Churg-Strauss syndrome is a rare disorder characterized by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. Vasculitis commonly affects the lungs, skin and peripheral nervous system. Outcome and long-term survival is usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides.
OBJECTIVE -To test the hypothesis that a reduction of somatostatin (SST) in the retina exists in patients without clinically detectable diabetic retinopathy and that it is associated with retinal neurodegeneration.RESEARCH DESIGN AND METHODS -Human diabetic postmortem eyes (n ϭ 10) without clinically detectable retinopathy were compared with eyes (n ϭ 10) from nondiabetic donors. SST mRNA (RT-PCR) and SST-28 immunoreactivity (confocal laser) were measured separately in neuroretina and retinal pigment epithelium (RPE). In addition, SST-28 (radioimmunoassay) was measured in the vitreous fluid. Glial fibrillar acidic protein (GFAP) was assessed by immunofluorescence and Western blot. Apoptotic cells were quantified using transferasemediated dUTP nick-end labeling.RESULTS -A higher expression of SST was detected in RPE than neuroretina in both groups. SST mRNA levels and SST-28 immunoreactivity were significantly lower in both RPE and the neuroretina from diabetic donors compared with nondiabetic donors. These results were in agreement with those obtained by measuring SST-28 in the vitreous fluid of the same donors. Increased GFAP and a higher degree of apoptosis were observed in diabetic retinas compared with nondiabetic retinas. These changes were most evident in patients with the higher deficit of SST.CONCLUSIONS -Underproduction of SST is an early event in the eyes of diabetic patients and is associated with glial activation and neural death. In addition, our results suggest that RPE is an important source of SST in the human eye. The possible role of the lower production of SST in the pathogenesis of diabetic retinopathy requires further investigation.
Diabetes Care 30:2902-2908, 2007D iabetic retinopathy is the most common complication of diabetes and remains the leading cause of new blindness among working-age individuals in developed countries (1). Although diabetic retinopathy has been traditionally viewed as a disorder of retinal vasculature, retinal neurodegeneration may be a primary pathology that gives rise to microvascular changes (2,3). The degenerative changes in the neuroretina include increased apoptosis, glial cell reactivity, microglial activation, and altered glutamate metabolism. When occurring together, these changes may explain some of the functional deficits in vision appearing in diabetes before the onset of vascular abnormalities. In fact, abnormal electroretinograms have been found in patients with type 1 diabetes previous to the development of clinically detectable vascular retinal pathology (4), as well as in rats at short time intervals after the onset of experimental diabetes (5).Somatostatin (SST) is a widely distributed peptide, and its diverse biological functions include neurotransmission and antisecretory and antiproliferative activities (6). SST and its receptors are found in the neuroretina of various species, including humans, and growing evidence suggests that in the retina, SST acts both as a neuromodulator and antiangiogenic factor (7). SST production in the human retina ha...
The early success described in this case report demonstrates the technical and clinical feasibility of transplanting all the tissues of the with all its aesthetic and functional units intact.
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