OBJECTIVE. To examine the effect of adenotonsillectomy (T&A) in children with obstructive sleep-disordered breathing on growth, hyperactivity, and sleep and waking motor activity. METHODS. We studied ...54 children who were aged 6 to 12 years and had adenotonsillar hypertrophy and an obstructive apnea-hypopnea index of greater than or equal to 1 before and 12 months after they all received adenotonsillectomy (T&A). We measured their height, weight, percentage overweight (patient BMI--BMI at 50th percentile)/BMI at 50th percentile * I00) and obtained a hyperactivity score from parent report on a standardized behavior questionnaire scale. A subset of 21 of these children were also studied for motor activity by wrist actigraphy for 7 consecutive days and nights before and 12 months after T&A. RESULTS. After T&A, mean obstructive apnea-hypopnea index decreased from 7.6 to 0.6. Height percentile did not change, but weight percentile increased; as a consequence, percentage overweight increased from 32.0% to 36.3%. Hyperactivity scores and total daily motor activity were reduced after T&A. From linear regression, the reduction in hyperactivity scores predicted an increase in percentage overweight. Reduced motor activity was correlated with increased percentage overweight. CONCLUSIONS. An increase in percentage overweight after T&A in children with obstructive sleep-disordered breathing is correlated to decreased child hyperactivity scores and to decreased measured motor activity in the subset studied. These associations suggest that the increase in overweight may be attributable to reductions in physical activity and fidgeting energy expenditure. KEY WORDS. sleep apnea, growth, height, weight, obesity, physical activity, hyperactivity, nonexercise activity thermogenesis. PEDIATRICS 2006;117:e200-e208. URL: www.pediatrics.org/cgi/doi/10.1542/peds.2005-1007
Pineocytomas are pineal parenchymal tumors composed of differentiated cells histologically similar to those of the mature pineal gland. Little is known about the incidence, pattern of growth, or ...response to treatment of pineocytomas. Between 1975 and 1985, six children with pineocytomas were treated at our institution, and pineocytomas constituted 11% of all newly diagnosed pineal region tumors. The clinical, radiographic, and pathologic features of these six patients with pineocytomas are presented. Initial treatment for these children included craniospinal plus supplemental local radiotherapy (three), local radiotherapy alone (one), or radiation therapy plus adjuvant chemotherapy (two). Four of six patients had tumor recurrence a median of two years after diagnosis. Three patients had leptomeningeal dissemination, one at the time of diagnosis and two following therapy. Our findings suggest that: (1) biopsy is necessary to distinguish pineocytomas from other pineal region tumors; (2) radiation therapy alone is inadequate; and (3) these tumors are aggressive in the pediatric population, with a high propensity for leptomeningeal dissemination.
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BFBNIB, FZAB, GIS, IJS, KILJ, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
A 50-year-old man was referred because of a painful lytic bone lesion of the right ulna and multiple hepatic masses that had been identified on computed tomography. Biopsy of the bone lesion ...disclosed adenocarcinoma. Fanconi's anemia had been diagnosed 10 years earlier, and squamous-cell carcinoma of the esophagus 4 years earlier. A diagnostic procedure was performed.
The human population is growing, and as a result we need to produce more food whilst reducing the impact of farming on the environment. Selective breeding and genomic selection have had a ...transformational impact on livestock productivity, and now transgenic and genome-editing technologies offer exciting opportunities for the production of fitter, healthier and more-productive livestock. Here, we review recent progress in the application of genome editing to farmed animal species and discuss the potential impact on our ability to produce food.
JAK2, a member of the Janus kinase super-family was found to interact functionally with Raf-1, a central component of the ras/mitogen-activated protein kinase signal transduction pathway. ...Interferon-$\gamma $ and several other cytokines that are known to activate JAK2 kinase were also found to stimulate Raf-1 kinase activity toward MEK-1 in mammalian cells. In the baculovirus coexpression system, Raf-1 was activated by JAK2 in the presence of p21$^{\text{ras}}$. Under these conditions, a ternary complex of p21$^{\text{ras}}$, JAK2, and Raf-1 was observed. In contrast, in the absence of p21$^{\text{ras}}$, coexpression of JAK2 and Raf-1 resulted in an overall decrease in the Raf-1 kinase activity. In addition, JAK2 phosphorylated Raf-1 at sites different from those phosphorylated by pp60$^{\text{v-src}}$. In mammalian cells treated with either erythropoietin or interferon-$\gamma $, a small fraction of Raf-1 coimmunoprecipitated with JAK2 in lysates of cells in which JAK2 was activated as judged by its state of tyrosine phosphorylation. Taken together, these data suggest that JAK2 and p21$^{\text{ras}}$ cooperate to activate Raf-1.
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BFBNIB, NMLJ, NUK, PNG, SAZU, UL, UM, UPUK
Fanconi anemia (FA) is an autosomal recessive cancer susceptibility syndrome with eight complementation groups. Four of the FA genes have been cloned, and at least three of the encoded proteins, ...FANCA, FANCC, and FANCG/XRCC9, interact in a nuclear complex, required for the maintenance of normal chromosome stability. In the current study, mutant forms of the FANCA and FANCG proteins have been generated and analyzed with respect to protein complex formation, nuclear translocation, and functional activity. The results demonstrate that the amino terminal two-thirds of FANCG (FANCG amino acids 1-428) binds to the amino terminal nuclear localization signal (NLS) of the FANCA protein. On the basis of 2-hybrid analysis, the FANCA/FANCG binding is a direct protein-protein interaction. Interestingly, a truncated mutant form of the FANCG protein, lacking the carboxy terminus, binds in a complex with FANCA and translocates to the nucleus; however, this mutant protein fails to bind to FANCC and fails to correct the mitomycin C sensitivity of an FA-G cell line. Taken together, these results demonstrate that binding of FANCG to the amino terminal FANCA NLS sequence is necessary but not sufficient for the functional activity of FANCG. Additional amino acid sequences at the carboxy terminus of FANCG are required for the binding of FANCC in the complex.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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