Liver transplantation for Wilson's disease

Wilson's disease is an inherited disorder of copper metabolism characterized by reduced biliary copper excretion, which results in copper accumulation in the tissues with liver injury and failure. Orthotopic liver transplantation (OLT) can be lifesaving for patients with Wilson's disease who present with fulminant liver failure and for patients' unresponsive to medical therapy. The aim of this study was to review our experience with OLT for patients with Wilson's disease. Between September 2001 and April 2007, 25 OLTs were performed in 24 patients (7 females and 17 males) with Wilson's disease of mean age 15.6 +/- 9.9 years (range, 5-51 years). Six patients underwent transplantation owing to coexistent fulminant hepatic failure and 18 with chronic advanced liver disease with (n = 8) or without (n = 10) associated neurologic manifestations. We performed 3 full-size, deceased-donor OLTs and 22 living-related donor OLTs. Eight patients had a family history of Wilson's disease. We detected a Kayser-Fleischer ring in 18 patients. All patients had a low serum ceruloplasmin level (mean, 27.8 mg/dL) and a high urinary copper excretion level (mean, 4119 mug/d) before OLT. Following successful OLT, there was a significant reduction in urinary copper excretion (median, 37.1 mug/d) in all patients. Mean follow-up was 21.7 +/- 19.8 months (range, 2-60 months). Retransplantation was required in 1 patient at 12 days after the first OLT owing to primary graft nonfunction. Five of the 24 patients died within 4 months of the surgery. The remaining 19 survivors (79%) have remained well, with normal liver function and no disease recurrence. In conclusion, OLT was a curative procedure for Wilson's disease among patients presenting with fulminant hepatic failure and others with end-stage hepatic insufficiency. After OLT, the serum ceruloplasmin level increased to the normal range, urinary copper excretion decreased, and neurologic manifestations improved.     

Utility of c2 monitoring in prediction of diastolic dysfunction in renal transplant recipients

BACKGROUND: A number of experimental studies have suggested that cyclosporine (CsA) toxicity induces cardiac modifications which may cause diastolic dysfunction over the course of time. Doppler echocardiography with tissue Doppler imaging (TDI) could consistently detect diastolic dysfunction. The purpose of this study was to assess diastolic dysfunction using C2 monitoring of CsA exposure in stable renal transplant patients. PATIENTS AND METHODS: Seventy-eight kidney recipients including 42 men and 36 women of overall mean age of 52 +/- 9 years were obtained in 47 living and in 31 cases from cadaveric donations over 12 or more months after transplantation using cases from CsA, mycophenolate mofetil, and steroid. C2 levels were measured by an enzyme multi-immune assay technique. The patients underwent conventional and Doppler echocardiography with TDI. RESULTS: The patients were divided into 2 groups according to C2 levels less than 500 mug/L (group 1, n = 40) versus greater than 500 mug/L (group 2, n = 38). The demographic parameters, serum creatinine and lipid levels, systolic and diastolic blood pressures, number and type of antihypertensive medications, and conventional echocardiographic parameters did not differ significantly between the groups. However, group 1 patients showed significantly higher isovolumic relaxation time (109 +/- 27 vs 86 +/- 14 ms), early diastolic deceleration time (189 +/- 52 vs 137 +/- 59 ms), and lower values of E velocity (56 +/- 32 vs 92 +/- 27 cm/s) and E/A ratios (0.81 +/- 0.23 vs 1.15 +/- 0.46) than group 2. TDI studies revealed significantly lower E'/A' (0.76 +/- 0.25 vs 1.09 +/- 0.32, P < .05) in group 1 versus group 2. CONCLUSION: The data suggested that the higher C2 levels may induce diastolic dysfunction in the hearts of kidney recipients without impairment of contractile performance.     

Surgical treatment of postinfarction pseudoaneurysms of the left ventricle

BACKGROUND: Left ventricular pseudoaneurysm is a rare and frequently fatal complication of acute myocardial infarction. It occurs as a consequence of rupture of the ventricular free wall that gets confined by a portion of the pericardium. The purpose of this study was to present our surgical experience of postinfarction left ventricular pseudoaneurysms and to evaluate mid-term results. METHODS: The study population comprised five symptomatic patients diagnosed with left ventricular pseudoaneurysm and treated surgically in a short period of time. There were three males and two females. The mean age of the patients was 66.8 +/- 10.8 years. The diagnosis was made initially by echocardiography and subsequently confirmed by angiography. An additional cardiac magnetic resonance imaging study was performed in three patients. Surgical resection of the pseudoaneurysm was combined with an endoaneurysmorrhaphy procedure in all patients. Associated cardiac operations were performed in three patients. Definitive diagnosis of pseudoaneurysm was confirmed by histopathological evaluation of the excised wall in all patients. RESULTS: All patients survived the operation and were discharged to home care. The mean duration of hospital stay was 11.6 +/- 4.6 days. Patients were either in class I or II of New York Heart Association classification at discharge. All patients are still being followed after surgery with a mean follow-up period of 10.4 +/- 6.6 months with no further problems. CONCLUSION: Surgical repair is indicated in left ventricular pseudoaneurysm as it carries a high risk of rupture and sudden cardiac death. Surgical repair combined with an endoaneurysmorrhaphy procedure carries a low mortality risk and improves functional capacity.     

Influence of Rifampin on Capsule Formation Around Silicone Implants in a Rat Model

This study investigated the effect of rifampin on the thickness of capsules around silicone implants by bactericidal activity against Stapylococcus epidermidis. Silicone blocks (1 × 1 cm) were placed into pockets created for each of the 40 rats included in the study. In group 1, the operation was performed under aseptic conditions. In group 2, standard S. epidermidis was inoculated into the pocket, whereas rifampin and S. epidermidis were applied in group 3. In group 4, only rifampin was applied topically on implants. After 12 weeks, the peri-implant capsules were removed and examined under a photomicroscope and a scanning electron microscope. The mean thickness of the capsules was 63.307 μm in group 1, 111.538 μm in group 2, 43.076 μm in group 3, and 30.384 μm in group 4. The differences between groups 2 and 3 and groups 2 and 4 were found to be statistically significant (p < 0.001). Rifampin appears to be an agent for preventing peri-implant capsule formation.     

Comparison of aesthetic outcomes and morbidity of nasal reconstruction with forehead flaps and free flaps

The most common etiology of nasal defects that require reconstruction is skin cancer, particularly basal cell carcinoma, the most common nasal skin cancer, as well as squamous cell carcinoma, and melanoma. Reconstruction of extensive nasal defects including nasal bone, septum, and esthetically defined units of the nose always represent with certain difficulties. Forehead flaps and free flaps can be utilized for reconstruction of extensive nasal defects. The forehead flap is a reliable and well-established reconstructive option for management of extensive nasal defects. We aimed to compare the esthetic and functional results of forehead flaps and free flaps for reconstruction of extensive nasal defects.