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Objective: To examine the incidence of sudden unexplained death in children 1-4 years old (SUDC) in Ireland and to compare the epidemiological profile of SUDC with that of SIDS., Design: All cases of sudden unexplained death in children <5 years in Ireland between 1994 and 2008 were reviewed. Epidemiological information obtained from parental questionnaires and post-mortem reports was examined, and data on cases ≥52 weeks compared with cases <52 weeks., Results: SUDC accounted for 5% (n=44) of deaths in children aged 1-4 years during 1994-2008. During this period, the SIDS rate dropped from 0.71 to 0.34 per 1000 live births, while the SUDC rate increased from 0.08 to 0.18 deaths per 10 000 population aged 1-4 years. The median age of SUDC cases was 71.5 weeks, and the male/female ratio was 1.3:1. All died during a sleep period, 71% between 10pm and 8am, and more than two-thirds were found prone. Fewest cases occurred during July-September (11%), and a greater proportion occurred at weekends (55%). 52% (17/33) had symptoms (any) in the 48 h before death, and 35% (11/31) visited their general practitioner because of illness in the week preceding death. SUDC differed from SIDS in prevalence of maternal smoking (38% vs 72%, p<0.001), bed-sharing (17% vs 49%, p<0.001), and whether found prone (72% vs 23%, p<0.001)., Conclusion: While SUDC shares some characteristics with SIDS, there are also some important differences. Further data collection will help determine whether SIDS and SUDC represent the same pathophysiological entity. Standardisation of protocols for investigating sudden deaths is urgently required for accurate diagnosis of cases.

The aim was to determine the rate of varicella infection and complications in children with disorders of intermediary metabolism (IEM) between the ages of 1 and 16 years attending our national metabolic referral centre. Of 126 children identified, a response was received from 122. A history of previous varicella infection was identified in 64 cases (53%) and of varicella vaccination in 5 (4%). Fifty-three (43%) patients apparently did not have a history of clinical varicella infection. Of the 64 children with a history of varicella infection, five required hospitalisation for complications, including life-threatening lactic acidosis in one patient with mitochondrial disease and metabolic decompensation in four patients. In conclusion, varicella infection may cause an increased risk of metabolic decompensation in patients with IEMs. We propose that a trial of varicella vaccination be considered for this cohort of patients with monitoring of its safety and efficacy.

Background: It is unclear if it is safe for babies to bed share with adults. In Ireland 49% of sudden infant death syndrome (SIDS) cases occur when the infant is bed-sharing with an adult., Objective: To evaluate the effect of bed-sharing during the last sleep period on risk factors for SIDS in Irish infants., Design: An 8 year (1994-2001) population based case control study of 287 SIDS cases and 831 controls matched for date, place of birth, and sleep period. Odds ratios and 95% confidence intervals were calculated by conditional logistic regression., Results: The risk associated with bed-sharing was three times greater for infants with low birth weight for gestation (UOR 16.28 v 4.90) and increased fourfold if the combined tog value of clothing and bedding was > or =10 (UOR 9.68 v 2.34). The unadjusted odds ratio for bed-sharing was 13.87 (95% CI 9.58 to 20.09) for infants whose mothers smoked and 2.09 (95% CI 0.98 to 4.39) for non-smokers. Age of death for bed-sharing and sofa-sharing infants (12.8 and 8.3 weeks, respectively) was less than for infants not sharing a sleep surface (21.0 weeks, p<0.001) and fewer bed-sharing cases were found prone (5% v 32%; p = 0.001)., Conclusion: Risk factors for SIDS vary according to the infant's sleeping environment. The increased risk associated with maternal smoking, high tog value of clothing and bedding, and low z scores of weight for gestation at birth is augmented further by bed-sharing. These factors should be taken into account when considering sleeping arrangements for young infants.

Aims: To investigate the influence of analytical design on the variability of published results in studies of sudden infant death syndrome (SIDS)., Methods: The results of a prospective case-control study, of 203 cases of SIDS, and 622 control infants are presented. All variables significant on univariate analysis were included in a multivariate model analysed in nine stages, starting with sociodemographic variables, then sequentially and cumulatively adding variables relating to pregnancy history, current pregnancy, birth, the interval from birth to the week prior to death, the last week, the last 48 hours, and the last sleep period. A ninth stage was created by adding placed to sleep prone for the last sleep period., Results: As additional variables are added, previously published SIDS risk factors emerged such as social deprivation, young maternal age, > or =3 previous live births, maternal smoking and drinking, urinary tract infection in pregnancy, reduced birth weight, and the infant having an illness, regurgitation, being sweaty, or a history of crying/colic in the interval from birth to the week before death, with co-sleeping and the lack of regular soother use important in the last sleep period. As the model progressed through stages 1-9, many significant variables became non-significant (social deprivation, young maternal age, maternal smoking and drinking) and in stage 9 the addition of placed to sleep prone for the last sleep period caused > or =3 previous live births and a reduced birth weight to become significant., Conclusion: The variables found to be significant in a case-control study, depend on what is included in a multivariate model.

Background: Little has been published on gratification disorder ("infantile masturbation") in early childhood., Aims: To expand on the profile of patients diagnosed with this condition., Methods: Retrospective case note review; Fraser of Allander Neurosciences Unit paediatric neurology outpatient department 1972-2002., Results: Thirty one patients were diagnosed (11 males and 20 females). Twenty one were referred for evaluation of possible epileptic seizures or epilepsy. The median age at first symptoms was 10.5 months (range 3 months to 5 years 5 months). The median age at diagnosis was 24.5 months (range 5 months to 8 years). The median frequency of events was seven times per week, and the median length 2.5 minutes. Events occurred in any situation in 10 children, and in a car seat in 11. Types of behaviour manifested were dystonic posturing in 19, grunting in 10, rocking in 9, eidetic imagery in 7, and sweating in 6. Two children had been previously diagnosed as having definite epilepsy. In nine cases home video was invaluable in allowing confident diagnosis., Conclusion: Gratification disorder, otherwise called infantile masturbation, is an important consideration in the differential diagnosis of epilepsy and other paroxysmal events in early childhood. Home video recording of events often prevents unnecessary investigations and treatments.

Aim: To identify risk factors for sudden infant death syndrome (SIDS) in the sleeping environment of Irish infants., Methods: A five year population based case-control study with parental interviews conducted for each case and three controls matched for age, place of birth, and last sleep period. A total of 203 SIDS cases and 622 control infants born 1994-98 were studied., Results: In a multivariate analysis, co-sleeping significantly increased the risk of SIDS both as a usual practice (adjusted OR 4.31; 95% CI 1.07 to 17.37) and during the last sleep period (adjusted OR 16.47; 95% CI 3.73 to 72.75). The associated risk was dependent on maternal smoking (OR 21.84; 95% CI 2.27 to 209.89), and was not significant for infants who were > or =20 weeks of age (OR 2.63; 95% CI 0.49 to 70.10) or placed back in their own cot/bed to sleep (OR 1.07; 95% CI 0.21 to 5.41). The use of pillows, duvets, and bedding with tog value > or =10 were not significant risk factors when adjusted for the effects of confounding variables, including maternal smoking and social disadvantage. However, the prone sleeping position remains a significant SIDS risk factor, and among infants using soothers, the absence of soother use during the last sleep period also significantly increased the SIDS risk (OR 5.83; CI 2.37 to 14.36)., Conclusion: Co-sleeping should be avoided in infants who are <20 weeks of age, or whose mothers smoked during pregnancy. The prone position remains a factor in some SIDS deaths, and the relation between soother use and SIDS is a complex variable requiring further study.

Objectives: Raised concentrations of antimony have been found in infants dying of sudden infant death syndrome (SIDS). The presumed source of this antimony is toxic gases generated from fire retardants that are present in cot mattresses. The aim of this study was to determine the role of antimony in SIDS., Design: Samples of liver, brain, serum, and urine were collected from all patients dying from SIDS and a group of aged matched control infants who had died of other causes., Setting: Nationwide study in Ireland., Subjects: 52 infants dying from SIDS and 19 control infants aged > 7 days and < 1 year., Results: The median concentration of antimony in the liver and brain of infants dying of SIDS was < 1 ng/g, with no difference detected between the infants dying from SIDS and the control infants. The range of antimony in the serum of infants dying of SIDS was 0.09-0.71 microg/litre (median, 0.26). Although no difference was found between infants dying from SIDS and control infants, SIDS infants were found to have higher concentrations when compared with healthy infants in the 1st year of life, probably as a result of release of antimony into serum after death. Urine antimony concentrations in infants dying from SIDS were < 3.91 ng/mg (corrected for creatinine) and similar to values found both in control infants and healthy infants., Conclusion: There is no evidence to support a causal role for antimony in SIDS.