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Sample records for death syndrome childhood

  1. Childhood Deaths from Physical Abuse.

    ERIC Educational Resources Information Center

    Kasim, Mohd. Sham; and Others

    1995-01-01

    This paper describes 30 cases of childhood deaths caused by physical abuse in Kuala Lumpur, Malaysia. Data presented include ethnic origins, age, causes of death, identity of perpetrators, and marital situation of parents. (DB)

  2. Sudden Infant Death Syndrome

    MedlinePlus

    Sudden infant death syndrome (SIDS) is the sudden, unexplained death of an infant younger than one year old. Some people call SIDS "crib death" because many babies who die of SIDS are found in their ...

  3. Sudden Infant Death Syndrome

    MedlinePlus

    Sudden infant death syndrome (SIDS) is the sudden, unexplained death of an infant younger than one year old. Some people call ... boys, African Americans, and American Indian/Alaska Native infants have a higher risk of SIDS. Although health ...

  4. Sudden Death Syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS) is an important disease of soybean in North and South America. SDS first occurred in South America in the early 1990s. In the U.S.A., SDS was first detected in AK in 1971. Now SDS occurs in most soybean production areas of the U.S. The SDS pathogen is a soil-borne fungu...

  5. Sudden Infant Death Syndrome.

    ERIC Educational Resources Information Center

    Barnett, Henry L.; And Others

    There is a growing body of evidence that Sudden Infant Death Syndrome (SIDS) victims are not completely normal and healthy, as was once believed. A variety of new information from several disciplines strongly suggests that the infant who dies suddenly and unexpectedly may do so because of subtle developmental, neurologic, cardiorespiratory, and…

  6. Sudden Infant Death Syndrome (SIDS)

    MedlinePlus

    ... Information Clinical Trials Resources and Publications Sudden Infant Death Syndrome (SIDS): Condition Information Skip sharing on social ... Share this: Page Content SIDS is the sudden death of an infant younger than 1 year of ...

  7. Childhood deaths from physical abuse.

    PubMed

    Kasim, M S; Cheah, I; Shafie, H M

    1995-07-01

    This paper gives a detailed account of 30 cases of childhood deaths caused by physical abuse, detected by the Suspected Child Abuse and Neglect (SCAN) team, General Hospital, Kuala Lumpur. They consisted of 12 Malays, 6 Chinese, 9 Indian, and 1 Indonesian child. Three cases could not be ascertained as to their ethnic origin. There were 13 male and 17 female children. The average age of the abused children was 2 years 5 months. The most frequent causes of death were intracranial hemorrhage and intraabdominal trauma. Of the 17 cases of intracranial hemorrhage, only four had X-ray evidence of skull fracture. This suggests the possibility of whiplash injuries with/without the abuser suspecting that he/she had injured the child. Of the 22 abusers who could be identified, there was no sex differentiation. Fathers formed the largest group of perpetrators, followed by mothers and childminders. Fifteen of the natural parents of the abused children were married, four were divorced and four were never married. Five of the abusers had aggressive personalities and three were drug addicts. Only one abuser was found to be an alcoholic even though a few were also under suspicion. For most cases, trigger factors could not be identified. PMID:7583742

  8. Sudden infant death syndrome

    PubMed Central

    Hunt, Carl E.; Hauck, Fern R.

    2006-01-01

    Sudden infant death syndrome (SIDS) continues to be the most common cause of postneonatal infant death. SIDS is a complex, multifactorial disorder, the cause of which is still not fully understood. However, much is known now about environmental risk factors, some of which are modifiable. These include maternal and antenatal risk factors such as smoking during pregnancy, as well as infant-related risk factors such as non-supine sleeping position and soft bedding. Emerging evidence also substantiates an expanding number of genetic risk factors. Interactions between environmental and genetic risk factors may be of critical importance in determining an infant's actual risk of SIDS. Although no practical way exists to identify which infants will die of SIDS, nor is there a safe and proven prevention strategy even if identification were feasible, reducing exposure to modifiable risk factors has helped to lower the incidence of SIDS. Current challenges include wider dissemination of guidelines to all people who care for infants, dissemination of guidelines in culturally appropriate ways, and surveillance of SIDS trends and other outcomes associated with implementation of these guidelines. PMID:16785462

  9. Sudden infant death syndrome.

    PubMed

    Adams, Stephen M; Ward, Chad E; Garcia, Karla L

    2015-06-01

    Sudden infant death syndrome (SIDS) is the sudden unexpected death of a child younger than one year during sleep that cannot be explained after a postmortem evaluation including autopsy, a thorough history, and scene evaluation. The incidence of SIDS has decreased more than 50% in the past 20 years, largely as a result of the Back to Sleep campaign. The most important risk factors relate to the sleep environment. Prone and side sleeping positions are significantly more dangerous than the supine position. Bed sharing with a parent is strongly correlated with an increased risk of SIDS, especially in infants younger than 12 weeks. Apparent life-threatening events are not a risk factor for SIDS. Parents should place infants on their backs to sleep, should not share a bed, and should avoid exposing the infant to tobacco smoke. Other risk-reducing measures include using a firm crib mattress, breastfeeding, keeping vaccinations up to date, avoiding overheating due to overbundling, avoiding soft bedding, and considering the use of a pacifier during sleep once breastfeeding is established. One consequence of the Back to Sleep campaign is a significant increase in the incidence of occipital flattening. Infants who develop a flat spot should be placed with the head facing alternating directions each time he or she is put to bed. Supervised prone positioning while the infant is awake, avoiding excessive use of carriers, and upright positioning while awake are also recommended. PMID:26034855

  10. Sudden infant death syndrome

    MedlinePlus

    Crib death; SIDS ... However, SIDS is still a major cause of death in infants under 1 year old. Thousands of ... affects boys more often than girls. Most SIDS deaths occur in the winter. The following may increase ...

  11. Sudden Unexpected Death in Fetal Life Through Early Childhood.

    PubMed

    Goldstein, Richard D; Kinney, Hannah C; Willinger, Marian

    2016-06-01

    In March 2015, the Eunice Kennedy Shriver National Institute of Child Health and Human Development held a workshop entitled "Sudden Unexpected Death in Fetal Life Through Early Childhood: New Opportunities." Its objective was to advance efforts to understand and ultimately prevent sudden deaths in early life, by considering their pathogenesis as a potential continuum with some commonalities in biological origins or pathways. A second objective of this meeting was to highlight current issues surrounding the classification of sudden infant death syndrome (SIDS), and the implications of variations in the use of the term "SIDS" in forensic practice, and pediatric care and research. The proceedings reflected the most current knowledge and understanding of the origins and biology of vulnerability to sudden unexpected death, and its environmental triggers. Participants were encouraged to consider the application of new technologies and "omics" approaches to accelerate research. The major advances in delineating the intrinsic vulnerabilities to sudden death in early life have come from epidemiologic, neural, cardiac, metabolic, genetic, and physiologic research, with some commonalities among cases of unexplained stillbirth, SIDS, and sudden unexplained death in childhood observed. It was emphasized that investigations of sudden unexpected death are inconsistent, varying by jurisdiction, as are the education, certification practices, and experience of death certifiers. In addition, there is no practical consensus on the use of "SIDS" as a determination in cause of death. Major clinical, forensic, and scientific areas are identified for future research. PMID:27230764

  12. [The Sudden Infant Death Syndrome.

    ERIC Educational Resources Information Center

    Florida's Health, 1976

    1976-01-01

    This collection of articles on the Sudden Infant Death Syndrome (SIDS), drawn from a southeastern regional symposium on the subject, summarizes much of what is known about the occurrence of SIDS, including current information about its causes. The background of state action in Florida is reviewed, with emphasis on the need for increased public and…

  13. Sudden Arrhythmia Death Syndromes (SADS) Foundation

    MedlinePlus

    ... all proceeds benefiting the SADS Foundation (Sudden Arrhythmia Death Syndrome). Each year 4,000 young Americans die ... Investigator Awardees 5/19/2016 The Sudden Arrhythmia Death Syndromes (SADS) Foundation announces the winners for the ...

  14. Sudden Infant Death Syndrome: Facts for Caregivers.

    ERIC Educational Resources Information Center

    Texas Child Care, 2000

    2000-01-01

    Presents risk factors and prevention measures related to Sudden Infant Death Syndrome. Offers infant sleep recommendations and five discussion questions to test knowledge of Sudden Infant Death Syndrome. (DLH)

  15. [Frey syndrome in childhood].

    PubMed

    Clarós, P; González-Enseñat, M A; Arimany, J; Vincente, M A; Clarós, A

    1993-01-01

    Frey's syndrome is distinguished by the appearing of erythema, sensation of hotness, sometimes pain, and transpiration discharge in the preauricular and temporal area when ingestion stars. We present an eleven month old child with this pathology and we review the etiology and clinic manifestations of this syndrome. PMID:8129975

  16. Sweet Syndrome in childhood*

    PubMed Central

    dos Santos, Talita Batalha Pires; Sales, Barbara Cristina Gouveia; Sigres, Marianne; Rosman, Fernando; de Cerqueira, Ana Maria Mosca

    2015-01-01

    Sweet syndrome or acute febrile neutrophilic dermatosis is a recurrent and rare skin disease caused by the release of cytokines, with diverse possible etiologic causes. It presents clinically with polymorphic skin lesions, fever, arthralgia, and peripheral leukocytosis. In general, it is associated with infections, malignancy and drugs. It usually regresses spontaneously and treatment is primarily to control the basic disease. The authors report the case of a child of 1 year and 11 months who developed Sweet syndrome. PMID:26375229

  17. Sweet Syndrome in childhood.

    PubMed

    Santos, Talita Batalha Pires dos; Sales, Barbara Cristina Gouveia; Sigres, Marianne; Rosman, Fernando; Cerqueira, Ana Maria Mosca de

    2015-01-01

    Sweet syndrome or acute febrile neutrophilic dermatosis is a recurrent and rare skin disease caused by the release of cytokines, with diverse possible etiologic causes. It presents clinically with polymorphic skin lesions, fever, arthralgia, and peripheral leukocytosis. In general, it is associated with infections, malignancy and drugs. It usually regresses spontaneously and treatment is primarily to control the basic disease. The authors report the case of a child of 1 year and 11 months who developed Sweet syndrome. PMID:26375229

  18. [Autoinflammatory syndromes in childhood].

    PubMed

    Horneff, G

    2015-08-01

    Systemic autoinflammatory diseases are a group of hereditary and non-hereditary diseases of the innate immune system, characterized by inflammation with no apparent cause, recurrence at irregular intervals and manifestation on the skin, mucous membranes, joints, bone, gastrointestinal tract, blood vessels and the central nervous system (CNS). Amyloidosis and other possibly severe long-term complications are important. Advances in genetics and molecular biology have improved understanding of the pathogenesis of these diseases, including familial Mediterranean fever, mevalonate kinase deficiency syndrome, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndrome and improved others. The vast majority of these diseases are based on activation of the interleukin-1 (IL-1) pathway, so that inhibition of IL-1 provides a therapeutic option. Other syndromes are characterized by a granulomatous inflammation. Newer autoinflammatory diseases, such as chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) and stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) are, however, driven by interferons. PMID:26238708

  19. Psychosocial Aspects of the Sudden Infant Death Syndrome ("Cot Death").

    ERIC Educational Resources Information Center

    Bluglass, Kerry

    1981-01-01

    Reviews literature on reactions of parents and siblings to Sudden Infant Death Syndrome (SIDS). The prospects for prolonged, adverse reactions are considered, and professional concerns regarding abnormal adaptation are noted. (Author/DB)

  20. Behavior of chickens prior to death from sudden death syndrome.

    PubMed

    Newberry, R C; Gardiner, E E; Hunt, J R

    1987-09-01

    A study was made to determine if chickens dying from sudden death syndrome (SDS) showed any unusual behavioral characteristics during the final 12 h preceding death. Continuous video recordings were made of floor pens of 50 to 120 individually marked male broiler chickens between 3 and 10 wk of age. Behavioral data were obtained from video tapes played back following death of chickens from SDS. Analysis of the video tapes revealed no significant differences between 10 SDS chickens and their matched controls in the frequencies or proportions of time spent in each of 19 different behavioral activities. All SDS chickens exhibited a sudden attack prior to death lasting an average of 53 s and characterized by loss of balance, violent flapping, and strong muscular contractions. There was no evidence that death was preceded by a particular environmental or behavioral event. It was concluded that there were no consistent behavioral symptoms which could be used to identify SDS chickens prior to death. PMID:3684869

  1. Early Childhood Injury Deaths in Washington State.

    ERIC Educational Resources Information Center

    Starzyk, Patricia M.

    This paper discusses data on the deaths of children aged 1-4 years in Washington State. A two-fold approach was used in the analysis. First, Washington State death certificate data for 1979-85 were used to characterize the deaths and identify hazardous situations. Second, death certificates were linked to birth certificates of children born in…

  2. Pathways to lifespan health following childhood parental death

    PubMed Central

    Luecken, Linda J.; Roubinov, Danielle S.

    2013-01-01

    The death of a parent is a profoundly stressful form of childhood adversity, increasing the short- and long-term risk of mental health problems. Emerging research suggests it may also disrupt biological regulatory systems and increase the risk of long-term physical health problems. This article presents a theoretical framework of the process by which the experience of parental death during childhood may influence mental and physical health outcomes over time. Drawing from a broad literature on adaptation following childhood parental loss, we focus on risk and protective factors in the childhood environment that are theoretically and empirically linked to emotional and biological regulatory responses to stress later in life, the effects of which may accumulate to impact long-term health. PMID:23555319

  3. The CAST (Childhood Asperger Syndrome Test)

    ERIC Educational Resources Information Center

    Williams, Jo; Scott, Fiona; Stott, Carol; Allison, Carrie; Bolton, Patrick; Baron-Cohen, Simon; Brayne, Carol

    2005-01-01

    The Childhood Asperger Syndrome Test (CAST) is a parental questionnaire to screen for autism spectrum conditions. In this validation study, the CAST was distributed to 1925 children aged 5-11 in mainstream Cambridgeshire schools. A sample of participants received a full diagnostic assessment, conducted blind to screen status. The sensitivity of…

  4. Childhood of Males with the XYY Syndrome

    ERIC Educational Resources Information Center

    Nielsen, Johannes; And Others

    1973-01-01

    Investigated to determine specific intelligence, personality characteristics, and behavioral patterns of boys with the XYY syndrome (a rare pattern of sex chromosome imbalance) were the childhood and adolescence of 20 males, 6- to 58-years of age at time of diagnosis. (Author/MC)

  5. [Metabolic syndrome in childhood and adolescence].

    PubMed

    Barkai, László; Paragh, György

    2006-02-12

    Metabolic syndrome has an outstanding impact on public health due to its increasing prevalence and poor prognosis. The development of insulin resistance, as a consequence of obesity, can be demonstrated even in childhood which has a pivotal role in the pathomechanism of the syndrome. Besides obesity, low birth weight, increased gain in body mass in early childhood, decreased pubertal insulin sensitivity and clinical markers of insulin resistance (acanthosis nigricans, polycystic ovarian syndrome, premature adrenarche) confer risk of metabolic syndrome. Currently, there are no consistent and consensus based diagnostic criteria of metabolic syndrome in children and adolescents. The most recent definition of the International Diabetes Federation [central obesity plus any two of four factors (raised triglyceride, reduced high-density lipoprotein-cholesterol, raised blood pressure and raised fasting plasma glucose)] is not approved for children and epidemiology data are not yet available. Applying the modified version of the most commonly used Adult Treatment Panel III diagnostic system for children and adolescents, the prevalence is given as 4.2% in the literature. As the components of the syndrome, frequency were as follows: 9.8-17.9% for abdominal obesity, 21.0-23.4% for elevated triglyceride, 18.3-23.3% for reduced high-density lipoprotein-cholesterol, 4.9-7.1% for elevated blood pressure and 0.8-1.7% for impaired fasting glucose. High frequency of morphological and functional disturbances of the vascular and endothelial systems seen frequently among children with signs of metabolic syndrome suggests early cardiovascular events and underlines the clinical significance of this entity. The most effective tool for prevention of metabolic syndrome is to avoid the development of childhood obesity. In case of established disease, the effective treatment should address the different components of the syndrome. The authors emphasize the need of elaboration of consensus

  6. Alcohol Use and Sudden Infant Death Syndrome

    ERIC Educational Resources Information Center

    Friend, Karen B.; Goodwin, Matthew S.; Lipsitt, Lewis P.

    2004-01-01

    Despite general evidence of fetal toxicities associated with sudden infant death syndrome (SIDS), there has been limited research focusing on the effects of parental alcohol use on SIDS occurrence, either directly or in interaction with other risk conditions. The purpose of this paper is to review the literature on parental, especially maternal,…

  7. Management of childhood onset nephrotic syndrome.

    PubMed

    Gipson, Debbie S; Massengill, Susan F; Yao, Lynne; Nagaraj, Shashi; Smoyer, William E; Mahan, John D; Wigfall, Delbert; Miles, Paul; Powell, Leslie; Lin, Jen-Jar; Trachtman, Howard; Greenbaum, Larry A

    2009-08-01

    The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967. The characteristics of children presenting with nephrotic syndrome have changed over recent decades with greater frequency of the challenging condition focal segmental glomerulosclerosis and a greater prevalence of obesity and diabetes mellitus, which may be resistant to glucocorticoids in the former and exacerbated by long-term glucocorticoid therapy in the latter 2 conditions. The Children's Nephrotic Syndrome Consensus Conference was formed to systematically review the published literature and generate a children's primary nephrotic syndrome guideline for use in educational, therapeutic, and research venues. PMID:19651590

  8. Accidental childhood death and the role of the pathologist.

    PubMed

    Byard, R W

    2000-01-01

    The following study provides an overview of accidental childhood death. This study is based on a review of 369 cases of fatal childhood accidents taken from the records of the Department of Histopathology, Women's and Children's Hospital, Adelaide, Australia, over a 34-year period from 1963 to 1996. Data provide information on deaths due to motor vehicle accidents, drownings, accidental asphyxia, burns, poisonings, electrocution, and miscellaneous trauma. In addition, certain categories have undergone further examination, including asphyxial deaths due to unsafe sleeping environments and unsafe eating practices, drowning deaths, and deaths on farms, following identification of significant child safety problems in these areas as part of the "Keeping Your Baby and Child Safe" program. Previously unrecognized dangers to children detected through this program include mesh-sided cots, V-shaped pillows, and certain types of stroller-prams. The production of information pamphlets and packages for parents and the recall of certain dangerous products following recommendations made by pathologists demonstrate that pediatric and forensic pathologists have an important role to play in preventive medicine issues and in formulating public health strategies. PMID:10890925

  9. Forensic epidemiology of childhood deaths in Nebraska, USA.

    PubMed

    Okoye, Cordelia N; Okoye, Matthias I

    2011-11-01

    In a 7-year period (April 1, 2003-March 31, 2010), all medico-legal childhood deaths aged 0-18 years investigated by the Lancaster County Coroner's Office under the auspices of Nebraska Institute of Forensic Sciences, Inc. (NIFS), were retrospectively reviewed (n = 140). This number of cases represents 10.9% of the 1287 forensic autopsies performed during the same period. Age, race, gender, cause and manner of deaths were analyzed for all victims categorized into five age groups: 0-1 year, 1-4 years, 5-9 years, 10-14 years, and 15-18 years. Male victims predominated with 98 cases (70%) versus 42 cases (30%) for females giving a male to female ratio of 2.3: 1. The mean age of the children was 7.6 years. The racial composition was 86.4% white, 10.7% Hispanic, 0.7% American Indian, 1.4% African American, and 0.7% Asian American. The majority of deaths occurred in the 0-1 age group (50 cases), followed in rank order by the 15-18 age group (40 cases), the 1-4 age group (23 cases), the 10-14 age group (17 cases), and the 5-9 age group (10 cases). The most common manner of death was accident, followed by natural, suicide, homicide, and undetermined. Accidents accounted for 71 cases (50.7%) of all the deaths and are amenable to prevention. Accidental blunt force trauma accounted for 41 cases or 58% of all the accident cases. The share of motor vehicle crashes in total blunt force trauma deaths was 33 cases. Natural deaths comprised 42 cases or 30% of all the deaths. Suicide (19 cases or 13.6% of all the deaths) was only encountered in the older age groups, the 10-14 age group (6 cases) and the 15-18 age group (13 cases). However, homicide which was observed as the least common manner of death (7 cases) was more predominant among the younger age groups (0-1 and 1-4 age groups). This review may provide useful information for the forensic pathologist, death investigators, law enforcement officers, policy makers, healthcare providers and Nebraska Child Death Review Team in

  10. Ethnic Differences in Childhood Nephrotic Syndrome

    PubMed Central

    Chanchlani, Rahul; Parekh, Rulan S.

    2016-01-01

    Nephrotic syndrome is a common glomerular disease in children with significant variability in both incidence and steroid responsiveness among various ethnic groups. The average incidence of nephrotic syndrome is 2–16.9 per 100,000 children worldwide. Understanding the variability by ethnicity may point to potential factors leading to nephrotic syndrome, which remains elusive, and may highlight factors accounting for differences in medication response. The emerging role of genetic factors associated with steroid responsive and steroid-resistant forms of nephrotic syndrome within an ethnic group can provide insight into potential biological mechanisms leading to disease. For example, among African-Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a twofold greater risk of progression to end-stage renal disease. Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes. In the future, the availability of whole-genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to a better understanding of the disease. PMID:27148508

  11. Sudden Unexpected Infant Death and Sudden Infant Death Syndrome: Reducing the Risk

    MedlinePlus

    ... organizations offer support: CJ Foundation for SIDS First Candle Sudden Unexplained Death In Childhood Foundation (SUDC) The ... and Caregivers Healthy Children Safe to Sleep First Candle CJ Foundation for SIDS Cribs for Kids Safe ...

  12. Escherichia coli and Sudden Infant Death Syndrome

    PubMed Central

    Bettelheim, Karl A.; Goldwater, Paul N.

    2015-01-01

    This review examines the association of strains of Escherichia coli with sudden infant death syndrome (SIDS) and the possible role these bacteria play in this enigmatic condition. The review addresses evidence for E. coli in SIDS infants, potential sources of E. coli in the environment, colonization by commensal and pathogenic strains, the variety of currently accepted pathotypes, and how these pathotypes could compromise intestinal integrity and induce inflammation. Both intestinal and extraintestinal pathotypes are compared in relation to the apparent liability in which virulence traits can be gained or lost by strains of E. coli. The way in which E. coli infections fit with current views on infant sleeping position and other SIDS risk factors is highlighted. PMID:26191064

  13. Endotoxemia in sudden infant death syndrome.

    PubMed

    Platt, M S; Elin, R J; Hosseini, J M; Smialek, J E

    1994-09-01

    Endotoxemia has been proposed as a significant cause of Sudden Infant Death Syndrome (SIDS). We examined postmortem sera from left and right heart samples of 21 SIDS cases (1989 definition) and 23 controls. The controls were < 1 year of age and had died suddenly and unexpectedly of infection, abuse, suffocation, blunt injury, or fire and smoke inhalation. Endotoxin was measured without knowledge of the clinical status by using a kinetic modification of the chromogenic limulus amoebocyte lysate assay. The SIDS cases had insignificant concentrations of endotoxin in serum, whereas some of the controls who experienced blunt injury, abuse, or severe infection exhibited moderately elevated concentrations. Postmortem interval and postmortem blood culture results did not materially affect endotoxin concentrations. Thus, we conclude that endotoxemia is not a substantial pathophysiologic event in SIDS. PMID:7825560

  14. Deaths among Children, Adolescents, and Young Adults with Down Syndrome

    ERIC Educational Resources Information Center

    Miodrag, Nancy; Silverberg, Sophie E.; Urbano, Richard C.; Hodapp, Robert M.

    2013-01-01

    Background: Although life expectancies in Down syndrome (DS) have doubled over the past 3-4 decades, there continue to be many early deaths. Yet, most research focuses on infant mortality or later adult deaths. Materials and Methods: In this US study, hospital discharge and death records from the state of Tennessee were linked to examine 2046…

  15. Sudden Infant Death Syndrome and Residential Altitude

    PubMed Central

    Shore, Supriya; Bandle, Brian; Niermeyer, Susan; Bol, Kirk A.; Khanna, Amber

    2015-01-01

    BACKGROUND: Theories of sudden infant death syndrome (SIDS) suggest hypoxia is a common pathway. Infants living at altitude have evidence of hypoxia; however, the association between SIDS incidence and infant residential altitude has not been well studied. METHODS: We performed a retrospective cohort study by using data from the Colorado birth and death registries from 2007 to 2012. Infant residential altitude was determined by geocoding maternal residential address. Logistic regression was used to determine adjusted association between residential altitude and SIDS. We evaluated the impact of the Back to Sleep campaign across various altitudes in an extended cohort from 1990 to 2012 to assess for interaction between sleep position and altitude. RESULTS: A total of 393 216 infants born between 2007 and 2012 were included in the primary cohort (51.4% boys; mean birth weight 3194 ± 558 g). Overall, 79.6% infants resided at altitude <6000 feet, 18.5% at 6000 to 8000 feet, and 1.9% at >8000 feet. There were no meaningful differences in maternal characteristics across altitude groups. Compared with residence <6000 feet, residence at high altitude (>8000 feet), was associated with an adjusted increased risk of SIDS (odds ratio 2.30; 95% confidence interval 1.01–5.24). Before the Back to Sleep campaign, the incidence of SIDS in Colorado was 1.99/1000 live births and dropped to 0.57/1000 live births after its implementation. The Back to Sleep campaign had similar effect across different altitudes (P = .45). CONCLUSIONS: Residence at high altitude was significantly associated with an increased adjusted risk for SIDS. Impact of the Back to Sleep campaign was similar across various altitudes. PMID:26009621

  16. Childhood Sexual Abuse and Psychosomatic Symptoms in Irritable Bowel Syndrome

    ERIC Educational Resources Information Center

    Ross, Colin A.

    2005-01-01

    Irritable bowel syndrome is characterized by chronic gastrointestinal symptoms without a demonstrable physical cause. In a subgroup of patients, irritable bowel syndrome may be part of a cluster of psychosomatic symptoms related to childhood sexual abuse. To investigate this possibility, the Dissociative Disorders Interview Schedule (DDIS), the…

  17. Drowning as a Cause of Death in Angelman Syndrome.

    ERIC Educational Resources Information Center

    Ishmael, Holly A.; Begleiter, Michael L.; Butler, Merlin G.

    2002-01-01

    This study reports on a 9-year-old boy previously diagnosed with Angelman syndrome who died unexpectedly by drowning in a shallow backyard wading pool. The case illustrates the fascination with water by individuals with Angelman syndrome and highlights that this fascination may lead to death. The need for supervision is stressed. (Contains 5…

  18. Training Emergency Responders: Sudden Infant Death Syndrome. An Instructor's Manual.

    ERIC Educational Resources Information Center

    Applied Science Associates, Inc., Reston, VA.

    This manual was developed to help instructors train police and emergency medical technicians, who often are the first persons to arrive at the scene of a death (first responders), to serve families who lose a child to Sudden Infant Death Syndrome (SIDS). The manual begins with an introduction that discusses the purpose of the training and…

  19. New Areas for Preventive Programing: Sudden Infant Death Syndrome.

    ERIC Educational Resources Information Center

    Lowman, Joseph

    Crisis intervention programs for persons experiencing the sudden death of family members or surviving natural disasters have been advocated as methods of primary prevention, although few have actually been implemented. A program utilizing nurses to deliver grief intervention to parents losing a baby to Sudden Infant Death Syndrome (SIDS) was…

  20. Diaphragm strength in near-miss sudden infant death syndrome.

    PubMed

    Scott, C B; Nickerson, B G; Sargent, C W; Dennies, P C; Platzker, A C; Keens, T G

    1982-06-01

    Diaphragm muscle strength was measured as maximal transdiaphragmatic pressure during airway occlusion in ten near-miss sudden infant death syndrome infants aged 4.1 +/- 0.6 (SE) months post-term, range 2 to 7 months, and ten control infants aged 4.5 +/- 0.8 months post-term, range 0.8 to 8 months. In the near-miss sudden infant death syndrome group, the mean maximal transdiaphragmatic pressure was 106 +/- 6 cm H2O, range 78 to 132 cm H2O, compared with a mean maximal transdiaphragmatic pressure 86 +/- 4 cm H2O, range 69 to 106 cm H2O, in the control group. Diaphragm strength is normal or increased in near-miss sudden infant death syndrome infants. PMID:7079044

  1. The Childhood Asperger Syndrome Test (Cast): Test--Retest Reliability

    ERIC Educational Resources Information Center

    Williams, Jo; Allison, Carrie; Scott, Fiona; Stott, Carol; Bolton, Patrick; Baron-Cohen, Simon; Brayne, Carol

    2006-01-01

    The Childhood Asperger Syndrome Test (CAST) is a 37-item parental self-completion questionnaire to screen for autism spectrum conditions in research. Good test accuracy was demonstrated in studies with primary school aged children in mainstream schools. The aim of this study was to investigate the test-retest reliability of the CAST. Parents of…

  2. Sudden Death Due to Undiagnosed Wilkie Syndrome.

    PubMed

    Baber, Yeliena Fay; OʼDonnell, Chris

    2016-06-01

    A 56-year-old transgender woman with mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes syndrome and diabetes presented to hospital with headaches and experiencing with malnutrition. She was agitated and refused medical and physical assistance. Soon after admission, she started to vomit and developed abdominal pain, becoming rapidly unresponsive on the ward after attending the radiology department, and was pronounced deceased. Autopsy revealed a cachectic transgender woman with a grossly distended stomach and proximal duodenum containing 2 L of liquid. The postmortem computed tomography scan showed compression of the duodenum by the superior mesenteric artery, diagnostic of Wilkie syndrome. Superior mesenteric artery syndrome, or Wilkie syndrome, was first described in 1861 by Von Rokitansky. It is an uncommon but well-recognized clinical entity characterized by compression of the third, or transverse, portion of the duodenum between the aorta and the superior mesenteric artery. This results in chronic, intermittent, or acute complete or partial duodenal obstruction. It is a well-recognized complication of anorexia. PMID:26963629

  3. Causes of death in 2877 patients with myelodysplastic syndromes.

    PubMed

    Nachtkamp, Kathrin; Stark, Romina; Strupp, Corinna; Kündgen, Andrea; Giagounidis, Aristoteles; Aul, Carlo; Hildebrandt, Barbara; Haas, Rainer; Gattermann, Norbert; Germing, Ulrich

    2016-05-01

    Patients with myelodysplastic syndromes face a poor prognosis. The exact causes of death have not been described properly in the past. We performed a retrospective analysis of causes of death using data of 3792 patients in the Düsseldorf registry who have been followed up for a median time of 21 months. Medical files as well as death certificates were screened and primary care physicians were contacted. Death after AML evolution, infection, and bleeding was considered to be clearly disease-related. Further categories of causes of death were heart failure, other possibly disease-related reasons, such as hemochromatosis, disease-independent reasons as well as cases with unclear causes of death. Median age at the time of diagnosis was 71 years. At the time of analysis, 2877 patients (75.9 %) had deceased. In 1212 cases (42.1 %), the exact cause of death could not be ascertained. From 1665 patients with a clearly documented cause of death, 1388 patients (83.4 %) succumbed directly disease-related (AML (46.6 %), infection (27.0 %), bleeding (9.8 %)), whereas 277 patients (16.6 %) died for reasons not directly related with myelodysplastic syndromes (MDS), including 132 patients with cardiac failure, 77 non-disease-related reasons, 23 patients with solid tumors, and 45 patients with possibly disease-related causes like hemochromatosis. Correlation with IPSS, IPSS-R, and WPSS categories showed a proportional increase of disease-related causes of death with increasing IPSS/IPSS-R/WPSS risk category. Likewise, therapy-related MDS were associated with a higher percentage of disease-related causes of death than primary MDS. This reflects the increasing influence of the underlying disease on the cause of death with increasing aggressiveness of the disease. PMID:27025507

  4. Long-term Outcomes of Childhood Onset Nephrotic Syndrome

    PubMed Central

    Hjorten, Rebecca; Anwar, Zohra; Reidy, Kimberly Jean

    2016-01-01

    There are limited studies on long-term outcomes of childhood onset nephrotic syndrome (NS). A majority of children with NS have steroid-sensitive nephrotic syndrome (SSNS). Steroid-resistant nephrotic syndrome (SRNS) is associated with a high risk of developing end-stage renal disease. Biomarkers and analysis of genetic mutations may provide new information for prognosis in SRNS. Frequently relapsing and steroid-dependent NS is associated with long-term complications, including dyslipidemia, cataracts, osteoporosis and fractures, obesity, impaired growth, and infertility. Long-term complications of SSNS are likely to be under-recognized. There remain many gaps in our knowledge of long-term outcomes of childhood NS, and further study is indicated. PMID:27252935

  5. Parental Perceptions of Siblings' Grieving after a Childhood Cancer Death: A Longitudinal Study

    ERIC Educational Resources Information Center

    Barrera, Maru; Alam, Rifat; D'Agostino, Norma Mammone; Nicholas, David B.; Schneiderman, Gerald

    2013-01-01

    We investigated longitudinally parental perceptions of siblings' bereavement after childhood cancer death. Parents were interviewed 6 months (n = 25) and 18 months (n = 15) post-death. Data are analyzed combined and over time. The following themes emerged: (a) expression of grief: missing deceased child (verbally, crying), behavioral problems,…

  6. The Development of the Concept of Death in Childhood: A Review of the Literature.

    ERIC Educational Resources Information Center

    Stambrook, Michael; Parker, Kevin C. H.

    1987-01-01

    A critical analysis of the current knowlege concerning the development of the concept of death in childhood is the purpose of this paper. Theoretical models and methodological limitations of the literature are addressed. Many factors have been implicated as contributing to the development of the concept of death. (Author/BN)

  7. Detection of sudden death syndrome using a multispectral imaging sensor

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS), caused by the fungus Fusarium solani f. sp. glycines, is a widespread mid- to late-season disease with distinctive foliar symptoms. This paper reported the development of an image analysis based method to detect SDS using a multispectral image sensor. A hue, saturation a...

  8. Sudden Infant Death Syndrome, FY 1983. Special Report to Congress.

    ERIC Educational Resources Information Center

    National Inst. of Child Health and Human Development (NIH), Bethesda, MD.

    This report describes research programs focusing on the sudden infant death syndrome (SIDS) and indicates some presently available results. Specific attention is given to research on sleep apnea, respiratory control, and hypoxia, as well as to infectious disease processes and immunology. Findings of a large-scale multidisciplinary SIDS project are…

  9. Research advances and management of soybean sudden death syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Fusarium virguliforme causes soybean sudden death syndrome (SDS) in the United States. The disease was first observed in Arkansas in 1971, and since has been reported in most soybean-producing states, with a general movement from the southern to the northern states. In addition to F. virguliforme, ...

  10. Pokkuri Death Syndrome; sudden cardiac death cases without coronary atherosclerosis in South Asian young males.

    PubMed

    Nakajima, Katsuyuki; Takeichi, Sanae; Nakajima, Yasuhiro; Fujita, Masaki Q

    2011-04-15

    Sudden death is one of the major concerns in forensic medicine. Especially when the deceased is a young subject without significant history, the case will be of major interest to the authorities. Sudden unexplained cardiac death has been known as "Pokkuri Death Syndrome" (PDS) in Japan, "Lai Tai" in Thailand, "Bangungut" in the Philippines, "Dream Disease" in Hawaii, and "Sudden Unexpected Nocturnal Death Syndrome" among South Asian immigrants in the USA. However, the clinical and pathological features of these sudden death cases, especially the characteristics of no coronary atherosclerosis, are surprisingly similar and mainly occur among Southeast Asian young males during sleep in the midnight. In this manuscript, we have reviewed the pathological characteristics and the possible mechanism of death in PDS cases, which were associated with significantly elevated remnant lipoproteins in plasma as revealed from our studies during the past 15 years in Japan. Although elevated plasma remnant lipoproteins have been known to be strongly atherogenic, coronary atherosclerosis was not observed in PDS cases. PDS cases were shown to be an interesting cardiovascular disease death discovered in forensic medicine research, which may suggest the difference between the occurrence of cardiovascular events and the severity of coronary atherosclerosis as separate factors. These observations in PDS cases suggest the possibility that the intervention could be more targeted to suppress the cardiovascular events rather than to slow down the progression of atherosclerosis, which is now most extensively targeted for the therapy of cardiovascular disease in Western countries. PMID:21084168

  11. Childhood Bereavement: Psychopathology in the 2 Years Postparental Death

    ERIC Educational Resources Information Center

    Cerel, Julie; Fristad, Mary A.; Verducci, Joseph; Weller, Ronald A.; Weller, Elizabeth B.

    2006-01-01

    Objective: Although the death of a parent is one of the most significant stressors a child can experience, the psychiatric sequelae of parental death are not fully understood. Method: A total of 360 parent-bereaved children (ages 6-17) and their surviving parents were directly interviewed four times during the first 2 years following the death (at…

  12. Underlying molecular and cellular mechanisms in childhood irritable bowel syndrome.

    PubMed

    Chumpitazi, Bruno P; Shulman, Robert J

    2016-12-01

    Irritable bowel syndrome (IBS) affects a large number of children throughout the world. The symptom expression of IBS is heterogeneous, and several factors which may be interrelated within the IBS biopsychosocial model play a role. These factors include visceral hyperalgesia, intestinal permeability, gut microbiota, psychosocial distress, gut inflammation, bile acids, food intolerance, colonic bacterial fermentation, and genetics. The molecular and cellular mechanisms of these factors are being actively investigated. In this mini-review, we present updates of these mechanisms and, where possible, relate the findings to childhood IBS. Mechanistic elucidation may lead to the identification of biomarkers as well as personalized childhood IBS therapies. PMID:26883355

  13. The metabolic syndrome and body composition in childhood cancer survivors

    PubMed Central

    Sohn, Young Bae; Kim, Su Jin; Park, Sung Won; Kim, Se-Hwa; Cho, Sung-Yoon; Lee, Soo Hyun; Yoo, Keon Hee; Sung, Ki Woong; Chung, Jae Hoon; Koo, Hong Hoe

    2011-01-01

    Purpose Long-term survivors of childhood cancer appear to have an increased risk for the metabolic syndrome, subsequent type 2 diabetes and cardiovascular disease in adulthood compared to healthy children. The purpose of this study was to investigate the frequency of the metabolic syndrome and associated factors in childhood cancer survivors at a single center in Korea. Methods We performed a retrospective review of medical records of 98 childhood cancer survivors who were diagnosed and completed anticancer treatment at Samsung Medical Center, Seoul, Korea between Jan. 1996 and Dec. 2007. Parameters of metabolic syndrome were evaluated between Jan. 2008 and Dec. 2009. Clinical and biochemical findings including body fat percentage were analyzed. Results A total of 19 (19.4%) patients had the metabolic syndrome. The median body fat percentage was 31.5%. The body mass index and waist circumference were positively correlated with the cranial irradiation dose (r=0.38, P<0.001 and r=0.44, P<0.00, respectively). Sixty-one (62.2%) patients had at least one abnormal lipid value. The triglyceride showed significant positive correlation with the body fat percentage (r=0.26, P=0.03). The high density lipoprotein cholesterol showed significant negative correlation with the percent body fat (r=-0.26, P=0.03). Conclusion Childhood cancer survivors should have thorough metabolic evaluation including measurement of body fat percentage even if they are not obese. A better understanding of the determinants of the metabolic syndrome during adolescence might provide preventive interventions for improving health outcomes in adulthood. PMID:21949520

  14. Sudden Unexplained Nocturnal Death Syndrome in Central China (Hubei)

    PubMed Central

    Chen, Zhenglian; Mu, Jiao; Chen, Xinshan; Dong, Hongmei

    2016-01-01

    Abstract A retrospective study was conducted at Tongji Forensic Medical Center in Hubei (TFMCH) from 1999 to 2014. Forty-nine cases of sudden unexplained nocturnal death syndrome (SUNDS) were collected. The SUNDS rate was 1.0% in the total number of cases, in which an incidence was fluctuating over the years. Interestingly, April and January, and 3:00 to 6:00 am were the peak months and times of death. Among the decedents, farmers and migrant workers accounted for 67.3%. The syndrome predominantly attacked males in their 30s. One victim had sinus tachycardia. Thirteen victims (26.5%) were witnessed and had abnormal symptoms near death. Macroscopically, compared to sudden noncardiac deaths, the weights of brain, heart, and lungs had no statistical difference in SUNDS. Microscopically, the incidence of lung edema (45 cases, 91.8%) was significantly higher in SUNDS group than in the control group (27 cases, 55.1%). 82.9% of 35 SUNDS cases examined displayed minor histological anomalies of the cardiac conduction system (CCS), including mild or moderate fatty, fibrous or fibrofatty tissue replacement, insignificant stenosis of node artery, and punctate hemorrhage in the node area. These findings suggested that minor CCS abnormalities might be the substrates for some SUNDS deaths. Therefore, SUNDS victims might suffer ventricular fibrillation and acute cardiopulmonary failure before death. Further in-depth studies are needed to unveil the underlying mechanisms of SUNDS. PMID:26945374

  15. Natural history of Wolff-Parkinson-White syndrome diagnosed in childhood.

    PubMed

    Cain, Nicole; Irving, Claire; Webber, Steven; Beerman, Lee; Arora, Gaurav

    2013-10-01

    Wolff-Parkinson-White (WPW) syndrome carries a risk for symptomatic arrhythmias and sudden death. The aim of this study was to examine the natural history of patients with Wolff-Parkinson-White syndrome diagnosed in childhood followed longitudinally at a single institution. The study population consisted of 446 patients. The median age of diagnosis was 7 years, and 61% were male. Associated heart disease was present in 40 patients (9%). Modes of presentation included supraventricular tachycardia (38%), palpitations (22%), chest pain (5%), syncope (4%), atrial fibrillation (0.4%), sudden death (0.2%), and incidental findings (26%); data were unavailable in 4%. During the study period, a total of 243 patients (54%) had supraventricular tachycardia, and 7 patients (1.6%) had atrial fibrillation. Of patients who presented at ≤3 months of age, 35% had resolution of manifest preexcitation compared with 5.8% who presented at >3 months of age (p <0.0001). There were 6 sudden deaths (1.3%), with an incidence of 2.8 per 1,000 patient-years. Two of these patients had structurally normal hearts (incidence 1.1 per 1,000 patient-years). Four of these patients had associated heart disease (incidence 27 per 1,000 patient-years) (p <0.01). In conclusion, in a large population of patients with Wolff-Parkinson-White syndrome diagnosed in childhood, 64% had symptoms at presentation, and an additional 20% developed symptoms during follow-up. There were 6 sudden deaths (1.3%), with an overall incidence of 1.1 per 1,000 patient-years in patients with structurally normal hearts and 27 per 1,000 patient-years in patients with associated heart disease. PMID:23827401

  16. Sudden arrhythmic death syndrome: a national survey of sudden unexplained cardiac death

    PubMed Central

    Behr, E R; Casey, A; Sheppard, M; Wright, M; Bowker, T J; Davies, M J; McKenna, W J; Wood, D A

    2007-01-01

    Objective To describe the characteristics of sudden arrhythmic death syndrome (SADS) and compare its incidence with official national mortality statistics for unascertained deaths. Design and setting Sudden unexplained deaths were prospectively surveyed through 117 coroners' jurisdictions in England. Consecutive cases meeting the following criteria were included: white Caucasian, aged 4–64 years, no history of cardiac disease, last seen alive within 12 h of death, normal coroner's autopsy, cardiac pathologist's confirmation of a normal heart and negative toxicology. Main outcome measures The estimated mortality from SADS was calculated and the official mortality statistics for unascertained causes of deaths in 4–64‐year‐olds was identified for the same time period. Results 115 coroner's cases were reported and 56 (49%) SADS victims were identified: mean age 32 years, range 7–64 years and 35 (63%) male. 7 of 39 cases (18%) had a family history of other premature sudden deaths (<45). The estimated mortality from SADS was 0.16/100 000 per annum (95% CI 0.12 to 0.21), compared with an official mortality of 0.10/100 000 per annum for International Classification of Diseases 798.1 (sudden death, cause unknown—instantaneous death) or 1.34/100 000 per annum for unascertained causes of death. Conclusions Deaths from SADS occur predominantly in young males. When compared with official mortality, the incidence of SADS may be up to eight times higher than estimated: more than 500 potential SADS cases per annum in England. Families with SADS carry genetic cardiac disease, placing them at risk of further sudden deaths. SADS should therefore be a certifiable cause of death prompting specialised cardiological evaluation of families. PMID:17237131

  17. Wolff-Parkinson-White syndrome and sudden cardiac death.

    PubMed

    Prystowsky, E N; Fananapazir, L; Packer, D L; Thompson, K A; German, L D

    1987-01-01

    Every year, individuals with no history of heart disease succumb to sudden cardiac death (SCD). Pathologic examination of the hearts usually reveals various forms of heart disease as hypertrophic cardiomyopathy or coronary artery disease. In other cases, however, there is no obvious structural heart disease, and it is possible that some of these individuals died because of a cardiac arrhythmia involving an accessory pathway. If this were the case, the most likely scenario would be onset of atrioventricular reciprocating tachycardia (AVRT), degeneration of the AVRT into atrial fibrillation with a rapid ventricular response over the accessory pathway, and subsequent death caused by the development of ventricular fibrillation. Although these events have been documented, albeit rarely, during intracardiac electrophysiologic studies, in reality very little is known about the natural history of asymptomatic and untreated patients with Wolff-Parkinson-White (WPW) syndrome. In fact, SCD in a previously asymptomatic patient with WPW syndrome is probably relatively rare. Whether asymptomatic WPW patients should undergo electrophysiologic or pharmacologic testing to determine their 'potential' to develop serious cardiac arrhythmias is controversial. The present paucity of data concerning the natural history of WPW syndrome in asymptomatic patients militates against successful identification of those patients who are at risk for sudden death. Long-term prospective studies are necessary to clarify which asymptomatic patients with WPW syndrome require treatment. PMID:3621280

  18. Causes of death in X chromatin positive males (Klinefelter's syndrome).

    PubMed Central

    Price, W H; Clayton, J F; Wilson, J; Collyer, S; De Mey, R

    1985-01-01

    The causes of death in 466 X chromatin positive males (Klinefelter's syndrome) studied prospectively over the last 25 years have been analysed. We have previously reported the overall mortality to be increased by 50% and life expectancy reduced by about five years. A highly significant increase in mortality from cerebrovascular disease was observed in the sub group considered to be most representative of X chromatin positive males in general. In the age group up to 45 years this increase could be attributed to deaths from subarachnoid haemorrhage. An increase in mortality from respiratory diseases was observed in those ascertained in psychiatric hospitals. In the sample as a whole there were small but highly significant numbers of deaths from carcinoma of the breast and aortic valve disease. The deaths from carcinoma of the breast were comparable with those expected if female mortality rates were applied. PMID:4086964

  19. Almost 19 million childhood injuries result in 11 thousand deaths.

    PubMed

    Waldman, H B

    1996-01-01

    Details are provided from a series of government and private agency reports on the accidents and related deaths of children and the effectiveness of efforts being made to reduce the incidence of these tragedies. In 1992 there were 83,000 accidental deaths and more than 17 million disabling injuries in the United States costing $399 billion. The death rate was down 10 percent from 1991, and also the lowest recorded in recent years. Included in these statistics are 19 million injured children and 11 thousand dead children. The leading cause of death of children less than ten years of age was an unintentional injury. The author presents details on the accidents and related deaths, as well as the effectiveness of efforts to reduce the incidence of these accidents. From the youngest ages to the teen years, a greater number of males than females are injured and die from accident-related causes. The number of accidental deaths of children, ages five to nine years, almost equalled the number of deaths from natural causes. For children ten to fourteen years old, the number of accidental deaths was one third greater than the number from natural causes. Statistics regarding death and injury from motor vehicles, firearms, consumer products, and poison are presented. PMID:8655752

  20. Childhood cancer and later development of the metabolic syndrome.

    PubMed

    Talvensaari, K; Knip, M

    1997-10-01

    The improving survival rate of patients with childhood cancer has led to a growing awareness of the long-term effects of malignant disease and its treatment. Various endocrine abnormalities have been reported as frequent long-term adverse effects of cancer treatment in childhood, and among these growth hormone (GH) deficiency is the most common one, especially after cranial irradiation. Besides promoting growth, GH has well-established metabolic effects. Patients with GH deficiency tend to be obese, and obesity per se is also associated with insulin resistance which plays a key role in a cluster of metabolic derangements including glucose intolerance, hypertension, lipid abnormalities and atherosclerotic cardiovascular disease. This condition is known as the metabolic syndrome. Our recent observations indicate that a combination of obesity, glucose intolerance, hyperinsulinaemia and an abnormal lipid profile can be observed in long-term survivors of childhood cancer. Every sixth patient had the triad of obesity, hyperinsulinaemia and low HDL cholesterol, whereas this combination was not seen in any of the controls. The survivors with such a high-risk profile for cardiovascular disease had markedly reduced spontaneous GH secretion, and also additional features of the metabolic syndrome, such as higher systolic blood pressure and higher plasma glucose and serum triglyceride levels. Accordingly, decreased GH secretion, or alternatively some other disturbance in the hypothalamic-pituitary axis, emerging as a consequence of cranial radiation, may expose long-term survivors of childhood cancer to premature evolution of the metabolic syndrome. This can have an important impact on the long-term prognosis in these patients, because the syndrome as such results in an increased risk of cardiovascular morbidity and mortality. PMID:9453278

  1. Practitioner Review: Chronic Fatigue Syndrome in Childhood

    ERIC Educational Resources Information Center

    Garralda, M. Elena; Chalder, Trudie

    2005-01-01

    RBackground: Chronic fatigue syndrome (CFS) is being increasingly recognized in children and adolescents. Yet comparatively little attention has been given in the literature to management. Methods: Description of the main features of the disorder, precipitating and maintaining factors and diagnostic assessment. Outline of different views on the…

  2. Early Parental Adjustment and Bereavement after Childhood Cancer Death

    ERIC Educational Resources Information Center

    Barrera, Maru; O'connor, Kathleen; D'Agostino, Norma Mammone; Spencer, Lynlee; Nicholas, David; Jovcevska, Vesna; Tallet, Susan; Schneiderman, Gerald

    2009-01-01

    This study comprehensively explored parental bereavement and adjustment at 6 months post-loss due to childhood cancer. Interviews were conducted with 18 mothers and 13 fathers. Interviews were transcribed verbatim and analyzed based on qualitative methodology. A model describing early parental bereavement and adaptation emerged with 3 domains:…

  3. Preventive Opportunities in Childhood Bereavement. (Death of a Parent Study).

    ERIC Educational Resources Information Center

    Kliman, Gilbert

    This lecture to clergymen presents a discussion of childhood bereavement and possible long-term psychological effects. A correlation between the loss of a parent and later-life mental illness is suggested, as well as the need to look closely at children's unique ways of grieving. The clergyman's role in helping bereaved families is emphasized.…

  4. Mechanisms underlying Phalaris aquatica "sudden death" syndrome in sheep.

    PubMed

    Bourke, C A; Carrigan, M J

    1992-07-01

    Twenty outbreaks of Phalaris aquatica "sudden death" syndrome in sheep were investigated between 1981 and 1991. Four were confirmed and one was suspected, to be a cardiac disorder; 5 were confirmed and 3 were suspected, to be a polioencephalomalacic disorder; the aetiology of the remaining 7 outbreaks could not be determined. Potentially toxic levels of hydrocyanic acid (20 to 36 mg/100 g) were measured in the 3 toxic phalaris pastures tested. The measurement of potentially toxic levels of nitrate nitrogen (2920 micrograms/g) in toxic phalaris pastures by others, was noted. It is suggested that phalaris "sudden death" syndrome could have as many as 4 different underlying mechanisms, and that these might reflect the presence in the plant of a cardio-respiratory toxin, a thiaminase and amine co-substate, cyanogenic compounds, and nitrate compounds. PMID:1445081

  5. [Sudden death in intermittent Wolff Parkinson White syndrome].

    PubMed

    Medeiros, A; Iturralde, P; Guevara, M; Mendoza, C; Colín, L

    2001-01-01

    Sudden death is a rare condition in asymptomatic patients with asymptomatic intermittent Wolff Parkinson syndrome (WPW); for this reason it is believed that these patients should not undergo to radiofrequency ablation. We report an asymptomatic 44 year old man who developed ventricular fibrillation with a pre-excited RR interval less than 200 msec during atrial fibrillation, as a first manifestation of WPW syndrome. The Holter monitoring showed intermittent pre-excitation at low heart rate (70 bpm). During the electrophysiological study a successfully radiofrequency catheter ablation of a right posteroseptal accessory pathway was performed. We concluded that intermittent pre-excitation may not be used to identify patients who are at risk of sudden death. Radiofrequency catheter ablation should be recommended in those patients with a very high success rate, and a low incidence of serious complications. PMID:11565363

  6. Melatonin concentrations in the sudden infant death syndrome

    NASA Technical Reports Server (NTRS)

    Sturner, W. Q.; Lynch, H. J.; Deng, M. H.; Gleason, R. E.; Wurtman, R. J.

    1990-01-01

    The melatonin levels in various body fluids of the sudden infant death syndrome (SIDS) infants are compared with those of infants of comparable age who died of other causes to examine a possible relationship between pineal function and SIDS. After adjusting for age differences, cerebrospinal fluid melatonin levels are found to be significantly lower in the SIDS infants. It is suggested that diminished melatonin production may be characteristic of SIDS and could represent an impairment in the maturation of physiologic circadian organization.

  7. Childhood obesity and the metabolic syndrome in developing countries.

    PubMed

    Gupta, Nidhi; Shah, Priyali; Nayyar, Sugandha; Misra, Anoop

    2013-03-01

    Rapidly changing dietary practices accompanied by an increasingly sedentary lifestyle predispose to nutrition-related non-communicable diseases, including childhood obesity. Over the last 5 y, reports from several developing countries indicate prevalence rates of obesity (inclusive of overweight) >15 % in children and adolescents aged 5-19 y; Mexico 41.8 %, Brazil 22.1 %, India 22.0 % and Argentina 19.3 %. Moreover, secular trends also indicate an alarming increase in obesity in developing countries; in Brazil from 4.1 % to 13.9 % between 1974 and 1997; in China from 6.4 % to 7.7 % between 1991 and 1997; and in India from 4.9 % to 6.6 % between 2003-04 to 2005-06. Other contributory factors to childhood obesity include: high socio-economic status, residence in metropolitan cities and female gender. Childhood obesity tracks into adulthood, thus increasing the risk for conditions like the metabolic syndrome, type 2 diabetes mellitus (T2DM), polycystic ovarian syndrome, hypertension, dyslipidemia and coronary artery disease later in life. Interestingly, prevalence of the metabolic syndrome was 35.2 % among overweight Chinese adolescents. Presence of central obesity (high waist-to-hip circumference ratio) along with hypertriglyceridemia and family history of T2DM increase the odds of T2DM by 112.1 in young Asian Indians (< 40 y). Therapeutic lifestyle changes and maintenance of regular physical activity are most important strategies for preventing childhood obesity. Effective health awareness educational programs for children should be immediately initiated in developing countries, following the successful model program in India (project 'MARG'). PMID:23334584

  8. "Excited delirium syndrome": is it a cause of death?

    PubMed

    Kodikara, Sarathchandra; Cunningham, Kristopher; Pollanen, Michael S

    2012-09-01

    Excited delirium syndrome (EDS) has become a controversial and vexing forensic issue due to its association with restraint and sudden unexpected death. Although some authorities and jurisdictions recognised EDS as a cause of death there is no consensus among the medical community in this regard. The overlapping nature of the spectrum of antemortem behaviours and signs with many natural disease processes complicates this issue further. We describe two deaths which initially presented as EDS-like behaviour during restraint. In the first case, the deceased was travelling on a long distance flight when he died while in the custody of air cabin crew. The autopsy revealed the cause of death as air travel-related pulmonary thromboembolism. Acute alcoholic intoxication, nicotine withdrawal, hypoxia due to acute pulmonary thromboembolism, and hypobaric environment in the air plane cabin appeared as the potential reasons for EDS-like behaviour. In the second case, the deceased died while in the custody of immigration officials. At autopsy the cause of death turned out to be tense pericardial effusion due to fibrinous pericarditis. In this case, hypoperfusion of the brain following systemic hypotension as a result of cardiac tamponade associated with pericardial effusion likely led to the EDS-like behaviour. Clinicopathologic correlation in these two cases would strongly suggest EDS as the cause of death, had the decedents not had fatal anatomical causes of death. This alerts the forensic pathologist that not all the individuals dying with signs and symptoms of EDS during restraint are accounted for EDS as the immediate cause of death. PMID:22622258

  9. Catch-up growth in childhood and death from coronary heart disease: longitudinal study

    PubMed Central

    Eriksson, J G; Forsén, T; Tuomilehto, J; Winter, P D; Osmond, C; Barker, D J P

    1999-01-01

    Objective To examine whether catch-up growth during childhood modifies the increased risk of death from coronary heart disease that is associated with reduced intrauterine growth. Design Follow up study of men whose body size at birth was recorded and who had an average of 10 measurements taken of their height and weight through childhood. Setting Helsinki, Finland. Subjects 3641 men who were born in Helsinki University Central Hospital during 1924-33 and who went to school in Helsinki. Main outcome measures Hazard ratios for death from coronary heart disease. Results Death from coronary heart disease was associated with low birth weight and, more strongly, with a low ponderal index at birth. Men who died from coronary heart disease had an above average body mass index at all ages from 7 to 15 years. In a simultaneous regression the hazard ratio for death from the disease increased by 14% (95% confidence interval 8% to 19%; P<0.0001) for each unit (kg/m3) decrease in ponderal index at birth and by 22% (10% to 36%; P=0.0001) for each unit (kg/m2) increase in body mass index at 11 years of age. Body mass index in childhood was strongly related to maternal body mass index, which in turn was related to coronary heart disease. The extent of crowding in the home during childhood, although related to body mass index in childhood, was not related to later coronary heart disease. Conclusion The highest death rates from coronary heart disease occurred in boys who were thin at birth but whose weight caught up so that they had an average or above average body mass from the age of 7 years. Death from coronary heart disease may be a consequence of poor prenatal nutrition followed by improved postnatal nutrition. Key messagesMen who had low birth weight or were thin at birth have high death rates from coronary heart diseaseDeath rates are even higher if weight “catches up” in early childhoodDeath from coronary heart disease may be a consequence of prenatal undernutrition

  10. A Teenager Revisits Her Father's Death during Childhood: A Study in Resilience and Healthy Mourning

    ERIC Educational Resources Information Center

    Hurd, Russell C.

    2004-01-01

    "Debbie," 14, was 8 when her father died. During 4 interviews over 3 months, Debbie described the impact of his death as she progressed from childhood to adolescence. Themes drawn from her experience were related to theories of development, bereavement, and resilience. Triangulating interviews with her mother and brother established validity.…

  11. Violent death in a rare peroxisomal disease--Zellweger syndrome.

    PubMed

    Malinescu, Bogdan; Martius, Eliza; Pelin, Ana Maria

    2015-10-01

    Peroxisomal diseases are rare (1:50,000), genetically determined disorders (autosomal recessive), systemic, multiorgan illnesses with prominent involvement of the nervous system, caused either by the failure to form or to maintain the peroxisome, or by a defect in the function of a single or multiple peroxisomal enzymes. Peroxisomes contain approximately 50 enzymes which are responsible for many metabolic reactions, and play an important role in the oxidation of saturated very-long-chain fatty acids (VLCFA). The authors present the case of a Romanian boy, who died at the age of 1.6 of one of the peroxisomal diseases-Zellweger syndrome. Newborn infants with Zellweger syndrome have a typical dysmorphic facies, neonatal seizures, profound hypotonia, and eye abnormalities. Major abnormalities are present in the liver (fibrotic), kidney (cortical cysts), and brain (lipid-laden macrophages and histiocytes in cortical and periventricular areas, demyelination, centrosylvian polymicrogyria and pachygyria)-cerebro-hepato-renal syndrome (CHRS) (Zellweger). Infants with Zellweger syndrome rarely live more than a few months, but in this case the survival was longer, and the cause of death was not directly the peroxisomal disease but a violent cause of death-mechanical asphyxia with tracheo-bronchial food aspiration. The authors present the results of investigations carried out during the child's life, but also data collected at the autopsy and hystopathological postnecroptic investigations. By presenting this case, the authors wish to bring to your attention a rare pathology in forensic practice by the paradox of finding a common violent cause of death, asphyxia with food aspiration, in a rare metabolic-genetic disease, which is usually fatal by itself. PMID:26235911

  12. Bed sharing and the sudden infant death syndrome.

    PubMed Central

    Klonoff-Cohen, H.; Edelstein, S. L.

    1995-01-01

    OBJECTIVE--To determine whether infants who died of the sudden infant death syndrome routinely shared their parents' bed more commonly than control infants. DESIGN--Case-control study. SETTING--Southern California. SUBJECTS--200 white, African-American, Latin American, and Asian infants who died and 200 living controls, matched by birth hospital, date of birth, sex, and race. MAIN OUTCOME MEASURES--Routine bedding (for example, crib, cradle), day and night time sleeping arrangement (for example, alone or sharing a bed); for cases only, sleeping arrangement at death. Differences in bed sharing practices among races. RESULTS--Of the infants who died of the syndrome, 45 (22.4%) were sharing a bed. Daytime bed sharing was more common in African-American (P < 0.001) and Latin American families (P < 0.001) than in white families. The overall adjusted odds ratio for the syndrome and routine bed sharing in the daytime was 1.38 (95% confidence interval 0.59 to 3.22) and for night was 1.21 (0.59 to 2.48). These odds ratios were adjusted for routine sleep position, passive smoking, breast feeding, intercom use, infant birth weight, medical conditions at birth, and maternal age and education. There was no interaction between bed sharing and passive smoking or alcohol use by either parent. CONCLUSIONS--Although there was a significant difference between bed sharing among African-American and Latin American parents compared with white parents, there was no significant relation between routine bed sharing and the sudden infant death syndrome. PMID:7496236

  13. Childhood trauma and metabolic syndrome in men and women.

    PubMed

    Lee, Chioun; Tsenkova, Vera; Carr, Deborah

    2014-03-01

    The long-term effects of childhood trauma on health are well-documented, but few population-based studies have explored how childhood trauma affects the risk of developing metabolic syndrome (MetS) in adulthood. Using data from 1234 adults in the second wave of Midlife in the United States (MIDUS), we investigate (1) the extent to which childhood abuse affects the risk of developing MetS in adulthood; (2) how the severity of different types of abuse (emotional, physical, sexual, or cumulative abuse) affects this risk; and (3) the extent to which adult socioeconomic status (SES), maladaptive stress responses, and unhealthy behaviors mediate the association. We also test whether these associations differ significantly by sex. We find that emotional and physical abuse increase the risk of developing MetS for both sexes, whereas sexual abuse is a predictor for women only. For both sexes, individuals who experienced more cumulative abuse have a greater risk of developing MetS. Adult SES partially explains the association between childhood abuse and MetS. Maladaptive stress responses and unhealthy behaviors further explain the association. Among the potential mediators, poor sleep quality was a significant pathway for men and women, while stress-induced eating was a significant pathway for women only. Our findings suggest that the well-documented health consequences of early life trauma may vary by the nature of the trauma, the victim's sex, and the coping mechanisms that he or she employs. PMID:24524907

  14. Perspectives on edema in childhood nephrotic syndrome.

    PubMed

    Teoh, Chia Wei; Robinson, Lisa A; Noone, Damien

    2015-10-01

    There have been two major theories surrounding the development of edema in nephrotic syndrome (NS), namely, the under- and overfill hypotheses. Edema is one of the cardinal features of NS and remains one of the principal reasons for admission of children to the hospital. Recently, the discovery that proteases in the glomerular filtrate of patients with NS are activating the epithelial sodium channel (ENaC), resulting in intrarenal salt retention and thereby contributing to edema, might suggest that targeting ENaC with amiloride might be a suitable strategy to manage the edema of NS. Other potential agents, particularly urearetics and aquaretics, might also prove useful in NS. Recent evidence also suggests that there may be other areas involved in salt storage, especially the skin, and it will be intriguing to study the implications of this in NS. PMID:26290369

  15. Cholestatic syndromes in infancy and childhood

    SciTech Connect

    Gates, G.F.; Sinatra, F.R.; Thomas, D.W.

    1980-06-01

    Of 33 children with various cholestatic syndromes who were studied with sonography, 19 also had /sup 131/I rose bengal scintigraphy, and 12 also had /sup 99m/Tc sulfur colloid scintigraphy. Patients were categorized into intra- or extrahepatic etiologies for their cholestasis. Of 19 children in the extrahepatic category, 17 had abnormal sonographic studies; two with biliary atresia appeared normal. All 14 patients in the intrahepatic category biliary patency. Some cases of biliary atresia with normal sonography and lack of rose bengal excretion into the intestinal tract could not be separated from cases of neonatal hepatitis using a similar combination of studies. Radiocolloid studies were neonatal hepatitis using a similar combination of studies. Radiocolloid studies were less valuable than other examinations except when demonstrating diffuse hepatic reticuloendothelial dysfunction as found in two cases of congenital syphilitic hepatitis.

  16. Parental Death in Childhood and Loneliness in Young Adults.

    ERIC Educational Resources Information Center

    Murphy, Patricia Ann

    1987-01-01

    Examined relationship between self-esteem and reported mourning behavior as it pertains to loneliness in young adults who, as children, had experienced parental death. Subjects (N=184) aged 18 to 25 completed four questionnaires. Revealed that self-esteem was the single best predictor of loneliness; reported mourning behaviors significantly added…

  17. Unusual cause of childhood anemia: Imerslund Grasbeck syndrome.

    PubMed

    Laxminarayana, Kishan Prasad Hosapatna; Yeshvanth, Sunil Kumar; Shetty, Jayaprakash K; Permi, Harish S; Rao, Chandrika

    2011-07-01

    Imerslund Grasbeck syndrome (IGS) is a rare autosomal recessive childhood disorder characterized by selective Vitamin (vit) B 12 malabsorption with asymptomatic proteinuria without any structural renal pathology. The patients stay healthy for decades with life-long parenteral vit B12. We report a case of young female who presented with pancytopenia and proteinuria, evaluated in local hospitals as chronic hemolytic anemia (autoimmune cause), finally diagnosed as IGS on complete evaluation. She was treated with injectable vit B12 (1000 μg cyanocobalalmin) and showed drastic recovery. IGS should be considered in patients with megaloblastic anemia not responding to oral vit B12 and associated proteinuria. PMID:22219566

  18. Rare cause of natural death in forensic setting: hemophagocytic syndrome.

    PubMed

    Ondruschka, B; Habeck, J-O; Hädrich, C; Dreßler, J; Bayer, R

    2016-05-01

    We report about the case of a sudden unexpected death of a 25-year-old male suffering from infectious disease. An autopsy was ordered with no final premortem diagnosis. Microscopic and microbiological examination revealed a pneumococcal bronchopneumonia and hemophagocytic lesions in the bone marrow. After integrating clinical and autopsy reports as well as additional postmortem investigations, the cause of death was found to be infectious-triggered hemophagocytic syndrome (HPS) with a final cytokine storm. This seems to be the first reported fatal case of a reactive form of HPS associated to Streptococcus pneumoniae to the best of our knowledge. HPS is a dangerous hyperinflammation with highly characteristic, but nonspecific, laboratory findings and symptoms. Autopsies in such cases must be carefully performed and include systematic tissue sampling done by an experienced pathologist. PMID:26718840

  19. A possible explanation of sudden infant death syndrome (SIDS).

    PubMed

    Christos, G A; Christos, J A

    1993-09-01

    Research into (lucid) dreaming has shown that the images of a dream are supported by the corresponding body actions, utilizing those muscles which remain active during dreaming. We suggest that Sudden Infant Death Syndrome (SIDS) or Cot Death may be a result of an infant dreaming about its life as a fetus. In the course of that dream, since a fetus does not breathe in the usual sense, the infant may cease to breathe and die. Our hypothesis is consistent with the known facts about SIDS, including social factors such as sleeping position and climatic variation. We suggest that the risk of SIDS can be reduced by making the environment of the infant, as much as possible, unlike that of the womb. PMID:8259083

  20. Seasonal relationship of sudden infant death syndrome and environmental pollutants

    SciTech Connect

    Hoppenbrouwers, T.; Calub, M.; Arakawa, K.; Hodgman, J.E.

    1981-06-01

    Evidence that chronic hypoxia precedes death from sudden infant death syndrome (SIDS) is mounting. Prolonged exposure to moderate levels of pollutants could be a contributing factor to hypoxia. Levels of carbon monoxide (CO), sulfur dioxide (SO/sub 2/), nitrogen dioxide (NO/sub 2/) and hydrocarbons (HC) are highest in the winter when incidence of SIDS is increased. SIDS cases in Los Angeles County were correlated with daily mean levels of these pollutants, temperature, barometric pressure and monthly lead levels with the aid of time series analyses. Peaks in CO, SO/sub 2/, NO/sub 2/, HC and lead preceded the seasonal increase in SIDS by seven weeks. Theoretical considerations, such as the hypoxia-inducing effects of CO, support the hypothesis that this temporal relation has functional significance. The role of pollution levels as a predisposing factor in risk for SIDS cannot be summarily dismissed.

  1. Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment.

    PubMed

    Blaes, Franz; Dharmalingam, Backialakshmi

    2016-06-01

    Opsoclonus-myoclonus syndrome (OMS) is a rare and primarily immune-mediated disease in children and adults. The main symptoms include opsoclonus, myoclonus and ataxia. In children, the symptoms also include irritability, and, over a long-term course, learning and behavioural disturbances. OMS can be idiopathic, parainfectious or occur as a paraneoplastic (tumour-associated) syndrome. Paraneoplastic OMS in children is almost exclusively associated with neuroblastoma, whereas in adults, small cell lung cancer and breast cancer are the main underlying tumours. An autoimmune pathophysiology is suspected because childhood OMS patients have functionally active autoantibodies, proinflammatory changes in the cytokine network and immunotherapy responses. Children appear to respond regularly to immunosuppressive treatment. However, although the neurological symptoms show a good response, most children continue to show neuropsychological disturbances. PMID:27095464

  2. Childhood trauma and metabolic syndrome in men and women

    PubMed Central

    Lee, Chioun; Tsenkova, Vera; Carr, Deborah

    2014-01-01

    The long-term effects of childhood trauma on health are well-documented, but few population-based studies have explored how childhood trauma affects the risk of developing metabolic syndrome (MetS) in adulthood. Using data from 1,234 adults in the second wave of the Midlife Development in the U.S. survey (2004), we investigate (1) the extent to which childhood abuse affects the risk of developing MetS in adulthood; (2) how the severity of different types of abuse (emotional, physical, sexual, or cumulative abuse) affects this risk; and (3) the extent to which adult socioeconomic status (SES), maladaptive stress responses, and unhealthy behaviors mediate the association. We also test whether these associations differ significantly by sex. We find that emotional and physical abuse increase the risk of developing MetS for both sexes, whereas sexual abuse is a predictor for women only. For both sexes, individuals who experienced more cumulative abuse have a greater risk of developing MetS. Adult SES partially explains the association between childhood abuse and MetS. Maladaptive stress responses and unhealthy behaviors further explain the association. Among the potential mediators, poor sleep quality was a significant pathway for men and women, while stress-induced eating was a significant pathway for women only. Our findings suggest that the well-documented health consequences of early life trauma may vary by the nature of the trauma, the victim’s sex, and the coping mechanisms that he or she employs. PMID:24524907

  3. Causes of accidental childhood deaths in China in 2010: A systematic review and analysis

    PubMed Central

    Chan, Kit Yee; Yu, Xin–wei; Lu, Jia–peng; Demaio, Alessandro Rhyll; Bowman, Kirsty; Theodoratou, Evropi

    2015-01-01

    Background Infectious causes of childhood deaths in the world have decreased substantially in the 21st century. This trend has exposed accidental deaths as an increasingly important future challenge. Presently, little is known about the cause structure of accidental childhood deaths in low– and middle–income country (LMIC) settings. In this paper, we aim to establish cause structure for accidental deaths in children aged 0–4 years in China in the year 2010. Methods In this paper, we explored the database of 208 multi–cause child mortality studies in Chinese that formed a basis for the first published estimate of the causes of child deaths in China (for the year 2008). Only five of those studies identified specific causes of accidental deaths. Because of this, we searched the Chinese medical literature databases CNKI and WanFang for single–cause mortality studies that were focused on accidental deaths. We identified 71 further studies that provided specific causes for accidental deaths. We used epidemiological modeling to estimate the number of accidental child deaths in China in 2010 and to assign those deaths to specific causes. Results In 2010, we estimated 314 581 deaths in children 0–4 years in China, of which 31 633 (10.1%) were accidental. Accidental deaths contributed 7240 (4.0%) of all deaths in neonatal period, 8838 (10.5%) among all post–neonatal infant deaths, and 15 554 (31.7%) among children with 1–4 years of age. Among four tested models, the most predictive was used to establish the likely cause structure of accidental deaths in China. We estimated that asphyxia caused 9490 (95% confidence interval (CI) 8224–11 072), drowning 5694 (95% CI 5061–6327), traffic accidents 3796 (95% CI 3163–4745), poisoning 3163 (95% CI 2531–3796) and falls 2531 (95% CI 2214–3163) deaths. Based on medians from a few rare studies, we also predict 633 (95% CI 316–1265) deaths to be due to burns and 316 (95% CI 0–633) due to falling

  4. [Sudden death in the Wolff-Parkinson-White syndrome].

    PubMed

    García-Cosío Mir, F

    1989-04-01

    Clinical electrophysiologic studies in patients with Wolff-Parkinson-White syndrome (WPW) suffering from ventricular fibrillation have shown a high prevalence of short anterograde refractory period of the accessory pathway (less than or equal to 250 ms), short preexcited RR intervals during atrial fibrillation (less than or equal to 250 ms), and multiple accessory pathways. Unfortunately the specificity of these findings is low, as they are present in almost 50% of patients with WPW without a history of ventricular fibrillation, and in 17% of patients with asymptomatic WPW. Pharmacologic and exercise testing detect a population of WPW with a low probability of having a short anterograde refractory period of the accessory pathway, but don't rule-out the ability of these patients to develop very short RR intervals during atrial fibrillation. Natural history studies show that sudden death in WPW occurs with an incidence less than or equal to 1:1,000 per year. The low predictive value of electrophysiologic and noninvasive studies for sudden death, makes then a poor means for screening patients at risk. Some clinical factors, such as the frequency of tachycardias and/or the detection of episodes of atrial flutter or fibrillation are markers of higher sudden death risk, and indications for aggressive electrophysiologic evaluation. PMID:2675223

  5. Rituximab for troublesome cases of childhood nephrotic syndrome

    PubMed Central

    Safdar, Osama Y; Aboualhameael, Adila; Kari, Jameela A

    2014-01-01

    Nephrotic syndrome (NS) is the most common glomerular disease of childhood. Steroid-dependent and steroid-resistant nephrotic syndrome present challenges in their pharmaceutical management; patients may need several immunosuppressive medication for optimum control, each of which medication has its own safety profile. Rituximab (RTX) is a monoclonal antibody that targets B cells and has been used successfully for management of lymphoma and rheumatoid arthritis. Recent clinical studies showed that rituximab may be an efficacious and safe alternative for the treatment of complicated nephrotic syndrome. In this review article, we aim to review the efficacy and safety of RTX therapy in nephrotic syndrome. We reviewed the literature pertaining to this topic by searching for relevant studies on PubMed and Medline using specific keywords. The initial search yielded 452 articles. These articles were then examined to ensure their relevance to the topic of research. We focused on multicenter randomized controlled trials with relatively large numbers of patients. A total of 29 articles were finally identified and will be summarized in this review. The majority of clinical studies of RTX in complicated pediatric NS showed that rituximab is effective in approximately 80% of patients with steroid-dependent NS, as it decreases the number of relapses and steroid dosage. However, RTX is less effective at achieving remission in steroid-resistant NS. RTX use was generally safe, and most side effects were transient and infusion-related. More randomized, double-blinded clinical studies are needed to assess the role of RTX in children with nephrotic syndrome. PMID:25512892

  6. Melatonin concentrations in the sudden infant death syndrome

    NASA Technical Reports Server (NTRS)

    Sturner, W. Q.; Lynch, H. J.; Deng, M. H.; Gleason, R. E.; Wurtman, R. J.

    1990-01-01

    To examine a possible relationship between pineal function and the sudden infant death syndrome (SIDS), samples of whole blood, ventricular cerebrospinal fluid (CSF) and/or vitreous humor (VH) were obtained at autopsy from 68 infants (45 male, 23 female) whose deaths were attributed to either SIDS (n = 32, 0.5-5.0 months of age; mean plus or minus S.E.M., 2.6 plus or minus 0.2 months) or other causes (non-SIDS, n = 36, 0.3-8.0 months of age 4.3 plus or minus 0.3 months). The melatonin concentrations were measured by radioimmunoassay. A significant correlation was observed for melatonin levels in different body fluids from the same individual. After adjusting for age differences, CSF melatonin levels were significantly lower among the SIDS infants (91 plus or minus 29 pmol/l; n = 32) than among those dying from other causes (180 plus or minus 27; n = 35, P less than 0.05). A similar, but non-significant trend was also noted in blood (97 plus or minus 23, n = 30 vs. 144 plus or minus 22 pmol/l, n = 33) and vitreous humor (68 plus or minus 21, n = 10 vs. 81 plus or minus 17 pmol/l, n = 15). These differences do not appear to be explainable in terms of the interval between death and autopsy, gender, premortem infection, or therapeutic measures instituted prior to death. Diminished melatonin production may be characteristic of SIDS and could represent an impairment in the maturation of physiologic circadian organization.

  7. Risk Factor Changes for Sudden Infant Death Syndrome After Initiation of Back-to-Sleep Campaign

    PubMed Central

    Trachtenberg, Felicia L.; Haas, Elisabeth A.; Kinney, Hannah C.; Stanley, Christina

    2012-01-01

    OBJECTIVE: To test the hypothesis that the profile of sudden infant death syndrome (SIDS) changed after the Back-to-Sleep (BTS) campaign initiation, document prevalence and patterns of multiple risks, and determine the age profile of risk factors. METHODS: The San Diego SIDS/Sudden Unexplained Death in Childhood Research Project recorded risk factors for 568 SIDS deaths from 1991 to 2008 based upon standardized death scene investigations and autopsies. Risks were divided into intrinsic (eg, male gender) and extrinsic (eg, prone sleep). RESULTS: Between 1991–1993 and 1996–2008, the percentage of SIDS infants found prone decreased from 84.0% to 48.5% (P < .001), bed-sharing increased from 19.2% to 37.9% (P < .001), especially among infants <2 months (29.0% vs 63.8%), prematurity rate increased from 20.0% to 29.0% (P = .05), whereas symptoms of upper respiratory tract infection decreased from 46.6% to 24.8% (P < .001). Ninety-nine percent of SIDS infants had at least 1 risk factor, 57% had at least 2 extrinsic and 1 intrinsic risk factor, and only 5% had no extrinsic risk. The average number of risks per SIDS infant did not change after initiation of the BTS campaign. CONCLUSIONS: SIDS infants in the BTS era show more variation in risk factors. There was a consistently high prevalence of both intrinsic and especially extrinsic risks both before and during the Back-to-Sleep era. Risk reduction campaigns emphasizing the importance of avoiding multiple and simultaneous SIDS risks are essential to prevent SIDS, including among infants who may already be vulnerable. PMID:22451703

  8. Cardiac and other abnormalities in the sudden infant death syndrome.

    PubMed

    Naeye, R L; Whalen, P; Ryser, M; Fisher, R

    1976-01-01

    Many victims of the sudden infant death syndrome (SIDS) have abnormally heavy cardiac right ventricles. The degree of this abnormality is directly proportional to: a) the mass of muscle about small pulmonary arteries, b) the amount of brown fat retention about adrenal glands, and c) the presence of hepatic erythropoiesis. The pulmonary arterial abnormality is probably the result of chronic alveolar hypoventilation, while brown fat retention and hepatic erythropoiesis are likely consequences of chronic hypoxemia. These abnormalities are found in both SIDS victims who die with and those who die without mild respiratory tract infections. However, there are some differences between the two SIDS groups. Infected victims die at an older age and have smaller thymus glands and larger spleens; there is a greater proportion of males in the infected victims than in the noninfected victims. PMID:1247080

  9. Sports and Marfan Syndrome: Awareness and Early Diagnosis Can Prevent Sudden Death.

    ERIC Educational Resources Information Center

    Salim, Mubadda A.; Alpert, Bruce S.

    2001-01-01

    Physicians who work with athletes play an important role in preventing sudden death related to physical activity in people who have Marfan syndrome. Flagging those who have the physical stigmata and listening for certain cardiac auscultation sounds are early diagnostic keys that can help prevent deaths. People with Marfan syndrome should be…

  10. Sudden unexplained death syndrome--a new manifestation in melioidosis?

    PubMed Central

    Yap, E. H.; Chan, Y. C.; Goh, K. T.; Chao, T. C.; Heng, B. H.; Thong, T. W.; Tan, H. C.; Thong, K. T.; Jacob, E.; Singh, M.

    1991-01-01

    The indirect haemagglutination (IHA) test using sensitized turkey erythrocytes and the indirect immunofluorescence assay (IgM-IFA) was confirmed to be sensitive in the detection of a recent or current Pseudomonas pseudomallei infection in 19 culture-confirmed Singapore melioidosis patients. All were found to have antibody titres from 4 to 32768 in the IHA test and 10 to 320 in the IgM-IFA test. When these tests were employed on sera from 16 immigrant Thai construction workers who died of sudden unexplained death syndrome (SUDS) and 73 healthy Thai fellow workers, 93.8% and 68.8% of SUDS cases had IHA titre of greater than or equal to 4 and IgM-IFA titre of greater than or equal to 10 respectively, in contrast to 39.7% and 12.3% found among healthy Thai workers. These data indicate that at the time of death, most of the SUDS patients had an active infection with P. pseudomallei, possibly resulting from reactivation of a latent infection. The aetiological role of P. pseudomallei as the major cause of SUDS is discussed. PMID:1721589

  11. Sudden infant death syndrome: review for the obstetric care provider.

    PubMed

    Van Nguyen, Julie My; Abenhaim, Haim A

    2013-10-01

    Sudden infant death syndrome (SIDS) is the leading cause of death among infants aged 1 to 12 months. In this article, we review risk factors that may predispose infants to increased vulnerability. Maternal characteristics, including nonmodifiable and modifiable factors, antenatal medical conditions, labor and delivery events, and infant characteristics, are reviewed, with the purpose of helping obstetric care providers target risk reduction efforts. We have reviewed over 85 case-control, retrospective, and prospective cohort studies published between 1975 and 2011. Major modifiable risk factors include maternal and paternal smoking, drug use, alcohol use, and insufficient prenatal care. Infants at increased risk include males, premature infants, infants of low birth weight or growth-restricted infants, and infants in multiple gestations. By targeting modifiable and nonmodifiable risk factors, it may be possible to decrease the incidence of SIDS. Efforts should be put on decreasing high-risk behaviors and encouraging sufficient antenatal follow-up. In view of recent increases in ethnic and social disparity with SIDS, it is essential that risk reduction guidelines, which have recently been expanded by the American Association of Pediatrics, be explained in a culturally sensitive manner. PMID:23292938

  12. Causes of childhood deaths in Bangladesh: results of a nationwide verbal autopsy study.

    PubMed Central

    Baqui, A. H.; Black, R. E.; Arifeen, S. E.; Hill, K.; Mitra, S. N.; al Sabir, A.

    1998-01-01

    While knowledge of causes of deaths is important for health sector planning, little is known from conventional sources about the causes of deaths in Bangladesh. This is partly due to deficiencies in the registration system and partly because few deaths are attended by qualified physicians. The present study was undertaken to update the information available on causes of deaths among under-5-year-olds, taking advantage of advances in verbal autopsy methodology and of the national Bangladesh Demographic and Health Survey conducted in 1993-94. About 25% of the deaths were associated with acute lower respiratory infections (ALRI) and about 20% with diarrhoea. Neonatal tetanus and measles remained important causes of death, and drowning was a major cause for 1-4-year-olds. Research and programmes to enable mothers to identify ALRI cases, particularly pneumonia, and to encourage timely and appropriate care-seeking and strengthening of ALRI case management at the primary care facilities are important priorities. While promotion of oral rehydration for watery diarrhoea and antibiotic treatment for dysentery should continue, broader preventive interventions including provision of safe water and sanitation, and improvements in personal hygiene require more attention. Further intensification of immunization programmes and innovative experimental interventions to reduce childhood from drowning should be designed and tested. PMID:9648357

  13. Teaching Child Care Providers to Reduce the Risk of SIDS (Sudden Infant Death Syndrome)

    ERIC Educational Resources Information Center

    Byington, Teresa; Martin, Sally; Reilly, Jackie; Weigel, Dan

    2011-01-01

    Keeping children safe and healthy is one of the main concerns of parents and child care providers. SIDS (Sudden Infant Death Syndrome) is the leading cause of death in infants 1 month to 12 months of age. Over 2,000 infants die from SIDS every year in the United States, and almost 15% of these deaths occur in child care settings. A targeted…

  14. The cost of inpatient death associated with acute coronary syndrome

    PubMed Central

    Page, Robert L; Ghushchyan, Vahram; Van Den Bos, Jill; Gray, Travis J; Hoetzer, Greta L; Bhandary, Durgesh; Nair, Kavita V

    2016-01-01

    Background No studies have addressed the cost of inpatient mortality during an acute coronary syndrome (ACS) admission. Objective Compare ACS-related length of stay (LOS), total admission cost, and total admission cost by day of discharge/death for patients who died during an inpatient admission with a matched cohort discharged alive following an ACS-related inpatient stay. Methods Medical and pharmacy claims (2009–2012) were used to identify admissions with a primary diagnosis of ACS from patients with at least 6 months of continuous enrollment prior to an ACS admission. Patients who died during their ACS admission (deceased cohort) were matched (one-to-one) to those who survived (survived cohort) on age, sex, year of admission, Chronic Condition Index score, and prior revascularization. Mean LOS, total admission cost, and total admission cost by the day of discharge/death for the deceased cohort were compared with the survived cohort. A generalized linear model with log transformation was used to estimate the differences in the total expected incremental cost of an ACS admission and by the day of discharge/death between cohorts. A negative binomial model was used to estimate differences in the LOS between the two cohorts. Costs were inflated to 2013 dollars. Results A total of 1,320 ACS claims from patients who died (n=1,320) were identified and matched to 1,319 claims from the survived patients (n=1,319). The majority were men (68%) and mean age was 56.7±6.4 years. The LOS per claim for the deceased cohort was 47% higher (adjusted incidence rate ratio: 1.47, 95% confidence interval: 1.37–1.57) compared with claims from the survived cohort. Compared with the survived cohort, the adjusted mean incremental total cost of ACS admission claims from the deceased cohort was US$43,107±US$3,927 (95% confidence interval: US$35,411–US$50,803) higher. Conclusion Despite decreasing ACS hospitalizations, the economic burden of inpatient death remains high. PMID

  15. The Effects of Childhood Maltreatment on Violent Injuries and Premature Death During Young Adulthood Among Urban High-Risk Men

    PubMed Central

    Lee, Chioun; White, Helene R.

    2013-01-01

    Objective To assess childhood maltreatment as a risk factor for violent injuries and premature death in young adulthood and whether these associations are mediated by adolescent heavy drinking, hard drug use, hard drug selling, and violent offending. Design A prospective longitudinal study of boys followed from childhood into young adulthood. Setting Pittsburgh, Pennsylvania Participants A total 1,009 men of the Pittsburgh Youth Study Main Outcome Measures Premature deaths between ages 18 and 38 from the Social Security Death Index and self-reports of violent injuries inflicted by gunshot or knife between ages 18 and 28. Results Young men who experienced childhood maltreatment, compared to their counterparts who did not experience it, had a greater risk of violent injuries (relative risk [RR], 1.61; 95% confidence interval [CI], 1.01–2.35) and death (hazard ratio, [HR], 2.85; 95% CI, 1.37–5.93) during young adulthood. Adolescent violent offending and hard drug selling explained the association between childhood maltreatment and violent injuries, and violent offending partially accounted for the association between childhood maltreatment and premature death. Although adolescent violent offending predicted both outcomes, maltreated boys still had an increased risk of premature death (HR, 2.54; 95% CI, 1.21–5.34) after accounting for their adolescent violence. Conclusions Childhood maltreatment significantly predicts premature death and violent injuries during young adulthood. These associations are partially explained by adolescent involvement in violence and drug dealing. Targeted interventions for maltreated boys to reduce their involvement in adolescent deviant behaviors may help decrease their risks for later serious injuries and premature death. PMID:22566518

  16. Child abuse, sudden infant death syndrome, infectious disease, and vaccinations.

    PubMed

    Walker, A; Chernoff, R; Joffe, A; Wilson, M E

    1994-04-01

    Clinicians all too often face the difficult dilemma of deciding whether a bone fracture in a young child was intentional. A structured expert consensus process suggests that all rib fractures; midshaft or metaphyseal fractures of the humerus; and fractures of the radius, ulna, tibia, or fibula in children younger than 1 year of age are highly likely to have been caused by abuse. Abused children are more likely to have negative social relationships with other children than their school-aged peers. Research on the causes of sudden infant death syndrome is still confounded by the likelihood that some deaths for which the label was misapplied are included in many studies; however, the presence of smokers in the household in the postnatal period appears to be yet another factor associated with increased risk. Congenital syphilis is on the rise. Detection of infants who have been infected is incomplete. Lack of prenatal care is strongly associated with infection. Cord serology is not sensitive enough to detect all possible cases. Testing of both maternal and neonatal sera results in detection of more infants at risk. Current tests still result in the treatment of some infants who are not themselves infected. Amoxicillin clavulante given twice daily rather then thrice, cefixime given once a day, and a single intramuscular dose of ceftriaxone have all shown acceptable--though not exceptional--cure rates for otitis media. Price is a consideration. Fewer courses of antibiotics would be necessary if more infants were breastfed. Exclusively breastfeeding to at least 4 months cuts the number of bouts of otitis media almost in half.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8032405

  17. Sudden infant death syndrome and abnormal metabolism of thiamin.

    PubMed

    Lonsdale, Derrick

    2015-12-01

    Although it has been generally accepted that moving the infant from the prone to the supine position has solved the problem of sudden infant death syndrome (SIDS), it has been hypothesized that this is an insufficient explanation and that a mixture of genetic risk, some form of stressful incident and marginal brain metabolism is proportionately required. It is suggested that each of these three variables, with dominance in one or more of them, act together in the common etiology. Much has been written about the association of thiamin and magnesium but the finding of extremely high concentrations of serum thiamin in SIDs victims has largely caused rejection of thiamin as being involved in the etiology. The publication of abnormal brainstem auditory evoked potentials strongly suggests that there are electrochemical changes in the brainstem affecting the mechanisms of automatic breathing and the control of cardiac rhythm. The brainstem, cerebellum and limbic system of the brain are known to be highly sensitive to thiamin deficiency (pseudo-hypoxia) and the pathophysiology is similar to a mild continued deprivation of oxygen. Little attention has been paid to the complex metabolism of thiamin. Dietary thiamin requires the cooperation of the SLC19 family of thiamin transporters for its absorption into cells and recent information has shown that transporter SNPs may be relatively common and can be expected to increase genetic risk. Thiamin must be phosphorylated to synthesize thiamin pyrophosphate (TPP), well established in its vital action in glucose metabolism. TPP is also a cofactor for the enzyme 2-hydroxyacyl-CoA lyase (HACL1) in the peroxisome, emphasizing its importance in alpha oxidation and plasmalogen synthesis in cell membrane physiology. The importance of thiamine triphosphate (TTP) in energy metabolism is still largely unknown. Thiamin metabolism has been implicated in hyperemesis gravidarum and iatrogenic Wernicke encephalopathy has been reported when the

  18. Impairment and Distress Judgments of Symptoms Composing Childhood Externalizing and Internalizing Syndromes

    ERIC Educational Resources Information Center

    Howell, Andrew J.; Watson, David C.

    2009-01-01

    The pattern of perceived dysfunction associated with symptoms composing the externalizing childhood disorder syndrome was compared to the pattern characterizing the internalizing syndrome. In Study 1, undergraduate students (N = 205) judged the social impairment, academic/occupational impairment and personal distress associated with symptoms from…

  19. The triple risk hypotheses in sudden infant death syndrome.

    PubMed

    Guntheroth, Warren G; Spiers, Philip S

    2002-11-01

    Sudden infant death syndrome (SIDS) victims were regarded as normal as a matter of definition (Beckwith 1970) until 1952 when Kinney and colleagues argued for elimination of the clause, "unexpected by history." They argued that "not all SIDS victims were normal," and referred to their hypothesis that SIDS results from brain abnormalities, which they postulated "to originate in utero and lead to sudden death during a vulnerable postnatal period." Bergman (1970) argued that SIDS did not depend on any "single characteristic that ordains a infant for death," but on an interaction of risk factors with variable probabilities. Wedgwood (1972) agreed and grouped risk factors into the first "triple risk hypothesis" consisting of general vulnerability, age-specific risks, and precipitating factors. Raring (1975), based on a bell-shaped curve of age of death (log-transformed), concluded that SIDS was a random process with multifactorial causation. Rognum and Saugstad (1993) developed a "fatal triangle" in 1993, with groupings similar to those of Wedgwood, but included mucosal immunity under a vulnerable developmental stage of the infant. Filiano and Kinney (1994) presented the best known triple risk hypothesis and emphasized prenatal injury of the brainstem. They added a qualifier, "in at least a subset of SIDS," but, the National Institute of Child Health and Development SIDS Strategic Plan 2000, quoting Kinney's work, states unequivocally that "SIDS is a developmental disorder. Its origins are during fetal development." Except for the emphasis on prenatal origin, all 3 triple risk hypotheses are similar. Interest in the brainstem of SIDS victims began with Naeye's 1976 report of astrogliosis in 50% of all victims. He concluded that these changes were caused by hypoxia and were not the cause of SIDS. He noted an absence of astrogliosis in some older SIDS victims, compatible with a single, terminal episode of hypoxia without previous hypoxic episodes, prenatal or postnatal

  20. Early repolarization syndrome: A cause of sudden cardiac death

    PubMed Central

    Ali, Abdi; Butt, Nida; Sheikh, Azeem S

    2015-01-01

    Early repolarization syndrome (ERS), demonstrated as J-point elevation on an electrocardiograph, was formerly thought to be a benign entity, but the recent studies have demonstrated that it can be linked to a considerable risk of life - threatening arrhythmias and sudden cardiac death (SCD). Early repolarization characteristics associated with SCD include high - amplitude J-point elevation, horizontal and/or downslopping ST segments, and inferior and/or lateral leads location. The prevalence of ERS varies between 3% and 24%, depending on age, sex and J-point elevation (0.05 mV vs 0.1 mV) being the main determinants. ERS patients are sporadic and they are at a higher risk of having recurrent cardiac events. Implantable cardioverter-defibrillator implantation and isoproterenol are the suggested therapies in this set of patients. On the other hand, asymptomatic patients with ERS are common and have a better prognosis. The risk stratification in asymptomatic patients with ERS still remains a grey area. This review provides an outline of the up-to-date evidence associated with ERS and the risk of life - threatening arrhythmias. Further prospective studies are required to elucidate the mechanisms of ventricular arrhythmogenesis in patients with ERS. PMID:26322186

  1. Sudden infant death syndrome (SIDS) and the immune response.

    PubMed

    Reid, G M

    1992-10-01

    Vitamin E pretreatment significantly prevented E. coli-induced Disseminated Intravascular Coagulation (DIC) in rats (1). DIC, a reduction in fibrinogen and a falling platelet count and diffuse haemorrhage are part of the clinical features of Haemorrhagic Shock Encephalopathy Syndrome (HSES), recognised as a disease entity in the 1980s (2). At the SIDS Conference 1974 Reisinger described the effect of Escherichia coli (E. coli) endotoxin on the rabbit (3). An early effect was a reduction in fibrinogen and a falling platelet count, resulting in the release of relatively large amounts of the neuro-transmitter serotonin, stored in platelets (3, 4). Fibrinogen inhibited the release of serotonin from platelets (24). Serotonin is released from platelets during platelet aggregation (14). Platelet aggregation is inhibited by vitamin E (1). Serotonin is a neuro-transmitter associated with deep sleep, respiratory movements and cardiovascular collapse (3). Death at a later stage involved vascular permeability, edema and haemorrhage. After fibrin-platelet clots had formed DIC was present in lungs, kidneys and other organs (3). Medical researchers in Australia linked almost half of SIDS victims with a poisonous strain of intestinal E. coli bacteria (5). Dietary selenium in the intestinal villous tip is considered a daily modulator of cytochrome P450-dependent metabolism of drugs and toxins absorbed by intestinal mucosa (6). Villous atrophy occurs in HSES (2). PMID:1461172

  2. A Systems-Level Perspective of Sudden Infant Death Syndrome

    PubMed Central

    Salomonis, Nathan

    2014-01-01

    Sudden Infant Death Syndrome (SIDS) remains one of the primary causes of infant mortality in developed countries. While the causes of SIDS remain largely inconclusive, some of the most informative associations implicate molecular, genetic, anatomical, physiological and environmental (i.e., infant sleep) factors. Thus, a comprehensive and evolving systems-level model is required to understand SIDS susceptibility. Such models, by being powerful enough to uncover indirect associations, could be used to expand our list of candidate targets for in-depth analysis. We present an integrated WikiPathways model for SIDS susceptibility that includes associated cell systems, signaling pathways, genetics and animal phenotypes. Experimental and literature-based gene-regulatory data has been integrated into this model, to identify intersecting upstream control elements and associated interactions. To expand this pathway model, we performed a comprehensive analysis of existing proteomics data from brainstem samples of SIDS infants. From this analysis, we discovered changes in the expression of several proteins linked to known SIDS pathologies, including factors involved in glial cell production, hypoxia regulation, and synaptic vesicle release, in addition to interactions with annotated SIDS markers. Our results highlight new targets for further consideration that further enrich this pathway model, which, over time, can improve as a wiki-based, community curation project. PMID:24964230

  3. TESTIN Induces Rapid Death and Suppresses Proliferation in Childhood B Acute Lymphoblastic Leukaemia Cells

    PubMed Central

    Weeks, Robert J.; Ludgate, Jackie L.; LeMée, Gwenn; Morison, Ian M.

    2016-01-01

    Background Childhood acute lymphoblastic leukaemia (ALL) is the most common malignancy in children. Despite high cure rates, side effects and late consequences of the intensive treatments are common. Unquestionably, the identification of new therapeutic targets will lead to safer, more effective treatments. We identified TES promoter methylation and transcriptional silencing as a very common molecular abnormality in childhood ALL, irrespective of molecular subtype. The aims of the present study were to demonstrate that TES promoter methylation is aberrant, to determine the effects of TES re-expression in ALL, and to determine if those effects are mediated via TP53 activity. Methods Normal fetal and adult tissue DNA was isolated and TES promoter methylation determined by Sequenom MassARRAY. Quantitative RT-PCR and immunoblot were used to confirm re-expression of TES in ALL cell lines after 5’-aza-2’-deoxycytidine (decitabine) exposure or transfection with TES expression plasmids. The effects of TES re-expression on ALL cells were investigated using standard cell proliferation, cell death and cell cycle assays. Results In this study, we confirm that the TES promoter is unmethylated in normal adult and fetal tissues. We report that decitabine treatment of ALL cell lines results in demethylation of the TES promoter and attendant expression of TES mRNA. Re-expression of TESTIN protein in ALL cells using expression plasmid transfection results in rapid cell death or cell cycle arrest independent of TP53 activity. Conclusions These results suggest that TES is aberrantly methylated in ALL and that re-expression of TESTIN has anti-leukaemia effects which point to novel therapeutic opportunities for childhood ALL. PMID:26985820

  4. [Two Surgical Cases of Loeys-Dietz Syndrome in Childhood].

    PubMed

    Sugawara, Masaaki; Oguma, Fumiaki; Hirahara, Hiroyuki

    2016-08-01

    Loeys-Dietz syndrome( LDS) is a recently recognized autosomal dominant connective tissue disorder. Mutations in the genes encoding transforming growth factor-beta( TGF-β) receptor 1 and (2 TGFBR1, TGFBR2)have been associated with LDS. We report here 2 cases of LDS in childhood. Case 1 was a 10-year-old man, who had aneurysm of both the pulmonary trunk and the ascending aorta, associated with pulmonary and aortic valve insufficiency. Surgical repair was performed successfully at the age of 17. The aortic valve was replaced with a mechanical valve. The aneurysmal ascending aorta was replaced with a Dacron graft. Pulmonary valvuloplasty and pulmonary arterioplasty was performed. Case 2 was a 3-month-old female infant, who had a patent ductus arteriosus( PDA) and aortic root dilation. A detailed physical examination revealed hypertelorism, bifid uvula, retrognathia, talipes equinovarus, and camptodactyly. Computed tomography and echocardiography demonstrated PDA, Valsalva sinus dilation, and arterial tortuosity. These findings were consistent with the clinical manifestations of LDS. Surgical ligation and clipping of the PDA was performed with good results. A molecular genetic analysis subsequently demonstrated a heterozygous missense mutation of the TGFBR2. Since aortic dissection occurs at smaller aortic diameters, early diagnosis and close monitoring are important for patients with LDS. PMID:27476563

  5. The non-immunosuppressive management of childhood nephrotic syndrome.

    PubMed

    McCaffrey, James; Lennon, Rachel; Webb, Nicholas J A

    2016-09-01

    Idiopathic nephrotic syndrome (INS) is one of the most common renal diseases found in the paediatric population and is associated with significant complications, including infection and thrombosis. A high proportion of children enter sustained remission before adulthood, and therapy must therefore mitigate the childhood complications, while minimising the long-term risk to health. Here we address the main complications of INS and summarise the available evidence and guidance to aid the clinician in determining the appropriate treatment for children with INS under their care. Additionally, we highlight areas where no consensus regarding appropriate management has been reached. In this review, we detail the reasons why routine prophylactic antimicrobial and antithrombotic therapy are not warranted in INS and emphasise the conservative management of oedema. When pharmacological intervention is required for the treatment of oedema, we provide guidance to aid the clinician in determining the appropriate therapy. Additionally, we discuss obesity and growth, fracture risk, dyslipidaemia and thyroid dysfunction associated with INS. Where appropriate, we describe how recent developments in research have identified potential novel therapeutic targets. PMID:26556028

  6. Childhood parental death and lifetime suicide attempt of the opposite-gender offspring in a nationwide community sample of Korea.

    PubMed

    Jeon, Hong Jin; Hong, Jin Pyo; Fava, Maurizio; Mischoulon, David; Nyer, Maren; Inamori, Aya; Sohn, Jee Hoon; Seong, Sujeong; Cho, Maeng Je

    2013-12-01

    Although previous studies have shown that childhood parental death influences suicide attempts of their offspring, few studies have examined influence of gender and age at exposure. Koreans show the third highest suicide rate in the world, and many children and adolescents lost their parents during and after the Korean War. A total of 12,532 adults, randomly selected through a one-person-per-household method, completed the Korean version of the Composite International Diagnostic Interview and questionnaire for suicidal ideation, plan, and attempt (response rate 80.2%). A total of 2,332 subjects experienced biological parental death in childhood (18.6%). Male suicide attempts were associated with age of exposure to maternal death from 0 to 4 years (adjusted OR = 4.48, 95% CI 1.32-15.18) and from 5 to 9 years (adjusted OR = 5.52, 95% CI 1.97-16.46), but not with paternal death, after adjusting for age, education years, marital status, monthly income, and psychiatric comorbidities. Female suicide attempts were associated with paternal death from 5 to 9 years (adjusted OR = 2.20, 95% CI 1.13-4.27), but not with maternal death. Childhood parental death is significantly associated with lifetime suicide attempt in the opposite-gender offspring, especially when exposure occurs before age 10. PMID:23834109

  7. Childhood diarrhoeal deaths in seven low- and middle-income countries

    PubMed Central

    Rahman, Ahmed Ehsanur; Moinuddin, Md; Molla, Mitike; Worku, Alemayehu; Hurt, Lisa; Kirkwood, Betty; Mohan, Sanjana Brahmawar; Mazumder, Sarmila; Bhutta, Zulfiqar; Raza, Farrukh; Mrema, Sigilbert; Masanja, Honorati; Kadobera, Daniel; Waiswa, Peter; Bahl, Rajiv; Zangenberg, Mike

    2014-01-01

    Abstract Objective To investigate the clinical characteristics of children who died from diarrhoea in low- and middle-income countries, such as the duration of diarrhoea, comorbid conditions, care-seeking behaviour and oral rehydration therapy use. Methods The study included verbal autopsy data on children who died from diarrhoea between 2000 and 2012 at seven sites in Bangladesh, Ethiopia, Ghana, India, Pakistan, Uganda and the United Republic of Tanzania, respectively. Data came from demographic surveillance sites, randomized trials and an extended Demographic and Health Survey. The type of diarrhoea was classified as acute watery, acute bloody or persistent and risk factors were identified. Deaths in children aged 1 to 11 months and 1 to 4 years were analysed separately. Findings The proportion of childhood deaths due to diarrhoea varied considerably across the seven sites from less than 3% to 30%. Among children aged 1–4 years, acute watery diarrhoea accounted for 31–69% of diarrhoeal deaths, acute bloody diarrhoea for 12–28%, and persistent diarrhoea for 12–56%. Among infants aged 1–11 months, persistent diarrhoea accounted for over 30% of diarrhoeal deaths in Ethiopia, India, Pakistan, Uganda and the United Republic of Tanzania. At most sites, more than 40% of children who died from persistent diarrhoea were malnourished. Conclusion Persistent diarrhoea remains an important cause of diarrhoeal death in young children in low- and middle-income countries. Research is needed on the public health burden of persistent diarrhoea and current treatment practices to understand why children are still dying from the condition. PMID:25378757

  8. Cigarette Smoking as a Risk Factor for Sudden Infant Death Syndrome: A Population-Based Study.

    ERIC Educational Resources Information Center

    Haglund, Bengt; Cnattingius, Sven

    1990-01-01

    Examines risk factors for sudden infant death syndrome based on Swedish births between 1983 and 1985. Results indicate that maternal smoking doubles the risk of infant death, and infants of smokers also died sooner. The more the mother smoked the more likely her infant was to die. (JS)

  9. Coping with Sudden Infant Death Syndrome: Intervention Strategies and a Case Study.

    ERIC Educational Resources Information Center

    Aadalen, Sharon

    1980-01-01

    Family-centered intervention after the death of a baby due to sudden infant death syndrome facilitates reorganization, growth, and development of the family system. A potentially defeating crisis becomes an opportunity to develop coping skills and strengthen family members. Public health nursing is an essential component of the program.…

  10. Sudden Infant Death Syndrome: review of implicated genetic factors.

    PubMed

    Weese-Mayer, Debra E; Ackerman, Michael J; Marazita, Mary L; Berry-Kravis, Elizabeth M

    2007-04-15

    Genetic studies in Sudden Infant Death Syndrome (SIDS) have been motivated by clinical, epidemiological, and/or neuropathological observations in SIDS victims, with subsequent pursuit of candidate genes in five categories: (1) genes for ion channel proteins based on electrocardiographic evidence of prolonged QT intervals in SIDS victims, (2) gene for serotonin transporter based on decreased serotonergic receptor binding in brainstems of SIDS victims, (3) genes pertinent to the early embryology of the autonomic nervous system (ANS) (and with a link to the 5-HT system) based on reports of ANS dysregulation in SIDS victims, (4) genes for nicotine metabolizing enzymes based on evidence of cigarette smoking as a modifiable risk factor for SIDS, and (5) genes regulating inflammation, energy production, hypoglycemia, and thermal regulation based on reports of postnatal infection, low birth weight, and/or overheating in SIDS victims. Evidence for each of these classes of candidate genes is reviewed in detail. As this review indicates, a number of genetically controlled pathways appear to be involved in at least some cases of SIDS. Given the diversity of results to date, genetic studies support the clinical impression that SIDS is heterogeneous with more than one entity and with more than one possible genetic etiology. Future studies should consider expanded phenotypic features that might help clarify the heterogeneity and improve the predictive value of the identified genetic factors. Such features should be evaluated to the extent possible in both SIDS victims and their family members. With 2,162 infants dying from SIDS in 2003 in the U.S. alone, and improved but still imperfect parent and caretaker compliance with known modifiable risk factors for SIDS, it behooves clinicians, researchers, and parents to combine efforts to reach a common goal. The message of the "Back to Sleep" campaign needs to be re-introduced/re-engineered to reach families and caretakers of all

  11. Cushing’s syndrome in childhood: update on genetics, treatment, and outcomes

    PubMed Central

    Lodish, Maya

    2015-01-01

    Purpose of review To provide an update on the genes associated with Cushing’s syndrome in children, as well as to familiarize the clinician with recent treatment guidelines and outcome data for children with Cushing’s syndrome. Recent findings The list of genes associated with Cushing’s syndrome continues to grow. In addition, treatment for childhood Cushing’s syndrome is evolving. As long-term follow-up data on children becomes available, clinicians need to be aware of the issues that require attention. Summary Knowledge of the specific genetic causes of Cushing’s syndrome has potential implications for treatment, surveillance, and counseling. Advances in surgical technique, radiation modalities, and medical therapies offer the potential for additional treatment options in Cushing’s syndrome. Early identification and management of post-treatment morbidities in children treated for Cushing’s syndrome is crucial in order to optimize care. PMID:25517021

  12. Childhood Ataxia with Cerebral Hypomyelination Syndrome: a Variant of Patient with Early Childhood Onset Related to EIF2B3 Mutation. A Case Report.

    PubMed

    Perfetto, F; Stoppino, L P; Calì, A; Milillo, P; Grilli, G; Vinci, R; Macarini, L

    2012-03-01

    Childhood ataxia with central nervous system hypomyelination (CACH) syndrome is an autosomal recessive transmitted leukodystrophy characterised by early childhood onset and acute deterioration following febrile illnesses or head trauma. We describe the case of a child with early onset of CACH syndrome. He presented with cerebellar ataxia beginning around two years of age with mild mental retardation. MRI showed diffuse white matter signal changes with thinning of the corpus callosum. PMID:24028880

  13. [Observations of play and verbal behavior of boys with fragile X syndrome in early childhood].

    PubMed

    Sarimski, K

    1999-08-01

    Reports on development and behaviour in boys with fragile-X syndrome support the idea of a characteristic behavioural phenotype in this special population. Preliminary results are presented for 10 boys with fragile-X syndrome in early childhood. Severe mental handicaps and communicative abnormalities are observed less frequently than was expected on the basis of results reported for school-age children or adults. Boys with fragile-X syndrome show goal-directed and cooperative play behaviours in a Montessori play session, but less persistence and organisation than children with normal development or a mental handicap of heterogeneous origin. Results confirmed these behavioural differences as characteristic aspects of a "behavioural phenotype" in children who already in early childhood have fragile-X syndrome. PMID:10478436

  14. Congenital myasthenic syndromes in childhood: diagnostic and management challenges.

    PubMed

    Kinali, M; Beeson, D; Pitt, M C; Jungbluth, H; Simonds, A K; Aloysius, A; Cockerill, H; Davis, T; Palace, J; Manzur, A Y; Jimenez-Mallebrera, C; Sewry, C; Muntoni, F; Robb, S A

    2008-09-15

    The Congenital Myasthenic Syndromes (CMS), a group of heterogeneous genetic disorders of neuromuscular transmission, are often misdiagnosed as congenital muscular dystrophy (CMD) or myopathies and present particular management problems. We present our experience of 46 children with CMS, referred to us between 1992-2007 with provisional diagnoses of congenital myopathy (22/46), CMS or limb-girdle myasthenia (9/46), central hypotonia or neurometabolic disease (5/46), myasthenia gravis (4/46), limb-girdle or congenital muscular dystrophy (4/46) and SMA (2/46). Diagnosis was often considerably delayed (up to 18y4 m), despite the early symptoms in most cases. Diagnostic clues in the neonates were feeding difficulties (29/46), hypotonia with or without limb weakness (21/46), ptosis (19/46), respiratory insufficiency (12/46), contractures (4/46) and stridor (6/46). Twenty-five children had delayed motor milestones. Fatigability developed in 43 and a variable degree of ptosis was eventually present in 40. Over the period of the study, the mainstay of EMG diagnosis evolved from repetitive nerve stimulation to stimulation single fibre EMG. The patients were studied by several different operators. 66 EMGs were performed in 40 children, 29 showed a neuromuscular junction abnormality, 7 were myopathic, 2 had possible neurogenic changes and 28 were normal or inconclusive. A repetitive CMAP was detected in only one of seven children with a COLQ mutation and neither of the two children with Slow Channel Syndrome mutations. Mutations have been identified so far in 32/46 children: 10 RAPSN, 7 COLQ, 6 CHRNE, 7 DOK7, 1 CHRNA1 and 1 CHAT. 24 of 25 muscle biopsies showed myopathic changes with fibre size variation; 14 had type-1 fibre predominance. Three cases showed small type-1 fibres resembling fibre type disproportion, and four showed core-like lesions. No specific myopathic features were associated with any of the genes. Twenty children responded to Pyridostigmine treatment alone

  15. The Genomic Load of Deleterious Mutations: Relevance to Death in Infancy and Childhood

    PubMed Central

    Morris, James Alfred

    2015-01-01

    The human diploid genome has approximately 40,000 functioning conserved genes distributed within 6 billion base pairs of DNA. Most individuals carry a few heterozygous deleterious mutations and this leads to an increased risk of recessive disease in the offspring of cousin unions. Rare recessive disease is more common in the children of cousin marriages than in the general population, even though <1% of marriages in the Western World are between first cousins. But more than 90% of the children of cousin marriages do not have recessive disease and are as healthy as the rest of the population. A mathematical model based on these observations generates simultaneous equations linking the mean number of deleterious mutations in the genome of adults (M), the mean number of new deleterious mutations arising in gametogenesis and passed to the next generation (N) and the number of genes in the human diploid genome (L). The best estimates are that M is <7 and N is approximately 1. The nature of meiosis indicates that deleterious mutations in zygotes will have a Poisson distribution with a mean of M + N. There must be strong selective pressure against zygotes at the upper end of the Poisson distribution otherwise the value of M would rise with each generation. It is suggested that this selection is based on synergistic interaction of heterozygous deleterious mutations acting in large complex highly redundant and robust genetic networks. To maintain the value of M in single figures over many thousands of generations means that the zygote loss must be of the order of 30%. Most of this loss will occur soon after conception but some will occur later; during fetal development, in infancy and even in childhood. Selection means genetic death and this is caused by disease to which the deleterious mutations predispose. In view of this genome sequencing should be undertaken in all infant deaths in which the cause of death is not ascertained by standard techniques. PMID:25852684

  16. Childhood Physical Abuse Is Associated with Incident Metabolic Syndrome in Mid-Life Women

    PubMed Central

    Midei, Aimee J.; Matthews, Karen A.; Chang, Yue-Fang; Bromberger, Joyce T.

    2013-01-01

    Objective Previous research has suggested that childhood emotional abuse, physical abuse, and sexual abuse are associated with an increased risk for ischemic heart disease. Our objective was to examine whether childhood abuse predicted incident metabolic syndrome, a precursor to heart disease, in mid-life women. Methods Participants were 342 (114 Black, 228 White) women from the Pittsburgh site of the Study of Women’s Health Across the Nation (SWAN). SWAN included a baseline assessment of premenopausal or early perimenopausal women in midlife (mean age = 45.7), and women were evaluated for presence of the metabolic syndrome over 7 annual follow-up visits. Women were classified as having metabolic syndrome if they met 3 of the following criteria: waist circumference > 88 cm, triglycerides ≥ 150 mg/dl, HDL < 50 mg/dl, SBP ≥ 130 or DBP ≥ 85 mmHg or on blood pressure medication, and fasting glucose ≥ 110 mg/dl or diabetic. The Childhood Trauma Questionnaire is a standardized measure that retrospectively assesses three domains of abuse in childhood and adolescence: emotional, physical, and sexual abuse. Results Approximately 34% of the participants reported a history of abuse. Cox model survival analysis showed that physical abuse was associated with incident metabolic syndrome over the course of seven years (HR = 2.12, p = .02), adjusted for ethnicity, age at baseline, and time-dependent menopausal status. Sexual abuse and emotional abuse were unrelated to the metabolic syndrome. Conclusion This is the first study to show that a history of childhood abuse, specifically physical abuse, is related to the development of metabolic syndrome in mid-life women. PMID:22775234

  17. Unique metabolic characteristics of the major syndromes of severe childhood malnutrition

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The major clinical syndromes of severe childhood malnutrition (SCM) are marasmus, kwashiorkor and marasmic-kwashiorkor. Whereas treatment of marasmus is straightforward and the associated mortality is low, kwashiorkor and marasmic-kwashiorkor are difficult to treat and have high morbidity and mortal...

  18. The Childhood Asperger Syndrome Test (CAST): Test-Retest Reliability in a High Scoring Sample

    ERIC Educational Resources Information Center

    Allison, Carrie; Williams, Jo; Scott, Fiona; Stott, Carol; Bolton, Patrick; Baron-Cohen, Simon; Brayne, Carol

    2007-01-01

    The Childhood Asperger Syndrome Test (CAST) is a 37-item parental self-completion questionnaire designed to screen for high-functioning autism spectrum conditions in epidemiological research. The CAST has previously demonstrated good accuracy for use as a screening test, with high sensitivity in studies with primary school aged children in…

  19. Patients Reporting Ritual Abuse in Childhood: A Clinical Syndrome. Report of 37 Cases.

    ERIC Educational Resources Information Center

    Young, Walter C.; And Others

    1991-01-01

    Thirty-seven adult dissociative disorder patients who reported ritual abuse in childhood by satanic cults are described. A clinical syndrome is presented that includes dissociative states with satanic overtones, posttraumatic stress disorder, survivor guilt, unusual fears, and substance abuse. Questions concerning reliability, credibility, and…

  20. Similar Developmental Trajectories in Autism and Asperger Syndrome: From Early Childhood to Adolescence

    ERIC Educational Resources Information Center

    Szatmari, Peter; Bryson, Susan; Duku, Eric; Vaccarella, Liezanne; Zwaigenbaum, Lonnie; Bennett, Teresa; Boyle, Michael H.

    2009-01-01

    Objective: The objective of this study was to chart the developmental trajectories of high-functioning children with autism spectrum disorders (ASD) from early childhood to adolescence using the presence and absence of structural language impairment (StrLI) as a way of differentiating autism from Asperger syndrome (AS). Method: Sixty-four…

  1. The Transition between the Phenotypes of Prader-Willi Syndrome during Infancy and Early Childhood

    ERIC Educational Resources Information Center

    Butler, Jill V.; Whittington, Joyce E.; Holland, Anthony J.; McAllister, Catherine J.; Goldstone, Anthony P

    2010-01-01

    Aim: Prader-Willi syndrome (PWS) is a genetic disorder historically characterized by two phenotypic stages. The early phenotype in infants is associated with hypotonia, poor suck, and failure to thrive. In later childhood, PWS is associated with intellectual disability, hyperphagia, as well as growth and sex hormone deficiency. Little is known…

  2. Infant dreaming and fetal memory: a possible explanation of sudden infant death syndrome.

    PubMed

    Christos, G A

    1995-04-01

    During rapid-eye-movement sleep, when we dream, the brain is thought to be processing stored memory. The memory of a newborn infant is dominated by its fetal experience, and the infant is likely to dream about its life in the womb. Research with lucid (or conscious) dreaming has shown that dream images are supported by the corresponding body actions, using those muscles which remain active during rapid-eye-movement sleep. We suggest that sudden infant death syndrome or cot death may be a result of an infant dreaming about its life (or memory) as a fetus. In the course of that dream, since a fetus does not breathe (in the usual sense) the infant may cease to breathe and may die. This simple hypothesis is consistent with all of the known facts about sudden infant death syndrome (pathological and epidemiological), such as the age at death curve (the observed exponential decay and possibly the peak at 2-3 months), the higher risk with the prone sleeping position (but not excluding the supine position), and the observed climatic variation (seasonal and regional) in the incidence of sudden infant death syndrome. Many of these well-established facts have no other known explanation and other theories can generally only account for a few of the known facts about sudden infant death syndrome. Our hypothesis is also supported by recent findings that, as a group, sudden infant death syndrome infants have a higher proportion of rapid-eye-movement sleep, and also that they have an average higher heart rate (corresponding to possible fetal dreams) but only during rapid-eye-movement sleep.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:7666822

  3. Case-control study of sudden infant death syndrome in Lithuania, 1997–2000

    PubMed Central

    Bubnaitienė, Vilija; Kalėdienė, Ramunė; Kėvalas, Rimantas

    2005-01-01

    Background To identify risk factors for sudden infant death syndrome relevant in Lithuania. Methods A nationwide case-control study surveying parents of 35 infants who died from sudden infant death syndrome during the period of 1997–2000 and parents of 145 control infants matched with SIDS infants for date of birth and for region of birth was carried out. Results Deaths incidence was greater in the warm period (60%) vs. cold period (40%). Prone and side sleeping positions both carried no increased risk of sudden infant death syndrome compared with supine because of a rare prone sleeping (4.1% of controls vs. 0% of dead infants) and more prevalent side than supine sleeping (84.8% of controls vs. 94.3% of dead infants) in the controls as well as the cases. Bed sharing for the whole night as a risk factor for sudden infant death syndrome has not been confirmed, either, as bed sharing was common only for the controls (13.8% of controls vs. 0% of dead infants). Routine sleeping environment factors such as heavy wrapping (≥4 togs) of an infant (odds ratio 8.49; 95% confidence interval 2.38 to 30.32), sleeping in a bassinet (4.22; 1.16 to 15.38) and maternal factors such as maternal education ≤12 years (4.48; 1.34 to 14.94), unplanned pregnancy (5.22; 1.49 to 18.18) and ≥2 previous live births (3.90; 1.00 to 15.10) were significantly associated with sudden infant death syndrome on multivariate analysis. Conclusion The results of this first population-based case-control study have shed some light on the epidemiology of the syndrome in Lithuania. Although the mortality of sudden infant death syndrome in Lithuania is not high, it might be lowered moreover by public informing about sudden infant death syndrome and related risk factors. Special attention must be paid to mothers with low education on potentially modifiable risk factors such as routine heavy wrapping of an infant during sleep, routine sleeping in a bassinet and unplanned pregnancy. PMID:16283946

  4. Forensic issues and possible mechanisms of sudden death in Rett syndrome.

    PubMed

    Byard, Roger W

    2006-02-01

    A 20-year-old female with an established diagnosis of Rett syndrome was found dead in bed. There had been no history of recent deterioration in health and at autopsy no acute lesions were found. There was no evidence of trauma. Toxicological analysis of blood revealed therapeutic levels of carbamazepine and clonazepam. Death was attributed to the complications of Rett syndrome, an uncommon developmental disorder characterized by autistic type behaviour, hypotonia, stereotyped movements, seizures and growth failure, caused by mutations in the MECP2 gene on the X chromosome. Establishing the precise cause of sudden death in individuals with Rett syndrome may be difficult as epilepsy, defective autonomic nervous system control and cardiac arrhythmias may relate more to functional problems rather than to defects that can be demonstrated at autopsy. Thus, although there are a variety of well-documented underlying mechanisms that may cause sudden death in this condition, determining the exact sequence of events in an unwitnessed death may be more by inference and elimination, given the absence of pathognomonic and acute lethal lesions that are able to be found histopathologically. 'Complications of Rett syndrome' may, therefore, be the most accurate designation when individuals with this condition are found unexpectedly dead and no anatomical cause of death can be identified at autopsy. PMID:16263320

  5. Adults with Asperger Syndrome: A Childhood Disorder Grows Up

    ERIC Educational Resources Information Center

    Wilkinson, Lee A.

    2007-01-01

    Asperger syndrome is a chronic developmental disorder characterized by problems in social relatedness, empathic communication and understanding, and circumscribed interests. The inclusion of Asperger's Disorder (Asperger syndrome) in the "Diagnostic and Statistical Manual of Mental Disorders" (DSM-IV; American Psychiatric Association, 1994), has…

  6. Next generation sequencing for molecular confirmation of hereditary sudden cardiac death syndromes.

    PubMed

    Márquez, Manlio F; Cruz-Robles, David; Ines-Real, Selene; Vargas-Alarcón, Gilberto; Cárdenas, Manuel

    2015-01-01

    Hereditary sudden cardiac death syndromes comprise a wide range of diseases resulting from alteration in cardiac ion channels. Genes involved in these syndromes represent diverse mutations that cause the altered encoding of the diverse proteins constituting these channels, thus affecting directly the currents of the corresponding ions. In the present article we will briefly review how to arrive to a clinical diagnosis and we will present the results of molecular genetic studies made in Mexican subjects attending the SCD Syndromes Clinic of the National Institute of Cardiology of Mexico City. PMID:25661095

  7. Ignored Disease or Diagnostic Dustbin? Sudden Infant Death Syndrome in the British Context

    PubMed Central

    Ferguson, Angus H.

    2015-01-01

    Sudden Infant Death Syndrome (SIDS) was defined in 1969 and incorporated into the International Classification of Diseases a decade later. To advocates of SIDS as a diagnosis, medical interest in sudden infant death was long overdue. However, the definition of SIDS lacked positive diagnostic criteria, provoking some to view it as a ‘diagnostic dustbin’ for the disposal of problematic cases where cause of death was unclear. This paper examines the development of medical interest in sudden infant death in Britain during the middle decades of the twentieth century. It highlights the importance of recognising the historicity of SIDS as a diagnosis facilitated by changes in law and medicine over the course of the nineteenth and twentieth centuries. It suggests that SIDS provides a definitive case study of the medicalisation of life and death, and a unique example of an officially recognised disease that had no symptoms, signs, pathology or patients. PMID:26217070

  8. Childhood trauma, parental death, and their co-occurrence in relation to current suicidality risk in adults: a nationwide community sample of Korea.

    PubMed

    Jeon, Hong Jin; Lee, Christina; Fava, Maurizio; Mischoulon, David; Shim, Eun-Jung; Heo, Jung-Yoon; Choi, Hong; Park, Jae-Hyun

    2014-12-01

    Although previous studies have suggested that childhood trauma and parental death are strongly associated with suicidality in adulthood, it is still unclear how these factors interact within the same population. A total of 1396 adults were recruited through nationwide multistage probability sampling in South Korea. Subjects were evaluated through face-to-face interviews using the Suicidality Module of the Mini-International Neuropsychiatric Interview and the Early Trauma Inventory Self Report-Short Form. Among the 1396 adults, the group that experienced both childhood trauma and parental death had the highest current suicidality risks (F = 12.16, p < 0.0001) and lifetime suicide attempt (χ2 = 35.81, p < 0.0001) compared with the other groups, which were only childhood trauma, only parental death, and neither. Multivariate logistic regression analyses revealed that middle-to-high current suicidality risk and lifetime suicide attempt were significantly associated with concurrent childhood trauma and parental death (odds ratio, 3.64; 95% confidence interval, 1.99-6.65) as well as with only childhood trauma (odds ratio, 1.95; 95% confidence interval, 1.33-2.87), after adjusting for age, sex, education, marital status, household monthly income, and living area. Emotional abuse was the only type of childhood trauma significantly associated with higher current suicidality scores in those who experienced childhood parental death than in those who did not (F = 3.26, p = 0.041). Current suicidality risk and lifetime suicide attempt are associated with experiencing both parental death and trauma, especially emotional abuse, in childhood, whereas experiencing only childhood parental death is associated with neither. PMID:25370752

  9. Effect of Fungicide Seed Treatments on Fusarium virguliforme and Sudden Death Syndrome of Soybean

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS) is a yield reducing disease increasing in prevalence across soybean producing states. Recent research indicates the SDS pathogen, Fusarium virguliforme, can infect as early as initial radicle emergence. This suggests fungicide seed treatments could offer some protection a...

  10. Soybean Sudden Death Syndrome Species Diversity within North and South America Revealed by Multilocus Genotyping

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden-death syndrome (SDS) of soybean and has become a serious constraint to the production of this crop in North and South America. Recently published phenotypic and multilocus molecular phylogenetic analyses, and pathogenicity experiments have demonstrated that four morphologically and phylogene...

  11. Transcriptional analysis of soybean roots response to Fusarium virguliforme, the causal agent of sudden death syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS) of soybean can be caused by any of four distinct Fusarium species, with F. virguliforme and F. tucumaniae being the main casual agents in North and South America, respectively. Although the fungal tissue is largely confined to the root, the fungus releases a toxin that is...

  12. A method for determining the severity of Sudden Death Syndrome in soybeans

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS), caused by the fungus Fusarium virguliforme, is a widespread mid- to late- season soybean disease with distinctive foliar symptoms that in some extreme cases may cause nearly 100% yield loss. This article reports on the development of an image analysis method to quantify ...

  13. First Report of Sexual Reproduction by the Soybean Sudden Death Syndrome Pathogen Fusarium tucumaniae in Nature

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Of the four fusaria that have been shown to cause soybean sudden death syndrome (SDS), field surveys indicate that Fusarium tucumaniae is the most important and genetically diverse SDS pathogen in Argentina. Although none of the SDS fusaria have been shown to produce perithecia in nature, a heteroth...

  14. Infant Temperament Characteristics Related to Sudden Infant Death Syndrome and Its Risk Factors

    ERIC Educational Resources Information Center

    Kelmanson, Igor A.

    2006-01-01

    Three major components have been repeatedly implicated for the origin(s) of sudden infant death syndrome (SIDS): system, minor sickness and surroundings. All these factors also frame infant temperament, and therefore it seems logical to suppose that the babies who either succumb to or are at risk of SIDS may present with certain behavioral…

  15. Evaluation of Aggressiveness of Fusarium virguliforme Isolates That Cause Soybean Sudden Death Syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Greenhouse studies were conducted to compare the ability of fungal isolates that cause soybean sudden death syndrome (SDS). After an initial evaluation of 123 isolates on soybean, 30 were selected for further tests where both foliar severity and root infection were evaluated. Difference among isolat...

  16. POSSIBLE RELATIONSHIP OF SUDDEN INFANT DEATH SYNDROME TO INDOOR AIR QUALITY

    EPA Science Inventory

    Sudden Infant Death Syndrome (SIDS) has been associated with higher rates of maternal smoking and higher body lead content of SIDS victims compared to control infants, matched for age and sex, who died of other causes. Hoppenbrouwers et al. demonstrated a temporal relationship be...

  17. Risk Factors of Sudden Infant Death Syndrome and Risk Factors for Sleep Disturbances

    ERIC Educational Resources Information Center

    Kelmanson, Igor A.

    2011-01-01

    Relationship between major risk factors of sudden infant death syndrome (SIDS) and sleep disorders in the infants is the subject of review and discussion. Improper micro-environmental characteristics (especially poor environmental organisation and lack of developmental stimulation), pre-term delivery and/or infant low birth weight, prone sleep…

  18. Greenhouse Inoculation Methods for Evaluating Resistance of Soybean to Sudden Death Syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS) caused by the soil borne fungus Fusarium solani f. sp. glycines (FSG) (syn. Fusarium virguliforme Akoi, O’Donnell, Homma and Lattanzi), is a major disease in soybean [Glycine max (L.) Merr.]. Selection for SDS resistance in the field is difficult because of the impact of ...

  19. Genome-wide association mapping of quantitative resistance to sudden death syndrome in soybean

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS) is a serious threat to soybean production that can be controlled by host plant resistance. To dissect the genetic architecture of quantitative resistance to the disease in soybean, two independent association panels of soybean elite cultivar, consisting of 392 and 300 uni...

  20. Statistical and Modeling Techniques for Studying the Sudden Infant Death Syndrome

    ERIC Educational Resources Information Center

    Lindsey, Helen L.

    1976-01-01

    The intention of this research is to contribute additional data, hopefully bearing on the solution to some of the problems and indirectly, the cause(s) of Sudden Infant Death Syndrome, and to present ideas for consideration for future SIDS research. (Author/RK)

  1. SIDS Family Adjustment Scale: A Method of Assessing Family Adjustment to Sudden Infant Death Syndrome.

    ERIC Educational Resources Information Center

    May, Harold J.; Breme, Frederick J.

    1982-01-01

    Discusses Sudden Infant Death Syndrome (SIDS) and the family's resultant grief process. Explores SIDS as a family crisis, and by identifying the psychological factors or tasks pertinent to family adjustment, proposes a SIDS Family Adjustment Scale which assists in recognizing adaptive and maladaptive grief responses. (Author)

  2. Metabolic syndrome in the survivors of childhood acute lymphoblastic leukaemia.

    PubMed

    Abu-Ouf, Noran M; Jan, Mohammed M

    2015-01-01

    Metabolic syndrome is a common complication encountered in children surviving acute lymphoblastic leukaemia (ALL). Affected patients develop obesity, insulin resistance, hypertension, and hyperlipidemia. Metabolic syndrome is a consequence of multiple factors, particularly hormonal imbalance induced by various ALL treatments. This review aims to evaluate the risk factors and mechanisms leading to the development of metabolic syndrome. Further research is needed to improve our understanding of the mechanisms leading to insulin resistance and the associated endothelial and adipose tissue dysfunction. Future studies should also examine other possible contributing factors, such as environmental and genetic factors. Understanding these factors will help in guiding modifications of the current ALL treatment protocols in order to prevent the development of this syndrome and hence improve the quality of life of ALL survivors. Until this is achieved, clinicians should continue to identify patients at risk early and use a therapeutic approach that combines dietary restrictions and enhanced physical activity. PMID:25081809

  3. [Sudden death of a 16-year-old girl with WPW syndrome: a case report].

    PubMed

    Wöllner, Kirsten; Doberentz, Elke; Madea, Burkhard

    2015-01-01

    The Wolff-Parkinson-White syndrome is a usually benign heart disease with accessory pathways. Circling excitations arise between atria and ventricles which can lead to cardiac arrhythmias. Cases of sudden cardiac death are rare (0.2 %). Risk factors for sudden cardiac death in patients with WPW syndrome are old age, several accessory pathways, male sex and previous syncopes. A 16-year-old girl was found lying dead in her bed. The evening before, she didn't feel well and complained about abdominal pain. The girl had known epilepsy and Wolff- Parkinson-White syndrome. The macroscopic and histological findings are presented and discussed with reference to the pertinent literature. PMID:26419085

  4. Philemon and Baucis syndrome: three additional cases of double deaths of married couples.

    PubMed

    Delannoy, Y; Tournel, G; Dedouit, F; Cornez, R; Telmon, N; Hedouin, V; Rouge, D; Gosset, D

    2013-03-10

    The simultaneous death of two people is immediately considered as a suspect. However, this feeling is reinforced when the individuals are spouses. In these situations, criminal and forensic investigations are required to establish whether or not the deaths were homicidal in nature. Despite many descriptions of simultaneous deaths being present in the literature, the simultaneous death of two spouses from natural causes is poorly described with Ciesiolka et al., Department of Legal Medicine in Gießen (Germany), being the only ones to have reviewed two case reports involving these circumstances. The scarcity of this type of information in the literature renders the task of claiming natural simultaneous death as the final outcome of an investigation difficult. We would like to report three additional cases with the aim of better describing this type of event. In all three cases, the bodies were those of a married couple in their 80s. The bodies were discovered in the same room. In each case, the death of one of the spouses could be attributed to natural cause; however the death of the other spouse could not be determined with certainty, and shared several similarities in all cases: simultaneity in death; a pre existing cardiovascular disease/disorder; a certain degree of fragility and dependence on the other spouse whose death could lead to acute psychological stress. Intense psychological disorder could trigger acute coronary or rhythmic disorders. The mechanisms by which brain activity influences cardiac electrophysiology are now known to take place via the autonomic nervous system mediation. This brain activity could provide an explanation for the death of the individuals with pre-existing heart conditions, who underwent significant stress upon occurrence of the death of their partners. The death of these individuals, which took place at the same place and time as their deceased spouses, can be attributed to natural causes: the Philemon and Baucis syndrome. PMID

  5. Pathogenesis of sudden death following water immersion (immersion syndrome)

    NASA Technical Reports Server (NTRS)

    Buhring, M.; Spies, H. F.

    1981-01-01

    Sympathetic activity under cold stress is investigated. Predominantly vagal cardio-depressive reflexes are discussed besides currently known mechanisms of sudden death after water immersion. Pronounced circulatory centralization in diving animals as well as following exposure in cold water indicates additional sympathetic activity. In cold water baths of 15 C, measurements indicate an increase in plasma catecholamine levels by more than 300 percent. This may lead to cardiac arrhythmias by the following mechanisms: cold water essentially induces sinus bradycardia; brady-and tachycardiarrhythmias may supervene as secondary complications; sinusbradycardia may be enhanced by sympathetic hypertonus. Furthermore, ectopic dysrhythmias are liable to be induced by the strictly sympathetic innervation of the ventricle. Myocardial ischemia following a rise in peripheral blood pressure constitutes another arrhythmogenic factor. Some of these reactions are enhanced by alcohol intoxication.

  6. Lights and shadows in autoinflammatory syndromes from the childhood and adulthood perspective.

    PubMed

    Rigante, Donato; Vitale, Antonio; Natale, Marco Francesco; Cantarini, Luca

    2016-03-01

    In a high percentage of cases, the monogenic autoinflammatory syndromes (AIS), caused by subversion in the inflammasome homeostasis leading to cytokine oversecretion and characterized by multiple inflammatory pictures, start in childhood. However, the description of tardive manifestations, veiled phenotypes, and atypical clinical signs beginning in adulthood has been more and more reported in recent times, requiring that many specialists become confident with concepts, details, and management strategies of AIS. Differences between child- and adult-onset syndromes raise the question of whether pathogenic mechanisms might differ when the timetable of AIS onset diverges, but show that carefulness is needed to establish a straightforward diagnosis. PMID:26631101

  7. Childhood Sjögren syndrome presenting as acute brainstem encephalitis.

    PubMed

    Matsui, Yoriko; Takenouchi, Toshiki; Narabayashi, Atsushi; Ohara, Kentaro; Nakahara, Tadaki; Takahashi, Takao

    2016-01-01

    Sjögren syndrome is an autoimmune disease characterized by dry mouth and eyes, known as sicca symptoms. The exact spectrum of neurological involvement, especially of the central nervous system, in childhood Sjögren syndrome has not been well defined. We report a girl who presented with acute febrile brainstem encephalitis. In retrospect, she had exhibited a preceding history of recurrent conjunctivitis and strong halitosis that could be considered as sicca symptoms. The histopathology results of a minor salivary biopsy, the presence of anti-SSA/Ro antibody, and keratoconjunctivitis confirmed the diagnosis of Sjögren syndrome. Commonly observed features in previously reported patients with childhood Sjögren syndrome and central nervous system complications have included fever at the time of neurologic presentation, cerebrospinal fluid pleocytosis, abnormal neuroimaging, and positivity for several specific antibodies. In children presenting with unknown acute febrile encephalopathy, Sjögren syndrome should be included in the differential diagnosis, especially when sicca symptoms are present. PMID:26006751

  8. The dangerous link between childhood and adulthood predictors of obesity and metabolic syndrome.

    PubMed

    Faienza, Maria Felicia; Wang, David Q H; Frühbeck, Gema; Garruti, Gabriella; Portincasa, Piero

    2016-03-01

    The purpose of this review is to evaluate whether some risk factors in childhood work as significant predictors of the development of obesity and the metabolic syndrome in adulthood. These factors include exposures to risk factors in the prenatal period, infancy and early childhood, as well as other socio-demographic variables. We searched articles of interest in PubMed using the following terms: 'predictors AND obesity OR Metabolic syndrome AND (children OR adolescents) AND (dyslipidemia OR type 2 diabetes OR atherosclerosis OR hypertension OR hypercholesterolemia OR cardiovascular disease)' AND genetic OR epigenetic. Maternal age, smoking and weight gain during pregnancy, parental body mass index, birth weight, childhood growth patterns (early rapid growth and early adiposity rebound), childhood obesity and the parents' employment have a role in early life. Furthermore, urbanization, unhealthy diets, increasingly sedentary lifestyles and genetic/epigenetic variants play a role in the persistence of obesity in adulthood. Health promotion programs/agencies should consider these factors as reasonable targets to reduce the risk of adult obesity. Moreover, it should be a clinical priority to correctly identify obese children who are already affected by metabolic comorbidities. PMID:26758061

  9. Case-control study of sudden infant death syndrome in Scotland, 1992-5.

    PubMed Central

    Brooke, H.; Gibson, A.; Tappin, D.; Brown, H.

    1997-01-01

    OBJECTIVE: To investigate the relation between routine infant care practices and the sudden infant death syndrome in Scotland. METHODS: National study of 201 infants dying of the sudden infant death syndrome (cases) and 276 controls by means of home interviews comparing methods of infant care and socioeconomic factors. RESULTS: Sleeping prone (odds ratio 6.96 (95% confidence interval 1.51 to 31.97) and drug treatment in the previous week (odds ratio 2.33 (1.10 to 4.94)) were more common in the cases than controls on multivariate analysis. Smoking was confirmed as a significant risk factor (odds ratio for mother and father both smoking 5.19 (2.26 to 11.91)). The risk increased with the number of parents smoking (P < 0.0001), with the number of cigarettes smoked by mother or father (P = 0.0001), and with bed sharing (P < 0.005). A new finding was an increased risk of dying of the syndrome for infants who slept at night on a mattress previously used by another infant or adult (odds ratio 2.51 (1.39 to 4.52)). However, this increased risk was not established for mattresses totally covered by polyvinyl chloride. CONCLUSIONS: Sleeping prone and parental smoking are confirmed as modifiable risk factors for the sudden infant death syndrome. Sleeping on an old mattress may be important but needs confirmation before recommendations can be made. PMID:9169398

  10. Fetal Alcohol Syndrome in Sudden Unexpected Death in Infancy: A Case Report in Medicolegal Autopsy.

    PubMed

    Tangsermkijsakul, Aphinan

    2016-03-01

    Fetal alcohol spectrum disorder is a range of birth defects associated with prenatal alcohol exposure. Fetal alcohol syndrome (FAS) is the most serious form of fetal alcohol spectrum disorder. Infants with FAS are prone to death because of various physical abnormalities. Consequently, infants with FAS may be presented in the medicolegal investigation as a form of sudden unexpected death in infancy. The author reported a 6-month-old male infant who was found dead at home. The history of maternal ethanol consumption during pregnancy was obtained. The infant was diagnosed with FAS at the autopsy because he was presented with postnatal growth retardation, multiple facial abnormalities, and abnormal brain structures, which met the criteria of FAS. The cause of death was severe aspiration pneumonia. The purposes of this case report are to show an uncommon manifestation of sudden unexpected death in infancy case for the forensic pathologists and to emphasize on the national healthcare problem. PMID:26730801

  11. Clinical Characteristics of Childhood Guillain-Barré Syndrome

    PubMed Central

    Koul, Roshan; Al-Futaisi, Amna; Chacko, Alexander; Fazalullah, Mohammed; Nabhani, Susan Al; Al-Awaidy, Salah; Al-Busaidy, Suleiman; Al-Mahrooqi, Salim

    2008-01-01

    Objectives To find the incidence, clinical pattern and outcome of Guillain-Barre syndrome in the Sultanate of Oman in children less than 15 years of age. Methods All children under fifteen years with acute flaccid paralysis were admitted to identify the underlying cause. The diagnosis of Gullain Barre syndrome was made by clinical criteria, cerebrospinal fluid findings and nerve conduction studies. Intravenous immunoglobulins were given to all and two needed plasmapharesis. Results Sixty-one children were diagnosed as Guillan-Barré syndrome and constituted 20% of cases of acute flaccid paralysis. Males 39 (63.9%) outnumbered females (36.1%).The annual incidence below 15 years was 0.45/100,000. Cranial nerves were involved in 31 (50.8%) children. Albumino-cytological dissociation in cerebrospinal fluid was seen in 42/45(93.3%) cases. Acute relapse was seen in six (9.8%) cases. Eleven children (18.3%) needed ventilation. Complete recovery was seen in 45 to 310 days (mean 69.1 days). Three children (4.9%) were left with minimal residual deficit. There was no mortality. Conclusions Guillain Barre syndrome is a serious disease, although recovery is the rule in children. The disease is associated with very low mortality and long term morbidity. Immunoglobulins have reduced the duration of hospital stay and the total time needed for recovery. PMID:22359705

  12. Underlying molecular and cellular mechanisms in childhood irritable bowel syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Irritable bowel syndrome (IBS) affects a large number of children throughout the world. The symptom expression of IBS is heterogeneous, and several factors which may be interrelated within the IBS biopsychosocial model play a role. These factors include visceral hyperalgesia, intestinal permeability...

  13. Down Syndrome Temperament: The Stereotype at Middle Childhood and Adolescence.

    ERIC Educational Resources Information Center

    Gunn, Pat; Cuskelly, Monica

    1991-01-01

    Behavioral ratings by mothers and teachers of 94 children with Down's Syndrome (between 8 and 14 years of age) indicated general support for the amiable personality stereotype, but ratings of low persistence were associated with maternal impressions of difficulty. There was little agreement between mothers and teachers regarding individual child…

  14. The CAST (Childhood Asperger Syndrome Test): Preliminary Development of a UK Screen for Mainstream Primary-School-Age Children.

    ERIC Educational Resources Information Center

    Scott, Fiona J.; Baron-Cohen, Simon; Bolton, Patrick; Brayne, Carol

    2002-01-01

    This article describes a pilot and follow-up study of the development of a screening test for Asperger Syndrome (AS) and relates social and communication conditions in children aged 4-11. Results suggest that the Childhood Asperger Syndrome Test may be useful for identifying children at risk for AS and related conditions, in a mainstream…

  15. Seasonal differences in risk factors for sudden infant death syndrome. The New Zealand Cot Death Study Group.

    PubMed

    Mitchell, E A; Clements, M; Williams, S M; Stewart, A W; Cheng, A; Ford, R P

    1999-03-01

    The aim of this study was to explore whether the risk of sudden infant death syndrome (SIDS) associated with prone sleeping position and other risk factors varies with season. The study was a large nation-wide case-control study, which compared 485 cases with 1800 controls. Parents of 393 (81.0%) cases and 1591 (88.4%) controls were interviewed. Obstetric records were also examined. Infants dying in winter were older and had lower birthweights than those dying in summer. The increased risk of SIDS associated with prone sleeping position was greater in winter than in summer. In contrast, the increased risk of SIDS associated with excess thermal insulation and bed sharing was less in winter than in summer. Prone sleeping position accounts for about half of the difference between the mortality rate in summer and that in winter. This suggests that some factor related to season modifies the effect of prone sleeping position. PMID:10229033

  16. [A proposal of essentials for forensic pathological diagnosis of sudden infant death syndrome (SIDS)].

    PubMed

    Takatsu, A; Misawa, S; Yoshioka, N; Nakasono, I; Sato, Y; Kurihara, K; Nishi, K; Maeda, H; Kurata, T

    2000-08-01

    There are many sudden unexpected infant death cases which are easily diagnosed as sudden infant death syndrome (SIDS) both with or without autopsy in Japan. A SIDS diagnosis may provide a cover for accidental or criminal death. SIDS should not be a convenient diagnostic box that shelters the cases of unexpected infant death which lack the necessary antemortem information to make the correct diagnosis. The authors consider that SIDS should be diagnosed according to the direction of the international definition of SIDS, and propose the following essentials for a forensic pathological diagnosis. 1) A thorough autopsy should be performed based on precise autopsy protocol, including not only histological observation, but also, if necessary, toxicological, bacteriological, viral and/or biochemical examinations. 2) The forensic pathologist should be provided with pertinent information regarding antemortem health status, past clinical history, social circumstances, death scene investigation, etc. In order to collect more precise information, the authors recommend using a questionnaire such as the example in this report to record information from the deceased's guardians. 3) Suspicion of accidental death or infanticide should be completely ruled out. SIDS should be diagnosed only after these three essentials have been satisfied. When there is even a slight suspicion of accidental death or infanticide, or when the forensic pathologist can not obtain pertinent information about the deceased, the causes and classification of the death should be diagnosed as unspecified or undetermined. That is, the causes and classification of the death are undetermined as to whether it is a natural or unnatural death. Furthermore, several warning flags indicating a possible SIDS diagnosis were proposed: a case found dead in a supine position, the existence of a foreign body in the respiratory tract or mild infectious findings. The authors also emphasize the physician's responsibility to

  17. Management patterns of childhood-onset nephrotic syndrome.

    PubMed

    MacHardy, Nathaniel; Miles, Paul V; Massengill, Susan F; Smoyer, William E; Mahan, John D; Greenbaum, Larry; Massie, Sara; Yao, Lynne; Nagaraj, Shashi; Lin, Jen-Jar; Wigfall, Delbert; Trachtman, Howard; Hu, Yichun; Gipson, Debbie S

    2009-11-01

    As an initial effort to identify opportunities to improve the management of children with nephrotic syndrome, the goal of this study was to assess the present-day management of children with primary nephrotic syndrome. A web-based survey was designed to assess the current management styles of all pediatric nephrology faculties at ten participating institutions. Ninety-one percent completed the initial survey. The duration of initial glucocorticoid therapy ranged from 4 to 24 weeks. Physicians reported that the recommendation for kidney biopsy was dependent on the response to initial corticosteroid therapy, with the minority always recommending a biopsy for frequently relapsing or steroid-dependent cases. All responding physicians recommended a kidney biopsy in steroid-resistant cases. Treatment strategies were reported to vary based upon the steroid response pattern and, where available, kidney histopathology. Striking variations in therapeutic preferences were described when alternatives to glucocorticoids were considered. The variability of management practices among pediatric nephrologists in the USA combined with the changing characteristics of our pediatric population raise concerns about our future strategies for improving healthcare for children coping with nephrotic syndrome. This variability is not unique to children's healthcare or to nephrology. However, a systematic approach to patient care and improvement in health outcomes has been shown to substantially improve morbidity and mortality outcomes in children with chronic health conditions. PMID:19672630

  18. Asbestos bodies in children's lungs. An association with sudden infant death syndrome and bronchopulmonary dysplasia

    SciTech Connect

    Haque, A.K.; Kanz, M.F.

    1988-05-01

    Lungs from 46 autopsied children (age range, 1 to 27 months) were examined for asbestos bodies using a bleach-digestion extraction technique. Ten (21.7%) of 46 children had asbestos bodies in their lungs. Of these ten children, seven were diagnosed with sudden infant death syndrome, and three were diagnosed with bronchopulmonary dysplasia. Thus, 46.6% of children with sudden infant death syndrome and 42.8% of children with bronchopulmonary dysplasia had asbestos bodies. Impaired lung-clearing mechanisms due to either abnormal lung physiology or reorganization of pulmonary architecture may be significant in the formation of asbestos bodies. Additionally, children with asbestos bodies may have been exposed to higher ambient levels of asbestos and other pollutants.

  19. [Guillain-Barre's syndrome in childhood (author's transl)].

    PubMed

    Mulas, F; Martínez Bermejo, A; Pascual Castroviejo, I

    1976-01-01

    Seventeen cases of Guillain-Barré's syndrome aged between 14 month ans 7 years are studied from clinical, analytical and evolutives aspects. Neither sex nor season predominance occurs. Sixteen of the patients had a previous viral or bacterial infection. Difficulty in walking was the main symptom at the onset of illness. Arreflexia and motot paralysis were always symmetrical and ascending. Albumino-cytological dissociation in the C.S.F. was found in the whole group. An evolutive study was made of the recovery of movement, C.S.F. and tendon reflexes which occurred in this order except in two cases which showed abnormal evolution. We emphasize the extent and frequency of respiratory difficulties at the onset of illness. Some etiopathogenic aspects, the lack of specifity of the syndrome and the differences of the diagnosis with that of poliomyelitis are commented. We point out the lack of effect of the corticosteroids treatment and the positive effects of phisiotherapy after the acute stage. PMID:1267306

  20. Splenic hypofunction in the nephrotic syndrome of childhood

    SciTech Connect

    McVicar, M.I.; Chandra, M.; Margouleff, D.; Zanzi, I.

    1986-05-01

    The reticuloendothelial system, including the spleen, subserves important immunologic functions. Loss of splenic function results in an increased incidence of severe bacterial infections and is accompanied by thrombocytosis. Several nephrotic children were noted to have remarkably high platelet counts and predisposition to bacterial infection with encapsulated organisms. We, therefore, investigated the splenic function of nine children with primary nephrotic syndrome and measured the phagocytic function of the spleen by sequestration of Technetium-99-labelled heat-treated autologous RBC, administered intravenously. Four children had decreased splenic function. Repeat studies performed in two of these children after remission of the nephrotic syndrome gave normal results. There were six episodes of bacterial infection (3 peritonitis, 1 septic arthritis, 1 cellulitis, and 1 Escherichia coli urinary tract infection) among the four patients with decreased splenic function. There were no episodes of bacterial infection among the five nephrotic children with normal splenic function. Nephrotic patients with decreased splenic function had significantly increased platelet counts (921,000 +/- 196,000; mean +/- SEM) compared to those with normal function (435,000 +/- 46,000; P less than 0.001). Our findings suggest the possibility that some nephrotic children may have decreased splenic function in association with increased susceptibility to bacterial infections.

  1. Childhood Sjögren Syndrome: Insights from adults and animal models

    PubMed Central

    Lieberman, Scott M.

    2014-01-01

    Purpose of review Sjögren syndrome (SS) is a chronic autoimmune disease affecting lacrimal and salivary glands and potentially involving extraglandular manifestations. While common in adults, the prevalence and prognosis of childhood SS is unknown, in part due to lack of child-specific diagnostic and classification criteria. This review discusses difficulties in diagnosing childhood SS and highlights recent findings in SS treatment and pathogenesis from studies in adults and animal models over the past 18 months. Recent findings Studies of rituximab show some therapeutic potential in adult SS while newer modalities including gene therapy and mesenchymal stem cell transfer are promising. The pathogenesis of SS is emerging, including roles of T and B lymphocytes, autoantibodies, interferons, and glandular epithelial cells. Specific recent notable findings in SS pathogenesis include identification of a type II interferon signature in salivary glands of SS patients, characterization of salivary gland-infiltrating T cell subsets, and characterization of anti-muscarinic acetylcholine receptor type 3 autoantibodies. Summary Childhood SS is a poorly defined and underdiagnosed autoimmune disease which requires child-specific criteria in order to study disease burden and prognosis. Studies in adults and animal models continue to elucidate new potential diagnostic and therapeutic targets, which may be relevant for childhood SS. PMID:23917159

  2. The course of severe chronic fatigue syndrome in childhood.

    PubMed Central

    Rangel, L; Garralda, M E; Levin, M; Roberts, H

    2000-01-01

    Little has been reported on prognostic indicators in children with chronic fatigue syndrome (CFS). We used interviews with children and parents, a mean of 45.5 months after illness onset, to follow up 25 cases of CFS referred to tertiary paediatric psychiatric clinics. At its worst, the illness had been markedly handicapping (prolonged bed-rest and school absence in two-thirds); mean time out of school was one academic year. Two-thirds, however, had recovered and resumed normal activities--mean duration of illness to recovery/assessment 38 months--and none had developed other medical conditions. Recovery was associated with specific physical triggers to the illness, with start of illness in the autumn school term and with higher socioeconomic status. Severe fatigue states in children can cause serious and longlasting handicap but most children recover. PMID:10741312

  3. Refractory Seizure in Childhood: Dyke-Davidoff-Masson Syndrome Revisited.

    PubMed

    Dutta, Abhijit; Bose, Sagar; Sen, Kaushik; Pandit, Narayan; Sharma, Samarth

    2016-07-01

    Dyke-Davidoff-Masson syndrome (DDMS) is a rare disorder characterized by recurrent seizures, facial asymmetry, contralateral hemiplegia, radiologic features of cerebral hemiatrophy, and ipsilateral compensatory hypertrophy of the skull bone and sinuses. We describe three cases of children with DDMS, who initially presented with refractory seizure to the pediatric department of North Bengal Medical College and Hospital, India. In each case, the clinical features noted along with computed tomography or magnetic resonance imaging helped confirm the diagnosis of DDMS. DDMS should be considered as a differential diagnosis of refractory seizures in children. We seek to emphasize the importance of thorough clinical and neuroimaging workup of seizure disorder in children for the proper management of the condition. PMID:27403244

  4. APECED syndrome in childhood: clinical spectrum is enlarging.

    PubMed

    Valenzise, Mariella; Alessi, Luca; Bruno, Enrico; Cama, Valeria; Costanzo, Daria; Genovese, Cristina; Mignosa, Cristina; Scuderi, Veronica; DE Luca, Filippo

    2016-06-01

    Autoimmune polyendocrinopathy-candidiasis-ectodermal-distrophy (APECED) is a rare autosomal recessive disease, which is mainly characterized by the association of many autoimmune diseases, with a classic triad including chronic mucocutaneous candidiasis, hypoparathyroidism and adrenocortical failure. Its clinical spectrum has significantly enlarged in the last years and other non-classic components have been recently described. Aim of this review was to alert pediatricians to these novel clinical aspects of this syndrome, that have been recently included among the autoimmune APECED manifestations: a) chronic lung disease, that may evolve to cor pulmonale and terminal respiratory failure; b) chronic inflammatory demyelinating polineuropathy, with progressive muscular weakness of both arms and legs and sensory loss; c) gastrointestinal dysfunction, with recurrent diarrhea, malabsorption and steatorrhea or chronic constipation. For each of these novel components of APECED, specific autoantibodies against either lung autoantigens or peripheral nerves or tryptophan hydroxylase have been just recently identified. PMID:25502918

  5. Refractory Seizure in Childhood: Dyke-Davidoff-Masson Syndrome Revisited

    PubMed Central

    Dutta, Abhijit; Bose, Sagar; Sen, Kaushik; Pandit, Narayan; Sharma, Samarth

    2016-01-01

    Dyke-Davidoff-Masson syndrome (DDMS) is a rare disorder characterized by recurrent seizures, facial asymmetry, contralateral hemiplegia, radiologic features of cerebral hemiatrophy, and ipsilateral compensatory hypertrophy of the skull bone and sinuses. We describe three cases of children with DDMS, who initially presented with refractory seizure to the pediatric department of North Bengal Medical College and Hospital, India. In each case, the clinical features noted along with computed tomography or magnetic resonance imaging helped confirm the diagnosis of DDMS. DDMS should be considered as a differential diagnosis of refractory seizures in children. We seek to emphasize the importance of thorough clinical and neuroimaging workup of seizure disorder in children for the proper management of the condition. PMID:27403244

  6. Early and treatment-related deaths in childhood acute myeloid leukaemia in the Nordic countries: 1984-2003.

    PubMed

    Molgaard-Hansen, Lene; Möttönen, Merja; Glosli, Heidi; Jónmundsson, Guðmundur K; Abrahamsson, Jonas; Hasle, Henrik

    2010-12-01

    Despite major improvements in the cure rate of childhood acute myeloid leukaemia (AML), 5-15% of patients still die from treatment-related complications. In a historical prospective cohort study, we analysed the frequency, clinical features and risk factors for early deaths (ED) and treatment-related deaths (TRD) in 525 children included in the Nordic Society of Paediatric Haematology and Oncology (NOPHO)-AML-84, -88 and -93 trials. Seventy patients (13%) died before starting treatment or from treatment-related complications. The death rate rose from 11% in NOPHO-AML-84 to 29% in -88, but then fell to 8% in -93. Sixteen patients (3%) died within the first 2 weeks, mainly from bleeding or leucostasis. Hyperleucocytosis, age <2 or ≥10 years were risk factors. After day 15, 10% of patients died from treatment-related complications with infection as the main cause of death. Risk factors were age <2 or ≥10 years and treatment according to the NOPHO-AML-88 protocol. The number of EDs and TRDs in AML is high. Therefore optimal antifungal prophylaxis is essential, and studies on the benefit of antibacterial prophylaxis and individual risk factors for ED and TRD are needed. PMID:20955398

  7. Leigh Syndrome in Childhood: Neurologic Progression and Functional Outcome

    PubMed Central

    Lee, Jin Sook; Kim, Hunmin; Lim, Byung Chan; Hwang, Hee; Choi, Jieun; Kim, Ki Joong; Hwang, Yong Seung

    2016-01-01

    Background and Purpose Few studies have analyzed the clinical course and functional outcome in Leigh syndrome (LS). The aim of this study was to determine the clinical, radiological, biochemical, and genetic features of patients with LS, and identify prognostic indicators of the disease progression and neurological outcome. Methods Thirty-nine patients who had been diagnosed with LS at the Seoul National University Children's Hospital were included. Their medical records, neuroimaging findings, and histological/biochemical findings of skeletal muscle specimens were reviewed. Targeted sequencing of mitochondrial DNA was performed based on mitochondrial respiratory chain (MRC) enzyme defects. Results Isolated complex I deficiency was the most frequently observed MRC defect (in 42% of 38 investigated patients). Mitochondrial DNA mutations were identified in 11 patients, of which 81.8% were MT-ND genes. The clinical outcome varied widely, from independent daily activity to severe disability. Poor functional outcomes and neurological deterioration were significantly associated with early onset (before an age of 1 year) and the presence of other lesions additional to basal ganglia involvement in the initial neuroimaging. Conclusions The neurological severity and outcome of LS may vary widely and be better than those predicted based on previous studies. We suggest that age at onset and initial neuroimaging findings are prognostic indicators in LS. PMID:27074294

  8. Sturge-Weber syndrome with spontaneous intracerebral hemorrhage in childhood.

    PubMed

    Nakajima, Madoka; Sugano, Hidenori; Iimura, Yasushi; Higo, Takuma; Nakanishi, Hajime; Shimoji, Kazuaki; Karagiozov, Kostadin; Miyajima, Masakazu; Arai, Hajime

    2014-01-01

    A girl aged 2 years 10 months suddenly went into a deep coma and demonstrated left hemiplegia. At birth, she had exhibited a left-sided facial port-wine stain typical of Sturge-Weber syndrome (SWS) and involving the V1 and V2 distributions of the trigeminal nerve. Computed tomography showed a right thalamic hemorrhage with acute hydrocephalus. Magnetic resonance imaging with Gd enhancement 8 months before the hemorrhage had shown a patent superior sagittal sinus (SSS) and deep venous system. Magnetic resonance imaging and MR angiography studies 2 months before the hemorrhage had revealed obstruction of the SSS and right internal cerebral vein (ICV). Given that a digital subtraction angiography study obtained after the hemorrhage did not show the SSS or right ICV, the authors assumed that impaired drainage was present in the deep venous system at that stage. The authors speculated that the patient's venous drainage pattern underwent compensatory changes because of the occluded SSS and deep venous collectors, shifting outflow through other cortical venous channels to nonoccluded dural sinuses. Sudden congestion (nearly total to total obstruction) of the ICV may have caused the thalamic hemorrhage in this case, which is the first reported instance of pediatric SWS with intracerebral hemorrhage and no other vascular lesion. Findings suggested that the appearance of major venous sinus occlusion in a child with SWS could be a warning sign of hemorrhage. PMID:24160667

  9. Childhood allergic bronchopulmonary aspergillosis presenting as a middle lobe syndrome.

    PubMed

    Shah, Ashok; Gera, Kamal; Panjabi, Chandramani

    2016-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) is infrequently documented in children with asthma. Although collapse is not uncommon, middle lobe syndrome (MLS) as a presentation of ABPA is rather a rarity. A 9-year-old female child with asthma presented with increase in intensity of symptoms along with a right midzone patchy consolidation on a chest radiograph. In addition, an ill-defined opacity abutting the right cardiac border with loss of cardiac silhouette was noted. A right lateral view confirmed a MLS, which was further corroborated by high resolution computed tomography. Central bronchiectasis was also observed, which prompted a work-up for ABPA. The child met 7/8 major diagnostic criteria for ABPA. She was then initiated on oral prednisolone that resulted in a marked clinical improvement within a fortnight. Radiological clearance occurred at 3 months with inflation of the middle lobe. ABPA presenting with MLS in a child is yet to be reported. A high index of suspicion is required to establish the diagnosis of ABPA in a child presenting with MLS. This would obviate the invasive investigations usually done to ascertain the cause of MLS. PMID:26844222

  10. Neurologic complications of cerebral angiography in childhood moyamoya syndrome.

    PubMed

    Robertson, R L; Chavali, R V; Robson, C D; Barnes, P D; Eldredge, E A; Burrows, P E; Scott, R M

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. PMID:9799310

  11. Hyperlipidemic profiles during remission in childhood idiopathic nephrotic syndrome.

    PubMed

    Mérouani, A; Lévy, E; Mongeau, J-G; Robitaille, P; Lambert, M; Delvin, E E

    2003-10-01

    Hyperlipidemia, an important characteristic of idiopathic nephrotic syndrome in children (NS), is usually observed during the active phase of the disease and disappears with the resolution of the proteinuria. However, persisting lipid anomalies during remission have been reported in a few studies and raise the question of the later development of atherosclerosis. Plasma lipid profiles in 25 children with NS at remission, with or without active prednisone treatment, were compared with those of an age-matched population. The results indicate that plasma total and LDL-cholesterol levels were above the 95(th) percentile for age and sex in 12 of the 25 patients (48%) with 7 of them having apolipoprotein B and triglyceride concentrations above the 95(th) percentile. Moreover, frequently relapsing children were more likely to have abnormal lipid profile during the remission. We conclude that close monitoring of lipid levels during the remission of the NS especially in those with frequent relapses, is necessary to select the high-risk patients. PMID:14563452

  12. Pathophysiology, Evaluation, and Management of Edema in Childhood Nephrotic Syndrome

    PubMed Central

    Ellis, Demetrius

    2016-01-01

    Generalized edema is a major presenting clinical feature of children with nephrotic syndrome (NS) exemplified by such primary conditions as minimal change disease (MCD). In these children with classical NS and marked proteinuria and hypoalbuminemia, the ensuing tendency to hypovolemia triggers compensatory physiological mechanisms, which enhance renal sodium (Na+) and water retention; this is known as the “underfill hypothesis.” Edema can also occur in secondary forms of NS and several other glomerulonephritides, in which the degree of proteinuria and hypoalbuminemia, are variable. In contrast to MCD, in these latter conditions, the predominant mechanism of edema formation is “primary” or “pathophysiological,” Na+ and water retention; this is known as the “overfill hypothesis.” A major clinical challenge in children with these disorders is to distinguish the predominant mechanism of edema formation, identify other potential contributing factors, and prevent the deleterious effects of diuretic regimens in those with unsuspected reduced effective circulatory volume (i.e., underfill). This article reviews the Starling forces that become altered in NS so as to tip the balance of fluid movement in favor of edema formation. An understanding of these pathomechanisms then serves to formulate a more rational approach to prevention, evaluation, and management of such edema. PMID:26793696

  13. Patients reporting ritual abuse in childhood: a clinical syndrome. Report of 37 cases.

    PubMed

    Young, W C; Sachs, R G; Braun, B G; Watkins, R T

    1991-01-01

    Thirty-seven adult dissociative disorder patients who reported ritual abuse in childhood by satanic cults are described. Patients came from a variety of separate clinical settings and geographical locations and reported a number of similar abuses. The most frequently reported types of ritual abuse are outlined, and a clinical syndrome is presented which includes dissociative states with satanic overtones, severe post-traumatic stress disorder, survivor guilt, bizarre self abuse, unusual fears, sexualization of sadistic impulses, indoctrinated beliefs, and substance abuse. Questions relating to issues of reliability, credibility and verifiability are addressed in depth, and the findings and implications are discussed. PMID:2043970

  14. Polymorphisms in genes of respiratory control and sudden infant death syndrome.

    PubMed

    Läer, Katharina; Dörk, Thilo; Vennemann, Marielle; Rothämel, Thomas; Klintschar, Michael

    2015-09-01

    Sudden infant death syndrome (SIDS) is a multifactorial syndrome and assumingly, among other mechanisms, a deficit in respiratory control leads to a failure of arousal and autoresuscitation when the child is challenged by a stressful homeostatic event, e.g., hypoxia. We hypothesize that genetic polymorphisms involved in respiratory control mediated in the medulla oblongata contribute to SIDS. Therefore, a total of 366 SIDS cases and 421 controls were genotyped for 48 SNPs in 41 candidate genes. Genotyping was performed using Fluidigm nanofluidic technology. Results were obtained for 356 SIDS and 406 controls and 38 SNPs. After correction for multiple testing, one SNP retained a nominally significant association with seasonal SIDS: rs1801030 in the phenol sulfotransferase 1A1 gene (subgroup: death occurring during summer). A borderline association could be also observed for rs563649 in the opioid receptor μ1 gene in a recessive model (subgroup: death occurring during autumn). As a conclusion, although these data suggest two SNPs to be associated with different subgroups of SIDS cases, none of them can fully explain the SIDS condition, consistent with its multifactorial etiology. Given the great complexity of respiratory control and our initial findings reported here, we believe it is worthwhile to further investigate genes involved in the respiratory system. PMID:26198620

  15. The relationships of attachment style and social maladjustment to death ideation in depressed women with a history of childhood sexual abuse.

    PubMed

    Smith, Phillip N; Gamble, Stephanie A; Cort, Natalie A; Ward, Erin A; Conwell, Yeates; Talbot, Nancy L

    2012-01-01

    The current study examined the interaction of attachment orientation and acute social maladjustment as risk factors for death ideation in a sample of women with Major Depression and histories of childhood sexual abuse. Social maladjustment was associated with greater endorsement of death ideation. Avoidant and anxious attachment orientations moderated the social maladjustment and death ideation associations in some domains. Work-related maladjustment was associated with greater odds of death ideation for those with higher attachment avoidance. Parent-role maladjustment was associated with greater odds of death ideation for those with lower attachment anxiety. Findings demonstrate strong associations between death ideation and social maladjustment, and suggest that death ideation may be specific to certain domains of adjustment for anxious and avoidant attachment styles. PMID:22125120

  16. Neuromyelitis Optica in Pregnancy Complicated by Posterior Reversible Encephalopathy Syndrome, Eclampsia and Fetal Death

    PubMed Central

    Igel, Catherine; Garretto, Diana; Robbins, Matthew S; Swerdlow, Michael; Judge, Nancy; Dayal, Ashlesha

    2015-01-01

    Neuromyelitis optica (NMO) is a demyelinating syndrome characterized by optic neuritis and acute myelitis with poor recovery and a progressive course. We report a poor outcome complicated by posterior reversible encephalopathy syndrome (PRES) and eclampsia and review available literature and current evidence for anticipation of adverse fetal and maternal effects. After a pregnancy complicated by multiple admissions for painful NMO exacerbations, a primiparous patient with seropositive NMO presented at 31 + 3/7 weeks with eclampsia, HELLP and subsequent fetal death. MRI confirmed PRES. NMO may be associated with eclampsia and leads to adverse maternal and fetal outcomes. Posited mechanisms include antibody-mediated placental damage and a heightened risk of eclampsia-associated PRES. Further characterization of the course of NMO and its relationship with pregnancy outcomes in larger series would be invaluable. PMID:25584107

  17. Computer polygraphic system for infants at risk for sudden infant death syndrome (SIDS).

    PubMed

    Dove, R; Brown, J; Fright, R; Tuffnell, C; Ford, R

    1990-12-01

    We have designed and developed a suite of equipment for polygraphic assessment of infants thought to be at risk for Sudden Infant Death Syndrome. A range of commercially available and custom made instrumentation is used to monitor cardio-respiratory function and thermal activity. The PC based system records continuous overnight trends and is able to detect apnoea, bradycardia, tachycardia, oxygen desaturation and other significant clinical events, producing summary data and graphs at the conclusion of the monitoring. The system is fully interactive and adaptable to various clinical and research requirements. PMID:2285374

  18. Malnutrition as an underlying cause of childhood deaths associated with infectious diseases in developing countries.

    PubMed Central

    Rice, A. L.; Sacco, L.; Hyder, A.; Black, R. E.

    2000-01-01

    INTRODUCTION: Recent estimates suggest that malnutrition (measured as poor anthropometric status) is associated with about 50% of all deaths among children. Although the association between malnutrition and all-cause mortality is well documented, the malnutrition-related risk of death associated with specific diseases is less well described. We reviewed published literature to examine the evidence for a relation between malnutrition and child mortality from diarrhoea, acute respiratory illness, malaria and measles, conditions that account for over 50% of deaths in children worldwide. METHODS: MEDLINE was searched for suitable review articles and original reports of community-based and hospital-based studies. Findings from cohort studies and case-control studies were reviewed and summarized. RESULTS: The strongest and most consistent relation between malnutrition and an increased risk of death was observed for diarrhoea and acute respiratory infection. The evidence, although limited, also suggests a potentially increased risk for death from malaria. A less consistent association was observed between nutritional status and death from measles. Although some hospital-based studies and case-control studies reported an increased risk of mortality from measles, few community-based studies reported any association. DISCUSSION: The risk of malnutrition-related mortality seems to vary for different diseases. These findings have important implications for the evaluation of nutritional intervention programmes and child survival programmes being implemented in settings with different disease profiles. PMID:11100616

  19. List-learning and verbal memory profiles in childhood epilepsy syndromes.

    PubMed

    Schraegle, William A; Nussbaum, Nancy L; Stefanatos, Arianna K

    2016-09-01

    Findings of material-specific influences on memory performance in pediatric epilepsy are inconsistent and merit further investigation. This study compared 90 children (aged 6years to 16years) with childhood absence epilepsy (CAE), frontal lobe epilepsy (FLE), and temporal lobe epilepsy (TLE) to determine whether they displayed distinct list-learning and verbal memory profiles on the California Verbal Learning Test - Children's Version (CVLT-C). Group comparison identified greater risk of memory impairment in children with TLE and FLE syndromes but not for those with CAE. While children with TLE performed worst overall on Short Delay Free Recall, groups with TLE and FLE performed similarly on Long Delay Free Recall. Contrast indices were then employed to explore these differences. Children with TLE demonstrated a significantly greater retroactive interference (RI) effect compared with groups with FLE and CAE. Conversely, children with FLE demonstrated a significantly worse learning efficiency index (LEI), which compares verbal memory following repetition with initial recall of the same list, than both children with TLE and CAE. These findings indicated shallow encoding related to attentional control for children with FLE and retrieval deficits in children with TLE. Finally, our combined sample showed significantly higher rates of extreme contrast indices (i.e., 1.5 SD difference) compared with the CVLT-C standardization sample. These results underscore the high prevalence of memory dysfunction in pediatric epilepsy and offer support for distinct patterns of verbal memory performance based on childhood epilepsy syndrome. PMID:27484747

  20. Caudate volumes in childhood predict symptom severity in adults with Tourette syndrome

    PubMed Central

    Bloch, Michael H.; Leckman, James F.; Zhu, Hongtu; Peterson, Bradley S.

    2008-01-01

    Background Most children with Tourette syndrome (TS) experience a marked decline in the severity of tic symptoms during adolescence. Currently no clinical measures can predict whose tic symptoms will persist into adulthood. Previous cross-sectional imaging studies have identified reduced caudate nucleus volumes in subjects with TS. Objective To evaluate whether caudate nucleus volumes in childhood can predict the severity of tic or obsessive–compulsive symptoms at follow-up in early adulthood. Methods In a prospective longitudinal study, clinical status and basal ganglia volumes of 43 children with TS were measured on high-resolution magnetic resonance images before age 14 years. Follow-up clinical assessments were conducted after age 16 years, an average of 7.5 years later. Linear regression and Tobit regression analyses were used to assess the association of basal ganglia volumes measured in childhood with the severity of tic and obsessive–compulsive disorder (OCD) symptoms at the time of childhood MRI and at follow-up in early adulthood. Results Volumes of the caudate nucleus correlated significantly and inversely with the severity of tic and OCD symptoms in early adulthood. Caudate volumes did not correlate with the severity of symptoms at the time of the MRI scan. Conclusions Caudate volumes in children with Tourette syndrome predict the severity of tic and obsessive–compulsive symptoms in early adulthood. This study provides compelling evidence that morphologic disturbances of the caudate nucleus within cortico-striatal-thalamo-cortical circuits are central to the persistence of both tics and obsessive–compulsive symptoms into adulthood. PMID:16247053

  1. Bottlenecks, barriers, and solutions: results from multicountry consultations focused on reduction of childhood pneumonia and diarrhoea deaths.

    PubMed

    Gill, Christopher J; Young, Mark; Schroder, Kate; Carvajal-Velez, Liliana; McNabb, Marion; Aboubaker, Samira; Qazi, Shamim; Bhutta, Zulfiqar A

    2013-04-27

    Millions of children still die unnecessarily from pneumonia and diarrhoea, mainly in resource-poor settings. A series of collaborative consultations and workshops involving several hundred academic, public health, governmental and private sector stakeholders were convened to identify the key barriers to progress and to issue recommendations. Bottlenecks impairing access to commodities included antiquated supply management systems, insufficient funding for drugs, inadequate knowledge about interventions by clients and providers, health worker shortages, poor support for training or retention of health workers, and a failure to convert national policies into action plans. Key programmatic barriers included an absence of effective programme coordination between and within partner organisations, scarce financial resources, inadequate training and support for health workers, sporadic availability of key commodities, and suboptimal programme management. However, these problems are solvable. Advocacy could help to mobilise needed resources, raise awareness, and prioritise childhood pneumonia and diarrhoea deaths in the coming decade. PMID:23582720

  2. Atypical "benign" partial epilepsy of childhood or pseudo-lennox syndrome. Part II: family study.

    PubMed

    Doose, H; Hahn, A; Neubauer, B A; Pistohl, J; Stephani, U

    2001-02-01

    Atypical benign partial epilepsy of childhood (ABPE = Pseudo-Lennox syndrome) shows semiologic parallels to Lennox-Gastaut syndrome, however--besides the lack of tonic seizures--it has an entirely different etiology and prognosis. Recently Hahn et al [17] investigated the long-term evolution of 43 cases with ABPE. Symptomatology, EEG findings, and course were found to overlap with Rolandic epilepsy, Landau-Kleffner syndrome and ESES. The incidence of seizures in relatives was determined in the whole series investigated by Hahn et al [17]. Five of 56 siblings suffered from seizures (3 Rolandic seizures; one febrile convulsions; one unclassified). Three fathers reported grand mal. In 29 families of the series of Hahn et al EEG recordings were performed: 22 brothers, 19 sisters and 16 pairs of parents. In 29% of the siblings a sharp wave focus was demonstrable. The rate rose to 40% when only siblings investigated at the age of maximum expression (3 to 10 years) were considered. Sharp wave foci were mostly multifocal and indistinguishable from those observed in siblings of children with Rolandic epilepsy. Photoparoxysmal response and generalized spikes and waves during rest and hyperventilation were also found to be significantly elevated (26% and 13% respectively). We conclude that ABPE is a subgroup of idiopathic partial epilepsy of childhood (representing a less benign part of a spectrum) that has to be ranked in a continuum with Rolandic epilepsy. The different clinical phenotype might be caused by a higher expressivity of the identical genetic trait, possibly facilitated by other genetic or acquired factors. Genetic heterogeneity represents another possibility. PMID:11315204

  3. Risk factors for early death in transient myeloproliferative disorder without phenotypic features of Down syndrome: a case report and literature review.

    PubMed

    Kawase, Koya; Azuma, Eiichi; Ohshita, Hironori; Tanaka, Tatsushi; Hanada, Yu; Sasaki, Tomoaki; Sugimoto, Mari; Togawa, Takao; Kouwaki, Masanori; Ito, Tsuyoshi; Hirayama, Masahiro; Koyama, Norihisa

    2012-08-01

    Not only in newborns with Down syndrome, but newborns without phenotypic features of Down syndrome also develop transient myeloproliferative disorder (TMD). In these cases, trisomy 21 and related chromosomal abnormalities are either constitutionally mosaic or limited to blood cells. Risk factors for early death of these patients are unknown so far. We here report a fatal case of TMD without phenotypic features of Down syndrome and review literature to identify risk factors associated with early death. Not only are gestational age and white blood cell count risk factors for early death in TMD with Down syndrome, but they also appear to be risk factors in TMD without Down syndrome. PMID:22510770

  4. Maternal recall of symptoms associated with childhood deaths in rural east Africa.

    PubMed

    Snow, R W; Basto de Azevedo, I; Forster, D; Mwankuyse, S; Bomu, G; Kassiga, G; Nyamawi, C; Teuscher, T; Marsh, K

    1993-08-01

    Verbal autopsies (VA) are frequently used to determine causes of death for individuals for whom there is no reliable clinical information regarding the terminal illness. VA interviews are used to note key symptoms and signs recalled by relatives of the deceased and diagnoses ascribed according to the symptom complexes. The VA technique assumes that individual disease entities have discrete symptom complexes and that these can be accurately recognized and recalled by the interviewees. We have examined the accuracy with which specific symptoms are recalled over time by mothers or normal guardians of 491 children who died on the paediatric wards of two district hospitals in East Africa. Kwashiorkor, measles, trauma, generalized convulsions and neonatal tetanus were all reported with a high degree of accuracy for children who died of these conditions and had low false positive rates for children without these conditions. Recall was similar within 1 month of death compared to recall after 6 months for most symptoms and signs except neonatal tetanus where false positive reports by mothers increased with time since death. Symptoms and signs commonly used to describe malaria, respiratory tract and diarrhoea-related deaths were reported by mothers to have been present during the terminal illness in 43% of cases where these features were absent. Recall abilities differed between the two communities studied for some symptoms and signs highlighting the importance of such studies in every setting where VA are applied. PMID:8225743

  5. Does Childhood Victimization Increase the Risk of Early Death? A 25-Year Prospective Study.

    ERIC Educational Resources Information Center

    White, Helene Raskin; Widom, Cathy Spatz

    2003-01-01

    This study compared mortality data and causes of death in a sample of 908 abused and/or neglected individuals and 667 matched controls followed for 25 years into young adulthood. The study found no significant differences in rates of mortality for the two groups and victims of child abuse and neglect were not more likely to experience a violent…

  6. Mechanisms of cardiac arrhythmias and sudden death in transgenic rabbits with long QT syndrome

    PubMed Central

    Brunner, Michael; Peng, Xuwen; Liu, Gong Xin; Ren, Xiao-Qin; Ziv, Ohad; Choi, Bum-Rak; Mathur, Rajesh; Hajjiri, Mohammed; Odening, Katja E.; Steinberg, Eric; Folco, Eduardo J.; Pringa, Ekatherini; Centracchio, Jason; Macharzina, Roland R.; Donahay, Tammy; Schofield, Lorraine; Rana, Naveed; Kirk, Malcolm; Mitchell, Gary F.; Poppas, Athena; Zehender, Manfred; Koren, Gideon

    2008-01-01

    Long QT syndrome (LQTS) is a heritable disease associated with ECG QT interval prolongation, ventricular tachycardia, and sudden cardiac death in young patients. Among genotyped individuals, mutations in genes encoding repolarizing K+ channels (LQT1:KCNQ1; LQT2:KCNH2) are present in approximately 90% of affected individuals. Expression of pore mutants of the human genes KCNQ1 (KvLQT1-Y315S) and KCNH2 (HERG-G628S) in the rabbit heart produced transgenic rabbits with a long QT phenotype. Prolongations of QT intervals and action potential durations were due to the elimination of IKs and IKr currents in cardiomyocytes. LQT2 rabbits showed a high incidence of spontaneous sudden cardiac death (>50% at 1 year) due to polymorphic ventricular tachycardia. Optical mapping revealed increased spatial dispersion of repolarization underlying the arrhythmias. Both transgenes caused downregulation of the remaining complementary IKr and IKs without affecting the steady state levels of the native polypeptides. Thus, the elimination of 1 repolarizing current was associated with downregulation of the reciprocal repolarizing current rather than with the compensatory upregulation observed previously in LQTS mouse models. This suggests that mutant KvLQT1 and HERG interacted with the reciprocal wild-type α subunits of rabbit ERG and KvLQT1, respectively. These results have implications for understanding the nature and heterogeneity of cardiac arrhythmias and sudden cardiac death. PMID:18464931

  7. Medullary Serotonin Defects and Respiratory Dysfunction in Sudden Infant Death Syndrome

    PubMed Central

    Paterson, David S; Hilaire, Gerard; Weese-Mayer, Debra E

    2009-01-01

    Sudden infant death syndrome (SIDS) is defined as the sudden and unexpected death of an infant less than 12 months of age that occurs during sleep and remains unexplained after a complete autopsy, death scene investigation, and review of the clinical history. It is the leading cause of postneonatal mortality in the developed world. The cause of SIDS is unknown, but is postulated to involve impairment of brainstem-mediated homeostatic control. Extensive evidence from animal studies indicates that serotonin (5-HT) neurons in the medulla oblongata play a role in the regulation of multiple aspects of respiratory and autonomic function. A subset of SIDS infants have several abnormalities in medullary markers of 5-HT function and genetic polymorphisms impacting the 5-HT system, informing the hypothesis that SIDS results from a defect in 5-HT brainstem-mediated control of respiratory (and autonomic) regulation. Here we review the evidence from postmortem human studies and animal studies to support this hypothesis and discuss how the pathogenesis of SIDS is likely to originate in utero during fetal development. PMID:19481178

  8. Metabolic Syndrome in Childhood: Rare Case of Alstrom Syndrome with Blindness.

    PubMed

    Ahmad, Afzal; D'Souza, Benedicta; Yadav, Charu; Agarwal, Ashish; Kumar, Anand; Nandini, M; D'Souza, Vivian; Poornima, A M; Kamath, Nutan

    2016-10-01

    Alstrom's syndrome (AS) is a rare autosomal recessive ciliopathic condition affecting 1:10,00,000 children. It's a single gene disorder of ALMS1 on chromosome 2 with multisystem involvement with cone-rod retinal dystrophy causing juvenile blindness, obesity, insulin resistance, type 2 Diabetes mellitus, hypogonadism and sensorineural hearing loss. Till now only 800 patients with this disorder has been identified so far. In this report, we describe the case of a 9-year old male boy from south India. He had been initially referred for polyphagia, polyuria, polydipsia, generalized weakness from 1 weeks. On examination he was demonstrated features suggestive of AS, including blindness, obesity, type 2 diabetes, altered lipid profile, hypogonadism, acanthosis nigricans, seborrheic dermatitis, right ear discharge and episodes of respiratory tract infections. So, diagnosis of AS is critical as it can easily be overlooked because of the many features associated with metabolic syndrome starting at age 7, a relatively early age. PMID:27605748

  9. [Parental death in childhood: the state of theoretical knowledge and clinical challenges in the future].

    PubMed

    Mentec, Margaux; Flahault, Cécile

    2015-03-01

    Although children's psychological adaptation to parental cancer is a wide field for psycho-oncological research, few empirical studies target children bereavement specifically following parental cancer. In this paper, our purpose is to make a state of art about literature concerning parental death. Literature dealing with grief concerns psychopathological consequences of parental loss and most recently post-traumatic growth. Although references about support programs have emerged, few of these programs have been scientifically evaluated. This review underlines that more studies are needed with prospective quantitative and qualitative studies, in order to describe more precisely children bereavement process and long term effects of bereavement. Psychological support for other family members and evaluation of support programs seem to be critical to improve children adaptation to parental death. PMID:25732046

  10. When the spirit leaves: Childhood death, grieving, and bereavement in Islam.

    PubMed

    Hedayat, Kamyar

    2006-12-01

    The death of a child has a profound and often long-lasting impact on families. The parent's relationship and their ability to bond with and take care of surviving children may be affected. It is important for healthcare workers to understand the dynamics associated with bereavement, especially when the family comes from a non-Western culture. Islam is one of the three most populous religions along with Christianity and Hinduism and the fastest growing religion in the United States but remains largely misunderstood. This paper seeks to explain what Islam is, who is a Muslim, where they live, and what they believe and practice. It also explains how Islamic beliefs contextualize the meaning of life and death for Muslims and how they are exhorted to grieve upon a child's death. Reading this paper will enable those who care for Muslim families to better attend to the social and emotional needs of Muslim parents and siblings after such a tragic event. PMID:17187536

  11. Unraveling the Enigma of Bangungut: Is Sudden Unexplained Nocturnal Death Syndrome (SUNDS) in the Philippines a Disease Allelic to the Brugada Syndrome?

    PubMed Central

    Gaw, Albert C.; Lee, Byron; Gervacio-Domingo, Giselle; Antzelevitch, Charles; Divinagracia, Romeo; Jocano, Felipe

    2012-01-01

    Background Sudden unexplained nocturnal death syndrome (SUNDS) has been reported worldwide. SUNDS is endemic in Southeast Asia and is colloquially known as Bangungut in the Philippines, Lai Tai in Thailand, and Pokkuri in Japan. Although SUNDS in Thailand and Japan have been determined to be phenotypically, genetically and functionally identical to the Brugada syndrome, the relationship between Bangungut/SUNDS in the Philippines and the Brugada syndrome has not been clarified. This paper explores the concordance between Bangungut/SUNDS and the Brugada syndrome. Methods We summarized autopsy studies on Bangungut retrieved from PubMed since 1917 and current epidemiological data on Philippine SUNDS to clarify its diagnostic features. We also reviewed current hypotheses of the pathophysiological mechanism of the Brugada syndrome to explore its applicability to Bangungut/SUNDS. Results The use of the term Bangungut is confusing as it includes many diseases that may cause SUNDS. However, our review reveals a notable subset of Bangungut, identified as Bangungut/SUNDS with no gross cardiac pathology that conforms to the clinical picture of the folk-belief of Bangungut and of the Brugada syndrome, namely: predominance among male in the 20-40 age range; sudden death during sleep or at rest, usually following ingestion of a large meal at night; and victims were in apparent good health prior to their demise. Current pathophysiological mechanisms of Brugada syndrome seemed plausible explanations for a majority of this subset of Bangungut/SUNDS. Conclusion Bangungut/SUNDS and the Brugada syndrome appear closely related. Pathophysiological mechanisms of the Brugada syndrome may explain the enigma of Bangungut/SUND. Whether Bangungut/SUNDS is phenotypically, genetically and functionally an allele of the Brugada syndrome remains inconclusive due to lack of research data. We therefore proposed a research agenda including genetic testing and pharmacological challenge of probands and

  12. Cot Deaths.

    ERIC Educational Resources Information Center

    Tyrrell, Shelagh

    1985-01-01

    Addresses the tragedy of crib deaths, giving particular attention to causes, prevention, and medical research on Sudden Infant Death Syndrome (SIDS). Gives anecdotal accounts of coping strategies used by parents and families of SIDS infants. (DT)

  13. Reversible posterior leukoencephalopathy syndrome in childhood: report of nine cases and review of the literature.

    PubMed

    Gümüş, Hakan; Per, Hüseyin; Kumandaş, Sefer; Yikilmaz, Ali

    2010-04-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is recently described disorder with typical radiological findings in the posterior regions of the cerebral hemisphere and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities, such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting and abnormalities of visual perception like cortical blindness. RPLS is caused by various heterogeneous factors, the commonest being hypertension, followed by non-hypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. We presented nine patients with RPLS who had primary diagnoses such as acute post-streptococcal glomerulonephritis, idiopathic hypertension, the performing of intravenous immunoglobulin for infection with crescentic glomerulonephritis, erythrocyte transfusion for severe iron deficiency, L: -asparaginase treatment for acute lymphoblastic leukemia and performing of granulocyte-colony stimulating factor for ulcerative colitis due to neutropenia. Early recognition of RPLS as complication during different diseases and therapy in childhood may facilitate precise diagnosis and appropriate treatment. PMID:19809787

  14. [Markers of metabolic syndrome and peptides regulating metabolism in survivors of childhood acute lymphoblastic leukemia].

    PubMed

    Skoczeń, Szymon; Tomasik, Przemysław; Balwierz, Walentyna; Surmiak, Marcin; Sztefko, Krystyna; Galicka-Latała, Danuta

    2011-01-01

    Along with the growing epidemic of overweight the risk of atherosclerosis, cardiovascular disease morbidity and mortality are increasing markedly. Metabolic syndrome (MS) is a condition clustering together several risk factors of those complications such as visceral obesity, glucose intolerance, arterial hypertension and dislipidemia. The risk of obesity in acute lymphoblastic leukemia (ALL) survivors is higher than in general population. We aimed to assess (1) the relationships between chosen adipokines and neuropeptides, chemotherapy, CRT, and body fatness and (2) evaluate adipokines and neuropeptides concentrations as a new markers of MS in children. We conducted cross-sectional evaluation of 82 ALL survivors (median age: 13.2 years; range: 4,8-26,2; median time from treatment: 3.2 years), including fasting laboratory testing: peptides (leptin, GLP-1, orexin, PYY, apelin), total cholesterol and its fractions, triglycerides; anthropometric measurements (weight, height), systolic and diastolic blood pressure. We estimated percentiles of body mass index and percentiles of blood pressure. Between 82 survivors overweight and diastolic hypertension was diagnosed in 31% of patients (35% in CRT group) and 15% respectively. At least one abnormality in lipids concentrations was found in 43%. Girls were more affected than boys. Statistically significant increased in leptin and apelin concentrations and decreased in soluble leptin receptor concentrations in the overweight group were observed compared to the non overweight subjects. Significant increase in orexin levels in females who had received CRT compared to those who had not received CRT was found. CRT is the main risk factor of elevated of body mass among survivors of childhood leukemia. Dyslipidemia and hypertension, along with increased adiposity indicate higher risk of MS development. Girls are more affected than boys. Leptin, orexin and apelin seem to be good markers of increased adiposity especially after CRT

  15. Treatment-related deaths in second complete remission in childhood acute myeloid leukaemia.

    PubMed

    Molgaard-Hansen, Lene; Möttönen, Merja; Glosli, Heidi; Jónmundsson, Guðmundur K; Abrahamsson, Jonas; Hasle, Henrik

    2011-03-01

    The frequency and causes of treatment-related deaths (TRD) in second complete remission (CR2) in acute myeloid leukaemia (AML) were investigated in a historical, prospective cohort study of 429 children included in the Nordic Society of Paediatric Haematology and Oncology (NOPHO)-AML-88 and -93 trials. Relapse occurred in 158 children (39%). Seventeen (18%) of the 96 patients entering CR2 suffered TRD. The main causes were infection (59%) and complications from graft-versus-host disease (22%). Fourteen (82%) of 17 TRDs occurred in children undergoing haematopoietic stem cell transplantations (HSCT). Optimal supportive care after HSCT is essential, and studies on risk factors for TRD are needed. PMID:21241281

  16. Nurses' Knowledge and Adherence To Sudden Infant Death Syndrome Prevention Guidelines.

    PubMed

    Bartlow, Kendra L; Cartwright, Sara B; Shefferly, Erin K

    2016-01-01

    The American Academy of Pediatrics (AAP) defines standard guidelines for infant positioning and sleep environment to reduce the rate of sudden infant death syndrome (SIDS), but recent data on nurses' knowledge and adherence to these guidelines in hospital settings are limited. An observational, quantitative, and descriptive study was conducted on well-baby postpartum nurseries at two urban Washington, DC, hospitals. Sixty-six direct observations of infant position and crib environment were conducted, and a 17-question survey was administered to determine nurses' knowledge and practice regarding AAP SIDS prevention guidelines. Of observed sleeping conditions, 69.7% failed the guidelines for infant positioning, crib environment, or both, despite nurses' reporting knowledge of the AAP guidelines. Further research is needed to determine if the study's findings are consistent with hospitals elsewhere, and to better understand the disconnect between nurses' knowledge and behavior regarding SIDS prevention guidelines. PMID:27019936

  17. Meaning-making in the aftermath of sudden infant death syndrome.

    PubMed

    Krueger, Guenther

    2006-09-01

    The reconstruction of meaning in the aftermath of sudden infant death syndrome (SIDS) is part of the grieving process but has to date been poorly understood. Earlier theorists including Freud, Bowlby and Kübler-Ross provided a foundation for what occurs during this time using stage theories. More recent researchers, often using qualitative techniques, have provided a more complex and expanded view that enhances our knowledge of meaning reconstruction following infant loss. This overview of representative contemporary authors compares and contrasts them with the longstanding models that are being supplanted within the emerging field of thanatology. Understanding parental reactions within this new framework can help healthcare professionals in dealing with those affected by SIDS and provide a more empathic and sensitive approach to individual differences. Parents' own accounts of their post-SIDS experience are consistent with these newer theories. Comprehending how parents cope and reconstruct their lives is an important element in providing appropriate psychological support services. PMID:16918783

  18. Interleukin-2 as a neuromodulator possibly implicated in the physiopathology of sudden infant death syndrome.

    PubMed

    Kadhim, Hazim; Deltenre, Paul; De Prez, Carine; Sébire, Guillaume

    2010-08-16

    Dysfunction in vital brainstem centers, including those controlling cardiorespiratory- and sleep/arousal pathophysiology, is reported in sudden infant death syndrome (SIDS). Biological mechanisms underlying SIDS, however, remain unclear. Cytokines are inter-cellular signaling chemicals. They can interact with neurotransmitters and might thus modify neural and neuroimmune functions. Cytokines could therefore act as neuromodulators. Interleukin (IL)-2 is a major immune-related cytokine. It has not been previously depicted in vital brainstem centers. We detected intense neuronal IL-2 immune-reactivity in the SIDS brainstem, namely in vital neural centers. This IL-2 overexpression might interfere with neurotransmitters in those critical brainstem centers, causing disturbed homeostatic control of cardiorespiratory and arousal responses, possibly leading to SIDS. PMID:20542085

  19. Near-Death Experiences in patients with locked-in syndrome: Not always a blissful journey.

    PubMed

    Charland-Verville, Vanessa; Lugo, Zulay; Jourdan, Jean-Pierre; Donneau, Anne-Françoise; Laureys, Steven

    2015-07-01

    Memories of Near-Death Experiences (NDEs) most often are recounted as emotionally positive events. At present, no satisfactory explanatory model exists to fully account for the rich phenomenology of NDEs following a severe acute brain injury. The particular population of patients with locked-in syndrome (LIS) provides a unique opportunity to study NDEs following infratentorial brain lesions. We here retrospectively characterized the content of NDEs in 8 patients with LIS caused by an acute brainstem lesion (i.e., "LIS NDEs") and 23 NDE experiencers after coma with supratentorial lesions (i.e., "classical NDEs"). Compared to "classical NDEs", "LIS NDEs" less frequently experienced a feeling of peacefulness or well-being. It could be hypothesized that NDEs containing less positive emotions might have a specific neuroanatomical substrate related to impaired pontine/paralimbic connectivity or alternatively might be related to the emotional distress caused by the presence of conscious awareness in a paralyzed body. PMID:25837796

  20. A case control study on autopsy findings in sudden unexplained nocturnal death syndrome

    PubMed Central

    Gervacio, G; Lim, M; Reganit, P; Encinas, M; Macapugay, L; Palmero, J; Nierras, C; De los Reyes, C; Geronimo, F

    2014-01-01

    Aim Sudden unexplained nocturnal death syndrome (SUNDS) has been linked to the Brugada syndrome. In some places, acute haemorrhagic pancreatitis is widely held to cause it. We conducted a systematic, controlled autopsy study on Filipino SUNDS victims to rule out structural heart findings as well as acute haemorrhagic pancreatitis as causes. Methods and results A case control autopsy study was conducted comparing SUNDS victims between 18 and 50 years of age who died within 1 h of symptom onset with age- and gender-matched controls. There were 24 SUNDS (mean age 34.5 years) and 24 controls (mean 32.7 years). The autopsy incidence of structural heart disease was 8.3% (95% CI (1% to 27%)) and focal pancreatic haemorrhage was 4.17% (95% CI (0.1% to 20%)) but zero for true acute haemorrhagic pancreatitis among SUNDS victims. Autopsy findings in SUNDS versus controls were not significantly different from each other, showing no diagnostic abnormality in any of the organs. There was no significant difference in the incidence of acute haemorrhagic pancreatitis in both the SUNDS and control groups. We did not find fetal dispersion of the atrioventricular (AV) node, sclerosis or fibrosis of the AV conduction system, in a substudy of SUNDS cases. Conclusions We have shown that there is no significant difference in the overall autopsy findings between SUNDS and controls. Autopsy findings were normal in 70% of SUNDS; no cardiac structural pathology was found in 87% of cases. Haemorrhagic pancreatitis is the cause of death in a minority of SUNDS. The cardiac conduction system is normal in a subgroup of SUNDS studied. PMID:27326155

  1. Kcne2 Deletion Creates a Multisystem Syndrome Predisposing to Sudden Cardiac Death

    PubMed Central

    Hu, Zhaoyang; Kant, Ritu; Anand, Marie; King, Elizabeth C.; Krogh-Madsen, Trine; Christini, David J.; Abbott, Geoffrey W.

    2014-01-01

    Background Sudden cardiac death (SCD) is the leading global cause of mortality, exhibiting increased incidence in diabetics. Ion channel gene perturbations provide a well-established ventricular arrhythmogenic substrate for SCD. However, most arrhythmia susceptibility genes - including the KCNE2 K+ channel β subunit - are expressed in multiple tissues, suggesting potential multiplex SCD substrates. Methods and Results Using “whole transcript” transcriptomics, we uncovered cardiac angiotensinogen upregulation and remodeling of cardiac angiotensinogen interaction networks in P21 Kcne2−/− mouse pups, and adrenal remodeling consistent with metabolic syndrome in adult Kcne2−/− mice. This led to the discovery that Kcne2 disruption causes multiple acknowledged SCD substrates of extracardiac origin: diabetes, hypercholesterolemia, hyperkalemia, anemia and elevated angiotensin II. Kcne2 deletion was also prerequisite for aging-dependent QT prolongation, ventricular fibrillation and SCD immediately following transient ischemia, and fasting-dependent hypoglycemia, myocardial ischemia and atrioventricular block. Conclusions Disruption of a single, widely expressed arrhythmia susceptibility gene can generate a multisystem syndrome comprising manifold electrical and systemic substrates and triggers of SCD. This paradigm is expected to apply to other arrhythmia susceptibility genes, the majority of which encode ubiquitously expressed ion channel subunits or regulatory proteins. PMID:24403551

  2. Ligninolytic Activity of Fusarium virguliforme (SYN. F. solani f. sp. glycines), the Causal Agent of Soybean Sudden Death Syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Fusarium virguliforme (syn. F. solani f. sp. glycines), a soil-borne fungus, is the causal agent of soybean sudden death syndrome (SDS), one of the most important diseases of soybean. Lignin degradation is not common in most soilborne fungi which are considered to be cellulose degraders only. In thi...

  3. Transcriptome analysis of Fusarium virguliforme provides additional evidence of toxins that contribute to foliar symptoms of soybean sudden death syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Toxins produced by the soil-borne fungus, Fusarium virguliforme, cause foliar symptoms in soybean. The disease in soybean is referred to as soybean sudden death syndrome (SDS). Three toxins produced by the fungus were reported to be associated with SDS foliar symptoms, but none produced identical S...

  4. Effect of fungicide seed treatments on Fusarium virguliforme infection of soybean and development of sudden death syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS), caused by Fusarium virguliforme (Fv), is a major yield-limiting disease of soybean in North America. Infection of soybean seedling roots by Fv results in severe root damage; therefore, fungicide seed treatments could potentially reduce these early-season infections and r...

  5. Restraint related deaths and excited delirium syndrome in Ontario (2004-2011).

    PubMed

    Michaud, Alain

    2016-07-01

    Restraint related death in individuals in excited delirium syndrome (ExDS) is a rare event that has been the subject of controversies for more than 3 decades. The purpose of this retrospective study was to retrieve data on all restraint related deaths (RRD) that occurred in Ontario during an 8-year period and compare them with an earlier study on RRD in ExDS covering the period 1988-1995 in Ontario. The Office of the Chief Coroner of Ontario website was consulted under verdicts and recommendations. The Canadian Legal Information Institute website was used to consult verdict explanations and coroner's summary of evidence. During the period 2004-2011, RRD occurred in 14 individuals in ExDS, a 33% reduction. Psychiatric illness as a cause of ExDS decreased from 57% to 14%. Cocaine was the cause of ExDS in 11 (79%) individuals. The number of RRD following a violent encounter in cocaine-induced ExDS (8) was identical in the 2 periods. RRD occurred in 6 individuals without ExDS following a violent encounter. Final restraint position preceding cardiorespiratory arrest was available in 36% of individuals with ExDS and 83% of individuals without ExDS. In both groups, cardiorespiratory arrests could be classified as immediate or delayed. All 4 individuals without ExDS who had immediate cardiorespiratory arrests were restrained in the prone position. Delayed cardiorespiratory arrest occurred in the non-prone position in both groups. Although many hypotheses may be put forward to explain changes in the epidemiology of RRD in ExDS in Ontario, multiple warnings and recommendations from coroners' inquests cannot be ignored. There is probably not a unique pathophysiological pathway leading to cardiorespiratory arrest in RRD. The death rate in RRD in ExDS is so low that drawing any conclusions based on statistical studies or on isolated case report could be hazardous. PMID:27126837

  6. Sudden unexplained nocturnal death syndrome in southern China: epidemiological survey and SCN5A gene screening

    PubMed Central

    Cheng, Jianding; Makielski, Jonathan C.; Yuan, Ping; Shi, Nianqing; Zhou, Feng; Ye, Bin; Tan, Bi-Hua; Kroboth, Stacie; Tang, Shuangbo; Lu, Ciyong

    2010-01-01

    Based on autopsy data collected in Southern China from 2001–2006, 975 cases of sudden unexplained nocturnal death syndrome (SUNDS) were surveyed. Genetic screening of SCN5A, the gene encoding the voltage dependent cardiac Na channel, was performed in 74 available SUNDS cases. The annual occurrence rate of SUNDS in the area was estimated to be 1 per 100,000 people. 80.6% of deaths occurred between the ages of 21 to 40 years and the case number peaked at age 30 years. In 75.4% of cases where witnesses were present, victims died in their sleep between 11 PM and 4 AM and many showed abrupt respiratory distress shortly preceding death. The monthly distribution of emergency fever cases in the area during the same period was positively correlated to that of SUNDS cases (rs = 0.611, P = 0.0025). Four polymorphisms in SCN5A were identified in both SUNDS and control groups. Compared with controls, the allele frequency of C5457 and C3666+69 were significant higher in SUNDS (P<0.005) while the genotypes of both 5457CC (P=0.012, OR=2.0, 95% CI=1.3–3.2) and 3666+69CC (P=0.004, OR=2.1, 95% CI=1.3–3.3) in SUNDS cases were significantly higher. This is the first report of an epidemiological survey and SCN5A gene screening in SUNDS in the Han population of China. The genotypes of 5457CC and 3666+69CC in SCN5A gene may be Chinese SUNDS susceptible polymorphisms. PMID:20110800

  7. Childhood stunting and the metabolic syndrome components in young adults from a Brazilian birth cohort study

    PubMed Central

    Grillo, L P; Gigante, D P; Horta, B L; de Barros, F C F

    2016-01-01

    Background/Objectives: The aim of this study was to investigate the association between stunting in the second year of life and metabolic syndrome components in early adulthood among subjects who have been prospectively followed-up since birth, in a city in Southern Brazil. Subjects/Methods: In 1984, we attempted to follow-up the entire cohort; the subjects were examined and their mothers interviewed. Stunting was defined by a length-for-age Z-score 2 s.d. or more below the mean, in accordance with the World Health Organization reference. Between 2004 and 2005, we again tried to follow the entire cohort; during this period the subjects were evaluated for the following metabolic syndrome components: high-density lipoprotein (HDL) cholesterol, triglycerides, random blood glucose, waist circumference and systolic and diastolic blood pressure. Family income at the time of the baby's birth, asset index, mother's education, mother's smoking during pregnancy and duration of breastfeeding were considered possible confounders. Linear regression was used in the unadjusted and adjusted analyses. Results: Among men, stunting was inversely associated with triglycerides (β=−11.90, confidence interval (CI)=−22.33 to −1.48) and waist circumference (β=−4.29, CI=−5.62 to −2.97), whereas for women stunting was negatively related to HDL-cholesterol (β=−4.50, CI=−6.47 to −2.52), triglycerides (β=−9.61, CI=−17.66 to −1.56) and waist circumference (β=−1.14, CI=−4.22 to −1.02). However, after controlling for confounding variables, these associations vanished. Conclusions: The findings suggest that stunting in childhood is not associated with metabolic syndrome components in young adults. PMID:26733042

  8. Long-term Impact of Childhood Adiposity on Adult Metabolic Syndrome Is Modified by Insulin Resistance: The Bogalusa Heart Study

    PubMed Central

    Zhang, Huijie; Zhang, Tao; Li, Shengxu; Li, Ying; Hussain, Azad; Fernandez, Camilo; Harville, Emily; Bazzano, Lydia A.; He, Jiang; Chen, Wei

    2015-01-01

    Childhood adiposity and insulin resistance are well-known risk factors for adult metabolic syndrome (MetS). This study aims to examine whether the association between childhood adiposity and adult MetS is modified by insulin resistance. The cohort consisted of 1,593 black and white subjects, aged 19–50 years at follow-up, who were examined 19 years apart on average as children and adults for MetS variables. The prevalence of adult MetS was compared between the insulin-sensitive obesity and insulin-resistant obesity groups in childhood. Adult MetS prevalence was higher in the insulin-resistant obesity group than in the insulin-sensitive obesity group (34.9% vs. 24.3%, p = 0.008). In multivariable logistic regression analyses adjusted for age, race, gender, and follow-up years, individuals with insulin-resistant obesity in childhood were 1.7 times (p = 0.011) more likely to have MetS 19 years later on average than those with insulin-sensitive obesity in childhood. Odds ratio did not differ significantly between blacks and whites (p = 0.724). ORs for the association of childhood BMI with adult MetS significantly increased with increasing tertiles of childhood HOMA (p < 0.001 for trend). These findings suggest that insulin resistance amplifies the association between childhood adiposity and adult MetS and underscore the importance of preventing both adiposity and insulin resistance in early life. PMID:26640243

  9. The risk of cardiovascular disease in adults who have had childhood nephrotic syndrome.

    PubMed

    Lechner, Brent Lee; Bockenhauer, Detlef; Iragorri, Sandra; Kennedy, Thomas Lyle; Siegel, Norman Joseph

    2004-07-01

    While increased risk of cardiovascular disease (CVD) in patients with hyperlipidemia, chronic kidney disease (CKD), or end-stage renal disease (ESRD) is well documented, transient hyperlipidemia or intermittent renal disease as a consequence of relapsing nephrotic syndrome (NS) has not been studied. To investigate this enigma, 62 patients, between 25 and 53 years of age, who had steroid-responsive/dependent NS during childhood, were identified from the records of the Division of Pediatric Nephrology at Yale School of Medicine. Forty patients were located and contacted to ascertain symptoms or occurrences of CVD via a telephone interview. At the time of follow-up, 23-46 years after cessation of NS, none of these patients had ESRD or CKD. Three patients had experienced a myocardial infarction (MI): a 32-year-old male with a family history of CVD; a 41-year-old male with a history of heavy smoking, hypertension, diabetes mellitus, and elevated cholesterol; a 31-year-old male after a cocaine overdose. The occurrence of events (8%) and mortality from CVD (none) in this cohort of patients is comparable to patients of a similar age in the general population and is lower than that of patients of the same age who are on dialysis. The data suggest that relapsing NS during childhood does not place patients at increased risk for CVD mortality or morbidity compared with the general population. Consequently, it would appear that factors related to persistent proteinuria or renal insufficiency, rather than transient proteinuria and renal disease, contribute to the CVD documented in patients with CKD or ESRD. PMID:15085419

  10. IQ in Childhood and the Metabolic Syndrome in Middle Age: Extended Follow-Up of the 1946 British Birth Cohort Study

    ERIC Educational Resources Information Center

    Richards, Marcus; Black, Stephanie; Mishra, Gita; Gale, Catharine R.; Deary, Ian J.; Batty, David G.

    2009-01-01

    IQ in early adulthood has been inversely associated with risk of the metabolic syndrome in midlife. We tested this association in the British 1946 birth cohort, which assessed IQ at age eight years and ascertained the metabolic syndrome at age 53 years based on modified (non-fasting blood) ATPIII criteria. Childhood IQ was inversely associated…

  11. Transcriptional analysis of soybean root response to Fusarium virguliforme, the causal agent of sudden death syndrome.

    PubMed

    Radwan, Osman; Liu, Yu; Clough, Steven J

    2011-08-01

    Sudden death syndrome (SDS) of soybean can be caused by any of four distinct Fusarium species, with F. virguliforme and F. tucumaniae being the main casual agents in North and South America, respectively. Although the fungal tissue is largely confined to the roots, the fungus releases a toxin that is translocated to leaf tissues, in which it causes interveinal chlorosis and necrosis leading to scorching symptoms and possible defoliation. In this study, we report on an Affymetrix analysis measuring transcript abundances in resistant (PI 567.374) and susceptible (Essex) roots upon infection by F. virguliforme, 5 and 7 days postinoculation. Many of the genes with increased expression were common between resistant and susceptible plants (including genes related to programmed cell death, the phenylpropanoid pathway, defense, signal transduction, and transcription factors), but some genotype-specific expression was noted. Changes in small (sm)RNA levels between inoculated and mock-treated samples were also studied and implicate a role for these molecules in this interaction. In total, 2,467 genes were significantly changing in the experiment, with 1,694 changing in response to the pathogen; 93 smRNA and 42 microRNA that have putative soybean gene targets were identified from infected tissue. Comparing genotypes, 247 genes were uniquely modulating in the resistant host, whereas 378 genes were uniquely modulating in the susceptible host. Comparing locations of differentially expressed genes to known resistant quantitative trait loci as well as identifying smRNA that increased while their putative targets decreased (or vice versa) allowed for the narrowing of candidate SDS defense-associated genes. PMID:21751852

  12. Neonatal carnitine palmitoyltransferase II deficiency associated with Dandy-Walker syndrome and sudden death.

    PubMed

    Yahyaoui, Raquel; Espinosa, María Gracia; Gómez, Celia; Dayaldasani, Anita; Rueda, Inmaculada; Roldán, Ana; Ugarte, Magdalena; Lastra, Gonzalo; Pérez, Vidal

    2011-11-01

    Neonatal onset of carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal recessive, often lethal disorder of the mitochondrial beta-oxidation of long-chain fatty acids. It is a rare multiorgan disease which includes hypoketotic hypoglycemia, severe hepatomuscular symptoms, cardiac abnormalities, seizures and lethargy, as well as dysmorphic features. Until now, only 22 affected families have been described in the literature. An increasing number of mutations are being identified in the CPT2 gene, with a distinct genotype-phenotype correlation in most cases. Herein we report a new case of neonatal CPT II deficiency associated with Dandy-Walker syndrome and sudden death at 13 days of life. CPT II deficiency was suggested by acylcarnitine analysis of dried-blood on filter paper in the expanded newborn screening. Genetic analysis of the CPT2 gene identified the presence of a previously described mutation in homozygosity (c.534_558del25bpinsT). All lethal neonatal CPT II deficiency patients previously described presented severe symptoms during the first week of life, although this was not the case in our patient, who remained stable and without apparent vital risk during the first 11 days of life. The introduction of tandem mass spectrometry to newborn screening has substantially improved our ability to detect metabolic diseases in the newborn period. This case illustrates the value of expanded newborn screening in a neonate with an unusual clinical presentation, combining hydrocephalus and sudden death, that might not commonly lead to the suspicion of an inborn error of metabolism. PMID:21641254

  13. Using a pacifier to decrease sudden infant death syndrome: an emergency department educational intervention.

    PubMed

    Walsh, Paul; Vieth, Teri; Rodriguez, Carolina; Lona, Nicole; Molina, Rogelio; Habebo, Emnet; Caldera, Enrique; Garcia, Cynthia; Veazey, Gregory

    2014-01-01

    Background. Pacifier use decreases the risk of sudden infant death syndrome (SIDS). An emergency department (ED) visit may provide an opportunistic 'teachable moment' for parents. Objectives. To test the hypotheses (1) that caregivers were less familiar with the role of pacifiers in sudden infant death (SIDS) prevention than other recommendations, and (2) that an ED educational intervention would increase pacifier use in infants younger than six months, and (3) that otitis media would not occur more frequently in pacifier users. Methods. We did an intervention-group-only longitudinal study in a county hospital ED. We measured pacifier use infants and baseline knowledge of SIDs prevention recommendations in caregivers. We followed up three months later to determine pacifier use, and 12 months later to determine episodes of otitis media. Results. We analyzed data for 780 infants. Parents knew of advice against co-sleeping in 469/780 (60%), smoking in 660/776 (85%), and prone sleeping in 613/780 (79%). Only 268/777 (35%) knew the recommendation to offer a pacifier at bedtime. At enrollment 449/780 (58%) did not use a pacifier. Of 210/338 infants aged less than 6 months followed up 41/112 (37%) non-users had started using a pacifier at bedtime (NNT 3). Over the same period, 37/98 (38%) users had discontinued their pacifier. Otitis media did not differ between users and non-users at 12 months. Conclusion. Caregiver knowledge of the role of pacifiers in SIDS prevention was less than for other recommendations. Our educational intervention appeared to increase pacifier use. Pacifier use was not associated with increased otitis media. PMID:24688883

  14. UK survey of broiler ascites and sudden death syndromes in 1993.

    PubMed

    Maxwell, M H; Robertson, G W

    1998-05-01

    1. The mean incidence of deaths from ascites in the UK in 1993 was 1.4% (0.7% in 1991 and 0.9% in 1992) and 0.8% from sudden death syndrome (SDS). In total, the economic loss to the UK Broiler Industry in 1993 as a result of these 2 conditions was 24 Pounds M. 2. Clear geographical differences emerged in the occurrence of ascites, with, not only the lowest incidences being observed in Northern Ireland, but also the peak of the mortality from ascites occurring much later in the rearing cycle than in other regions on the mainland. 3. In all regions the incidence of SDS was lower than that of ascites but the reason for this disparity remains to be established. 4. Some of the variables associated with the road transportation of day-old chicks from the hatchery to the farm appeared to influence the incidence of ascites. These included distance or time travelled, stocking density, internal lorry temperature and the length of time the lorry was heated before transport as well as the time the shed was heated before chick arrival. Temperature was also an important factor during growth (brooding and finishing). 5. Negative pressure-powered ventilation was preferred in most organisations but more ascites was seen with positive pressure ventilation. However, the lowest incidence of ascites occurred with natural ventilation. There was more ascites relative to shed orientation when the wind direction was from the west compared to the east. 6. This survey identifies the extent of the problem of broiler ascites in the UK and also highlights the importance of good management control of day-old chicks, not only following placement, but even before their arrival on the farm. PMID:9649872

  15. Identification of rare variants of DSP gene in Sudden Unexplained Nocturnal Death Syndrome in the southern Chinese Han population

    PubMed Central

    Zhao, Qianhao; Chen, Yili; Peng, Longlun; Gao, Rui; Liu, Nian; Jiang, Pingping; Liu, Chao; Tang, Shuangbo

    2016-01-01

    Sudden unexplained nocturnal death syndrome (SUNDS) is a perplexing disorder to both forensic pathologists and clinic physicians. Desmoplakin (DSP) gene was the first desmosomal gene linked to arrhythmogenic right ventricular cardiomyopathy (ARVC) which was associated with sudden death. To identify the genetic variants of the DSP gene in SUNDS in the southern Chinese Han population, we genetically screened the DSP gene in 40 sporadic SUNDS victims, 16 Brugada syndrome (BrS) patients and 2 Early Repolarization syndrome (ERS) patients using Next Generation Sequencing (NSG) and direct Sanger sequencing. A total of 10 genetic variants of the DSP gene were detected in 11 cases, comprised of two novel missense mutations (p.I125F and p.D521A) and eight previously reported rare variants. Of eight reported variants, two were previously considered pathogenic (p.Q90R and p.R2639Q), three were predicted in silico to bepathogenic (p.R315C, p.E1357D and p.D2579H), and the rest three were predicted to be benign (p.N1234S, p.R1308Q and p.T2267S). This is the first report of DSP genetic screening in Chinese SUNDS and Brugada syndrome. Our results implies that DSP mutations contribute to the genetic cause of some SUNDS victims and maybe a new susceptible gene for Brugada syndrome. PMID:26585738

  16. Identification of rare variants of DSP gene in sudden unexplained nocturnal death syndrome in the southern Chinese Han population.

    PubMed

    Zhao, Qianhao; Chen, Yili; Peng, Longlun; Gao, Rui; Liu, Nian; Jiang, Pingping; Liu, Chao; Tang, Shuangbo; Quan, Li; Makielski, Jonathan C; Cheng, Jianding

    2016-03-01

    Sudden unexplained nocturnal death syndrome (SUNDS) is a perplexing disorder to both forensic pathologists and clinic physicians. Desmoplakin (DSP) gene was the first desmosomal gene linked to arrhythmogenic right ventricular cardiomyopathy (ARVC) which was associated with sudden death. To identify the genetic variants of the DSP gene in SUNDS in the southern Chinese Han population, we genetically screened the DSP gene in 40 sporadic SUNDS victims, 16 Brugada syndrome (BrS) patients, and 2 early repolarization syndrome (ERS) patients using next generation sequencing (NSG) and direct Sanger sequencing. A total of 10 genetic variants of the DSP gene were detected in 11 cases, comprised of two novel missense mutations (p.I125F and p.D521A) and eight previously reported rare variants. Of eight reported variants, two were previously considered pathogenic (p.Q90R and p.R2639Q), three were predicted in silico to be pathogenic (p.R315C, p.E1357D and p.D2579H), and the rest three were predicted to be benign (p.N1234S, p.R1308Q, and p.T2267S). This is the first report of DSP genetic screening in Chinese SUNDS and Brugada syndrome. Our results imply that DSP mutations contribute to the genetic cause of some SUNDS victims and maybe a new susceptible gene for Brugada syndrome. PMID:26585738

  17. Inherited arrhythmia syndromes leading to sudden cardiac death in the young: A global update and an Indian perspective

    PubMed Central

    Chockalingam, Priya; Wilde, Arthur A.

    2014-01-01

    Inherited primary arrhythmias, namely congenital long QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia, account for a significant proportion of sudden cardiac deaths in young and apparently healthy individuals. Genetic testing plays an integral role in the diagnosis, risk-stratification and treatment of probands and family members. It is increasingly obvious that collaborative efforts are required to understand and manage these relatively rare but potentially lethal diseases. This article aims to update readers on the recent developments in our knowledge of inherited arrhythmias and to lay the foundation for a national synergistic effort to characterize them in the Indian population. PMID:24568830

  18. Sexual reproduction in the soybean sudden death syndrome pathogen Fusarium tucumaniae.

    PubMed

    Covert, S F; Aoki, T; O'Donnell, K; Starkey, D; Holliday, A; Geiser, D M; Cheung, F; Town, C; Strom, A; Juba, J; Scandiani, M; Yang, X B

    2007-08-01

    We investigated the sexual reproductive mode of the two most important etiological agents of soybean sudden death syndrome, Fusarium tucumaniae and Fusarium virguliforme. F. tucumaniae sexual crosses often were highly fertile, making it possible to assign mating type and assess female fertility in 24 South American isolates. These crosses produced red perithecia and oblong-elliptical ascospores, as is typical for sexual members of the F. solani species complex. Genotyping of progeny from three F. tucumaniae crosses confirmed that sexual recombination had occurred. In contrast, pairings among 17 U.S. F. virguliforme isolates never produced perithecia. Inter-species crosses between F. tucumaniae and F. virguliforme, in which infertile perithecia were induced only in one of the two F. tucumaniae mating types, suggest that all U.S. F. virguliforme isolates are of a single mating type. We conclude that the F. tucumaniae life cycle in S. America includes a sexual reproductive mode, and thus this species has greater potential for rapid evolution than the F. virguliforme population in the U.S., which may be exclusively asexual. PMID:17300967

  19. Sudden infant death syndrome and placental disorders: the thyroid-selenium link.

    PubMed

    Reid, G M; Tervit, H

    1997-04-01

    Placental insufficiency, inducing hypoxia-ischaemia, is considered a major cause of neuronal injury and impaired post natal development. Placental insufficiency alters the metabolism of arachidonic acid and its oxidation products. Premature labour and low-birth-weight infants are associated with reduced intrauterine blood-flow and infections of the reproductive tract. Thyroidal activity is depressed in undernutrition (placental insufficiency). Premature infants require extra vitamin C for normal tyrosine metabolism (tyrosine is the thyroxine precursor). Among the symptoms indicating infantile cretinism, which appear during 3-5 months of age are: delayed union of skull bones, torpid behaviour, slow feeding, cyanosis during feeding, excessive sleepiness, enlarged tongue, umbilical herniation, flabby musculature, short stature and delayed development. These symptoms have all been described in low-birth-weight infants and sudden infant death syndrome victims by various authors. Bacteria utilize selenium (at the expense of host tissue). Escherichia coli is among the bacteria invading the reproductive tract. E. coli produce thiouracil and are goitrogenic. Some strains of E. coli produce phospholipase A2 which releases arachidonic acid from phospholipids for prostaglandin synthesis. Phospholipase A2 is more active against peroxidized than non-peroxidized lipids. Bacterial competition for intrauterine selenium and goitrogenic bacterial infections of the reproductive tract during pregnancy, depress thyroid function in the fetus but not in the mother. PMID:9160285

  20. Hypothesis on supine sleep, sudden infant death syndrome reduction and association with increasing autism incidence

    PubMed Central

    Bergman, Nils J

    2016-01-01

    AIM To identify a hypothesis on: Supine sleep, sudden infant death syndrome (SIDS) reduction and association with increasing autism incidence. METHODS Literature was searched for autism spectrum disorder incidence time trends, with correlation of change-points matching supine sleep campaigns. A mechanistic model expanding the hypothesis was constructed based on further review of epidemiological and other literature on autism. RESULTS In five countries (Denmark, United Kingdom, Australia, Israel, United States) with published time trends of autism, change-points coinciding with supine sleep campaigns were identified. The model proposes that supine sleep does not directly cause autism, but increases the likelihood of expression of a subset of autistic criteria in individuals with genetic susceptibility, thereby specifically increasing the incidence of autism without intellectual disability. CONCLUSION Supine sleep is likely a physiological stressor, that does reduce SIDS, but at the cost of impact on emotional and social development in the population, a portion of which will be susceptible to, and consequently express autism. A re-evaluation of all benefits and harms of supine sleep is warranted. If the SIDS mechanism proposed and autism model presented can be verified, the research agenda may be better directed, in order to further decrease SIDS, and reduce autism incidence. PMID:27610351

  1. Assessing Field-Specific Risk of Soybean Sudden Death Syndrome Using Satellite Imagery in Iowa.

    PubMed

    Yang, S; Li, X; Chen, C; Kyveryga, P; Yang, X B

    2016-08-01

    Moderate resolution imaging spectroradiometer (MODIS) satellite imagery from 2004 to 2013 were used to assess the field-specific risks of soybean sudden death syndrome (SDS) caused by Fusarium virguliforme in Iowa. Fields with a high frequency of significant decrease (>10%) of the normalized difference vegetation index (NDVI) observed in late July to middle August on historical imagery were hypothetically considered as high SDS risk. These high-risk fields had higher slopes and shorter distances to flowlines, e.g., creeks and drainages, particularly in the Des Moines lobe. Field data in 2014 showed a significantly higher SDS level in the high-risk fields than fields selected without considering NDVI information. On average, low-risk fields had 10 times lower F. virguliforme soil density, determined by quantitative polymerase chain reaction, compared with other surveyed fields. Ordinal logistic regression identified positive correlations between SDS and slope, June NDVI, and May maximum temperature, but high June maximum temperature hindered SDS. A modeled SDS risk map showed a clear trend of potential disease occurrences across Iowa. Landsat imagery was analyzed similarly, to discuss the ability to utilize higher spatial resolution data. The results demonstrated the great potential of both MODIS and Landsat imagery for SDS field-specific risk assessment. PMID:27070424

  2. The incidence of sudden infant death syndrome in North Carolina's cities and counties: 1972--1974.

    PubMed Central

    Blok, J H

    1978-01-01

    Between January 1, 1972 and December 31, 1974, 534 Sudden Infant Death Syndrome cases were reported in North Carolina. All but the out-of-state cases were mapped by county and city locations to determine if urban or rural cases predominated. The mapping was also undertaken to see if significant spatial variabilities could be detected between the county and city populations of infants at risk. The state had an overall SIDS rate of 2.06 per thousand live births. The mapping revealed that counties had a range from zero to a high of 6.6 and that cities with populations of over 10,000 had SIDS rates which ranged from zero to a high of 10.6. The proportions of SIDS cases occurring in either urban or rural locations roughly approximated the distribution of the state's population, with neither location accounting for disproportionately more cases. The larger cities, however, reported more cases than did their suburbs and the immediately surrounding rural areas. The largest and smallest cities, when grouped accordingly, had the lowest urban SIDS rates. The summary SIDS rates for whites was 1.23 per thousant live births, for blacks it was 3.75, and for Indians it was 6.56 per thousand live births. Images Figure 1 PMID:645982

  3. Sleeping position and sudden infant death syndrome in Norway 1967-91.

    PubMed Central

    Irgens, L M; Markestad, T; Baste, V; Schreuder, P; Skjaerven, R; Oyen, N

    1995-01-01

    OBJECTIVE--To investigate, in a population based national study, the association between sleeping position of infants and the occurrence of sudden infant death syndrome (SIDS). DESIGN--A retrospective survey and registry based ecological study. A questionnaire based surveillance of sleeping position was obtained in a random sample (n = 34,799) and surveillance of SIDS was based on all infants born in Norway 1967-91, surviving the perinatal period. Variables studied from the questionnaire were usual sleeping position (placed), breast feeding at 3 months, and maternal smoking in pregnancy, and from the Medical Birth Registry maternal age, birth order, and birth weight. RESULTS--Proportion of infants sleeping prone increased from 1970 (7.4%) to 1989 (49.1%) and dropped in 1990 (26.8%) and 1991 (28.3%). Occurrence of SIDS increased from 1970 (1.1/1000) to 1989 (2.0) before dropping in 1990 and 1991 (1.1). IMPLICATION AND RELEVANCE OF RESULTS--A cause effect relationship between prone sleeping and SIDS as suggested in previous studies is supported by the present; and so far only, national study of infants' sleeping position. PMID:7618929

  4. Hypoxic-ischaemic encephalopathy after near miss sudden infant death syndrome.

    PubMed

    Constantinou, J E; Gillis, J; Ouvrier, R A; Rahilly, P M

    1989-05-01

    Between 1982 and 1985, 14 infants aged 3-26 weeks presented with severe hypoxic episodes as a result of the 'near miss' sudden infant death syndrome (SIDS). They all had metabolic acidosis, cardiovascular instability, acute renal failure, ischaemic colitis, or acute neurological dysfunction. Investigation of the cause excluded infection and trauma, or a primary metabolic, pulmonary, cardiac, or seizure disorder. Seven infants were deeply comatose on admission, never regained consciousness, and died within 60 hours. A characteristic evolution of hypoxic-ischaemic encephalopathy not previously clearly described after near miss SIDS was seen in the seven who lived. Five of the seven were conscious within one hour of resuscitation and showed a striking interval of near normality before neurological deterioration that was characterised by status epilepticus, deep coma, and brain stem dysfunction from 36-96 hours after the event. A biphasic course was not apparent in the remaining two, each of whom was comatose on admission, though refractory seizures did develop. Computed tomograms of the brain more than a week after the event showed cortical infarction or cerebral atrophy. Six of the survivors, followed up from 16-55 months, have serious residual deficits including spastic quadriplegia, delayed development, cortical blindness, or infantile spasms. PMID:2730124

  5. Heritability of sudden death syndrome and its associated correlations to ascites and body weight in broilers.

    PubMed

    Moghadam, H K; McMillan, I; Chambers, J R; Julian, R J; Tranchant, C C

    2005-02-01

    (1) Genetic parameters for the sudden death syndrome (SDS) were estimated in meat-type chickens. Data were collected over 11 generations of selection for body weight within two distinct breeds (Cornish and White Rock). (2) The animal model was used exclusively with linear methods (LM) to estimate genetic parameters. Heritability (h2) of SDS on the liability scale was 0.30 +/- 0.002 and 0.25 +/- 0.002 in the Cornish and White Rock breeds, respectively. (3) A positive genetic correlation (r(g)) with ascites (AS) was determined (approximately 0.3 +/- 0.006). However, it was not possible to estimate the rg of SDS with body weight because of the low prevalence of the defect trait studied (1.8% in the Cornish and 1-5% in the White Rock). (4) Heritability of SDS calculated using male records only was 0.45 +/- 0.009 and 0.35 +/- 0.009, and r(g) with body weight was 0.30 +/- 0.010 and 0.27 +/- 0.009, in the Cornish and White Rock breeds, respectively. (5) In conclusion, the heart defect investigated was heritable with a positive genetic correlation with AS and body weight. PMID:15835252

  6. Gut Microbiota and Immunity: Possible Role in Sudden Infant Death Syndrome

    PubMed Central

    Goldwater, Paul N.

    2015-01-01

    The gut microbiome influences the development of the immune system of young mammals; the establishment of a normal gut microbiome is thought to be important for the health of the infant during its early development. As the role of bacteria in the causation of sudden infant death syndrome (SIDS) is backed by strong evidence, the balance between host immunity and potential bacterial pathogens is likely to be pivotal. Bacterial colonization of the infant colon is influenced by age, mode of delivery, diet, environment, and antibiotic exposure. The gut microbiome influences several systems including gut integrity and development of the immune system; therefore, gut microflora could be important in protection against bacteria and/or their toxins identified in SIDS infants. The aims of the review are to explore (1) the role of the gut microbiome in relation to the developmentally critical period in which most SIDS cases occur; (2) the mechanisms by which the gut microbiome might induce inflammation resulting in transit of bacteria from the lumen into the bloodstream; and (3) assessment of the clinical, physiological, pathological, and microbiological evidence for bacteremia leading to the final events in SIDS pathogenesis. PMID:26089821

  7. Prevalence of SCN1A mutations in children with suspected Dravet syndrome and intractable childhood epilepsy.

    PubMed

    Wang, Ji-wen; Shi, Xiu-yu; Kurahashi, Hirokazu; Hwang, Su-Kyeong; Ishii, Atsushi; Higurashi, Norimichi; Kaneko, Sunao; Hirose, Shinichi

    2012-12-01

    Mutations of the gene encoding the α1 subunit of neuronal sodium channel, SCN1A, are reported to cause Dravet syndrome (DS). The prevalence of mutations reported in such studies (mainly in clinically confirmed DS) seems high enough to make genetic diagnosis feasible. In fact, commercially operating genetic diagnostic laboratories offering genetic analyses of SCN1A are available. Still, the exact prevalence of mutations of SCN1A remains elusive. Fukuoka University has been serving as a genetic diagnostic laboratory for DS for the last 10 years. In this study, we determined the prevalence of SCN1A mutations (SCN1A, SCN2A, SCN1B and SCN2B) in 448 patients with suspected DS and intractable childhood epilepsy. A total of 192 SCN1A mutations were identified in 188 of 448 patients (42.0%). The frequencies of SCN1A mutations in suspected severe myoclonic epilepsy of infancy (SMEI), its borderline phenotype (SMEB) and intractable epilepsy were 56.2%, 41.9% and 28.9% respectively. In addition, four SCN2A mutations were identified in 4 of 325 patients. No mutations of SCN1B and SCN2B were identified. These results are potentially helpful for the diagnosis of DS at early stage. PMID:23195492

  8. Childhood apraxia of speech without intellectual deficit in a patient with cri du chat syndrome.

    PubMed

    Marignier, Stéphanie; Lesca, Gaetan; Marguin, Jessica; Bussy, Gérald; Sanlaville, Damien; des Portes, Vincent

    2012-06-01

    We report an 11-year-old girl for whom the diagnosis of cri du chat syndrome (CdCS) was made during a genetic investigation of childhood apraxia of speech. The patient presented with the classic chromosome 5 short arm deletion found in CdCS. The microdeletion, characterised using aCGH (array Comparative Genomic Hybridisation), was 12.85 Mb, overlapping the 5p15.2 and 5p15.3 critical regions. CdCS is typically associated with severe mental retardation while this patient had normal intellectual performance, confirmed by normal results from categorisation tasks. This mild phenotype was assessed using a comprehensive cognitive battery. Language evaluation showed normal receptive vocabulary scores, in contrast with obvious oro-facial dyspraxia. Disabled fine motor skills were confirmed as well as weak visuo-spatial reasoning abilities. In conclusion, fine cognitive assessment may be worthwhile for patients with CdCS since good intellectual functioning may be masked by severe speech and gestural dyspraxia, thus requiring specific teaching and rehabilitation strategies. PMID:22510527

  9. Less efficient and costly processes of frontal cortex in childhood chronic fatigue syndrome.

    PubMed

    Mizuno, Kei; Tanaka, Masaaki; Tanabe, Hiroki C; Joudoi, Takako; Kawatani, Junko; Shigihara, Yoshihito; Tomoda, Akemi; Miike, Teruhisa; Imai-Matsumura, Kyoko; Sadato, Norihiro; Watanabe, Yasuyoshi

    2015-01-01

    The ability to divide one's attention deteriorates in patients with childhood chronic fatigue syndrome (CCFS). We conducted a study using a dual verbal task to assess allocation of attentional resources to two simultaneous activities (picking out vowels and reading for story comprehension) and functional magnetic resonance imaging. Patients exhibited a much larger area of activation, recruiting additional frontal areas. The right middle frontal gyrus (MFG), which is included in the dorsolateral prefrontal cortex, of CCFS patients was specifically activated in both the single and dual tasks; this activation level was positively correlated with motivation scores for the tasks and accuracy of story comprehension. In addition, in patients, the dorsal anterior cingulate gyrus (dACC) and left MFG were activated only in the dual task, and activation levels of the dACC and left MFG were positively associated with the motivation and fatigue scores, respectively. Patients with CCFS exhibited a wider area of activated frontal regions related to attentional resources in order to increase their poorer task performance with massive mental effort. This is likely to be less efficient and costly in terms of energy requirements. It seems to be related to the pathophysiology of patients with CCFS and to cause a vicious cycle of further increases in fatigue. PMID:26594619

  10. Frontotemporal Dementia-Like Syndrome Following Recall of Childhood Sexual Abuse.

    PubMed

    Cohen, Lisa J; Brody, David

    2015-06-01

    Numerous psychopathological syndromes have been attributed to posttraumatic stress, both at the time of the trauma and many years later. To date, however, there is little literature on pseudodementia as a delayed traumatic stress response. The authors present a case history of a 50-year-old woman who developed severe cognitive impairment following retrieval of previously forgotten memories of childhood sexual abuse. Her cognitive condition deteriorated rapidly and dramatically. Neuropsychological assessment and clinical presentation led to a diagnosis of frontotemporal dementia (vs. corticobasal degeneration). Detailed neurologic and medical evaluations could not identify any underlying physical cause. Her condition progressively worsened over 9 months, at which point memantine, an N-methyl-D-aspartate receptor antagonist, was begun. The patient regained full functioning over the next year. Although an organic cause could not be ruled out, it was likely that recovery of traumatic memories was contributory to the patient's condition, as ongoing psychotherapy had begun 1 year into the course. If additional cases with similar presentations are reported, such cases would corroborate the notion that persistent, severe, and reversible cognitive impairment constitutes a previously unrecognized and atypical posttraumatic response. PMID:25991053

  11. Less efficient and costly processes of frontal cortex in childhood chronic fatigue syndrome

    PubMed Central

    Mizuno, Kei; Tanaka, Masaaki; Tanabe, Hiroki C.; Joudoi, Takako; Kawatani, Junko; Shigihara, Yoshihito; Tomoda, Akemi; Miike, Teruhisa; Imai-Matsumura, Kyoko; Sadato, Norihiro; Watanabe, Yasuyoshi

    2015-01-01

    The ability to divide one's attention deteriorates in patients with childhood chronic fatigue syndrome (CCFS). We conducted a study using a dual verbal task to assess allocation of attentional resources to two simultaneous activities (picking out vowels and reading for story comprehension) and functional magnetic resonance imaging. Patients exhibited a much larger area of activation, recruiting additional frontal areas. The right middle frontal gyrus (MFG), which is included in the dorsolateral prefrontal cortex, of CCFS patients was specifically activated in both the single and dual tasks; this activation level was positively correlated with motivation scores for the tasks and accuracy of story comprehension. In addition, in patients, the dorsal anterior cingulate gyrus (dACC) and left MFG were activated only in the dual task, and activation levels of the dACC and left MFG were positively associated with the motivation and fatigue scores, respectively. Patients with CCFS exhibited a wider area of activated frontal regions related to attentional resources in order to increase their poorer task performance with massive mental effort. This is likely to be less efficient and costly in terms of energy requirements. It seems to be related to the pathophysiology of patients with CCFS and to cause a vicious cycle of further increases in fatigue. PMID:26594619

  12. Loss of B cells and their precursors is the most constant feature of GATA-2 deficiency in childhood myelodysplastic syndrome

    PubMed Central

    Nováková, Michaela; Žaliová, Markéta; Suková, Martina; Wlodarski, Marcin; Janda, Aleš; Froňková, Eva; Campr, Vít; Lejhancová, Kateřina; Zapletal, Ondřej; Pospíšilová, Dagmar; Černá, Zdeňka; Kuhn, Tomáš; Švec, Peter; Pelková, Vendula; Zemanová, Zuzana; Kerndrup, Gitte; van den Heuvel-Eibrink, Marry; van der Velden, Vincent; Niemeyer, Charlotte; Kalina, Tomáš; Trka, Jan; Starý, Jan; Hrušák, Ondřej; Mejstříková, Ester

    2016-01-01

    GATA-2 deficiency was recently described as common cause of overlapping syndromes of immunodeficiency, lymphedema, familiar myelodysplastic syndrome or acute myeloid leukemia. The aim of our study was to analyze bone marrow and peripheral blood samples of children with myelodysplastic syndrome or aplastic anemia to define prevalence of the GATA2 mutation and to assess whether mutations in GATA-2 transcription factor exhibit specific immunophenotypic features. The prevalence of a GATA2 mutation in a consecutively diagnosed cohort of children was 14% in advanced forms of myelodysplastic syndrome (refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and myelodysplasia-related acute myeloid leukemia), 17% in refractory cytopenia of childhood, and 0% in aplastic anemia. In GATA-2-deficient cases, we found the most profound B-cell lymphopenia, including its progenitors in blood and bone marrow, which correlated with significantly diminished intronRSS-Kde recombination excision circles in comparison to other myelodysplastic syndrome/aplastic anemia cases. The other typical features of GATA-2 deficiency (monocytopenia and natural killer cell lymphopenia) were less discriminative. In conclusion, we suggest screening for GATA2 mutations in pediatric myelodysplastic syndrome, preferentially in patients with impaired B-cell homeostasis in bone marrow and peripheral blood (low number of progenitors, intronRSS-Kde recombination excision circles and naïve cells). PMID:27013649

  13. Loss of B cells and their precursors is the most constant feature of GATA-2 deficiency in childhood myelodysplastic syndrome.

    PubMed

    Nováková, Michaela; Žaliová, Markéta; Suková, Martina; Wlodarski, Marcin; Janda, Aleš; Froňková, Eva; Campr, Vít; Lejhancová, Kateřina; Zapletal, Ondřej; Pospíšilová, Dagmar; Černá, Zdeňka; Kuhn, Tomáš; Švec, Peter; Pelková, Vendula; Zemanová, Zuzana; Kerndrup, Gitte; van den Heuvel-Eibrink, Marry; van der Velden, Vincent; Niemeyer, Charlotte; Kalina, Tomáš; Trka, Jan; Starý, Jan; Hrušák, Ondřej; Mejstříková, Ester

    2016-06-01

    GATA-2 deficiency was recently described as common cause of overlapping syndromes of immunodeficiency, lymphedema, familiar myelodysplastic syndrome or acute myeloid leukemia. The aim of our study was to analyze bone marrow and peripheral blood samples of children with myelodysplastic syndrome or aplastic anemia to define prevalence of the GATA2 mutation and to assess whether mutations in GATA-2 transcription factor exhibit specific immunophenotypic features. The prevalence of a GATA2 mutation in a consecutively diagnosed cohort of children was 14% in advanced forms of myelodysplastic syndrome (refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and myelodysplasia-related acute myeloid leukemia), 17% in refractory cytopenia of childhood, and 0% in aplastic anemia. In GATA-2-deficient cases, we found the most profound B-cell lymphopenia, including its progenitors in blood and bone marrow, which correlated with significantly diminished intronRSS-Kde recombination excision circles in comparison to other myelodysplastic syndrome/aplastic anemia cases. The other typical features of GATA-2 deficiency (monocytopenia and natural killer cell lymphopenia) were less discriminative. In conclusion, we suggest screening for GATA2 mutations in pediatric myelodysplastic syndrome, preferentially in patients with impaired B-cell homeostasis in bone marrow and peripheral blood (low number of progenitors, intronRSS-Kde recombination excision circles and naïve cells). PMID:27013649

  14. Separate loci underlie resistance to root infection and leaf scorch during soybean sudden death syndrome.

    PubMed

    Kazi, S; Shultz, J; Afzal, J; Johnson, J; Njiti, V N; Lightfoot, D A

    2008-05-01

    Soybean [Glycine max (L.) Merr.] cultivars show differences in their resistance to both the leaf scorch and root rot of sudden death syndrome (SDS). The syndrome is caused by root colonization by Fusarium virguliforme (ex. F. solani f. sp. glycines). Root susceptibility combined with reduced leaf scorch resistance has been associated with resistance to Heterodera glycines HG Type 1.3.6.7 (race 14) of the soybean cyst nematode (SCN). In contrast, the rhg1 locus underlying resistance to Hg Type 0 was found clustered with three loci for resistance to SDS leaf scorch and one for root infection. The aims of this study were to compare the inheritance of resistance to leaf scorch and root infection in a population that segregated for resistance to SCN and to identify the underlying quantitative trait loci (QTL). "Hartwig", a cultivar partially resistant to SDS leaf scorch, F. virguliforme root infection and SCN HG Type 1.3.6.7 was crossed with the partially susceptible cultivar "Flyer". Ninety-two F5-derived recombinant inbred lines and 144 markers were used for map development. Four QTL found in earlier studies were confirmed. One contributed resistance to leaf scorch on linkage group (LG) C2 (Satt277; P = 0.004, R2 = 15%). Two on LG G underlay root infection at R8 (Satt038; P = 0.0001 R2 = 28.1%; Satt115; P = 0.003, R2 = 12.9%). The marker Satt038 was linked to rhg1 underlying resistance to SCN Hg Type 0. The fourth QTL was on LG D2 underlying resistance to root infection at R6 (Satt574; P = 0.001, R2 = 10%). That QTL was in an interval previously associated with resistance to both SDS leaf scorch and SCN Hg Type 1.3.6.7. The QTL showed repulsion linkage with resistance to SCN that may explain the relative susceptibility to SDS of some SCN resistant cultivars. One additional QTL was discovered on LG G underlying resistance to SDS leaf scorch measured by disease index (Satt130; P = 0.003, R2 = 13%). The loci and markers will provide tagged alleles with which to improve

  15. A Neonate with Susceptibility to Long QT Syndrome Type 6 who Presented with Ventricular Fibrillation and Sudden Unexpected Infant Death

    PubMed Central

    Sauer, Charles W.; Marc-Aurele, Krishelle L.

    2016-01-01

    Patient: Female, 19-day Final Diagnosis: 19 day old neonate with susceptibility to Long QT syndrome • ventricular fibrillation Symptoms: Cardiac arrest • cardiac arrhythmia • encephalopathy Medication: — Clinical Procedure: Cardioversion Specialty: Pediatrics and Neonatology Objective: Rare disease Background: This is a case of a neonate with susceptibility to long QT syndrome (LQTS) who presented with a sudden unexpected infant death. Experts continue to debate whether universal electrocardiogram (ECG) screening of all newborns is feasible, practical, and cost-effective. Case Report: A 19-day-old neonate was found unresponsive by her mother. ECG showed ventricular fibrillation and a combination of a lidocaine drip plus multiple defibrillations converted the rhythm to normal sinus. Unfortunately, MRI brain imaging showed multiple infarcts and EEG showed burst suppression pattern with frequent seizures; life supportive treatment was stopped and the infant died. Genetic testing revealed two mutations in the KCNE2 gene consistent with susceptibility to LQTS type 6. Conclusions: We believe this case is the first to demonstrate both a precipitating electrocardiographic and genetic cause of death for an infant with LQTS, showing a cause-and-effect relationship between LQTS mutation, ventricular arrhythmia, and death. We wonder whether universal ECG newborn screening to prevent LQTS death could have saved this baby. PMID:27465075

  16. Aluminum toxicity and Ca depletion may enhance cell death of tobacco cells via similar syndrome

    PubMed Central

    Matsumoto, Hideaki

    2008-01-01

    -mentioned sequence of events, induced by Al application looks, to a great extent, similar to Ca depletion syndrome leading finally to cell death of tobacco cells. PMID:19513221

  17. Aluminum toxicity and Ca depletion may enhance cell death of tobacco cells via similar syndrome.

    PubMed

    Basset, Refat Abdel; Matsumoto, Hideaki

    2008-05-01

    -mentioned sequence of events, induced by Al application looks, to a great extent, similar to Ca depletion syndrome leading finally to cell death of tobacco cells. PMID:19513221

  18. Soybean sudden death syndrome species diversity within north and South america revealed by multilocus genotyping.

    PubMed

    O'Donnell, Kerry; Sink, Stacy; Scandiani, María Mercedes; Luque, Alicia; Colletto, Analía; Biasoli, Marisa; Lenzi, Lisandro; Salas, Graciela; González, Victoria; Ploper, Leonardo Daniel; Formento, Norma; Pioli, Rosanna N; Aoki, Takayuki; Yang, X B; Sarver, Brice A J

    2010-01-01

    Sudden death syndrome (SDS) of soybean has become a serious constraint to the production of this crop in North and South America. Phenotypic and multilocus molecular phylogenetic analyses, as well as pathogenicity experiments, have demonstrated that four morphologically and phylogenetically distinct fusaria can induce soybean SDS. Published molecular diagnostic assays for the detection and identification of these pathogens have reported these pathogens as F. solani, F. solani f. sp. glycines, or F. solani f. sp. phaseoli, primarily because the species limits of these four pathogens were only recently resolved. In light of the recent discovery that soybean SDS and Phaseolus and mung bean root rot (BRR) are caused by four and two distinct species, respectively, multilocus DNA sequence analyses were conducted to assess whether any of the published molecular diagnostic assays were species-specific. Comparative DNA sequence analyses of the soybean SDS and BRR pathogens revealed that highly conserved regions of three loci were used in the design of these assays, and therefore none were species-specific based on our current understanding of species limits within the SDS-BRR clade. Prompted by this finding, we developed a high-throughput multilocus genotyping (MLGT) assay which accurately differentiated the soybean SDS and two closely related Phaseolus and mung BRR pathogens based on nucleotide polymorphism within the nuclear ribosomal intergenic spacer region rDNA and two anonymous intergenic regions designated locus 51 and 96. The single-well diagnostic assay, employing flow cytometry and a novel fluorescent microsphere array, was validated by independent multilocus molecular phylogenetic analysis of a 65 isolate design panel. The MLGT assay was used to reproducibly type a total of 262 soybean SDS and 9 BRR pathogens. The validated MLGT array provides a unique molecular diagnostic for the accurate identification and molecular surveillance of these economically important

  19. Improved Diagnoses and Quantification of Fusarium virguliforme, Causal Agent of Soybean Sudden Death Syndrome.

    PubMed

    Wang, Jie; Jacobs, Janette L; Byrne, Jan M; Chilvers, Martin I

    2015-03-01

    Fusarium virguliforme (syn. F. solani f. sp. glycines) is the primary causal pathogen responsible for soybean sudden death syndrome (SDS) in North America. Diagnosis of SDS is difficult because symptoms can be inconsistent or similar to several soybean diseases and disorders. Additionally, quantification and identification of F. virguliforme by traditional dilution plating of soil or ground plant tissue is problematic due to the slow growth rate and plastic morphology of F. virguliforme. Although several real-time quantitative polymerase chain reaction (qPCR)-based assays have been developed for F. virguliforme, the performance of those assays does not allow for accurate quantification of F. virguliforme due to the reclassification of the F. solani species complex. In this study, we developed a TaqMan qPCR assay based on the ribosomal DNA (rDNA) intergenic spacer (IGS) region of F. virguliforme. Specificity of the assay was demonstrated by challenging it with genomic DNA of closely related Fusarium spp. and commonly encountered soilborne fungal pathogens. The detection limit of this assay was determined to be 100 fg of pure F. virguliforme genomic DNA or 100 macroconidia in 0.5 g of soil. An exogenous control was multiplexed with the assay to evaluate for PCR inhibition. Target locus copy number variation had minimal impact, with a range of rDNA copy number from 138 to 233 copies per haploid genome, resulting in a minor variation of up to 0.76 cycle threshold values between strains. The qPCR assay is transferable across platforms, as validated on the primary real-time PCR platform used in the Northcentral region of the National Plant Diagnostic Network. A conventional PCR assay for F. virguliforme detection was also developed and validated for use in situations where qPCR is not possible. PMID:25302524

  20. Usefulness of 10 genomic regions in soybean associated with sudden death syndrome resistance.

    PubMed

    Luckew, A S; Leandro, L F; Bhattacharyya, M K; Nordman, D J; Lightfoot, D A; Cianzio, S R

    2013-09-01

    Sudden death syndrome (SDS) is an important soybean [Glycine max (L) Merrill] disease caused by the soilborne fungus Fusarium virguliforme. Currently, 14 quantitative trait loci (QTL) had been confirmed associated with resistance or tolerance to SDS. The objective of the study was to evaluate usefulness of 10 of these QTL in controlling disease expression. Six populations were developed providing a total of 321 F2-derived lines for the study. Recombinant inbred lines (RIL) used as parents were obtained from populations of 'Essex' × 'Forrest' (EF), 'Flyer' × 'Hartwig' (FH), and 'Pyramid' × 'Douglas' (PD). Disease resistance was evaluated in the greenhouse at three different planting times, each with four replications, using sorghum infested with F. virguliforme homogeneously mixed in the soil (Luckew et al., Crop Sci 52:2215-2223, 2012). Four disease assessment criteria-foliar disease incidence (DI), foliar leaf scorch disease severity (DS), area under the disease progress curve (AUDPC), and root rot severity-were used. QTL were identified in more than one of the disease assessment criteria, mainly associated with lines in the most resistant categories. Five QTL (qRfs4, qRfs5, qRfs7, qRfs12, and Rfs16) were associated with at least one of the disease assessments across multiple populations. Of the five, qRfs4 was associated with DI, AUDPC, and root rot severity, and Rfs16 with AUDPC and root rot severity. The findings suggest it may be possible for plant breeders to focus on stacking a subset of the previously identified QTL to improve resistance to SDS in soybean. PMID:23793550

  1. Attention in Williams Syndrome and Down's Syndrome: Performance on the New Early Childhood Attention Battery

    ERIC Educational Resources Information Center

    Breckenridge, Kate; Braddick, Oliver; Anker, Shirley; Woodhouse, Margaret; Atkinson, Janette

    2013-01-01

    Attentional problems are commonly reported as a feature of the behavioural profile in both Williams syndrome (WS) and Down's syndrome (DS). Recent studies have begun to investigate these impairments empirically, acknowledging the need for an approach that considers cross-syndrome comparisons and developmental changes across the different…

  2. Temporal organization of rest defined by actigraphy data in healthy and childhood chronic fatigue syndrome children

    PubMed Central

    2013-01-01

    Background Accumulating evidence has shown a universality in the temporal organization of activity and rest among animals ranging from mammals to insects. Previous reports in both humans and mice showed that rest bout durations followed long-tailed (i.e., power-law) distributions, whereas activity bouts followed exponential distributions. We confirmed similar results in the fruit fly, Drosophila melanogaster. Conversely, another report showed that the awakening bout durations, which were defined by polysomnography in bed, followed power-law distributions, while sleeping periods, which may correspond to rest, followed exponential distributions. This apparent discrepancy has been left to be resolved. Methods Actigraphy data from healthy and disordered children were analyzed separately for two periods: time out of bed (UP period) and time in bed (DOWN period). Results When data over a period of 24 h were analyzed as a whole, rest bouts showed a power law distribution as previously reported. However, when UP and DOWN period data were analyzed separately, neither showed power law properties. Using a newly developed strict method, only 30% of individuals satisfied the power law criteria, even when the 24 h data were analyzed. The human results were in contrast to the Drosophila results, which revealed clear power-law distributions for both day time and night time rest through the use of a strict method. In addition, we analyzed the actigraphy data from patients with childhood type chronic fatigue syndrome (CCFS), and found that they showed differences from healthy controls when their UP and DOWN data were analyzed separately. Conclusions These results suggested that the DOWN sleep, the bout distribution of which showed exponential properties, contributes to the production of long-tail distributions in human rest periods. We propose that separate analysis of UP and DOWN period data is important for understanding the temporal organization of activity. PMID:24188379

  3. Thimerosal-Preserved Hepatitis B Vaccine and Hyperkinetic Syndrome of Childhood

    PubMed Central

    Geier, David A.; Kern, Janet K.; Hooker, Brian S.; Sykes, Lisa K.; Geier, Mark R.

    2016-01-01

    (1) Background: Hyperkinetic syndrome of childhood (HKSoC) is an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9) category in which the majority of the children are also diagnosed under the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision (DSM-IV-TR), where the umbrella term is “Attention-Deficit and Disruptive Behavior Disorders”. The diagnostic criteria for HKSoC are developmentally inappropriate inattention, hyperactivity, and impulsivity. Some studies have implicated mercury (Hg) exposure as a risk factor. (2) Methods: This hypothesis testing study; using the Vaccine Safety Datalink; assessed the toxicological effects of bolus exposure to organic-Hg from Thimerosal-containing vaccines (TCVs) by examining the relationship between Thimerosal-preserved hepatitis B vaccines (TM-HepB) given at varying levels and at specific intervals in the first six months after birth and the risk of a child being diagnosed with HKSoC. (3) Results: Children diagnosed with HKSoC were significantly more likely to be exposed to increased organic-Hg from TM-HepB doses given within the first month (odds ratio = 1.45; 95% confidence interval (CI) = 1.30–1.62); within the first two months (odds ratio = 1.43; 95% CI = 1.28–1.59); and within the first six months (odds ratio = 4.51; 95% CI = 3.04–6.71) than controls. (4) Conclusion: The results indicate that increasing organic-Hg exposure from TCVs heightens the risk of a HKSoC diagnosis. PMID:26999226

  4. Thimerosal-Preserved Hepatitis B Vaccine and Hyperkinetic Syndrome of Childhood.

    PubMed

    Geier, David A; Kern, Janet K; Hooker, Brian S; Sykes, Lisa K; Geier, Mark R

    2016-01-01

    (1) BACKGROUND: Hyperkinetic syndrome of childhood (HKSoC) is an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9) category in which the majority of the children are also diagnosed under the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision (DSM-IV-TR), where the umbrella term is "Attention-Deficit and Disruptive Behavior Disorders". The diagnostic criteria for HKSoC are developmentally inappropriate inattention, hyperactivity, and impulsivity. Some studies have implicated mercury (Hg) exposure as a risk factor. (2) METHODS: This hypothesis testing study; using the Vaccine Safety Datalink; assessed the toxicological effects of bolus exposure to organic-Hg from Thimerosal-containing vaccines (TCVs) by examining the relationship between Thimerosal-preserved hepatitis B vaccines (TM-HepB) given at varying levels and at specific intervals in the first six months after birth and the risk of a child being diagnosed with HKSoC. (3) RESULTS: Children diagnosed with HKSoC were significantly more likely to be exposed to increased organic-Hg from TM-HepB doses given within the first month (odds ratio = 1.45; 95% confidence interval (CI) = 1.30-1.62); within the first two months (odds ratio = 1.43; 95% CI = 1.28-1.59); and within the first six months (odds ratio = 4.51; 95% CI = 3.04-6.71) than controls. (4) CONCLUSION: The results indicate that increasing organic-Hg exposure from TCVs heightens the risk of a HKSoC diagnosis. PMID:26999226

  5. Brugada syndrome and right ventricle morphofunctional abnormalities on echocardiography in young male with family anamnesis of sudden cardiac death.

    PubMed

    Steiner, Robert; Makarovic, Sandra; Makarovic, Zorin; Bilic-Curcic, Ines

    2014-03-01

    First presented by Brugada and Brugada in 1992, Brugada Syndrome (BrS) is a primary electrical disease of the heart that causes sudden cardiac death or life-threatening ventricular arrhythmias. This disease is hereditary autosomic dominant transmitted and genetically determined. The syndrome has been linked to mutations in SCN5A, the gene encoding for the a-subunit of the sodium channel. Electrocardiogram (ECG) abnormalities indicating Brugada syndrome, include repolarization and depolarization abnormalities in the absence of identifiable structural cardiac abnormalities or other conditions or agents known to lead to ST-segment elevation in the right precordial leads (V1-V3). Intravenous administration of sodium channel blocking drugs may modify the ECG pattern. Ajmaline, flecainide, procainamide and propafenone exaggerate the ST-segment elevation or unmask it when it is initially absent. An implantable cardioverter-defibrillator (ICD) is the only proven effective device treatment for the disease. Although BrS is primary electrical disease, some authors have suggested the presence of morphological and functional abnormalities mainly located in the right ventricle (RV), notably in the outflow tract (RVOT). In this short report we will present a young male, with predisposition and positive family history of sudden cardiac death, with complete diagnostic procedure including propafenon testing unmasking Brugada syndrome. An echosonography revealed dilated apical right ventricle, suggesting BrS is not only electrical disorder, but may include morphofunctional abnormalities, described in previous reports. In addition, we reviewed the possible connection between Brugada syndrome and morphological abnormalities in RV. PMID:24851643

  6. The evidential value of intra-alveolar haemosiderin-macrophages in cases of sudden infant death syndrome (SIDS).

    PubMed

    Kernbach-Wighton, G; Albalooshi, Y; Madea, B

    2012-10-10

    Intra-alveolar deposits of haemosiderin have repeatedly been brought into connection with some diagnostic value, such as markers for previous imposed suffocation, smothering due to Munchausen syndrome by proxy or sudden infant death syndrome (SIDS). This study is based on 104 SIDS cases and 14 controls (causes of death, e.g. inflammatory changes, internal haemorrhages, asphyxia, blunt force trauma or acute toxicity). The SIDS group comprised 44 females (aged 7 days to 12 months) and 60 males (aged 12 days to 16 months 8 days) with the ages of the controls ranging from 2 months 3 days to 47 months. Routine histology samples from the lungs were stained with Prussian blue and haemosiderin foci were counted in 20 hpf for each lung lobe by a pathologist blinded to the cause of death. Results were assigned to one of five categories for haemosiderin positivity. Data were analysed by the Levene-test revealing identical variances in both groups and with a two-sample t-test showing the mean values for haemosiderin counts not being significantly different between SIDS and control groups. Although the sizes of both samples differed considerably it is our opinion that the haemosiderin counts did not show sufficient diagnostic value. This outcome supports the latest results of other comparable investigations. Furthermore, it highlights the necessity to assess carefully positive haemosiderin findings to avoid false suspicion. PMID:22704554

  7. The rationale and design of Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT): a prospective cohort study of childhood nephrotic syndrome

    PubMed Central

    2013-01-01

    Background Nephrotic syndrome is one of the most commonly diagnosed kidney diseases in childhood and its progressive forms can lead to chronic kidney disease (CKD) and/or end-stage renal disease (ESRD). There have been few longitudinal studies among a multi-ethnic cohort to determine potential risk factors influencing disease susceptibility, treatment response, and progression of nephrotic syndrome. Temporal relationships cannot be studied through cross-sectional study design. Understanding the interaction between various factors is critical to developing new strategies for treating children with kidney disease. We present the rationale and the study design of a longitudinal cohort study of children with nephrotic syndrome, the Insight into Nephrotic Syndrome: Investigating Genes, Health and Therapeutics (INSIGHT) study. The specific aims are to determine: 1) socio-demographic, environmental, and genetic factors that influence disease susceptibility; 2) rates of steroid treatment resistance and steroid treatment dependence, and identify factors that may modify treatment response; 3) clinical and genetic factors that influence disease susceptibility and progression to CKD and ESRD; and 4) the interaction between the course of illness and socio-demographic, environmental, and clinical risk factors. Methods/design INSIGHT is a disease-based observational longitudinal cohort study of children with nephrotic syndrome. At baseline, participants complete questionnaires and provide biological specimen samples (blood, urine, and toenail clippings). Follow-up questionnaires and repeat biological specimen collections are performed annually for up to five years. Discussion The proposed cohort will provide the structure to test various risk factors predicting or influencing disease susceptibility, treatment response, and progression to CKD among children with nephrotic syndrome. Trial registration ClinicalTrials.gov Identifier NCT01605266. PMID:23351121

  8. Sudden infant death syndrome in Australian aboriginal and non-aboriginal infants: an analytical comparison.

    PubMed

    Alessandri, L M; Read, A W; Burton, P R; Stanley, F J

    1996-07-01

    Our previous research has shown that the sudden infant death syndrome (SIDS) rate for Aboriginal infants in Western Australia (WA) is markedly higher than that for non-Aboriginal infants. The aim of this study was to identify factors that may be important in explaining this disparity. A case-control study was conducted based on routinely collected data for the population of WA singleton births from 1980 to 1990 inclusive. Cases were infants born and classified as dying from SIDS in WA (Aboriginal n = 88, non-Aboriginal n = 409). Controls were infants born in WA and not classified as dying from SIDS; 2% samples of both Aboriginal and non-Aboriginal infants were included. The risk of dying from SIDS in Aboriginal infants was 3.86 times [95% confidence interval (CI) = 2.98 to 5.02] that in non-Aboriginal infants. Statistically significant univariable risk factors for SIDS in Aboriginal infants were preterm birth, low birthweight and small-for-gestational-age; for non-Aboriginal infants they included these factors as well as single marital status, young maternal age, parity of one or greater and male sex. Comparing Aboriginal with non-Aboriginal controls, most of the risk factors were more common in the Aboriginal population. Multiple logistic regression analysis indicated that Aboriginal infants were 1.43 times [95% CI = 1.04 to 1.95] more likely to die from SIDS than non-Aboriginal infants. Differences in the risk factor profile for Aboriginal and non-Aboriginal infants were sought using interaction terms. The only important differences were that the risk of SIDS in Aboriginal infants, unlike that in non-Aboriginal infants, appeared not to be strongly related to male sex or to single marital status. Thus, the results show that the disparity between the incidence of SIDS in the Aboriginal and non-Aboriginal populations can be explained largely, although not totally, by the high prevalence of routinely recorded risk factors in the Aboriginal population. A limitation of

  9. mTOR inhibition by everolimus in childhood acute lymphoblastic leukemia induces caspase-independent cell death.

    PubMed

    Baraz, Rana; Cisterne, Adam; Saunders, Philip O; Hewson, John; Thien, Marilyn; Weiss, Jocelyn; Basnett, Jordan; Bradstock, Kenneth F; Bendall, Linda J

    2014-01-01

    Increasingly, anti-cancer medications are being reported to induce cell death mechanisms other than apoptosis. Activating alternate death mechanisms introduces the potential to kill cells that have defects in their apoptotic machinery, as is commonly observed in cancer cells, including in hematological malignancies. We, and others, have previously reported that the mTOR inhibitor everolimus has pre-clinical efficacy and induces caspase-independent cell death in acute lymphoblastic leukemia cells. Furthermore, everolimus is currently in clinical trial for acute lymphoblastic leukemia. Here we characterize the death mechanism activated by everolimus in acute lymphoblastic leukemia cells. We find that cell death is caspase-independent and lacks the morphology associated with apoptosis. Although mitochondrial depolarization is an early event, permeabilization of the outer mitochondrial membrane only occurs after cell death has occurred. While morphological and biochemical evidence shows that autophagy is clearly present it is not responsible for the observed cell death. There are a number of features consistent with paraptosis including morphology, caspase-independence, and the requirement for new protein synthesis. However in contrast to some reports of paraptosis, the activation of JNK signaling was not required for everolimus-induced cell death. Overall in acute lymphoblastic leukemia cells everolimus induces a cell death that resembles paraptosis. PMID:25014496

  10. mTOR Inhibition by Everolimus in Childhood Acute Lymphoblastic Leukemia Induces Caspase-Independent Cell Death

    PubMed Central

    Baraz, Rana; Cisterne, Adam; Saunders, Philip O.; Hewson, John; Thien, Marilyn; Weiss, Jocelyn; Basnett, Jordan; Bradstock, Kenneth F.; Bendall, Linda J.

    2014-01-01

    Increasingly, anti-cancer medications are being reported to induce cell death mechanisms other than apoptosis. Activating alternate death mechanisms introduces the potential to kill cells that have defects in their apoptotic machinery, as is commonly observed in cancer cells, including in hematological malignancies. We, and others, have previously reported that the mTOR inhibitor everolimus has pre-clinical efficacy and induces caspase-independent cell death in acute lymphoblastic leukemia cells. Furthermore, everolimus is currently in clinical trial for acute lymphoblastic leukemia. Here we characterize the death mechanism activated by everolimus in acute lymphoblastic leukemia cells. We find that cell death is caspase-independent and lacks the morphology associated with apoptosis. Although mitochondrial depolarization is an early event, permeabilization of the outer mitochondrial membrane only occurs after cell death has occurred. While morphological and biochemical evidence shows that autophagy is clearly present it is not responsible for the observed cell death. There are a number of features consistent with paraptosis including morphology, caspase-independence, and the requirement for new protein synthesis. However in contrast to some reports of paraptosis, the activation of JNK signaling was not required for everolimus-induced cell death. Overall in acute lymphoblastic leukemia cells everolimus induces a cell death that resembles paraptosis. PMID:25014496