Fibrovascular tissue in bilateral juxtafoveal telangiectasis.

Science.gov (United States)

Park, D; Schatz, H; McDonald, H R; Johnson, R N

1996-09-01

To study the natural history and retinal findings associated with the intraretinal and subretinal fibrovascular tissues that develop in the late phases of bilateral juxtafoveal telangiectasis. The records of 10 patients (11 eyes) with bilateral juxtafoveal telangiectasis who developed these fibrovascular tissues were examined. Throughout the follow-up period (average 44 months), only 2 eyes (18%) lost 2 or more lines of vision; the final visual acuities were similar for the eyes both with and without fibrovascular tissues. Sixty-four percent of fibrovascular tissues showed little to no growth. Eyes with fibrovascular tissue commonly had retinal pigment epithelial hyperplasia (72%), draining retinal venules (82%), and retinal vascular distortion (64%). Fibrovascular tissues of bilateral juxtafoveal telangiectasis have little proliferative potential and minimal effects on visual acuity. Nevertheless, these fibrovascular tissues do remodel over time, leading to retinal vascular distortion. Given these benign findings, the role of laser photocoagulation treatment of these tissues is questionable.

Long non-coding RNA expression profiles in hereditary haemorrhagic telangiectasia

DEFF Research Database (Denmark)

Tørring, Pernille M; Larsen, Martin Jakob; Kjeldsen, Anette D

2014-01-01

transcriptome, we wanted to assess whether lncRNAs play a role in the molecular pathogenesis of HHT manifestations. By microarray technology, we profiled lncRNA transcripts from HHT nasal telangiectasial and non-telangiectasial tissue using a paired design. The microarray probes were annotated using the GENCODE...... v.16 dataset, identifying 4,810 probes mapping to 2,811 lncRNAs. Comparing HHT telangiectasial tissue with HHT non-telangiectasial tissue, we identified 42 lncRNAs that are differentially expressed (qUsing GREAT, a tool that assumes cis-regulation, we showed that differently expressed lncRNAs...... to the TGF-β signalling pathway. The exact mechanism of how haploinsufficiency of ENG and ACVRL1 leads to HHT manifestations remains to be identified. As long non-coding RNAs (lncRNAs) are increasingly recognized as key regulators of gene expression and constitute a sizable fraction of the human...

Evaluation of protective effect of hydroalcoholic extract of Crocus sativus petals on preventing of gentamicin induced peliosis hepatis and hepatic telangiectasis in rats: short communication

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Arash Omidi

2013-02-01

Full Text Available Peliosis hepatis is a rare liver disease characterized by blood-filled cavities scattered irregularly throughout the liver. Risk factors for peliosis include chronic illness such as AIDS, tuberculosis, cancer also use of some drugs such as anabolic steroids and azathioprine. The aim of the present study was to evaluate the curative properties of crocus sativus petals on induced peliosis hepatis in rats. Thirty two male Wistar rats (weight: 180-220 g were randomly divided into four equal groups: group 1 (healthy group received only IP normal saline, group2 received IP 80mg/kg.bw gentamicin, group3 IP 80mg/kg.bw gentamicin+ 40mg/kg crocus sativus petal extract, and group 4 was given IP 80mg/kg.bw gentamicin+ 40mg/kg crocus sativus petal extract. At the end of the experiment, the rats were anesthetized and their blood samples were collected through cardiac puncture for AST and ALT measurement.Then, the livers of the subjects were excised and fixed in formalin. It was found that AST significantly increased in gentamicin group (P<0.05 compared to the healthy group and groups treated by means of crocus sativus petal extract .Moreover, there was no significant differences between the groups administered the extract and those given gentamicin. Histologically,heterogeneous multiple blood-filled cavities were observed in gentamicin group (2 and the treatment groups (3 and 4. The results of the present study show that doses of hydroalcoholic extract of crocus sativus do not effect on peliosis hepatic and telangiectasis due to gentamicin sulfate in rats

Injeção intravítrea de triancinolona no tratamento da telangiectasia retiniana justafoveolar idiopática Intravitreal triamcinolone injection in the treatment of idiopathic juxtafoveal telangiectasis

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Otacílio Oliveira Maia Júnior

2006-12-01

Full Text Available Relato de caso de um paciente com telangiectasia justafoveal idiopática (TJI tipo 1A, no olho direito, submetido a 4 mg de triancinolona intravítrea. O resultado foi avaliado por meio da acuidade visual e da tomografia de coerência óptica. A acuidade visual e a espessura retiniana macular medida na tomografia de coerência óptica, antes da injeção intravítrea de triancinolona, foram respectivamente de 20/100 e 569 µm e, após três semanas do tratamento foram de 20/60 e 371 µm e na sexta semana de 20/100 e 614 µm. A estabilização da parede vascular obtida com injeção intravítrea de triancinolona proporciona melhora transitória da visão e do edema macular em olhos com TJI-1A. Não foi demonstrada nenhuma ajuda permanente à fotocoagulação prévia.Case report of one idiopathic juxtafoveal telangiectasis (IJT 1A patient whose right eye was treated with a 4 mg intravitreal triamcinolone acetonide injection. The outcome was evaluated by visual acuity and optic coherence tomography. The visual acuity and the caliper retinal thickness before triamcinolone injection were respectively 20/100 and 569 µm, and 20/60 and 371 µm after three weeks and 20/100 and 614 µm after six week of follow-up. The stabilization of the vascular wall due to the intravitreal triamcinolone injection leads to a transitory improvement in vision and reduction in macular edema in the TJI 1A eyes. No permanent help by the photocoagulation could be shown.

Coats' disease in Tanzania: first case report and literature review ...

African Journals Online (AJOL)

Background: Coats' disease is an exudative retinal detachment with vascular telangiectasis occurring mostly in male children, the age group most affected by retinoblastoma. Objectives: To compare the differential diagnoses of Coats' disease. To establish recommendation to early disease detection. Materials and Methods:

[Mutations of ACVRL1 gene in a pedigree with hereditary hemorrhagic telangiectasia].

Science.gov (United States)

Luo, Jie-wei; Chen, Hui; Yang, Liu-qing; Zhu, Ai-lan; Wu, Yan-an; Li, Jian-wei

2008-06-01

To identify the activin A receptor type II-like 1 gene (ACVRL1) mutations in a Chinese family with hereditary hemorrhagic telangiectasia (HHT2). The exons 3, 7 and 8 of ACVRL1 gene of the proband and her five family members were amplified by polymerase chain reaction (PCR), and the PCR products were sequenced. The proband had obvious telangiectasis of gastric mucosa, and small arteriovenous fistula in the right kidney. All the patients in the HHT2 family had iterative epistaxis or bleeding in other sites, and had telangiectasis of nasal mucosa, tunica mucosa oris and finger tips. ACVRL1 gene analysis confirmed that there is frameshift mutation caused by deletion of G145 in exon 3 in the 4 patients, but the mutation is absent in 2 members without HHT2. The HHT2 family is caused by a 145delG mutation of ACVRL1 gene, resulting in frameshift and a new stop codon at codon 53.

Hereditary haemorrhagic telangiectasia: study of hepatic vascular alterations with multi-detector row helical CT and reconstruction programs; Telangiectasia emorragica ereditaria: TC multidetettore multifasica e programmi di ricostruzione nello studio delle alterazioni vascolari epatiche

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Memeo, Maurizio; Stabile Ianora, Amato Antonio; Scaldapane, Arnaldo; Rotondo, Antonio; Angelelli, Giuseppe [Policlinico Universitario, Bari (Italy). DiMIMP Sezione di Diagnostica per Immagini; Suppressa, Patrizia; Cirulli, Anna; Sabba' , Carlo [Policlinico Universitario, Bari (Italy). Centro Interdipartimentale per lo studio dell' HHT

2005-02-01

Purpose: To evaluate hepatic alterations in patients affected by Hereditary Haemorrhagic Telangiectasia (HHT) by using multidetector row helical CT (MDCT) and new reconstruction programs. Materials and methods: An MDCT multiphasic study of the liver was performed in 105 consecutive patients: 89 considered to be affected by HHT and 16 with suspicion of disease alone. The scan delay was determined by using a test bolus of contrast material. The CT examination was performed with a triphasic technique (double arterial phase and portal venous phase). multiplanar and angiographic reconstructions were then obtained, and the images checked for the presence of shunts, hepatic perfusion disorders, vascular lesions (telangiectasis and large confluent vascular masses), indirect signs of portal hypertension, and anatomical vascular variants. Results: Hepatic vascular alterations were found in 78/105 cases (67/89) patients affected by HHT and 11/16 patients with clinical suspicion alone). Therefore HHT diagnosis was excluded in 5 patients. 78/100 (78%) patients with HHT had intrahepatic vascular alterations: arterioportal shunts in 40/78 (51.2%) arteriosystemic shunts in 16/78 (20.5%) and both shunt types in 22/78 (28.3%). Intraparenchymal perfusion disorders were found in 46/78 (58.9%) patients. Telangiectasis were recognised in 50/78 (64.1%) patients. Large confluent vascular masses (LCVMs) were identified in 20/78 (25.6%) patients. indirect signs of portal hypertension were found in 46/78 (58.9%) cases. Variant hepatic arterial anatomy was present in 38/100 cases (38%). Conclusions: Multiphasic MDCT and the new reconstruction programs enable the identification and characterisation of the complex vascular alterations typical of HHT. [Italian] Scopo: Valutare le alterazioni epatiche nei pazienti affetti da Telangiectasia Emorraica Ereditaria (TEE) utilizzando una TC multidetettore (TCMD) ed in nuovi programmi di ricostruzione. Materiale e metodi: E' stato eseguito uno

Clinical picture of delayed radiation effects in the skin

International Nuclear Information System (INIS)

Hundeiker, M.

1987-01-01

Chronic radiation injuries of the skin develop over years or decades. Gradually increasing atrophy, sclerosis, telangiectasis, possibly - in highly exposed parts of the skin - keratosis due to radiation ulcers, carcinomas and basilomas occur after a latency period of decades, not so much in X-ray-injured skin after tumour therapy as in diffusely altered X-ray-injured skin after multiple exposure to low doses. Radiotherapy is indispensable, but like other effective methods of treatment it requires stringent indications, accurate execution and careful after-treatment. (TRV) [de

Hereditary haemorrhagic telangiectasia: study of hepatic vascular alterations with multi-detector row helical CT and reconstruction programs

International Nuclear Information System (INIS)

Memeo, Maurizio; Stabile Ianora, Amato Antonio; Scaldapane, Arnaldo; Rotondo, Antonio; Angelelli, Giuseppe; Suppressa, Patrizia; Cirulli, Anna; Sabba', Carlo

2005-01-01

Purpose: To evaluate hepatic alterations in patients affected by Hereditary Haemorrhagic Telangiectasia (HHT) by using multidetector row helical CT (MDCT) and new reconstruction programs. Materials and methods: An MDCT multiphasic study of the liver was performed in 105 consecutive patients: 89 considered to be affected by HHT and 16 with suspicion of disease alone. The scan delay was determined by using a test bolus of contrast material. The CT examination was performed with a triphasic technique (double arterial phase and portal venous phase). multiplanar and angiographic reconstructions were then obtained, and the images checked for the presence of shunts, hepatic perfusion disorders, vascular lesions (telangiectasis and large confluent vascular masses), indirect signs of portal hypertension, and anatomical vascular variants. Results: Hepatic vascular alterations were found in 78/105 cases (67/89) patients affected by HHT and 11/16 patients with clinical suspicion alone). Therefore HHT diagnosis was excluded in 5 patients. 78/100 (78%) patients with HHT had intrahepatic vascular alterations: arterioportal shunts in 40/78 (51.2%) arteriosystemic shunts in 16/78 (20.5%) and both shunt types in 22/78 (28.3%). Intraparenchymal perfusion disorders were found in 46/78 (58.9%) patients. Telangiectasis were recognised in 50/78 (64.1%) patients. Large confluent vascular masses (LCVMs) were identified in 20/78 (25.6%) patients. indirect signs of portal hypertension were found in 46/78 (58.9%) cases. Variant hepatic arterial anatomy was present in 38/100 cases (38%). Conclusions: Multiphasic MDCT and the new reconstruction programs enable the identification and characterisation of the complex vascular alterations typical of HHT [it

Hereditary hemorrhagic telangiectasia: progress of diagnostic imaging and vascular therapeutic embolization

International Nuclear Information System (INIS)

Lu Chuan; Liu Zuoqin

2008-01-01

Hereditary haemorrhagic telangiectasia (HHT) is a genetic autosomal-dominant disorder characterized by the presence of epistaxis, vascular telangiectasis in mucosal and cutaneous tissues, with visceral lesions and family history. However, many specialists or radiologists are still in lack of appreciation concerning the full range of consequences in diagnosis and their family relationship resulting the poor recognition of the disease. Understanding the diagnostic imaging and therapeutic measure for HHT will be critical, because of the continuous growth and risk existance of these arteriovenous malformations arousing early diagnosis, proper treatment, adequate follow-up and screening of the family. (authors)

SEM study of the effects of crude oil on the gills and air breathing organs of climbing perch, Anabas testudineus

International Nuclear Information System (INIS)

Prasad, M.S.

1991-01-01

Ultrastructural studies on the effects of crude oil on the gills are scanty. Recently, researchers studied the effect of crude oil on the air breathing organs of striped gourami using scanning electron microscope and observed mucous cell hyperplasia coupled with telangiectasis in the epithelia of air breathing organs. The present investigation has been undertaken to study crude oil toxicity by observing the morphological changes occurring in the epithelia of gills and air breathing organs of climbing perch, Anabas testudineus at SEM level. Since the epithelia of gills and air breathing organs function in two different media, a comparative account for their sensitivity to crude oil solutions would be informative

Global Gene Expression Profiling of Telangiectasial Tissue from Patients with Hereditary Haemorrhagic Telangiectasia

DEFF Research Database (Denmark)

Tørring, Pernille M; Larsen, Martin Jakob; Kjeldsen, Anette D

2015-01-01

and arteriovenous malformations in visceral organs, primarily the lungs, brain and liver. The most common symptom in HHT is epistaxis originating from nasal telangiectasia, which can be difficult to prevent and can lead to severe anaemia. The clinical manifestations of HHT are extremely variable, even within family...

Deformities of contour after breast conserving therapy and the possibilities of correction

International Nuclear Information System (INIS)

Deutinger, M.; Tairych, G.

1999-01-01

Background: Breast conserving treatment is increasing for primary treatment of breast carcinoma because of the importance of the cosmetic outcome. Patients and method: We examined 195 patients after breast conserving therapy which was performed between 1983 and 1992. For evaluation of the cosmetic result symmetry, contour of the breast and location of the areola were examined. Radiation effect on breast tissue was evaluated by the Lent score. 72% of the patients had been treated with quadrantectomy and 28% with lumpectomy. Results: Deformities of the contour were visible in 59% of the patients depending on the primary location of the tumor. Lumpectomy from medial quadrants caused poor results. Dislocation of the areola of more than 2 cm was detected in 32% of the patients. The dislocation depended on the primary kind of incision and resulted in 89% of the patients after a radial incision and only in 11% after curvilinear incisions. Telangiectasies were absent in 84% of the patients, the others showed telangiectasies Grade 1 to 3. In 48% of the patients no signs of fibrosis could be detected, in 49% fibrosis Grade 1 to 2 was found. 68% of the patients estimated the cosmetic result as very good or good. Only 10% of the patients estimated the result as fair or bad. The examiner estimated the results as good or very good in 28%. Examples of operative procedures for primary and secondary correction are demonstrated. Conclusions: Our results showed an adverse effect of long radial incisions. For lumpectomy and axillary node dissection separate incisions should be used. Correction of contour deformities should be done primarily in breast conserving procedures. This is possible by using modified reduction mammaplasties, local flaps of the breast tissue or switching a latissimus dorsi muscle flap. For secondary correction of defects after breast conserving treatment a latissimus dorsi muscle can be used as well as z-plasty for scar contracture. (orig.) [de

Tratamiento de algunas dermatosis por los rayos X de bucky: desarrollo histórico de la radioterapia superficial

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Gonzalo Reyes Garcia

1940-11-01

En los primeros años la Roentgenterapia, puso todo su interés en las reacciones cutáneas. Hasta el año de 1902, se trataron únicamente afecciones de la piel, sin buscar ningún efecto sobre los órganos profundos. Más tarde, Sinns, hacía publicaciones sobre la influencia de los rayos en la leucemia y poco a poco, con el perfeccionamiento de los aparatos y el aumento del voltaje, se fue desarrollando la radioterapia profunda, sobre todo en las afecciones cancerosas y otras de los órganos situados profunda mente; al mismo tiempo, se fueron fijando las técnicas y aplicaciones de la terapia superficial y se observaban las lesiones producidas por los rayos en la piel, tales como: atrofias, telangiectasis, úlceras renguenianas, eritemas, pigmentaciones, etc.

Imaging of Hereditary Hemorrhagic Telangiectasia

International Nuclear Information System (INIS)

Carette, Marie-France; Nedelcu, Cosmina; Tassart, Marc; Grange, Jean-Didier; Wislez, Marie; Khalil, Antoine

2009-01-01

This pictorial review is based on our experience of the follow-up of 120 patients at our multidisciplinary center for hereditary hemorrhagic telangiectasia (HHT). Rendu-Osler-Weber disease or HHT is a multiorgan autosomal dominant disorder with high penetrance, characterized by epistaxis, mucocutaneous telangiectasis, and visceral arteriovenous malformations (AVMs). The research on gene mutations is fundamental and family screening by clinical examination, chest X-ray, research of pulmonary shunting, and abdominal color Doppler sonography is absolutely necessary. The angioarchitecture of pulmonary AVMs can be studied by unenhanced multidetector computed tomography; however, all other explorations of liver, digestive bowels, or brain require administration of contrast media. Magnetic resonance angiography is helpful for central nervous system screening, in particular for the spinal cord, but also for pulmonary, hepatic, and pelvic AVMs. Knowledge of the multiorgan involvement of HHT, mechanism of complications, and radiologic findings is fundamental for the correct management of these patients.

Diagnostic management of pulmonary arteriovenous malformations in children

International Nuclear Information System (INIS)

Lynch-Nyhan, A.; White, R.I.; Terry, P.; Mitchell, S.E.

1988-01-01

Pulmonary arteriovenous malformations (PAVMs) are uncommon in childhood and adolescence. In the past 8 years, the authors have studied 21 patients. Symptoms included dyspnea (n = 13), epistaxis (n = 13), migraine headaches (n = 9), transient ischemic attacks (n = 6), hemoptysis (n = 3), and seizures (n = 3). Seventeen patients had hereditary hemorrhagic telangiectasis (HHT). All patients underwent diagnostic angiography, and 19 underwent detachable balloon embolotherapy. In patients with focal PAVMs, treatment increased the average preembolization arterial oxygen pressure of 63 mm Hg to a posttreatment average of 83 mm Hg. Marked clinical improvement was documented at follow-up (3 - 90 months), except in four patients with diffuse disease. Six patients underwent repeated embolotherapy of PAVMs that were not occluded initially. The authors conclude that PAVMs in children and adolescents are frequently symptomatic and associated with HHT. Detachable balloon embolotherapy is a safe and effective therapeutic modality

Leber's Hereditary Optic Neuropathy: A Case Report

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Chi-Wu Chang

2003-10-01

Full Text Available Leber's hereditary optic neuropathy (LHON is a maternally inherited mitochondrial disease that primarily affects the optic nerve, causing bilateral vision loss in juveniles and young adults. A 12-year-old boy had complained of blurred vision in both eyes for more than 1 year. His best-corrected visual acuity was 0.08 in the right eye and 0.1 in the left. Ophthalmologic examination showed bilateral optic disc hyperemia and margin blurring, peripapillary telangiectasis, and a relative afferent pupil defect in his right eye. Fluorescein angiography showed no stain or leakage around the optic disc in the late phase. Visual field analysis showed central scotoma in the left eye and a near-total defect in the right. Upon examination of the patient's mitochondrial DNA, a point mutation at nucleotide position 11778 was found, and the diagnosis of LHON was confirmed. Coenzyme Q10 was used to treat the patient.

Efectos del parasitismo sobre el tejido branquial en peces cultivados: estudio parasitologico e histopatologico Effect of the parasitism on the gill tissue of cultivated fishes: parasitological and histopathological studies

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Maurício Laterça Martins

1996-01-01

Full Text Available One hundred four out of 225 diagnosticated cases were myxosporidian, monogenean, Ichthyophthirius multifiliis Fouquet, 1876 and bacterial diseases in Piaractus mesopotamicus Holmberg, 1887 (pacu, Colossoma macropomum Cuvier, 1818 (tambaqui and tambacu, at Aquaculture Center, Universidade Estadual Paulista (UNESP, Jaboticabal, São Paulo, Brazil, between 1992 and 1995. The gills were fixed in 10% buffered formalin solution to posterior histologic routine. It was observed Henneguya sp. cysts into the capillaries of the secondary lamellae, encapsulated by the respiratory epithelium cells. It provoked adherence of the adjacent lamellae, hyperplasia, congestion, oedema and epithelium displacement. Monogeneans infestations by Anacanthorus penilabiatus Boeger, Husak & Martins, 1995 and Ancyrocephalinae sub-family caused light inflammatory reaction and hyperplasia. In severe infestations was observed hyperplasia of primary lamellae, necrosis, oedema, respiratory epithelium displacement, ruptured pillar cells and telangiectasis. Response to I. multifiliis was limited to surrounding epithelial cells in young fishes and hyperplasia, necrosis, inflammatory infiltrate and oedema in old fishes. Such lesions iniciate hyperplasic and oedematous process that with inflammation of the parasitic sites, provoked alterations over gases and ions interchange surface and consequently fish metabolism.

Radiodermatitis of the hands in a dental practitioner

International Nuclear Information System (INIS)

Warin, A.P.

1979-01-01

A man aged 43, presented with a 4 year history of a rash affecting his hands. He had been a dental practitioner for 20 years and admitted to lack of care with X-rays, especially in holding the X-ray film within the patient's mouth himself. He never withdrew behind a lead screen and so had been constantly exposed to X-rays over a prolonged period. Shortly prior to presentation, he had been using a cream containing amethocaine in an attempt to relieve the pain in his hands. On examination he had a weeping eczema of his hands but he was also noted to have atrophy and sclerosis of the skin of the fingers with telangiectasia and some erosions. A diagnosis of radiodermatitis was made, with superimposed contact eczema. Patch testing showed a positive reaction to amethocaine, and with the use of topical corticosteroids, the eczema settled quickly, leaving the typical signs of chronic radiodermatitis. Histopathology during the eczematous phase showed spongiosis with an eroded epidermis. The dermis showed telangiectasis and scarring of collagen. Although the epidermis showed some basal cell proliferation and acanthosis, there was no epidermal dysplasia. (author)

Radiodermatitis of the hands in a dental practitioner

Energy Technology Data Exchange (ETDEWEB)

Warin, A P [Saint John' s Hospital for Diseases of the Skin, London (UK)

1979-03-01

A man aged 43, presented with a 4 year history of a rash affecting his hands. He had been a dental practitioner for 20 years and admitted to lack of care with X-rays, especially in holding the X-ray film within the patient's mouth himself. He never withdrew behind a lead screen and so had been constantly exposed to X-rays over a prolonged period. Shortly prior to presentation, he had been using a cream containing amethocaine in an attempt to relieve the pain in his hands. On examination he had a weeping eczema of his hands but he was also noted to have atrophy and sclerosis of the skin of the fingers with telangiectasia and some erosions. A diagnosis of radiodermatitis was made, with superimposed contact eczema. Patch testing showed a positive reaction to amethocaine, and with the use of topical corticosteroids, the eczema settled quickly, leaving the typical signs of chronic radiodermatitis. Histopathology during the eczematous phase showed spongiosis with an eroded epidermis. The dermis showed telangiectasis and scarring of collagen. Although the epidermis showed some basal cell proliferation and acanthosis, there was no epidermal dysplasia.