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Objective: To determine whether the onset of rapid eye movement (REM) sleep behavior disorder (RBD) is associated with changes in brainstem neuronal pathway dysfunction as reflected by vestibular-evoked myogenic potentials (VEMPs) and to evaluate associations between VEMPs and REM sleep without atonia (RSWA) in patients with early-stage Parkinson's disease (PD) and isolated RBD (iRBD)., Methods: Eighty-two early-stage PD patients, 40 iRBD patients, and 41 healthy control individuals underwent one-night video-polysomnography (vPSG) and VEMPs examination. We compared cervical (cVEMP), ocular (oVEMP), and masseter (mVEMP) VEMP parameters among PD with RBD (PD + RBD), PD without RBD (PD-RBD), iRBD, and control groups and analyzed correlations between VEMPs and RSWA in PD and iRBD groups., Results: The PD + RBD group showed delays in bilateral cVEMP (Lp13, Ln23, Rn23: all p < 0.05) and oVEMP (Ln10, Rn10, Rp15: all p < 0.05) peak latencies compared with the PD-RBD group. Total cVEMP scores were higher in the PD + RBD group than in the iRBD group (p = 0.033). In PD patients, phasic RSWA was correlated with total cVEMP scores (p = 0.003), and tonic RSWA was correlated with left oVEMP scores (p = 0.013)., Conclusions: Brainstem neurophysiology as evidenced by altered VEMPs in patients with PD and iRBD could reflect disease evolvement. Moreover, VEMPs alterations may vary depending on the presence of RBD in PD patients. The associations between altered RSWA and VEMP parameters highlight the meaningfulness of detecting brainstem dysfunction in early-stage PD., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Introduction: Loss of REM sleep muscle atonia (RWA) and dream-enactment behavior (DEB) are two associated features of REM sleep behavior disorder (RBD), which is frequently associated with Parkinson's disease (PD). Few studies have examined both DEB and RWA simultaneously in patients with PD. This study aimed to evaluate relationships between RWA, DEB and clinical characteristics of PD., Methods: We conducted overnight polysomnography in 145 patients with PD. DEB (motor behaviors and/or vocalizations during REM) and increased RWA (IRWA; tonic and phasic chin EMG density ≥ 30% and ≥15%, respectively) were identified. Patients were categorized as clinical RBD (DEB and IRWA), sub-DEB positive (DEB only), subclinical RBD (IRWA only), or normal REM sleep., Results: Patients with DEB had higher Hoehn and Yahr (H&Y) stage, Unified Parkinson's Disease Rating Scale (UPDRS) III score, levodopa equivalent dose(LEDs), and worse cognition. RWA was associated with H&Y stage, LEDs, cognition, and sleep structure in all patients. PD duration was associated with RWA, but not DEB. The PD patients who exhibited clinical or subclinical RBD, compared to sub-DEB positive, had higher H&Y stage, UPDRS III score and LEDs, lower cognitive score, worse sleep structure than the PD + cREM group., Conclusion: Both DEB and RWA were associated with severity of PD illness. Subclinical RBD might have different disease progression from sub-DEB positive. DEB symptoms may fluctuate or disappear whereas RWA may continue to develop as PD progresses. Differences in the course of DEB and RWA may reflect the difference in the degeneration process of neurodegenerative disorders., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Objective: To analyze our experiences of pediatric testicular tumors and investigate the management of pediatric testicular germ cell tumors. Pediatric testicular tumors are rare and the treatment of them has not been well defined., Methods: Children treated for primary testicular tumors between January 1998 and July 2010 were retrospectively analyzed. For yolk sac tumor, the difference of survival rates between patients with and without retroperitoneal lymph node dissection (RPLND) was calculated., Results: Eighty-seven cases met our criteria and 78 were germ cell tumors, including 40 cases with yolk sac tumor. Patients were 3-128 months old (median 19), and 53 patients were diagnosed at younger than 2 years of age. For germ cell tumors, serum α-fetoprotein and β-human chorionic gonadotropin were elevated in 48 and 7 patients, respectively, including 38 and 2 in those with yolk sac tumor. RPLND and chemotherapy were performed in 13 and 19 patients, respectively, and surveillance was performed in 50 patients. With median follow-up of 50 months, 6 patients had recurrence, 4 patients died, and the others achieved complete remission. For stage I yolk sac tumor, the difference of survival rates between patients with and without RPLND was not significant (P = .808)., Conclusion: Yolk sac tumor is the most common type of pediatric testicular tumor. For stage I yolk sac tumor, radical inguinal orchiectomy is effective, salvage chemotherapy is promising, and RPLND may not be necessary., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Pulmonary hypertension is associated with remodeling of the smooth muscle layer of pulmonary arteries, manifested by reduced smooth muscle cell (SMC) contractility and enhanced motility and growth. These responses are underlied by increased dynamics of the peripheral actin network. Thus, we hypothesized that pulmonary hypertension is associated with upregulation of two proteins that regulate the dynamics of peripheral actin filaments, i.e., profilin and cofilin. We also analyzed the expression of LIMK2, which regulates the actin remodeling capacity of cofilin by phosphorylation. Experimental inflammation was induced by incubation of cultured pulmonary artery SMCs (PASMCs) with inflammatory mediators in vitro, and by subcutaneous administration of monocrotaline to Sprague-Dawley rats in vivo. Expression of messenger RNA (mRNA) was assessed by quantitative RT-PCR, protein levels and phosphorylation were analyzed by immunoblotting. Immune and Masson trichrome stained lung cryosections were analyzed by microscopy. PDGF, IL-1beta, ET-1 and TNFalpha upregulated the profilin, cofilin-2 and LIMK2 mRNA in cultured pulmonary artery SMCs (PASMCs). Along with the development of rat pulmonary artery and right ventricular hypertrophy, monocrotaline treatment also induced the mRNA and protein contents of profilin, cofilin-2 and LIMK2 in PASMCs. The cofilin upregulation was paralleled by a relative decrease of the phospho-cofilin content. The upregulation of profilin, cofilin and LIMK2 in experimental inflammation suggests that by intensifying the remodeling of subcortical actin filaments these proteins may contribute to the enhanced invasiveness and growth of SMCs, and to the development of increased vascular resistance and pulmonary hypertension.

Incorporation of pyrimidine ribonucleotides in Giardia intestinalis occurs via uracil phosphoribosyltransferase (UPRTase). The enzyme was purified over 1000-fold to apparent homogeneity from parasite extracts, using Fast Protein Liquid Chromatography, namely Mono Q anion exchange, Mono P chromatofocusing and Superose 12 chromatography. The specific activity of the purified enzyme was 3100 nmol min-1 mg protein-1. The enzyme was found to be a dimer of mol. wt. 76,000. Kinetic analysis, including initial velocity and product inhibition studies, indicated that it obeyed a rapid-random equilibrium mechanism. GTP and dGTP caused a dramatic increase in the activity of the enzyme, though there was no effect on the Michaelis constants. All other nucleotides tested were without effect or were inhibitory. The effect of GTP is similar to that observed for UPRTase from E. coli but not from other eukaryotes.