Attempted treatment of factor H deficiency by liver transplantation

Complement factor H (FH) deficiency is one of the causes of atypical hemolytic uremic syndrome (HUS). Most patients with FH deficiency associated HUS progress to end-stage renal disease despite plasma therapy. Moreover, the disease invariably recurs in the graft kidney and causes graft failure. We confirmed FH deficiency in a 30-month-old boy with recurrent HUS of 2 years duration, and attempted an auxiliary partial orthotopic liver transplantation (APOLT) to overcome the sustained intractable dependency on plasma therapy. APOLT restored the plasma FH level, without HUS recurrence, for 7 months. However, thereafter he suffered from serious infectious complications associated with immunosuppression and finally died 11 months after APOLT. In conclusion, although APOLT showed clinical and laboratory improvement for some period in this patient, the final fatal outcome suggests that liver transplantation should be cautiously applied to patients with HUS associated with FH deficiency.     

Use of an oxygen therapeutic as an adjunct to intraoperative autologous donation to reduce transfusion requirements in patients undergoing coronary artery bypass graft surgery

BACKGROUND: The benefits of intraoperative autologous donation (IAD) in reducing the need for allogeneic blood transfusion in surgery have been debated for several years. The purpose of this study was to determine if IAD alone or in conjunction with hemoglobin raffimer (HR) confers a reduction in red cell or blood component transfusion compared with results in standard clinical practice. STUDY DESIGN: The Phase III clinical trial was a multicenter, randomized, double-blind study to determine the efficacy and safety of HR versus 10% pentastarch when used to facilitate IAD in 299 patients undergoing primary coronary artery bypass grafting. The patients received HR or pentastarch as an adjunct to IAD immediately before cardiopulmonary bypass. Results were compared with transfusion requirements for 150 matched patients in the reference group. RESULTS: The frequency of allogeneic RBC transfusion in the HR, pentastarch, and reference groups was 56%, 76%, and 95%, respectively. The number of allogeneic red cell units used was 49 in the HR group, 104 in the pentastarch group, and 480 in the reference group (p < 0.001). The total number of non-RBC units administered was 150 in the HR group, 238 in the pentastarch group, and 270 in the reference group. CONCLUSIONS: In this study, patients treated with HR in conjunction with IAD received fewer transfusions overall and a lower volume of allogeneic RBCs and non-RBC allogeneic blood products than did the two comparison groups. This confers a real benefit on the overall blood supply by decreasing use and increasing availability.     

Phyllodes tumor of the seminal vesicle: case report and literature review

A 39-year-old man presented with urinary retention and lower abdominal discomfort at our hospital, and a computed tomography scan showed a huge cystic mass posterior to the urinary bladder. During surgical exploration, a mass superior to the prostate in the region of the left seminal vesicle was found. Histologically, the tumor was characterized by cystically dilated or slit-like glands mixed in a densely cellular stroma with pleomorphism and resembled those of phyllodes tumor of the breast or prostate. The glandular epithelium within the tumor showed focal lipofuscin pigment and negative staining for prostate specific antigen (PSA). The stromal cells showed positive immunoreactivity for vimentin and CD34, and focal positive reactions for desmin and alpha-smooth muscle actin. Mitosis was present 0 to 1 per 10 high power fields of magnification in the stromal cells. Approximately 20% of the stromal cells were positive for progesterone receptor. The patient is alive with no evidence of disease 12 months after surgery. Mixed epithelial-stromal tumors of the seminal vesicle are extremely rare. A combination of stromal cellularity, atypia and mitosis might be used for the histological grading, and a prostatic origin might be excluded by the location of the primary lesion itself and by the failure to show PSA.     

Evaluations of bioactivity and mechanical properties of poly (epsilon-caprolactone)/silica nanocomposite following heat treatment

A composite material consisting of poly(epsilon-caprolactone) (PCL) and silica was prepared and evaluated as a bioactive bone substitute. The composite was synthesized by the co-condensation of tetraethyl orthosilicate and PCL and end-capped with triethoxysilane (Si-PCL). The as-prepared specimens were subjected to an initial heat treatment of 2 days at 60 degrees C, followed by further heat-treatments at 100 degrees C, 150 degrees C, and 200 degrees C for 24 h. The tensile mechanical properties of the heat-treated specimens were determined, and additional specimens were exposed to a simulated body fluid (SBF) for different periods of time. The SBF exposure led to the deposition of a layer of apatite crystals on the surface of the composites. It was found that increasing the second heat-treatment temperature produced an increase in tensile strength and Young's modulus of the composite but a decrease in the initial rate of apatite formation. These phenomena are explained in terms of the condensation reaction that takes place between the silanol groups in the silica and Si-PCL as the heat-treatment temperature is increased.     

Evaluation of groEL gene analysis for identification of Borrelia burgdorferi sensu lato

The nucleotide sequences of the groEL genes, the flagellin genes, and the 16S rRNA genes from 22 reference strains of Borrelia were compared. groEL sequence analysis is useful not only in interspecies differentiation but also in intraspecies differentiation of Borrelia afzelii and Borrelia garinii isolates.     

Burkitt's lymphoma representing periportal infiltrating mass on CT

Imaging findings of secondary hepatic lymphoma have been reported as variable, ranging from single or multiple small nodules to diffuse infiltrative tumor patterns. We hear present a rare case report concerning aggressive B cells, secondary Burkitt's lymphoma in non-AIDS demonstrating a surprising periportal lymphoma infiltration, without upper abdominal lymphadenopathy or splenomegaly on the sonography and CT scans. Clinically, the case was characterized by atypical and highly aggressive course, with the patient presenting an abruptly developed obstructive jaundice with rapidly deteriorating hepatic function that could be indicative of cholestatic hepatitis, which differs in its clinical manifestations from hepatic lymphoma without functional deterioration in respect of its non-tissue destructive growth pattern. We suggest that hepatic lymphoma can sometimes be consistent with periportal infiltrating homogeneous mass, with no lymphadenopathy or splenomegaly on the imaging examination, with a predictable aggressive clinical course of the disease and poor prognosis.     

Transplant graft vasculopathy: an emerging target for prevention and treatment of renal allograft dysfunction

Maintenance of healthy endothelium is essential to vascular homeostasis, and preservation of endothelial cell function is critical for transplant allograft function. Damage of microvascular endothelial cells is now regarded as a characteristic feature of acute vascular rejection and chronic allograft nephropathy, which is an important predictor of graft loss and is often associated with transplant vasculopathy. In this review, we will discuss the role of microvascular endothelium, in renal allograft dysfunction, particularly as it relates to markers of endothelial dysfunction and endothelial repair mechanisms. We also discuss the potential for therapies targeting endothelial dysfunction and transplant graft vasculopathy.