Renal disease is a major cause of mortality in TSC. Lifelong surveillance and early intervention is warranted. SUDEP is also an important cause of mortality. Patients with learning disabilities are at significantly greater risk of early mortality and this implies the need for greater vigilance for TSC-related complications in this group. Female patients are vulnerable to pulmonary and renal disease. Pancreatic lesions are a rare but potentially treatable cause of mortality.
SummaryObjectiveEpidemiological studies have shown an association between increased bone mineral density (BMD) and osteoarthritis (OA), but whether this represents cause or effect remains unclear. In this study, we used a novel approach to investigate this question, determining whether individuals with High Bone Mass (HBM) have a higher prevalence of radiographic hip OA compared with controls.DesignHBM cases came from the UK-based HBM study: HBM was defined by BMD Z-score. Unaffected relatives of index cases were recruited as family controls. Age-stratified random sampling was used to select further population controls from the Chingford and Hertfordshire cohort studies. Pelvic radiographs were pooled and assessed by a single observer blinded to case-control status. Analyses used logistic regression, adjusted for age, gender and body mass index (BMI).Results530 HBM hips in 272 cases (mean age 62.9 years, 74% female) and 1702 control hips in 863 controls (mean age 64.8 years, 84% female) were analysed. The prevalence of radiographic OA, defined as Croft score ≥3, was higher in cases compared with controls (20.0% vs 13.6%), with adjusted odds ratio (OR) [95% CI] 1.52 [1.09, 2.11], P = 0.013. Osteophytes (OR 2.12 [1.61, 2.79], P < 0.001) and subchondral sclerosis (OR 2.78 [1.49, 5.18], P = 0.001) were more prevalent in cases. However, no difference in the prevalence of joint space narrowing (JSN) was seen (OR 0.97 [0.72, 1.33], P = 0.869).ConclusionsAn increased prevalence of radiographic hip OA and osteophytosis was observed in HBM cases compared with controls, in keeping with a positive association between HBM and OA and suggesting that OA in HBM has a hypertrophic phenotype.
Presently, sirolimus (rapamycin) is used as both induction and maintenance immunosuppression in solid organ transplants, including whole pancreas and kidney, and islet transplantation. Sirolimus has been suggested to have deleterious effects on islet beta-cell and renal function. We investigated the effect of sirolimus on the viability of islets, podocytes, and renal tubular cells. Sirolimus reduced the viability of islets and HK-2 human proximal renal tubular cells in vitro. This toxic effect was associated with a reduction of vascular endothelial growth factor (VEGF) release by islets but not the proximal tubular cells. Sirolimus reduced both viability and VEGF production by murine beta-cells, and blockade of VEGF-164 was associated with a reduction in viability. Transfection of murine islets with adenoviral VEGF-165 improved islet viability. These data are consistent with the hypothesis that sirolimus is toxic to islets and beta-cells by blockade of VEGF-mediated survival pathways.
Summary The performance of multidetector computed tomography (CT) angiography was assessed in the pre‐operative evaluation of live renal donors. Between July 1998 and March 2006, 156 consecutive patients underwent open donor nephrectomy following pre‐operative multidetector CT angiography (MDCTA). Operative notes were compared with radiological reports and discrepancies identified. MDCTA missed five of 28 accessory arteries (four visible with hindsight), accuracy of 96%. Of 30 early‐branching renal arteries, eight were missed (all visible with hindsight), accuracy 95%. MDCTA missed only one of 13 venous anomalies (accuracy 97%) and also missed the only duplicated collecting system: both were undetectable with hindsight. Following modifications to image acquisition and interpretation sensitivity, negative‐predictive value and accuracy were significantly increased. The results were compared with pooled data from published studies of live donor imaging. This study and previous studies of MDCTA had improved sensitivity for arterial and venous anomalies over single detector CT angiography and MR angiography. We conclude that multidetector CT angiography is an accurate modality in the pre‐operative evaluation of live renal donors. Regular communication between the transplant surgeon and the radiologist is paramount to improve reporting of surgically relevant anatomy. Mechanisms should exist for auditing and improving pre‐operative imaging in any live donor programme.
This study demonstrates the frequency of nerve injury during primary great saphenous vein surgery. It will be useful for clinicians providing informed consent and may provide a benchmark for comparison with newer techniques.
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