This excellent metabolic control was associated with a markedly improved general condition, mood and behavior, and small developmental achievements. Twelve years after liver transplantation, the patient has a stable cognitive impairment without progression of spastic diplegia.
 
  This first case of liver transplantation in HHH syndrome demonstrates that this procedure is a therapeutic option for HHH patients with difficult metabolic control.
 This first case of liver transplantation in HHH syndrome demonstrates that this procedure is a therapeutic option for HHH patients with difficult metabolic control.
  The purpose of this study was to examine the reliability and validity of the Korean version of the Illness Cognition Questionnaire.
 
  A total of 237 adolescent Participants ages 13-20 years were collected from two hospitals for purposes of the study. The participants were diagnosed with blood cancer, congenital heart disease, paediatric rheumatoid arthritis, multiple sclerosis and diabetes mellitus.
 
  The Illness Cognition Questionnaire is composed of three subscales and 18 items. Exploratory factor analysis and confirmatory factor analysis were performed for all 18 items. The data used in the exploratory factor analysis were obtained from 126 adolescents with blood cancer. The data used for confirmatory factor analysis were obtained from 111 adolescents who had chronic diseases. The three-factor model of 18 items showed general fitness close to the standard but not a very good fit.
 
  This study indicated that the Korean version of the Illness Cognition Questionnaire is reasonable to use for Korean adolescents with chronic illness. The authors recommend that the meaning of Item 10 be clarified from 'I have learned to accept the limitations imposed by my illness' to 'I have learned to positively accept the limitations imposed by my illness'.
 This study indicated that the Korean version of the Illness Cognition Questionnaire is reasonable to use for Korean adolescents with chronic illness. The authors recommend that the meaning of Item 10 be clarified from 'I have learned to accept the limitations imposed by my illness' to 'I have learned to positively accept the limitations imposed by my illness'.Extracellular vesicles (EVs), a broad term for the lipid microparticles known as microvesicles and exosomes, are discharged by cells into their surrounding space. Microvesicles are discharged upon outward plasma membrane budding, while exosomes are secreted after multivesicular body (MVB) fusion with the plasma membrane. The majority of information regarding EV biology comes from studies performed in non-polarized cells. Here we characterize EV release in polarized cells. We found a substantial asymmetry in the number and composition of EVs produced and released from the apical membrane of epithelial cells as compared to the basolateral membrane. We showed that the quantitative difference is related to the polarized distribution of two phosphoinositide species between the two cell surfaces and that the peculiar biochemical composition of resultant EVs reflects their site of origin. In particular, apical and basolateral exosomes may derive from distinct classes of MVBs originating from and fusing with the same plasma membrane. We identify VAMP8/Endobrevin as a regulator of the basolateral release of exosomes, whereas the mechanism responsible for apical EV release requires further study.
  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare inherited disease caused by NOTCH3 gene mutations. Although the main clinical features reflect brain injury, CADASIL is a systemic microangiopathy, and cardiac involvement has been observed but not systematically assessed. We aimed to study the prevalence and severity of coronary microvascular dysfunction (CMD) in CADASIL patients.
 
  Seventeen patients with genetically confirmed CADASIL, aged <60years (mean age 40±9years), with ≤1 cardiovascular risk factor underwent neurological and neuropsychological evaluation, 3T brain magnetic resonance imaging (MRI), 12-lead electrocardiography (ECG), standard echocardiography, and measurement of myocardial blood flow at rest (resting MBF) and of maximal myocardial blood flow following Regadenoson infusion (Reg-MBF) by 
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  positron emission tomography (PET). Coronary flow reserve (CFR) was defined as Reg-MBF/resting MBF.  https://www.selleckchem.com/products/talabostat.html  PET results were compared to thtemic microcirculation disease, and active surveillance of cardiac symptoms should be considered in these patients.
  Anterior rectocele is usually an asymptomatic condition in many women, yet it can be associated with obstructed defaecation syndrome (ODS). Transperineal repair of rectocele (TPR) has been followed by variable rates of improvement in ODS. The present pilot randomized clinical trial aimed to evaluate the outcome of TPR with vertical plication (VP) of the rectovaginal septum compared to horizontal plication (HP).
 
  Adult women with anterior rectocele were recruited to the study and were randomly allocated to one of two equal groups. The first group underwent TPR with VP of the rectovaginal septum and the second group underwent TPR with HP. The main outcome measures were improvement in ODS, recurrence of rectocele, complications and dyspareunia.
 
  The trial included 40 female patients with anterior rectocele. There was no significant difference between the two groups regarding the postoperative Wexner score. Complete cure and significant improvement in ODS symptoms were comparable after the two techniques. The reduction in rectocele size after HP was significantly greater than after VP (1.7 vs. 2.6, P<0.0001). Significant improvement in dyspareunia was recorded after HP (P=0.001) but not after VP (P=0.1). There was no significant difference between the two groups with regard to operating time, complications and recurrence.
 
  VP and HP of the rectovaginal septum in TPR were associated with a comparable improvement in ODS symptoms and similar complication rates. HP was followed by a greater reduction in the rectocele size and greater improvement in dyspareunia than VP.
 VP and HP of the rectovaginal septum in TPR were associated with a comparable improvement in ODS symptoms and similar complication rates. HP was followed by a greater reduction in the rectocele size and greater improvement in dyspareunia than VP.