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PubMed1996

Cerebral palsy: effects of twinning, birthweight, and gestational age.

AIMS: To determine the effects of birthweight and gestational age on the risk of cerebral palsy for multiple and singleton births. METHODS: Children on the North East Thames Regional Health Authority Interactive Child Health System, born between 1 January 1980 and 31 December 1986, and notified as having cerebral palsy, were included. Cases of postneonatal onset, of known progressive, or non-cerebral pathology and with only mild signs were excluded. Rates and relative risks were calculated using the most complete data, which related to 1985-86, and comprised 102,059 singletons and 2367 twins. Logistic regression was used to examine the associations between being a twin, gestational age, and birthweight. RESULTS: The crude rate per 1000 survivors at 1 year of age was 1.0 in singletons and 7.4 in twins. The relative risk was greatest in twins weighing more than 2499 g (4.5). However, after adjusting for reduced birthweight of twins it was the relative risk of twins weighing less than 1400 g that was significantly increased. Logistic regression confirmed that lower fetal growth, lower gestational age, and being a twin are all independent risk factors for cerebral palsy. CONCLUSION: The increased risk to twins of cerebral palsy is not entirely explained by their increased risk of prematurity and low birthweight.

Birth Weight↗
PubMed1987

A comparison of mothers and infants of different ethnic origins delivering in the British Military Hospital, Hong Kong, 1976-1985.

The majority of infants being delivered in the British Military Hospital, Hong Kong are of British, Chinese or Nepalese origin. This paper compares perinatal data from these three groups of mothers and their babies, and in particular looks at the variations in perinatal mortality rates over the 10 year period 1976-1985. Mortality among the British babies has declined in a way similar to many areas of Britain. The Chinese have a very low perinatal mortality rate probably due to fewer preterm, multiple and abnormal births, but mortality has increased over the last 10 years and there is evidence that the rise may be due to changes in maternal health. The incidence of pregnancy associated hypertension has increased threefold among the Chinese. The Nepalese have a perinatal mortality similar to that of the British, much lower than that expected had they delivered in Nepal, and this probably reflects the application of modern obstetric care to these mothers. In all groups there has been a rise in instrumental deliveries but this is most marked among the Chinese in whom there has also been a significant increase in infants with low apgar scores. The Nepalese have the lowest incidence of forceps and operative deliveries and possible reasons for these differences are discussed.

Adult↗
PubMed2005

Socio-demographic factors associated with smoking and smoking cessation among 426,344 pregnant women in New South Wales, Australia.

BACKGROUND: This study explores the socio-demographic characteristics of pregnant women who continue to smoke during the pregnancy, and identifies the characteristics of the smokers who were likely to quit smoking during the pregnancy period. METHODS: This was secondary analysis of the New South Wales (NSW) Midwives Data Collection (MDC) 1999-2003, a surveillance system covering all births in NSW public and private hospitals, as well as home births. Bivariate and multiple logistic regression analyses were performed to explore the associations between socio-demographic characteristics and smoking behaviour during pregnancy. RESULTS: Data from 426,344 pregnant women in NSW showed that 17.0% continued to smoke during pregnancy. The smoking rate was higher among teenage mothers, those with an Aboriginal (indigenous) background, and lower among more affluent and overseas-born mothers. This study also found that unbooked confinements, and lack of antenatal care in the first trimester were strongly associated with increased risk of smoking during pregnancy. About 4.0% of the smoking women reported they may quit smoking during their pregnancy. Findings showed that mothers born overseas, of higher socio-economic status, first time mothers and those who attended antenatal care early showed an increased likelihood of smoking cessation during pregnancy. Those who were heavy smokers were less likely to quit during pregnancy. CONCLUSION: Although the prevalence of smoking during pregnancy has been declining, it remains a significant public health concern. Smoking cessation programs should target the population subgroups of women at highest risk of smoking and who are least likely to quit. Effective antismoking interventions could reduce the obstetric and perinatal complications of smoking in pregnancy.

Adolescent↗
PubMed2006

Three decades of antidepressant, anxiolytic and hypnotic use in a national population birth cohort.

BACKGROUND: Psychotropic medication use is common and increasing. Use of such drugs at the individual level over long periods has not been reported. AIMS: To describe antidepressant, anxiolytic and hypnotic drug use, and associations between such medication use and common mental disorder, over a 22-year period. METHOD: Questions about psychotropic medication use and symptoms of common mental disorder were asked of more than 3000 members of the 1946 British birth cohort at multiple time points between ages 31 and 53 years. RESULTS: Prevalence of any antidepressant, anxiolytic or hypnotic use increased significantly from 1977 (30.6 per 1000) to 1999 (59.1 per 1000) as the cohort aged. Less than 30% with mental disorder used antidepressants, anxiolytics or hypnotics. Previous use of antidepressant, anxiolytic or hypnotic was a strong predictor of future use during an episode of mental disorder (odds ratios 3.0-8.4); this association became weaker over time. CONCLUSIONS: Pharmacotherapy is infrequently used by individuals with common mental disorder in Britain; this has not changed in the past three decades.

Adult↗
PubMed1992

Periodic alternating gaze deviation in infancy.

Periodic alternating gaze is a rarely reported phenomenon. We have observed two cases that are unique in their early onset at birth and infancy. Multiple congenital defects of the posterior cranial fossa were present on MRI in both cases. A prominent abnormality shared by both was absence of normal structures in the region of the inferior cerebellar vermis. Periodic alternating gaze appears to be associated with pathologic changes in the hindbrain in these and other reported cases.

Brain↗
PubMed2004

Tbx1 regulates fibroblast growth factors in the anterior heart field through a reinforcing autoregulatory loop involving forkhead transcription factors.

Birth defects, which occur in one out of 20 live births, often affect multiple organs that have common developmental origins. Human and mouse studies indicate that haploinsufficiency of the transcription factor TBX1 disrupts pharyngeal arch development, resulting in the cardiac and craniofacial features associated with microdeletion of 22q11 (del22q11), the most frequent human deletion syndrome. Here, we have generated an allelic series of Tbx1 deficiency that reveals a lower critical threshold for Tbx1 activity in the cardiac outflow tract compared with other pharyngeal arch derivatives, including the palatal bones. Mice hypomorphic for Tbx1 failed to activate expression of the forkhead transcription factor Foxa2 in the pharyngeal mesoderm, which contains cardiac outflow precursors derived from the anterior heart field. We identified a Fox-binding site upstream of Tbx1 that interacted with Foxa2 and was necessary for pharyngeal mesoderm expression of Tbx1, revealing an autoregulatory loop that may explain the increased cardiac sensitivity to Tbx1 dose. Downstream of Tbx1, we found a fibroblast growth factor 8 (Fgf8) enhancer that was dependent on Tbx1 in vivo for regulating expression in the cardiac outflow tract, but not in pharyngeal arches. Consistent with its role in regulating cardiac outflow tract cells Tbx1 gain of function resulted in expansion of the cardiac outflow tract segment derived from the anterior heart field as marked by Fgf10. These findings reveal a Tbx1-dependent transcriptional and signaling network in the cardiac outflow tract that renders mouse cardiovascular development more susceptible than craniofacial development to a reduction in Tbx1 dose, similar to humans with del22q11.

Alleles↗
PubMed2005

Six1 and Six4 homeoproteins are required for Pax3 and Mrf expression during myogenesis in the mouse embryo.

In mammals, Six5, Six4 and Six1 genes are co-expressed during mouse myogenesis. Six4 and Six5 single knockout (KO) mice have no developmental defects, while Six1 KO mice die at birth and show multiple organ developmental defects. We have generated Six1Six4 double KO mice and show an aggravation of the phenotype previously reported for the single Six1 KO. Six1Six4 double KO mice are characterized by severe craniofacial and rib defects, and general muscle hypoplasia. At the limb bud level, Six1 and Six4 homeogenes control early steps of myogenic cell delamination and migration from the somite through the control of Pax3 gene expression. Impaired in their migratory pathway, cells of the somitic ventrolateral dermomyotome are rerouted, lose their identity and die by apoptosis. At the interlimb level, epaxial Met expression is abolished, while it is preserved in Pax3-deficient embryos. Within the myotome, absence of Six1 and Six4 impairs the expression of the myogenic regulatory factors myogenin and Myod1, and Mrf4 expression becomes undetectable. Myf5 expression is correctly initiated but becomes restricted to the caudal region of each somite. Early syndetomal expression of scleraxis is reduced in the Six1Six4 embryo, while the myotomal expression of Fgfr4 and Fgf8 but not Fgf4 and Fgf6 is maintained. These results highlight the different roles played by Six proteins during skeletal myogenesis.

Animals↗
PubMed2005

Selective expression of presenilin 1 in neural progenitor cells rescues the cerebral hemorrhages and cortical lamination defects in presenilin 1-null mutant mice.

Mice with a null mutation of the presenilin 1 gene (Psen1(-/-)) die during late intrauterine life or shortly after birth and exhibit multiple CNS and non-CNS abnormalities, including cerebral hemorrhages and altered cortical development. The cellular and molecular basis for the developmental effects of Psen1 remain incompletely understood. Psen1 is expressed in neural progenitors in developing brain, as well as in postmitotic neurons. We crossed transgenic mice with either neuron-specific or neural progenitor-specific expression of Psen1 onto the Psen1(-/-) background. We show that neither neuron-specific nor neural progenitor-specific expression of Psen1 can rescue the embryonic lethality of the Psen1(-/-) embryo. Indeed neuron-specific expression rescued none of the abnormalities in Psen1(-/-) mice. However, Psen1 expression in neural progenitors rescued the cortical lamination defects, as well as the cerebral hemorrhages, and restored a normal vascular pattern in Psen1(-/-) embryos. Collectively, these studies demonstrate that Psen1 expression in neural progenitor cells is crucial for cortical development and reveal a novel role for neuroectodermal expression of Psen1 in development of the brain vasculature.

Animals↗
PubMed2003

Agenesis of the epiglottis and false vocal folds with maxillary hypoplasia in an adult.

Hypoplasia or absence of the epiglottis in an adult is a rarely reported congenital anomaly that usually occurs in association with congenital anomalies of other organ systems. Most epiglottic anomalies usually present in infancy and early childhood with respiratory and feeding problems and the affected individual dies shortly after birth due to multiple congenital anomalies. We present a case of congenital absence of the epiglottis and false vocal folds with hypoplastic maxillae in an adult.

Adult↗
PubMed1975

Induction of multiple ovulation in the cow with single injections of FSH and HCG.

A gonadotrophin regimen was developed to induce a high incidence of double and triple ovulations in the beef cow using a single injection of FSH on Day 16 followed by a low dose of HCG on the day of oestrus. Conception rate was not affected by gonadotrophin treatment and 1/9 cows experiencing multiple ovulations gave birth to twin calves.

Animals↗
PubMed1982

Congenital coronary arterio-ventricular fistulae, associated with absence of proximal coronary artery from aorta.

A male newborn expired 5 days after birth, because of multiple congenital cardiac anomalies. The postmortem examination demonstrated tricuspid stenosis, pulmonary atresia, hypoplastic right ventricle, persistent venous valve, and so on. In addition, he had coronary arterio-ventricular fistulous connection, associated with absence of the proximal connection of the coronary artery to the aorta or the pulmonary artery. As the result, the coronary blood was totally supplied via the right ventricular-coronary channels. Histologic examination revealed that these arterio-luminal channels were characterized by sinusoidal mural structure.

Abnormalities, Multiple↗
PubMed2001

Growth and development in term infants fed long-chain polyunsaturated fatty acids: a double-masked, randomized, parallel, prospective, multivariate study.

OBJECTIVE: To evaluate the effects of dietary intake of the long-chain polyunsaturated fatty acids, arachidonic acid (AA), and docosahexaenoic acid (DHA) on multiple indices of infant growth and development. DESIGN: A double-masked, randomized, parallel trial was conducted with term infants fed formulas with or without AA+DHA for 1 year (N = 239). Reference groups of breastfed infants (N = 165) weaned to formulas with and without AA+DHA were also studied. Infants in the formula groups were randomized at </=9 days of age to a control formula with no AA or DHA (n = 77) or 1 of 2 otherwise identical formulas containing AA+DHA (AA, 0.46% and DHA, 0.14% of total fatty acids) from either egg-derived triglyceride (egg-DTG [n=80]) or fish oil and fungal oil (fish/fungal [n = 82]) at levels similar to the average in breast milk samples as measured in the reference group. All formulas contained 50% of energy from fat with the essential dietary fatty acids, linoleic acid (20% fatty acids) and alpha-linolenic acid (2% fatty acids). The main study outcomes were AA and DHA levels in plasma and red blood cells, and multiple measures of infant development at multiple ages from birth to 14 months: growth, visual acuity, information processing, general development, language, and temperament. RESULTS: AA and DHA levels in plasma and red cells were higher in AA+DHA-supplemented groups than in the control formula group and comparable to those in reference groups. No developmental test results distinguished these groups. Expected differences in family demographics associated with breastfeeding were found, but no advantages to breastfeeding on any of the developmental outcome demonstrated. CONCLUSIONS: These findings do not support adding AA+DHA to formulas containing 10% energy as linoleic acid and 1% energy as alpha-linolenic acid to enhance growth, visual acuity, information processing, general development, language, or temperament in healthy, term infants during the first 14 months after birth.infant development, breast feeding, infant formula, long-chain polyunsaturated fatty acids, docosahexaenoic acid.

Arachidonic Acid↗
PubMed2004

[Mortality due to infectious and parasitic diseases in the elderly in Brazil].

OBJECTIVE: To describe the mortality due to infectious and parasitic diseases in persons over 65 years of age in all of Brazil and in individual states and to study the association between that mortality due to these causes and specific socioeconomic variables over the period from 1980 to 1995. METHODS: Data were obtained from Brazil's national Mortality Information System. Standardized mortality rates were calculated for tuberculosis, Chagas' disease, and sepsis. The relative contribution of these causes of death to overall mortality was also calculated. The study also analyzed the relationship between mortality rates and gender and the following socioeconomic variables: per capita gross domestic product; number of hospital beds per inhabitant; proportion of the total population living in urban areas; number of benefits provided per capita by the social welfare system; number of deaths reported for every 100 hospitalizations; and life expectancy at birth. A normal multiple linear regression model was used for this analysis. RESULTS: There were no significant changes in the proportion of deaths due to infectious and parasitic diseases in the elderly population during the period studied, regardless of sex. However, for both men and women there was a sizeable decrease in the impact of tuberculosis and Chagas' disease, while that of sepsis appeared to increase. According to the results of the ecological analysis, socioeconomic indicators had little power to explain the differences in mortality patterns seen in the different states of the country. CONCLUSIONS: Our results suggest that policies to prevent and control infectious and parasitic diseases among the elderly in Brazil have stagnated. Given this situation and the progressive increase in the elderly population, mortality due to infectious and parasitic diseases is not likely to decrease in this age group, at least in the near future.

Aged↗
PubMed1995

Suicide in Québec, 1951-1986.

Data from Fortier, et al. (1989) were reanalysed using multiple regression. While birth and divorce rates were significantly associated with both male and female suicide rates, unemployment rates and cirrhosis death rates were associated only with male suicide rates.

Cross-Sectional Studies↗
PubMed1985

Predisposing and risk factors of umbilical cord prolapse.

Altogether 117 deliveries (0.14%) out of 82 308 were complicated by umbilical cord prolapse (UCP) in the obstetrical department of Tampere University Central Hospital in the period 1962-81. The gross perinatal mortality rate in the present study was 16.2%, but during the last 5-year period the incidence of UCP (0.09%) as well as the perinatal mortality rate (5.9%) declined significantly. UCP seemed to be more prevalent during the first half of the year. The most prominent precipitating factors for the occurrence of UCP seemed to be abnormal presentation, multiple pregnancy, low birth weight, and multiparity. Amniotomy seemed not to increase the risk for UCP. Diminished perinatal mortality figures were closely associated with increased cesarean section rates in the management of deliveries complicated by UCP. Cesarean section is therefore recommended as the treatment of choice for deliveries complicated by UCP.

Delivery, Obstetric↗
PubMed1999

Sociodemographic and behavioural determinants of blood lead concentrations in children aged 11-13 years. The Port Pirie Cohort Study.

OBJECTIVE: To describe the determinants of blood lead concentration in children with long term environmental exposure to lead. DESIGN: Prospective cohort study. SETTING: The lead smelting town of Port Pirie, South Australia, and surrounding townships. PARTICIPANTS: 326 children born in and around Port Pirie, 1979-1982, followed up until age 11-13 years in 1993-1994. MAIN OUTCOME MEASURES: Blood lead concentrations assessed at birth and at multiple ages up to 11-13 years; average lifetime blood lead concentration. RESULTS: Mean blood lead concentration rose sharply over the ages 6 to 15 months, reached a maximum around 2 years of age, and declined steadily as the children grew older. There was no difference in blood lead concentration between boys and girls until they reached the age of 11-13 years, when mean blood lead concentration in boys (8.4 micrograms/dL [0.41 mumol/L]) was slightly higher than in girls (7.5 micrograms/dL [0.36 mumol/L]). Residential area and father's employment site were the two variables most strongly predictive of a child's blood lead concentration at the end of primary school. Poorer-quality home environment was also found to be an independent contributor to blood lead concentrations. CONCLUSIONS: Age-related factors, and possibly recent concerted efforts to decrease entry or re-entrainment of lead into the environment at Port Pirie, have resulted in most children in our study having blood lead concentrations below 10 micrograms/dL (0.48 mumol/L) at the end of their primary school years. Lead exposure during a child's early years remains an important contributor to average lifetime exposure.

Age Factors↗
PubMed2000

Timing of exogenous surfactant administration in clinical practice. Report of a multi-center Italian Observational Study.

UNLABELLED: We now have convincing data that surfactant administration in newborns with the respiratory distress syndrome (RDS) is effective in reducing the need for respiratory supports and improves the clinical outcomes. To evaluate the different strategies adopted in clinical practice for the surfactant use we performed an observational multicenter study. All newborns with gestational age (g.a.) between 24-32 wk. with RDS and that were in need of mechanical ventilation and treated with exogenous surfactant from 1994 to 1996 in twelve Neonatal Intensive Care Units have been evaluated. The patients have been divided into two groups: one includes babies that were given surfactant within the first 15 minutes of life (very early -VE- treated) and the other includes those that received the treatment at age 16- 180 minutes (early -E- treated). The analysis concerned 246 cases, 54 VE treated and 192 E treated. The two groups were comparable with regards to sex, Apgar score at one and five minutes, maternal treatment with steroids and multiple pregnancy. Mean birth weight and g.a. were significantly lower and the rate of caesarean sections was higher in the VE than in the E. Almost 80% of the VE newborns had a g.a. equal or less than 28 wks. One single dose of surfactant was administered in 77.8% of VE babies and 45.3% in the E group, while 18.5% VE and 36.8% E received two doses, 3.7% VE and 16.3% E were given three doses. The mean number of doses used per patient was 1.3 in VE and 1.7 in E. 62.5% of cases with g.a. equal to or less than 26 wks and 80.8% of those with g.a. 27-28 wk. received a single dose if VE, compared to 31.9% and 49.1% respectively if E treated. Prenatal prophylaxis of RDS with steroids, considering only the mothers that received a complete course between 48 hrs and 7 days before the delivery, did not modify the number of doses of surfactant required in both groups. The main outcomes did not differ in the two groups: mortality at 28 days of life, age at death, pneumothorax, intraventricular haemorrhage grade 3-4, retinopathy of prematurity grade 3-4, patent ductus arteriosus and oxygen dependency at 36 wks post-conceptional age. CONCLUSIONS: Very early surfactant administration in clinical practice reserved for newborns with the lowest g.a., improves the outcome and does not increase surfactant consumption.

Female↗
PubMed2001

Giant congenital pigmented naevus with unusual presentation and early malignant transformation in a Nigerian infant: case report and review of the literature.

A six month-old female infant with a giant congenital pigmented naevus is reported because of the rarity and unusual presentation and early malignant transformation. It was the first case to be documented in our centre. There was an extensive giant, darkly pigmented naevus of the "bathing trunk" distribution noted at birth, associated with multiple darkly pigmented smaller naevi scattered over the trunk and limbs. Bv the third month of life large pigmented lesion began to demonstrate small papillomatous projections studding the entire surface and at age six months nodular and warty excrescences appeared including a large pedunculated spherical mass attached to the anal verge. The labia majora became anatomically disfigured by these nodular, warty lesions of various sizes associated with the large pigmented hairy naevus. Histological examination of the excised large perianal and vulvar nodules showed the typical picture of an intradermal naevus consistent with giant congenital pigmented (melanocytic) naevus. The deeper portions of the lesion showed features of malignant transformation. The rapidity of evolution of the lesions including histologic features of early malignant transformation and the gross disfiguring of the genitalia and perineum are uncommon presentation of this melanocytic naevus. In view of the early malignant transformation immediate surgical treatment was indicated. Unfortunately the patient was lost to follow-up. The case report is complimented with a review of the literature.

Cell Transformation, Neoplastic↗