RETINOBLASTOMA ASSOCIATED WITH TOTAL EXUDATIVE RETINAL DETACHMENT: Treatment and Outcomes.

PURPOSE: To study the clinical presentation, treatment, and outcomes of eyes presenting with retinoblastoma and total retinal detachment. METHODS: A retrospective study of 50 eyes in 43 patients. RESULTS: The mean age at presentation was 15 months (median, 9 months). Retinoblastoma-associated total retinal detachment was unilateral (n = 36; 84%) or bilateral (n = 7; 16%). The mean tumor basal diameter was 17 mm (median, 18 mm), and the mean tumor thickness was 13 mm (median, 12 mm). Associated features included subretinal seeds (n = 26; 52%), vitreous seeds (n = 13; 26%), and subretinal exudation (n = 4; 8%). Based on eighth edition of American Joint Committee on Cancer classification, the tumors were classified as cT2a (n = 19; 38%), cT2b (n = 28; 56%), or cT3c (n = 3; 6%). Primary treatment included intravenous (n = 48; 96%) or intraarterial chemotherapy (n = 2; 4%). Over a mean follow-up period of 30 months (median, 29 months; range, 7-62 months), retinal detachment resolved in 41 eyes (82%), tumor recurrence was noted in 39 eyes (78%), globe salvage was achieved in 35 eyes (70%), and one patient (2%) died due to metastasis. CONCLUSION: Relatively high tumor recurrence rate is noted in retinoblastoma presenting with total retinal detachment. With appropriate treatment, 70% of the globes can be salvaged.

Lacrimal Drainage Anomalies in Goldenhar, Rubinstein-Taybi, and Ectodermal-Ectrodactyly-Clefting Syndromes.

OBJECTIVE: To describe in detail the lacrimal drainage system anomalies and review of literature in patients with Goldenhar syndrome, Rubinstein-Taybi syndrome (RTS), and Ectodermal-Ectrodactyly-Clefting syndrome (EECS), their management and outcomes. METHODS: A retrospective chart review from January 2011-June 2023 of all cases presenting to the Dacryology clinic with Goldenhar syndrome, RTS, and EECS was obtained. Data collected included demographics, laterality, clinical presentations, proximal and distal lacrimal drainage anomalies, associated systemic features, management, and outcomes. RESULTS: Eight children with Goldenhar syndrome (n = 13), three with RTS (n = 5) and three with EECS (n = 5) presented with lacrimal drainage system involvement. Cases with Goldenhar syndrome showed male predominance (5/8), and the mean age at presentation was 14.75 months. Four cases had simple CNLDO, seven cases with complex CNLDO (4 - buried probe and 3 - atonic sacs) and a single neonate presented with bilateral dacryocele. Patients with RTS presented with mean age of 36.33 months with male predominance. Probing under endoscopic guidance explored the anatomy thoroughly and those with altered nasal anatomy increased the probability of complex CNLDO. Those with EECS (n = 5) presented with a greater involvement of proximal lacrimal drainage system compared with Goldenhar syndrome and RTS, including anomalies like punctal agenesis, incomplete punctal canalization (IPC), ectopic puncta, canalicular stenosis, and complex CNLDO. CONCLUSIONS: A step-wise approach to assessing the proximal and lacrimal drainage system in those affected with craniofacial malformations and addressing them can result in satisfactory outcomes for the majority of patients.

Studies of human hemoglobin modified with peroxynitrite: A cytotoxic metabolite generated in numerous disorders.

OBJECTIVES: Peroxynitrite interacts with biomolecules through oxidative reactions or radical-mediated mechanisms leading to oxidative damage and committing cells to necrosis or/and apoptosis. Hemoglobin (Hb) is the oxygen-transporting metalloprotein found in blood that carries oxygen from the lungs to the tissues and subsequently releases it to carry out various metabolic functions. In the present study, we have isolated Hb from human blood and subjected it to modify by peroxynitrite generated in vitro. The native and modified Hbs were characterized using various biochemical methods. METHODS: The native and modified Hbs were characterized using absorption spectroscopy, thermal melting profile analysis, and other biochemical techniques. We have also tried to ascertain the effect of various scavengers such as uric acid, ascorbic acid, tocopherol, and reduced glutathione as potent peroxynitrite quenchers. RESULTS: The isolated Hb produces distinct peaks while the Hb modified with peroxynitrite showed marked hyperchromicity and the distinct peaks were lost. The chemical denaturation and thermal denaturation studies along with carbonyl content data show that the modified Hb is unstable and shows higher absorbance due to denaturation of the protein. CONCLUSION: Thus, the formation and effect of peroxynitrite on Hb are deleterious and antioxidant scavengers of the peroxynitrite show that the modification of the Hb can reverse the effect of peroxynitrite modification. The in vitro studies presented here show that peroxynitrite is toxic to human Hb and its inhibition by various antioxidants may be helpful in prevention of numerous disorders.

Depressive symptoms after acute myocardial infarction: evidence for highest rates in younger women.

BACKGROUND: Depression is common in patients hospitalized with acute myocardial infarction (AMI). In the community, younger women are uniquely prone to depression. Whether younger women are also more likely to have depression during hospitalization with AMI is unknown. METHODS: A total of 2498 AMI patients (1284 patients