[Peripheral facial palsy accompanied by a vesicular rash on the ear and hard palate]. / Periphere Fazialisparese mit Krusten an der Ohrmuschel und Bläschen am Gaumen. Der pädiatrische PRAXIS-Fall.

Ramsay Hunt syndrome (RHS) is defined as a peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) and hard palate. It is known that varicella zoster virus (VZV) causes RHS. History and neurological examination remain the mainstay of diagnosis. Prednisolone and acyclovir/valacyclovir is helpful in RHS when given within three days of onset. We report on a 14-year-old boy who had RHS accompanied by meningitis. Polymerase chain reaction identified VZV in exudates from the geniculate zone of the ear, the hard palate and in the CSF.

Large deletions in the CFTR gene: clinics and genetics in Swiss patients with CF.

Cystic fibrosis (CF) is the most common life-shortening autosomal recessive disorder in Caucasians, and is associated with at least one mutation on each CF transmembrane conductance regulator (CFTR) allele. Some patients, however, with only one identifiable point mutation carry on the other allele, a large deletion that is not detected by conventional screening methods. The overall frequency of large deletions in patients with CF is estimated to be 1-3%. Using the CFTR Multiplex Ligation dependent Probe Amplification Kit (MRC-Holland, Amsterdam, Netherlands) that allows the exact detection of copy numbers from all 27 exons in the CFTR gene, we screened 50 patients with only one identified mutation for large deletions in the CFTR gene. Each detected deletion was confirmed using our real-time polymerase chain reaction (PCR) assay and deletion-specific PCR reactions using junction fragment primers. We detected large deletions in eight patients (16%). These eight CF alleles belong to four different deletion types (CFTRindel2, CFTRdele14b-17b, CFTRdele17a-17b and CFTRdele 2-9) whereof the last is novel. Comparing detailed clinical data of all these patients with CF and the molecular genetic findings, we were able to elaborate criteria for deletion screenings and possible genotype-phenotype associations. In conclusion, we agree with other authors that deletion screenings should be implemented in routine genetic diagnostics of CF.

Characteristics of memory dysfunction in body dysmorphic disorder.

Although body dysmorphic disorder (BDD) is receiving increasing empirical attention, very little is known about neuropsychological deficits in this disorder. The current study investigated the nature of memory dysfunction in BDD, including the relationship between encoding strategies and verbal and nonverbal memory performance. We evaluated 17 patients with BDD and 17 healthy controls using the Rey-Osterrieth Complex Figure Test (RCFT) and the California Verbal Learning Test (CVLT). BDD patients differed significantly from healthy controls on verbal and nonverbal learning and memory indices. Multiple regression analyses revealed that group differences in free recall were statistically mediated by deficits in organizational strategies in the BDD cohort. These findings are similar to patterns previously observed in obsessive-compulsive disorder (OCD), suggesting a potential relationship between OCD and BDD. Studies in both groups have shown that verbal and nonverbal memory deficits are affected by impaired strategic processing.

Comparison of immunogenicity and safety of a virosome influenza vaccine with those of a subunit influenza vaccine in pediatric patients with cystic fibrosis.

The objective of this study was to compare the immunogenicity and safety of a single-dose regimen and a two-dose regimen of a trivalent virosome influenza vaccine (Inflexal Berna V) with those of a trivalent subunit influenza vaccine (Influvac) in children and adolescents with cystic fibrosis (CF). In an open, randomized, multicenter study with parallel groups, 11 young children with CF (1 to 6 years old) and 53 older children and adolescents with CF (>6 years old) were randomly assigned to one of the following immunization regimens: virosome vaccine at 0.5 ml on study day 0 or 0.25 ml on days 0 and 28 or a standard regimen of subunit vaccine, i. e., 0.5 ml on day 0 for older children and 0.25 ml on days 0 and 28 for younger children. Safety assessments, i.e., recording of systemic and local adverse events (AEs) and vital signs, were made for a 5-day observation period after each immunization. Hemagglutination inhibition (HI) titers were determined at baseline and 4 weeks after the single-dose and the two-dose immunizations, respectively. Immunogenicity was assessed according to the criteria of the European Agency for the Evaluation of Medicinal Products (EMEA). Both vaccines induced comparable HI antibody titers. Seroconversion (> or =4-fold rise in HI antibody titers, reaching a titer of > or =1:40) was achieved in 41 to 100% of the participants. Seroprotection (HI titer, > or =1:40) and a >2.5-fold increase in geometric mean titers were achieved in 100% of the participants. Thus, all three EMEA requirements for influenza vaccine efficacy were met by all treatment groups and for both vaccines. The virosome vaccine, when administered as a single dose, seemed to induce superior immunogenicity compared with the standard pediatric two-dose regimen. Totals of 42 and 57% of vaccinees receiving virosome and subunit vaccines, respectively, reported at least one local AE (predominantly pain). Totals of 84 and 71% of subjects receiving virosome and subunit vaccines, respectively, complained in response to questions of at least one systemic AE (mainly cough, fatigue, coryza, or headache). The majority of events were mild or moderate and lasted 1 or 2 days only. No obvious relationship was found between AE reporting rate and vaccine formulation, age group, or dose regimen. The relatively high AE reporting rate seemed to be partly related to the symptomatology of the underlying CF disease. In summary, the virosome and subunit vaccines induced in both age groups and against all three influenza strains an efficient immune response and were well tolerated by the children and adolescents with CF.

Ozone exposure-response relationships for biomass and root/shoot ratio of beech (Fagus sylvatica), ash (Fraxinus excelsior), Norway spruce (Picea abies) and Scots pine (Pinus sylvestris).

Current-year seedlings of beech, ash, Norway spruce and Scots pine were exposed during one growing season to different, but moderate, ozone (O(3)) scenarios representative for Switzerland (50, 85, 100% ambient, 50% ambient+30 nl l(-1)) in open-top chambers (OTCs) and to ambient O(3) concentrations in the field. Biomass significantly decreased with increasing O(3) dose in all species except for spruce. Losses of 25.5% (ash), 17.4% (beech), 9.9% (Scots pine) were found per 10 microl l(-1) h accumulated O(3) exposure over a threshold concentration of 40 nl l(-1) during daylight hours (AOT40). Ratios of root/shoot biomass (RSR) also significantly decreased with increasing AOT40 levels in beech and ash, but not in Norway spruce and Scots pine. The data show that the deciduous species beech and ash were more susceptible to O(3) with respect to RSR and biomass than the coniferous species Norway spruce and Scots pine.

National infrastructures for delivery of hepatitis B vaccination to different age groups.

If hepatitis B vaccination is to be introduced into a universal vaccination programme, it must be given before young people become sexually active. Two age groups are of main interest: infants and early adolescents. In Australia, North America and Europe, vaccination programmes for infants are well established but not yet for adolescents. From a developmental point of view, targeting adolescents seems to be a demanding task. Nevertheless, there are examples of successful approaches: e.g. rubella vaccination in the UK, where since its introduction in 1976 the immunization coverage among 14-year-olds has remained high at 80-85%, or the more recent hepatitis B strategies in Italy and Spain, where young adolescents are included with high success rates. In summary, whereas the inclusion of HBV vaccine in the infant immunization programme is not a problem in most counties, the approach to adolescents needs more thought.

[Intellectual and neurological development of 9-15-year-olds who were born as at-risk neonates]. / Intellektuelle und neurologische Entwicklung von 9- bis 15jährigen ehemaligen Risikoneugeborenen.

This retrospective study deals with 296 children born between 1, Jan. 1974 and 31, Dec. 1980, who had been hospitalized as newborns at the Department of Pediatrics of the University of Berne and who had been considered at risk for abnormal psychomotor development because of well defined perinatal risk factors. Their psychomotor development had first been evaluated in the first months and years of life at the Cerebral Palsy Centre at Berne. In 1989, when the children were 9-15 years old, their neuro-intellectual development was investigated through a questionnaire sent to the families dealing with the subsequent psychosocial and scholastic course. In the CP-Centre, at a median age of 10 and mean age of 26.4 months, 247 (83.5%) of the children had been discharged with the finding of normal psychomotor development, 25 (8.4%) had been considered to show questionable findings and 24 (8.1%) to show obvious abnormal psychomotor development. On the basis of the answers to the questionnaire (median age 11 8/12, mean age 11 10/12 years; 62% return rate), 39.3% of the children had had no further problem, and 57.4% had used special support such as speech therapy or educational consulting and/or had had school problems. 3.3% showed distinct learning disabilities. More than 90% were attending a normal public school. This corresponds to the attendance in the general population. However, fewer children were in higher schools. To investigate whether the judgement in the first months and years (CP-Centre) of life corresponded to the further development (questionnaire), a subpopulation was specially evaluated in addition.(ABSTRACT TRUNCATED AT 250 WORDS)

[Chronic disease and adolescence]. / Chronische Krankheit und Adoleszenz.

Chronic disease is not a strictly defined term and includes a large number of illnesses ranging from physical to mental impairment. It is estimated that between 10% and 20% of adolescents have a chronic disease. Independence and new relations, acceptance of a new body image and sexuality, career plans and cognitive maturation are core topics in development to adulthood. Chronic disease may interfere with these developmental tasks. Most often there is no specific psychopathology, but the type of impairment, its influence on family life and functioning, age at onset, gender, and other factors will interact with psychosocial maturation. Because of the important role of the family, not only the adolescent patient him/herself, but also parents and siblings need to be included in all major decisions. As hospitalizations may be disruptive they must be planned, taking in account the patient's plans and opinions. Chronic disease may lead to death during the period of adolescence. It is believed that the concept of one's own mortality develops at age 14 to 17 years, a fact that will influence care during the terminal stage of a disease. Whatever the problems and questions raised by the family, the developmental stage of the adolescent has always to be considered when dealing with specific issues of chronic disease. Periodic reassessment of psychosocial development is therefore one of the main tasks of the primary care physician. Counselling will address not only the disease but also the developmental tasks of any teenager.

Chronic immune thrombocytopenic purpura--immunological analyses of a patient pre- and post-HIV seroconversion.

A seven year follow-up of immune parameters is reported for a patient with chronic immune thrombocytopenic purpura (ITP) pre and post human immunodeficiency virus (HIV) seroconversion. Therapies such as intravenous IgG, prednisone, vincristine, or Ciclosporin A had no clear-cut beneficial effect on platelet counts. A long-term normalization of platelet counts was achieved by splenectomy. At splenectomy the patient was seropositive for HIV, most likely transmitted by blood products received half a year prior to laparatomy. Mean plasma levels of the second component of complement, C2, were half of the normal values prior to and within the lowest normal range post HIV seroconversion. Nevertheless, the T cell-dependent B cell response to HIV, which is dependent on the activation of C3 via the classical pathway of complement, was normal: Western blot analysis of total IgG and of IgG subclass responses to individual HIV antigens proved to be unimpaired.

Caring for an adolescent with a chronic illness.

Although early, mid, and late adolescence are transient psychological periods, the teenager must master these three phases to complete the psychological stages and tasks of adolescence. Because chronic disease delays or alters these phases, it becomes imperative for the primary care physician to reassess psychological development periodically for appropriate and intensive counseling. With the advantage of continuous contact with the family and the understanding of the family's structure and interpersonal relationships, the primary care physician may be able to: alleviate struggles for control that may seriously impede care, encourage the teenager to accomplish the psychological tasks of adolescence, both during hospitalization and in follow-up outpatient care visits, promote the adolescent's participation in his or her own health care, and ultimately enhance both the family's and the teenager's adaptation to a chronic illness. Finally, during the terminal phase of an illness, the primary care physician will be able to help the adolescent find meaning in his or her short life, provide the support to help the teenager to disengage from life with dignity, and provide a supportive relationship to the parents and siblings.

[Toxic hemolytic anemia in the newborn infant following ingestion of a phenazone derivative (Cibalgin) via breast milk]. / Toxische hämolytische Anämie beim Neugeborenen nach Ingestion eines Phenazon-Derivates (Cibalgin) via Muttermilch.

After the administration of Cibalgin (a Phenazone-derivative) to a lactating mother during the first days after delivery, her healthy newborn infant developed a toxic hemolytic anemia with precipitation of globin, forming large inclusion bodies in the erythrocytes. Phenazone was found in the mothers milk up to 8 days after the end of Cibalgin administration. During the acute phase of hemolysis it could also be demonstrated in the infants serum. A list of medicaments that pass into the milk and can cause a toxic hemolytic anemia in the child is included.

[Drug withdrawal syndrome in the neonate]. / Drogenentzugssyndrom beim Neugeborenen.

The relationship between maternal abuse of methadone and neonatal signs of withdrawal has been studied. The main symptoms are frequent vomiting or diarrhea, hyperpyrexia, moderate or severe irritability and tremors, inability to sleep between feedings, and clinically apparent convulsions. Physicians caring for such high-risk infants should recognize the potential seriousness of this problem.