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Endobronchial telangiectasias and hemoptysis in scleroderma.

Hemoptysis is considered a rare event in scleroderma and to date only two previous cases could be identified. The occurrence of hemoptysis with bleeding and friable telangiectasias is reported in a patient with rapidly progressing systemic sclerosis. This represents the first report of this association, although bleeding telangiectasias have been reported in other systems. A brief review of the relevant literature is included.

Bronchi↗

COATS' DISEASE.

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Exudates and Transudates↗

Rhegmatogenous retinal detachment with retinal telangiectasia.

A 34-year-old man with unilateral retinal telangiectasia developed a bullous retinal detachment. A horseshoe retinal tear was found at 12 o'clock. The detachment resolved with placement of an encircling scleral buckle. The prompt and permanent resolution of subretinal fluid supported our belief that this was a rhegmatogenous retinal detachment.

Adult↗

Abnormal glucose metabolism and parafoveal telangiectasia.

Parafoveal telangiectasia is a microvascular abnormality of the macula that may be developmental or acquired. Twenty-eight patients with this condition and normal fasting blood glucose levels underwent ophthalmologic evaluation and glucose tolerance testing. Bilateral telangiectasia was more frequently associated with laboratory evidence of abnormal glucose metabolism than unilateral disease (five of eight cases vs six of 17 cases). In five of our patients, results of glucose tolerance testing were consistent with diabetes even though fasting blood glucose levels were normal. Right-angle venules, which have been reported to be an important sign of bilateral disease, were also found in unilateral telangiectasia. Unilateral telangiectasia occurs in females as well as males. Glucose tolerance testing should be performed in all patients who have bilateral parafoveal telangiectasia.

Adult↗

Vascular lesions of the intestines.

This article reviews the pathology, pathophysiology, diagnosis, and treatment of vascular lesions of the intestine as a cause of gastrointestinal bleeding. In addition, a modified system for classifying such lesions, based on the author's personal experience with such lesions, is presented.

Arteriovenous Malformations↗

DNA repair enzymes in ataxia telangiectasia and Bloom's syndrome fibroblasts.

Ataxia telangiectasia, Bloom's syndrome and normal fibroblasts were compared as to the capacity of their cellular extracts to enhance the priming activity of gamma-irradiated colicin E1 DNA for purified DNA polymerase. It was found that an ataxia strain had substantially lower, and a Bloom's syndrome strain had slightly lower capacity than a normal strain; while the activities of apurinic site specific endonuclease in these extracts were comparable.

Ataxia Telangiectasia↗

Argon laser therapy for cutaneous lesions.

This paper presents a review of the results of treatment of a series of cutaneous vascular lesions with the argon laser. The lesions were evaluated before treatment, both clinically and histologically. Argon laser therapy appears to be most effective for purple port wine stains on the faces of adults and telangiectatic lesions involving the nose and forehead. Complications seem to be particularly associated with its use in children and about the upper lip and chin.

Adolescent↗

Sclerotherapy for venous flare.

The aetiology of venous flare is discussed and a method of microsclerotherapy described. Visualisation of the injection of the sclerosant has been seen to be helped by the addition of a gelatin solution.

Capillaries↗

Copper vapour laser treatment of port-wine stains and other vascular malformations.

Over a 2-year period more than 1350 treatment sessions have been conducted with yellow 578 nm light from a copper vapour laser on a variety of vascular malformations. Of these sessions half were of port-wine stains, from 297 patients. The light is applied by scanning a 1 mm optical fibre approximately 2 mm above the lesion along the lines of Langer. We use a maximum scan rate of 3 s/cm2, which is the highest rate at which minimal blanching can be produced. Up to 6.5 watts of light has been used, and a typical energy fluence is 20 to 30 J/cm2. Topical melanin suppressing creams are used before and after treatment, in order to maximise the effectiveness of the treatment. Non-vasoconstricting anaesthetics are always used. Repeat treatments are 3 months apart. The scan rate we used was directly correlated with the light intensity. The degree to which the colour of the port-wine stains lightened after the first treatment was also well correlated with the scan rate (p less than 0.0005). The faster the scan rate, the greater the colour reduction. This provides some supporting evidence for the short illumination time that theoretical models have indicated as "ideal". The lightening was more marked for patients whose lesion colour was initially dark (p less than 0.0005). Those whose skin had a high melanin content (Maori, Polynesian and Asian) responded more slowly than others. Thirty-six patients have had a greater than 70% reduction in the size of their lesions after an average of four treatments. Ten patients have responded poorly after at least four treatments (10% of all those who have had four or more treatments). The occurrence of scarring, hyperpigmentation and hypopigmentation was low in comparison with that reported previously from the green light of argon ion lasers.

Adolescent↗

Characterization of a polypeptide associated with coated vesicles and the cytoskeleton which is recognized by a CREST serum.

Serum from an individual with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dismotility, sclerodactyly, telangiectasia) reacts not only with kinetochores, but also with a cytoplasmic, phosphorylatable polypeptide, which is shown by immunofluorescence in whole cells and immunoelectronmicroscopy in sections to be associated with actin stress fibres in cultured mammalian cells. The antigen shows some variation in molecular weight between species, estimated by immunoblotting to range from 68 to 76 kD between mouse, Chinese hamster, sheep and human cells. Much of the polypeptide copurifies with coated vesicles, of which approx. 5% bound antibody from the serum, as detected by immunogold electronmicroscopy.

Actins↗

Neuroendocrine cell proliferations in gastric antral vascular ectasia.

Gastric antral vascular ectasia is a recently recognized cause of chronic gastrointestinal blood loss. Immunohistochemical and electron-microscopic studies were performed on the surgically resected antrum of a patient with antral vascular ectasia. These revealed extraepithelial and intraepithelial neuroendocrine cell proliferations. Staining for a wide range of neural and endocrine markers demonstrated that these cells contain large quantities of 5-hydroxytryptamine and vasoactive intestinal polypeptide. Gastric vascular ectasia is of unknown etiology but has been associated with achlorhydria and liver cirrhosis. The findings suggest that locally released neurotransmitters may be responsible for local vasodilation and hence a propensity to bleed.

Aged↗