1,720,961 research outputs found
Ocular features and management in the mucopolysaccaridosis
No full textPurpose
To evaluate the ocular findings and the visual outcome in a group of patients with mucopolysaccaridoses(MPS) followed and treated with new therapeutic options.
Methods
18 pazients with MPS (9 males and 9 females) were examinated: 3 cases with MPS type I, 4 with MPS type II,7 with MPS type III, 1 with MPS type IV and 3 with MPS type VI. The patients underwent an ophthalmological assessment with : visual acuity (with Teller Acuity Cards, Lea Symbols o letter chart, depending on patient age or degree of cohoperation), slit-lamp examination, fundus ophthalmoscopy, intraocular pressure, refractometry, electroretinography and eye echography.
Results
The mean follow-up was 48 months (ranged from 3 to 190 months).The mean age at first ophtalmologic evaluation was 6,9 years ( ranged from 1 to 20 years). 8 case presented progressive corneal clouding, 5 case retinal pigmentary degeneration, and 1 papilledema. Visual acuity worsened severely in 3 cases with MPS type I: 1 case from 10/10 to 5/10, 1 case from 5/10 to 1/10; in another case visual acuity decreased to 1/20 and underwent to corneal transplantation with good visual outcome (RE 5/10 and LE 7/10). The other cases showed slightly reduction of visual function.
3 patients had ocular hypertension and were treated with hypotensive eye drops.
8 patients underwent enzyme replacement therapy.
Conclusions
The mucopolysaccaridoses(MPS) are rare systemic disorders caused by accumulation of glycosaminoglycans. Ophthalmological manifestations are frequent in MPS, particularly in MPS I, II and VI, characterized by corneal clouding, retinal distrophy and blindness.Regular ophthalmic monitoring to determine disease progression and management of complications are necessary as a part of multidisciplinary approach
Inquadramento diagnostico delle distrofie retiniche ereditarie in età pediatrica: casi clinici
No full text
Treatmen of conjunctival squamous cell carcinoma with topical 5-Fluorouracil
Full text linkAbstract:
Aim:To evaluate the efficacy of topical 5-fluorouracil (5-FU) alone, without concurrent surgery or radiotherapy for the treatment of conjunctival squamous cell. carcinoma.
Methods:Eight patients affected by conjunctival squamous cell carcinoma (three recurrent cases, three incompletely excised, and two untreated cases) were treated with 1% 5-FU eye drops. Topical 1% 5-FU was administered four times daily for 4 weeks (one course). Clinical examination (biomicroscopy and photography) and morphological evaluation of conjunctival cytological specimens were used to monitor the efficacy of local chemotherapy, side effects, and recurrences;
Results:All patients showed clinical regression of conjunctival carcinoma after topical 1% 5-FU treatment. Neoplastic conjunctiva was completely replaced by normal epithelium within 3 months. Mean follow up was 27 months. One patient needed two courses of local chemotherapy for recurrent disease. An acute transient toxic keratoconjunctivitis was observed in all treated cases; it was easily controlled with topical therapy. No long term side effects were found.
Conclusions:Topical 1% 5-FU is effective in the treatment of recurrent, incompletely excised, and selected untreated conjunctival squamous cell carcinomas. Topical 1% 5-FU has no major complications. This study suggests that topical conjunctival chemotherapy with 1% 5-FU may be useful, at least as adjunctive therapy, in the treatment of conjunctival squamous cell carcinoma
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Long-term outcome and morphology/function correlates in cases of shaken baby syndrome
No full textPURPOSE: Haken Baby Syndrome (SBS) presents with acute encephalopathy, subdural and retinal haemorrhages, with inconsistent history, in < 2 years old. Long-term sequelae are still under study. Aim is to present the long-term outcome in a case series of SBS followed-up by a multidisciplinary team.
METHODS: Case series of 14 children (9M,%F) with confirmed SBS has been examined and followed-up. Patients underwent funds evaluation (indirect ophtalmoscopy) and wide-field digital ophthalmic camera (RetCam II) until bleeding reabsorption. Assessment was repeated at follow-up combined with ocular motility evaluation, visual field (BEFIE test), visual acuity (Teller acuity cards), refractometry, cognitive-behavioural evaluations (Griffiths scales, Child Behaviour Check List), and family stress measurement (Parenting Stress Index), segmentation analysis of MRI.
RESULTS: Mean age at acute episode of SBS: 6.5 months (range 2-20), 9/14 in the first 6 months of life. Mean age at last follow-up evaluation: 32 months (range 8-65). 4 cases were lost to follow-up. At last follow-up evaluation: 4 out of 10 had a decreased visual acuity (cortical visual impairment), 4 out of 10 had visual field deficits, 3 out of 10 had strabismus. None of the cases showed significant refractive errors. Cognitive/behavioural assessment demonstrated global delay and impairments in neuromotor ability, speech/language development and attention problems. MRI analysis showed atrophic alterations in several brain areas.
CONCLUSION:Multidisciplinary assessment including ophthalmologic examination and neurobehavioral evaluation provides crucial prognostic information in cases of suspected SBS. Functional outcome is largely dependent on brain injury and atrophy
- …
