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Biomedical subjects

M Hadders-Algra

Publications and source records attributed to M Hadders-Algra.

At least 19 recordsLinked to original sources
PubMed2006

Improved motor development and good long-term glycaemic control with sulfonylurea treatment in a patient with the syndrome of intermediate developmental delay, early-onset generalised epilepsy and neonatal diabetes associated with the V59M mutation in the KCNJ11 gene.

AIMS/HYPOTHESIS: Activating mutations in the KCNJ11 gene encoding the Kir6.2 subunit of the K(ATP) channels in pancreatic beta cells are a common cause of neonatal diabetes. One-third of patients also have developmental delay, which probably results from mutated K(ATP) channels in muscle, nerve and brain. Sulfonylureas, which bind to the sulfonylurea receptor 1 subunit of the K(ATP) channel, can replace insulin injections in patients with KCNJ11 mutations. The aim of this study was to investigate the long-term outcome and impact on neurological features of sulfonylurea treatment. METHODS: We report the response to sulfonylurea treatment in a boy with neonatal diabetes and marked developmental delay resulting from the KCNJ11 mutation V59M. RESULTS: Glibenclamide (glyburide) treatment was started at 23 months and resulted in insulin being discontinued, lower overall glycaemia, reduced glucose fluctuations and reduced hypoglycaemia. Good control (HbA(1c) 6.5%) was maintained 2 years after discontinuing insulin, despite a reduction in the glibenclamide dose (from 0.41 to 0.11 mg.kg(-1).day(-1)). Within 1 month of starting glibenclamide there was marked improvement in motor function, resulting in the patient progressing from being unable to stand unaided to walking independently, but there was no improvement in mental function. CONCLUSIONS/INTERPRETATION: This 2-year follow-up of a patient highlights that sulfonylurea treatment can result in prolonged, excellent glycaemic control and may improve motor features, but not mental features, associated with KCNJ11 mutations. This suggests that the neurological actions of sulfonylurea are initially principally on peripheral (nerve or muscle) rather than on central (brain) K(ATP) channels. Early molecular diagnosis is important in patients with neonatal diabetes and neurological features.

Amino Acid Substitution↗
PubMed2006

Psychiatric morbidity is related to a chain of prenatal and perinatal adversities.

BACKGROUND: Most studies on the effects of early risk factors on future mental health focus on specific obstetric complications as risk factors for specific disorders. However, obstetric complications rarely occur in isolation, and the same holds for psychiatric problems. AIMS: To study prenatal and perinatal risk factors for psychiatric multimorbidity in early adulthood and to determine whether these differ from risk factors for monomorbidity. STUDY DESIGN: Monomorbidity and multimorbidity of six types of psychiatric disorders were determined by a standardised psychiatric interview. Using univariate and multivariate logistic and ordinal logistic regression, associations of mono- and multimorbidity with prospectively collected early risk factors were examined. SUBJECTS: Two hundred and eighty-five young adults selected from a prospective birth cohort on the basis of their Obstetric Optimality Scores. OUTCOME MEASURES: Six types of psychiatric disorder and their co-occurrence. RESULTS: Monomorbidity was related to isolated early risk factors such as low birth weight or a low Apgar score, and to an accumulation of unfavourable pre- and perinatal events. Multimorbidity on the other hand, was only related to a chain of pre- and perinatal adversities. CONCLUSION: Research and prevention strategies should not focus solely on isolated early risk factors, but also on the entire pre- and perinatal situation.

Adult↗
PubMed2006

Prescription of the first prosthesis and later use in children with congenital unilateral upper limb deficiency: A systematic review.

BACKGROUND: The prosthetic rejection rates in children with an upper limb transversal reduction deficiency are considerable. It is unclear whether the timing of the first prescription of the prosthesis contributes to the rejection rates. OBJECTIVE: To reveal whether scientific evidence is available in literature to confirm the hypothesis that the first prosthesis of children with an upper limb deficiency should be prescribed before two years of age. We expect lower rejection rates and better functional outcomes in children fitted at young age. METHODS: A computerized search was performed in several databases (Medline, Embase, Cinahl, Amed, Psycinfo, PiCarta and the Cochrane database). A combination of the following keywords and their synonyms was used: "prostheses, upper limb, upper extremity, arm and congenital". Furthermore, references of conference reports, references of most relevant studies, citations of most relevant studies and related articles were checked for relevancy. RESULTS: The search yielded 285 publications, of which four studies met the selection criteria. The methodological quality of the studies was low. All studies showed a trend of lower rejection rates in children who were provided with their first prosthesis at less than two years of age. The pooled odds ratio of two studies showed a higher rejection rate in children who were fitted over two years of age (pooled OR = 3.6, 95% CI 1.6 - 8.0). No scientific evidence was found concerning the relation between the age at which a prosthesis was prescribed for the first time and functional outcomes. CONCLUSION: In literature only little evidence was found for a relationship between the fitting of a first prosthesis in children with a congenital upper limb deficiency and rejection rates or functional outcomes. As such, clinical practice of the introduction of a prosthesis is guided by clinical experience rather than by evidence-based medicine.

Age Factors↗
PubMed2005

Long-chain polyunsaturated fatty acids and neurological developmental outcome at 18 months in healthy term infants.

AIM: Previously, we found a beneficial effect of 2 mo supplementation of infant formula with long-chain polyunsaturated fatty acids (LC-PUFA) on neurological condition at 3 mo in healthy term infants. The aim of the present follow-up study was to evaluate whether the effect on neurological condition persists until 18 mo. METHODS: A prospective, double-blind, randomized control study was conducted. Three groups were formed: a control (CF; n = 169), an LC-PUFA-supplemented (LF; n = 146) and a breastfed (BF; n = 159) group. Information on potential confounders was collected at enrolment. At the age of 18 mo, neurodevelopmental condition was assessed by the age-specific neurological examination of Hempel and the Bayley scales. The Hempel assessment resulted in a clinical neurological diagnosis, a total optimality score and a score on the fluency of motility. The Bayley scales resulted in mental and psychomotor developmental indices. Attrition at 18 mo was 5.5% and non-selective. Multivariate regression analyses were carried out to evaluate the effect of type of feeding while adjusting for confounders. RESULTS: None of the children had developed cerebral palsy and 23 (CF: n = 8; LF: n = 10; BF: n = 5) showed minor neurological dysfunction. The groups did not show statistically significant differences in clinical neurological condition, neurological optimality score, fluency score, and the psychomotor and mental development indices. Multivariate analysis confirmed that there was no effect of type of feeding on neurological condition. CONCLUSION: This study indicates that the beneficial neurodevelopmental effect of 2 mo LC-PUFA supplementation in healthy term infants can not be detected at the age of 18 mo.

Age Factors↗
PubMed2004

Obstetric optimality and emotional problems and substance use in young adulthood.

BACKGROUND: Pregnancy and delivery are complex processes, and isolated obstetric complications rare and often accompanied or followed by a number of others. AIMS: To study the relationship between the overall obstetric situation (as opposed to single obstetric risk factors) and emotional and substance use disorders in young adulthood, and to analyse whether these links are mediated by temperaments in childhood. STUDY DESIGN: In a prospective birth cohort (n=3162), questionnaires were sent to mothers and teachers when the child was 7-10 years old, and to the children when they were 20-25 years old. SUBJECTS: Six hundred and eighty-two cohort members with complete data sets at three ages (perinatal, childhood, and young adulthood). OUTCOME MEASURES: Emotional problems and substance use in young adulthood. MAIN RESULTS: Substance use in young adulthood was predicted better by the overall obstetrical optimality score than emotional problems were. Links studied were stronger for men than for women. CONCLUSIONS: This study demonstrated the use of an aggregated obstetrical optimality score in analysing the associations between early risk factors and emotional problems and substance use in young adulthood.

Adult↗
PubMed2003

Effect of antenatal exposure to maternal smoking on behavioural problems and academic achievement in childhood: prospective evidence from a Dutch birth cohort.

AIM: To examine effects of maternal smoking during pregnancy on academic achievement and emotional and behavioural problems during childhood. METHODS: Least squares regression was used to examine associations between maternal smoking prior to delivery and subsequent academic performance and behaviour of 1186 children aged 5.5-11 years. Crude associations were adjusted for risk factors that were significantly related to the respective outcomes. MAIN OUTCOME MEASURES: Parents and teachers were asked to indicate, on a 10-item questionnaire, whether they regarded the child as more, the same, or less shy/withdrawn (internalising), troublesome (externalising), and attention deficient than the average child. Reading, spelling, and arithmetic performance levels were assessed with short standardised Dutch tests. RESULTS: After adjustment for confounders like socio-economic status and pre- and perinatal complications, children of mothers who smoked during pregnancy showed more signs of attention deficit and displayed higher levels of troublesome (externalising) behaviour than non-cigarette-exposed children. Also, children of smoking mothers performed worse on arithmetic and spelling tasks. Spelling problems were more pronounced when the mother continued to smoke after the child's birth. Excessively withdrawn (internalising) behaviour was not related to maternal smoking but to factors like the mother's use of psychotropic drugs and bottle-instead of breastfeeding. CONCLUSION: Perinatal antecedents of internalising behaviour on the one hand and externalising behaviour, attention deficit, and learning problems on the other seem to be distinct. Only the latter are independently associated with maternal smoking during pregnancy and thus potentially amenable to early preventive effort, for instance, through continued health education emphasising the health hazards of nicotine use by pregnant women.

Adult↗
PubMed2003

Is hypotension a major risk factor for neurological morbidity at term age in very preterm infants?

OBJECTIVE: To investigate the influence of perinatal risk factors, especially hypotension, on neuromotor status at term in surviving preterm infants born before 32 weeks of gestation. METHODS: This study is part of the Leiden Follow-Up Project on Prematurity: a prospective, regional study of 266 live born infants with a gestational age (GA) < 32 weeks born in 1996-1997. Twenty-eight infants died before term age. Two hundred and eleven infants were examined neurologically at term according to Prechtl. The findings were classified as normal (N), mildly abnormal (MA) or definitely abnormal (DA). Hypotension was defined as a mean arterial blood pressure (MABP) < 30 mm Hg on at least two occasions. RESULTS: One hundred and six (50%) infants were classified as neurologically N, 92 (44%) infants were classified as MA and 13 (6%) infants as DA. Hypotension, bronchopulmonary dysplasia (BPD), flaring and cystic periventricular leucomalacia (PVL) were risk factors for neurological morbidity. Of the 68 infants with hypotension, 33 (49%) were classified as MA and 7 (10%) as DA. Of the 141 infants without hypotension, 58 (41%) were MA, and 5 (4%) were DA. The odds ratio of hypotension for neurological morbidity was 1.9 (95% CI 1.06-3.40), adjusted for gestational age, birth weight, small for gestational age (SGA) and gender, it was 1.96 (95% CI 1.02-3.77). The adjusted odds ratio of PVL was 18.6 (4.4-78.5), of flaring was 2.37 (1.18-4.74) and of BPD was 2.44 (1.08-5.5). CONCLUSIONS: Apart from gestational age, periventricular leucomalacia, and bronchopulmonary dysplasia, hypotension in preterm infants is a major risk factor for neurological morbidity at term.

Gestational Age↗
PubMed2003

The neurology of learning and behavioural problems in pre-adolescent children.

OBJECTIVE: The aim of the study was to evaluate whether in pre-adolescent children specific types of minor neurological dysfunction (MND) are related to specific types of learning and behavioural problems, and whether it is the type or the severity of neurological dysfunction that matters most. METHOD: A total of 1186, 5.5-11-year-old, children were examined for the presence of clusters of signs of MND. Parents and teachers completed brief behaviour rating scales. In school-aged children (n = 580) cognitive achievement was assessed using standardized Dutch tests. Least-squares regression was used to examine neurobehavioural and neurocognitive links. RESULTS: Children with more MND clusters performed worse scholastically and had more signs of attention deficit than others. Externalizing and internalizing behaviour were only linked to specific forms of MND. CONCLUSION: Evaluation of the neurological condition of the child with learning and behavioural problems offers insight into the aetiology and pathogenesis of these problems.

Child↗
PubMed2003

Exclusive breastfeeding of healthy term infants for at least 6 weeks improves neurological condition.

To investigate the minimal duration of exclusive breastfeeding for optimal neurological outcome, we assessed the quality of general movements (GM) at 3 mo of 147 breastfed healthy term infants that were followed from birth. The quality of GM is a sensitive marker of neurological condition. The quality of GM was classified as normal-optimal, normal-suboptimal, mildly abnormal and definitely abnormal. Information on social and pre- and perinatal conditions and the duration of breastfeeding was collected prospectively. Logistical regression analyses were used to adjust for confounders. There was a positive association between breastfeeding duration and movement quality, with a saturation effect at the age of approximately 6 wk. In the group of infants breastfed for < or = 6 wk (n = 55), 18% exhibited normal-optimal GM, 47% normal-suboptimal GM, and 47% mildly abnormal GM. In contrast, in the group of infants breastfed for > 6 wk (n = 92), 43% exhibited normal-optimal GM, 45% normal-suboptimal GM, and 12% mildly abnormal GM. Exclusive breastfeeding for >6 wk was therefore associated with markedly less abnormal and more normal-optimal GM. Thus, we conclude that breastfeeding for > 6 wk might improve the neurological condition in infants.

Breast Feeding↗
PubMed2001

Influence of two different sitting positions on postural adjustments in children with spastic diplegia.

The present study addressed the question whether the deviant postural adjustments in children with spastic diplegia can be attributed to their crouched sitting position or primarily to their neural deficit. Postural adjustments during sitting in an erect and in a crouched position on a movable platform were assessed in 10 children, aged 3 to 7 years 6 months, with mild-to-severe forms of spastic diplegia and 10 age- and sex-matched control children. Multiple surface EMGs of neck, trunk, and leg muscles and kinematics of head, body sway, and pelvis were recorded during forward and backward translations. The children with normal motor development showed a distinct adaptation of postural adjustments to sitting position. The children with cerebral palsy (CP) had a deficient adaptational capacity which was more pronounced in the erect than in the crouched position. Thus, the crouched sitting position did not induce postural deficiency but seemed to offer a solution to the sensory-motor problem of the instability experienced. Children with severe diplegia exhibited a lack of direction specificity in the leg muscles during backward body sway, which points to a basic deficit in postural control. In addition, these children showed marked dysfunctions in the precise tuning of the postural adjustments to task-specific conditions. In the children with mild-to-moderate forms of CP the basic level of control was intact.

Adaptation, Physiological↗
PubMed2001

Early brain damage and the development of motor behavior in children: clues for therapeutic intervention?

The Neuronal Group Selection Theory (NGST) could offer new insights into the mechanisms directing motor disorders, such as cerebral palsy and developmental coordination disorder. According to NGST, normal motor development is characterized by two phases of variability. Variation is not at random but determined by criteria set by genetic information. Development starts with the phase of primary variability, during which variation in motor behavior is not geared to external conditions. At function-specific ages secondary variability starts, during which motor performance can be adapted to specific situations. In both forms, of variability, selection on the basis of afferent information plays a significant role. From the NGST point of view, children with pre- or perinatally acquired brain damage, such as children with cerebral palsy and part of the children with developmental coordination disorder, suffer from stereotyped motor behavior, produced by a limited repertoire or primary (sub)cortical neuronal networks. These children also have problems in selecting the most efficient neuronal activity, due to deficits in the processing of sensory information. Therefore, NGST suggests that intervention in these children at early age should aim at an enlargement of the primary neuronal networks. With increasing age, the emphasis of intervention could shift to the provision of ample opportunities for active practice, which might form a compensation for the impaired selection.

Brain Damage, Chronic↗
PubMed2001

Are moderate degrees of hyperbilirubinemia in healthy term neonates really safe for the brain?

In 1994 the American Academy of Pediatrics recommended more liberal rules for the treatment of hyperbilirubinemia in healthy term newborns. Yet, the safety of moderate degrees of hyperbilirubinemia in healthy term newborns is debated. To evaluate the safety of moderate degrees of hyperbilirubinemia, we assessed neurologic condition of 20 healthy nonhemolytic term newborns with peak total serum bilirubin levels of 233-444 micromol/L and 20 control infants matched for sex and gestational age at birth. Neurologic condition was evaluated with techniques focusing on the presence of minor neurologic dysfunction: in the newborn period according to Prechtl, at 3 mo on the basis of the quality of general movements, and at 12 mo according to Touwen. Moderate hyperbilirubinemia turned out to be associated with a significant increase in minor neurologic dysfunction throughout the first year of life. At 12 mo a strong dose-response relationship between the degree of hyperbilirubinemia and the severity of minor neurologic dysfunction was present. Our results indicate that total serum bilirubin levels 335 micromol/L should be avoided.

Bilirubin↗
PubMed2001

Evaluation of motor function in young infants by means of the assessment of general movements: a review.

PURPOSE: Optimal management of children with developmental disorders, such as cerebral palsy (CP), requires detection at an early age. The purpose of this paper is to review the predictive value of various forms of traditional neonatal neurological examination and that of a new form of neuromotor assessment of young infants, based on the assessment of the quality of general movements (GMs). SUMMARY OF KEY POINTS: The technique of GM assessment is presented and the features of normal, mildly abnormal and definitely abnormal GMs discussed. Essential to GM assessment is the Gestalt evaluation of movement complexity and variation. The quality of GMs at two to four months postterm has been found to have the highest predictive value. The presence of definitely abnormal GMs at this age, ie, GMs devoid of complexity and variation, puts a child at very high risk for CP. CONCLUSIONS: This implies that definitely abnormal GMs at two to four months are an indication for early physical therapy intervention.

Journal Article↗
PubMed2000

Goal directed reaching and postural control in supine position in healthy infants.

The present study focussed on the development and interaction of reaching and posture in supine position in young infants. The kinematics of goal directed reaches and the concurrent ground reaction forces of the total body centre of pressure (COP) in cranial-caudal and medial-lateral direction were recorded in twelve healthy fullterm infants at 4 and 6 months of age. From eight infants, data were available at both ages. The results indicated that between 4 and 6 months, postural stability during reaching in supine position increased, as the oscillatory COP displacement in the medial-lateral direction decreased significantly with increasing age. Concurrently, reaching performance improved, which was reflected by a decrease in the number of movement units (MU), the length of the displacement-path of the hand and an increase of the length and duration of the first MU. The kinematic data of the reaching movements and the COP data were correlated at both ages, but the nature of the relation changed. At 4 months of age, the number of MU was related to the number of COP oscillations in the cranial-caudal direction, whereas at 6 months the maximum velocity of the hand movement was related to the maximum velocity of COP displacement in cranial-caudal and medial-lateral direction. This change might point to the development of a more subtle control of postural adjustments during reaching movements in supine position.

Aging↗