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Kuo D.S.,University of California at San Francisco | Nyong'O O.L.,Pediatric Ophthalmology
Journal of AAPOS | Year: 2010

Trichoepitheliomas are unusual, benign tumors of hair follicle origin. They may present in children and adults as acquired lesions on the body and face, but they rarely involve the eyelids. Solitary trichoepitheliomas have not previously been reported in infants. We present a first report of congenital solitary eyelid trichoepithelioma in an infant and review the classification of trichoepitheliomas. © 2010 by the American Association for Pediatric Ophthalmology and Strabismus. Source

ElKhamary S.,King Khaled Eye Specialist Hospital | Al-Mesfer S.,Pediatric Ophthalmology | AlKatan H.M.,Pathology and Laboratory Medicine
American Journal of Neuroradiology | Year: 2012

BACKGROUND AND PURPOSE: Pathologic prognostic parameters for retinoblastoma have been defined. Our purpose was to correlate ADC values at 3T with prognostic parameters of retinoblastoma. MATERIALS AND METHODS: This study included 72 children (30 boys and 42 girls, mean age 19 ± 2.6 months) with retinoblastoma. Pretreatment diffusion-weighted MR imaging was performed on a 3T scanner with b factors of 0, 500, and 1000 seconds/mm2. ADC values were calculated and pathologic specimens were analyzed. ADC values of the tumors were then correlated with prognostic parameters, including degree of histologic differentiation, tumor size, bilaterality, choroidal invasion, and optic nerve extension. RESULTS: The mean ADC value of retinoblastoma was 0.49 ± 0.12 × 10-3 mm2/s. The ADC values of well- and moderately differentiated tumors were significantly different (P = .007) from poorly and undifferentiated retinoblastoma. There was also a significant difference in the ADC value among small, medium, and large tumors (P = .015), as well as between unilateral and bilateral retinoblastoma (P = .001), and this was independent of the degree of differentiation. The ADC value was also significantly lower (P = .003) when optic nerve invasion was present. There was no correlation of ADC value with growth pattern or choroidal invasion (P = .640 and 0.661, respectively). The ADC value of retinoblastoma was well correlated with the degree of differentiation of the tumor (r = 0.87, P = .007) and inversely correlated with the size of the tumor (r = -0.68, P = .015). CONCLUSIONS: ADC correlated with some of the accepted parameters of poor prognosis for retinoblastoma and may serve as a noninvasive prognostic parameter for assessment of newly diagnosed retinoblastoma. Source

Marrani E.,Pediatric Rheumatology | Paganelli V.,Pediatric Rheumatology | de Libero C.,Pediatric Ophthalmology | Cimaz R.,Pediatric Rheumatology | Simonini G.,Pediatric Rheumatology
Graefe's Archive for Clinical and Experimental Ophthalmology | Year: 2015

Background: Non-infectious uveitis represents one of the most common causes of blindness, even at pediatric age; in particular, idiopathic chronic uveitis can pose significant difficulties during treatment, due to a partial response to TNF-α antagonists. To date, very few case series exist describing the treatment of idiopathic uveitis not adequately controlled by TNF-α antagonists. The aim of our study is to describe the role of abatacept in achieving remission in patients with idiopathic uveitis previously treated with TNF-α antagonists, and to assess how long abatacept efficacy is maintained during follow-up. The treatment’s safety profile and tolerability were also specifically investigated. Methods: Three patients affected with chronic idiopathic uveitis, who have been treated with abatacept due to loss of efficacy of TNF-α antagonists, were reviewed. Details of the demographic and clinical characteristics were recorded, and a summary of the medical history was obtained. Patients were regularly reviewed in the ophthalmology and rheumatology clinics. Assessment of their ocular condition was characterized according to the Standardization of Uveitis Nomenclature (SUN) group. Results: In our patients, abatacept was able to induce remission and to discontinue systemic corticosteroids after a mean of 30 weeks; the drug maintained its efficacy through a long follow-up period (42, 33, and 18 months respectively), with an excellent safety profile. Conclusion: Our small case series seems to suggest abatacept to be a promising therapy in children affected with chronic idiopathic uveitis not adequately controlled by TNF-α antagonists. © 2015, Springer-Verlag Berlin Heidelberg. Source

Jethani J.,Pediatric Ophthalmology | Shah K.,M and J Western Regional Institute of Ophthalmology | Jethani M.,Eye-Q
Indian Journal of Ophthalmology | Year: 2015

Background and Aim: Central corneal thickness (CCT) of term and preterm infants in Indian population is not known. We did a prospective noninterventional study to measure the CCT in term and preterm infants. Materials and Methods: An ultrasonic pachymeter was used. The data regarding the date of birth, expected date of delivery, birth weight were recorded. The preterm and the term infants were followed up at 8 weeks, 20 weeks and at 1-year. Results: A total of 85 (170 eyes) children were included in the study. The mean age was 264.6 ± 21.8 days postconception. The mean birth weight and CCT were 1834.4 ± 512.1 g and 595.8 ± 72.4 μ respectively. A comparison of CCT on the basis postgestational age showed a mean thickness of 620.7 ± 88.8 and 574.4 ± 78.3 μ in the <260 days and >260 days age groups respectively. The difference was statistically significant (Student's test, P = 0.002). The CCT of preterm infants (<260 days) decreased from a mean value of 620.7 ± 88.8 μ to 534.1 ± 57.6 μ at the end of 1-year. Conclusion: We present the data of CCT in term and preterm infants in Indian population. We believe that the premature babies have slightly thicker corneas than mature term babies. Source

Ketterl T.G.,University of Minnesota | Messinger Y.H.,University of Minnesota | Niess D.R.,University of Minnesota | Gilles E.,University of Minnesota | And 3 more authors.
Pediatric Blood and Cancer | Year: 2013

Opsoclonus-myoclonus syndrome (OMS) may be associated with ANNA-1 (anti-Hu) autoantibodies. The standard treatment with IVIG, steroids, and anti-CD20 monoclonal antibody may fail, and optimal therapy is unknown. A patient developed OMS with high-titer ANNA-1 following recovery from neuroblastoma. She failed standard therapy and had only transient response to rituximab. Treatment with the humanized anti-CD20 monoclonal antibody ofatumumab combined with methotrexate resulted in transient neurologic improvement and decrease of ANNA-1. This suggests that ofatumumab combined with methotrexate should further be considered OMS patients, particularly in refractory disease. Pediatr Blood Cancer 2013;60:E163-E165. © 2013 Wiley Periodicals, Inc. Source

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