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Biomedical subjects

M H Ens-Dokkum

Publications and source records attributed to M H Ens-Dokkum.

15 recordsLinked to original sources
PubMed2000

[Neonatal hearing screening].

Approximately 1 to 2 per thousand live-born infants suffer from a serious perceptive hearing loss. Normal hearing from birth is essential for optimal human development (language and speech, social and emotional development, communicative skills and learning). The earlier the hearing loss is diagnosed the better the prognosis for the infant with a hearing impairment. Suitable methods are now available for neonatal hearing screening: automated measurement of auditory brain stem response and measurement of oto-acoustic emissions. Screening must be viewed as only the first step in a program of diagnosis, treatment and habilitation of these children. The ultimate goal of the implementation of neonatal hearing screening is: identification of bilateral hearing losses before the age of 3 months and start of therapy and counselling before the age of 6 months.

Age Factors↗
PubMed2000

Thiamine-responsive megaloblastic anemia syndrome (TRMA) with cone-rod dystrophy.

Thiamine-responsive megaloblastic anemia (TRMA) is an autosomal recessive disease in which the active thiamine uptake into cells is disturbed. The molecular basis underlying the disorder has been related to mutations in the gene SLC19A2 on chromosome 1q23.3 that encodes a functional thiamine transporter. The protein is predicted to have 12 transmembrane domains. TRMA is characterized by sensorineural deafness, diabetes mellitus, megaloblastic anemia, and cardiomyopathy. Optic nerve atrophy and retinal dystrophy have been reported in a small number of patients. We report a 15-year-old girl with TRMA and cone-rod dystrophy and confirm that retinal dystrophy may form part of the syndrome. Differential diagnosis of syndromes with deafness, diabetes mellitus, and optic nerve atrophy or retinal dystrophy are discussed. The authors suggest that ERG be performed in all patients with TRMA.

Adolescent↗
PubMed1994

Otitis media, respiratory tract infections and hearing loss in pre-term and low birthweight infants.

In 1983, 1338 liveborn infants with a gestational age of less than 32 weeks and/or a birthweight of less than 1500 g, were enrolled in a national follow-up study in The Netherlands. At the age of 5 years, 966 children were alive. Of these, 927 (96%) were assessed on a home visit 2-6 weeks after their fifth birthday by three specially trained paediatricians. An assessment of ENT morbidity was made and compared with ENT morbidity in full-term children of the same age group. Markedly preterm birth or very low birthweight does not seem to be a risk factor for developing middle ear disease in childhood, however, the rate of ENT problems seems to be higher than in the general population of Dutch pre-school children.

Child, Preschool↗
PubMed1994

Comparison of mortality and rates of cerebral palsy in two populations of very low birthweight infants.

Comparisons of mortality and rates of cerebral palsy in different populations can be confusing. This is illustrated by comparing two populations of very low birthweight infants born in the 1980s, one from the Netherlands, the other from the UK (Oxford region). Although a number of biases were controlled for while comparing two large geographically defined populations, by assessing the survivors at similar ages and describing their health status in a standard way, some problems in interpretation of outcome remained. Differences in registration practice of live births at early gestational ages, as well as differences in withholding or withdrawing treatment, which occurred in about half of the cases of neonatal death in the Netherlands and in about one third of those in the Oxford region, may have influenced the incidence of registered live births, neonatal mortality, and the rate of cerebral palsy.

Blindness↗
PubMed1994

Sex difference in disability and handicap at five years of age in children born at very short gestation.

OBJECTIVE: The objective of this study was to examine the relationship between sex and disabilities or handicaps at 5 years of age in infants born at less than 32 weeks gestation. DESIGN: From the nationwide collaborative survey starting in 1983, including perinatal data obtained during routine perinatal care and follow-up assessments by the attending pediatricians, data from 1008 infants fulfilling the criteria were used. At age 5 years, a detailed assessment was performed by three specially trained pediatricians in 96% surviving infants (n = 648), of which 345 were boys. Each child was categorized as disabled or handicapped, using World Health Organization definitions. RESULTS: The prevalence of handicaps was three times greater in boys than in girls (21% vs 7%, odds ratio 3.2). Adjustment for gestational age and birth weight (logistic regression analysis) did not change this (odds ratio 3.5). Further adjustment by including perinatal variables such as idiopathic respiratory distress syndrome did not alter the odds ratios. The male excess in handicaps was not related to lower mortality, and therefore was not a mere consequence of a higher survival rate. The excess in handicaps was found in all assessed areas. CONCLUSIONS: Infants' sex seems to be an important determinant of handicaps. The perinatal variables used in the present study do not explain the difference in handicaps. These findings emphasize the need to include the sex distribution of a study population more systematically in analyses in future studies concerning long-term outcome of very preterm birth or low birth weight.

Child, Preschool↗
PubMed1993

Hearing loss in very preterm and very low birthweight infants at the age of 5 years in a nationwide cohort.

In a geographically defined population of very preterm and very low birthweight infants (gestational age < 32 weeks and/or birthweight < 1500 g) hearing was evaluated in 890 children by pure-tone audiometry at the age of 5 years. Hearing loss was conductive/unspecified in 123 (13.8%) and sensorineural in 13 (1.5%) children. The prevalence of sensorineural hearing loss was 15 times as high as in 5-7 year old children in the Dutch population at large. The sensorineural hearing loss prevalence in very low birthweight and extremely low birthweight infants was similar. On account of communication disorders 10 (1.1%) children were classified as disabled and 6 (0.7%) as handicapped, following the definitions of the International Classification of Impairments, Disabilities, and Handicaps of the World Health Organisation. Children with conductive hearing loss had a higher risk of impairments, disabilities and handicaps of language and speech development, than children with normal hearing, the difference being statistically significant. The same holds for children with sensorineural hearing loss; moreover they had a significantly higher risk of impairments, disabilities and handicaps of mental development. Overall comparison of children with and without sensorineural hearing loss proved that the children with sensorineural hearing loss had a significantly less favourable outcome, based on 15 perinatal factors simultaneously. The age at which sensorineural hearing loss in very preterm and/or very low birthweight infants is detected has to be improved.

Audiometry, Pure-Tone↗
PubMed1993

Breech delivery in very preterm and very low birthweight infants in The Netherlands.

OBJECTIVE: To study the relation between various perinatal factors and the sequelae of very preterm birth, applying logistic regression analysis. DESIGN: In a nationwide collaborative study in the Netherlands, perinatal and follow up data were collected on 899 liveborn singleton nonmalformed infants with gestational age less than 32 weeks or birthweight less than 1500 g born in 1983. MAIN OUTCOME MEASURES: Neonatal mortality rate and total handicap rates (minor and major) in surviving children at two years and five years of age. RESULTS: Comparing breech with vertex presentation, the odds ratio for neonatal mortality (adjusted for duration of pregnancy, birthweight, maternal hypertension and prolonged rupture of membranes) is 1.6 (P < 0.05). Comparing abdominal versus vaginal delivery, the odds ratio indicates equal risks. When breech and vertex presentation are analysed separately it appears that breech presenting infants have a significantly lower mortality risk when born by caesarean section compared with vaginal delivery. However, comparing abdominal versus vaginal delivery in breech presentation, the odds ratio for handicap at five years (0.9) is not significantly different from 1. CONCLUSION: The data presented suggest a reduced neonatal mortality rate in breech presenting infants born by caesarean section but because of the observational design of the study the statistical analysis described only identifies a possible trend and cannot prove the issue.

Breech Presentation↗
PubMed1993

Prediction of height achievement at five years of age in children born very preterm or with very low birth weight: continuation of catch-up growth after two years of age.

To predict height at five years in a cohort of 565 very preterm and/or very low-birth-weight children, hypothesized growth determinants were subjected to discriminant analysis. Many neonatal parameters were not significantly associated with short stature at five years of age. A correct classification of stature (smaller/larger than the 10th percentile at five years of age) could be obtained in 85% of children, using the following variables: height at two years of age; total (or mid) parental height; parental level of education; length at one year of age; hypertension during pregnancy; sex; weight at two years of age; length percentile at one year of age. However, when compared to actual longitudinal data, the false-positive rate was 37%. The survey also demonstrated the continuing catch-up growth in very preterm and very low-birth-weight infants after two years of age.

Biometry↗
PubMed1992

Standardised method of follow-up assessment of preterm infants at the age of 5 years: use of the WHO classification of impairments, disabilities and handicaps. Report from the collaborative Project on Preterm and Small for gestational age infants (POPS) in The Netherlands, 1983.

A nationwide, prospective study was initiated in The Netherlands in 1983, involving 1338 liveborn infants with a gestational age less than 32 weeks and/or a birthweight less than 1500 g. Pre- and perinatal data, methods and results of follow-up until the corrected age of 2 years have been published previously. In this paper, methods of follow-up at the age of 5 years are described. At that age, 966 children were alive, of which 927 (96%) were assessed during a home visit 2 to 6 weeks after their fifth birthday by three specially trained paediatricians. A questionnaire served to collect data on medical history, respiratory function, behaviour and socio-economic factors. Standardised tests were carried out covering the following 10 areas: congenital malformations, neuromotor function, mental development, hearing, visual function, language and speech development, behaviour, musculoskeletal system, respiratory tract and ENT problems, and growth. The outcome was recorded for separate areas and for the child as a whole using the WHO classification of impairments, disabilities and handicaps.

Abnormalities, Multiple↗
PubMed1992

Evaluation of care for the preterm infant: review of literature on follow-up of preterm and low birthweight infants. Report from the collaborative Project on Preterm and Small for Gestational Age Infants (POPS) in The Netherlands.

Since the introduction of neonatal intensive care in the 1960s, mortality in very preterm and very low birthweight infants has been decreasing steadily. Consequently, interest in the outcome of surviving infants is growing. Restriction of health care resources has stressed the need for information concerning the effect of individual treatment components on mortality and morbidity. Concern about the quality in apparently normal survivors has been increasing as well. The current flood of papers on these subjects illustrates the interest in these issues. The first part of this paper reviews the methodology used in follow-up studies in the past decades. It aims at methodological problems that hamper comparison between studies and preclude unequivocal conclusions. New treatment techniques seldom were but should be evaluated by randomised trials. To monitor the combined effects of changing obstetric and neonatal techniques on perinatal outcome, studies in geographically defined populations are recommended using data from early pregnancy until at least preschool age. Comparability of outcomes could be enhanced by international agreement on standardisation of assessment methods and outcome measures. In the second part the results concerning gestational age- and birthweight-specific mortality, impairments and disabilities and the risk factors for such disorders are discussed. Increased survival of even the tiniest infants is clearly established. This increase in survival has not yet been accompanied by an apparent increase in major morbidity. However, many minor impairments are reported, occurring often in combination and predisposing these children to deviations of normal development. Important changes in the manifestation of brain damage appear to occur during development. These findings stress the importance of long-term follow-up studies.

Follow-Up Studies↗
PubMed1992

Five-year outcome of preterm and very low birth weight infants: a comparison between maternal and neonatal transport.

OBJECTIVE: To determine the 5-year outcome of very low birth weight infants referred to tertiary perinatal centers. METHODS: This study was part of a Dutch national collaborative survey of 1338 newborn infants younger than 32 weeks' gestation and/or with a birth weight of less than 1500 g born in 1983. Comparisons were made between maternal transport to university hospital perinatal centers versus delivery in local hospitals, and between neonatal transport to these centers versus treatment in local hospitals. For the 252 survivors meeting the entry criteria for this part of the study, adverse outcome at 5 years of age was evaluated by logistic regression analysis, including 26 perinatal risk factors as confounding variables. Outcome variables were disabilities and handicaps at 5 years as defined by the World Health Organization. RESULTS: There were no differences in handicaps and disabilities between infants born after maternal transport and those born in local hospitals. Handicaps and disabilities in neonates transported versus those treated in local hospitals were also not statistically different despite selection bias. CONCLUSIONS: The previously reported decrease in neonatal mortality risk after maternal transfer is not accompanied by an increased risk of adverse outcome for the survivors. In threatening very preterm delivery, maternal transport to a tertiary center is recommended.

Persons with Disabilities↗
PubMed1991

Impairments, disabilities, and handicaps of very preterm and very-low-birthweight infants at five years of age. The Collaborative Project on Preterm and Small for Gestational Age Infants (POPS) in The Netherlands.

The Project On Preterm and Small for gestational age infants (POPS) was started in the Netherlands in 1983 to investigate the relation between prenatal/perinatal factors and mortality/morbidity in very preterm and very-low-birthweight infants. Of the 1338 liveborn infants (less than 32 weeks and/or less than 1500 g) 966 were enrolled in the five-year (chronological age) follow-up programme; 96% of these children were assessed during a home visit. The overall outcome was expressed as impairments, disabilities, and handicaps according to World Health Organisation criteria. Of the assessed children, 13% had a disability and 14% were handicapped, which are much higher frequencies than those found in the general population. Handicaps were due mainly to abnormalities of neuromotor function, mental development, or language and speech development. Compared with the handicap frequency in the same cohort at two years of age, a more favourable outcome at five years of age was seen in 10%, and a less favourable outcome in 7% of the children. The findings show that most of those high-risk children survived without handicap or serious disability at preschool age.

Child, Preschool↗
PubMed1985

[Infantile myositis].

An eleven-months-old boy with motor developmental delay, muscle weakness, hypotonia and normal tendon reflexes was diagnosed as having infantile polymyositis, histologically with a vasculitis component. Corticosteroid treatment was successful. At the age of 5 years he showed a normal motor pattern.

Humans↗