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Sample records for telangiectasis

  1. 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.

  2. 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

    Directory of Open Access Journals (Sweden)

    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

  3. 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.

  4. 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...

  5. 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...

  6. 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:

  7. [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.

  8. 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

  9. 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

  10. 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)

  11. 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.

  12. 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.

  13. 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.

  14. 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

    Energy Technology Data Exchange (ETDEWEB)

    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

  15. 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

  16. 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

  17. 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

  18. Response of sensitive human ataxia and resistant T-1 cell lines to accelerated heavy ions

    International Nuclear Information System (INIS)

    Tobias, C.A.; Blakely, E.A.; Chang, P.Y.; Lommel, L.; Roots, R.

    1983-07-01

    The radiation dose responses of fibroblast from a patient with Ataxia telangiectasis (AT-2SF) and an established line of human T-1 cells were studied. Nearly monoenergetic accelerated neon and argon ions were used at the Berkeley Bevalac with various residual range values. The LET of the particles varied from 30 keV/μm to over 1000 keV/μm. All Ataxia survival curves were exponential functions of the dose. Their radiosensitivity reached peak values at 100 to 200 keV/μm. Human T-1 cells have effective sublethal damage repair as has been evidenced by split dose experiments, and they are much more resistant to low LET than to high LET radiation. The repair-misrepair model has been used to interpret these results. We have obtained mathematical expressions that describe the cross sections and inactivation coefficients for both human cell lines as a function of the LET and the type of particle used. The results suggest either that high-LET particles induce a greater number of radiolesions per track or that heavy-ions at high LET induce lesions that kill cells more effectively and that are different from those produced at low LET. We assume that the lesions induced in T-1 and Ataxia cells are qualitatively similar and that each cell line attempts to repair these lesions. The result in most irradiated Ataxia cells, however, is either lethal misrepair or incomplete repair leading to cell death. 63 references, 10 figures, 1 table

  19. 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

    Directory of Open Access Journals (Sweden)

    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.

  20. Conditional Müllercell ablation causes independent neuronal and vascular pathologies in a novel transgenic model.

    Science.gov (United States)

    Shen, Weiyong; Fruttiger, Marcus; Zhu, Ling; Chung, Sook H; Barnett, Nigel L; Kirk, Joshua K; Lee, SoRa; Coorey, Nathan J; Killingsworth, Murray; Sherman, Larry S; Gillies, Mark C

    2012-11-07

    Müller cells are the major glia of the retina that serve numerous functions essential to retinal homeostasis, yet the contribution of Müller glial dysfunction to retinal diseases remains largely unknown. We have developed a transgenic model using a portion of the regulatory region of the retinaldehyde binding protein 1 gene for conditional Müller cell ablation and the consequences of primary Müller cell dysfunction have been studied in adult mice. We found that selective ablation of Müller cells led to photoreceptor apoptosis, vascular telangiectasis, blood-retinal barrier breakdown and, later, intraretinal neovascularization. These changes were accompanied by impaired retinal function and an imbalance between vascular endothelial growth factor-A (VEGF-A) and pigment epithelium-derived factor. Intravitreal injection of ciliary neurotrophic factor inhibited photoreceptor injury but had no effect on the vasculopathy. Conversely, inhibition of VEGF-A activity attenuated vascular leak but did not protect photoreceptors. Our findings show that Müller glial deficiency may be an important upstream cause of retinal neuronal and vascular pathologies in retinal diseases. Combined neuroprotective and anti-angiogenic therapies may be required to treat Müller cell deficiency in retinal diseases and in other parts of the CNS associated with glial dysfunction.

  1. Absceso intramedular en paciente con enfermedad de Rendu-Osler-Weber Spinal abscess in a patient with hereditary hemorrhagic telangiectasia

    Directory of Open Access Journals (Sweden)

    Lorena V Maldonado

    2007-12-01

    Full Text Available La enfermedad de Rendu-Osler-Weber es una enfermedad autosómica dominante que se manifiesta por telangiectasias en piel y mucosas y malformaciones arteriovenosas en diversos órganos. El compromiso neurológico puede ocurrir por la presencia de malformaciones arterio-venosas cerebrales, hemorragia intracraneal, y más habitualmente por accidentes cerebrovasculares isquémicos y abscesos cerebrales secundarios a embolia paradojal, en pacientes con malformaciones arteriovenosas pulmonares. El absceso intramedular es una entidad rara, inusual como complicación de la enfermedad de Rendu-Osler-Weber. Presentamos el caso de una mujer de 56 años de edad, con antecedentes familiares de enfermedad de Rendu-Osler- Weber, que ingresó a Terapia Intensiva con cuadriplejia aguda e hipoxemia, en quien se diagnosticó fístulas arteriovenosas pulmonares y un absceso intramedular cervical.Rendu-Osler-Weber syndrome is an autosomal dominant disorder characterized by multiple skin and mucosal telangiectasis and multiorgan arteriovenous malformations. Neurological manifestations may occur because of cerebral arteriovenous malformations, intracranial hemorrhage, and most commonly by ischemic stroke and brain abscess secondary to paradoxical embolization in patients with pulmonary arteriovenous malformations. Intramedullary abscess is a rare, unusual condition, in Rendu-Osler-Weber syndrome. We report the case of a 56 years old woman, with a familial history of Rendu-Osler-Weber syndrome, admitted to intensive care with acute quadriplegia and hypoxemia. Our diagnosis was pulmonary arteriovenous malformations and intramedullary abscess.

  2. Multimodality imaging in macular telangiectasia 2: A clue to its pathogenesis

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    Lihteh Wu

    2015-01-01

    Full Text Available Macular telangiectasia type 2 also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A is an acquired bilateral neurodegenerative macular disease that manifests itself during the fifth or sixth decades of life. It is characterized by minimal dilatation of the parafoveal capillaries with graying of the retinal area involved, a lack of lipid exudation, right-angled retinal venules, refractile deposits in the superficial retina, hyperplasia of the retinal pigment epithelium, foveal atrophy, and subretinal neovascularization (SRNV. Our understanding of the disease has paralleled advances in multimodality imaging of the fundus. Optical coherence tomography (OCT images typically demonstrate the presence of intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic (FA finding is a deep intraretinal hyperfluorescent staining in the temporal parafoveal area. With time, the staining may involve the whole parafoveal area but does not extend to the center of the fovea. Long-term prognosis for central vision is poor, because of the development of SRNV or macular atrophy. Its pathogenesis remains unclear but multimodality imaging with FA, spectral domain OCT, adaptive optics, confocal blue reflectance and short wave fundus autofluorescence implicate Müller cells and macular pigment. Currently, there is no known treatment for this condition.

  3. Lead (Pb) induced ATM-dependent mitophagy via PINK1/Parkin pathway.

    Science.gov (United States)

    Gu, Xueyan; Qi, Yongmei; Feng, Zengxiu; Ma, Lin; Gao, Ke; Zhang, Yingmei

    2018-07-01

    Lead (Pb), a widely distributed environmental pollutant, is known to induce mitochondrial damage as well as autophagy in vitro and in vivo. In this study, we found that Pb could trigger mitophagy in both HEK293 cells and the kidney cortex of male Kunming mice. However, whether ataxia telangiectasis mutated (ATM) which is reported to be linked with PTEN-induced putative kinase 1 (PINK1)/Parkin pathway (a well-characterized mitophagic pathway) participates in the regulation of Pb-induced mitophagy and its exact role remains enigmatic. Our results indicated that Pb activated ATM in vitro and in vivo, and further in vitro studies showed that ATM could co-localize with PINK1 and Parkin in cytosol and interact with PINK1. Knockdown of ATM by siRNA blocked Pb-induced mitophagy even under the circumstance of enhanced accumulation of PINK1 and mitochondrial Parkin. Intriguingly, elevation instead of reduction in phosphorylation level of PINK1 and Parkin was observed in response to ATM knockdown and Pb did not contribute to the further increase of their phosphorylation level, implying that ATM indirectly regulated PINK1/Parkin pathway. These findings reveal a novel mechanism for Pb toxicity and suggest the regulatory importance of ATM in PINK1/Parkin-mediated mitophagy. Copyright © 2018 Elsevier B.V. All rights reserved.

  4. 眼底荧光血管造影诊断早期糖尿病性视网膜病变%Fluorescein angiography in diagnosis of early diabetic retinopathy

    Institute of Scientific and Technical Information of China (English)

    李敏

    2013-01-01

    Objective: To assess the value of fundus fluorescence angiogiaphy(FFA) in diagnostic of early diabetic retinopathy (DR) that had failed to be detected by ophthalenosope. Methods: One hundred and one patients ( 202 eyes) with non-insulin-dependent diabetes mellitus who had been proved to have no DR by ophthalenosope received FFA examination. Results: Forty-five patients (90 eyes) were found to have early DR by FFA. The lesions included capillaries lump, capillary non-perfusion, capillary fluorescence leakage, telangiectasis and macular edema. Conclusions: Fundus fluorecein angiography can detect diabetic retinopathy earlier than ophthalenosope.%目的:评价眼底荧光血管造影(fundus fluorescein angiography,FFA)诊断在检眼镜下未发现病变的早期糖尿病性视网膜病变(diabetic retinopathy,DR)的价值.方法:对101例(202眼)检眼镜下未发现DR的2型糖尿病患者进行FFA检查.结果:45例(90眼)FFA检查可见不同程度的早期DR,病变包括微血管瘤、毛细血管无灌注区、毛细血管荧光渗漏、毛细血管扩张及黄斑水肿.结论:FFA能更早发现早期DR.

  5. 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.

  6. 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)

  7. Evaluation of the use of common sculpin (Myoxocephalus scorpius) organ histology as bioindicator for element exposure in the fjord of the mining area Maarmorilik, West Greenland

    Energy Technology Data Exchange (ETDEWEB)

    Sonne, Christian, E-mail: csh@dmu.dk [Aarhus University, Faculty of Science and Technology, Department of Bioscience, Arctic Research Centre (ARC), Frederiksborgvej 399, P.O. Box 358, DK-4000 Roskilde (Denmark); Bach, Lis; Søndergaard, Jens; Rigét, Frank F.; Dietz, Rune; Mosbech, Anders [Aarhus University, Faculty of Science and Technology, Department of Bioscience, Arctic Research Centre (ARC), Frederiksborgvej 399, P.O. Box 358, DK-4000 Roskilde (Denmark); Leifsson, Pall S. [University of Copenhagen, Faculty of Health and Medical Sciences, Department of Veterinary Disease Biology, Bülowsvej 17, DK-1870 Frederiksberg (Denmark); Gustavson, Kim [Aarhus University, Faculty of Science and Technology, Department of Bioscience, Arctic Research Centre (ARC), Frederiksborgvej 399, P.O. Box 358, DK-4000 Roskilde (Denmark)

    2014-08-15

    The former Black Angel lead–zinc mine in Maarmorilik, West Greenland, is a historic example of how mining activity may result in a significant impact on the surrounding fjord system in terms of elevated concentrations of especially lead (Pb) and zinc (Zn) in seawater, sediments and surrounding biota. In order to shed light on the present contamination and possible effects in the fjord we initiated a range of studies including a pilot study on gill and liver morphology of common sculpins (Myoxocephalus scorpius) around Maarmorilik. Sculpins were caught and sampled at five different stations known to represent a gradient of Pb concentrations. Fish livers from all specimens were analyzed for relevant elements in the area: Fe, Zn, As, Cu, Se, Cd, Pb, Ag, Hg, Co and Ni. Lead, As and Hg showed significant differences among the five stations. For 20% of the sculpins, Hg concentrations were in the range of lowest observed effect dose (LOED) of 0.1–0.5 μg/g ww for toxic threshold on reproduction and subclinical endpoints. Likewise LOEDs for tissue lesions, LOEDs for biochemistry, growth, survival and reproduction were exceeded for Cd (0.42–1.8 μg/g ww) and for As (11.6 μg/g ww) in 28% and 85% of the sculpins, respectively. Similar to this, the no observed effect dose (NOED) for biochemistry was exceeded for Pb (0.32 μg/g ww) and for growth, mortality and reproduction for Zn (60–68 μg/g ww) in 33% and 24% of the sculpins, respectively. For all sculpins, females were significantly larger than males and for five of the elements (Fe, Co, Ni, Cu, Se) females had higher concentrations. The chronic lesions observed in liver (mononuclear cell infiltrates, necrosis, vacuolar hepatocytes, portal fibrosis, bile duct hyperplasia, active melanomacrophage centers) and gills (fusion and edema of secondary lamellae, laminar telangiectasis, mononuclear cell infiltrates, blebs) were similar to those in the literature studies for both wild and laboratory exposed sculpins and

  8. Evaluation of the use of common sculpin (Myoxocephalus scorpius) organ histology as bioindicator for element exposure in the fjord of the mining area Maarmorilik, West Greenland

    International Nuclear Information System (INIS)

    Sonne, Christian; Bach, Lis; Søndergaard, Jens; Rigét, Frank F.; Dietz, Rune; Mosbech, Anders; Leifsson, Pall S.; Gustavson, Kim

    2014-01-01

    The former Black Angel lead–zinc mine in Maarmorilik, West Greenland, is a historic example of how mining activity may result in a significant impact on the surrounding fjord system in terms of elevated concentrations of especially lead (Pb) and zinc (Zn) in seawater, sediments and surrounding biota. In order to shed light on the present contamination and possible effects in the fjord we initiated a range of studies including a pilot study on gill and liver morphology of common sculpins (Myoxocephalus scorpius) around Maarmorilik. Sculpins were caught and sampled at five different stations known to represent a gradient of Pb concentrations. Fish livers from all specimens were analyzed for relevant elements in the area: Fe, Zn, As, Cu, Se, Cd, Pb, Ag, Hg, Co and Ni. Lead, As and Hg showed significant differences among the five stations. For 20% of the sculpins, Hg concentrations were in the range of lowest observed effect dose (LOED) of 0.1–0.5 μg/g ww for toxic threshold on reproduction and subclinical endpoints. Likewise LOEDs for tissue lesions, LOEDs for biochemistry, growth, survival and reproduction were exceeded for Cd (0.42–1.8 μg/g ww) and for As (11.6 μg/g ww) in 28% and 85% of the sculpins, respectively. Similar to this, the no observed effect dose (NOED) for biochemistry was exceeded for Pb (0.32 μg/g ww) and for growth, mortality and reproduction for Zn (60–68 μg/g ww) in 33% and 24% of the sculpins, respectively. For all sculpins, females were significantly larger than males and for five of the elements (Fe, Co, Ni, Cu, Se) females had higher concentrations. The chronic lesions observed in liver (mononuclear cell infiltrates, necrosis, vacuolar hepatocytes, portal fibrosis, bile duct hyperplasia, active melanomacrophage centers) and gills (fusion and edema of secondary lamellae, laminar telangiectasis, mononuclear cell infiltrates, blebs) were similar to those in the literature studies for both wild and laboratory exposed sculpins and

  9. Safety of florfenicol administered in feed to tilapia (Oreochromis sp.)

    Science.gov (United States)

    Gaikowski, Mark P.; Wolf, Jeffrey C.; Schleis, Susan M.; Tuomari, Darrell; Endris, Richard G.

    2013-01-01

    The safety of Aquaflor® (50% w/w florfenicol [FFC]) incorporated in feed then administered to tilapia for 20 days (2x the recommended duration) at 0, 15, 45, or 75 mg/kg body weight/day (0, 1, 3, or 5x the recommended dose of 15 mg FFC/kg BW/d) was investigated. Mortality, behavioral change, feed consumption, body size, and gross and microscopic lesions were determined. Estimated delivered doses were >96.9% of target. Three unscheduled mortalities occurred but were considered incidental since FFC-related findings were not identified. Feed consumption was only affected during the last 10 dosing days when the 45 and 75 mg/kg groups consumed only 62.5% and 55.3% of the feed offered, respectively. There were significant, dose-dependent reductions in body size in the FFC-dose groups relative to the controls. Treatment-related histopathological findings included increased severity of lamellar epithelial hyperplasia, increased incidence of lamellar adhesions, decreased incidence of lamellar telangiectasis in the gills, increased glycogen-type and lipid-type hepatocellular vacuolation in the liver, decreased lymphocytes, increased blast cells, and increased individual cell necrosis in the anterior kidney, and tubular epithelial degeneration and mineralization in the posterior kidney. These changes are likely to be of minimal clinical relevance, given the lack of mortality or morbidity observed. This study has shown that FFC, when administered in feed to tilapia at the recommended dose (15 mg FFC/kg BW/day) for 10 days would be well tolerated.

  10. Current treatments for radiation retinopathy

    Energy Technology Data Exchange (ETDEWEB)

    Giuliari, Gian Paolo; Simpson, E. Rand (Princess Margaret Hospital, Univ. of Toronto, Dept. of Ophthalmology and Vision Sciences, Toronto (Canada)), e-mail: gpgiuliari@gmail.com; Sadaka, Ama (Schepens Eye Research Inst., Boston, MA (United States)); Hinkle, David M. (Massachusetts Eye Research and Surgery Institution, Cambridge, MA (United States))

    2011-01-15

    Background. To review the currently available therapeutic modalities for radiation retinopathy (RR), including newer investigational interventions directed towards specific aspects of the pathophysiology of this refractory complication. Methods. A review of the literature encompassing the pathogenesis of RR and the current therapeutic modalities available was performed. Results. RR is a chronic and progressive condition that results from exposure to any source of radiation. It might be secondary to radiation treatment of intraocular tumors such as choroidal melanomas, retinoblastomas, and choroidal metastasis, or from unavoidable exposure to excessive radiation from the treatment of extraocular tumors like cephalic, nasopharyngeal, orbital, and paranasal malignancies. After the results of the Collaborative Ocular Melanoma Study, most of the choroidal melanomas are being treated with plaque brachytherapy increasing by that the incidence of this radiation complication. RR has been reported to occur in as many as 60% of eyes treated with plaque radiation, with higher rates associated with larger tumors. Initially, the condition manifests as a radiation vasculopathy clinically seen as microaneurysms and telangiectasis, with posterior development of retinal hard exudates and hemorrhages, macular edema, neovascularization and tractional retinal detachment. Regrettably, the management of these eyes remains limited. Photodynamic therapy, laser photocoagulation, oral pentoxyphylline and hyperbaric oxygen have been attempted as treatment modalities with inconclusive results. Intravitreal injections of anti-vascular endothelial growth factor such as bevacizumab, ranibizumab and pegaptanib sodium have been recently used, also with variable results. Discussion. RR is a common vision threatening complication following radiation therapy. The available therapeutic options are limited and show unsatisfactory results. Further large investigative studies are required for developing

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

    Science.gov (United States)

    Hidano, A; Arai, Y

    1987-01-01

    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)

  12. Abnormal regulation of DNA replication and increased lethality in ataxia telangiectasia cells exposed to carcinogenic agents

    International Nuclear Information System (INIS)

    Jaspers, N.G.; de Wit, J.; Regulski, M.R.; Bootsma, D.

    1982-01-01

    The effect of different carcinogenic agents on the rate of semiconservative DNA replication in normal and ataxia telangiectasis (AT) cells was investigated. The rate of DNA synthesis in all AT cell strains tested was depressed to a significantly lesser extent than in normal cells after exposure to X-rays under oxia or hypoxia or to bleomycin, agents to which AT cells are hypersensitive. In contrast, inhibition of DNA replication in normal human and AT cells was similar after treatment with some DNA-methylating agents or mitomycin C. Colony-forming ability of AT cells treated with these agents was not different from normal cells. Treatment with 4-nitroquinoline 1-oxide elicited a variable response in both AT and normal cell strains. In some strains, including those shown to be hypersensitive to the drug by other workers, the inhibition of DNA synthesis was more pronounced than in other cell strains, but no significant difference between AT and normal cells could be detected. The rejoining of DNA strand breaks induced by X-rays, measured by DNA elution techniques, occurred within l2 hr after treatment and could not be correlated with the difference in DNA synthesis inhibition in AT and normal cells. After low doses of X-rays, AT cells rejoined single-strand breaks slightly more slowly than did normal cells. The rate of DNA replication in X-irradiation AT and normal cells was not affected by nicotinamide, an inhibitor of poly(adenosine diphosphate ribose) synthesis. These data indicate that the diminished inhibition of DNA replication in carcinogen-treated AT cells (a) is a general characteristic of all AT cell strains, (b) correlates with AT cellular hypersensitivity, (c) is not directly caused by the bulk of the DNA strand breaks produced by carcinogenic agents, and (d) is not based on differences in the induction of poly(adenosine diphosphate ribose) synthesis between X-irradiated AT and normal cells

  13. Global gene expression analysis in a mouse model for Norrie disease: late involvement of photoreceptor cells.

    Science.gov (United States)

    Lenzner, Steffen; Prietz, Sandra; Feil, Silke; Nuber, Ulrike A; Ropers, H-Hilger; Berger, Wolfgang

    2002-09-01

    Mutations in the NDP gene give rise to a variety of eye diseases, including classic Norrie disease (ND), X-linked exudative vitreoretinopathy (EVRX), retinal telangiectasis (Coats disease), and advanced retinopathy of prematurity (ROP). The gene product is a cystine-knot-containing extracellular signaling molecule of unknown function. In the current study, gene expression was determined in a mouse model of ND, to unravel disease-associated mechanisms at the molecular level. Gene transcription in the eyes of 2-year-old Ndp knockout mice was compared with that in the eyes of age-matched wild-type control animals, by means of cDNA subtraction and microarrays. Clones (n = 3072) from the cDNA subtraction libraries were spotted onto glass slides and hybridized with fluorescently labeled RNA-derived targets. More than 230 differentially expressed clones were sequenced, and their expression patterns were verified by virtual Northern blot analysis. Numerous gene transcripts that are absent or downregulated in the eye of Ndp knockout mice are photoreceptor cell specific. In younger Ndp knockout mice (up to 1 year old), however, all these transcripts were found to be expressed at normal levels. The identification of numerous photoreceptor cell-specific transcripts with a reduced expression in 2-year-old, but not in young, Ndp knockout mice indicates that normal gene expression in these light-sensitive cells of mutant mice is established and maintained over a long period and that rods and cones are affected relatively late in the mouse model of ND. Obviously, the absence of the Ndp gene product is not compatible with long-term survival of photoreceptor cells in the mouse.