[Monosystemic, oligolesional Langerhans cell histiocytosis. A rare congenital dermatosis]. / Monosystemische, oligoläsionale Langerhans-Zell-Histiozytose der Haut. Eine seltene kongenitale Dermatose.

A five-day-old female infant presented with congenital red-livid papules and nodules on the head, chest, back and left arm. The nodule on the chest was ulcerated at birth. The pregnancy and delivery were uneventful. There was no history of birth trauma to account for the ulcerated lesion. The parents and the three older siblings were healthy without similar skin lesions. The skin biopsy showed in the deep layer of the dermis a multinodular, granulomatous histiocytic infiltrate. The histiocytic cells expressed S-100 and CD1a. There were additionally many eosinophils. The suspected diagnosis Langerhans cell histiocytosis was confirmed by the histologic results. The patient was referred to pediatric hematology-oncology where evaluation showed no evidence of systemic involvement. The clinical, radiological, sonographic and histological results led to the diagnosis of a congenital, monosystemic, oligolesional Langerhans cell histiocytosis of the skin. In addition to the case presentation, we review the current stand of knowledge of the pathogenesis, the clinical classification and the therapy of the Langerhans cell histiocytosis.

Volunteers: An Effective Medium for Delivering Therapeutic Lifestyle Interventions.

PURPOSE: Lifestyle modification programs have been shown to effectively treat chronic disease. The Coronary Health Improvement Program has been delivered by both paid professional and unpaid volunteer facilitators. This study compared participant outcomes of each mode in the United States. DESIGN: Pre-/post-analysis of CHIP interventions delivered between 1999 and 2012. SETTING: Professional-delivered programs in Rockford Illinois 1999-2004 and volunteer-delivered programs across North America 2005-2012. SUBJECTS: Adults ≥21 years (professional programs N = 3158 34.3% men, mean age = 54.0 ± 11.4 years; volunteer programs N = 7115 33.4% men, mean age = 57.4 ± 13.0 years). MEASURES: Body mass index, blood pressure (systolic and diastolic), blood lipid profile (total cholesterol, high-density lipoprotein, triglycerides, low-density lipoprotein), and fasting plasma glucose. ANALYSIS: Analysis of Covariance, with adjustment for age, gender, BMI change and baseline biometric and effect sizes. RESULTS: The professional-delivered programs achieved significantly greater reductions in BMI (.4%, P < .001) and HDL (1.9%, P < .001) and the volunteer-delivered programs achieved greater reductions in SBP (1.4%, P < .001), DBP (1.1%, P < .001), TC (1.4%, P = .004), LDL (2.3%, P < .001), TG (4.0%, P = .006), and FPG (2.7%, P < .001). However, the effect size differences between the groups were minimal (Cohen's d .1-.2). CONCLUSIONS: Lifestyle modification programs have been shown to effectively treat chronic disease. The Complete Health Improvement Program (CHIP) lifestyle intervention has been delivered by both paid professional and unpaid volunteer facilitators. This study compared selected chronic disease biometric outcomes of participants in each mode in the United States. It found volunteer-delivered programs do not appear to be any less effective than programs delivered by paid professionals, which is noteworthy as volunteers may provide important social capital in the combat of chronic disease.

Legionellosis must be kept in mind in case of pneumonia with lung abscesses in children receiving therapeutic steroids.

A 10-year-old boy, who had received recurrent short-course treatments with steroids to control severe autoimmune thrombocytopaenia, developed Legionnaires' disease as community-acquired pneumonia. Legionella pneumophila pneumonia was complicated by an extended abscess of the right inferior lobe, leading to residual lung cavities. Legionellosis must be kept in mind as the differential diagnosis in the case of severe pneumonia and with lung abscesses in children receiving therapeutic steroids. Legionella-specific diagnostic tests (polymerase chain reaction [PCR] in respiratory samples or urine antigen assay) and, also, specific empirical antibiotic combination therapy are required for the early detection and treatment of L. pneumophila pneumonia in childhood.

A combination of palm oil tocotrienols and citrus peel polymethoxylated flavones does not influence elevated LDL cholesterol and high-sensitivity C-reactive protein levels.

BACKGROUND/OBJECTIVES: Lipid-lowering and anti-inflammatory effects have been individually described for tocotrienols (TTs) and polymethoxylated flavones (PMFs). This study investigated low-density lipoprotein-cholesterol (LDL-C)- and high-sensitivity C-reactive protein (hsCRP)-reducing effects of combined TT-PMF treatment in low doses in hypercholesterolemic individuals with subclinical inflammation. SUBJECTS/METHODS: In the double-blind, placebo-controlled study, 240 Caucasians with LDL-C ⩾3.36 mmol/l and hsCRP ⩾1 mg/l were enrolled and randomized into group S1 (12 mg/day TT and 103 mg/day PMF), group S2 (27 mg/day TT and 32 mg/day PMF) or placebo. RESULTS: Twenty-three subjects dropped out of the study, 13 were excluded from the analysis because of lack of compliance. A total of 204 subjects per-protocol analysis were included. After 12 weeks of treatment, no significant differences in LDL-C levels (primary outcome) were observed between groups. LDL-C levels significantly decreased in all intervention groups (S1: -5.2%, S2: -4.8% and P: -4.2%). Total cholesterol and hsCRP (secondary outcome) did not change significantly. CONCLUSIONS: PMF-TT supplements had no effect beyond that of placebo on elevated LDL-C and hsCRP levels.

Mechanistic modelling of toxicokinetic processes within Myriophyllum spicatum.

Effects of chemicals are, in most cases, caused by internal concentrations within organisms which rely on uptake and elimination kinetics. These processes might be key components for assessing the effects of time-variable exposure of chemicals which regularly occur in aquatic systems. However, the knowledge of toxicokinetic patterns caused by time-variable exposure is limited, and gaining such information is complex. In this work, a previously developed mechanistic growth model of Myriophyllum spicatum is coupled with a newly developed toxicokinetic part, providing a model that is able to predict uptake and elimination of chemicals, as well as distribution processes between plant compartments (leaves, stems, roots) of M. spicatum. It is shown, that toxicokinetic patterns, at least for most of the investigated chemicals, can be calculated in agreement with experimental observations, by only calibrating two chemical- specific parameters, the cuticular permeability and a plant/water partition coefficient. Through the model-based determination of the cuticular permeabilities of Isoproturon, Iofensulfuron, Fluridone, Imazamox and Penoxsulam, their toxicokinetic pattern can be described with the model approach. For the use of the model for predicting toxicokinetics of other chemicals, where experimental data is not available, equations are presented that are based on the log (P oct/wat) of a chemical and estimate parameters that are necessary to run the model. In general, a method is presented to analyze time-variable exposure of chemicals more in detail without conducting time and labour intensive experiments.

Relation between perceived driving disability and scores of vision screening tests.

AIM: To determine the relation between perceived driving disability and vision screening tests. METHODS: 93 subjects, aged 50 years and over, with binocular visual acuity of at least 20/80. Perceived driving disability (PDD) was assessed by a questionnaire. Subtracting daytime from night-time driving question scores revealed PDD at night (PDDN), subtracting scores of questions for driving in familiar places from those in unfamiliar places revealed PDD at unfamiliar places (PDDU). RESULTS: PDD was strongly related to visual acuity, contrast sensitivity and useful field of view (UFOV). Specific relations existed between PDDN and Nyktotests and Mesotests and between PDDU and UFOV. These associations were enhanced in a subset of subjects with better visual acuities. CONCLUSIONS: Vision screening tests correlate well with perceived driving disabilities, especially when a subtraction method is used in the questionnaire to reveal condition dependent disabilities. Additional tests for visual acuity are useful, especially in subjects with better visual acuity.

The cultural construction of self-enhancement: an examination of group-serving biases.

Self-serving biases, found routinely in Western samples, have not been observed in Asian samples. Yet given the orientation toward individualism and collectivism in these 2 cultures, respectively, it is imperative to examine whether parallel differences emerge when the target of evaluation is the group. It may be that Asians show a group-serving bias parallel to the Western self-serving bias. In 2 studies, group-serving biases were compared across European Canadian, Asian Canadian, and Japanese students. Study 1 revealed that Japanese students evaluated a family member less positively than did both groups of Canadian students. Study 2 replicated this pattern with students' evaluations of their universities. The data suggest that cultural differences in enhancement biases are robust, generalizing to individuals' evaluations of their groups.

Divergent consequences of success and failure in japan and north america: an investigation of self-improving motivations and malleable selves.

Self-enhancing and self-improving motivations were investigated across cultures. Replicating past research, North Americans who failed on a task persisted less on a follow-up task than those who succeeded. In contrast, Japanese who failed persisted more than those who succeeded. The Japanese pattern is evidence for a self-improving orientation: Failures highlight where corrective efforts are needed. Japanese who failed also enhanced the importance and the diagnosticity of the task compared with those who succeeded, whereas North Americans did the opposite. Study 2 revealed that self-improving motivations are specific to the tasks on which one receives feedback. Study 3 unpackaged the cultural differences by demonstrating that they are due, at least in part, to divergent lay theories regarding the utility of effort. Study 4 addressed the problem of comparing cultures on subjective Likert scales and replicated the findings with a different measure.

Effects of light and temperature fluctuations on the growth of Myriophyllum spicatum in toxicity tests--a model-based analysis.

Laboratory toxicity tests are a key component of the aquatic risk assessments of chemicals. Toxicity tests with Myriophyllum spicatum are conducted based on working procedures that provide detailed instructions on how to set up the experiment, e.g., which experimental design is necessary to get reproducible and thus comparable results. Approved working procedures are established by analyzing numerous toxicity tests to find a compromise between practical reasons (e.g., acceptable ranges of ambient conditions as they cannot be kept completely constant) and the ability for detecting growth alterations. However, the benefit of each step of a working procedure, e.g., the random repositioning of test beakers, cannot be exactly quantified, although this information might be useful to evaluate working procedures. In this paper, a growth model of M. spicatum was developed and used to assess the impact of temperature and light fluctuations within the standardized setup. It was analyzed how important it is to randomly reassign the location of each plant during laboratory tests to keep differences between the relative growth rates of individual plants low. Moreover, two examples are presented on how modeling can give insight into toxicity testing. Results showed that randomly repositioning of individual plants during an experiment can compensate for fluctuations of light and temperature. A method is presented on how models can be used to improve experimental designs and to quantify their benefits by predicting growth responses.

[Therapy of inherited diseases of platelet function. Interdisciplinary S2K guideline of the Permanent Paediatric Committee of the Society of Thrombosis and Haemostasis Research (GTH e. V.)]. / Therapie hereditärer Thrombozytopathien. Interdisziplinäre S2K-Leitlinie der Ständigen Kommission Pädiatrie der Gesellschaft für Thrombose- und Hämostaseforschung e. V.

Inherited disorders of platelet function are a heterogeneous group. For optimal prevention and management of bleeding, classification and diagnosis of the underlying defect are highly recommended. An interdisciplinary guideline for a diagnostic approach has been published (AWMF # 086-003 S2K; Hämostaseologie 2014; 34: 201-212). Underlying platelet disorder, platelet count, age and clinical situation modify treatment. Exclusive transfusion of platelet concentrates may be inappropriate as potentially adverse effects can outweigh its benefit. A stepwise and individually adjusted approach for restitution and maintenance of haemostasis is recommended. Administration of antifibrinolytics is generally endorsed, but is of particular use in Quebec disease. Restricted to older children, desmopressin is favourable in storage pool disease and unclassified platelet disorders. Although licensed only for patients with Glanzmann thrombasthenia and alloantibodies, in clinical practice rFVIIa is widely used in inherited platelet disorders with severe bleeding tendency. This guideline aims at presenting the best available advice for the management of patients with inherited platelet function disorders.

Sequential myelin protein expression during remyelination reveals fast and efficient repair after central nervous system demyelination.

To understand the mechanisms of remyelination and the reasons for regeneration failure is one of the major challenges in multiple sclerosis research. This requires a good knowledge and reliable analysis of experimental models. This work was undertaken to characterize the pattern of myelin protein expression during experimental remyelination. Acute demyelination of the corpus callosum was induced by feeding of 0.3% cuprizone for 6 weeks, followed by a 10-week remyelination period. We used a combination of Luxol fast blue (LFB) myelin staining, electron microscopy (EM) and immunohistochemistry for the myelin proteins 2',3'-cyclic nucleotide 3' phosphodiesterase (CNPase), myelin basic protein (MBP), proteolipid protein (PLP) and myelin oligodendrocyte glycoprotein (MOG). Early remyelination was detected by the re-expression of CNPase, MBP and PLP as early as 4 days. MOG, as a marker for late differentiation of oligodendrocytes, was not detectable until 2 weeks of remyelination. EM data correlated well with the LFB myelin staining and myelin protein expression, with 50% of the axons being rapidly remyelinated within 2 weeks. While particularly MBP but also PLP and CNPase are re-expressed very early before significant remyelination is observed by EM, the late marker MOG shows a lag behind the remyelination detected by EM. The presented data indicate that immunohistochemistry for various myelin proteins expressed early and late during myelin formation is a suitable and reliable method to follow remyelination in the cuprizone model. Furthermore, investigation of early remyelination confirms that the intrinsic repair programme is very fast and switched on within days.

Self as cultural product: an examination of East Asian and North American selves.

In the past decade a wealth of research has been conducted on the cultural foundation of the self-concept, particularly with respect to East Asian and North American selves. The present paper discusses how the self differs across these two cultural contexts, particularly with respect to an emphasis on consistency versus flexibility, an intraindividual versus an extraindividual focus, the malleability of the self versus world, the relation of self to others, and self-enhancing versus self-critical motivations. These differences reveal the manifold ways that culture shapes the self.

[Intrapalpebral migration of a form stable contact lens: a rare complication in contact lens practice]. / Intrapalpebral eingewachsene, formstabile Kontaktlinse: Eine seltene Komplikation in der Kontaktologie.

PURPOSE: To demonstrate a rare complication in contactology. CASE REPORT: A 26-year-old female had "lost" her hard contact lens in the right eye 2 years previously. Afterwards, new contact lenses were not tolerated. The patient had a slight intermittent epiphora but no further complaints. She consulted her ophthalmologist for new spectacles. At presentation, there was a firm tumour without signs of inflammation in the right upper lid area. When the lid was everted a hard contact lens was found within the tarsal plate which could be easily removed in the operating room. Microbiologic investigation disclosed no bacteria. Histology showed a circumscribed papillary reaction and a chronic non-specific inflammation with few eosinophils and no giant cells. Two weeks later the lens-related cavity was only slightly filled up by granulation tissue. CONCLUSIONS: After "loss" of a contact lens superior dislocation and finally tarsal implantation should be kept in mind. The process of contact lens migration reveals some interesting features: 1. Generally, it causes only minor symptoms though it may last for years. 2. Bacterial contamination rarely occurs. 3. Histologically, the inflammation is often mild or even absent. Eosinophils and giant cells are of minor or no importance indicating that allergy and giant-cell reaction play no significant role. 4. The lens related cavity probably heals slowly, possibly because of a (partial) epithelialization.

[Clinical significance of objective vision assessment using visually evoked cortical potentials induced by rapid pattern sequences of different spatial frequency]. / Zur klinischen Bedeutung der objektiven Sehschärfenschätzung mit Hilfe der visuell evozierten kortikalen Potentiale durch schnelle Reizmustersequenzen unterschiedlicher Ortsfrequenz.

BACKGROUND: In patients where reliable subjective assessment of visual acuity is impossible, further diagnostics should be enhanced by an objective method. PATIENTS AND METHODS: A group of 34 patients was examined by objective assessment of visual acuity using visual evoked potentials (VEP) as described by Hajek and Zrenner in 1988. The presentation of five checkerboards with different spatial frequency in repetitive sequences on a TV-monitor elicits a series of transient visual evoked potentials. Shape and amplitude of each wavelet depends on check size and directly reflect a spatial tuning function with a low- and high-frequency cut-off. This amplitude is described by a polynomial fit (2nd order). The function's intersection with the x-axis at higher spatial frequencies leads to an estimation of the visual acuity. RESULT: This result is compared to the subjectively determined visual acuity. In the majority of the presented cases the suspected malingering was confirmed. CONCLUSION: Patients with suspected malingering represent the primary indication of the described method.

Substitution of anti-human globulin by protein A-bearing staphylococci in the detection of Brucella antibodies.

A coagglutination test using protein A-bearing staphylococci has been developed for the detection of Brucella antibodies. Comparing the results of a random sample of 57 sera collected from Malta fever patients, suggestive titers of 1: greater than or equal to 160 were found in 8 sera (14%) with the standard agglutination test, in 22 sera (39%) with the Coombs test, and in 23 sera (40%) with the coagglutination test. The titers in the Coombs test and the coagglutination test coincided in 54 (95%) of the 57 sera, in 3 sera (5%) the difference was no more than one dilution step. Sera from healthy subjects and patients with infections other than brucellosis showed titers up to 1:40 in all three tests. Because of its sensitivity and specificity in detecting non-agglutinating antibodies, the Brucella-antibody coagglutination test may replace the Coombs test as a complementary assay to the standard agglutination. Native sera from Malta fever patients frequently show a prozone phenomenon in the standard agglutination test and a reduced agglutinate formation in both the Coombs test and the coagglutination test. The inhibitors of agglutination lattice formation are apparently serum beta-lipoproteins which become attached to the Brucella antigen and can be removed from the serum by treatment with MnCl2-heparin.