Vesiculobullous Hailey-Hailey disease: Successful treatment with oral retinoids

A 56-year-old male presented with a pruritic, generalized vesiculobullous eruption. His past history revealed classical symptoms of limited Hailey-Hailey disease for 34 years. Clinically, vesicles, bullae and occasional pustules were present and multiple biopsies confirmed this to be an unusual presentation of Hailey-Hailey disease. Various therapeutic modalities including topical and oral antibiotics, oral prednisone and dapsone failed to achieve sustained remission. Treatment with lowdose oral etretinate (25 mg daily) produced marked clinical improvement with complete suppression of new vesicle formation after 6 weeks.     

Dubin-Johnson综合征1例

Dubin-Johnson综合征是一种先天性非溶血性黄疸,在临床中较罕见,需与其他原因所导致的黄疸相鉴别.本文报告了我院1例Dubin- Johnson综合征的诊治情况,有助于加深对该病的认识.     

自体骨髓干细胞移植治疗失代偿期肝硬化

选择36例失代偿期肝硬化患者,年龄37～59岁,患者在无菌条件下,从髂后上棘抽取骨髓100～200mL,在体外分离纯化骨髓干细胞后,局部麻醉下经股动脉插管经肝动脉将分离的骨髓干细胞移植于肝脏。自移植后12周,25例(69.4%)患者谷丙转氨酶逐渐降低,由平均(2788.56±357.90)nkat/L降至(1077.05±440.25)nkat/L;22例(61.1%)患者总胆红素逐渐下降,由平均(151.47±25.77)μmol/L降至(69.93±18.86)μmol/L;27例(75%)患者白蛋白逐渐升高,由平均(25.17±11.79)g/L升至(30.87±12.17)g/L。在干细胞移植后凝血酶原活动度逐渐上升,由术前平均(25.89±12.67)%上升至术后12周的(50.39±19.38)%,患者凝血机制明显改善。移植后大多数患者身体状况有明显的改善;移植后12周腹水减轻的19例(52.7%),食欲改善的28例(77.7%),体力好转20例(58.3%),腹胀减轻17例(47.2%),36例干细胞移植患者未出现严重并发症。     

Corrosion and Microstructural Investigation on Additively Manufactured 316L Stainless Steel: Experimental and Statistical Approach

The use of metal additive manufacturing (AM) has strongly increased in the industry during the last years. More specifically, selective laser melting (SLM) is one of the most used techniques due to its numerous advantages compared to conventional processing methods. The purpose of this study is to investigate the effects of process parameters on the microstructural and corrosion properties of the additively manufactured AISI 316L stainless steel. Porosity, surface roughness, hardness, and grain size were studied for specimens produced with energy densities ranging from 51.17 to 173.91 J/mm³ that resulted from different combinations of processing parameters. Using experimental results and applying the Taguchi model, 99.38 J/mm³ was determined as the optimal energy density needed to produce samples with almost no porosity. The following analysis of variance ANOVA confirmed the scanning speed as the most influential factor in reducing the porosity percentage, which had a 74.9% contribution, followed by the position along the building direction with 22.8%, and finally, the laser energy with 2.3%. The influence on corrosion resistance was obtained by performing cyclic potentiodynamic polarization tests (CPP) in a 3.5 wt % NaCl solution at room temperature for different energy densities and positions (Z axis). The corrosion properties of the AM samples were studied and compared to those obtained from the traditionally manufactured samples. The corrosion resistance of the samples worsened with the increase in the percentage of porosity. The process parameters have consequently been optimized and the database has been extended to improve the quality of the AM-produced parts in which microstructural heterogeneities were observed along the building direction.     

Reduced Circulating Androgen Levels After Gastric Bypass Surgery in Severely Obese Women

Background

Previous studies have shown a reduction of elevated androgen levels in premenopausal women after marked weight loss induced by bariatric surgery. In this study, we aimed to assess whether circulating androgen levels also decline after bariatric surgery in women displaying normal values preoperatively as well as in postmenopausal women.

Methods

In 36 severely obese women (six postmenopausal), levels of total testosterone, dehydroepiandresterone sulfate (DHEA-S), and sex hormone-binding globulin (SHBG) were assessed before and at ~1 year after gastric bypass. Free and bioavailable testosterone levels as well as the free androgen index were calculated by established formulas.

Results

After the surgery, women had lost on average 43.1?±?1.8 kg. Independently of the pre/postmenopausal state, women showed a marked reduction in all testosterone-related androgen markers and DHEA-S levels, while SHBG levels markedly increased (all P?<?0.001). Respective changes were found in both women with and without preoperatively elevated levels. Changes after the surgery in testosterone-related markers as well as in SHBG levels but not in DHEA-S levels were correlated with changes in insulin levels independently of body weight changes.

Conclusions

Data show a marked reduction of androgen levels in severely obese women after a surgically induced weight loss, which is independent from the menopausal state and preoperative levels. The mechanisms and consequences of these hormonal changes induced by bariatric surgery should be addressed in further studies.     

CT and MRI of ruptured intracranial dermoids

Summary Two patients with ruptured intracranial dermoids, examined with both CT and MRI are reported. Clinical presentation was transient cerebral ischemia in one patient and acute meningeal signs in the other. CT scan showed typical fat density of the tumor and the subarachnoid space. On MRI both the tumor and the subarachnoid fat, were strongly hyperintense on T1-weighted images.     

Basic principles of MRA

Two types of MR angiography techniques are used in the radiological practice for neurovascular applications: flow based techniques and ultrafast contrast enhanced acquisitions. Both techniques have their specific advantages and limitations. Whereas flow based techniques can be run on most MR scanners, high quality contrast enhanced studies require a state-of-the-art system with high slew rates and dedicated tools to match bolus passage and MR scanning. In this text, we focus on the physical acquisition principles and we illustrate the different phenomena in clinical examples. Numerous studies have proven the clinical applications for the 2 acquisition strategies. So far, an understanding of the basic physics remains necessary to explain occasional artefacts: MR angiography techniques are not yet fully robust. Further optimizations of the current approaches can be expected as there is still a need to improve image quality.     

Inositol 1,4,5-trisphosphate turnover enzymes--activities and subcellular distribution in hepatocarcinogenesis

The metabolism of inositol 1,4,5-trisphosphate and inositol 1,3,4,5- tetrakisphosphate in homogenates and sub-fractions from normal rat liver and premalignant liver nodules was investigated. The activities of 5-phosphatase, expressed as pmol converted substrate per minute and mg protein, were equal when using the two substrates, and did not differ between normal and nodular homogenates. Subcellular fractions were purified by sequential steps of differential centrifugation and density gradient fractionation procedures. The total phosphatase activity was found to be distributed between cytosol (15%) and membraneous fractions (75%), with most of the enzyme activity residing in the plasma membranes. A doubling of phosphatase specific activity was seen in the nodular low density membrane fraction, containing Golgi apparatus and endosomes, as compared with normal liver. Inositol 1,4,5- trisphosphate 3-kinase activity was found to be exclusively cytosolic. No difference in this enzyme was seen between the two tissue types studied. Vasopressin (0.2 or 2 microM) had no effect either on phosphatase or kinase activity. The compartmentalization of inositol polyphosphate 5-phosphatase activity presents a possible explanation of earlier findings that premalignant liver tissue was able to respond with inositol 1,4,5-trisphosphate, but not inositol 1,3,4,5- tetrakisphosphate formation after agonist stimulation.