Article

Sudden unexpected death in childhood: a report of 50 cases.

Department of Pathology, Children's Hospital and Health Center, San Diego, CA 92123, USA.
Pediatric and Developmental Pathology (Impact Factor: 0.86). 01/2005; 8(3):307-19. DOI: 10.1007/s10024-005-1155-8
Source: PubMed

ABSTRACT Sudden unexplained death in childhood (SUDC) is rare, with a reported incidence in the United States of 1.5 deaths per 100,000 live births compared with 56 deaths per 100,000 live births for sudden infant death syndrome in 2001. The objectives of this study include a proposal for a general definition for SUDC and presentation of 36 cases of SUDC and 14 cases of sudden unexpected death in childhood. Cases were accrued through referrals or unsolicited via our Web page (http://www.sudc.org ). Our analyses tentatively suggest a SUDC profile characterized by cases being 1 to 3 years in age, predominantly male, and frequently having a personal and family history of seizures that are often associated with a fever. A history of recent minor head trauma is not uncommon. They are usually born at term as singletons and occasionally have a family history of sudden infant death syndrome or SUDC. Most are found prone, often with their face straight down into the sleep surface. Minor findings are commonly seen at postmortem examination but do not explain their deaths. Comprehensive review of the medical history and circumstances of death and performance of a complete postmortem examination including ancillary studies and extensive histologic sampling of the brain are critical in determining the cause of death in these cases of sudden unexpected childhood death. Legislation enabling research and formation of a multicenter research team is recommended to unravel the mystery of SUDC.

0 Followers
 · 
109 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Long considered a rare and unique disorder of respiratory control, congenital central hypoventilation syndrome has recently been further distinguished as a disorder of autonomic regulation. Similarly, more recent evidence suggests that sudden infant death syndrome is also a disorder of autonomic regulation. Congenital central hypoventilation syndrome typically presents in the newborn period with alveolar hypoventilation, symptoms of autonomic dysregulation and, in a subset of cases, Hirschsprung disease or tumors of neural crest origin or both. Genetic investigation identified PHOX2B, a crucial gene during early autonomic development, as disease defining for congenital central hypoventilation syndrome. Although sudden infant death syndrome is most likely defined by complex multifactorial genetic and environmental interactions, it is also thought to result from central deficits in the control of breathing and autonomic regulation. The purpose of this article is to review the current understanding of these autonomic disorders and discuss the influence of this information on clinical practice and future research directions.
    Seminars in pediatric neurology 03/2013; 20(1):44-55. DOI:10.1016/j.spen.2013.01.005 · 1.88 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Abstract Objective: To study histological variations and abnormalities in Unclassified Sudden Infant Death (USID) including Sudden Infant Death Syndrome (SIDS) in The Netherland. Design: two hundred Dutch USID cases between 1984 and 2005 were identified. The histology slides and autopsy reports of 187 cases were available for systematic review, including brain autopsy in 135 cases. Results: An explanation for the cause of death of 19 patients (10,2%) was found. Twelve had bronchopneumonia, three showed extensive aspiration, two had signs of a metabolic disorder, one had sepsis, and one had meningitis. Frequent non-specific findings were; congestion (66%), oedema (47%), small haemorrhages (18%) and lymphoid aggregates (51%) in the lungs; congestion of the liver (23%); asphyctic bleeding in the kidney (44%), adrenal gland (23%), and thymus (17%). Statistical associations were found for infection with starry sky macrophages in the thymus (p=0,004), with calcification (p=0,023), or with debris in the Hassal's corpuscles (p=0,034). Conclusion: in this study, in 10,2% of cases the histological findings were incompatible with SIDS or USID. Furthermore, several frequent non-specific histological findings in the thymus that point towards an infection were found.
    Pediatric and Developmental Pathology 01/2013; DOI:10.2350/12-10-1262-OA.1 · 0.86 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: In the past century, child mortality has fallen to very low rates in all developed countries. However, rates between and within countries vary widely, and factors can be identified that could be modified to reduce the risk of future deaths. An understanding of the nature and patterns of child death and of the factors contributing to child deaths is essential to drive preventive initiatives. We discuss the epidemiology of child deaths in England and Wales. We use available data, particularly that of death registration and other available datasets, and published literature to emphasise issues relevant to reduction of child deaths in developed countries. We examine the different patterns of mortality at different ages in five broad categories of death: perinatal causes, congenital abnormalities, acquired natural causes, external causes, and unexplained deaths. For each category, we explore what is known about the main causes of death and some of the contributory factors. We then explain how this knowledge might be used to help to drive prevention initiatives.
    The Lancet 09/2014; 384(9946):904–914. DOI:10.1016/S0140-6736(13)61090-9 · 39.21 Impact Factor