Mit dem Problemfeld "sexueller Misbrauch an Kindern und Jugendlichen" ist ein breites Spektrum verschiedenster Institutionen konfrontiert. Bislang gibt es nur wenige systematische Beschreibungen des institutionellen Umgangs mit betroffenen Kindern und Jugendlichen und dessen Auswirkungen auf die Betroffenen. In diesem Beitrag werden erste Auswertungen einer Studie dargestellt, die sich mit individuellen und institutionellen Reaktionen auf die Problematik des sexuellen Misbrauchs von Kindern und Jugendlichen befast. Rechtliche Bestimmungen spielen gerade im Umgang mit dieser Problematik eine wesentliche Rolle. Die hier vorgestellten ersten Forschungsergebnisse basieren auf quantitativen Fragebogenerhebungen und qualitativen Experteninterviews, die in Berlin und Koln durchgefuhrt wurden. Befragt wurden 195 Mitarbeiter/innen (Berufserfahrungsmedian 8 Jahre) des gesamten institutionellen Spektrums (Beratungsstellen, Kriseneinrichtungen, Kliniken, Jugendamter, Staatsanwaltschaften, Gerichte, Polizei), von denen 40 Personen zusatzlich an dem Experteninterview teilnahmen Im Mittelpunkt der hier dargestellten Ergebnisse steht die Frage, wie die verschiedenen Institutionen mit rechtlichen Bestimmungen umgehen. Am Beispiel der Nutzung von Strafrecht zeigt sich die unterschiedliche Einflusnahme gesetzlicher Rahmenbedingungen auf das Handeln der versorgenden Einrichtungen Die enge Verknupfung zwischen der Bewertung strafrechtlicher Masnahmen und Beratungskonzepten kann am Beispiel der vermeintlich homogenen Gruppe der spezialisierten Beratungsstellen gezeigt werden.

This article describes experiences of parents of sexual abused children and their evaluations of institutional interventions on sexual child abuse. Results are presented of a study investigating 'individual and institutional reactions on sexual child abuse'. The number of contacted institutions, personnel experiences with these institutions and resulting requirements are described. The results are based on a sample of 47 (82.5%) girls and 10 (17.5%) boys (range 6-18 years) and interviews with their 'non-abusing' parents. 28 (49.1%) of these children were abused by a member of the family, 29 (50.9%) children by non-familiar persons. It could be shown that 70.2% of the children had contacted four or more different institutions soon after the sexual abuse had been revealed. In cases of sexual abuse by a family member the first contacted institution was the Youth-and-Health-Care-System whereas in cases of sexual abuse by non-familiar persons mostly the police was contacted. Nevertheless in most cases both judicial and supporting approach were chosen. Additionally to concrete advices and professional competences the interviewed parents reported that emotional warmth was a very helpful aspect of the professional intervention. Generally the perspective of the parents seems to give some interesting informations about 'consumer satisfaction'.

This article describes experiences of parents of sexual abused children and their evaluations of institutional interventions on sexual child abuse. Results are presented of a study investigating 'individual and institutional reactions on sexual child abuse: The number of contacted institutions, personel experiences with these institutions and resulting requirements are described. The results are based on a sample of 47 (82,5%) girls and 10 (17,5%) boys (range 6-18 years) and interviews with their 'non-abusing' parents. 28 (49,1%) of these children were abused by a member of the family, 29 (50.9%) children by non-familiar persons. It could be shown that 70,2% of the children had contacted four or more different institutions soon after the sexual abuse had been revealed. In cases of sexual abuse by a family member the first contacted institution was the Youth-and-Health-Care-System whereas in cases of sexual abuse by non-familiar persons mostly the police was contacted. Nevertheless in most cases both judical and supporting approach were chosen. Additionally to concrete advices and professional competences the interviewed parents reported that emotional warmth was a very helpful aspect of the professional intervention. Generally the perspective of the parents seems to give some interesting informations about 'consumer satisfaction'.

A clinical study of 204 patients with unilateral multicystic renal dysplasia referred to 30 nephrology departments was undertaken to assess the frequency of complications in children who underwent nephrectomy (n=40) versus those who were treated conservatively (n=164). Six patients required antihypertensive treatment, 30 (13% of girls, 17% of boys) had at least one episode of urinary tract infection. The number of clinical complications did not differ in patients who underwent nephrectomy in comparison to those who did not. The dysplastic kidney decreased in size in 65% of kidneys with repeated ultrasound values; no change occurred in 16%, while an increase in maximal diameter was observed in 19%. Contralateral kidney length of more than 2 standard deviation scores (SDS) was seen in 30% of patients. There is evidence for a slight impairment of renal function in the whole study group given by a median of serum creatinine level of 0.63 SDS in all patients available for analysis. Among those 35 patients with contralateral anomalies (mainly obstructive changes and vesico-ureteral reflux), all 3 patients with contralateral changes suggestive of hypoplasia and 22% with obstruction, but only 1/7 (14%) with reflux showed elevated serum creatinine level >2 SDS.

This collaborative group describes the clinical course of 66 boys and 49 girls with autosomal recessive polycystic kidney disease (ARPKD). The patients, enrolled from 1987 to 1993, were followed up after birth and all survived at least 1 month The patients were followed up clinically and with laboratory examination at entry and usually at least once per year thereafter. Body weight, height, blood pressure measurements, serum creatinine, calculated glomerular filtration rate (GFR), urinalysis, and ultrasound were obtained. Age at diagnosis varied from prenatal to greater than 5 years.In 103 children diagnosed after birth, the clinical features leading to further examination included: palpable abdominal masses in 47, hypertension in 25, urinary tract infections in 13, and a family history of ARPKD in 9. Other manifestations included hepatosplenomegaly, respiratory failure, failure to thrive, eosphageal varices, and enuresis. During the study 70% of the children were treated with antihypertensive drugs; 51% were hypertensive at initial diagnosis, requiring drug therapy. In 68% of the children the total kidney length was above the with percentile. Urinary tract infections were observed more often in girls than in boys, 43% vs 20%. Proteinuria was observed in almost half of the patients. Twenty-five percent of the children had a height, below die 3rd percentile for age. This was likely due to renal failure since 34 of the 28 had decreased GFR. Forty-six percent of the children presented with clinical signs of hepatic fibrosis assessed by liver involvement, esophageal varices, or spleen enlargement.A total of 13 patients died during the observation period, with a female predominance. Death occur-red in the first year of life in 10 patients and was due to sepsis, asphyxia, cardiac failure, sudden infant death syndrome, and Potter's syndrome. Kaplen-Meier survival curves showed that the 1-year survival for boys was 94% and 82% for girls. Beyond the third year of life the survival probability decreased to 79% for girls; conversely no male patient died after the first year of life. The time to dialysis and. transplantation was variable. Median age of onset of an increase in serum creatinine or decrease in GFR was about 1 year of age. The mean age of the children with a serum creatinine greater than 2 standard deviations above the mean was age 4 years. End-stage renal disease occurred in a total of 11 patients, 7 females and 1 males.

The aim of this study was to assess rates for tic disorders and obsessive compulsive psychopathology in families of children and adolescents with Gilles de la Tourette syndrome (TS). Diagnoses were based on the DSM III-R criteria. Obsessive compulsive psychopathology, that did not fulfill the criteria for obsessive compulsive disorder (OCD) was additionally assessed and termed obsessive compulsive symptoms (OCS). The authors hypothesized that comorbid OCD or OCS in TS patients predicts a higher familial loading with obsessive compulsive symptomatology. The study cohort included 87 patients with TS who were evaluated clinically and with the use of a structured psychiatric interview. All available parents (152/174; 87%), several sibs (49/93; 53%) and some second degree relatives (27/659; 4.1%) were also interviewed. For other first and second degree relatives the family history method was used. Familial rates for TS were clearly elevated. Rates for chronic tic disorders (CT) were considerably lower than in previous studies. Additionally, tic disorders not otherwise specified (TDNOS) were diagnosed in a substantial number of first degree (15/267; 5.6%) and second degree relatives (36/659; 5.5%). OCD in parents (4/174; 2.3%) did not occur in an above baseline rate. However, both OCD (14/87; 16.1%) and OCS (15/87; 17.2%) were frequently associated with TS in index patients. Interestingly, 10 of 16 fathers with OCS also had a tic disorder. Obsessive compulsive psychopathology clustered in families. It is concluded that genetic studies in TS could profit from adhering to a conservative diagnostic approach to both tic disorders and OCD. The familial clustering of OCS/OCD in conjunction with the elevated paternal rate for the co-occurrence of tic disorders and OCS might indicate heterogeneity of TS.

A broad spectrum of very different institutions is confronted with the problem of sexual child abuse. So far, only a few systematic descriptions of institutional interventions and their impact on sexually abused children exist. In this article, first results are presented of a study investigating "individual and institutional reactions on sexual child abuse'. In dealing with this problem legal provisions are of special importance. The results presented are based on data obtained from questionnaires and qualitative interviews with experts (median of vocation experience: 8 years) in two german cities (berlin/cologne). Questionnaires were filled in by 195 experts from the entire institutional spectrum (counselors, clinical centres, youth welfare departments, public prosecutors, courts of justice, police), of whom 40 additionally took part in a detailed interview. This article focuses on the question, how different institutions handle legal provisions. The use of criminal law exemplarily shows the different impact of legal provisions on the interventions of counselors. The close connection between the assessment of legal interventions and counseling concepts could be shown at the example of the allegedly homogeneous group of specific counselors.

The clinical course of 66 boys and 49 girls with autosomal recessive polycystic kidney disease recruited from departments of paediatric nephrology was investigated over a mean observation period of 4.92 years. This is a selected study group of children from departments of paediatric nephrology who in most cases survived the neonatal period, since birth clinics did not participate. The median age at diagnosis was 29 days (prenatal to 14.5 years). We observed decreased glomerular filtration rates (GFRs) in 72% (median age at onset of decrease of GFR < 2SD, 0.6 years; range. 0‐18.7 years), and 11 patients developed end‐stage renal disease. Hypertension requiring drug treatment was found in 70% (median age at start of medication, 0.5 years; range, 0‐16.7 years). Kidney length was above the 97th centile in 68% of patients, and kidney length did not increase with age or deterioration of renal function. Urinary tract infections occurred in 30%, growth retardation in 25%. and clinical signs of hepatic fibrosis were detected in 46%. Thirteen patients (11 %) died during the observation period. 10 of them in the first year of life. There was a statistically significant sex difference in terms of a more pronounced progression in girls. The survival probability at 1 year was 94% for male patients and 82'% for female patients ( p < 0.05) in this study. Urinary tract infections occurred more frequently in girls ( p < 0.025) and were observed earlier. In addition, more girls had impaired renal function. developed end‐stage renal disease and showed growth retardation; these differences, however. were not significant. For the children in this study, however, our results indicate that the long‐term prognosis in the majority of cases is better throughout childhood and youth than often stated.

What has been changed in the system of social help since sexual abuse became a public topic? Is social work capable of providing adequate help and support for the survivors? The article presents the results of a three-year-lasting research project. In three different regions the authors have scrutinized 1. the institutions offering help, 2. the knowledge of and the attitude towards sexual violence of the professionals and 3. the conceptional assumptions leading social work. The results show that the situation for survivors of sexual abuse has definitely improved, because there are more helping institutions and the professional awareness has increased. But for a profound reorganization of help, restrictive financial politics, internalized prejudices and institutional concepts need to be changed.

Forty-two children out of 20 sibships with autosomal recessive polycystic kidney disease were observed pro- and retrospectively over a mean period of 3.7 years in a long-term study on cystic kidney diseases in children. The intra- and interfamilial variability in terms of age at diagnosis, administration of antihypertensive therapy, liver affection, and renal function were evaluated. According to the 1971 subclassification of Blyth & Ockenden, defining different grades of severity, 12 patients were assigned to the perinatal, nine to the neonatal, 13 to the infantile, and eight to the juvenile subtype of autosomal recessive polycystic kidney disease. In 11 of the 20 families different subtypes were observed among affected siblings. In seven families, affected sibs belonged to adjacent subtypes, while major intrafamilial differences were observed in only four families. The defined subtypes, therefore, cannot be regarded as appropriate in distinguishing genetic groups of autosomal recessive polycystic kidney disease. With respect to the severity of autosomal recessive polycystic kidney disease, there is a wide spectrum of phenotypic manifestations, ranging from stillbirths to mildly affected of phenotypic manifestations, ranging from stillbirths to mildly affected adults, while intrafamilial variability of the clinical picture is generally small with multiple allelism as the most likely genetic explanation. Age at death, however, showed gross variation in eight sibships. Differences in the clinical course between several siblings cannot be explained by a sex influence in autosomal recessive polycystic kidney disease.

In a systematic study on the clinical picture and genetics of cystic kidneys in children, in association with the German working group on paediatric nephrology (Arbeitsgemeinschaft für Pädiatrische Nephrologie), we have investigated 79 children with early manifestation of autosomal dominant polycystic kidney disease (ADPKD). They belonged to 64 families (64 index patients and 15 affected sibs). Early manifestation was defined in this study as clinical symptoms (hypertension, proteinuria, impaired renal function, palpably enlarged kidneys) occurring before the age of 15 years. In order to estimate the recurrence risk to sibs of a previously diagnosed patient with early manifesting ADPKD, we found that 15 out of a total of 65 sibs of the 64 index patients (45% of the theoretically expected 32.5 gene carriers) showed comparable early manifestation. Another 10 symptom free children were diagnosed sonographically as having ADPKD before the age of 18 years, so that the total number of affected sibs was 25/65 in the study group, representing 76% of the gene carriers. Although the gene in childhood manifesting ADPKD can be transmitted through both sexes, a statistically significant (p < 0.05) maternal predominance was observed (M:F = 23:41). In affected sibs ages of onset, initial presentation, and the development of complications appeared to be similar in the majority of families. Our data indicate a high recurrence risk to sibs for early manifestation of ADPKD which has important implications for genetic counselling and clinical care of affected families and gives clues to the underlying genetic mechanism of childhood onset ADPKD.

Cilazapril is an orally active angiotensin converting enzyme (ACE) inhibitor which lowers peripheral vascular resistance without affecting heart rate. Like enalapril and ramipril it is a prodrug, and is hydrolysed after adsorption to cilazaprilat, which has a long terminal phase elimination half-life permitting once daily administration. Given once daily at doses between 2.5 and 5mg, cilazapril reduces arterial blood pressure in patients with mild to moderate essential and renal hypertension. Patients who do not respond adequately to cilazapril monotherapy usually respond with the addition of a diuretic such as hydrochlorothiazide. Preliminary data suggest that cilazapril is of comparable antihypertensive efficacy to usual therapeutic dosages of hydrochlorothiazide, slow release propranolol, nitrendipine, captopril and enalapril. In small studies cilazapril has produced sustained beneficial haemodynamic effects in patients with congestive heart failure. Cilazapril has been well tolerated and exhibits tolerability typical of ACE inhibitors as a class, including their lack of detrimental effect on glucose or lipid metabolism. Cilazapril should provide an effective alternative in the treatment of hypertension and, if preliminary data are confirmed, in congestive heart failure.