Older pediatric patients had no treatment failures, and tended to have fewer postoperative complications compared to younger patients. (C) 2011 Elsevier KPT-8602 mouse Ireland Ltd. All rights reserved.”
“Background: Chronically increased intestinal iron uptake in genetic hemochromatosis (HC) may cause organ failure. Whilst iron loading from blood transfusions

may cause dilated cardiomyopathy in conditions such as thalassemia, the in-vivo prevalence of myocardial siderosis in HC is unclear, and its relation to left ventricular (LV) dysfunction is controversial. Most previous data on myocardial siderosis in HC has come from post-mortem studies.

Methods: Cardiovascular magnetic resonance (CMR) was performed at first presentation of 41 HC patients (58.9 +/- 14.1 years) to measure myocardial iron and left ventricular (LV) ejection fraction (EF).

Results: In 31 patients (genetically confirmed HFE-HC), the HFE genotype was C282Y/C282Y (n = 30) and C282Y/H63D (n = 1). Patients with other genotypes (n = 10) were labeled genetically unconfirmed HC. Of the genetically confirmed HFE-HC patients, 6 (19%) had myocardial siderosis (T2* <20 ms). Of these,

5 (83%) had heart failure and reduced LVEF which was correlated to the severity of siderosis (R-2 0.57, p = 0.049). Two patients had follow-up scans and both had marked improvements in T2* and LVEF following venesection. Myocardial siderosis was present in 6/18 (33%) of patients with presenting ferritin >= 1000 mu g/L at diagnosis but in 0/13 (0%) patients with

ferritin <1000 mu g/L (p = 0.028). Overall AZ 628 however, the relation between myocardial siderosis and ferritin was weak (R-2 0.20, p = 0.011). In the 10 genetically unconfirmed HC patients, 1 patient had mild myocardial GSK872 siderosis but normal EF. Of all 31 patients, 4 had low LVEF from other identifiable causes without myocardial siderosis.

Conclusion: Myocardial siderosis was present in 33% of newly presenting genetically confirmed HFE-HC patients with ferritin >1000 mu g/L, and was the commonest cause of reduced LVEF. Heart failure due to myocardial siderosis was only found in these HFE-HC patients, and was reversible with venesection. Myocardial iron was normal in patients with other causes of LV dysfunction.”
“Objectives: To report the results of cochlear implantation via the middle fossa approach in 4 patients, discuss the complications, and present a detailed description of the programming specifications in these cases.

Study Design: Retrospective case review.

Setting: Tertiary-care referral center with a well-established cochlear implant program.

Patients: Four patients with bilateral canal wall down mastoid cavities who underwent the middle fossa approach for cochlear implantation.

Interventions: Cochlear implantation and subsequent rehabilitation.