Inborn errors of lysine metabolism

A wide variety of inborn errors of lysine catabolism have been described. They include: abnormalities of the mitochrondrial catabolism via the "saccharopine pathway", abnormalities of the peroxisomal catabolism via the pipecolic pathway, and "lysine intolerance with periodic ammonia intoxication" linked to urea cycle pathway. These inborn errors are associated with a wide variety of clinical symptoms and biochemical profiles as persistent hyperlysinemia, saccharopinuria, "amino and ketoadipic aciduria, and glutaric aciduria.     

Fine needle aspiration biopsy of the liver

The liver is a commonly targeted deep-seated organ in which fine-needle aspiration biopsy (FNAB) is performed. By far, the most frequently encountered lesions on FNAB of the liver are metastases; however, a wide variety of other lesions have been described and are likely to be encountered. Because of this wide variety, attention to architectural as well as cytologic features on smears and cell block preparations, ancillary techniques, and special studies are often required. In the setting of proper specimen handling and judicious use of ancillary procedures, FNAB of hepatic lesions can be an accurate diagnostic procedure.     

Painful truths about depressives' cognitions

Cognitive theories assert that depressed persons' cognitions are distorted. Most of the empirical literature directly contradicts this assertion. Using a wide variety of methods to study a wide variety of cognitive processes, experiments consistently find that depressed people suffer significantly less cognitive distortion than do both normals and nondepressed psychiatric patients. It was speculated that childhood traumas predispose depression by preventing the normal formation of a defensive screen against painful realities.     

A system for computing neuromorphometry from magnetic resonance images.

A new technique for the automated measurement of a variety of gross neuroanatomical structures (such as cerebral ventricles) is described. The system exploits the local statistical properties of dual-echo magnetic resonance images of the brain to produce a fully unsupervised classification of these images into a variety of tissue types. Following this, a simple region agglomeration procedure aggregates the segmented regions by class, and submits these regions to a computational-geometric analysis from which a wide variety of measures are derived.     

Recent developments in soft tissue tumours

This article reviews the clinicopathological features of several recently described soft tissue tumours, namely ossifying fibromyxoid tumour, angiomyofibroblastoma, epithelioid angiosarcoma, retiform haemangioendothelioma, intra-abdominal desmoplastic small cell tumour, spindle cell liposarcoma and low grade fibromyxoid sarcoma. Conceptual changes are also discussed. These include the relationship between Ewing's sarcoma and peripheral primitive neuroectodermal tumour, the proposed use of the term atypical lipoma for a subset of well differentiated liposarcomas, and the occurrence at a wide variety of sites of inflammatory myofibroblastic lesions of uncertain biological potential. In addition, advances in the study of soft tissue lesions at the molecular and cytogenetic levels are outlined, with particular emphasis on the recent identification of tumour-specific karyotypic abnormalities in a wide variety of sarcomas.     

Hormonally Functional Ovarian Neoplasms

Hormonally functional ovarian neoplasms are those tumors that secrete one or more hormones that are clinically manifested in the patient. The hormone production may have implications for the diagnosis, management, or treatment of the patient. Hormonally functional ovarian neoplasms include tumors that belong to various histologic categories and produce a variety of hormonal effects. Functional ovarian tumors most commonly produce steroid hormones, and such tumors frequently belong in the sec cordstromal and steroid cell categories. In addition, a wide variety of peptide hormones may be produced by ovarian tumors. Although in most instances the neoplastic cells themselves produce the hormones, a wide variety of tumors may induce their stroma to produce steroid hormones. The stroma of ovarian tumors is derived from the ovarian stroma and may, on occasion, resemble specialized ovarian stroma and its derivatives. Cells resembling luteinized stromal cells or luteinized theca cells may be present and appear to be responsible for the resultant hormone secretion.     

Innate lymphoid cells,possible interaction with microbiota

Seminars in Immunopathology - Recent studies have identified novel lymphocyte subsets named innate lymphoid cells (ILCs) lacking antigen-specific receptors. ILCs are present in a wide variety of...     

Gene therapy with helper-dependent adenoviral vectors: lessons from studies in large animal models

Helper-dependent adenoviral vectors (HDAd) are deleted of all viral genes and they can efficiently transduce a wide variety of dividing and non-dividing cells to mediate high transgene expression levels. Unlike early generation adenoviral vectors, the absence of viral genes in HDAd results in long-term transgene expression without chronic toxicity and permits a large cloning capacity of 36 kb. Moreover, HDAd genomes exist extra-chromosomally thus minimizing the risks of germline transmission and insertional mutagenesis. For these reasons, HDAd offers tremendous potential for in vivo gene therapy. This chapter reviews preclinical studies using HDAd in large animal models to assess safety and efficacy in a wide variety of gene therapy applications.     

Ultrastructure of the human breast and its disorders.

The extant literature pertaining to the ultrastructural features of disorders of the human breast is reviewed and annotated with findings derived from a personal study of 82 patients whose specimens encompassed a wide variety of such lesions. In addition, observations of lesions heretofore undescribed are presented.     

基因组CpG岛甲基化检测技术研究进展

启动子CpG岛甲基化所致的抑癌基因失活和肿瘤发生密切相关，因而了解抑癌基因CpG岛甲基化情况具有重要意义。目前CpG岛甲基化的检测方法众多，其原理分别基于甲基化敏感的限制性内切酶切割和亚硫酸氢钠修饰，用于全基因组大量CpG岛甲基化检测和基因特异性CpG岛的一个或多个CpG位点甲基化检测。     

提高抗体芯片检测灵敏度的方法

抗体芯片作为蛋白质芯片的一种,具有高通量、高特异性及平行性分析的优点,目前已被应用到许多领域,成为蛋白质组学研究过程中不可缺少的技术。但是如何构建高敏感度、微型化及检测动力学范围广的抗体芯片以满足样品检测的需要,仍然是抗体芯片发展过程中所面临的重要挑战。就如何提高其检测灵敏度作一综述。     

A sampler of hotlines and helplines

A wide variety of hotlines and helplines exist in New York City and nationally. Sixteen hotlines and helplines are described. Information is provided on the types of clients each hotline or helpline target. Hours of operation and contact information are included.     

The pulmonary biology of isoprostanes

Isoprostanes were first recognized as convenient markers of oxidative stress, but their powerful effects on a variety of cell functions are now also being increasingly appreciated. This is particularly true of the lung, which is comprised of a wide variety of different cell types (smooth muscle, innervation, epithelium, lymphatics, etc.), all of which have been shown to respond to exogenously applied isoprostanes. In this review, we summarize these biological responses in the lung, and also consider the roles that isoprostanes might play in a range of pulmonary clinical disorders.     

蛋白质组学技术在泛素-蛋白酶体系统研究中的应用

泛素-蛋白酶体系统(ubituitin-proteasome system,UPS)是一条高效的、具有选择性并依赖能量的蛋白降解途径。目前应用蛋白质组学技术的重要研究工具之一质谱(mass spectrometry,MS)技术研究UPS系统还处于起步阶段。本综述主要介绍了应用蛋白质组学技术研究UPS系统的研究现状以及存在的问题。     

The emerging roles of eosinophils in mucosal homeostasis

Eosinophils are granulocytes, typically implicated as end-stage effector cells in type-II immune responses. They are capable of producing a wide array of pre-formed molecules which render them with vast potential to influence a wide variety of processes. Nonetheless, eosinophil research has traditionally focused on their role in anti-helminthic responses and pathophysiological processes in type-II immune disorders, such as allergy and asthma, where eosinophilia is a hallmark phenotype. However, a number of key studies over the past decade have placed this restricted view of eosinophil function into question, presenting additional evidence for eosinophils as critical regulators of various homeostatic processes including immune maintenance, organ development, and tissue regeneration.     

如何引导医学生学好“抗生素”

 药理学是临床医学专业学生一门重要的基础课,涉及药物种类繁多,授课内容复杂,需掌握的知识点多,学生畏难情绪较重。在药理学抗生素教学实践中,教师可根据医学生特点和具体的授课内容,灵活运用多种授课技巧和方法,以提高学生的兴趣和教学质量,本文一一举例。     

Meningioma with Chordoid Features

Meningioma is a relatively common intracranial tumor, occurring most frequently in adults, and is capable of a wide variety of growth patterns. We describe a meningioma in a child that had a peculiar chordomalike appearance. The pathologic findings and distinction from chordoma are discussed.     

Caspase-1 inflammasomes in infection and inflammation

Nucleotide-binding and oligomerization domain-like receptors (NLRs) constitute a family of germline-encoded pattern-recognition receptors, which allow the host to respond rapidly to a wide variety of pathogenic microorganisms. Here, we discuss recent advances in the study of a subset of NLRs, which control the activation of caspase-1 through the assembly of large protein complexes, inflammasomes. The NALP1b inflammasome recognizes anthrax lethal toxin, and flagellin from Salmonella and Legionella induces assembly of the Ipaf inflammasome. Cryopyrin/NALP3 mediates caspase-1 activation in response to a wide variety of bacterial ligands, imidazoquinolines, dsRNA, and the endogenous danger signal uric acid. The importance of these cytosolic receptors in immune regulation is underscored by the identification of mutations in cryopyrin/NALP3, which are genetically linked to human autoinflammatory disorders.     

RNA interference: on the road to an alternate therapeutic strategy!

RNA interference (RNAi) is a newly described natural biological phenomenon mediated by small interfering RNA (siRNA) molecules which target viral mRNA for degradation by cellular enzymes. RNAi has become a method of choice for studying gene function, especially in mammalian systems. With proof-of-concept studies already presented against a wide variety of human pathogens and several innovative methods of delivering the siRNA to a wide variety of primary cells available, the role for siRNA as a potential therapeutic strategy is becoming increasingly clear. This review presents recent advances in this direction.     

The efficacy of habit reversal therapy for tics, habit disorders, and stuttering: a meta-analytic review

A meta-analysis based on 575 participants in 18 studies found Habit Reversal Therapy (HRT) to be an efficacious intervention for a wide variety of maladaptive repetitive behaviors, including stuttering, tics, nail biting, temporomandibular disorder, thumb sucking, and mixed repetitive oral-digital habits. Compared to control conditions, HRT showed a large effect size pre-treatment to final post-treatment assessment, d = 0.80. Moderator analyses revealed significant treatment effects for HRT for most moderator levels, indicating that HRT is efficacious in a number of variations for a variety of types of maladaptive behaviors, across a wide range of sample characteristics. The findings provide substantial support for the efficacy of HRT for disorders it is commonly used to treat. The findings are consistent with recent arguments for the classification of HRT as a well-established treatment for tic and habit disorders.