Incidence of Cystic Fibrosis in Ethnic Italians and Greeks and in Australians of Predominantly British Origin

The incidence of cystic fibrosis among the Australian born children of parents born in Italy was 1: 3 625 live births and among Australian born children of parents born in Greece 1: 3726. These incidences were significantly lower than the incidence for cystic fibrosis of 1: 2021 in children born to Australian born parents, about 90% of whom have ancestors born in the British Isles. The figures suggest there are significant variations in the incidence of cystic fibrosis in different European populations.     

Incidence of cystic fibrosis in ethnic Italians and Greeks and in Australians of predominantly British origin

The incidence of cystic fibrosis among the Australian born children of parents born in Italy was 1:3 625 live births and among Australian born children of parents born in Greece 1:3 726. These incidences were significantly lower than the incidence for cystic fibrosis of 1:2 021 in children born to Australian born parents, about 90% of whom have ancestors born in the British Isles. The figures suggest there are significant variations in the incidence of cystic fibrosis in different European populations.     

Fetal growth regulation and intrauterine growth retardation

Gestational age and neonatal anthropometric parameters are currently used to evaluate fetal growth and are predictive factors of perinatal and postnatal morbidity and mortality. We performed a retrospective analysis of neonatal anthropometric parameters (weight, vertex-heel length and head circumference) in 1,470 live preterm neonates born between 1997 and 2002 and a prospective analysis of the same parameters in 1,786 live newborns of both sexes born in 2001 and 2002, products of single 37-42 week uncomplicated pregnancies in healthy Spanish Caucasian mothers. A progressive increase in these parameters with gestational age and sexual dimorphism were observed from the 30th week of gestational age onwards, with statistically-significant differences (p<0.05) at 38-42 weeks of gestational age. An increase in weight and length values in relation to previous Spanish studies was also documented in preterm newborns. It is estimated that 10-15% of children born small for gestational age (SGA) do not experience catch-up growth by the age of 3 years and may have short stature in adulthood. Preliminary data of a cross-sectional study on spontaneous growth in boys and girls born SGA without postnatal catch-up growth show that their +2 SD values of height are similar to -2 SD values of our normal control population of children born with adequate weight and length for gestational age (AGE). However, weight +2 SD values are similar to mean values of control children born AGE. In summary, our data show sexual dimorphism in neonatal anthropometric growth parameters and that these parameters change with time and may be updated. In addition children born SGA without postnatal catch-up are shorter and have higher weight than age-, height- and sex-matched controls born AGE.     

Post-mortem findings in fetal and neonatal congenital diaphragmatic hernia.

Analyses of survival data of neonates born with congenital diaphragmatic hernia (CDH) can be misleading. There is a hidden mortality only apparent when fetuses with CDH are included in the analysis. A retrospective review of all post mortems with a diagnosis of CDH in the West of Scotland over a 10-year period was carried out. Congenital anomalies were identified and heart and lung weights were compared with controls (infants dying of non-cardiorespiratory causes). 70 Pm reports were studied. Major congenital anomalies were present in 53% (18/ 47 live born, 19/23 not live born). Neural tube defects, cardiac and chromosomal anomalies were the most common. Antenatal detection rate was 17% in live-born infants. In infants dying within the first week of life lung weights showed severe pulmonary hypoplasia, but heart weights were within the normal range. Detailed antenatal scanning needs to be considered if the detection rate for CDH is to improve in this region.     

Survival of children born with congenital anomalies

Aim: To describe the survival to age 5 years of children born with congenital anomalies. Methods: Between 1980 and 1997, 6153 live born cases of congenital anomaly were diagnosed and registered by the population based Glasgow Register of Congenital Anomalies. They were retrospectively followed to assess their survival status from birth up to the age of 5 years. Results: The proportions of all live born infants with congenital anomalies surviving to the end of the first week, and first and fifth year were 94%, 89%, and 88%, respectively. Survival to age 5, the end point of follow up, was significantly poorer for infants with chromosomal anomalies (48%) compared to neural tube defects (72%), respiratory system anomalies (74%), congenital heart disease (75%), nervous system anomalies (77%), and Down''s syndrome (84%). Conclusion: Although almost 90% of all live born infants with congenital anomalies survive to 5 years, there are notable variations in survival between anomaly types. Our findings should be useful for both clinicians and geneticists to assess the prognosis of congenital anomalies. This information is also important for affected families and for the planning of health care needs for this high risk population.     

The risk of psychiatric disorders in individuals born prematurely in Denmark from 1974 to 1996

Aim: To investigate the risk of developing psychiatric disorders in individuals born prematurely (born before 37 weeks of gestation). Methods: The study is a longitudinal register‐based study including all live‐born individuals in Denmark from 1974 to 1996. Poisson regression models including well‐known confounders were used to investigate the rate ratio of psychiatric disorders at the age of 11–34 years in the individuals born prematurely. Results: A total of 1 545 641 individuals were born from 1974 to 1996. Of these, 1 348 106 live‐born individuals constituted the study cohort. Rate ratios of psychiatric disorders, in total, increased in proportion to the degree of prematurity. The rate ratio of major psychiatric disorder was increased in individuals born with a gestational age below 28 weeks compared to persons born with a gestational age of 39–45 weeks (RR = 1.88 (95% CI: 1.25–2.83)). Similarly, the rate ratios of minor psychiatric disorder (RR = 1.63 (95% CI: 1.29–2.06)) and psychotropic drugs purchased (RR = 1.35 (95% CI: 0.98–1.86)) were increased. Conclusion: The risk of minor as well as major psychiatric disorders during adolescence and young adulthood increases in proportion to the degree of prematurity. The relative risk, however, is small compared to the risks of cerebral palsy or low intelligence.     

Survival of children born with congenital anomalies.

AIM: To describe the survival to age 5 years of children born with congenital anomalies. METHODS: Between 1980 and 1997, 6153 live born cases of congenital anomaly were diagnosed and registered by the population based Glasgow Register of Congenital Anomalies. They were retrospectively followed to assess their survival status from birth up to the age of 5 years. RESULTS: The proportions of all live born infants with congenital anomalies surviving to the end of the first week, and first and fifth year were 94%, 89%, and 88%, respectively. Survival to age 5, the end point of follow up, was significantly poorer for infants with chromosomal anomalies (48%) compared to neural tube defects (72%), respiratory system anomalies (74%), congenital heart disease (75%), nervous system anomalies (77%), and Down's syndrome (84%). CONCLUSION: Although almost 90% of all live born infants with congenital anomalies survive to 5 years, there are notable variations in survival between anomaly types. Our findings should be useful for both clinicians and geneticists to assess the prognosis of congenital anomalies. This information is also important for affected families and for the planning of health care needs for this high risk population.     

甲肝减毒活疫苗及加服牛磺酸在接种乙肝疫苗免疫失败儿童中的致免疫性

目的为了估价甲肝减毒活疫苗在接种乙肝疫苗免疫失败儿童中的致免疫性和牛磺酸对该疫苗免疫性的影响。方法应用随机对照临床试验。选择９０名健康儿童随机分成二组：４３名接种甲肝减毒活疫苗和口服牛磺酸（Ⅰ组）；４７名接种疫苗加口服安慰剂（Ⅱ组）。另选择６２名由携带ＨＢＶ的无症状母亲所生接种过乙肝疫苗但是免疫失败者（Ⅲ组）和４４名同龄由ＨＢｓＡｇ阴性母亲所生接种乙肝疫苗正常免疫反应的健康儿童（Ⅳ组），均接种甲肝减毒活疫苗作比较。所有对象均除外甲肝感染，接种后观察反应，２个月后检测血清－ＨＡＶＩｇＧ抗体。结果Ⅰ组抗ＨＡＶＩｇＧ平均值显著高于Ⅱ组（ｔ＝７．１０９，Ｐ＜０．０１）；Ⅳ组接种甲肝疫苗后抗－ＨＡＶＩｇＧ平均值与Ⅲ组比较，差异有显著性，（ｔ＝１．９９８，Ｐ＜０．０５）。结论接种乙肝疫苗免疫失败儿童接种甲肝减毒活疫苗的免疫效果比健康儿童差。甲肝减毒活疫苗接种后随访未发现明显副反应。牛磺酸对疫苗接种效果的辅助作用值得推广应用。     

Congenital defects in the newborn--difficulties in prenatal diagnosis

The aim of this work is an analysis of developmental defects in newborn infants, born in the Clinical Department of Pregnancy Pathology and Reproduction in Zabrze, during the 19 months of its existence. Difficulties in prenatal diagnosis of developmental defects are presented and their structure is discussed. The study included 1524 live born infants with birth weight over 500 g and 12 stillborn. In the live born group developmental defects were found in 71 (4.6%) infants. Among the 12 stillborn infants developmental defects were present in one child (8.3%). The most common developmental defects, in our material, were those of the skeletal system and heart defects. Prenatally, developmental defects were diagnosed in 8% of the examined foetuses. The quality of health care in pregnant mothers of the investigated newborn infants was assessed, with relation to medical examination, ultrasonographic scan and biochemical screening tests. In many cases, modern standards of management were not maintained.     

Race- and rank-specific infant mortality in a US military population.

Mortality among black infants in the United States is approximately twice that among white infants. The disparity has been attributed in large part to the higher incidence of poverty and limited access to health care among black Americans. We investigated race- and rank-specific infant mortality rates among dependents of military officers and soldiers at Madigan Army Medical Center, Tacoma, Wash, between 1985 and 1990. The overall infant mortality rate was 9.3 deaths per 1000 live births compared with 10.1 deaths per 1000 live births in the United States in 1987. Mortality rates for infants born to families of junior enlisted soldiers were similar to those for infants born to families of noncommissioned and commissioned officers. The mortality rate among black infants was 11.1 deaths per 1000 live births compared with 17.9 deaths per 1000 live births among all black Americans in 1987. These lower rates of mortality among black infants may be due to guaranteed access to health care and higher levels of family education and income in the multiracial subpopulation served by our medical center compared with the nation as a whole.     

Prevalence of cleft lip and/or palate in children from Lodz between years 1981–2010

Congenital malformations constitute a serious problem of both medical and social nature. Cleft lip and/or palate represent the most common congenital anomaly of the face that is why it is essential to know the real frequency of the described phenomenon. The aim of this paper is to determine the frequency of cleft lip and/or palate and the types of malformations that occurred in Lodz city between the years 1981–2010. Our clinic has been carrying on the studies concerning the incidence of cleft lip and/or palate since 1981. The Polish Registry of Congenital Malformations has been operating in Poland since 1 April 1997. The team has managed to obtain data, from the Registry, concerning the total number of all live born infants and the number of children with cleft lip and/or palate, who were born in Lodz, between 1998 and 2010. In years 1981–2010, 319 children, in 210 952 live born infants, were born with cleft lip and/or palate in Lodz. The isolated cleft palate was observed more frequently in girls and the unilateral cleft of lip and palate in boys. In all three decades palate clefts are more common whereas bilateral lip, alveolus and palate clefts are more infrequent. A small tendency to decrease in actual cleft lip and/or palate frequency among children, in the period of 30 years, is observed in Lodz. Over the years it has still been observed that the isolated cleft palate is the most common type of defect.     

The changing panorama of cerebral palsy in Sweden. IX. Prevalence and origin in the birth-year period 1995-1998

AIM: This is the ninth report from the western-Swedish study of the prevalence and origin of cerebral palsy. METHODS: A population-based study covering the 88 371 live births in the area in 1995-1998. Birth characteristics, neuroimaging findings and risk factors in children with cerebral palsy were recorded, prevalence was calculated, and aetiology was analysed. RESULTS: The study comprised 170 children with cerebral palsy, i.e. a prevalence of 1.92 per 1000 live births. Excluding eight post-neonatally derived cases, the gestational age-specific prevalences were 77 per 1000 for children born before 28 wk of gestation, 40 for children born at 28-31 wk, 7 for children born at 32-36 wk and 1.1 for children born after 36 wk of gestation. Spastic hemiplegia, diplegia and tetraplegia accounted for 38%, 35% and 6%, respectively, dyskinetic cerebral palsy for 15%, and ataxia for 6%. For the first time, hemiplegia was now most common, due to the decline in preterm diplegia. There was a further increase in full-term dyskinetic cerebral palsy. The origin of cerebral palsy in children born at term was considered to be prenatal in 38%, peri/neonatal in 35% and unclassifiable in 27%, while in children born preterm it was 17%, 49% and 33%, respectively. CONCLUSION: The decreasing trend from the period 1991-1994 continued, both in children born at term and especially in those born preterm. However, the increase in dyskinetic cerebral palsy in children born at term was a matter of concern. In this group, a perinatal hypoxic ischaemic encephalopathy had been present in 71%.     

Epidemiological characteristics of new borns in a rural community

All births (1211) were recorded in a rural area for 1985 by monthly domiciliary visits and the sociological characteristics were collected from mothers within a month. The analysis of 1201 births is presented: 52.7% were male births, 95.7% were delivered in village itself, 80.4% were delivered by trained dais, 99.5% were live born, 12.9% were born before 37 weeks of gestation, 61.5% were put to breast feed within 6 hours, 35% were of gravida 4 and above, 68.9% did not receive any antenatal care, 67.1% had not received any tetanus toxoid, 70.8% did not receive any folifer tablet, 0.8% had abortion and another 0.8% had still birth in preceeding outcome, 1.58% had lost a live born dead in preceding out come, 89.6% stopped breast feeding at conception, 31.7% had birth interval less than 2 years, but 2.2% had less than ore year, 2/3rd were born in second half of the year, 17.8% had taken extra food during pregnancy, 21.2% reduced the work after becoming pregnant, 9.5% were born to mothers of less than 20 years age, 98.5% of mothers were housewifes and 95.9% mothers were illiterate, 33.5% were born in nuclear family, 22.3% were living in Kacha' house, 34.8% had no electricity connection, 37.0% were born to land less, 42.2% had no radio in the house, 7.1% had no conveyance, 52.0% families had piped water supply. These findings have been discussed here.     

Need for measles revaccination in adolescents: correlation with birth date prior to 1972

Measles hemagglutination inhibition (HI) antibody titers of less than 1:4 were significantly (P less than 0.05) more prevalent among subjects born from 1962 through 1971 and vaccinated with a single dose of live measles virus vaccine at 12 months of age (14.5%) than among subjects born during the same years but vaccinated at 13 months or older (2.3%). For subjects born in 1972 through 1976, however, this difference was not statistically significant; titers of less than 1:4 occurred in 6.2% of those vaccinated at 12 months, compared to 0% in those vaccinated at 13 months or older. A decline in maternally derived measles HI antibody may be related to the increased rate of HI antibody titers of 1:4 or greater following vaccination of more recently born subjects. Following revaccination of subjects whose measles HI antibody titers were less than 1:4, measles HI titers were lower than would be expected after successful primary vaccination. Nevertheless, measles HI antibody persisted at a level of 1:4 or more until the latest titer measurement of this study (one to two years after revaccination) in 87.5% of those whose initial vaccination had been at 11 or 12 months of age. No adverse reactions to revaccination occurred. Revaccination programs should be considered for adolescents and young adults born before 1972 who received live measles virus vaccine at or before 12 months. Children born from 1972 through 1976 who were vaccinated at 12 months or later are not in need of revaccination.     

Very low birthweight infants: outcome in a sub-Arctic population

Objective : To evaluate the outcome for very low birthweight (VLBW) infants in northern Norway. Subjects and methods: All live born infants ( n = 536) with birthweight ≤1500g born during 1978–89 to women residing in the northern health region of Norway were studied retrospectively. Data were from the Medical Birth Registry (MBR), hospital records and from follow-up recordings to 4 y of age at maternal and child health centres. Stillborn infants ( n = 269) with birthweight ≤1500g during the same period were also registered. Results : The annual incidence of live born VLBW infants (7.1/1000 live births) did not change, but the proportion of infants born alive before 26 weeks'gestation increased and the stillborn part decreased significantly. The Caesarean section (CS) rate, antenatal transfer and the use of a neonatal transport team increased significantly. Four hundred and seventy-five infants (89%) were considered viable at birth, 347 (65%) survived to 1 y and 343 (64%) to 4y. The likelihood of survival was independently related to female gender. The trend for survival to 4y of age did not increase significantly. Thirty children suffered from cerebral palsy (8.7% of survivors, 5.6% of live births) and the cerebral palsy rate for infants with birthweight 751-1000 g decreased. The proportion of survivors considered to be normal or mild disabled increased and the part suffering from moderate or severe disability decreased significantly. Conclusions : In spite of long distances and unfavourable climatic conditions VLBW infants can be adequately cared for in this sparsely populated region of Norway.     

Infant Mortality in Madura, Indonesia. Implications for Action

Infant mortality by age at death and birth weight was assessed in a prospective, population-based study in Madura, East Java. Even after elimination of neonatal tetanus by vaccination during pregnancy as a major cause of death, the infant mortality rate was high, viz. 121 per 1000 live births. The majority of live born infants died in the first 6 months of life. Birth weight was the best practical indicator of survival. The implications for Primary Health Care are discussed.     

The changing panorama of cerebral palsy in Sweden. X. Prevalence and origin in the birth‐year period 1999–2002

Abstract Aim: The aim of the study was to describe the prevalence and origin of cerebral palsy (CP), which is the tenth report from the western Swedish study. Methods: A population‐based study covering 85 737 live births in the area in 1999–2002. Birth characteristics and neuroimaging findings were recorded, prevalence of CP was calculated and aetiology was analysed. Results: CP was found in 186 children. The crude prevalence was 2.18 per 1000 live births. The gestational age‐specific prevalence for <28 gestational weeks was 55.6 per 1000 live births, whereas it was 43.7 for 28–31 weeks, 6.1 for 32–36 weeks and 1.43 per 1000 for >36 weeks. There was a female majority among children born at term and a male predominance in children born preterm. Hemiplegia accounted for 38%, diplegia for 32%, tetraplegia for 7%, whereas 17% had dyskinetic CP and 5% ataxia. Neuroimaging showed white‐matter lesions in 31% and cortical/subcortical lesions in 29%. The aetiology was considered to be prenatal in 36%, peri/neonatal in 42%, whereas it remained unclassified in 21%. Conclusion: The decrease in CP prevalence observed since the 1980s had ceased. An increase in children born at term and in dyskinetic CP was found. In children born before 28 weeks of gestation, the prevalence decreased significantly. White‐matter and cortical/subcortical lesions dominated on neuroimaging.     

Early neonatal streptococcal infection

Objective To determine the incidence of early onset Group B Streptococcal (GBS) infection in infants born over a two year period and to determine the outcome of sepsis evaluation in infants born to mothers with GBS colonization. Methods The charts of infants born to mothers with GBS colonization were reviewed for details of sepsis evaluation and management. The microbiology records were used to identify proven cases of GBS septicemia and meningitis in neonates born during the study period. Results Out of a total of 4636 live births in 2 years, there was one infant with culture-proven GBS septicemia, an incidence of 0.2 per 1000 live births. During the study period 83 infants were born to mothers who were known to have GBS carriage at the time of delivery. 73 out of these 83 infants (88%) had sepsis evaluation and received empirical parenteral penicillin for at least 5 days. There were no cases of blood culture-proven GBS sepsis among these 83 infants. However, there were 2 cases of probable sepsis giving an attack rate of 2.4%. All the three infants with definite or probable sepsis were preterm; there were no deaths among these affected infants. Conclusion The overall incidence of early onset GBS sepsis was found to be low when compared to previous reported studies. The strategy of sepsis evaluation and management was found to be effective in preventing death and definite GBS septicemia in infants born to GBS colonized mothers.     

The incidence of congenital angiocardiopathies

Among 229,846 children born alive between 1971 and 1980 in the districts Erfurt and Suhl--this figure coincide with the total number of live births a year in the GDR--1638 patients with structural congenital heart disease were identified. The study is based on an analysis of cardiac patients of this districts and autopsy protocols of all deceased children of the 10-year period. Additional attempt has been made to assess the proportion of cases in adults undergoing cardiac catheterisation with congenital heart disease, undetected in childhood. In our study we found malformations of the heart in 7.13 of 1,000 live births. There is agreement with the results of foreign studies. It is evident, however, that the result requires correction to 8-10/1,000. Our study of the incidence of congenital heart disease in children born between 1971 and 1980 concludes that over the 10-year period the overall incidence has remained constant.     

Fetal and infant death in mono- and dizygotic twins in England and Wales 1982-91

AIM: To quantify the level of risk for stillbirth and infant death in singleton compared with twin pregnancies, using national data; to determine the independent effects of zygosity, sex, and birthweight on these risks in twin pregnancies. METHODS: A retrospective national study was carried out of all singleton and twin birth and death registrations in England and Wales 1982-91, according to sex and birthweight group. Weinberg's rule was applied to the twin pairs to differentiate mono- from dizygotic twins. Relative risks for mono- compared with dizygous twins for both twins being stillbirths and for one of the pair being a stillbirth were determined. For twins where one was stillborn and the other live born, the relative risk of neonatal and infant mortality in the surviving co-twin was determined. RESULTS: There were 6 563 834 registered singletons and 70772 registered twin pairs for the period under study. Monozygotic twins had a relative risk of: 18.91 (95% CI 12.48-28.64) for both twins being stillborn; 1.63 (95% CI 1.48-1.79) for one twin being a stillbirth; and 2.26 (95% CI 1.45-3.52) for the live born co-twin dying as a neonate. When both twins were live born and among singletons, the odds ratio for neonatal mortality of being male was 1.41 (95% CI 1.37-1.45) and there was a highly significant negative association with birthweight. After adjusting for birthweight group and sex, twins had a reduced neonatal mortality compared with singletons: odds ratio 0.91 (95% CI 0.85-0.96). CONCLUSIONS: Fetal death in one of monozygotic twins has serious implications for survival of the co-twin. Monochorionicity is probably the essential feature of the increased risk to the co-twin. It is imperative that all fetal deaths in multiple pregnancies are recorded and chorionicity determined if parents are to be adequately counselled.