[Pulmonary siderosis in a welder, diagnosis on unusual pneumopathy]. / Pneumopathie atypique chez un soudeur : sidérose pulmonaire ?

INTRODUCTION: Pulmonary siderosis or welder's lung is an occupational lung disease that is usually observed after chronic exposure to iron dust. OBSERVATION: A 55-years-old welder visited hospital with dyspnea linked to occupational exposure. Pulmonary function studies revealed lung function abnormalities with decerase of FEV1 and TPC. Based on the chest Tomography CT results, he was diagnosed with obliterans bronchiolitis. A chest biopsy was performed and the specimen is for a pulmonary siderosis aspect. DISCUSSION: This case of pulmonary siderosis is an unusual one by symptoms, CT images and short latency of exposure. An intense inhalation of iron particles could explain this case and inflammatory process and it highlights need of histological analysis of chest biopsy.

[A very unusual pleural presentation]. / Une pleurésie très inhabituelle.

Urinothorax refers to the presence of urine in the pleural space. Urinothorax is an infrequent and underdiagnosed pathology, with few cases reported, and these often suspected only with hindsight. It is usually a transudative pleural effusion. We report a case of urinothorax presenting as a purulent pleural effusion. Management of the urinothorax required antibiotics and surgical unblocking of the urinary tract. Currently, no test is available to confirm the diagnosis. The ratio of serum creatinine/pleural creatinine could suggest the presence of urinothorax but this parameter needs to be validated by complementary studies. Urinothorax should be suspected in the context of pleural effusion occurring after a recent urologic surgery.

[Pulmonary Mycobacterium abscessus sensu lato infection in patients without cystic fibrosis]. / Infection pulmonaire à Mycobacterium abscessus sensu lato chez des patients exempts de mucoviscidose.

INTRODUCTION: Infection with Mycobacterium abscessus sensu lato is uncommon in patients without cystic fibrosis. We are interested in these patients and have collected cases in Finistère between 2007 and 2011. CASE REPORTS: Four patients met the infection criteria recommended by the American Thoracic Society in 2007. Among them, all had Aspergillus spp. in sputum, 3 had gastroesophageal reflux and two had the criteria for allergic bronchopulmonary aspergillosis. We identified Mycobacterium massiliense in the single patient in our series whose therapeutic outcome was successful. CONCLUSION: By comparing these data with those in the literature, we believe that the search for allergic bronchopulmonary aspergillosis and gastroesophageal reflux is necessary in these patients and that species identification is essential for prognosis.

[Pulmonary arterial hypertension in the elderly subject]. / Hypertension artérielle pulmonaire du sujet âgé. La cohorte d'un centre régional.

OBJECTIVE: To describe the features of pulmonary arterial hypertension (PAH) in elderly patients. METHODS: A single centre, descriptive study of PAH patients consecutively referred to a regional centre, from September 2002 to February, 1st, 2009. The group of patients aged 65 and above at the time of the diagnosis was compared to the younger patients. RESULTS: Sixty-six patients suffering from PAH (group 1) have been investigated by means of right heart catheterisation. There were 24 patients aged 65 and above. Mean pulmonary arterial pressure was lower in the patients aged over 65. The older patient group had more respiratory and/or cardiac co-morbidities, a lower median distance in the 6minute walk test and a higher median Pro-BNP level. Specific PAH treatments were prescribed in both groups. Fifteen patients aged 65 and above were on long-term oxygen therapy (vs four younger patients, p<0.0001). The elderly patients had a median survival of 32 months. CONCLUSION: The diagnosis of PAH in elderly patients is associated with a poor prognosis. The management of these patients needs further studies.

Posthypnotic amnesia for material learned before or during hypnosis: explicit and implicit memory effects.

This article focuses on dissociations between explicit and implicit expressions of memory during posthypnotic amnesia (PHA). Despite evidence of such dissociations, experimental design in this area has not always been consistent with contemporary memory research. Within a paradigm that aimed for conceptual and methodological clarity, we presented 40 high and 38 low hypnotizable individuals with a word list either before or during hypnosis, gave them a PHA suggestion for the word list, and tested them on explicit and implicit memory tasks. In the absence of conscious recollection, highs showed equivalent levels of priming (perceptual and semantic) to lows. However, when analysis focused only on those highs who remained amnesic after the implicit memory tasks, we confirmed perceptual, but not semantic, priming. These findings highlight the impact of methodological choices on theoretical interpretations of memory performance following a suggestion for PHA.

Posthypnotic responding: the relevance of suggestion and test congruence.

Thirty real, hypnotized subjects and 34 simulating, unhypnotized subjects were given either a suggestion to respond when they heard a cue (general) or a suggestion to respond when they heard a cue after hypnosis (posthypnotic). Half the subjects were given the cue during hypnosis (hypnotic test) and half were given it after hypnosis (posthypnotic test). Those who were given the cue during hypnosis were also given it after hypnosis. Between- and within-group comparisons were made of subjects' behavioral responses, latencies to respond, and ratings of experiential compulsion. The findings indicated that subjects' behavior and experience were influenced by congruence between information conveyed by the suggestion and the test about when and how they should respond.

Information processing during hypnotically suggested sex change.

During hypnotically suggested sex change, 36 real (12 virtuoso and 24 high hypnotizable) and 18 simulating (low hypnotizable) individuals listened to a story involving a male and a female character. They subsequently reported their experience and recall of the story. Virtuosos were less likely than highs and simulators to identify with the character consistent with their suggested sex. However, virtuosos recalled more information about the character consistent with their suggested sex than did highs and simulators. The authors discuss the findings in terms of attention and the selective processing of information during hypnosis. They conclude that character identification was not the major factor that influenced the recall of virtuosos and suggest that virtuosos may have processed aspects of the information in a more self-referential way and thus encoded and recalled it more effectively.

Indexing the experience of sex change in hypnosis and imagination.

The authors suggested a change of sex to high hypnotizable participants in hypnosis and imagination conditions and indexed the subjects' experiences with a continuous, concurrent behavioral measure that involved them turning a dial to indicate changes in the strength of the suggested effect. In addition, the researchers indexed the participants' experiences through retrospective ratings of realness, involuntariness, and active thinking. The dial rating showed that the onset of the experience was more rapid for hypnotic than for imagination participants. Moreover, there were differences in the relationship between dial ratings and retrospective ratings across the conditions as well as across the suggestion, test, and cancellation phases of the item. The findings are discussed in terms of how the dial method provides a better understanding of suggested sex change as well as a better understanding of the private experience of hypnosis and imagination.

Specific autobiographical memory following hypnotically induced mood state.

This study investigated the impact of hypnotically induced mood on the specificity of autobiographical memory. High (n = 24) and low (n = 21) hypnotizable participants were administered a hypnotic induction for sad, neutral, or happy mood and were asked to retrieve specific autobiographical memories in response to positive and negative cue words. Whereas high hypnotizable participants in the sad condition provided fewer specific memories in response to positive rather than negative cues, those in the neutral and happy conditions responded similarly to positive and negative cues. Findings suggest that impaired recall of specific memories may be mediated by state factors associated with sad mood. These results point to the utility of hypnotic mood induction as a means to experimentally investigate the relationship between mood and autobiographical memory.

Identification of four novel PMM2 mutations in congenital disorders of glycosylation (CDG) Ia French patients.

We screened 11 unrelated French patients with congenital disorders of glycosylation (CDG) Ia for PMM2 mutations. Twenty one missense mutations on the 22 chromosomes (95%) including four novel mutations were identified: C9Y (G26A) in exon 1, L32R (TA95GC) in exon 2, and T226S (C677G) and C241S (G722C) in exon 8. We studied the PMM activity of these four novel mutant proteins and of the R141H mutant protein in an E coli expression system. The T226S, C9Y, L32R, and C241S mutant proteins have decreased specific activity (23 to 41% of normal), are all more or less thermolabile, and R141H has no detectable activity. Our results indicate that the new mutations identified here are less severe than the inactive R141H mutant protein, conferring residual PMM activity compatible with life.

Defect in N-glycosylation of proteins is tissue-dependent in congenital disorders of glycosylation Ia.

The biochemical hallmark of Congenital Disorders of Glycosylation (CDG) including type Ia is a defective N-glycosylation of serum glycoproteins. Hypoglycosylated forms of alpha1-antitrypsin have been detected by Western blot in serum from CDG Ia patients. In contrast we were not able to detect hypoglycosylation in alpha1-antitrypsin synthesized by fibroblasts, keratinocytes, enterocytes, and leukocytes. Similarly no hypoglycosylation was detectable in a membrane-associated N-linked glycoprotein, the facilitative glucose transporter GLUT-1 and also in serum immunoglobulin G isolated from sera of CDG Ia patients. We conclude that the phenotypic expression of CDG Ia is tissue-dependent.

Eliciting autobiographical pseudomemories: the relevance of hypnosis, hypnotizability, and attributions.

The authors investigated the roles of hypnosis, hypnotizability, and attributions in autobiographical pseudomemories. Experiment 1 administered a suggestion for recall of their second birthday to hypnotized high and low hypnotizable participants and nonhypnotized, high hypnotizable participants; Experiment 2 administered a similar suggestion to real and simulating participants. Recall was tested during hypnosis, after hypnosis, and after a challenge procedure. In Experiment 1, more highs than lows reported a memory during hypnosis; however, following the challenge, half the walking highs but none of the hypnosis highs retracted their memory. Notably, highs attributed their memories to reconstructions based on other birthdays. In Experiment 2, whereas an equal number of reals and simulators reported a memory of their second birthday during hypnosis and then retracted following the challenge, they made different attributions about their memories. These findings highlight the value of a closer investigation of attributional processes that reconcile believed-in autobiographical memories with conflicting evidence.

Autobiographical remembering and forgetting: what can hypnosis tell us?

Autobiographical memory can be characterized in terms of its reconstructive nature, its relationship with self-identity, and its shifting accessibility. Hypnosis research on personal memory has focused for the most part on its reconstructive nature. The authors examine selected contributions of hypnosis research to understand the nature and function of autobiographical memory and consider further ways in which hypnosis can make specific contributions to theoretical understanding and empirical inquiry into personal recollection. The authors provide some examples of research on various aspects of hypnosis and autobiographical memory and suggest particular ways for adding to the value and impact of such work. They argue that hypnosis researchers should continue to look for ways in which they can demonstrate and communicate the vigor and relevance of their work.

Characterization of the 415G>A (E139K) PMM2 mutation in carbohydrate-deficient glycoprotein syndrome type Ia disrupting a splicing enhancer resulting in exon 5 skipping.

Carbohydrate-deficient glycoprotein syndrome type Ia (CDGS) is an autosomal recessive disorder, characterized by a central nervous system dysfunction and multiorgan failure associated with defective N-glycosylation and phosphomannomutase (PMM) deficiency related to mutations in the PMM2 gene. A total of 26 different missense mutations and one single base pair deletion have already been described. We found by sequencing and restriction analysis, in two unrelated French patients with CDG type Ia a compound heterozygosity for two mutations in exon 5: a new mutation 415G>A (E139K) and the most frequent mutation 425G>A (R141H ). The 415G>A mutation disrupted a splicing enhancer sequence: (GAR)n-(GAR)n resulting in exon 5 skipping. We studied the activity of these mutant proteins expressed in E Coli. Compared to the normal PMM protein activity, the R141H and transcript without exon 5 expressed a protein with undetectable specific activity when the E139K mutant protein expressed a residual activity of 25%. The E139K mutant protein could be expressed at a sufficient level in vivo to confer residual activity compatible with life in these patients when absence of residual PMM activity is likely lethal.

Measuring change in the subjective experience of hypnosis.

The authors indexed the subjective experience of hypnosis through the use of a continuous behavioral measure of the strength of the participant's experience at the time of the suggestion. Specifically, subjects turned a dial to indicate changes in their experience of the suggested effect during that experience. Thirty-three high, 47 medium, and 28 low hypnotizable subjects were asked to use the dial during the suggestion, test, and cancellation phases of three hypnotic items: arm levitation, arm rigidity, and anosmia. The pattern of ratings differed according to the nature of the suggestion. Also, across the items, subjects who passed according to behavioral criteria experienced the suggested effect to a greater degree than those who failed. Notably, whereas the ratings of highs and mediums did not differ for any item, they differed from lows on all three items. The authors discuss the implications of these findings in terms of the potential for this method to provide insight into the experience of hypnosis.

Posthypnotic amnesia for material learned before hypnosis.

The impact of a suggestion for posthypnotic amnesia on material learned either before or during hypnosis was investigated across 2 experiments. In Experiment 1, very high, high, and low hypnotizable participants learned a word list either before or immediately after a hypnotic induction. During hypnosis, participants were given a suggestion for posthypnotic amnesia for the word list. After hypnosis, they were tested on recall, word-fragment, and word-recognition tasks. Experiment 2 replicated and extended Experiment 1 through application of the real-simulating paradigm. Across the 2 experiments, there was no difference in the performance of participants who learned the word list either before or during hypnosis. Although amnesia on direct memory measures was associated with high hypnotizability (Experiment 1), an explanation based on demand characteristics could not be excluded (Experiment 2). The implications of these findings for the use of post-hypnotic amnesia as a laboratory analog of disorders of autobiographical memory are discussed.