[Advocacy for pediatric care]. / Politische Kindermedizin: Für eine bessere Zukunft aller Kinder.

Advocacy for pediatric care ("Politische Kindermedizin") is an Austrian association of engaged pediatricians, aiming at identifying, analyzing and evaluating pressing issues of pediatric care and to suggest improvements to the responsible politicians. The aim was and is to achieve an improvement of pediatric care for children and adolescents in the sense of patient advocacy. The history and concerns of the initiative "Politische Kindermedizin" from 1997 until the present are presented.

Alterations in nocturnal serum melatonin levels in humans with growth and aging.

The available data on potential alterations in serum melatonin (MLT) levels during a human lifetime are fragmentary and inconsistent. We, therefore, measured day- and nighttime serum MLT concentrations in 367 subjects (210 males and 157 females), aged 3 days to 90 yr. Blood samples were collected between 0730 and 1000 h and between 2300 and 0100 h. Serum MLT levels were measured by RIA. The mean nighttime serum MLT concentration was low during the first 6 months of life, i.e. 27.3 +/- 5.4 (+/- SE) pg/mL (0.12 +/- 0.02 nmol/L). It then increased to a peak value at 1-3 yr of age [329.5 +/- 42.0 pg/mL; (1.43 +/- 0.18 nmol/L)], and it was considerably lower [62.5 +/- 9.0 pg/mL; (0.27 +/- 0.04 nmol/L)] in individuals aged 15-20 yr. During the following decades serum MLT declined moderately until old age (70-90 yr of age), i.e. 29.2 +/- 6.1 pg/mL (0.13 +/- 0.03 nmol/L). This biphasic MLT decline follows 2 exponential functions with different slopes (from age 1-20 yr: r = -0.56; P less than 0.001; y = 278.7 X e -0.09x; from age 20-90 yr: r = -0.44; P less than 0.001; y = 84.8 X e -0.017x). The decrease in nocturnal serum MLT in children and adolescents (1-20 yr) correlated with the increase in body weight (r = -0.54; P less than 0.001) and body surface area (r = -0.71; P less than 0.001). At a later age (20-90 yr) there was no correlation among these variables. Daytime serum MLT levels were low and no age-related alterations were found. This study revealed major age-related alterations in nocturnal serum MLT levels. The negative correlation between serum MLT and body weight in childhood and adolescence is evidence that expansion of body size is responsible for the huge MLT decrease during that period. The moderate decline at older ages must derive from other factors.

Psychogenic relapses in childhood epilepsy in puberty and adolescence.

Epileptic patients who, after years of being free from symptoms, have relapses during puberty or adolescence (some-times coinciding with a reduction in therapy) pose special therapeutic and diagnostic problems. Because of pubertal lability, the cause of a relapse might seem to be "organic", especially if the EEG also shows a "deterioration", yet psychogenic factors must not be disregarded. On the basis of typical case studies, a characteristic constellation is presented. The achievement of a "well-behaved" child at first dramatically improves and this correlates with the success of antiepileptic therapy. Then individuation and further development cannot adequately take place because the family unit is not functioning properly. At a critical stage of development, the excessive expectations of the parents lead to too much stress on the child, thus destroying the balance within the family system, which has hitherto been maintained only with difficulty. Attempts to improve the situation by changing the medication (increasing the dose or switching to another drug) fail. If, however, it is realized that the symptoms are of psychogenic origin, adequate therapeutic interventions (e.g. adequate schooling or professional training as well as psychotherapy) promise good results.

Carbamazepine and benzodiazepines in combination--a possibility to improve the efficacy of treatment of patients with 'intractable' infantile spasms?

Therapeutical efforts in epilepsies with infantile spasms (IS) often show unsatisfying results, especially if neurological impairments are found. In a clearly negatively selected group of 24 children with IS and 10 patients with symptomatic myoclonic-astatic epilepsies--pretreated without success with ACTH and/or benzodiazepines (BDZ) alone or combined with other anticonvulsants--we tried a two-drug therapy of BDZ with carbamazepine (CBZ). Dosage of both drugs was within the usual range. In a follow-up period of 1-5 years, 8 of the IS patients and 4 of those with myoclonic-astatic seizures became seizure-free; furthermore, 6 children showed a marked reduction in their seizure frequency: 3 more than 80%, 3 more than 50%. Besides the fact that the patients did not develop a so-called escape-phenomenon--as often seen in therapy with benzodiazepines--they also showed fewer and less intensive side-effects. Without optioning for antiepileptic polytherapy in general, we conclude that in cases of "intractable" IS the combination of BDZ with CBZ might be more successful than the single drug. To confirm these preliminary findings further controlled studies have to be carried out.

Discrimination of taste and preference for sweet in premature babies.

A modified method for registering non-nutritive sucking behaviour with and without taste stimulation enabled us to study taste perception of premature babies. We wanted to study how babies who had no extrauterine taste experience reacted to one or more stimuli with water and 33% glucose. Eight randomly selected healthy newborns (three of them being SGA without further symptoms), exclusively fed by gastric tube, were studied. The mean gestational age at the time of the study was 35.5 weeks. They were studied five times with water and five times with glucose just before feeding. In seven babies the sucking response to glucose was greater than that to water from the first trial onwards. Sucking response increased with repeated glucose stimulation, but remained the same with water stimulation. The eighth baby behaved completely differently. We concluded from our results that premature babies with a postconceptional age of 35 weeks can discriminate between sweet and not-sweet. The greater sucking response to glucose than to water at the first trial implies a genetic factor in the preference for sweet in humans.

[Determination of antiepileptic drug levels in the blood: day-to-day experience in an out-patient clinic for epilepic children (author's transl)]. / Blutspiegelbestimmungen von Antiepileptika--Erfahrungen aus der Praxis einer Anfallsambulanz.

The possibility of determining the blood levels of antiepileptic drugs represents a considerable advance, under certain circumstances, objective supervision of anticonvulsant therapy. In particular in the case of an unsatisfactory therapeutic result, this procedure helps to reveal the causes of failure (e.g. incorrect administration, poor absorption, interaction with other drugs). Like many new diagnostic methods this method is rather costly; hence, its use within the daily routine work should be limited to definite indications. Apart from its use in cases of inadequate control of epilepsy and the appearance of side effects, other indications for implementation of the method are the establishement of correct dosation of antiepileptic drugs in new patients and drug intercurrent illness, which frequently necessitates the use of additional drugs.

[Psychosocial dwarfism--a rare form of growth disorder]. / Psychosozialer Minderwuchs--eine seltene Form der Wachstumsstörung.

Psychosocial dwarfism is a syndrome caused by emotional deprivation (maternal deprivation), characterized by symptoms of delayed motor and intellectual development, abnormal eating and drinking habits, enuresis and encopresis, aggressiveness and a pathological family structure. Diagnosis of psychosocial dwarfism is easy if the case history is carefully elicited and the growth hormone level is determined within the first few days following change in environment (e.g. hospitalization). Difficulty in reaching the correct diagnosis or misdiagnosis can occur if the symptomatology is not studied in its entirety. Diagnosis at the earliest possible stage is very important for the further development of the child, since behavioural disturbances and growth retardation are reversible with environmental change. This is demonstrated by the presentation of the case history of a 6 year-old boy--the third case reported in the German literature.

[Discrimination between epileptic and non-epileptic seizures using defined prolactin studies]. / Diskriminierung epileptischer und nichtepileptischer Anfälle mittels genormter Prolaktinuntersuchungen.

Prolactin blood levels (HPRL) increase within 20 minutes postictally after generalized epileptic, especially generalized tonic-clonic seizures and return to normal values within one hour. Elevated HPRL levels were also observed after complex partial seizures, but usually in less extent, exceeding normal ranges only slightly. Therefore baselin HPRL measurements are necessary for estimation of spontaneous fluctuations in comparison to changes after seizures. Unchanged PRL levels after attacks do not support their epileptic origin. Rage attacke showed no clear pattern of PRL changes.

[Function and structure of families with an epileptic child]. / Funktion und Struktur von Familien mit einem epilepsiekranken Kind.

Although the impact of psycho-social factors on the individual patient with epilepsy has been widely investigated, the influence of the illness on the family as a whole is still underestimated. By means of the Family Assessment Measure (FAM III), a well-validated instrument, we investigated which measurable influence the epilepsy of one child had on the functioning of the families. Data from a group of 72 families with a child suffering from epilepsy (EG), but without any other handicap were analysed and compared with those of 75 families with a child with severe mental retardation (SMG) and 76 control families (CG). Data were gathered through home visits. Only complete families were studied. Results showed that the EG was similar to the SMG in all 3 scales of FAM III, but differed significantly from the CG. In 26% of the families in EG and in 19% of the SMG clear signs of family malfunctioning were found, as compared with 6.5% of the CG. Family disfunctioning in EG was of a greater variety that in SMG. We found that within the EG the type of seizures (41 patients with generalized tonic clonic fits, 10 with complex partial seizures and 21 with absences) did not have any impact on the results. In addition, neither the duration of the illness nor the absolute length of seizure-free periods seemed to matter. Only families who had the subjective feeling that their children were still suffering from epilepsy showed significantly higher rates of family malfunctioning. Summarizing, we found that epilepsy in a child can have a severe impact, not only on his individual life and on the mother-child relationship, but on the functioning of his family as the whole. This fact should be taken into account in the treatment of these children and should influence family counselling, as well.

[Work with psychiatrically ill parents in a pediatric psychiatry treatment facility]. / Arbeit mit psychisch gestörten Eltern in einer kinderpsychiatrischen Einrichtung.

Since the new law for hospitalization has come into effect (1991) in Austria more than before children from parents who have psychoses or other serious mental disorders have to be taken into in-patient wards in psychiatric institutions for children-often according to a court decision. This article discusses the cases of 21 such children and their 16 (respective) families. The various difficulties in dealing with the mentally ill mothers and fathers as well as some first experiences with possible strategies for working with these parents, which were gathered in therapeutic practice, are described individually. Work with these parents means trying to establish reality or risking losing reality.

Is the therapy with carbamazepine more effective when measuring blood levels?

41 patients receiving Carbamazepine under regular measurements of blood levels were compared with 77 patients with no or irregular controls of the blood level. 71% of the patients in the first group had a good seizure control in contrast to 61% of the patients in the second group. Furthermore, patients with regular controls reached the desired therapeutical effect earlier (on an average within 21/2 months) compared with patients with no or irregular controls (the average was 10 months). Patients who did nor react to medication with CBZ were put on another drug within a shorter time if their blood levels were measured (on the average 10 months compared with 20 months). We conclude that regular blood level controls make handling of CBZ easier. This allowed us to extend the range of indications for CBZ to a greater number to epilepsy with grand mal and focal seizures as well as to few therapy-resistant cases of epilepsy of early childhood with tonic seizures.

[Threatened occupational situation of parents as a pathogenetic factor in childhood behavior disorders]. / Bedrohte berufliche Situation von Eltern als pathogenetischer Faktor kindlicher Verhaltensstörungen.

Real or imminent loss of employment of family supporters (mostly fathers)--unfortunately nowadays a rather frequent event--has gained more and more importance with respect to genesis of child's behavioral disturbances. Different interaction patterns are listed here: family systems primarily regarded as free of conflicts can be desequilibrated by financial distress; especially by loss of self-value-feelings; by appearance of different child rearing methods. Sometimes the tolerance for already striking children decreases, resulting in decompensation.

[Conversations with parents of children with seizures]. / Gespräche mit Eltern anfallskranker Kinder.

When treating children with seizures, repeated and extensive talks with parents and child are an integrating part of diagnosis and therapy. The form of the conversation depends on its topic and the timing. From the physician's point of view, there are 3 forms of such conversations. 1. Informative, 2. counselling, 3. therapeutic. The subjects are in particular 1. getting the anamnestic data, 2. breaking the diagnosis to the parents and discussing the prognosis, 3. helping them in their confrontation with the "fate" of their child having epilepsy, 4. discussing special pedagogical and psycho-social problems. This kind of talk ranges from preventing faulty educational attitudes to intervening therapeutically within the patient's family.

[Treatment result and treatment resistance in early childhood petit mal epilepsy]. / Therapieerfolg und Therapieresistenz bei frühkindlichen Epilepsien mit kleinen Anfällen.

The data of 115 children with age-dependent epilepsy in early childhood and treated in the out-patient department of the university clinic for pediatrics in Vienna in the past 20 years were prepared for computerization and subsequently processed. The average observation period was 10,9 years (SD = 6,81 years). At the time of the study 20 out of 35 children with infantile spasms and 61 out of 80 children with myoclonic-astatic seizures were free from seizures. We consider Benzidiazepines to be the optimum treatment for both of the above-mentioned kinds of seizures. If they failed to achieve the desired success, ACTH and steroids proved to be quite effective. In cases of myoclonicastatic seizures with generalized EEG patterns, VPA is indicated. In order to be able to judge the dynamics in psychic development more effectively, we divided our patients into three groups: children with stable development, those with obvious retardation, and those with obvious positive development in the course of therapy. There was no statistically significant correlation between a delayed onset of therapy and the absence of seizures as well as a more satisfactory psychic development (according to the Man-Whitney-Test and the Kruskal-Wallis-Test). In children with infantile spasms we found a statistically significant correlation (p less than 0.05) between relief from seizures and a satisfactory psychic development. In general, the somatic and psychic prognosis of Petit-Mal epilepsies in early childhood seems to depend on pretherapeutical factors.

[Articulation disorder as the initial manifestation of facioscapulohumeral muscular dystrophy in childhood]. / Artikulationsstörung als Erstmanifestation einer facioscapulohumeralen Muskel-dystrophie im Kindesalter.

A five year old boy who had received logopedic treatment for more than two years was seen as an outpatient because of speech retardation. He presented with myopathic face, incomplete closure of both lids and severe weakness of facial muscles, bilateral winging of scapulae and hyperlordosis. Extraocular and pharyngeal muscles were not affected. Motor and sensory nerve fibre conductions and electromyography were within normal limits. CPK was moderately elevated (320 U/I). Muscle biopsy of right deltoid muscle revealed unspecific myopathic changes. The patients brother aged 7 also presented with facial weakness, elevated CPK and neurogenic changes in EMG of deltoid muscle. Both parents were clinically and electrophysiologically unremarkable. Although problems to speak distinctly are usually not the first manifestation, we found in this family facio-scapulo-humeral muscular dystrophy.