Sudden unexplained death in childhood | |
---|---|
Specialty | Pediatrics |
Symptoms | Usually none but may be preceded by a fever |
Complications | Death |
Usual onset | Sudden |
Duration | Unknown |
Types | Unknown |
Causes | Not definitive, most likely heart arrhythmias, seizures, or a mix |
Risk factors | Family history of sudden death |
Diagnostic method | Unexplained death of a child of 1–18 years |
Prevention | None |
Treatment | None |
Medication | None |
Prognosis | Death |
Frequency | ~1.2 out of 100,000 |
Deaths | ~1.2 out of 100,000 |
Sudden unexplained death in childhood (SUDC) is the death of a child over the age of 12 months which remains unexplained after a thorough investigation and autopsy. There has not been enough research to identify risk factors, common characteristics, or prevention strategies for SUDC.
SUDC is similar in concept to sudden infant death syndrome (SIDS). Like SIDS, SUDC is a diagnosis of exclusion, the concrete symptom of both being death. However, SIDS is a diagnosis specifically for infants under the age of 12 months while SUDC is a diagnosis for children 12 months and older. The causes of SIDS and SUDC are not definitively known but there are good chances heart arrhythmias and seizures are the main causes. Although it can happen to any child under the age of 18 (after which the deaths are classified as Sudden arrhythmic death syndrome (SADS)), it is most common in those aged between 1–4 years, where according to the SUDC foundation, it is the 5th leading cause of death in this age group.
Researchers are exploring a possible connection between sudden unexplained death in toddlers, febrile seizures, and hippocampal anomalies. The occurrence of fever before the sudden deaths later in sleep, could explain the febrile seizure hypotheses [1] [2]
SUDC is rare, with a reported incidence in the United States of 1.2 deaths per 100,000 children, compared to 54 deaths per 100,000 live births for SIDs. There are approximately 400 deaths per year of SUDC in the U.S, with over 200 of these cases being the children aged 1–4 years. [3] SUDC deaths have occurred at the following sites: [4]
The placed and found positions were as follows:
*applies only to youngest children
At the SIDS Alliance national convention in Atlanta, GA in 1999 Dr. Henry Krous gave a presentation titled "Post-Infancy SIDS: Is it on the rise?" This led to the beginning of the San Diego SUDC Research Project. The first definition of SUDC was published in 2005 in Pediatric and Developmental Pathology . [5] [6] [7]
A convulsion is a medical condition where the body muscles contract and relax rapidly and repeatedly, resulting in uncontrolled shaking. Because epileptic seizures typically include convulsions, the term convulsion is often used as a synonym for seizure. However, not all epileptic seizures result in convulsions, and not all convulsions are caused by epileptic seizures. Non-epileptic convulsions have no relation with epilepsy, and are caused by non-epileptic seizures.
Sudden infant death syndrome (SIDS), sometimes known as cot death, is the sudden unexplained death of a child of less than one year of age. Diagnosis requires that the death remain unexplained even after a thorough autopsy and detailed death scene investigation. SIDS usually occurs during sleep. Typically death occurs between the hours of midnight and 9:00 a.m. There is usually no noise or evidence of struggle. SIDS remains the leading cause of infant mortality in Western countries, constituting half of all post-neonatal deaths.
A febrile seizure, also known as a fever fit or febrile convulsion, is a seizure associated with a high body temperature but without any serious underlying health issue. They most commonly occur in children between the ages of 6 months and 5 years. Most seizures are less than five minutes in duration, and the child is completely back to normal within an hour of the event. There are two types: simple febrile seizures and complex febrile seizures. Simple febrile seizures involve an otherwise healthy child who has at most one tonic-clonic seizure lasting less than 15 minutes in a 24-hour period. Complex febrile seizures have focal symptoms, last longer than 15 minutes, or occur more than once within 24 hours. About 80% are classified as simple febrile seizures.
Roseola, also known as sixth disease, is an infectious disease caused by certain types of human herpes viruses. Most infections occur before the age of three. Symptoms vary from absent to the classic presentation of a fever of rapid onset followed by a rash. The fever generally lasts for three to five days, while the rash is generally pink and lasts for less than three days. Complications may include febrile seizures, with serious complications being rare.
Medium-chain acyl-CoA dehydrogenase deficiency is a disorder of fatty acid oxidation that impairs the body's ability to break down medium-chain fatty acids into acetyl-CoA. The disorder is characterized by hypoglycemia and sudden death without timely intervention, most often brought on by periods of fasting or vomiting.
A pacifier is a rubber, plastic, or silicone nipple substitute given to an infant or toddler to suckle upon between feedings to quiet its distress by satisfying the need to suck when it does not need to eat. Pacifiers normally have three parts: an elongated teat, a handle, and a mouth shield which prevents the child from swallowing or choking on it.
Failure to thrive (FTT), also known as weight faltering or faltering growth, indicates insufficient weight gain or absence of appropriate physical growth in children. FTT is usually defined in terms of weight, and can be evaluated either by a low weight for the child's age, or by a low rate of increase in the weight.
Sidney Farber was an American pediatric pathologist. He is regarded as the father of modern chemotherapy for his work using folic acid antagonists to combat leukemia, which led to the development of other chemotherapeutic agents against other malignancies. Farber was also active in cancer research advocacy and fundraising, most notably through his establishment of the Jimmy Fund, a foundation dedicated to pediatric research in childhood cancers. The Dana–Farber Cancer Institute is named after him.
Lennox–Gastaut syndrome (LGS) is a complex, rare, and severe childhood-onset epilepsy syndrome. It is characterized by multiple and concurrent seizure types including tonic seizure, cognitive dysfunction, and slow spike waves on electroencephalogram (EEG), which are very abnormal. Typically, it presents in children aged 3–5 years and most of the time persists into adulthood with slight changes in the electroclinical phenotype. It has been associated with perinatal injuries, congenital infections, brain malformations, brain tumors, genetic disorders such as tuberous sclerosis and numerous gene mutations. Sometimes LGS is observed after infantile epileptic spasm syndrome. The prognosis for LGS is marked by a 5% mortality in childhood and persistent seizures into adulthood.
In the field of neurology, temporal lobe epilepsy is an enduring brain disorder that causes unprovoked seizures from the temporal lobe. Temporal lobe epilepsy is the most common type of focal onset epilepsy among adults. Seizure symptoms and behavior distinguish seizures arising from the medial temporal lobe from seizures arising from the lateral (neocortical) temporal lobe. Memory and psychiatric comorbidities may occur. Diagnosis relies on electroencephalographic (EEG) and neuroimaging studies. Anticonvulsant medications, epilepsy surgery and dietary treatments may improve seizure control.
Sudden unexpected death in epilepsy (SUDEP) is a fatal complication of epilepsy. It is defined as the sudden and unexpected, non-traumatic and non-drowning death of a person with epilepsy, without a toxicological or anatomical cause of death detected during the post-mortem examination.
Dravet syndrome (DS), previously known as severe myoclonic epilepsy of infancy (SMEI), is an autosomal dominant genetic disorder which causes a catastrophic form of epilepsy, with prolonged seizures that are often triggered by hot temperatures or fever. It is very difficult to treat with anticonvulsant medications. It often begins before one year of age, with six months being the age that seizures, characterized by prolonged convulsions and triggered by fever, usually begin.
Benign Rolandic epilepsy or self-limited epilepsy with centrotemporal spikes is the most common epilepsy syndrome in childhood. Most children will outgrow the syndrome, hence the label benign. The seizures, sometimes referred to as sylvian seizures, start around the central sulcus of the brain.
Epilepsy is a neurological condition of recurrent episodes of unprovoked epileptic seizures. A seizure is an abnormal neuronal brain activity that can cause intellectual, emotional, and social consequences. Epilepsy affects children and adults of all ages and races, and is one of the most common neurological disorders of the nervous system. Epilepsy is more common among children than adults, affecting about 6 out of 1000 US children that are between the age of 0 to 5 years old. The epileptic seizures can be of different types depending on the part of the brain that was affected, seizures are classified in 2 main types partial seizure or generalized seizure.
Febrile infection-related epilepsy syndrome (FIRES), is onset of severe seizures following a febrile illness in someone who was previously healthy. The seizures may initially be focal; however, often become tonic-clonic. Complications often include intellectual disability, behavioral problems, and ongoing seizures.
An epilepsy syndrome is defined as "a characteristic cluster of clinical and Electroencephalography (EEG) features, often supported by specific etiological findings ."
Infant and toddler safety are those actions and modifications put into place to keep babies and toddlers safe from accidental injury and death. Many accidents, injuries and deaths are preventable.
John Lewis Emery was a British-born paediatric pathologist and emeritus professor at the University of Sheffield. Emery was most notable for being one of the founding fathers of paediatric pathology in the country, and for conducting research into haematology, developmental anatomy, congenital deformities, particularly hydrocephalus, and was probably Britain's leading scientist in the subject of unexplained infant deaths, or cot death.
Marta C. Cohen OBE is a clinical pediatric pathologist, and currently head of the Department of Histopathology and Clinical Director of Pharmacy, Pathology, and Genetics at Sheffield Children's Hospital, and an honorary professor at the University of Sheffield. Her clinical work focusses on sudden infant death.
Millard Bass, was an American forensic pathologist and former deputy medical examiner for the State of New York. In 1970 he coined the term "sudden sniffing death syndrome".