[Imerslund-Gräsbeck syndrome]. / Maladie d'Imerslund-Gräsbeck.

Anemia is a very common symptom encountered in numerous clinical situations in pediatrics. Etiologies range from classic iron-deficiency anemia to the more particular etiologies. We report on a clinical history where usual symptoms such as asthenia, drowsiness and proteinuria provided a rare diagnosis: Imerslund-Gräsbeck syndrome. We discuss the exams to be done with aregenerative macrocytic anemia so as not to underestimate these diagnoses, which each require adapted treatments.

[Obstructive sleep apnoea syndrome (OSAS) in women: a specific entity?]. / Pathologie pulmonaire au féminin: le SAOS de la femme, une entité particulière?

Despite a variable prevalence in the literature, OSAS is characterized by a higher frequency in men. This study involved a review of published data describing the impact of gender on features of OSAS. In women, OSAS seems to be associated with certain anatomical factors such as more significant obesity, a thinner oropharyngeal junction, a shorter uvula and reduced upper airway (UA) collapsibility, in comparison with men. Sleep related breathing disorders observed during pregnancy are more likely UA resistance syndrome than true OSAS, and are associated with severe fetal and maternal complications that could be improved by the use of nasal CPAP. Though OSAS symptoms are underestimated by women, the Epworth score as well as snoring are not influenced by gender. Gender does not seem to be a risk factor for increased mortality. Compliance with nasal CPAP is identical in both sexes, but oral appliance seem to be more effective in women. Further studies are needed to produce the required complementary data to confirm specific sex-related features in the diagnosis and treatment of OSAS.

[Syringe drug labels: a French national survey]. / Enquête nationale sur le marquage des seringues d'anesthésie en France.

INTRODUCTION: The French Society of Anaesthesiology (SFAR) recommends the use of pre printed self-stick syringe labels. However, French anaesthesia-syringe labelling customs is yet unknown. STUDY DESIGN: Therefore, a national phone survey was performed in order to investigate this issue. RESULTS: Forty-five percent of the answering centers (324/722) used colour self-stick labels, with a larger proportion in public centers and a large regional variability. The kind of colour code differed from an area to another. Among centers using colour labels, the international recommended colour code was used in only 36% of them. The majority of health care providers declare to be favourable to the use of the colour self-stick labels and the standardization of the colour code as well. A relationship between the University hospital and the surrounded area has been observed for the use of the colour labels and the choice of the colour code. CONCLUSION: Colour labels are used in France by nearly half of the centers, but international colour code is less spread.

[Gaucher disease in childhood]. / Maladie de Gaucher: particularités cliniques chez l'enfant.

Gaucher disease is well-known in adult patients and must be regarded as a pediatric disease, two thirds of the patients manifesting before the age of 20. Three clinical forms have been defined based on the presence of neurological involvement. Gaucher disease type 1, without neurological signs, generally begins before the five years age with splenomegaly as the main symptom. The bone crises are more frequent than in adulthood. Gaucher disease type 2 or acute neuronopathic form begins between three and six months and do not have any treatment. Type 3 or chronic neuronopathic form appears like a type 1 with progressive horizontal saccade-initiation failure and developmental delay. Onset in childhood is predictive of a severe and progressive phenotype. The presence of neurological symptoms induces important consequences for treatment, prognosis and genetic counselling.

[Pneumoperitoneum and pneumomediastinum complicating non-invasive ventilation]. / Un pneumoperitoine compliquant un pneumomediastin au cours de la ventilation non invasive.

INTRODUCTION: Pneumoperitoneum is known to be a rare complication of invasive mechanical ventilation. However it has not previously been described as a consequence of non-invasive ventilation. CASE REPORT: The authors report a case of pneumoperitoneum associated with pneumomediastinum occurring in a 64-year-old patient treated for 3 years with bilevel ventilation via a nasal mask (expiratory pressure = 9 cm H2O, inspiratory pressure = 15 cm H2O) for obesity hypoventilation syndrome. Respiratory and gastroenterological investigations did not demonstrate a cause for this complication which resolved spontaneously following the cessation of ventilation. Nine months later, clinical deterioration and a worsening of blood gas parameters led to a recommencement of non-invasive mechanical ventilation at the same levels as previously. Over two years of follow up there have been no clinical or radiological signs of a recurrence of pneumomediastinum or pneumoperitoneum. CONCLUSION: In the absence of any other explanation, barotrauma due to nasal ventilation appears to be the most likely explanation for this complication.

[Polyarteritis nodosa revealed by acute cholecystitis]. / Cholécystite aiguë révélant une périartérite noueuse. A propos de deux cas.

UNLABELLED: Acute cholecystitis revealing polyarteritis nodosa is classic but rare in adulthood. We report two cases observed during childhood. CASE REPORTS: The association of a persistent inflammatory syndrome, positive antineutrophil cytoplasm antibodies and of a vasculitis with fibrinoïd necrosis led to the diagnosis in two pediatric patients. CONCLUSION: The diagnosis of polyarteritis nodosa is difficult and often delayed. Cholecystectomy can help to such a diagnosis in the presence of acute cholecystitis.