Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32

Some MCP SNP and aHUS-associated MCP mutation     

Recurrent venous thrombosis following free flap surgery: The role of heparin-induced thrombocytopenia

Complications following free tissue transfer have been well established in the literature. Common and rare causes of free flap failure must be addressed by the treating surgeon when microvascular patency is threatened. With the evolution and prevalence of microsurgery, ‘rare’ causes of free flap failure will become increasingly frequent. A high index of suspicion must be established in patients with multiple failed operative interventions. A case of recurrent free flap failure secondary to heparin-induced thrombocytopenia is presented in a patient with a history of squamous cell carcinoma of the floor of the mouth, and a long-standing history of alcohol and tobacco consumption.     

Primary Localized Cutaneous Lichen Amyloidosus of the vulva

A woman, age 72, gravida 5, para 5, was hospitalized for a vulvar biopsy. Her past history revealed a generally healthy woman, menopausal from the age of 53, who had a vaginal hysterectomy and repair at the age of 68 because of genital prolapse. One year before her present admission, the woman started complaining of pruritus vulvae, which progressively worsened. On admission, general physical examination revealed no outstanding pathologic findings; gynecologic examination revealed advanced atrophy of the external genitalia and the vaginal wall. The clitoris and the small labia were atrophic, scarred, and only slightly elevated off the surface (Fig. 1). The vulvar skin was shiny with scattered white patches that were not elevated and had no shiny borders. The rectal examination was normal, and inguinal lymph nodes were not palpable. Laboratory tests revealed the following: sedimentation rate 15/26, hemoglobin 15 gidl, WBC 6400/mm³ (with normal differential count), thrombocytes 150,000/mm-³, serum alkaline phosphatase 100 mu/ml (normal 30–85), uric acid 7 mg/dl (upper normal value 6.4). Serum electrolytes, blood urea nitrogen, glucose, cholesterol, creatinine, total proteins, albumin, glutamic ox-aloacetic transaminase, lactic dehydrogenase, creatine phosphokinase, diastase, urine analysis, chest x-ray, and electrocardiogram were normal. Vulvar biopsy was per-formed under general anesthesia. Histologic examination of vulvar skin was as follows: the epidermis was hyperkeratotic with granulosis and acanthosis (Fig. 2). There were enlarged blood vessels in the dermis with thickened and hyalinized walls and foci of infiltration of round cells. The papillaris and reticularis revealed amorphic areas that stained positively with Congo red (Fig. 3). The histologic diagnosis was amy-loidosis of the vulvar skin. After the histologic report was received, further investiga-tions were done, as follows: urine Bence Jones protein was negative, serum protein electrophoresis and immunoelec-trophoresis were normal, and no free light chains were found in the plasma. Serum antinuclear factor and tubercu-lin skin test were negative. The patient refused to undergo rectal biopsy. She was initially treated with local application of testosterone ointment. After 2 months of treatment, the itching worsened and we started with applications of iso-conazole nitrate and diflucortolone valerate. This treatment was followed by prompt relief of symptoms, although the gross appearance of the lesions had not changed. One year later, the patient feels well and refuses repeat biopsy.     

Ultrasonographic evaluation of the endometrium and correlation with endometrial sampling in postmenopausal patients treated with tamoxifen

Seventy-two asymptomatic, postmenopausal women treated with tamoxifen for breast cancer were studied prospectively with vaginal ultrasonography, followed by endometrial sampling. Seventy-one patients demonstrated an endometrial echo of more than 5 mm, and one displayed an endometrial echo of less than 5 mm. No patient with an endometrial echo of less than 5 mm displayed any endometrial pathology. Different endometrial pathologic conditions were found when the endometrial echo was over 5 mm. When classifying those patients whose ultrasonic endometrial widths were wider than 5 mm, on the basis of different endometrial histologic findings, no obvious correlation was found between the various pathologic endometrial findings and endometrial thickness. Thus, a "thicker" endometrial sonographic image did not necessarily correlate with pathologic endometrial findings. When ultrasonic endometrial thickness of 5 mm was considered the upper limit of normal, the sensitivity of ultrasonography in correlating to positive histologic findings was 91% and the specificity was 96%. These findings suggest that there is good correlation between endometrial width measured by ultrasonographic assessment and histologic findings.     

The Rayleigh-Taylor condition for the evolution of irrotational fluid interfaces

For the free boundary dynamics of the two-phase Hele-Shaw and Muskat problems, and also for the irrotational incompressible Euler equation, we prove existence locally in time when the Rayleigh–Taylor condition is initially satisfied for a 2D interface. The result for water waves was first obtained by Wu in a slightly different scenario (vanishing at infinity), but our approach is different because it emphasizes the active scalar character of the system and does not require the presence of gravity.     

An artificial sphincter: A preliminary report

Summary A new method of providing continence to patients with fecal stomas is presented. The device, used as an artificial sphincter, consists of an inflatable Silastic balloon, which is implanted in the subcutaneous tissue around the stoma; it is easily handled by the patient. The artificial sphincter was used in six patients with colostomies. In all cases, satisfactory continence of the stoma was achieved, obviating the need to use enemas, bags or other appliances. In three patients subcutaneous infections developed around the prosthesis. In two cases, this was readily controlled; in one case, the prosthesis had to be removed. The other five patients are well and continent. None of the patients experienced pain or discomfort during use of the prosthesis. There has been no stomal ischemia. Read at the meeting of the American Society of Colon and Rectal Surgeons, San Diego, California, June 11 to 15, 1978.     

Clinical outcome and prognostic factors of patients with Richter syndrome: real-world study of the Spanish Chronic Lymphocytic Leukemia Study Group (GELLC)

Richter syndrome (RS) is an uncommon evolution of chronic lymphocytic leukaemia (CLL) with a dismal prognosis. Clinical-biological features predicting outcome and best therapeutic approach for these patients remain to be established. In this study, 128 patients with RS, including 112 diffuse large B-cell lymphoma (DLBCL)-type RS, 15 Hodgkin lymphoma (HL)-type RS, and one plasmablastic lymphoma, were identified in 11 centres of the Spanish CLL Study Group (GELLC). The median overall survival (OS) was 5·9 months for DLBCL-type RS and 30·8 months for HL-type RS. Eastern Cooperative Oncology Group Performance Status, haemoglobin level, platelet count, serum lactate dehydrogenase and β2-microglobulin levels, tumour protein p53 (TP53) abnormalities in the CLL clone concomitant to RS, number of prior therapies, and clonal relationship between CLL and RS, were associated with OS in patients with DLBCL-type RS. A platelet count of <100 × 10⁹/l, prior CLL therapy (0 vs. ≥1), and presence of TP53 alterations maintained an independent prognostic impact in the multivariate analysis. Patients without any of these factors had a better clinical outcome, with a median OS of 75·3 months, while patients with one or two or more of these factors presented a median OS of 25·5 and 3 months, respectively. Although OS of patients with RS is generally poor, a proportion of patients achieved prolonged survival. Treatment of RS remains a medical need, and further therapeutic approaches with novel therapies are warranted.     

Co-administration of non-carrier nanoparticles boosts antigen immune response without requiring protein conjugation

Nanotechnology promises a revolution in medicine including through new vaccine approaches. The use of nanoparticles in vaccination has, to date, focused on attaching antigen directly to or within nanoparticle structures to enhance antigen uptake by immune cells. Here we question whether antigen incorporation with the nanoparticle is actually necessary to boost vaccine effectiveness. We show that the immunogenicity of a sub-unit protein antigen was significantly boosted by formulation with silica nanoparticles even without specific conjugation of antigen to the nanoparticle. We further show that this effect was observed only for virus-sized nanoparticles (50 nm) but not for larger (1000 nm) particles, demonstrating a pronounced effect of nanoparticle size. This non-attachment approach has potential to radically simplify the development and application of nanoparticle-based formulations, leading to safer and simpler nanoparticle applications in vaccine development.