Avascular necrosis of bone following renal transplantation

This study was undertaken to determine the incidence and clinical features of avascular necrosis of bone in 69 transplant recipients all of whom had a functioning allograft for a least 12 months. An attempt was also made to identify any potential predisposing factors. The patients were assessed by an orthopaedic surgeon. The diagnosis of avascular necrosis was tnade on the basis of radiographs and isotope bone scans. Fourteen patients (20,2%) developed avascular necrosis with a mean onset of 19 months post-transplantation.The hip joint was most commonly affected. The isotope bone scan was the most sensitive diagnostic tool; abnonnalities were detected before the onset of symptoms in 4 patients. Avascular necrosis was more common in Indian transplant recipients and was also associated with: (i) cadaver transplants; (ii) more frequent bouts of acute rejection (P < 0,05); and (iii) a greater incidence of other steroid-associated side-effects (P < 0,05).Alcohol conswnption and radiological evidence of osteoporosis were more prevalent in the avascular necrosis group (42,8% v. 29,0% and 28,5% v. 7,2% respectively). Avascular necrosis did not correlate with age, sex, renal function at 1 year or severe secondary hyperparathyroidism.This study suggests that corticosteroid therapy plays an important role in the pathogenesis of avascular necrosis. Excessive alcohol conswnption and osteoporosis also appear to be risk factors

Diagnosis, monitoring and treatment of tuberous sclerosis complex: A South African consensus response to international guidelines

Tuberous sclerosis complex (TSC) is a genetic disorder with multi-system manifestations and a high burden of disease. In 2013, an international panel of TSC experts revised the guidelines for the diagnosis, surveillance and treatment of the disorder. In South Africa (SA), a local multidisciplinary group of healthcare professionals and TSC researchers reviewed the international guidelines to generate an SA consensus clinical update on the identification, diagnosis, treatment and lifelong monitoring of individuals who live with TSC. We strongly endorse dissemination and use of the international guidelines for the assessment, monitoring and treatment of TSC. In addition, we strongly support access to genetic testing and to mTOR (mammalian target of rapamycin) inhibitors to treat subependymal giant cell astrocytomas not amenable to surgery and renal angiomyolipomas larger than 3 cm, and as adjunctive treatment for refractory focal seizures. We await with interest results from mTOR inhibitor trials of skin and TSC-associated neuropsychiatric disorders (TAND). With regard to training, we recommend the inclusion of TSC in undergraduate and postgraduate medical and health sciences curricula, and the promotion of other continuing professional development events to raise awareness about TSC. We also support the development of a TSC user/carer/parent organisation to provide an informal support network for families across SA. We acknowledge that some progress has been made in recent years in SA, but much remains to be done. We hope that this SA consensus clinical update based on the international guidelines will make a positive contribution to increase knowledge and improve clinical care for all patients who live with TSC in SA, and their families