Phototesting and photoprotection in LE

Photosensitivity and induction of skin lesions following UV radiation is a common problem of patients with cutaneous and systemic forms of lupus erythematosus. The detrimental effect of UV radiation to patients with lupus erythematosus was already recognized in the last century. Skin lesions can now be provoked under standardized conditions allowing the diagnosis and classification of patients with photosensitive disorders. The aim of this review is to give an overview on the history, test procedure and test results in patients with lupus erythematosus

The low energy nuclear mechanics and the independent particle model

The author analizes the independent particle model of the nucleus in its definition as an approximation toweek incoherent interaction and its applicability to general problems of low energy nuclear mechanics. In zero approximation, defined. by a condition of self consistency, the potential wells which determine the states of the single nucleons in the nucleus have a mean depth of the order of twice the mean potential energy per nucleon. It results, for instance, that direct emission and evaporation processes of nucleons have different thresholds. It is emphasized that the optical model for nuclear reactions can be directly derived from the model considered

The health state preferences and logistical inconsistencies of New Zealanders: a tale of two tariffs

Notwithstanding the proposed use of Cost-Utility Analysis (CUA) to inform health care priority setting in New Zealand, to date there has been no research into New Zealanders’ valuations of health-related quality of life. This paper reports the results of a study of the health state preferences of adult New Zealanders generated from a postal survey to which 1360 people responded (a 50% response rate). The survey employed a self-completed questionnaire in which a selection of health states were described using the EQ-5D health state classification system and respondents’ valuations were sought using a visual analogue scale (VAS). Close attention is paid to the quality of the data, in particular to the ‘logical inconsistencies’ in respondents’ valuations. Regression analysis is used to interpolate values over the 245 possible EQ-5D states. Two tariffs of health state preferences, arising from contrasting treatments of the logical inconsistencies, are reported.New Zealand, EuroQol, EQ-5D

Kaposi's sarcoma in a patient with erythroblastopenia and thymoma: Reactivation after topical corticosteroids

We report a 69-year-old female with erythroblastopenia and thymoma who developed lesions of Kaposi's sarcoma (KS) after thymectomy, 2 months after the initiation of therapy with methylprednisolone. Control of mucocutaneous KS lesions was obtained with radiotherapy, interferon alfa-2b and withdrawal of systemic immunosuppressive therapy. Erosive oral lichen planus appeared later, and after therapy with topical corticosteroids a new lesion of KS developed that regressed after withdrawal of topical corticosteroids. The detection of HHV-8 only in lesional skin supports the hypothesis that this virus can trigger the development of KS lesions

FMRP sustains presynaptic function via control of activity-dependent bulk endocytosis

Synaptic vesicle (SV) recycling is essential for the maintenance of neurotransmission, with a number of neurodevelopmental disorders linked to defects in this process. Fragile X syndrome (FXS) results from a loss of fragile X mental retardation protein (FMRP) encoded by the FMR1 gene. Hyperexcitability of neuronal circuits is a key feature of FXS, therefore we investigated whether SV recycling was affected by the absence of FMRP during increased neuronal activity. We revealed that primary neuronal cultures from male Fmr1 knock-out (KO) rats display a specific defect in activity-dependent bulk endocytosis (ADBE). ADBE is dominant during intense neuronal activity, and this defect resulted in an inability of Fmr1 KO neurons to sustain SV recycling during trains of high-frequency stimulation. Using a molecular replacement strategy, we also revealed that a human FMRP mutant that cannot bind BK channels failed to correct ADBE dysfunction in KO neurons, however this dysfunction was corrected by BK channel agonists. Therefore, FMRP performs a key role in sustaining neurotransmitter release via selective control of ADBE, suggesting intervention via this endocytosis mode may correct the hyperexcitability observed in FXS. SIGNIFICANCE STATEMENT Loss of fragile X mental retardation protein (FMRP) results in fragile X syndrome (FXS), however whether its loss has a direct role in neurotransmitter release remains a matter of debate. We demonstrate that neurons lacking FMRP display a specific defect in a mechanism that sustains neurotransmitter release during intense neuronal firing, called activity-dependent bulk endocytosis (ADBE). This discovery provides key insights into mechanisms of brain communication that occur because of loss of FMRP function. Importantly it also reveals ADBE as a potential therapeutic target to correct the circuit hyperexcitability observed in FXS