Orbital retinoblastoma: present status and future challenges-a review

Orbital retinoblastoma is a catastrophic event traditionally carrying a dismal prognosis. Although its incidence is less in the developed countries it continues to be one of the major diagnosis at presentation in the developing world. Orbital retinoblastoma encompasses a wide range of distinct clinical entities with varying tumor load. There are no standard treatment protocols as of now but the current preferred management is multimodal with a combination of initial high-dose chemotherapy, surgery, external beam radiotherapy and prolonged chemotherapy for twelve cycles. In spite of progress on all fronts including surgical, medical, diagnostic, genetic and rehabilitative with improving survival rates, however, lack of access to medical facilities, lack of education about the need for early medical attention and cultural resistance to enucleation continue to contribute to an epidemic of extra ocular disease at diagnosis in the developing world. This review introduces the various terminologies used in the spectrum of orbital retinoblastoma, discusses in details the clinical aspects and management protocols, current status and the future directions

Oncogenic osteomalacia, you say? better start looking then - a case report

Tumour-induced or oncogenic osteomalacia (OOM) is a rare paraneoplastic syndrome characterized by bone pain and muscle weakness. A biochemical profile consisting of normocalcaemia, hypophosphataemia, phosphaturia, increased serum alkaline phosphatase and inappropriately low serum levels of 1, 25-dihydroxyvitamin-D is diagnostic. OOM is usually caused by an osseous or soft-tissue tumour of mesenchymal origin that secretes phosphaturic substances leading to increased urinary phosphate wasting. These tumours are small and slow growing. The diagnosis continues to be easily missed and when eventually made, localization of the tumour can be difficult. We describe the case of a young man who presented with severe generalized pain associated with muscle weakness. He was extensively investigated and eventually diagnosed to have OOM 3 years after initial presentation. Specialized investigations were necessary to localize the offending tumour.

Carbimazole-induced aplastic anaemia - a case report

Antithyroid drugs have been used for more than 50 years for the management of hyperthyroidism. Most patients tolerate treatment well, but some may develop rare life threatening side effects such as agranulocytosis and aplastic anaemia. Clinical experience with the latter condition is extremely limited. We report on a case of carbimazole-induced aplastic anaemia caused by hypocellular bone marrow and associated plasmacytosis in a thyrotoxic patient chronically treated with carbimazole. This resolved after substitution with propylthiouracil. The clinical course was complicated by neutropaenic septicaemia and atrial fibrillation.

Diabetes control- the legacy of a memory

Achieving and maintaining good glycaemic control remains an important goal in the management of this common and prevalent disorder. Recent evidence from important megatrials, ACCORD, ADVANCE, VADT, UKPDS-10 year follow-up as well as the STENO-2 follow-up study, have cleared doubts concerning the benefits of targeting good glycaemic control. For the first time, we have the reassurance that macrovascular benefits can be realised from good glycaemic control. The legacy effect of prior good glucose control from the UKPDS-10 year follow-up, reinforces the results seen from the DCCT-EDIC (for Type 1 diabetes). The Intervention Phase of the UKPDS revealed benefits for reduction of microvascular complications, while it was only at the end of the Post-Trial Monitoring Phase where significant improvements in both micro and macrovascular outcomes were seen. The other three Trials assessing the effect of glycaemic control on cardiovascular outcomes, although largely negative for CV benefit, give valuable insight towards appropriate patient characteristics for which aggressive glucose control can and should be instituted. Individualising glycaemic targets, which has been the approach that many clinicians have been practising, has received new impetus albeit with clearer details. Getting to glycaemic goal early in the course of T2DM and Doing to Safely (Avoiding hypoglycaemia) are the key ingredients to successful management. The legacy of the memory of initial good metabolic/glycaemic control is investment in good health with benefits of reductions in both micro and more importantly, macrovascular disease, years later. Multifactorial interventions that include blood pressure, lipid lowering in addition to glucose control in these individuals with the Metabolic Syndrome result in more immediate beneficial additive effects on cardiovascular outcomes.