Childhood chronic inflammatory demyelinating neuropathies: clinical course and long-term follow-up

Chronic inflammatory demyelinating neuropathy (CIDP) is a rare disease in childhood. We reviewed the clinical characteristics, response to therapy, and long-term prognosis in 13 children (1.5 to 16 years of age) diagnosed with CIDP at Washington University Medical Center, St. Louis, and the Royal Children's Hospital, Melbourne, Australia, between 1979 and 1994. The most common presenting symptom (in 11/13 [85%]) was lower extremity weakness associated with difficulty in walking. Preceding events within 1 months of onset, mostly intercurrent infections or vaccinations, occurred in seven children (54%). The disease was monophasic in three children (23%). One relapse occurred in four (30%) and multiple relapses in six (46%). All patients had at least short-term response to steroids. Three children (23%) recovered completely during the first year. Ten children (77%) had residual weakness after an average follow-up of 6 years. There seems to be two populations of children with CIDP. One subgroup, with a favorable prognosis, progressed to peak disability over less than 3 months; these children often have a monophasic course with complete resolution of symptoms and signs and withdrawal from all medications by 1 year after onset. A second subgroup progressed for 3 months or longer; these children all required substantial does of prednisone for prolonged periods and had considerable long-term morbidity with persistent weakness.     

Guanosine tetra- and pentaphosphate promote accumulation of inorganic polyphosphate in Escherichia coli

High levels of guanosine tetraphosphate (ppGpp) and guanosine pentaphosphate (pppGpp), generated in response to amino acid starvation in Escherichia coli, lead to massive accumulations of inorganic polyphosphate (polyP). Inasmuch as the activities of the principal enzymes that synthesize and degrade polyP fluctuate only slightly, the polyP accumulation can be attributed to a singular and profound inhibition by pppGpp and/or ppGpp of the hydrolytic breakdown of polyP by exopolyphosphatase, thereby blocking the dynamic turnover of polyP. The Ki values of 10 microM for pppGpp and 200 microM for ppGpp are far below the concentrations of these nucleotides in nutritionally stressed cells. In the complex metabolic network of pppGpp and ppGpp, the greater inhibitory effect of pppGpp (compared with ppGpp) leading to the accumulation of polyP, may have some significance in the relative roles played by these regulatory compounds.     

The course of pulmonary tuberculosis in adolescents

The authors summarize data obtained at the examinations of 178 tuberculous adolescents aged 15-17. Primary forms of the disease were diagnosed in 52% of new-onset cases, while infiltrative tuberculosis prevailed among secondary forms (48%). The tuberculosis was detected at prophylactic fluorography in 63.5% of the examinees. Late disease diagnosis was mostly consequent to poor symptoms of tuberculosis.     

X chromosome inactivation in mammals

The most important results of the last 30 years of studies on mammalian X-chromosome inactivation are reviewed. The data on X-chromosome inactivation in cells of embryonic and extraembryonic tissues and in male and female germ cell lines are discussed. Special attention is paid to data on mapping and functioning of the X-inactivation center and of recently discovered gene XIST. The main hypotheses concerning the mechanisms responsible for X-chromosome inactivation are considered. A new model of X-inactivation is proposed, which regards heterochromatin as a nonspecific activator of nucleation of the X-chromosome on which it is located.     

Eosinophil sequestration and activation are associated with the onset and severity of systemic adverse reactions following the treatment of onchocerciasis with ivermectin

To investigate the role of eosinophil activation and sequestration in the development and severity of adverse reactions after the treatment of Onchocerca volvulus infection, 40 O. volvulus-infected Ghanaians were randomized to receive placebo or standard- or high-dose ivermectin. Subjects were examined for typical physiologic and clinical events before and up to 48 h after treatment. Plasma samples were tested for interleukin (IL)-5 and eosinophil degranulation products (e.g., eosinophil-derived neurotoxin, EDN). After treatment, peripheral eosinophil counts declined in ivermectin-treated groups (P<.001), whereas circulating levels of IL-5 (P<.01) and EDN (P<.05) increased. Cumulative levels of IL-5 and EDN correlated with reaction scores (P<.01). High-dose ivermectin was associated with more-severe reactions, more-profound eosinopenia, and higher circulating levels of IL-5 and EDN, compared with the standard dose. These results suggest that eosinophil sequestration and activation/degranulation are associated with the initiation and severity of ivermectin-associated adverse reactions.     

The organization of automated information systems on occupational diseases and of the hygienic monitoring of working conditions

Creation of automated database "Archive of occupational diseases clinic" is an important task of informational support for occupational pathology centers. Constructing the database, the authors designed an "Accounting chart of patient with occupational disease for diagnostic survey in occupational pathology center" and a "Diagnosis of occupational disease" code book adjusted to X ICD with instruction. Analysis of the information obtained forms a basis for forecasting the course of pathologic process, for justifying a complex of treatment and prophylaxis.     

Morphological characteristics of lungs in embryos and fetuses in women living in regions contaminated with radionuclides after accident at the Chernobyl power plant

The possibility of transplacental passage of Cr-90 and Cs-137 as well as their traces appearance in descendants muscles and bones beginning from the 2nd pregnancy trimester (and this was sometimes associated with teratogenic effects) was demonstrated on the abortion material (human embryos and fetuses) from the controlled zones of the Bryansk region. Statistically significant inhibition of the bronchial branching (according to the morphometry of the lung histological section) this indicating the disturbance of the lung prenatal morphogenesis during the "pseudoglandular" developmental stage was observed in the 1st trimester of the pregnancy in the descendants of the irradiated mothers. Lung hypoplasia was observed in some cases in the 2nd and 3rd trimesters of the pregnancy. Activation of lymphoid cells in the fetal lungs and the tendency to the enhancement in them of the ectopic erythroid hemopoiesis was revealed. These processes are considered as a reflection of the compensatory adaptive cellular reaction in the descendants respiratory organs in response to the tissue hypoxia and antigenic stimuli from the organism of the pregnant woman irradiated after the Chernobyl accident.     

胃肠道间质瘤的诊断及预后探讨

目的探讨胃肠道间质瘤的诊断及预后相关因素。方法采用S-P免疫组化方法标记42例消化道及腹腔胃肠道间质瘤(GISTS)。结果42例GISTS中Des(+)4例(9.5%),SMA(+)14例(33%),Actin(+)12例(28.6%),CD34(+)36例(85.7%),CD117(+)40例(95.2%),S-100(+)12例(28.6%),Ki-67(+)22例(52.4%),bcl-2(+)28例(66.7%)。结论GISTS的诊断CD117阳性率高于CD34,但在诊断中应两者结合以提高诊断率;肿瘤的大小及核分裂的多少是判断良恶性的重要指标,而所有间质瘤具有潜在恶性。     

Characterization of the three-dimensional solution structure of human profilin: 1H, 13C, and 15N NMR assignments and global folding pattern

Human profilin is a 15-kDa protein that plays a major role in the signaling pathway leading to cytoskeletal rearrangement. Essentially complete assignment of the 1H, 13C, and 15N resonances of human profilin have been made by analysis of multidimensional, double- and triple-resonance nuclear magnetic resonance (NMR) experiments. The deviation of the 13C alpha and 13C beta chemical shifts from their respective random coil values were analyzed and correlate well with the secondary structure determined from the NMR data. Twenty structures of human profilin were refined in the program X-PLOR using a total of 1186 experimentally derived conformational restraints. The structures converged to a root mean squared distance deviation of 1.5 A for the backbone atoms. The resultant conformational ensemble indicates that human profilin is an alpha/beta protein comprised of a seven-stranded, antiparallel beta-sheet and three helices. The secondary structure elements for human profilin are quite similar to those found in Acanthamoeba profilin I [Archer, S. J., Vinson, V. K., Pollard, T. D., & Torchia, D. A. (1993), Biochemistry 32, 6680-6687], suggesting that the three-dimensional structure of Acanthamoeba profilin I should be analogous to that determined here for human profilin. The structure determination of human profilin has facilitated the sequence alignment of lower eukaryotic and human profilins and provides a framework upon which the various functionalities of profilin can be explored. At least one element of the actin-binding region of human profilin is an alpha-helix. Two mechanisms by which phosphatidylinositol 4,5-bisphosphate can interfere with actin-binding by human profilin are proposed.