Quality of life and phonatory and morphological outcomes in cognitively unimpaired adolescents with Pierre Robin sequence: a cross-sectional study of 72 patients.

BACKGROUND: Pierre Robin sequence (PRS) is a heterogeneous condition involving retro(micro)gnathia, glossoptosis and upper airway obstruction, very often with posterior cleft palate. Patients with PRS, either isolated or associated with Stickler syndrome have good intellectual prognosis. Nevertheless, the quality of life in adolescence and the phonatory and morphological outcomes are rarely analysed. We assessed the phonatory and morphological outcomes of 72 cognitively unimpaired adolescents with PRS, studied their oral (COHIP-SF19), vocal (VHI-9i) and generic quality of life (QoL; KIDSCREEN-52), and searched for determinants of these outcomes. RESULTS: Two-thirds of our adolescents retained low or moderate phonation difficulties, but risk factors were not identified. For 14%, morphological results were considered disharmonious, with no link to neonatal retrognathia severity. Only one vs two-stage surgery seemed to affect final aesthetic results. The oral QoL of these adolescents was comparable to that of control patients and was significantly better than that of children with other craniofacial malformations (COHIP-SF19 = 17.5, 15.4 and 25.7, respectively). The oral QoL of the adolescents with non-isolated PRS was significantly worse (COHIP-SF19 = 24.2) than that of control patients and close to that of children with other craniofacial malformations. The vocal QoL of the adolescents (mean [SD] VHI-9i = 7.5 [5.4]) was better than that of patients with other voice pathologies and better when phonation was good. The generic QoL of the adolescents was satisfactory but slightly lower than that of controls, especially in dimensions concerning physical well-being, relationships and autonomy. QoL results were lower for adolescents with non-isolated than isolated PRS. Only non-isolated PRS and low oral QoL affected generic QoL. CONCLUSION: Morphological or phonatory impairments remain non-rare in adolescents with PRS but do not seem to be directly responsible for altered QoL. These adolescents, especially those with non-isolated PRS, show self-confidence and social-relation fragility. We must focus on long-term functional and psychological results for PRS patients and improve therapy protocols and follow-up, notably those affecting the oral aspects of the disease.

A Program for Weaning Children from Enteral Feeding in a General Pediatric Unit: How, for Whom, and with What Results?

OBJECTIVE: To describe a series of children who were hospitalized for a tube-weaning program in the general pediatric ward of a pediatric tertiary university hospital: describe our method, to determine the success rate of our inpatient pediatric tube weaning program, and search for relevant factors linked to its success or failure. METHOD: We analyzed the medical files of consecutive children who were hospitalized for gastric-tube weaning over an 8-year period. We analyzed outcomes in terms of feeding and growth with at least 2 years of data. Success (weaning within 3 months) and failure were compared by characteristics of children. RESULTS: We included 37 children (29 females) with mean (SD) age 31.4 (21) months. Most had a severe medical history (30% prematurity; 50% intrauterine growth restriction, 50% neurological and genetic anomalies). The weaning program was successful for half of the children. Factors linked to success of the program were female sex (p = 0.0188), normal neurodevelopment (p = 0.0016), nasogastric tube (p = 0.0098), and with <24 months on EF before the stay (p = 0.0309). DISCUSSION: Comparing the efficiency of various methods and results among teams was difficult, which indicates the need to establish consensus about the outcome criteria. We confirm the need for these types of stays and programs.

Is there any impact of cognitive remediation on an ecological test in schizophrenia?

INTRODUCTION: Cognitive deficits are commonly reported in schizophrenia and have a significant impact on the daily life of patients and on their social and work inclusion. Cognitive remediation therapies (CRT) may enhance the capabilities of schizophrenia patients. Although social and work integration is the ultimate goal of CRT, previous studies have failed to carry out a detailed assessment of the effects on everyday life. METHODS: Fifty-nine schizophrenia patients were randomised into two groups (remediation or usual treatment) to test the effects of a new remediation programme, which included both rehearsal and strategy learning, on cognitive functions. An ecological test was used to evaluate its transfer to daily living skills. RESULTS: Cognitive improvements are revealed in CRT patients, mainly in memory and executive functions. Patients showing some deficiencies to perform the ecological test had better scores after the CRT. Moreover, they significantly improve their social activity scores. CONCLUSIONS: CRT would facilitate mental load monitoring by enhancing or reallocating cognitive resources, facilitating the patient's organisation and autonomy. The rehearsal learning approach improves the ability to carry out automatic operations that are less demanding in terms of cognitive resources, thereby increasing the resources available for acquisition and efficient use of strategies provided during the strategy learning approach.

Explicit and implicit emotional processing modifications in pharmacoresistant left temporal lobe epilepsy and anxiodepressive disorders.

We examined whether anxiodepressive patients with left temporal lobe epilepsy could be differentiated from those with depression but without epilepsy on tasks that investigate attentional bias toward and explicit judgment of emotional stimuli. Eight depressive patients, eight anxiodepressive patients with epilepsy, and eight controls participated in the present study. Anxiodepressive with epilepsy and depressive patients had comparable depression scores and the same cognitive profile. Two distinct emotional tasks were used: the decision lexical task and the number comparison task. Three emotional connotations were presented: neutral, positive, and negative. The pattern of results showed an attentional bias toward negative words and pictures in depressive patients and only toward negative words in anxiodepressive patients with epilepsy. Moreover, depressive patients explicitly judged negative stimuli with lower intensity and anxiodepressive patients judged neutral stimuli with higher intensity. The present study specifies the emotional functioning in depression with or without left temporal lobe epilepsy.

Modulation of the default mode network is task-dependant in chronic schizophrenia patients.

The activity of brain regions of the so-called default mode network (DMN) attenuates during the performance of goal-directed tasks. These activity decreases (named task-induced deactivations; TID) are though to reflect the reallocation of cognitive resources from the DMN to areas implicated in the execution of the task. Recently, DMN activity suppression has been studied in schizophrenia patients. Although these works showed that TID are altered in schizophrenia, they also revealed inconsistent findings. We hypothesized that reallocation of resources is altered in schizophrenia patients and is context or task specific. We investigated TID using functional MRI in 26 schizophrenic patients and 13 control subjects while performing two different goal-directed tasks (the Hayling Sentence Completion Test and the N-Back task). Both whole brain and region of interest conjunction analyses were conducted to investigate brain areas commonly deactivated in the two tasks (task unspecific deactivations). Task-unspecific deactivations were not observed in the schizophrenia group, although these were strongly significant in the control group. Differences between patient and control participants were observed in different regions of the DMN depending whether the subjects performed the Hayling or the N-back task. These results suggest that reallocation of cognitive resources is altered in our patient sample. Moreover, TID were task-unspecific indicating that resources reallocation is context dependent in schizophrenia. DMN activity attenuates differently in schizophrenia patients depending on the cognitive processes involved in the task.

Brain activation during executive processes in schizophrenia.

Schizophrenia patients show some deficits in executive processes (impaired behavioural performance and abnormal brain functioning). The aim of this study is to explore the brain activity of schizophrenia patients during different inhibitory tasks. We used functional magnetic resonance imaging to investigate to investigate the restraint and deletion aspects of inhibition in 19 patients with schizophrenia and 12 normal subjects during the performance of the Hayling and the N-back tasks. The patients demonstrated impaired performance (more errors and longer reaction times) in the Hayling task. Schizophrenia subjects activated the same fronto-parietal network as the control subjects but demonstrated stronger parietal activations. For the N-back task, the deficit shown by the patients was limited to the number of target omissions. The reaction times and the number of false alarms did not differ in the two groups. We interpret this pattern of deficit as an alteration of working memory processes (and unaltered inhibition). Schizophrenia subjects showed higher activations in a fronto-parietal network. Since schizophrenia patients reached normal inhibitory performances in the N-back task and not in the Hayling task, the frontal hyperactivation may reflect an increased effort or a compensatory mechanism that facilitates the performance of executive tasks. During the Hayling task, this frontal hyperactivation was not achieved, and its absence was associated with a performance deficit relative to the performance of normal subjects.