Hyper IgE (Job's) syndrome: a primary immune deficiency with oral manifestations

Autosomal dominant hyper IgE (HIES or Job's) syndrome is a rare primary immune deficiency characterized by eczema, recurrent skin and lung infections, extremely elevated serum IgE, and a variety of connective tissue and skeletal abnormalities. Individuals with HIES share a characteristic facial appearance and many oral manifestations including retained primary dentition, a high arched palate, variations of the oral mucosa and gingiva, and recurrent oral candidiasis. Mutations in STAT3 account for the majority, if not all, of the cases of autosomal dominant HIES, but the pathogenesis of the many varied features remains poorly understood. In this review, we discuss the clinical phenotype of HIES including immunologic and non-immunologic features, the genetics of HIES, and treatment.     

Gastric ulcer localization by direct in vivo labeling of sucralfate. Work in progress

We developed and evaluated a new procedure for imaging gastric ulcer disease with technetium 99m-labeled sucralfate. The new method employs direct in vivo labeling of sucralfate instead of in vitro labeling using human serum albumin, as previously reported in the literature. Tests using hydrochloric acid and a rabbit ulcer model showed the efficacy of the direct in vivo labeling technique and the ability of the tagged material to bind to ulcers, respectively. In 26 studies using humans with sucralfate labeled directly in vivo, 15 gave true-negative results and 11 gave true-positive results. Of 14 studies using humans with in vitro labeled sucralfate, three gave true-negative results, three gave true-positive results, and the results of eight were either false-negative or could not be interpreted because of high levels of activity remaining in the stomach. We suggest that the direct in vivo labeling method significantly improves the sucralfate gastric ulcer imaging technique.     

补肾补骨汤治疗绝经后骨质疏松症的疗效及分子机制

目的:明确补肾补骨汤(BuShenBuGuTang,BSBGT)治疗绝经后骨质疏松症的分子机制。方法:SD成年雌鼠分为阴性对照组、去势组、中药治疗组和雌激素治疗组4组,12周后检测4组骨密度、骨形态计量学参数及I型胶原的变化并比较各组间差异。体外实验采用大鼠骨髓基质细胞的原代培养体系,加用不同药物处理后,用半定量RT-PCR方法检测破骨细胞分化因子和护骨因子的变化情况。结果:补肾补骨汤对SD大鼠上述3项指标的维持具有明显的效果,而且在体外可明显地抑制破骨细胞分化因子的表达和促进护骨因子的表达。结论:补肾补骨汤对于绝经后骨质疏松症有明显的治疗作用,是通过调节破骨细胞分化因子和护骨因子的表达和分泌而发挥作用的。     

Ontogeny of gonadotrophin and inhibin secretion in normal girls through puberty based on overnight serial sampling and a comparison with normal boys

We measured luteinizing hormone (LH) and follicle stimulating hormone (FSH) by immunofluorometric assays and alpha-inhibin by radioimmunoassay in serum sampled every 10 min throughout the night (2100-0500 h) from 44 normal girls. Mean overnight LH values rose log- linearly from a mean of 0.2 IU/l in prepubertal girls to 3.0 IU/l in late pubertal girls. Log2 mean overnight FSH rose rapidly through early puberty and then remained constant; mean FSH rose from 1.0 IU/l in prepubertal girls to approximately 2.8 IU/l in Tanner III-V girls. Mean overnight inhibin increased through puberty, rising from 151 ng/l in prepubertal girls to 432 ng/l in fully pubescent girls. Within each of the first three Tanner stages, LH differed approximately 100-fold between the smallest and largest mean concentrations but differed <10- fold within stages IV or V. Such within-pubertal stage variability was less pronounced for FSH, which differed approximately 16-fold among Tanner I subjects and 4-10-fold at later stages, and for inhibin, which varied approximately 4-fold within each Tanner stage. The frequency of LH pulses during overnight sampling increased significantly during puberty, but the frequency of FSH and inhibin pulses remained constant. We compared the results from girls to those from 50 normal boys [Manasco et al. (1995) J. Clin. Endocrinol. Metab., 80, 20462052]. At each pubertal stage, girls had approximately the same mean overnight LH values as boys; girls had higher mean overnight FSH, particularly during Tanner stages II-IV; and boys had mean overnight alpha-inhibin immunoreactivity approximately 1.5 times that of girls at each pubertal stage. Still, hormone concentrations for individuals of both sexes intergraded at each pubertal stage.