[Febrile convulsions, Treatment and prognosis]. / Feberkramper. Behandling og prognose.

Epidemiological data indicate that the long-term prognosis in the vast majority of children with febrile seizures is good. Three main problems are important for the treatment: febrile seizures are extremely upsetting for the parents, the recurrence rate is 30-40% and the febrile status occurs unpredictably and is potentially damaging to the CNS. There is universal agreement that daily prophylaxis with valproate, primidone, or phenobarbital should only be used in highly selected cases, if at all. The effectiveness of phenytoin and carbamazepine has not been documented. Antipyretic treatment does not reduce the recurrence rate. Intermittent diazepam prophylaxis at times of fever may or may not reduce the recurrence rate significantly, but there is no data to suggest that it improves the long-term outcome, as compared with short-term seizure control, in terms of IQ, cognition, academic progress, motor control, and subsequent epilepsy. Acute anticonvulsive treatment with rectal diazepam in solution or other benzodiazepines is effective in aborting recurrent seizures with almost the effectiveness of i.v. treatment and is safe, simple, and easy to use for the parents. The long-term prognosis is probably uninfluenced by the type of treatment given in early childhood. It has not been established that acute anticonvulsive treatment with benzodiazepine is better than placebo.

[Child abuse and osteogenesis imperfecta. How do we distinguish?]. / Børnemishandling og osteogenesis imperfecta. Hvordan ser vi forskel?

Osteogenesis imperfecta is a hereditary connective tissue disorder. Typical manifestations are fragile bones with multiple bone fractures and bone deformities. A history of minimal or no trauma and recurrent fractures is a feature of OI, but is also typical of non-accidental injury (NAI). OI and NAI are relevant differential diagnoses when a child presents with unexplained fractures. The differential diagnostic problems are reviewed, all of which are important for the child both in terms of treatment and for prognosis, socially and medicolegally. We conclude that comprehensive clinical evaluation is adequate for differential diagnosis and that both OI and NAI can be diagnosed by positive anamnestic and objective signs. Mild OI IV without other signs than fracture(s) is very rare and the new entity temporary brittle bone disease is hypothetical; the diagnosis of these two clinical pictures is unacceptable in small children. Routine analysis of collagens should not be performed.

Febrile seizures: treatment and prognosis.

Recent epidemiologic data indicate that the vast majority of children with febrile seizures have a normal longterm outcome. A precise knowledge of the short- and long-term outcome with or without treatment, and short- and long-term side effects is an important prerequisite for assessing the various treatment strategies. We focus on the impact of short-term or prophylactic treatment on the short- and long-term outcome of various types of febrile seizures. There is universal agreement that daily prophylaxis with antiepileptic agents should never be used routinely in simple febrile seizures, but only in highly selected cases, if at all. Intermittent diazepam (DZP) prophylaxis at times of fever may or may not reduce the recurrence rate, but it does not appear to improve the long-term outcome as compared with short-term seizure control. The treatment may be used to reduce the recurrence rate for a small arbitrarily defined group with multiple simple febrile seizures, complex febrile seizures, especially focal, prolonged or both, febrile status, and when parental anxiety is severe. However, there is no evidence that treatment of simple febrile seizures can prevent the rare cases of later epilepsy, and many children with complex febrile seizures have a benign long-term outcome, even without treatment. Many prefer a "wait and see" policy. An attractive alternative is to treat new febrile seizures with rectal DZP in solution at seizure onset, given by the parents at home to prevent febrile status. Newer, less well documented short-term strategies include nasal, oral, or rectal administration of other benzodiazepines. Short-term seizure control of febrile status and careful parental counseling are the two most important targets of treatment.

Epidemiologic, bacteriologic, and long-term follow-up data of children with acute hematogenous osteomyelitis and septic arthritis: a ten-year review.

A total of 69 children with acute hematogenous osteomyelitis and 48 with septic arthritis admitted in the period 1978 through 1987 were included in a retrospective review. Epidemiologic and bacteriologic data were analyzed and compared with those of an earlier study (1965 through 1975), confined to the same geographic area. Long-term outcome was evaluated by a questionnaire and clinical and radiographic follow-up. A significant increase in the admission rate for both disorders was observed. The long-term outcome was favorable: major sequelae were found in three patients (3%), minor sequelae in two patients (2%). The benign long-term outcome may well be related to rapid hospital admission and appropriate long-lasting antibiotic treatment.

[Clinical value of SPECT scanning in children with neurological disorders]. / Den kliniske vaerdi af SPECT-scanning hos børn med neurologiske sygdomme.

We retrospectively evaluated the clinical value of Tc-99-HMPAO brain single photon emission tomography (SPECT) in 21 children with neurological disorders of varied aetiology. All 21 patients were examined with electroencephalograms (EEGs), 17 with computed tomography (CT) and ten with magnetic resonance imaging (MR). New relevant information was obtained from SPECT in all 21 cases and in four of these cases the investigation directly led to a change in diagnosis and better assessment of the prognosis. It is concluded that SPECT is an important investigational method in children with neurological symptoms of partly or completely unknown aetiology.

[Shaken baby syndrome]. / Shaken baby syndrome--ruskede børn.

Shaken baby syndrome is a serious form of physical child abuse, which is frequently overlooked. It should be suspected in all children younger than one year of age, who present with drowsiness, coma, seizures or apnoea. A combination of subdural haematomas and retinal haemorrhages with minimal or no trauma and no coagulopathy is almost pathognomonic of the syndrome. The findings are caused by shaking with or without impact. Physical signs of violence are often absent and the syndrome may easily be mistaken for serious infection or seizure disorder. Many cases are fatal or lead to severe disability including blindness, cerebral palsy, mental retardation or epilepsy in about 60% of the children. There are many unresolved problems regarding diagnosis, pathophysiology, treatment, prognosis, prophylaxis and legal actions. We discuss these problems and in addition present eleven children with shaken baby syndrome.

[Incidence of child neglect and child abuse in the region of Copenhagen]. / Forekomst af omsorgssvigt mod børn oq børnemishandling i Københavns Amt.

The aim of the study was to evaluate the incidence of physical violence, neglect and sexual abuse against children as reported to the local authorities, in the county of Copenhagen during the year 1993. A questionnaire was mailed to the local authorities in the 18 districts in the county and to 18 schools and 18 general practitioners (GP's). They were asked whether, and how many new, verified and suspected cases of physical, emotional and sexual abuse had come to their attention in 1993. A total of 300 cases of child abuse were identified in the area with a total population of 675.000 i.e. a total incidence of 2.7 cases per 1000 children aged 0-17 years (2.7%), with 0.7% for physical violence, 0.5% for sexual abuse and 1.5% for emotional neglect. Some 180 cases were considered verified and 120 cases were suspected. A considerable variation between the different districts was noted, i.e. the local incidence varied from 0-5.4%. A statistically significant inverse correlation between the average local tax-income and the incidence of child abuse and neglect was found. There were several examples of schools having knowledge of more cases than the local authorities. Very few cases came to be attention of the GP.

[Long-term prognosis in febrile convulsions with and without prophylaxis]. / Feberkrampers langtidsprognose med og uden profylakse.

This is a long-term follow-up of occurrence of epilepsy, neurological, motor, intellectual, cognitive, and scholastic achievements in a cohort of children with febrile convulsions (n = 289), randomized in early childhood to either intermittent prophylaxis (diazepam at fever) or no prophylaxis (diazepam at seizures). At follow-up the two groups were of almost identical age (14.0 vs. 14.1 years), body weight (58.2 vs. 57.2 kg), height (168.2 vs. 167.7 cm) and head circumference (55.9 vs. 56.2 cm). The neurological examination, fine and gross motor development on Stott motor test, intellectual performance on the Wechsler Intelligence Scale for Children verbal IQ (105 vs. 105), performance IQ (114 vs. 111) and full scale IQ (110 vs. 108). cognitive abilities on an neuropsychological test battery, including short and long term, auditory and visual memory, visuomotor tempo, computer reaction time, reading test, scholastic achievements and the occurrence of subsequent epilepsy were also very similar. Children with simple and complex febrile convulsions had the same benign outcome. The long term prognosis in terms of subsequent epilepsy, neurological, motor, intellectual, cognitive, and scholastic ability was not influenced by the type of treatment applied in early childhood. Preventing new febrile convulsions appears no better in the long run than abbreviating them.

Febrile seizures--treatment and outcome.

Assessment of treatment strategies in febrile seizures should be based on short- and long-term outcomes, with and without acute, intermittent, or chronic medical intervention, as well as short- and long-term side effects. Febrile seizures are a benign condition with a normal neurological, motor, intellectual, and cognitive long-term outcome and have a low risk of later epilepsy in most cases. Even many complex febrile seizures have a benign outcome. Prophylaxis may or may not reduce the recurrence rate, but does not appear to improve the long-term outcome as compared to acute treatment of seizures in progress. All agree that chronic prophylaxis with anti-epileptic agents is justified only in highly selected cases, if at all. Treatment with benzodiazepines during febrile episodes appears to effectively reduce the recurrence rate, provided adequate doses are given and compliance problems minimized. A selective approach to intermittent diazepam prophylaxis seems rational, as the recurrence risk and response to treatment are highly variable. An attractive alternative is acute treatment at seizure onset with rectal diazepam in solution given by the parents at home in order to prevent prolonged recurrent seizures. This regimen has the potential of moving the first line of anti-convulsant defence close to the child. It appears to be effective, inexpensive, feasible even for non-professionals, has few side effects and is well accepted by the parents. A reasonable policy would be to treat simple febrile seizures solely with acute rectal diazepam in solution and reserve intermittent diazepam prophylaxis for selected cases including those with multiple or prolonged recurrences, several risk factors for recurrent febrile seizures and other special situations.

[Low dose ACTH in infantile spasms. Effects and adverse effects]. / Lavdosis-ACTH ved infantile spasmer. Effekt og bivirkninger.

A total of 20 infants with cryptogenic (n = 7) or symptomatic (n = 13) infantile spasms (West syndrome) who received low-dose ACTH treatment 0.25 mg x 2 i.m. (25 units x 2) per week for four to eight weeks were evaluated in an uncontrolled retrospective study for short- and long-term efficacy and side-effects of the treatment. In ten infants (50%) cessation of the seizures was seen, and six improved, i.e. a response rate of 84%. Side-effects in the form of infections were seen in four patients. Two developed pneumonia, one fever of unknown origin and one a purulent meningitis 12 hours after start of treatment, but a cause and effect relationship is questionable.

Long term outcome of prophylaxis for febrile convulsions.

A cohort of 289 children with febrile convulsions who had been randomised in early childhood to either intermittent prophylaxis (diazepam at fever) or no prophylaxis (diazepam at seizures) was followed up 12 years later. The study focused on the occurrence of epilepsy and on neurological, motor, intellectual, cognitive, and scholastic achievements in the cohort. At follow up the two groups were of almost identical age (14.0 v 14.1 years), body weight (58.2 v 57.2 kg), height (168.2 v 167.7 cm), and head circumference (55.9 v 56.2 cm). The occurrence of epilepsy (0.7% v 0.8%), neurological examination, fine and gross motor development on the Stott motor test, intellectual performance on the Wechsler intelligence scale for children verbal IQ (105 v 105), performance IQ (114 v 111), and full scale IQ (110 v 108), cognitive abilities on a neuropsychological test battery, including short and long term, auditory and visual memory, visuomotor tempo, computer reaction time, reading test, and scholastic achievement were also very similar. Children with simple and complex febrile convulsions had the same benign outcome. The long term prognosis in terms of subsequent epilepsy, neurological, motor, intellectual, cognitive, and scholastic ability was not influenced by the type of treatment applied in early childhood. Preventing new febrile convulsions appears no better in the long run than abbreviating them.

Risk factors for seizure recurrence in children with febrile seizures: a pooled analysis of individual patient data from five studies.

To reassess the relations between postulated risk factors and seizure recurrence after a first febrile seizure (FS), the individual data from five follow-up studies that used similar definitions of FSs and risk factors were pooled and reanalyzed. The risk of frequent recurrent seizures and of the occurrence of complex seizures in previously healthy, untreated children was studied. Seizure recurrence hazard was described as a function of the child's attained age. The influence of various risk factors on the recurrence hazard was assessed, with control for other factors. Of a total of 2496 children with 1410 episodes of recurrent seizures, 32% had one, 15% had two, and 7% had three or more recurrent seizures after a first FS; 7% had a complex seizure. The hazard of recurrent seizures was highest between the ages of 12 and 24 months. After a first and a second recurrence, the risk of further FSs was two and two and one-half times higher, respectively. A history of febrile or unprovoked seizures in a first-degree family member and a relatively low temperature at the time of the first seizure were also associated with an increased risk of subsequent recurrences. Young age at onset (< 12 months), a family history of unprovoked seizures, and a partial initial FS were all associated with an increased risk of complex seizures. A higher recurrence rate in clinic-based studies compared with population-based studies could not be explained by a difference in the presence of the risk factors studied. Thus other factors must influence seizure recurrence after an initial FS.

Acute osteomyelitis and septic arthritis in the neonate, risk factors and outcome.

Twenty-two neonates with acute osteomyelitis (AO) or septic arthritis (SA) were included in a study based on a review of medical reports and a long-term clinical and radiological follow up. Clinical symptoms, bacteriology, risk factors, and outcome are discussed. The diagnoses were difficult, the clinical symptoms vague, fever rare and white cell count normal. Detection by plain radiological films was more efficient than by radionuclide bone scan. Staphylococcus aureus was the predominant causative organism and a shift towards group B Streptococcus in recent years was not identified. Risk factors for AO and SA were prematurity (13/22), respiratory distress syndrome (15/22) and perhaps most important: umbilical artery catheterisation (15/22). Severe sequelae were found in only 1 patient, while 3 patients had slight asymptomatic changes. The relatively favourable long-term outcome is unexplained, but may be related to early and appropriate, long lasting antibiotic treatment.

Intermittent diazepam prophylaxis in febrile convulsions. Pros and cons.

Major cohort studies document that the long-term prognosis for most children with febrile convulsions (FC) is excellent. The 2 main treatment alternatives so far have been long-term prophylaxis with phenobarbital or valproate or no prophylaxis at all. Phenobarbital at times of fever is ineffective and obsolete. Consensus has emerged that long-term prophylaxis with antiepileptic drugs is rarely justified in FC considering the side effects and the favourable prognosis. No treatment at all does not appear quite satisfactory either, as FC have a high recurrence rate, disrupt family life and may have emotional consequences for the family. Moreover, all FC children face a risk, although admittedly low, of subsequent long-lasting potentially central nervous system (CNS)-damaging seizures. However, 2 further options exist: treatment with rapid-acting benzodiazepines solely at times of greatest risk, i.e., at high fever or at renewed seizures. Several clinical trials have confirmed that intermittent diazepam prophylaxis by way of a few doses of the drug per year provides effective seizure control and reduces the recurrence rate by one half or two thirds. The treatment is feasible and cheap, well tolerated by the child and well accepted by the parents. Compliance problems are common and only partly abatable. Trivial side effects are frequent. Transient respiratory apnoea does occur, but 15 years' experience substantiates that serious side effects are remarkably rare. Acute anticonvulsant treatment with rectal diazepam in solution given by the parents to stop ongoing seizures and to prevent immediate recurrences is an attractive alternative. It is feasible, is probably effective and minimizes the use of drugs, but compliance problems are common and protracted seizures are not always controlled. The subsequent management should include a risk profile approach considering a combination of risk factors for new FC rather than a single factor. By means of a risk index, based on simple clinical data including age at onset, family seizure history, seizure type and frequency of fever, children may be identified as being at low, intermediate or high risk for further febrile fits. However, risk factors for new FC and not for subsequent epilepsy should be used. It is concluded that preventing or abbreviating new FC with benzodiazepines appears to be a useful, although not ideal, drug-minimizing approach in managing many children with simple or complex FC. From a health hazard viewpoint, treatment is not strictly mandatory, although advisable. A selective strategy seems rational. Intermittent diazepam prophylaxis may preferably be offered to children at high risk for new FC.(ABSTRACT TRUNCATED AT 400 WORDS)

Intermittent prophylaxis in febrile convulsions: diazepam or valproic acid?

In an open, prospective, randomized, and hospital-based study, comprising 219 consecutive children, 169 were given intermittent prophylaxis for one year, receiving either diazepam or valproic acid after their first febrile convulsion. Children admitted on odd dates (n = 89) were given rectal diazepam in solution every 12 h, whenever the temperature was 38.5 degrees C or more. Children admitted on even dates (n = 80) were given valproic acid as suppositories at times of fever. Twenty-three children in the diazepam group had a recurrence within 1 year versus 14 in the valproic acid group. On an intention-to-treat basis the 12-month recurrence rates in the 2 groups were similar, 27% vs 20%. The latter is well below figures for untreated controls from Denmark (32%), suggesting that intermittent valproic acid at times of fever may be effective, but further studies are needed. The number of complex recurrences, however, were significantly higher in the valproic acid group than in the diazepam group. Parental non-compliance was a major problem, and in the 2 study groups only 5 and 12 children, respectively, with recurrences were treated adequately. Sixty-nine children receiving diazepam had side-effects vs 37 receiving valproic acid. None were serious.

Total body bone mineral in light-for-gestational-age infants and appropriate-for-gestational-age infants.

Dual-photon-absorptiometry using 153Gd in a whole body scanner was used to measure total body bone mineral in 51 newborn infants. In preterm light-for-gestational-age infants total body bone mineral was 12.6 g v.s. 25.6 g in preterm appropriate-for-gestational-age infants (p less than 0.05). In term light-for-gestational-age infants total body bone mineral was 41.4 g v. 84.2 g in term appropriate-for-gestational-age infants (p less than 0.001). The correlations between gestational age and total body bone mineral was best described by exponential regression lines.