Periodic limb movement in sleep in children with Williams syndrome

Difficulty differentiating physiologic genu varum from early Blount's disease persists. Drennan's metaphyseal-diaphyseal (MD) angle remains the most consistently valuable radiographic parameter despite measurement error. Clinical risk factors also should be considered. All patients receiving orthoses for genu varum since 1985 were reviewed. The focus of the study was those patients with an MD angle of >16 degrees or between 9 and 16 degrees with a clinical risk factor for progression. Risk factors considered were ligamentous instability, obesity, asymmetry, and being female, black, or Hispanic. Thirty-eight patients with 60 tibiae were included. The success rate was 90%. Risk factors for failure (six cases) were instability, obesity, and delayed bracing. In cases with MD angles >16 degrees, the success rate was 86%. The results of orthotic treatment, restricted to patients meeting the stated parameters, represent improvement on the reported natural history.     

Angiotensin II signal transduction pathways

It has been 100 years since the discovery of renin by Tigerstedt and Bergman. Since that time, numerous discoveries have advanced our understanding of the renin-angiotensin system, including the observation that angiotensin II is the effector molecule of this system. A remarkable aspect of angiotensin II is the many different physiological responses this simple peptide induces in different cell types. Here, we focus on the signal transduction pathways that are activated as a consequence of angiotensin II binding to the AT1 receptor. Classical signaling pathways such as the activation of heterotrimeric G proteins by the AT1 receptor are discussed. In addition, recent work examining the role of tyrosine phosphorylation in angiotensin II-mediated signal transduction is also examined. Understanding how these distinct signaling pathways transduce signals from the cell surface will advance our understanding of how such a simple molecule elicits such a wide variety of specific cellular responses.     

Evidence suggesting that galanin (GAL), melanin-concentrating hormone (MCH), neurotensin (NT), proopiomelanocortin (POMC) and neuropeptide Y (NPY) are targets of leptin signaling in the hypothalamus

Leptin (OB protein) reduces food intake by acting at the hypothalamic level. The purpose of the present study was to identify potential targets of leptin signaling in the hypothalamus in ad-lib fed rats. Central administration of leptin (5 microg) for 3 days decreased food intake and body weight gain in association with a decrease in hypothalamic galanin (GAL), melanin-concentrating hormone (MCH), proopiomelanocortin (POMC) and neuropeptide Y (NPY) gene expression and with an increase in neurotensin (NT) gene expression. In pair-fed rats, NPY gene expression was increased and there was no change in either MCH, GAL, POMC or NT gene expression. This study identifies GAL, MCH, POMC and NT as non-NPY targets of leptin signaling and suggests that leptin's action on food intake and body weight is most likely mediated by inhibiting excitatory (e.g. NPY, MCH, GAL, POMC) and stimulating inhibitory (e.g., NT) signals in the feeding circuitry.     

Rehabilitation outcomes of long-term survivors treated for head and neck cancer

BACKGROUND: Little is known about the rehabilitation outcomes of long-term survivors following treatment for head and neck cancer. There are, for example, no studies on physical and psychosocial rehabilitation outcomes of T1 glottic larynx carcinoma, despite the fact that these form the majority of head and neck cancer sites. Thus, this investigation afforded a unique opportunity for examining similarities and differences among T1 glottic larynx patients, laryngectomy patients, and those who had surgery for cancer of the oral cavity and/or oropharynx along a variety of physical and psychosocial dimensions. METHODS: To describe the impact of these three types of head and neck cancer and their treatment on the physical and psychosocial functioning of long-term survivors, a self-report questionnaire was completed by 110 patients treated between 2 and 6 years previously in a major cancer center. RESULTS: Data indicate that a higher percentage of patients treated with laryngectomy or commando procedures still experience severe psychosocial distress between 2 and 6 years after their last treatment than do patients treated with radiotherapy for a T1 carcinoma of the glottic larynx. Psychosocial and physical complaints are still reported by many laryngectomy patients, apparently the result of problems in effective communication with others. Many commando procedure patients experience problems with respect to food intake, and with disfigurement and its consequences. T1 larynx patients mainly experience a considerable number of physical complaints. The greater the time that had elapsed since treatment, the fewer the psychosocial problems associated with head and neck tumors. Open discussion of the illness in the family, social support, and perceptions of adequate information from the specialist are the most important predictors of positive rehabilitation outcomes. CONCLUSIONS: This study indicates that T1 larynx patients report many physical complaints even though several years had elapsed since treatment. Also, laryngectomy patients may need psychosocial guidance for a longer posttreatment period and that health care personnel must involve the partner as much as possible in all communications. Commando procedure patients in particular feel hindered by their disfigurement and its consequences. Future research with respect to validation of the specific head and neck modules is needed.     

The prevalence, structure and clinical problems of multiple sclerosis in the Transcarpathian area based on epidemiological study data

As many as 230 patients with disseminated sclerosis were examined in the Transcarpathian region. Sick male- and female populations were found out to be almost similar. There were more sick persons in the medium-range areas of the Carpathian Mountains, with a mean age being 35 +/- 5 years. It has been established that intake of spirulina makes for lengthening of remission in those patients with disseminated sclerosis.     

Preparation of the myristoylated and nonmyristoylated form of recombinant recoverin in E. coli cells and comparison of their functional activity]

Numerous data strongly suggest the involvement of cytokines and the matrix metalloproteinase collagenase (MMP-1) in the pathogenesis of periodontitis. Recently, we have demonstrated that, upon culturing under the influence of IL-1 alpha + EGF, a large amount of inactive procollagenase (MMP-1) is stored in the extracellular matrix of periosteal tissue. We now show that this endogenous reservoir of proenzyme can be operative after activation with plasmin and is able to induce a rapid and almost complete breakdown of the collagenous extracellular matrix. The level of collagen degradation following activation showed a strong correlation with the amount of proenzyme that was incorporated in the tissue. The highest level of degradation (70% of the total amount of collagenous proteins) was found with the IL-1 alpha + EGF-treated explants, followed by those treated with IL-1 alpha alone (35%). Explants cultured with EGF or in the absence of cytokines, containing only small amounts of procollagenase, showed little collagen breakdown following plasmin activation (7%). Inhibition of metalloproteinases by EDTA, or blockage of plasmin by PMSF, prevented the degradation in all explants irrespective of the amount of proenzyme present in the tissue. Our findings demonstrate that endogenous proenzyme stored in a native connective tissue matrix can be activated at a later time interval which results in a massive breakdown of the tissue. This study shows a possible pathway of collagenase-induced breakdown without recent de novo synthesis of the enzyme. Such a sequence may be operative in chronic inflammatory diseases, such as periodontitis, where production of procollagenase under the influence of cytokines spans a longer time period, whereas breakdown is often characterized by a cyclic behaviour.     

Quantitative determination of spleen added to ground beef

Simultaneous measurement of cardiac output distribution with 86Rubidium and 57Cobalt-tagged microspheres in rats implanted with liver tumors by intraportal injection of sarcoma cells enables quantitation of arterial and portal tumor circulation. The portal circulation was found to be increased in small tumors as compared to the liver, but as the tumor grew there was a decrease in the portal tumor circulation. When the tumor growth became massive even the total liver circulation was reduced, as measured with 133Xenon wash-out. All the tumors had increased arterial circulation. This arterial hyperperfusion was changed into ischemia when the liver artery was occluded through embolization with degradable microspheres.