Sudden Unexplained Death in Childhood

Sudden Unexplained Death in Childhood

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Sudden Unexplained Death in Childhood is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • SUDC

Disorder Subdivisions

  • None

General Discussion

Sudden unexplained death in childhood (SUDC) is defined as the sudden death of a child greater than one year of age that remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene, and review of the clinical history.



SUDC deaths have occurred at the following sites:



Death at home, history provided: 79%

Crib or bassinet: 54%

Adult bed: 36%



The placed and found positions were as follows:



Placed supine, side, prone*: 10%, 2%, 3%

Found prone: 89%

Found face position: down, side: 10%, 8%

Co-sleeping, sweating when found: 3%, 1%



*applies only to youngest children

Symptoms

Nearly all of the children with SUDC were thought to be sleeping before becoming unresponsive. The older children who died of SUDC were more likely to have clinically recognizable but nonspecific symptoms before their deaths.



The seizure history among the cases and their families is noteworthy:



Child seizure history: 31%

Febrile seizures: 80%

Family seizures history: 37%

Febrile seizures: 57%

Child & family seizure history: 26%

Causes

The causes of SUDC are unknown. However, several studies have now established that SUDC is characterized by a frequent history of seizures that are usually associated with a fever in the affected child and their family members as well as occasional histories of irritability, excessive crying, vomiting, and histories of recent minor head trauma. Detailed neuropathologic studies have identified a subset of children with abnormal development of the hippocampus. It has been proposed that they represent a group of SUDC cases whose sudden death was caused by an unwitnessed seizure arising during sleep in the anomalous hippocampus and producing cardiopulmonary arrest. Fever, infection, and/or minor head trauma may be the precipitating factors that cause the seizure and it appears that a history of febrile seizures and/or a family history of febrile seizures are important risk factors.



A recent study tested the hypothesis that a SUDC subset is characterized by hippocampal maldevelopment and an individual and/or family history of simple familial seizures. The study concluded that a potential new entity may account for the majority of SUDC in toddlers, defined by sleep-related death in the prone position, individual/family history of febrile seizures, and hippocampal anomalies. The mechanism of death appears analogous to sudden death in (temporal lobe) epilepsy (SUDEP), with a putative unwitnessed seizure during sleep leading to airway occlusion and death. This study mandates further research into the potential link between simple febrile seizures and death.

Affected Populations

Children who die of SUDC are generally toddlers between 1 and 4 years of age, but older children have been uncommonly affected. Nearly 80% have been less than 3 years of age. Approximately two thirds of the cases are males. A predilection for a particular ethnic group has not been established. Nearly 90% are born at term and nearly half are first born. Nearly six percent of the cases are part of a multiple birth gestation.

Standard Therapies

SUDC is a diagnosis of exclusion which means that it is only determined as the cause of death after thorough investigation of the clinical history, scene of death and autopsy reveal no other cause. Because the diagnosis of SUDC is made after death, there is no treatment.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

JOURNAL ARTICLES

Kinney H, Chadwick A, Crandall LA, et al. Sudden Death, Febrile Seizures, and Hippocampal Maldevelopment in Toddlers: A New Entity. Pediatr Dev Pathol. 2009:1.



Kinney HC, Armstrong DL, Chadwick AE, et al. Sudden death in toddlers associated with developmental abnormalities of the hippocampus: a report of five cases. Pediatr Dev Pathol. 2007;10:208-223.



Krous HF, Chadwick AE, Crandall L, Nadeau-Manning JM. Sudden unexpected death in childhood: a report of 50 cases. Pediatr Dev Pathol. 2005;8:307-319.

Resources

First Candle-SIDS Alliance

1314 Bedford Avenue

Suite 210

Baltimore, MD 21208

Tel: (800)221-7437

Email: info@firstcandle.org

Internet: http://www.firstcandle.org



National Sudden and Unexpected Infant/Child Death Resource Center

2115 Wisconsin Avenue, NW

Suite 601

Washington, DC 20007

Tel: (202)687-7466

Fax: (202)784-9777

Tel: (866)866-7437

Email: info@sidscenter.org

Internet: http://www.sidscenter.org



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



SIDS Educational Services, Inc.

PO Box 2426

Hyattsville, MD 20784-0426

Tel: (301)322-2620

Fax: (301)322-9822

Email: sidses@aol.com

Internet: http://www.sidssurvivalguide.org



NIH/National Institute of Child Health and Human Development

31 Center Dr

Building 31, Room 2A32

MSC2425

Bethesda, MD 20892

Fax: (866)760-5947

Tel: (800)370-2943

TDD: (888)320-6942

Email: NICHDInformationResourceCenter@mail.nih.gov

Internet: http://www.nichd.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



CJ Foundation for SIDS, Inc.

HUMC: WFAN Pediatric Center

30 Prospect Avenue

Hackensack, NJ 07601

Tel: (551)996-5301

Fax: (551)996-5326

Tel: (888)825-7437

Email: info@cjsids.org

Internet: http://www.cjsids.org



Sudden Unexplained Death In Childhood Program

c/o CJ Foundation for SIDS

The Don Imus WFAN Pediatric Center

30 Prospect Ave.

Hackensack, NJ 07601

Tel: (201)996-5080

Fax: (201)996-5326

Tel: (800)620-7832

Email: info@sudc.org

Internet: http://www.sudc.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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