Functional characterization of the Haemophilus influenzae 4.5S RNA

General registration of congenital malformations was established in Denmark in the early 1960s as a consequence of the thalidomide tragedy. The aims of the present paper are to describe the registration of congenital malformations in Denmark since the thalidomide tragedy, to discuss the limitations of these data and to point towards possibilities for improving the registration of congenital malformations in the future. Important components in a surveillance system are compulsory detailed reporting of malformations shortly after the diagnoses have been made and continuous expert evaluation of all reports. However, since 1995 the registration of congenital malformations in Denmark is based mainly on routine discharge diagnoses from hospitals. A high quality registration of congenital malformations in Denmark would be particularly valuable, because such a register together with existing health related registers and an ongoing large scale cohort study of pregnant women would provide a unique resource for etiological research in congenital malformations. Considering the consequences of congenital malformations for the affected children, their families and society, the establishment of a high quality registration of congenital malformations seems justifiable--and the Swedish experience shows that it is feasible.     

Cold pressor pain: comparing responses of juvenile arthritis patients and their parents

Neuroblastoma is one of the most common cancers of childhood. Some studies have shown an excess of congenital abnormalities in children who have been diagnosed with neuroblastoma. In this study we examined the medical records of all children with neuroblastoma seen at St. Justine Children's Hospital between the years 1977 and 1993. A total of 141 children (131 of French-Canadian ancestry) were included in this study. Twelve children (8.5%) had 21 defined congenital abnormalities (1,490 per 10,000 children). This compared with a rate of 444.3 children with abnormalities per 10,000 live births (4.44%) for all congenital abnormalities in the British Columbia Health Surveillance Registry, 1979-1988 (relative risk = 1.91, P = 0.03). Six of the 12 children had cardiovascular malformations. These and previous results suggest that there may be a common developmental origin to neuroblastoma and to some congenital malformations. Genes that control development may be worthy of further study in these children.     

Registry of activities of the Section of Hemodynamics and Interventional Cardiology in 1997

The results of the Spanish Registry of Hemodynamic and Interventional Cardiology in 1997 are presented. The Registry collects the activity of 83 centers which constitute all the cardiac catheterization laboratories in Spain. The main activity was adult cardiac catheterization in 75 centers and exclusively pediatric cardiac catheterization in 8. A total of 72,370 diagnostic catheterization procedures, 80% coronary angiographies (57,960; 1,462 per million inhabitants), were performed which represents a 13% total increase compared to 1996. Coronary intervention increased by 23% compared to 1996, for a total number of 18,545 procedures. The ratio of coronary interventions per million inhabitants was 468. Success rates of coronary interventions (91.3%) and complications (3.7%) were similar to those registered in previous years. In the specific field of revascularization devices, there has been a dramatic increase in the use of stents. In 1997, coronary stents were employed in 11,417 cases (a 61% increase compared to 1996) which represents 61.5% of all coronary revascularizations procedures. A total of 14,170 prosthesis were implanted, 72% in a elective way, with a low rate of complications (0.95% subacute closure; 1.45% myocardial infarction and 0.75% mortality). Compared to 1996, directional coronary atherectomy (92 procedures) showed a slight decrease, whereas rotational atherectomy (554 procedures) increased by 49% with double the number of centers performing this technique. As in previous years, a slight decrease (7% compared to 1996) in adult valvuloplasties (559 vs 599) was noted. Pediatric interventional procedures decreased by 17% (465 vs 558 procedures) compared to the 1996 Registry.     

Cancer incidence in The Netherlands in 1989 and 1990: first results of the nationwide Netherlands cancer registry. Coordinating Committee for Regional Cancer Registries

The first results are presented of the newly established Netherlands Cancer Registry, which covers the whole Dutch population (approximately 15 million people). The registry receives data on incident cancer cases from nine autonomous regional cancer registries. Notification occurs primarily through the national registry of all pathology and haematology departments, with additional reporting by medical records' departments of all hospitals. Data on cancer patients are abstracted directly from the medical records by trained registration clerks. In the years 1989-1990, the most common cancer sites among males were cancers of the lung, prostate and colon. For females, breast cancer ranked first, followed by cancer of the colon and lung. A comparison with age-adjusted (world standard population) incidence rates reported by other western cancer registries showed a relatively high incidence of lung cancer among males (72.9 per 100,000) and breast cancer among females (76.2 per 100,000). Through its near completeness and the high quality of the registered data, the Netherlands Cancer Registry offers excellent opportunities for epidemiological and clinical research.     

Pediatric rheumatology in the United Kingdom: data from the British Pediatric Rheumatology Group National Diagnostic Register

OBJECTIVE: To establish a computerized national diagnostic register for pediatric rheumatology in the UK; to describe the demography and diagnostic classification of children referred to pediatric rheumatology clinics; to estimate the current incidence of juvenile arthritis (JA) in the UK. METHODS: A diagnostic register was established in 1989: 23 centers have contributed data on all new cases seen since they joined the register; 18 centers have also contributed data on all current attenders. For 2 centers with well defined catchment areas, the incidence of JA was estimated. RESULTS: A total of 4948 cases were registered, of whom 2962 (60%) were female. 1991 (40%) had a diagnosis of JA. The 2nd largest category was mechanical/orthopedic problems (24%). Pauciarticular juvenile chronic arthritis was the most common subtype of JA. Seropositive RA was rare. Tertiary referral centers saw proportionately more JA and district general hospitals saw proportionately more mechanical problems. The annual incidence rate for JA from 2 centers was 10/100,000, and for all rheumatic disorders was 32-42/100,000 children under age 16. CONCLUSION: The relative proportion of patient diagnoses varies between centers. Nevertheless, the incidence of hospital referred JA seems very uniform.     

Sequestration of neutrophils in the hepatic vasculature during endotoxemia is independent of beta 2 integrins and intercellular adhesion molecule-1

Nearly one million of the fifteen million inhabitants of the Netherlands are directly descending from migrant parents. Of these inhabitants, 75% come from former colonies (Surinam and the Netherlands Antilles) and Mediterranean countries like Turkey and Morocco. The mortality rate of Turkish and Moroccan children under 15 years of age is two to three times higher compared to Dutch children. Main causes are perinatal death (including congenital malformations), accidents and drowning, infectious diseases and death during holidays in the country of origin. Inequalities in health between the migrant and Dutch children are demonstrated in several surveys conducted at both national and local levels. Apart from socio-economic differences, this can be attributed to three main causes; different pathology due to imported infectious diseases or inherited disorders, different life style and socio-cultural factors. The cumulative factor explains the differences in health, comparable with several other countries in Europe where migrants from Mediterranean countries and former colonies live. CONCLUSION: Migration has an increasing impact on the daily practice of Dutch paediatricians as well as elsewhere in Europe. Inclusion of intercultural and international aspects of health in the curriculum of the medical paediatric education is paramount.     

Cardiac teratogenesis of halogenated hydrocarbon-contaminated drinking water

OBJECTIVES: The purpose of this study was to test the hypothesis that administration of trichloroethylene and dichloroethylene to pregnant rats during organogenesis would produce a significant fetal cardiac teratogenic effect. It was also hypothesized that administration of these compounds only before pregnancy would not be associated with fetal cardiac teratogenesis. BACKGROUND: Epidemiologic observations demonstrated an increased number of congenital cardiac defects in children whose mother resided in an area with drinking water contaminated by trichloroethylene and dichloroethylene. A prior provocative intrauterine exposure study in rats established a positive link between these contaminants and an increased number of fetal hearts with congenital cardiac defects. METHODS: Sprague-Dawley rats were given pure tap drinking water (control subjects) or water contaminated with high or low dose of trichloroethylene or dichloroethylene (experimental groups) during prepregnancy only, prepregnancy and pregnancy or during pregnancy alone. RESULTS: A total of 2,045 fetuses were examined. Trichloroethylene or dichloroethylene delivered exclusively in the period before pregnancy caused no increase in congenital cardiac malformations over the control level. Compared with the control group, rats exposed to these agents both before and during pregnancy, had a significantly greater number of fetuses with cogenital cardiac malformations. Trichloroethylene (high dose only) administered only during pregnancy produced a significant increase in cardiac defects. Other fetal variables, including noncardiac congenital abnormalities, showed no significant difference between control and treated groups. CONCLUSIONS: Trichloroethylene and dichloroethylene administered during organogenesis are cardiac, but not general, teratogens. The data indicate that these agents administered in drinking water to pregnant rats caused an increased number of congenital cardiac defects in rat fetuses.     

Towards achieving the Health of the Nation target for cervical cancer: accuracy of cancer registration

To measure the accuracy of incidence rates for invasive cervical cancer derived from cancer registration data, a review of histopathology records was undertaken for all cases of invasive cervical cancer, a 20 per cent random sample of CIN 3 registrations and all cases of cancer of the uterus not otherwise specified (NOS) from Greater Manchester, which were registered by the North Western Regional Cancer Registry between 1988 and 1989. The subjects were 386 cases of invasive cervical cancer, 313 CIN 3 registrations and 69 cases of cancer of the uterus NOS. The main outcome measure was the incidence rate of invasive cervical cancer in Greater Manchester after adjusting for errors in cancer registration. It was found that 375 (97 per cent) of the 386 cases of invasive cervical cancer were verified against the original pathology report but 11 cases (3 per cent) had been registered as invasive cervical cancer in error. Nine cases of invasive cervical cancer were found to have been misclassified as CIN 3, and 10 cases as cancer of the uterus NOS. As the CIN 3 cases were a 20 per cent random sample of all CIN 3 registrations, the best estimate of the number of cases of invasive cervical cancer misclassified as CIN 3 is 45, which makes a total under-registration of 55 cases over the two-year period. After adjusting for under-and over-registration, the incidence rate of invasive cancer of the cervix in Greater Manchester for 1988-1989 increased from 16.5 to 18.1 per 100,000 population.(ABSTRACT TRUNCATED AT 250 WORDS)     

The validity of data in registration of operations. A quality analysis

In Denmark all operations are registered centrally and identified by a unique number describing the exact operation. The aim of this study has been to validate the data, which are registered. Within a period of four months all registrations were carried out in duplo in two independent registers. The contents of the two registers were compared manually. A total of 1568 operations were registered. In one of the registrations 102 (6.5%) were missing and in the other 112 (7.1%). In 70 cases (4.5%) a discrepancy was found between the registers according to type or date of operation. It is concluded, that about 10% of all registrations in such a register are wrong. A better validity of data might be obtained by a simplification of registration procedures. Double registration is an effective way to locate the mistakes.     

Clinically applicable bulk isolation of blood CD34+ cells for autografting in children

The quality of population-based cancer registries has been measured by the indices of the proportion of total incident cases (DCO%) registered by death certificate only (DCO), and the ratio of incidence to mortality (I/D ratio). Recently it has been recommended that DCO% should be used as an index for the reliability of diagnosing cancers and that the proportion of cases first notified via death certificate (DCN, DCN%) be used as an index for the completeness of registration. Parkin introduced a method to estimate the registration rate, the estimated proportion of the "true incidence" that are registered in population-based registries. We recommend a modified method for estimating the registration rate for cancer registries where DCN% is relatively high, as it is in Japan, as Parkin's method may overestimate the registration rate. The method is as follows: the registration rate = (1-DCN% x 1/D ratio)/(1-DCN%). The registration rates at the Osaka Cancer Registry between 1966 and 1992 were estimated using our method. During this period, the yearly registration rate was 74.6-78.4% for males and 69.1-73.3% for females. When the cancer cases were looked at according to site, the yearly registration rate was 74.2-81.6% for stomach cancer, 81.2-89.3% for lung cancer, and 71.3-76.9% for uterine cancer. These results show that the registration rate is high for cancers that have an unfavorable prognosis and low for cancers that have a favorable prognosis. We recommend that all cancer registries in Japan calculate the completeness of registration by utilizing DCN defined as the sum of DCO plus cases not reported as cancer but with supportive clinical information of such obtained through survey of the registry for DCN.     

Observations of the treatment of women in the United States with myocardial infarction: a report from the National Registry of Myocardial Infarction-I

BACKGROUND: To determine whether there are sex differences in the demographics, treatment, and outcome of patients with acute myocardial infarction in the United States, data from the National Registry of Myocardial Infarction-I from September 1990 to September 1994 were examined. METHODS: The National Registry of Myocardial Infarction-I is a national observational database consisting of 1234 US hospitals in which each hospital submits data from each patient with acute myocardial infarction to a central data collection center. For these analyses, the following variables were examined in 354 435 patients with acute myocardial infarction: demographics; use of medical therapy including thrombolytic agents; use of procedures including cardiac catheterization, percutaneous transluminal coronary angioplasty, and coronary artery bypass surgery; length of hospital stay; adverse events (stroke, major bleeding, or recurrent myocardial infarction); and causes of death. RESULTS: In comparison with men, women experiencing acute myocardial infarction in the United States are older, with 55.7% older than 70 years. Women have a higher mortality rate than men even when controlled for age and die less often from arrhythmia but more often from cardiac rupture whether or not thrombolytic therapy is used. Treatment with aspirin, heparin, or beta-blockers is less frequent in women. When thrombolytic therapy is used, women are treated an average of almost 14 minutes later than men and experience a greater incidence of major bleeding. Cardiac catheterization, percutaneous transluminal coronary angioplasty, and coronary artery bypass surgery are used less often in women. CONCLUSIONS: Observations from the National Registry of Myocardial Infarction-I document important sex differences in demographics, treatment, and outcome of patients with acute myocardial infarction in the United States.     

Seasonal distribution of acute myocardial infarction in the second National Registry of Myocardial Infarction

OBJECTIVES: This observational study sought to determine whether cases of acute myocardial infarction (AMI) reported to the second National Registry of Myocardial Infarction (NRMI-2) varied by season. BACKGROUND: The existence of circadian variation in the onset of AMI is well established. Examination of this periodicity has led to new insights into pathophysiologic triggers of atherosclerotic plaque rupture. Although a seasonal pattern for mortality from AMI has been previously noted, it remains unclear whether the occurrence of AMI also displays a seasonal rhythmicity. Documentation of such a pattern may foster investigation of new pathophysiologic determinants of plaque rupture and intracoronary thrombosis. METHODS: We analyzed the number of cases of AMI reported to NRMI-2 by season during the period July 1, 1994 to July 31, 1996. Data were normalized so that seasonal occurrence of AMI was reported according to a standard 90-day length. RESULTS: A total of 259,891 cases of AMI were analyzed during the study period. Approximately 53% more cases were reported in winter than during the summer. The same seasonal pattern (decreasing occurrence of reported cases from winter to fall to spring to summer) was seen in men and women, in different age groups and in 9 of 10 geographic areas. In-hospital case fatality rates for AMI also followed a seasonal pattern, with a peak of 9% in winter. CONCLUSION: The present results suggest that there is a seasonal pattern in the occurrence of AMIs reported to NRMI-2 that is characterized by a marked peak of cases in the winter months and a nadir in the summer months. This pattern was seen in all subgroups analyzed as well as in different geographic areas. These findings suggest that the chronobiology of seasonal variation in AMI may be affected by variables independent of climate.     

Descriptive epidemiology of IDDM in Hokkaido, Japan: the Childhood IDDM Hokkaido Registry

OBJECTIVE: To identify the incidence of IDDM with regard to sex, age, family history of diabetes, season, and 5-year period of childhood IDDM among children ages 0-14 years from a population-based epidemiological study in Hokkaido, Japan, from 1973 to 1992. RESEARCH DESIGN AND METHODS: Registration of all new IDDM cases in Hokkaido was conducted by the Childhood IDDM Hokkaido Registry Study Group from 1973 to 1992. The cases were selected from among 1) patients who were admitted to the member hospitals of the study group, 2) patients who answered a questionnaire distributed to hospitals and diabetic clinics throughout Hokkaido, and 3) patients whose cases were recorded in free-treatment medical records of urban and rural districts. The case ascertainment rate was estimated to be 100%. Differences in incidence with regard to sex, age, family history of diabetes, season, and year period were analyzed by the Poisson regression analysis by GENMOD. RESULTS: During the 20-year period studied, 396 cases (181 boys, 215 girls) of abrupt-onset IDDM were registered. Statistically significant differences in annual incidence were found according to sex (female), age (8-14 years), history (having no diabetes in family), season (spring), and 5-year period. CONCLUSIONS: This is the first population-based, long-term epidemiological study of childhood IDDM from Japan. We observed a significantly higher annual incidence (per 100,000/year) of IDDM in female subjects (1.81), older age-groups (2.25 for 8-14 years), subjects with no family history of diabetes (1.26), diabetes onset in the spring (2.20), and an increased trend over the 20 years. In addition, the heterogeneity of IDDM among Japanese children needs to be elucidated.     

Registry of experimental cancers of the National Cancer Institute

This report describes the organization and functions of the Registry of Experimental Cancers and gives the chronology of events that led to its establishment. Currently 21,500 accessions have been coded; the vast majority are spontaneous and induced cancers, chiefly of rodents, and also a wide variety of nonneoplastic diseases. Accessions are accepted from contributors working in laboratories in this country and abroad. The material is available for study by responsible scientists, and a limited number of study sets is available for loan on request.     

Visual impairment in Swedish children. III. Diagnoses

PURPOSE: To gain an overview of the spectrum of diagnoses among Swedish visually impaired children. METHODS: An epidemiological study of all known visually impaired children was made by review of medical records. RESULTS AND CONCLUSION: In all we found 2373 children, 0-19 years of age, with an age-specific prevalence of 10.9/10,000. The two largest diagnostic groups included neuro-ophthalmological and retinal diseases. The most frequent disorders were cerebral visual impairment, non-hereditary optic atrophy, retinal dystrophy (when regarded as a general entity), congenital hypoplasia of the optic nerve and congenital cataract. Nystagmus secondary to brain disorder, albinism, congenital nystagmus, retinopathy of prematurity and high myopia were also found in a considerable number of patients. The leading diagnoses in children with WHO-defined childhood blindness were non-hereditary optic atrophy, cerebral visual impairment and retinopathy of prematurity. A large proportion of the children, especially in the groups with neuro-ophthalmological disorders and malformations of the posterior segment had additional impairments, emphasizing the importance of a multi-disciplinary approach when assessing multi-handicapped children.     

Children conceived by in vitro fertilisation after fresh embryo transfer

AIMS: To compare the outcome in in vitro fertilisation (IVF) children (after fresh embryo transfer) from multiple and singleton births with one another, and with normally conceived control children. METHODS: A cohort of 278 children (150 singletons, 100 twins, 24 triplets and four quadruplets), conceived by IVF after three fresh embryos had been transferred, born between October 1984 and December 1991, and 278 normally conceived control children (all singletons), were followed up for four years after birth. They were assessed for neonatal conditions, minor congenital anomalies, major congenital malformations, cerebral palsy and other disabilities. Control children, all born at term, were matched for age, sex and social class. RESULTS: The ratio of male:female births was 1.03. Forty six per cent of IVF children were from multiple births; 34.9% were from preterm deliveries; and 43.2% weighed less than 2500 g at birth. The IVF singletons were on average born one week earlier than the controls, weighed 400 g less, and had a threefold greater chance of being born by caesarean section. The higher percentage of preterm deliveries was largely due to multiple births and they contributed to neonatal conditions in 45.0% of all IVF children. The types of congenital abnormalities varied: 3.6% of IVF children and 2.5% of controls had minor congenital anomalies, and 2.5% of IVF children and none of the controls had major congenital malformations. The numbers of each specific type of congenital abnormality were small and were not significantly related to multiple births. IVF children (2.1%) and 0.4% of the controls had mild/moderate disabilities. They were all from multiple births, including two children with cerebral palsy who were triplets. CONCLUSIONS: The outcome of IVF treatment leading to multiple births is less satisfactory than that in singletons because of neonatal conditions associated with preterm delivery and disabilities in later childhood. A reduction of multiple pregnancies by limiting the transfer of embryos to two instead of three remains a high priority.     

Is detection and treatment of familial hypercholesterolemia indicated in children?

Familial hypercholesterolaemia (FH) is a congenital metabolic disorder predisposing to severe atherosclerosis resulting in coronary heart disease sometimes even at early adult age. Children with FH lack the stigmata at physical examination and measuring the cholesterol level does not always enable the clinician to make the diagnosis. In about 70% of the cases, the diagnosis of FH in childhood can be made by means of molecular-biological examination, by demonstrating the underlying defect of the LDL cholesterol receptor gene. In the remaining cases, the combination of the positive family history for cardiovascular diseases and increased total cholesterol and LDL cholesterol levels should suggest the diagnosis of FH. Pharmaceutical agents inhibiting the cholesterol synthesis have been researched very little in children and are not registered in the Netherlands. Nevertheless, drug treatment of children with FH is advisable because of the better possibilities to make a definite diagnosis and the early occurrence of coronary heart disease. If this treatment were indicated before patients reach adult age, the question arises whether screening for FH of children in families in which this disorder prevails, should not be promoted more strongly.     

Urolithiasis in childhood: surgery and lithotripsy

From 1982 through 1993, 174 interventions in 160 infants and children with urolithiasis were performed at our department. There were 101 boys and 59 girls, mean age 8.9 years. The main causes of the interventions were congenital malformations in 62, followed by recurrent urinary tract infections in 29, previous operations in 17, and hypercalciuria in 35 patients. In 17 children, the cause of urolithiasis remained unknown. As a therapeutic option, extracorporeal shock-wave lithotripsy (ESWL) has become available since 1988. The malformations and postoperative conditions are surgically corrected, and the calculi removed in the same act. For post-infectious, idiopathic or hypercalciuric calculi, ESWL has been used in the majority of patients since 1988. Thus, a total of 129 calculi were removed by operation and 47 by ESWL, whereas 18 calculi were endoscopically extracted. There were no complications after either ESWL or open surgery. Calculi in congenital malformations or as a result of operation are still removed by open surgery. In other cases, ESWL is the method of choice.     

Is Down syndrome a risk factor for poor outcome after repair of congenital heart defects?

Down syndrome is commonly associated with significant congenital heart disease with the potential for early development of pulmonary hypertension. As such, children with Down syndrome may be at increased risk for both perioperative and long-term mortality. The purpose of this study, using data collected from a population-based outcomes study, is to analyze the potential role that Down syndrome plays in the outcome of surgically "corrected" congenital heart disease. Data were collected from a registry of all Oregon residents who, in the period 1958 to the present, had a reparative operation for one of 14 congenital cardiac malformations when younger than 18 years (N = 3965 patients). Down syndrome was present in 289 (7%) of the total registry patients. In evaluating the cardiac mortality associated with Down syndrome for each of the repaired cardiac malformations, only complete atrioventricular septal defect was associated with significantly higher perioperative (13% vs 5%) as well as higher overall late cardiac mortality through 20 years after the operation (20% vs 5%; p = 0.04). The survival outcomes for each of the other cardiac malformations were similar for children with and without Down syndrome.     

Detection of subtle changes in the brains of infants and children via subvoxel registration and subtraction of serial MR images

PURPOSE: To compare conventional two-dimensional multisection images with registered three-dimensional volume and subtraction images for detecting subtle changes in the brains of infants and children. METHODS: Twenty-six patients (24 with hemorrhagic/ischemic lesions) and one each with perinatal infection and Sturge-Weber disease were examined on two or more occasions with conventional multisection T1- and T2-weighted sequences as well as with 3-D T1-weighted volume sequences. A registration program was used to match the volume images to subvoxel dimensions, and subtracted images (second volume set minus the first) were obtained. The multisection images were compared with the 3-D and subtracted images and graded for detection of changes in a variety of brain structures. RESULTS: In 16% to 33% of comparisons of different structures, the multisection images and the 3-D registered and subtracted images showed changes equally well. The 3-D registered and subtracted images were better than the multisection images in 67% to 84% of comparisons for detection of changes in the cerebral hemispheres, ventricles, brain stem, cerebellum, and in lesions. Statistically significant differences were found between the graded performance of the registered 3-D images and the conventional 2-D images in detecting cerebral infarction and hypoxic ischemic encephalopathy. In the late phase following neonatal cerebral infarction (1 to 11 months), the 3-D registered and subtracted images revealed growth of the brain at the margins of the lesions. CONCLUSION: Subvoxel registration of serial MR images may be of value in detecting subtle changes in the brains of infants and children.