FACT - The First G-APD Cherenkov Telescope: Status and Results

The First G-APD Cherenkov telescope (FACT) is the first telescope using silicon photon detectors (G-APD aka. SiPM). It is built on the mount of the HEGRA CT3 telescope, still located at the Observatorio del Roque de los Muchachos, and it is successfully in operation since Oct. 2011. The use of Silicon devices promises a higher photon detection efficiency, more robustness and higher precision than photo-multiplier tubes. The FACT collaboration is investigating with which precision these devices can be operated on the long-term. Currently, the telescope is successfully operated from remote and robotic operation is under development. During the past months of operation, the foreseen monitoring program of the brightest known TeV blazars has been carried out, and first physics results have been obtained including a strong flare of Mrk501. An instantaneous flare alert system is already in a testing phase. This presentation will give an overview of the project and summarize its goals, status and first results

Hereditary sensory and autonomic neuropathies: types II, III, and IV

The hereditary sensory and autonomic neuropathies (HSAN) encompass a number of inherited disorders that are associated with sensory dysfunction (depressed reflexes, altered pain and temperature perception) and varying degrees of autonomic dysfunction (gastroesophageal reflux, postural hypotention, excessive sweating). Subsequent to the numerical classification of four distinct forms of HSAN that was proposed by Dyck and Ohta, additional entities continue to be described, so that identification and classification are ongoing. As a group, the HSAN are rare diseases that affect both sexes. HSAN III is almost exclusive to individuals of Eastern European Jewish extraction, with incidence of 1 per 3600 live births. Several hundred cases with HSAN IV have been reported. The worldwide prevalence of HSAN type II is very low. This review focuses on the description of three of the disorders, HSAN II through IV, that are characterized by autosomal recessive inheritance and onset at birth. These three forms of HSAN have been the most intensively studied, especially familial dysautonomia (Riley-Day syndrome or HSAN III), which is often used as a prototype for comparison to the other HSAN. Each HSAN disorder is likely caused by different genetic errors that affect specific aspects of small fiber neurodevelopment, which result in variable phenotypic expression. As genetic tests are routinely used for diagnostic confirmation of HSAN III only, other means of differentiating between the disorders is necessary. Diagnosis is based on the clinical features, the degree of both sensory and autonomic dysfunction, and biochemical evaluations, with pathologic examinations serving to further confirm differences. Treatments for all these disorders are supportive

Expert committee recommendations for acne management.

In 2003, an international committee of physicians and researchers in the field of acne, working together as the Global Alliance to Improve Outcomes in Acne, developed consensus guidelines for the treatment of acne. These guidelines were evidence based when possible but also included the extensive clinical experience of this group of international dermatologists. As a result of the evaluation of available data and the experience, significant changes occurred in the management routines for acne. The greatest change arose on the basis of improved understanding of acne pathophysiology. The recommendation now is that acne treatments should be combined to target as many pathogenic factors as possible. A topical retinoid should be the foundation of treatment for most patients with acne, because retinoids target the microcomedo, the precursor to all acne lesions. Retinoids also are comedolytic and have intrinsic antiinflammatory effects, thus targeting 2 pathogenic factors in acne. Combining a topical retinoid with an antimicrobial agent targets 3 pathogenic factors, and clinical trials have shown that combination therapy results in significantly faster and greater clearing as opposed to antimicrobial therapy alone. Oral antibiotics should be used only in moderate-to-severe acne, should not be used as monotherapy, and should be discontinued as soon as possible (usually within 8-12 weeks). Because of their effect on the microcomedo, topical retinoids also are recommended as an important facet of maintenance therapy

Hormonal Treatment of Acne and Hidradenitis Suppurativa in Adolescent Patients.

Homeostasis of the cutaneous microenvironment is complex and depends on multiple intrinsic and extrinsic factors, including the influence of hormones. Hormones exert action on the hair follicle and associated sebaceous glands via both endocrine and intracrine mechanisms. The profound impact of hormonal action on follicular homeostasis can be leveraged in the treatment of disorders, such as acne and hidradenitis suppurativa. The clinician must have an intimate knowledge of the rationale for use, mechanism of action, and possible side effects of hormonal therapy when using these agents to treat adolescents with cutaneous disease

Congenital self-healing reticulohistiocytosis with eye involvement.

Congenital self-healing reticulohistiocytosis (CSHR) represents the benign end of the spectrum of Langerhans cell histiocytoses, with spontaneous resolution of lesions within the first year of life. However, involvement of organ systems other than the skin has been described occasionally and recurrence of disease at sites distant from the skin has been documented. We report a case of CSHR with eye involvement that spontaneously resolved concurrent with resolution of skin lesions. Because multiple organ systems can be involved and recurrences are possible, long-term follow-up of these patients is indicated

Neutrophilic eccrine hidradenitis: a case report of an unusual annular presentation.

Neutrophilic eccrine hidradenitis (NEH) is an acute, self-limited, inflammatory neutrophilic dermatosis. It is most commonly described in patients with acute myelogenous leukemia (AML) following chemotherapy. NEH generally presents as erythematous papules and plaques on the head, trunk, and extremities. Histologically, NEH is characterized by a neutrophilic infiltrate surrounding eccrine ducts and coils with occasional necrosis. We describe a case of NEH with an unusual presentation of annular plaques. A search of the literature revealed only one other case report of NEH presenting as an annular eruption