for SIDS. 3. Understand the relationship between apnea and SIDS. 4. Discuss the American Academy of Pediatrics SIDS Task Force recommendations and underlying rationale. Sudden infant death syndrome (SIDS) is defined as the sudden unexplained death of an infant younger than 1 year of age. It usually occurs in a previously healthy infant, and the cause of death remains unexplained despite a thorough case investigation, including a complete autopsy, death scene investigation, and review of the clinical history. Every year in the United States, approximately 2,500 infants die from SIDS. Risk factors have been identified through epidemiologic studies. SIDS is more likely to occur in male infants (3:2 ratio). Other risk factors include prone and side sleeping positions, maternal smoking during pregnancy, environmental tobacco smoke, overheating, soft bedding, inadequate prenatal care, young maternal age, prematurity or low birthweight, and African-American or American Indian/Alaskan Native heritage. For many years, apnea was believed to be the predecessor of SIDS, and home apnea monitors have been used in an attempt to prevent SIDS. However, studies such as the Collaborative Home Infant Monitoring Evaluation (CHIME) have demonstrated that apnea neither precedes nor predicts SIDS. Although apnea monitors may be useful in selected patients who have experienced apparent life-threatening events (ALTEs), there is no evidence that they are useful in reducing the risk of SIDS, and the American Academy of Pediatrics (AAP) recommends against using monitors as a SIDS prevention technique. (A more detailed discussion of ALTEs appears in this issue of Pediatrics in Review on page 203.) In 1992, the AAP first published its recommendation that infants should sleep in a nonprone position to reduce the risk of SIDS, and a national public …

Do All Infants With Apparent Life-Threatening Events Need to Be Admitted?

The goal was to identify criteria that would allow low-risk infants presenting with an apparent life-threatening event to be discharged safely from the emergency department. We completed data forms prospectively on all previously healthy patients <12 months of age presenting to the emergency department of an urban tertiary care children's hospital with an apparent life-threatening event over a 3-year period. These patients were then observed for subsequent events, significant interventions, or final diagnoses that would have mandated their admission (eg, sepsis). In our population of 59 infants, all 8 children who met the aforementioned outcome measures, thus requiring admission, either had experienced multiple apparent life-threatening events before presentation or were in their first month of life. In our study group, the high-risk criteria of age of <1 month [corrected] and multiple apparent life-threatening events yielded a negative predictive value of 100% to identify the need for hospital admission. Our study suggests that >30-day-old infants who have experienced a single apparent life-threatening event may be discharged safely from the hospital, which would decrease admissions by 38%.

Severe Human Metapneumovirus Infection in Hospitalized Children

Human metapneumovirus is a recently discovered pathogen that causes upper and lower respiratory tract disease in children. This study describes the course of illness in hospitalized children with this infection. During a 6-month period, 11 children were diagnosed with human metapneumovirus infection by reverse transcription-polymerase chain reaction. Oxygen supplementation was required for 82% of patients. Severe disease developed in 45%, and mechanical ventilation was required. An apparent life-threatening event was the indication for hospitalization of 27% of patients infected with human metapneumovirus. Children with underlying asthma or neuromuscular disease had a prolonged hospitalization.

Why do ALTE infants not die in SIDS?

To compare known risk factors for sudden infant death syndrome (SIDS) amongst infants with apparent life threatening events (ALTE) with their matched controls, and ALTE infants who subsequently died of SIDS with infants surviving an ALTE. Questionnaires with replies were obtained from 58 ALTE infants and 56 sex and age matched ALTE control infants. 244 SIDS cases and 868 SIDS controls were used as comparison. The incidence of ALTE was found to be 1.9% among SIDS controls, but 7.4% among infants who later on died of SIDS. The parents sought medical advice in 0.9% vs 3.7%. ALTE infants did not differ from their matched controls. In the ALTE group 13.3% of the survivors had the combination of prone sleeping and maternal smoking compared with 33.3% of those who became SIDS victims. Our results show some major differences between the ALTE infants and SIDS victims not supporting that these conditions belong to the same entity. However, we cannot exclude the possibility that there is a subpopulation of ALTE infants who did not die in SIDS due to that they were sleeping on the back and not exposed to nicotine.

The Role of β-Amyloid Precursor Protein (β-APP) Staining in the Neuropathologic Evaluation of Sudden Infant Death and in the Initiation of Clinical Investigations of Subsequent Siblings

This report highlights the importance of undertaking immunohistochemical staining of the brains of infants who die unexpectedly, as it may not only assist with the evaluation of the cause of death in an individual infant but may also help with the clinical management of subsequent siblings. A 5-month-old male infant who died suddenly was found to have diffuse beta-amyloid precursor protein (beta-APP) staining in the brain, with no unusual features in his history, death scene examination, routine autopsy dissection, and ancillary tests to suggest any definite cause of death. Due to the beta-APP staining, the possibility of previous episodes of occult trauma, apparent life threatening events (ALTEs), and accidental or inflicted suffocation was raised in the autopsy report. As detailed analyses and investigations provided no supportive evidence for trauma or inflicted injury, hypoxia was clinically considered the most likely cause. Because of these concerns, sleeping oxygen saturation levels were monitored following the birth of a subsequent sibling who had normal APGAR scores and no evidence of any health problems. Oxygen desaturation to 70% occurred in association with a color change while on the postnatal ward, and a subsequent polysomnogram showed multiple episodic significant desaturations to around 80% in association with central apnea. Other testing was unremarkable. These cases demonstrate that beta-APP staining of the brain may not only provide clues as to possible mechanisms of death in pediatric forensic cases but may indicate a need for careful clinical evaluation of subsequent siblings for possible central apnea requiring oxygen therapy.

Child abuse by suffocation: A cause of apparent life-threatening events

Despite decades of attention, child maltreatment is a pervasive problem that remains under-recognized (1,2), leaving younger children and infants at great risk of lethal child maltreatment (3). Recurrent intentional suffocation of a child by a caregiver – termed suffocatory abuse – is a particularly deadly form of maltreatment. The present article summarizes the importance of considering suffocatory abuse as a differential diagnostic possibility, especially in infants with apparent life-threatening events (ALTEs) or apparent sudden infant death syndrome (SIDS).

Smaller mandibular size in infants with a history of an apparent life-threatening event

To examine small mandibular size and preference for a hand-to-chin posture as salient characteristics in infants with a history of an apparent life-threatening event (ALTE). This was a prospective case-control study of term infants, from birth to 6 months of age, admitted post-ALTE and matched 1:2 with healthy control infants (age within 2 weeks and weight within 0.5 kg). Infants with confirmed gastroesophageal reflux and congenital anomalies, including severe micrognathia, were excluded. Infants with a history of an ALTE (n = 25) were matched to 47 healthy controls. Infants with a history of an ALTE had mandibular indices (larger index indicates a smaller mandible) that were 3.8 mm greater on the left side (95% CI: 2.0-5.6, P < .001) and 4.2 mm greater on the right side (95% CI: 2.7-5.6, P < .001) adjusting for length and non-white race. Controlling for matching and length, a 1-mm increase in the average mandibular index increased the odds of an ALTE by 62% (OR = 1.62, 95% CI: 1.22-2.44, P < .001). Smaller mandibular size was associated with ALTE, suggesting airway obstruction as a potential cause of ALTE. The association of this characteristic with ALTE also offers the potential for prospective quantification of ALTE risk.

Seizure-like States

Seizure-like States

Infantile Restrictive Cardiomyopathy Resulting From a Mutation in the Cardiac Troponin T Gene

Here we report the first infantile case of restrictive cardiomyopathy caused by a de novo mutation of the cardiac troponin T gene. The patient presented with an apparent life-threatening event. She developed malignant arrhythmias and hemodynamic instability, requiring initial rescue support with extracorporeal membrane oxygenation, and subsequently underwent insertion of a biventricular assist device (VAD). She successfully received an orthotopic heart transplant 172 days after VAD implantation.

Apparent Life-threatening Event or Child Abuse?

Children with apparent life-threatening events (ALTEs) often pose a diagnostic and management dilemma in a pediatric emergency department. Although the term ALTE is nonspecific and symptoms are vague, it may have alarming connotations and may signal serious life threatening conditions. It may be a subtle presentation of child abuse. We report an infant who was presented with an ALTE and subsequently determined to have subdural hematoma caused by nonaccidental injury.

Pathogenesis of apparent life‐threatening events in infants with esophageal atresia

Many infants with a repaired esophageal atresia (EA) undergo fundoplication, aortopexy, or glossopexy because the mechanisms most responsible for airway obstruction and/or apparent life-threatening event (AO/ALTE) syndrome are considered to be gastroesophageal reflux (GER), tracheal compression (TC), or obstructive apnea, respectively. In the present study, we investigated whether these mechanisms are independent or interrelated. We developed a database of 120 consecutive patients with EA treated by the senior author between 1967-2002. We studied the clinical manifestations of patients with a cervical esophagostomy and/or blind lower esophageal stump, which ruled out TC and/or proximal esophageal GER as a mechanism for AO/ALTE. Of 25 neonates who underwent section/ligation of lower tracheo-esophageal fistula and/or feeding gastrostomy, 10 critically ill neonates died. Of 15 survivors, 9 infants had a feeding gastrostomy without an esophagostomy. Of these, 6 infants presented one or more episodes of AO, and 8 presented ALTE with or without AO. Subsequently, 5 of the 9 infants underwent an esophagostomy. Eventually, 11 infants had a feeding gastrostomy with an esophagostomy. Of the latter, 5 infants presented one or more episodes of AO, and 6 presented ALTE without AO. In conclusion, oral feeding, proximal esophageal GER, and TC are not essential for AO/ALTE syndrome to occur. They are probably factors which offer evidence of an underlying problem with control of upper airway patency.

Pulmonary Intra-Alveolar Siderophages in SIDS and Suffocation: A San Diego SIDS/SUDC Research Project Report

Pulmonary intra-alveolar siderophages (PS) have been suggested as a marker of previous attempts at imposed suffocation in infants dying suddenly and unexpectedly. The aims of this study were to (1) compare PS counts between cases of sudden infant death syndrome (SIDS) and a control group comprised of infants whose deaths were attributed to accidental or inflicted suffocation, (2) compare clinical variables in SIDS and control suffocation cases, and (3) review individual cases irrespective of the cause and manner of death with an average PS count greater than 200 per 20 high-power fields (hpf) per lung lobe. Retrospective assessment of siderophages in available iron-stained lung sections was undertaken in 91 SIDS cases and 29 cases of death due to suffocation (27 accidents and 2 homicides) from the San Diego SIDS and Sudden Unexplained Death in Childhood (SUDC) Research Project (SDSSRP) database. Neither the means of the log-transformed PS counts nor the medians of the raw PS counts were significantly different between the SIDS and control suffocation groups. The distributions of the PS data were different, however-the range was wider in the SIDS group. Only 6% of each group had a history of prior apparent life-threatening events. Approximately three fourths of the families from both groups had no prior referral to Child Protective Services. The number of PS varies widely in cases of sudden infant death caused by SIDS and accidental or inflicted suffocation and cannot be used as an independent variable to ascertain past attempts at suffocation.

Relationship between gastro‐oesophageal reflux and gastric activity in newborns assessed by combined intraluminal impedance, pH metry and epigastric impedance

The pathogenesis of gastro-oesophageal reflux disease (GORD) is complex and multifactorial. A motility disorder resulting from immaturity of the gastro-oesophageal tract may be involved. We have combined multichannel intraluminal impedance (MII) and pH monitoring with epigastric impedance (EGI) to evaluate the activity of this tract in neonates with suspected GORD. Multichannel intraluminal impedance, pH and EGI were followed for 3 h in 30 newborns displaying apparent life-threatening events and signs of GORD. Simultaneous application of MII and pH monitoring identifies reflux episodes and illustrates their duration, height and pH. Episodes detected by MII were placed on the EGI curve and the contemporaneous gastric filling state and emptying velocity were calculated. During the total measuring time, 248 reflux episodes were revealed. An inverse correlation was evident for reflux frequency and gastric emptying velocity (r2 = 0.94; P < 0.001), and between acid refluxes and the gastric filling state (r2 = 0.95; P < 0.001), whereas a positive correlation was found between the reflux level and the gastric filling state (r2 = 0.52; P < 0.05). Simultaneous MII, pH and EGI monitoring provided new information on the relationship between refluxes and gastric activity. Data suggest that gastric emptying patterns influence the frequency, level and pH of reflux episodes.

Idiopathic apparent life‐threatening event in Northern Israel

To note whether the incidence of idiopathic apparent life-threatening event (IALTE) has decreased since 1993 in parallel to the decline in the incidence of sudden unexpected death in infancy (SUDI) 2. To compare their epidemiological profile to infants with acute unrelated illness. The discharge diagnoses of each infant investigated for apparent life-threatening event or apnea in the five major hospitals in Northern Israel were reviewed over the period 1991-2000. Infants with identified aetiology or apnea only were excluded. Each infant was matched with two other infants admitted for an acute respiratory illness unrelated to apnea. IALTE rates were compared to the national rates of SUDI. Two hundred and forty-three infants were diagnosed with IALTE. No evidence for a decline in incidence was noted over the period of the study, while a consistent decline in the incidence of SUDI was observed. Only few differences in the epidemiological profile were noted between the study and the comparison group, that is, increased rate of prematurity and first-born infants. (i) The two conditions probably do not share a common aetiology in the majority of cases; and (ii) the epidemiological profile of IALTE is predominantly similar to that of infants hospitalized for respiratory illness.

Home and hospital monitoring for ALTE

Apparent life-threatening events (ALTE) results from various causes. Documented monitoring of cardiorespiratory parameters and Hb-O(2) saturation by pulse oximetry (SpO(2)) has proven useful in the identification of significant events both in hospital and at home. Home monitoring might be especially helpful for infants with recurrent but infrequent clinical events not captured during a hospital admission.

Yield of Diagnostic Testing in Infants Who Have Had an Apparent Life-Threatening Event

To the Editor.—We read the article by Brand et al1 with great interest. In 2002, we also saw the need for an evidence-based diagnostic-testing guideline for evaluating apparent life-threatening events (ALTEs), but we took a different approach. Brand et al looked at the yield of different diagnostic tests to try to establish which are useful. We systematically reviewed the medical literature to determine the diagnoses reported after infants first presented with ALTEs and used these diagnoses to suggest an algorithm of investigation.2Brand et al conclude that, in infants whose history and examination are unhelpful in directing diagnostic investigations, only the following screening tests are useful: screening for gastroesophageal reflux disease, urine analysis and culture, brain imaging, chest radiograph, pneumogram, and white blood count.In our review, we looked at 728 diagnoses among 8 studies. Metabolic disease was diagnosed in 4 studies (n = 11 [1.5% of all diagnoses]), ingestion of drugs or toxins was reported in 3 studies (n = 11 [1.5% of all diagnoses]), and factitious illness was diagnosed in 1 study (n = 2 [0.3% of all diagnoses]). Therefore, a wider investigation plan may be particularly useful when the history and physical examination do not suggest a cause. Brand et al note this limitation of the sample size in their article.In our review, we also encountered the difficulties noted by the authors.Many studies looking at ALTEs are observational and, as such, lack uniform testing and diagnostic criteria. (In this study, gastroesophageal reflux was diagnosed by different methods [eg, upper gastrointestinal series or by pH probe].) It also can be difficult to determine when diagnoses are causal or coexistent, and long-term follow-up for new diagnoses is often limited.We strongly agree that a diagnostic-testing strategy should depend on the outcome of the initial clinical assessment as suggested and are encouraged to see that in 70% of the patients studied, the history and examination were helpful. We estimated that >50% of the time this would be true. Our algorithm suggests a stepwise approach, and investigations are initially directed by the history and clinical findings. The possibility of child abuse is highlighted at the first step. When the initial history and examination are not helpful, we suggest that full blood count, blood culture, calcium, magnesium, glucose, blood gas, C-reactive protein, urea, and electrolytes levels, metabolic screen, toxicology screen, urine culture, chest radiograph, electrocardiograph, electroencephalogram, ultrasound brain, investigations for gastroesophageal reflux, and an analysis of respiratory secretions be obtained. In addition, if the ALTE is severe or recurrent, the infant will often require additional specialized investigations.