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Biomedical subjects

E Fernell

Publications and source records attributed to E Fernell.

35 records · Page 2Linked to original sources

Mild mental retardation in schoolchildren in a Swedish suburban municipality: prevalence and diagnostic aspects.

The prevalence of mild mental retardation (MMR) (IQ 50-72) was investigated in a Swedish suburban municipality with special demographic characteristics. The study population consisted of the 6397 children aged 9-15 years residing in the municipality on December 31, 1994. A total of 82 children fulfilled psychometric and adaptive criteria of MMR, corresponding to a prevalence of 12.8/1000. The prevalence was higher than that reported in previous Swedish and Finnish studies using similar case-finding methods. This can probably be explained by the lower socioeconomic status in the municipality studied and by the fact that testing is now more acceptable. Nearly 90% of the MMR children had symptoms in their preschool years, mainly motor, speech/language deviations and a relative inability to perform certain tasks. These symptoms also occur in children with minimal brain dysfunction/ deficits in attention, motor control and perception which should be taken into consideration when selecting screening tests.

Adolescent↗

Decrease in 6R-5,6,7,8-tetrahydrobiopterin content in cerebrospinal fluid of autistic patients.

The levels of pterin compounds in the lumbar cerebrospinal fluid (CSF) of ten neurologically normal and twenty autistic children were measured by two different liquid chromatographic systems, a conventional and a newly developed direct method. Among pterin compounds 7,8-dihydroneopterin (NH2) and 6R-5,6,7,8-tetrahydrobiopterin (R-BH4) levels in autistic children were significantly reduced to 66.1 and 41.5%, respectively, of those found in the controls. The autistic children were divided into three subgroups according to their CSF homovanillic acid (HVA) level and age: the group with an elevated HVA level, the younger (< 7 year old) group with a normal HVA level, and the older (> 7 year old) group with a normal HVA level. In comparison, the further reduction was observed in NH2 and R-BH4 levels in the younger group and in the 7,8-dihydropterin (PH2) level in the group with an elevated HVA level. Thus, these results suggest that the endogenous biosynthesis of R-BH4 in the brain may be reduced in autistic children as compared with that in neurologically normal children.

Adolescent↗

Infantile hydrocephalus epidemiology: an indicator of enhanced survival.

The epidemiology of infantile hydrocephalus in the birth years 1973-90 was investigated in west Sweden. The study revealed a significant increase in prevalence from 1973-8 and 1979-82 considered due to the enhanced survival of very and extremely preterm infants. The increase did not continue from 1983-90. This could indicate an improved outcome in preterm survivors as the neonatal survival rate continued to increase. The striking predominance of a perinatal/neonatal aetiology in very preterm hydrocephalic infants could be confirmed: 89% born from 1983-90 had suffered a confirmed postpartum intraventricular haemorrhage. In infants born at term, prenatal origins, mainly maldevelopments, dominated. The outcome in very preterm surviving infants with infantile hydrocephalus was poor: 73% had cerebral palsy, 52% epilepsy, 22% severe visual disability, and 55% were mentally retarded. Despite the increased survival resulting in a majority of healthy infants, there is an accumulating cohort of hydrocephalic children.

Blindness↗

Infantile hydrocephalus in preterm, low-birth-weight infants--a nationwide Swedish cohort study 1979-1988.

All Swedish infants with shunt-treated infantile hydrocephalus, born during the period 1979-88 at < or = 34 weeks gestational age and of low birth weight, were studied. Ninety-six infants were born before 32 weeks and 50 at 32-34 weeks. The mean gestational age in the very preterm group gradually decreased from 29.5 to 27.3 weeks. The mean live birth prevalence was 15.9 per 1000 very preterm infants, and 5.1 per 1000 moderately preterm infants. No significant secular prevalence trends were found. The perinatal mortality decreased successively. The slowly decreasing trend in moderately preterm infants may imply better outcome in survivors. The slightly increasing trend in very preterm infants could be explained by more survivors in the low gestational age group. The aetiology was considered perinatal in 94% of the very preterm group and in 56% of the moderately preterm group; prenatal in 1% and 32% of infants, respectively. Additional neuro-impairments were present in 82% of infants, cerebral palsy being the commonest (74%).

Cerebrospinal Fluid Shunts↗

Behavioural problems in children with infantile hydrocephalus.

The occurrence of behavioural problems in a population-based series of children with infantile hydrocephalus (non-spina bifida) was analysed, using parent questionnaires. Children with both infantile hydrocephalus and mental retardation had significantly more behavioural problems compared with those with no mental retardation and controls. Inattentiveness and hyperactivity were particularly typical. No differences were found between children with infantile hydrocephalus and no mental retardation and the control group.

Adolescent↗

Autistic symptoms in children with infantile hydrocephalus.

From a population-based series of children with Infantile Hydrocephalus (IH) 69 patients (mean age 11.7 years) were examined with respect to the occurrence of autistic symptoms. Autistic symptomatology was evaluated according to a modified short Swedish version of the so-called Autism Behavior Checklist. Sixteen of the 69 IH children (23%) reached a score which was considered indicative of autistic symptoms (AS) in the child. This group was compared with the remaining 53 IH children without autistic symptoms (non AS). Significant differences were found between these two groups with respect to aetiological and clinical data. In the AS group 44% were born preterm as compared to 9% in the non AS group. CT scan showed major abnormalities in 64% of the AS children while this was present in 28% in the non AS group. The occurrence of major neuroimpairments--epilepsy, mental retardation and cerebral palsy--was 50%, 88% and 50% in the AS group as compared to 9%, 23% and 19% respectively in the non AS group. It was concluded that the more severe the brain damage in children with IH the more likely that autistic symptomatology would ensue. This implies that specific neuropsychiatric services to these families are required.

Autistic Disorder↗

Functions and complications of shunts in different etiologies of childhood hydrocephalus.

Shunt function and complications in different etiologies of childhood hydrocephalus were studied in a series of 306 patients involving 1102 shunt operations. Shunts in patients with hydrocephalus caused by neoplasms proved to be most prone to shunt complications. The patency time for shunts in these patients was significantly shorter than for shunts in other patient categories [Standard number of deviations (SND) 5.9; P less than 0.001, Meyer-Kaplan life table analysis]. When the two main groups of infantile hydrocephalus-congenital obstructive hydrocephalus and hydrocephalus caused by perinatal intracerebral hemorrhage-were compared, the latter group proved to be significantly more prone to shunt infections (P less than 0.01), with an infection rate of 17.8% compared with 8.9% for the former group. The importance of this fact is stressed by the observation that these patients appear to constitute an increasing percentage of hydrocephalic patients. According to the present study, patients with congenital intracranial cysts and hydrocephalus are less prone to shunt complications, i.e., the infection rate is 6.8%, which is significantly less than that of patients with other types of hydrocephalus (P less than 0.01; chi-square test).

Brain Diseases↗

Infantile hydrocephalus--the impact of enhanced preterm survival.

The epidemiology of infantile hydrocephalus (IH) in the birth years 1983-86 was investigated in the south-western health care region of Sweden. The study was made as a continuation of a previous one which had shown a significant increase in the prevalence of IH in 1967-82. That rise was entirely referable to the relatively larger number of preterm IH infants born in 1979-82 and was considered to be due to the enhanced survival, especially of very preterm infants. The present series comprised 57 liveborn IH infants--27 preterms and 30 born at term. The livebirth prevalence of IH was 0.64 per 1,000, 0.30 for preterm and 0.34 for fullterm infants. The high prevalence of preterm IH infants in 1979-82 had persisted, but had not increased further. This might indicate an improved outcome in preterm survivors in the period 1983-86, as the survival rate had continued to increase. The striking predominance of a perinatal aetiology of IH in very preterm infants could be confirmed: 90% had had intraventricular haemorrhages verified by ultrasound in the postpartum period. The outcome in very preterm surviving infants with IH was still alarmingly poor: 78% had cerebral palsy, 72% mental deficiency, and 56% epilepsy.

Cerebral Hemorrhage↗

Epidemiology of infantile hydrocephalus in Sweden. Reduced optimality in prepartum, partum and postpartum conditions. A case-control study.

The optimality concept developed by Prechtl was adopted to investigate a population-based series of infantile hydrocephalus (IH). The results were compared with those from a control series of newborns. The case series comprised 128 IH children born at term and 50 born preterm, and the control series 269 and 176, respectively. Cases with a prenatal cause of IH, as compared with those with a perinatal cause and controls, had significantly increased risk of IH by reduced optimality in the prepartum period. Peaks in the flow of non-optimal items in the prenatal group were repeated abortions or perinatal death in previous pregnancies, maternal disorder and twin birth. The profile of reduced optimality in term IH cases of undefined cause was similar to that of term cases with a prenatal cause. All IH cases had significantly increased reduced optimality in the postpartum period compared with controls. The increase was massive in cases where IH was of perinatal cause, with peaks in items of acidosis, apnea, respiratory treatment, infection and cerebral irritation. Reduced optimality in partum conditions did not discriminate between IH of pre- and perinatal cause. Reduced optimality in the prepartum, partum and postpartum periods in IH children, as compared with those with cerebral palsy syndromes, was nearly identical to that of hemiplegic, and significantly lower than that of diplegic and dyskinetic, cerebral palsy.

Female↗

Epidemiology of infantile hydrocephalus in Sweden: a clinical follow-up study in children born at term.

The long-term outcome of infantile hydrocephalus (IH) in children born at term during a period of active shunt treatment was studied in a population-based survey. The series consisted of 68 children greater than or equal to 6 years old and born in 1967-78 in the south-western Swedish health care region. The clinical follow-up included neuro-paediatric assessment, Stott's test of motor impairment, the WISC test, CT and EEG analyses. Nineteen of the 68 children (28%) had cerebral palsy, 17 (25%) minor motor dysfunction and 32 (47%) no motor dysfunction; mental retardation was present in 26 (38%), 16 with an IQ 50-70 and 10 with IQ less than 50; 42 children (62%) had normal intelligence and epilepsy was found in 15 (22%). Compared with a non-shunted IH series from the 1950s, the survival of IH children had considerably increased. Of constituents characterizing the IH syndrome from the time prior to shunting, ataxia, divergent squint and the special "Cocktail-party behaviour" had significantly decreased, all of which conditions are highly related to chronic expansion of the ventricular system. The frequencies of other impairments such as mental retardation and epilepsy were fairly similar, reflecting the present increased survival of IH children with primarily non-IH-dependent brain damage. IH children with associated brain parenchymal defects had the poorest outcome, and those without had in general a much more favourable one. Thus the single most important factor for the outcome of IH was found to be the presence or absence of associated primary brain damage or maldevelopment.

Cerebral Palsy↗

Epidemiology of infantile hydrocephalus in Sweden. Current aspects of the outcome in preterm infants.

The outcome in a population-based series of 61 Swedish preterm infants born in 1967-82 with infantile hydrocephalus (IH) was investigated. Sixteen (26%) died before the age of two years. The available information was updated when the 45 surviving children were at least four years and six months old. A structured follow-up examination was performed in the 13 children who had passed the age of six years. Among the 45 survivors, 47% had cerebral palsy, 51% mental retardation and 33% epilepsy. The overall outcome for preterm infants with IH was found to be poorer than that for fullterm ones. Prognostic factors correlating to a poor outcome were an obvious origin of IH (pre- or perinatal) and a gestational age of less than 28 weeks. It is concluded that handicapped IH children born very or extremely prematurely constitute a new, and to a large extent severely brain-damaged group that has entered the Swedish IH panorama since the end of the 1970s.

Female↗

Overt hydrocephalus at birth--origin and outcome.

Based on a survey of the population, which included 202 infants with infantile hydrocephalus those who had had overt hydrocephalus at birth were selected and analyzed with respect to origin and outcome. The present series consisted of 47 infants (23% of the total series), 83% of whom were born at term and 17% before term. In the study period 1967-1982, the prevalence was 0.12/1,000 births. Among infants with maldevelopment of the CNS and/or other organs, the mortality before 2 years of age was 37%, and among the survivors the rate of neurological sequelae was 88%. The corresponding figures for infants with uncomplicated hydrocephalus, i.e., without other maldevelopment, was 20% and 44%, respectively. The prognosis was especially poor for infants born before term. It was concluded that the prognosis was largely determined by maldevelopment affecting the CNS macro- or microarchitecture.

Central Nervous System↗

Epidemiology of infantile hydrocephalus in Sweden. II. Origin in infants born at term.

The aetiology of infantile hydrocephalus (IH) was studied in a population-based series of 141 children with IH, born at term in southwestern Sweden 1967-82. A prenatal aetiology was present in 81 children (57%), a pre- and perinatal in 6 (4%), a perinatal in 27 (19%), and a postnatal in 8 (6%); the origin in 19 children (13%) remained untraceable. A variety of aetiologies were revealed or indicated among prenatal conditions. The dominant intrauterine infection was toxoplasmosis. The predominant perinatal condition was posthaemorrhagic IH. The broad outline of outcome differed between pathogenetic groups. Children with a clear prenatal onset of IH were found to be at high risk for early death or multiple neurological impairments. Thirteen of 63 (21%) within this group had died before 2 years of age and 34 of the 50 (68%) survivors showed major neurological dysfunction. This contrasted to the incidences of 3% deaths and 30% sequelae in children with IH of other onset.

Adult↗

Epidemiology of infantile hydrocephalus in Sweden. III. Origin in preterm infants.

The aetiology of infantile hydrocephalus (IH) was studied in a population-based series of 61 children with IH born 1967-82 at less than 37 weeks of gestation. A prenatal origin was present in 17 children (28%), a pre- and perinatal in 17 (28%), a perinatal in 26 (43%) and a postnatal in one (1%). The predominant single cause was postaemorrhagic IH, which was diagnosed in 19 (31%). In addition, an undiagnosed cerebral haemorrhage was considered to be the cause in another 25%. The outcome differed between pathogenetic groups. Children with a clear onset of IH (pre-, peri- or postnatal) were found to be at high risk for early death or multiple impairments. Sixteen of 39 (41%) within these groups had died before 2 years of age and 18 of the 23 (78%) survivors showed major neurological dysfunction. This contrasted to no mortality and 41% major dysfunction in children with a less clear onset of IH. A new subgroup consisting of infants born before 28 weeks of gestation emerged in the early 1980s. All infants with IH in this group developed a severe multihandicap condition.

Child, Preschool↗

Epidemiology of infantile hydrocephalus in Sweden. I. Birth prevalence and general data.

The livebirth prevalence of infantile simple hydrocephalus (IH) was investigated in a Swedish population-based survey. The study comprised all liveborn infants with IH apparent before the age of one year and born in the study area between 1967-82. A total of 202 infants fulfilled these criteria; of these, 141 (70%) were born at term and 61 (30%) were preterm. The mean prevalence was 0.53 per 1,000, with a slightly increasing trend from 0.48 in 1967-70 to 0.63 in 1979-82. The increase was entirely referable to the preterm group, in which the prevalence rose from 0.13 per 1,000 in 1967-70 to 0.30 in 1979-82. There was no tendency to an increase in the term group. In 12 of 23 children born very preterm the origin of the IH was a diagnosed cerebral haemorrhage. This is compatible with the increased risk of the latter condition that has been found in very preterm newborns. The mortality among children with IH was highest before the age of one year (15%), after which it was 1.2% per year.

Female↗

Ventriculoatrial or ventriculoperitoneal shunts in the treatment of hydrocephalus in children?

The data on all 881 primary or revision shunt operations performed on 158 paediatric patients treated in Gothenburg, Sweden from 1967 to 1984 and 101 patients treated in Oulu, Finland from 1968 to 1983 were pooled for the purpose of comparative evaluation of the function of ventriculoatrial (VA) and ventriculoperitoneal (VP) shunts. Ventriculoperitoneal shunting was the method of choice in Gothenburg and ventriculoatrial shunting in Oulu. The results of the 723 operations (305 VA and 418 VP shunts) were evaluated as the other 158 operations were for ventriculostomas, shunt removals and other procedures. 80 children had exclusively VA shunts and 133 children had exclusively VP shunts. Irrespective of the method of analysis the VP shunts were more frequently infected. The estimated relative risk for obstruction of the shunt (Meyer-Kaplan method) was shown to be significantly higher in VA shunts, but only at a low level of statistical significance (p less than 0.1). All other shunt complications were distributed uniformly in both groups. There was, however, a trend towards a higher mortality among children with exclusively VA shunts. Therefore it was concluded that despite the higher risk for infection in VP shunts, these still should be considered a safer choice, as the complications of VA shunts present greater risks.

Adolescent↗