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.     

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。     

The genome sequence of the probiotic intestinal bacterium Lactobacillus johnsonii NCC 533

Lactobacillus johnsonii NCC 533 is a member of the acidophilus group of intestinal lactobacilli that has been extensively studied for their "probiotic" activities that include, pathogen inhibition, epithelial cell attachment, and immunomodulation. To gain insight into its physiology and identify genes potentially involved in interactions with the host, we sequenced and analyzed the 1.99-Mb genome of L. johnsonii NCC 533. Strikingly, the organism completely lacked genes encoding biosynthetic pathways for amino acids, purine nucleotides, and most cofactors. In apparent compensation, a remarkable number of uncommon and often duplicated amino acid permeases, peptidases, and phosphotransferase-type transporters were discovered, suggesting a strong dependency of NCC 533 on the host or other intestinal microbes to provide simple monomeric nutrients. Genome analysis also predicted an abundance (>12) of large and unusual cell-surface proteins, including fimbrial subunits, which may be involved in adhesion to glycoproteins or other components of mucin, a characteristic expected to affect persistence in the gastrointestinal tract (GIT). Three bile salt hydrolases and two bile acid transporters, proteins apparently critical for GIT survival, were also detected. In silico genome comparisons with the >95% complete genome sequence of the closely related Lactobacillus gasseri revealed extensive synteny punctuated by clear-cut insertions or deletions of single genes or operons. Many of these regions of difference appear to encode metabolic or structural components that could affect the organisms competitiveness or interactions with the GIT ecosystem.     

A humanised tissue‐engineered bone model allows species‐specific breast cancer‐related bone metastasis in vivo

Bone metastases frequently occur in the advanced stages of breast cancer. At this stage, the disease is deemed incurable. To date, the mechanisms of breast cancer‐related metastasis to bone are poorly understood. This may be attributed to the lack of appropriate animal models to investigate the complex cancer cell–bone interactions. In this study, two established tissue‐engineered bone constructs (TEBCs) were applied to a breast cancer‐related metastasis model. A cylindrical medical‐grade polycaprolactone‐tricalcium phosphate scaffold produced by fused deposition modelling (scaffold 1) was compared with a tubular calcium phosphate‐coated polycaprolactone scaffold fabricated by solution electrospinning (scaffold 2) for their potential to generate ectopic humanised bone in NOD/SCID mice. While scaffold 1 was found not suitable to generate a sufficient amount of ectopic bone tissue due to poor ectopic integration, scaffold 2 showed excellent integration into the host tissue, leading to bone formation. To mimic breast cancer cell colonisation to the bone, MDA‐MB‐231, SUM1315, and MDA‐MB‐231BO breast cancer cells were cultured in polyethylene glycol‐based hydrogels and implanted adjacent to the TEBCs. Histological analysis indicated that the breast cancer cells induced an osteoclastic reaction in the TEBCs, demonstrating analogies to breast cancer‐related bone metastasis seen in patients.     

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.     

Rabbit serum albumin hydrolyzes the carbamate carbaryl

One of the main detoxification processes of the carbamate insecticides is the hydrolysis of the carbamic ester bond. Carboxylesterases seem to play important roles in the metabolization of carbamates. This study performs a biochemical characterization of the capabilities of rabbit serum albumin (RSA) to hydrolyze the carbamate carbaryl. Rabbit serum albumin was able to hydrolyze carbaryl with a K(cat) of 7.1 x 10(-5) s(-1). The K(m) for this hydrolysis reaction was 240 microM. Human, chicken, and bovine serum albumins were also able to hydrolyze carbaryl. The divalent cation Cu(2+) at 1 mM concentration inhibited around 50% of the hydrolysis of carbaryl by RSA. Other mono- and divalent cations at 1 mM concentration and 5 mM EDTA exerted no significant effects on the hydrolysis of carbaryl by RSA. The inhibition of the carbaryl hydrolysis by sulfydril blocking agents suggests that a cysteine residue plays an important role in the active center of the catalytic activity. Both caprylic and palmitic acids were noncompetitive inhibitors of the carbaryl hydrolysis by RSA. The carboxyl ester p-nitrophenyl butyrate is a substrate of RSA and competitively inhibited the hydrolysis of carbaryl by this protein, suggesting that the hydrolysis of carbaryl and the hydrolysis of carboxyl esters occur in the same catalytic site and through a similar mechanism. This mechanism might be based on the carbamylation of a tyrosine residue of the RSA. Serum albumin is a protein universally present in nontarget species of insecticides; therefore, the capability of this protein to hydrolyze other carbamates must be studied because it might have important toxicological and ecotoxicological implications.     

Mapping of the human visual cortex using image-guided transcranial magnetic stimulation

We describe a protocol using transcranial magnetic stimulation (TMS) to systematically map the visual sensations induced by focal and non-invasive stimulation of the human occipital cortex. TMS is applied with a figure of eight coil to 28 positions arranged in a 2x2-cm grid over the occipital area. A digitizing tablet connected to a PC computer running customized software, and audio and video recording are used for detailed and accurate data collection and analysis of evoked phosphenes. A frameless image-guided neuronavigational device is used to describe the position of the actual sites of the stimulation coils relative to the cortical surface. Our results show that TMS is able to elicit phosphenes in almost all sighted subjects and in a proportion of blind subjects. Evoked phosphenes are topographically organized. Despite minor inter-individual variations, the mapping results are reproducible and show good congruence among different subjects. This procedure has potential to improve our understanding of physiologic organization and plastic changes in the human visual system and to establish the degree of remaining functional visual cortex in blind subjects. Such a non-invasive method is critical for selection of suitable subjects for a cortical visual prosthesis.