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PubMed2003

Intrahepatic Osler's disease: report of two cases and review of the literature.

Osler-Weber-Rendu disease is a hereditary vascular disease with multiple manifestations. The liver is involved rarely. Vascular abnormalities include telangiectasis and arteriovenous fistulas, sometimes associated with fibrosis and cirrhosis. Hepatic arteriovenous shunting may include secondary portal hypertension, reduced liver function and high cardiac output. Two cases of Osler-Weber-Rendu disease with extensive hepatic arteriovenous fistulation were described in detail and we report on their clinical features. In the first patient, treatment was symptomatic since liver transplantation is not indicated because the patient was asymptomatic. Embolization treatment of hepatic arteries was indicated in the second patient because he had biliary disease and recurrent cholangitis secondary to vasculo-biliary shunts. Therapy with arterial embolization, banding, or ligature of hepatic arteries is still limited and provides unsatisfactory long-term results. Liver transplantation offers now another therapeutic option for patients with intrahepatic high shunting and secondary pulmonary hypertension.

Adolescent↗

PubMed2004

Chronic venous diseases: roles of various pathophysiological factors.

Disturbances in haemodynamic, biochemical and enzymatic factors have been observed in chronic venous diseases (CVD). These changes lead to the development of varices, telangiectasies and skin disorders. They affect vessels, blood, skin tissues and cells. It is now possible to describe their time course and interdependance of these changes. Orthostatism pressure on vein wall may lead to fluid leakage and oedema, these resulting in vein enlargement. These processes may be further influenced by genetic or acquired risk factors. Skin microvessels suffer more from hypoxia than from hypertension. Indeed, hypoxia affects not only endothelial cells, but also red and white blood cells and modifies particularly, but not exclusively, TGF-beta1 production. This substance is, an important modulator of zinc dependent-metallo-proteinases and their tissue inhibitor of metallo-proteinases (TIMP) in the skin. Imbalance in this enzymatic system seems to lead either to sclerosis or ulcer. Of course, other biochemical events (also in this review) play a role in vessel wall and skin deterioration in CVD. The aim of the present review is to assess the role of pathophysiological factors in CVD and the influence of different therapies, including the venotropic agent calcium dobesilate, on some of these haemodynamic or biochemical aspects.

Capillaries↗

PubMed2004

[Treatment of the keratinizing skin disorder, psoriasis, by topical vitamin D3].

The active vitamin D(3) regulates proliferation and differentiation of epidermal keratinocytes. Although topical application of steroid has been principal therapy for psoriasis, there occurs some side effects such as skin atropy, telangiectasis, and purpura. Recently topical vitamin D(3), tacalcitol, calcipotriol, and maxacalcitol, which avoid these side effects, are widely used for psoriasis. In this review, we discussed the mechanism, effect, and side effect of topical vitamin D(3) for psoriasis.

Administration, Topical↗

PubMed2005

[A case of Osler-Weber-Rendu syndrome: therapeutic embolization of the pulmonary artery and bronchial artery].

We encountered a case in which the symptoms of hypoxia and hemoptysis improved after performing repeated pulmonary artery embolizations and bronchial artery embolization. A 22-year-old man was hospitalized in our institution with high fever, headache and unconsciousness in May of 2001. Subendocranial abscess was diagnosed, so we performed antibiotic therapy and drainage, but at that time he showed symptoms of hypoxia. We diagnosed Osler-Weber-Rendu syndrome because of multiple small nodular shadows in his chest CT, multiple arteriovenous fistulae in both lungs on pulmonary arteriography, and telangiectasis. We performed pulmonary artery embolization 6 times, and his hypoxia and right to left shunts improved. In the course of our therapy, hemoptysis appeared and dilatation of vessels was detected in the left bronchial arteriography, so we performed embolization of the left bronchial artery and the symptom improved. We concluded that we should take care to detect not only pulmonary arterial lesions but also bronchial arterial leisions in the diagnosis and therapy of respiratory symptoms of Osler-Weber-Rendu syndrome.

Adult↗

PubMed2005

[ATM expression in K562 and SiHA cell lines in relation to their cell cycle restardation after gamma-irradiation].

Ataxia telangiectasis is caused by the mutation of AT gene (ATM) and it is characterized by hypersensitivity to the radiation. In order to investigate the relationship between ATM mRNA expression of K562 and SiHA two kinds of tumor cell lines and their cell cycle restardation after gamma-irradiation, their ATM mRNA expressions were measured by semi-quantitive RT-PCR and the cells were irradiated at the dose of 6, 10 and 15 Gy of (60)Co gamma ray and the change of the apoptosis and cell cycle arrest phenomenon were observed at the time of 6, 12, 24, 48 and 60 hours after irradiation. The results showed that the ATM mRNA relative expression level of K562 cell line was 0.04, that of SiHA cell line was 0.80, the ATM transcript levels in SiHA cells were 20 times as much as that in K562. In conclusion, the G(2)/M phase restardation after irradiation was observed in both cell lines, whereas SiHA exhibited a much stronger cell cycle restardation, a self-protection function, than that of K562.

Apoptosis↗

PubMed2005

[Castleman disease of the inter-lobar lymph node origin].

We report a case of Castleman disease which originated from the inter-lobar lymph node, with a review of literatures. A 19-year-old woman complaining of cough was pointed out to have an abnormal shadow in the left lung field on chest X-ray. Chest computed tomography (CT) and magnetic resonance imaging (MRI) with enhancement revealed a homogeneous mass lesion at the left inter-lobar portion of the lung. Bronchoscopic findings demonstrated mucosal telangiectasis of the left lower bronchus. We performed the usual axillary thoracotomy and succeeded in extirpation of the tumor without large amount of bleeding. The tumor was elastic and hard, and 70 x 55 x 45 mm in size. Her postoperative course was uneventful and she was discharged on the 12th postoperative day.

Adult↗

PubMed1975

Hereditary hemorrhagic telangiectasia. A case with hepatocellular carcinoma and acquired hepatocerebral degeneration.

A patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease), treated with ethinyl estradiol, multiple blood transfusions, and iron-dextran, developed hepatocellular carcinoma and acquired hepatocerebral degeneration. In addition to the carcinoma, the liver contained extensive arteriovenous maliformations, telangiectasis, and changes of Osler atypical cirrhosis. The carcinoma possibly had its genesis in the presence of an ocongenic serum hepatitis virus, or the cirrhosis, or both.

Aged↗

PubMedDate not supplied

[Severe upper gastrointestinal hemorrhage of a rare cause: aorto-esophageal fistula successfully treated].

Aorto-esophageal fistula is a rare entity, determining huge digestive bleeding with a very poor prognostic most often. We present the case of a patient with aorto-esophageal fistula of unknown origin, treated successfully in the Center of Surgery and Liver Transplantation from Fundeni Clinical Institute. A young man, 21 years old, was admitted in our clinic from another hospital for repetitive severe upper gastrointestinal bleeding, with hematemesis and melena, pale, asthenia, without any pain; the bleeding stopped when he was admitted. The upper digestive endoscopy and esophago-gastro-duodenal radioscopy with barium did not show any lesion. During the 7th day the patient presents sudden massive hematemesis with hemodynamic instability; during the surgery we found two subcardial ulcers (stress ulcers?) for which we have done excision and suture which temporary stopped the bleeding. After 14 days, the patient had another massive bleeding; the upper endoscopy shows 28 cm far from the dental arch a protrusive formation of 6-7 mm with a telangiectasis aspect and with a white spot (possibly a central ulceration); 1 ml pure alcohol was injected inside it with temporary bleeding ceasing. After 3 days the patient is bleeding again, with marked decrease in blood pressure; a Blakemore tube stopped the bleeding until surgery was performed. During the intervention an aorto-oesophageal fistula is detected; we made the excision of the fistula with simple suture of the aorta, subtotal esophagectomy, cervico-stoma and gastrostomy, with good postoperative evolution. After 4 months the digestive transit is restored by esophagoplasty with tubulized stomach placed behind the sternum. The diagnostic and treatment difficulties encountered in these kind of cases need to consider also an aorto-esophageal fistula diagnosis, especially for the cases with Chiari triad (mild thoracic pain, sentinel digestive bleeding and exsanguination). The Blakemore tube can save patient's life by ensuring temporary hemostasis and allowing the time for a definitive surgical intervention. The subtotal esophagectomy with cervicostoma and feeding gastrostomy, followed after few months by a esophageal reconstruction , is a solution for this extreme severe cases.

Adult↗

PubMed2006

[A case of multiple pulmonary arteriovenous fistulae associated with hereditary hemorrhagic telangiectasia that deteriorated during pregnancy].

A 29-year-old woman was admitted to our hospital because of persistent breathlessness on exertion after the delivery of her second child. Although at the age of 26 she had been given a diagnosis of multiple pulmonary arteriovenous fistula (PAVF), treatment was not done because of the absence of symptoms. An intrauterine growth retardation (IUGR) due to hypoxemia occurred during the second pregnancy, and then she delivered a 1,283g baby by cesarean section in the 36th gestation week. Her symptoms and hypoxemia improved after transcatheter embolization. Hereditary hemorrhagic telangiectasia was diagnosed on the basis of recurrent epistaxis since her childhood, pulmonary and hepatic vascular abnormality (PAVF and hepatic arterioportal shunt) and telangiectasis of the buccal mucosa and tongue. We should consider an early treatment of PAVF for young female because pregnancy may induce increase of arteriovenous shunt, fatal hemoptysis, infertility, miscarriage and IUGR.

Adult↗

PubMed2006

Topical steroid abuse: its use as a depigmenting agent.

This study was undertaken to document prevalence, motives and observed complications of steroid use as a depigmenting agent amongst African blacks in southeast Nigeria. This practice is very common in the African environment. Consecutive new patients attending the dermatology clinic of the University of Nigeria TeachingHospital, Enugu, from June to December 2004 were recruited. Active substances of products used were determined from packages, while unknown concoctions were analyzed. Chi-squared and Fischer tests were used for statistical analysis, with a significant threshold fixed at 5%. Females aged 18-69 years accounted for 75% (414) of patients. Main topical steroids used by both women and men were class-1 steroids, and these were often compounded with other bleaching products. Median duration of usage was 9 years +/- 1.3. Disorders observed included steroid-induced acne (45.3%), macular hyperpigmentation of face (37.2%), mycoses (40.4%), striae (28.3%), telangiectasis (21.3%), hypertrichosis (13.9%) and diabetes mellitus (2.1%). Duration of utilization of these topical steroids was significantly associated with severe local and systemic consequences, while withdrawal of the offending steroids usually resulted in severe withdrawal dermatitis that was unpleasant to patients. This may suggest that discontinuation is impossible.

Administration, Topical↗

PubMed1991

[Massive hemothorax: an exceptional complication of Rendu-Osler-Weber disease].

Hereditary familial telangiectasis is a vascular dysplasia characterized by a triple syndrome: hereditary character, telangiectasias and hemorrhages. Its association with systemic and/or pulmonary arterio-venous fistulae is frequent and can condition several complications: cerebral abscess, digestive hemorrhages, epistaxis, hemoptysis, hypoxemia, polyglobulia, and rarely the appearance of hemothorax. We describe a patient who was admitted in hospital with a hypovolemic shock secondary to a massive hemothorax; during emergency surgery, which was necessary to perform, a pulmonary A-V fistula was observed in the middle lobe. The studies afterwards performed demonstrated that it was a Rendu-Osler-Weber disease with duodenal, hepatic, and cerebral involvement.

Adult↗

PubMed1991

[Corticosteroid for hemangioma in infants and young children].

500 infants and young children suffering from hemangioma treated by corticosteroid (Prednisone) are reported. This disorder is most commonly seen in the head and neck (64.2%). Clinically there are 3 types of hemangioma: intradermal (34.1%), subdermal (11.3%), and mixed (51.5%). Our patients were treated with steroid (Prednisone 20-40 mg/QOD at 8 am). All patients were responsive. In 411 evaluated patients, 151 (36.7%) gave CR, 83 (20.1%) gave CR with residual telangiectasis, 172 (41.5%) gave PR, and in 5 patients, the lesion remained or slightly faded. During and within 3 years of treatment, none developed undesirable side effects, though the treatment was protracted to 2 years.

Child, Preschool↗

PubMed1990

Defective DNA topoisomerase II in ataxia-telangiectasia cells.

A number of characteristics in the human genetic disorder ataxia-telangiectasia are compatible with an alteration to chromatin structure or the recognition of that structure by an enzyme or DNA binding protein. We describe here reduce activity of DNA topoisomerase type II in a number of Epstein Barr Virus-transformed ataxia-telangiectasia lymphoblastoid cell lines. Enzyme activity was reduced 10-fold or greater in 4 out of 5 cell lines compared to controls. In the remaining cell line approximately a 2-3 fold reduction was evident in partially purified extracts. DNA topoisomerase type I activity was found to be the same as controls in all the cell lines. Northern blot analysis revealed that the same level of DNA topoisomerase II mRNA was expressed in ataxia-telangiectasia and control cell lines. The size and amount of the enzyme did not differ appreciably from that observed in control cells. The reduced activity of DNA topoisomerase II in ataxia-telangiectasis cells might be explained by amino acid substitutions, small deletions in DNA or by a defect in post-translational modification in these cells.

Ataxia Telangiectasia↗

PubMed1990

Methods for the assessment of the effects of topical retinoic acid in photo-ageing and actinic keratoses.

Quantitative techniques for assessing the effects of tretinoin on photo-aged skin mainly involve humans, although hairless mice were used in initial studies. Ideally techniques should be non-invasive but occasionally biopsies have to be taken, especially when studying the effects of tretinoin on different skin compartments. Characteristic features of photo-aged skin, including the development of fine and coarse wrinkles, skin discoloration, rosy cheeks and telangiectasis, have been assessed subjectively using a visual analogue scale. Effects of tretinoin on wrinkle depth have been measured non-invasively by quantifying contours of silicone rubber replicas using either a mechanical tracking device or an optical technique employing an image-analysing computer. Changes in skin colour have been measured using an erythema meter and the stimulatory effect of tretinoin on blood flow has been established by laser doppler flowmetry. Skin thickness has been measured non-invasively using pulsed A-scan ultrasound which showed that tropical tretinoin increased thickness. Biopsies have also been used but no changes in the thickness of the dermal repair zone have been noted in humans, in contrast to the situation in hairless mice. Epidermal dysplasia has been measured by a visual analogue scale or by objective image analysis.

Humans↗

PubMed1989

[Clinical aspects of Coats].

Coats' disease associates on exudative retinopathy to vascular telangiectasis. The authors analyse the different factors which take part to many clinical aspects of the Coats' exudation: the age of the patients and the vascular trouble which causes the exudative retinopathy.

Adult↗

PubMed1989

Multiple focal nodular hyperplasia of the liver associated with vascular malformations of various organs and neoplasia of the brain: a new syndrome.

Focal nodular hyperplasia (FNH) is a lesion of the liver in which a large anomalous artery is located within a region of hyperplastic hepatic parenchyma. Patients with FNH commonly have other lesions, often vascular in nature, in the liver or other organs. We have noted that these associated lesions almost always occur in patients with multiple FNH. We therefore studied 27 autopsied patients with FNH. All 13 with multiple FNH had other lesions such as hemangioma of liver, meningioma, astrocytoma, telangiectasis of the brain, berry aneurysm, dysplastic systemic arteries, and portal vein atresia. One patient had several of these lesions including multiple FNH, meningioma, astrocytoma, vascular malformation of the brain stem, and hemangioma of the liver. In contrast, among the 14 patients with solitary FNH there were no associated lesions, except for hepatic hemangioma in one patient. The prevalence of this syndrome was estimated by examination of 2500 serial autopsies and autopsies with various components of the syndrome. On review of 73 consecutive autopsies with meningioma, three had multiple FNH, compared with seven of 2500 consecutive adult autopsies (P less than 0.001). Multiple FNH was found in two of 83 autopsies with astrocytoma (P less than 0.05) and in one of 139 autopsies with berry aneurysm (not significant). We describe a telangiectatic subtype of FNH which occurs in this syndrome as well as in a minority of patients with solitary FNH. The existence and character of this syndrome suggest that there may be an underlying systemic abnormality in some patients having components of the syndrome. Investigation of patients with multiple FNH lesions may reveal significant treatable lesions.

Adolescent↗

PubMed1987

[Congenital hemihypertrophy associated with cutaneous pigmento-vascular, cerebral, visceral and bone abnormalities].

A case of hemihypertrophy associated with multiple anomalies of the skin, bone and visceral organs is presented. A 31-year-old female was admitted for evaluation of her skin conditions. Her family history is noncontributory, while her past history discloses operations for syndactyly of the right foot, tonsillar hypertrophy, anal prolapse and ovarial cyst. Erythemas of the face and the left upper extremity were noticed during the neonatal period and hypertrophy of the right side of the body started at age 2 months. On admission, hemihypertrophy was observed in the face, trunk and extremities. Multiple faint nevi flammei were seen on the right half of the face and on the left side of the trunk and extremities. Telangiectasis and nevus anemicus were seen in the upper chest. The left upper extremity showed diffuse brown patches that was histologically basal pigmentation with some giant melanosomes. Visceral anomalies consisted of fibromatous tumors of the tip of the tongue and mitral prolapse. Angiography and computed tomography revealed a possible arteriovenous malformation of the right occipital region, small hemangiomas around the patella, dilation of the lateral ventricle, and calcification of the choroid plexus. Tortuous superficial veins were noted in the right leg. She had no seizure, but her IQ was 68. The bone disorders consisted of scoliosis, short forth metacarpus, hypoplastic mandible and peroneal exostosis. Examination revealed a slight diminution of urinary corticosteroid, but no other endocrinological disorders were found. The hemihypertrophy in this case is at least partially due to an arteriovenous shunt, suggested by elevated oxygen saturation of the blood obtained from the internal saphenous vein.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗

PubMed1977

[Recurrent massive hemorrhage of the distal part of the digestive tract due to hemorrhagic hereditary telangiectasia].

Massive gastrointestinal bleeding is known to occur in patients presenting the hemorrhagic hereditary telangiectasis condition known as Rendu-Osler-Weber (ROW) disease. Bleeding is most frequent in the fourth decade. Endoscopic coagulation of upper gastrointestinal telangiectatic spots is nowadays possible even in the presence of active bleeding. In the lower digestive tract the naevi are basicallly located on the terminal ileum and the right colon; emergency coloscopic examination is difficult when active bleeding occurs. 10-15% of ROW patients present sudden life-threatening episodes of bleeding. The danger is aggravated through delayed diagnosis as well as difficulty in localizing the hemorrhagic area. Selective angiogram of the three digestive arteries is a safe and efficient method of diagnosing the nature and source of the bleeding. It should be obtained routinely, on an emergency basis, before surgery. Operation includes removal of the involved part of the digestive tract. A specimen angiogram should be obtained during the procedure, to make sure that the bleeding malformation has been removed. Laparotomy without preliminary angiogram is likely to be unsuccessful. An illustrative case is presented.

Aged↗