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Thirty-five members of a family affected with von Hippel-Lindau's disease (VHL) were examined and lesions were found in nine of them. Four of the lesions were located in the central nervous system; two cerebellar, one spinal, one cerebral. Eye lesions were present in eight of the nine patients and complications developed in seven; six unilateral and one bilateral blindness. Both mortality and morbidity were found to be higher than in other series. This is attributed to insufficient screening of the patients with familial VHL, the late diagnosis was established only after the onset of complications. The importance of screening tests in VHL risk patients is emphasized. 相似文献
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Jeremy Turk 《Journal of Applied Research in Intellectual Disabilities》1998,11(3):175-191
This study examined whether boys with Fragile X syndrome have a characteristic behavioural profile associated with the underlying genetic abnormality. Parents of 49 boys with Fragile X syndrome, 45 boys with Down's syndrome and 42 boys with intellectual disabilities of unknown aetiology were interviewed. Measures included the Childhood Behaviour Checklist, parent and teacher versions (CBCL), Parental Account of Childhood Symptoms (PACS) and Vineland social age and social quotient derived from the MRC Schedule of Handicaps, Behaviour and Skills (HBS). 相似文献
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Okan Bilge Yelda Pinar Mehmet Asim Ozer Figen Govsa 《Journal of plastic, reconstructive & aesthetic surgery》2007,60(10):1120-1126
The lumbrical muscles are located in the midpalm, dorsal to the palmar aponeurosis. The main function of these muscles is an indirect contribution to interphalangeal joint extension by decreasing the flexor effect of the flexor digitorum profundus muscle. Due to their minor biomechanical functions and suitable constructions, these muscles have been preferred in reconstructive surgery as local transposition flaps or pedicled flaps. Despite the surgical and clinical importance, vascular anatomical studies of these muscles are not well represented in the current literature. This study was performed in the Department of Anatomy of the Faculty of Medicine of the Ege University. Thirty-four cadaver hands, injected with red-coloured latex were used, and we aimed to describe the morphometry and vascular anatomy of the lumbrical muscles. We measured the length and width of the muscles, after removing their epimisium, and the diameter and length of the arteries to the muscles. The outcomes of our study determined that the length and width of the lumbrical muscles were reduced significantly from radial towards ulnar sides. The lumbrical muscles were supplied from both their palmar and dorsal surfaces by both superficial and deep palmar arches and/or their branches. We also described the level of entry of the dominant arteries for each lumbrical muscle and measured the size of the vessels and muscles to guide some surgical approaches. This anatomical study could guide for some surgical approaches and reduce the deficiency about the vascular anatomical patterns of the lumbrical muscles in the literature. 相似文献
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A CMEC which occurred within the right maxillary canine-third molar region of a 34-year-old male was presented. The clinical symptoms were swelling and pain. Radiologically the lesion looked like an ameloblastoma or residual cyst. Hemimaxillectomy was the treatment. No evidence of recurrence has been observed for ten months after the operation. 相似文献
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Bilge Türk Bilen Hidir Kilin? Nezih Alaybeyoglu Mehmet Celik Mustafa Iraz Nurzen Sezgin Ahmet Gültek 《Nordisk plastikkirurgisk forening [and] Nordisk klubb for handkirurgi》2006,40(2):73-78
Oxygen-derived free radicals have been implicated in the pathogenesis of tissue injury after ischaemia-reperfusion. Caffeic acid phenethyl ester (CAPE), an active ingredient of honeybee propolis, has been identified as having potent antioxidant and anti-inflammatory properties. We evaluated the ability of CAPE applied intraperitoneally in reducing tissue injury after ischaemia-reperfusion. To investigate whether treatment with CAPE modifies the concentrations of the endogenous indices of oxidant stress, we examined its effects on a model of flap ischaemia-reperfusion injury in rats. CAPE (10 micromol/kg) was given through the peritoneum before reperfusion. CAPE given intraperitoneally had an inhibitory effect on tissue injury after ischaemia-reperfusion comparable to that of a control group. The anti-inflammatory and antioxidant properties of CAPE may contribute to its suppression of tissue injury. 相似文献