Subclinical course of adult visceral Niemann–Pick type C1 disease. A rare or underdiagnosed disorder?

Summary  We present the third case of Niemann–Pick disease type C without neurological symptoms. The patient was a 53-year-old woman without significant prior health problems who died of acute pulmonary embolism. Autopsy findings of hepatosplenomegaly, lymphadenopathy and ceroid-rich foam cells raised the suspicion of the visceral form of acid sphingomyelinase deficiency (Niemann–Pick disease type B; NPB) or a much rarer disorder, variant adult visceral form of Niemann–Pick disease type C (NPC). To verify the histopathological findings, SMPD1, NPC1 and NPC2 genes were analysed. Two novel sequence variants, c.1997G>A (S666N) and c.2882A>G (N961S) were detected in the NPC1 gene. No pathogenic sequence variants were found either in the SMPD1 gene mutated in NPB or in NPC2 gene. The pathogenicity of both NPC1 variants was supported by their location in regions important for the protein function. Both variations were not found in more than 300 control alleles. Identified sequence variations confirm the diagnosis of the extremely rare adult visceral form of Niemann–Pick disease type C, which is otherwise dominated by neurovisceral symptoms. Although only three patients have been reported, this (most probably underdiagnosed) form of NPC should be considered in differential diagnosis of isolated hepatosplenomegaly with foam cells in adulthood. Electronic supplementary material  Supplementary material is available for this article at     

Peripheral Vascular Complication from Coronary Angiography: a Case Report of Cholesterol Embolization

A 65-year-old man developed acute limb ischemia, severe abdominal wall and lower limb livedo reticularis following a coronary angiogram. The differential diagnoses of acute limb ischemia and multiple cholesterol emboli syndrome (MCES) are discussed. This work was performed at Long Island Jewish Medical Center, 270-05, 76^th Avenue, New Hyde Park, NY 11040.     

精神分裂症首次发病患者的磁共振弥散张量显像研究

目的探讨从未用过药的精神分裂症首次发病(以下简称首发)患者的重要白质及部分灰质区的磁共振弥散张量显像(DTI)的特点。方法选取9例首发精神分裂症患者(患者组)及9名年龄、性别、受教育程度与患者组相配对的健康者,应用DTI成像技术检测脑额颞交界处、内囊等白质区和颞中回灰质、中央前回、中央后回等灰质区的各向异性(FA)、表观扩散系数(ADC)及双侧海马体积。结果患者组及对照组组内左右两侧兴趣区FA值及ADC值的差异无统计学意义(P>0.05);患者组与对照组各感兴趣区FA值差异无统计学意义(P>0.05),但患者组颞中回灰质(8.655×10-9)、中央前回(7.816×10-9)、中央后回(7.855×10-9)ADC值高于对照组(分别为7.428×10-9,6.921×10-9,7.013×10-9;P=0.049,0.009,0.005);两组内及组间双侧海马体积比较,差异均无统计学意义(P>0.05)。结论首发精神分裂症患者与健康者DTI参数之间没有明显区别。     

Internal thoracic vessels as recipient vessels for free flap reconstruction in head and neck surgery.

In rare cases the usage of the internal thoracic vessels as recipient vessels in reconstructive surgery of the head and neck region with free tissue transfer is a challenging but valid alternative if local recipient vessels are unusable.     

术中电生理动态监护下显微手术切除椎管神经鞘瘤（附65例报告）

目的 探讨术中电生理监护对椎管神经鞘瘤显微手术中的作用及意义，提高对椎管内神经鞘瘤的治疗水平。方法 回顾性分析65例术中电生理动态监护下，显微手术切除椎管内神经鞘瘤。结果 治愈60例（占92．3％），好转5例（占7．7％），无死亡；肿瘤全切62例，次全切除3例，全切率95．4％。结论 常规动态电生理监测下显微手术切除椎管神经鞘瘤，能保全脊髓神经的功能，减少副损伤，提高手术安全性；显微手术有助于提高肿瘤全切率，可有效减少术后复发。对影响脊柱稳定性的行脊柱融合内固定。     

Wirkung und Wirkfaktoren von psychodramatischem Coaching — Eine experimentelle Evaluationsstudie

Zusammenfassung Diese Studie untersucht, wie Erfolg im Einzelcoaching entsteht und was Psychodrama dazu beitragen kann. Dabei werden zun?chst die vier Berner Wirkfaktoren dargestellt, die im Rahmen einer allgemeinen psychologischen Coachingkonzeption die vier m?glichen Erfolgsstrategien darstellen. über Videoauswertung und schriftliche Befragung aller Beteiligten wurden 35 Einzelsitzungen evaluiert. Dabei zeigten sich die vier Wirkfaktoren tats?chlich als elementar in kurz- und mittelfristig erfolgreichen Coachingprozessen. In dem experimentellen Vergleich wurde das psychodramatische Coaching zudem wesentlich besser bewertet als das Vergleichscoaching, was durch den Ressourcen- und Wachstumsfokus sowie die sehr gute Beziehungskompetenz der Psychodramatiker erkl?rt werden kann. Des Weiteren zeigen die Auswertungen, dass ein dosierter und gezielter Einsatz des psychodramatischen Methodenrepertoires fruchtbarer zu sein scheint als der zeitlich umfassende Einsatz des Psychodramas als Hauptinterventionsmethode.

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Summary The present study investigates the process of success development in one-to-one-coaching and examines how the method of psychodrama can contribute to this process. First the four change factors are presented. They represent the four success strategies in a general psychological coaching approach that can be chosen by the coach. Second 35 one-to-one-coaching sessions are evaluated by a video-based rating-system and questionnaires that were filled in by all coaches, clients and employees of the clients. In these analyses the four change factors were shown to be critical for short- and medium-term success. In an experimentally designed assessment the psychodramatic coachings had a considerably better result than the comparative coaching. This outcome could be explained by the focus on resources and growth and the high competence of the psychodramatic coaches to set up an appreciative coaching relationship. The analyses demonstrate furthermore that the well-aimed and specific use of psychodrama methods is more fruitful than its time-extensive use as the main intervention method.

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Peter Behrendt geb. 1977, Dipl. Psych., ausgebildeter Psychodrama-Therapeut und Mediator, seit 2004 freiberuflich Coaching, Moderation sowie Konzeption und Durchführung von Fortbildung und Trainings. Er konzipierte und leitete die Evaluationsstudie, die diesem Artikel zugrunde liegt.