Rückfallprognosen in der forensischen Psychiatrie

Ziel der Arbeit war, die prädiktive Validität der Prognoseinstrumente ILRV, HCR-20 und PCL-R zu vergleichen und den VRAG erstmals an einer deutschen Stichprobe zu validieren. Hierzu wurde das englische Originalmanual des VRAG und des SORAG ins Deutsche übersetzt. Zu Beginn der Studie lagen die Ergebnisse von Voruntersuchungen vor, in denen unter anderem die prognostische Überlegenheit der modifizierten Basisrate für Rückfälligkeit, des PCL-R Gesamtwertes und der historischen Variablen festgestellt worden war. Diese Ergebnisse wurden an der vorliegenden Stichprobe getestet. Die Prognoseinstrumente wurden anhand von Gutachten für 136 Straftäter ausgefüllt, die 1994 und 1995 an der Universität München auf Schuldunfähigkeit hin begutachtet worden waren. Das Ergebniskriterium Rückfälligkeit wurde im März 2003 für die Probanden anhand der Einträge in das Bundeszentralregister seit ihrer Begutachtung ermittelt. Die durchschnittliche time-at-risk betrug 58.06 Monate. 43 von 113 Probanden (38.1%) wurden rückfällig, 21 Probanden mit einem gewalttätigen Rückfall, 22 Probanden mit einem nicht-gewalttätigen Rückfall. Die prädiktive Validität der Prognoseinstrumente wurde mittels der AUC in ROC- Analysen bestimmt. Die erstmalige Validierung des VRAG an einer deutschen Stichprobe wurde durch verschiedene Untersuchungen ergänzt: Korrelation mit der Rückfälligkeit für Probanden mit VRAG-Summenwerten unter oder über dem Mittelwert, ROC-Analysen für die gesamte Stichprobe, für Probanden mit gewalttätigen Indexdelikten und für das Ergebniskriterium Rückfälligkeit allgemein bzw. gewalttätige Rückfälligkeit, Korrelation der beobachteten Rückfallsrate mit der von den Autoren des VRAG vorhergesagten Rückfallswahrscheinlichkeit für die 9 Gruppen der VRAG Summenwerte und die Kaplan-Meier Überlebensanalyse für Probanden mit VRAG Summenwerten über und unter dem Mittelwert. Der VRAG Gesamtwert (AUC .703), die modifizierte Basisrate (AUC .661) und der PCL-R Gesamtwert (AUC .630) korrelierten am besten mit der Rückfälligkeit, sie sollten standardisiert in die klinische Beurteilung des Rückfallrisikos aufgenommen werden. Der VRAG weist auch für diese deutsche Stichprobe die höchste prädiktive Validität aller Prognoseinstrumente auf. Entwicklungsmethode und Bewertungssystem des VRAG stellen wichtige Grundlagen für die zukünftige Prognoseforschung dar. Die Validierung des im Aufbau dem VRAG ähnlichen SORAG für Sexualstraftäter steht in Deutschland noch aus

Retained primary teeth in STAT3 hyper-IgE syndrome: early intervention in childhood is essential

Background STAT3 hyper-IgE syndrome (STAT3-HIES) is a rare primary immunodeficiency that clinically overlaps with atopic dermatitis. In addition to eczema, elevated serum-IgE, and recurrent infections, STAT3-HIES patients suffer from characteristic facies, midline defects, and retained primary teeth. To optimize dental management we assessed the development of dentition and the long-term outcomes of dental treatment in 13 molecularly defined STAT3-HIES patients using questionnaires, radiographs, and dental investigations. Results Primary tooth eruption was unremarkable in all STAT3-HIES patients evaluated. Primary tooth exfoliation and permanent tooth eruption was delayed in 83% of patients due to unresorbed tooth roots. A complex orthodontic treatment was needed for one patient receiving delayed extraction of primary molars and canines. Permanent teeth erupted spontaneously in all patients receiving primary teeth extraction of retained primary teeth during average physiologic exfoliation time. Conclusions The association of STAT3-HIES with retained primary teeth is important knowledge for dentists and physicians as timely extraction of retained primary teeth prevents dental complications. To enable spontaneous eruption of permanent teeth in children with STAT3-HIES, we recommend extracting retained primary incisors when the patient is not older than 9 years of age and retained primary canines and molars when the patient is not older than 13 years of age, after having confirmed the presence of the permanent successor teeth by radiograph

Categorizing diffuse parenchymal lung disease in children

Background Aim of this study was to verify a systematic and practical categorization system that allows dynamic classification of pediatric DPLD irrespective of completeness of patient data. Methods The study was based on 2322 children submitted to the kids-lung-register between 1997 and 2012. Of these children 791 were assigned to 12 DPLD categories, more than 2/3 belonged to categories manifesting primarily in infancy. The work-flow of the pediatric DPLD categorization system included (i) the generation of a final working diagnosis, decision on the presence or absence of (ii) DPLD and (iii) a systemic or lung only condition, and (iv) the allocation to a category and subcategory. The validity and inter-observer dependency of this workflow was re-tested using a systematic sample of 100 cases. Results Two blinded raters allocated more than 80 % of the re-categorized cases identically. Non-identical allocation was due to lack of appreciation of all available details, insufficient knowledge of the classification rules by the raters, incomplete patient data, and shortcomings of the classification system itself. Conclusions This study provides a suitable workflow and hand-on rules for the categorization of pediatric DPLD. Potential pitfalls were identified and a foundation was laid for the development of consensus-based, international categorization guidelines

GATA2 deficiency in children and adults with severe pulmonary alveolar proteinosis and hematologic disorders

Background The majority of cases with severe pulmonary alveolar proteinosis (PAP) are caused by auto-antibodies against GM-CSF. A multitude of genetic and exogenous causes are responsible for few other cases. Goal of this study was to determine the prevalence of GATA2 deficiency in children and adults with PAP and hematologic disorders. Methods Of 21 patients with GM-CSF-autoantibody negative PAP, 13 had no other organ involvement and 8 had some form of hematologic disorder. The latter were sequenced for GATA2. Results Age at start of PAP ranged from 0.3 to 64 years, 4 patients were children. In half of the subjects GATA2-sequence variations were found, two of which were considered disease causing. Those two patients had the typical phenotype of GATA2 deficiency, one of whom additionally showed a previously undescribed feature – a cholesterol pneumonia. Hematologic disorders included chronic myeloic leukemia, juvenile myelo-monocytic leukemia, lymphoblastic leukemia, sideroblastic anemia and two cases of myelodysplastic syndrome (MDS). A 4 year old child with MDS and DiGeorge Syndrome Type 2 was rescued with repetitive whole lung lavages and her PAP was cured with heterologous stem cell transplant. Conclusions In children and adults with severe GM-CSF negative PAP a close cooperation between pneumologists and hemato-oncologists is needed to diagnose the underlying diseases, some of which are caused by mutations of transcription factor GATA2. Treatment with whole lung lavages as well as stem cell transplant may be successful

Lung disease caused by ABCA3 mutations

Background Knowledge about the clinical spectrum of lung disease caused by variations in the ATP binding cassette subfamily A member 3 (ABCA3) gene is limited. Here we describe genotype-phenotype correlations in a European cohort. Methods We retrospectively analysed baseline and outcome characteristics of 40 patients with two disease-causing ABCA3 mutations collected between 2001 and 2015. Results Of 22 homozygous (15 male) and 18 compound heterozygous patients (3 male), 37 presented with neonatal respiratory distress syndrome as term babies. At follow-up, two major phenotypes are documented: patients with (1) early lethal mutations subdivided into (1a) dying within the first 6 months or (1b) before the age of 5 years, and (2) patients with prolonged survival into childhood, adolescence or adulthood. Patients with null/null mutations predicting complete ABCA3 deficiency died within the 1st weeks to months of life, while those with null/other or other/other mutations had a more variable presentation and outcome. Treatment with exogenous surfactant, systemic steroids, hydroxychloroquine and whole lung lavages had apparent but many times transient effects in individual subjects. Conclusions Overall long-term (>5 years) survival of subjects with two disease-causing ABCA3 mutations was <20%. Response to therapies needs to be ascertained in randomised controlled trials

The basidiomycetous yeast Trichosporon may cause severe lung exacerbation in cystic fibrosis patients - clinical analysis of Trichosporon positive patients in a Munich cohort

Background: The relevance of Trichosporon species for cystic fibrosis (CF) patients has not yet been extensively investigated. Methods: The clinical course of CF patients with Trichosporon spp. in their respiratory secretions was analysed between 2003 and 2010 in the Munich CF center. All respiratory samples of 360 CF patients (0 - 52.4 years; mean FEV1 2010 81.4% pred) were investigated. Results: In 8 patients (2.2%, 3 male, mean age 21.8 years) Trichosporon was detected at least once. One patient carried T. asahii. One patient carried T. mycotoxinivorans and one patient T. inkin as determined by DNA sequencing. As potential risk factors for Trichosporon colonization steroid treatment, allergic bronchopulmonary aspergillosis (ABPA) and CF associated diabetes were identified in 6, 5, and 2 patients respectively. For one patient, the observation period was not long enough to determine the clinical course. One patient had only a single positive specimen and exhibited a stable clinical course determined by change in forced expiratory volume in one second (FEV1), body-mass-index (BMI), C-reactive protein (CRP) and immunoglobulin G (IgG). Of 6 patients with repeatedly positive specimen (mean detection period 4.5 years), 4 patients had a greater decline in FEV1 than expected, 2 of these a decline in BMI and 1 an increase in IgG above the reference range. 2 patients received antimycotic treatment: one patient with a tormenting dry cough subjectively improved under Amphotericin B inhalation; one patient with a severe exacerbation due to T. inkin was treated with i.v. Amphotericin B, oral Voriconazole and Posaconazole which stabilized the clinical condition. Conclusions: This study demonstrates the potential association of Trichosporon spp. with severe exacerbations in CF patients

Recomindation: New Functions for Augmented Memories

Abstract. Advances in technological support for augmented personal memories make possible new ways of enhancing the process of product recommendation. Instead of simply analyzing information about a user’s past behavior in order to generate recommendations, a recominder 1 system can additionally supply various types of information from the user’s augmented memory that allows the user to take a more active role in the search for suitable products. We illustrate the paradigm of recomindation with reference to a prototype implementation of the system SPECTER in a CD shopping scenario and the results of a study with 20 subjects, who found most of the recomindation functionality to constitute a useful enhancement of their shopping experience. 1 An Introduction to Recomindation One development of the past few years that promises to bring substantial innovations in the area of adaptive hypermedia (among others) concerns augmented personal memories: It is becoming feasible for a system, with the user’s consent, to store a vast amount of information about the user’s actions, experiences, and contexts over a long perio