ABSTRACT
In recent years, we have considerably widened our knowledge of the pathophysiology of sepsis and some procedures, aiming both to relieve symptoms and control the inflammation/coagulation reaction, have proven to be effective in increasing survival. This improves when mechanical ventilation is applied with low tidal volumes, fluid replacement and the use of cardioactive drugs are titrated on the oxygen saturation of hemoglobin in the central venous system and blood glucose does not exceed certain limits. It is also evident that inflammation and coagulation are closely related to each other. The inhibition of only one pathway, such as the inhibition of inflammation with high dosage steroids or the inhibition of coagulation with antithrombin, does not produce a survival improvement. The only molecule which has proven to be notably effective in reducing mortality is Activated Protein C interacting on coagulation/fibrinolysis, as well as on inflammation processes. Multinodal modulation of several interdependent processes may be the probable reason for the proven effectiveness of this treatment.
Subject(s)
Systemic Inflammatory Response Syndrome , Blood Proteins/physiology , Humans , Models, Biological , Multiple Organ Failure/etiology , Protein C/therapeutic use , Recombinant Proteins/therapeutic use , Systemic Inflammatory Response Syndrome/classification , Systemic Inflammatory Response Syndrome/epidemiology , Systemic Inflammatory Response Syndrome/metabolism , Systemic Inflammatory Response Syndrome/physiopathology , Systemic Inflammatory Response Syndrome/therapyABSTRACT
BACKGROUND: von Hippel-Lindau disease (VHL) is a hereditary cancer syndrome in which affected individuals are at risk of developing tumors in multiple organs, including eyes, cerebellum, spinal cord, kidneys, inner ear, adrenal glands and pancreas. METHODS: We performed a fundus examination and fluorescein and indocyanine green (ICG) angiography in both eyes of a young woman affected by VHL with bilateral pheochromocytoma, retinal angioma, retinal microaneurysms and unusual alterations of the deep retinal layers. A molecular analysis of the VHL gene was carried out. RESULTS: Ophthalmoscopy disclosed in her right eye a small retinal hemangioma, some microaneurysms in both eyes at the posterior pole and multiple, small, whitish, dome-shaped lesions scattered in the retinal pigment epithelium (RPE) of the posterior retina. Fluorescein angiograms revealed in the early phase multiple hyperfluorescent spots that showed progressive discoloration in the late phase of angiography. Some of these spots were ophthalmoscopically undetectable. The late phase of ICG angiography showed some small hyperfluorescent points located at the level of the RPE, and some of them corresponded to the hyperfluorescent spots seen on fluorescein angiography. The molecular analysis revealed the presence of a "missense" mutation of the VHL gene at nucleotide 683. CONCLUSIONS: Alterations in the RPE have never been observed in the VHL syndrome. We describe an unusual case of VHL with a capillary hemangioma associated to diffuse alterations with the RPE of the posterior retina. The possibility exists that these lesions form part of the eye modifications in VHL.
Subject(s)
Adrenal Gland Neoplasms/genetics , Genes, Tumor Suppressor/genetics , Hemangioma, Capillary/genetics , Ligases , Mutation, Missense , Pheochromocytoma/genetics , Proteins/genetics , Retinal Neoplasms/genetics , Tumor Suppressor Proteins , Ubiquitin-Protein Ligases , von Hippel-Lindau Disease/genetics , Adrenal Gland Neoplasms/pathology , Adult , Blotting, Southern , DNA, Neoplasm/genetics , Female , Fluorescein Angiography , Fundus Oculi , Genetic Markers , Hemangioma, Capillary/pathology , Humans , Indocyanine Green , Pheochromocytoma/pathology , Polymerase Chain Reaction , Retinal Neoplasms/pathology , Von Hippel-Lindau Tumor Suppressor Protein , von Hippel-Lindau Disease/diagnosisABSTRACT
BACKGROUND: The effect of external beam radiation therapy (teletherapy) on the choroidal circulation is poorly known. Eyes irradiated with teletherapy represent a good model to study, without confounding factors, the pathophysiologic and clinical aspects of radiation-induced chorioretinal damage. This study used fluorescein and indocyanine green choroidal angiography to investigate the late effects of external eye irradiation on the choroidal circulation. METHODS: Fluorescein angiography and indocyanine green choroidal videoangiography were performed on patients with radiation retinopathy because of external eye irradiation for orbital and paranasal sinus malignancies. Patients were divided into two groups according to the treatment field (anterior unilateral or bilateral). RESULTS: Indocyanine green angiograms showed areas of choriocapillaris hypoperfusion in all eyes-unilateral or bilateral irradiation-affected by radiation retinopathy. Late indocyanine green choroidal staining was found in five eyes (28%) of the patients who received unilateral anterior irradiation. In the same group, nine eyes (52%) had signs of choroidal precapillary occlusion and four eyes (23%) had rubeosis iridis without retinal neovascularization. One case of subfoveal choroidal neovascularization was documented in the bilateral irradiation group. CONCLUSIONS: Radiation side effects are not limited to the retinal vessels but also involve choroidal circulation. The damage to the choroid is primarily vascular, and its clinical aspects depend on the treatment fields. Anterior irradiation may be a critical factor for the appearance of unusual rubeosis iridis and neovascular glaucoma.
