Long term NIV in an infant with Hallermann-Streiff syndrome: A case report and overview of respiratory morbidity.

Hallermann-Streiff syndrome (HSS) is a rare congenital syndrome with different anomalies including midface hypoplasia, beak nose and micrognathia. The upper airways narrowness can lead to severe respiratory complications such as obstructive sleep apnoea syndrome (OSAS), particularly in infancy. The management of these severe OSAS is difficult and poorly documented in literature. We report the case of an infant with HSS complicated by severe and early OSAS successfully managed with non-invasive ventilation (NIV), provide an overview of respiratory morbidities and discuss treatment options for HSS-related OSAS.

Remodelling and inflammation in preschoolers with severe recurrent wheeze and asthma outcome at school age.

BACKGROUND: The influence of airway remodelling and inflammation in preschoolers with severe recurrent wheeze on asthma outcomes is poorly understood. OBJECTIVE: To assess their association with asthma symptoms and lung function at school age. METHODS: Preschoolers (38.4 months) initially investigated with bronchial biopsies were re-assessed for asthma symptoms and lung function at school age. RESULTS: Thirty-six of 49 preschoolers (73.5%) were assessed at 10.9 years. Twenty-six (72.2%) had persistent asthma. Submucosal eosinophil counts were higher in children with severe exacerbations at school age than in those without (16/0.1 mm2 [11.2-30.4] vs 8/0.1 mm2 [2.4-17.6], P = .02), and correlated with the number of severe exacerbations (P = .04, r = .35). Submucosal neutrophil counts correlated with FEV1/FVC (P < .01, r = .47) and FEF25-75% predicted (P = .02, r = .43). Airway smooth muscle (ASM) area correlated with FEV1/FVC (P < .01, r = .51). Vessel numbers negatively correlated with FEV1% predicted and FEV1/FVC (P = .03, r = -.42; P = .04, r = -.41; respectively) and FEF25-75% predicted (P = .02, r = -.46). CONCLUSION: Eosinophilic inflammation in preschoolers with severe recurrent wheeze might be predictive of future severe exacerbations, neutrophilia might be associated with better lung function. Changes in ASM and vascularity might affect lung function at school age.

[Congenital ciliary dyskinesia. Focus]. / Dyskinésie ciliaire congénitale. Mise au point.

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disease, caused by specific primary structural and/or functional abnormalities of the motile cilia. Prevalence, about 1/15,000 to 1/30,000, is probably underestimated, as diagnosis might not be evocated in absence of Kartagener syndrome. Diagnosis is confirmed in presence of abnormal ciliary motility as well as ciliary ultrastructure. Disease-causing mutations in at least 16 genes have already been identified; analysis will be guided by the type of ultrastructural abnormalities. An early and adequate diagnosis and therapy can theoretically improve the prognosis of the disease.

[Pneumocystis jiroveci pneumonia during prolonged corticosteroid therapy in an immunocompetent infant]. / Pneumonie àPneumocystis jiroveci au cours d'une corticothérapie prolongée chez un nourrisson immunocompétent.

INTRODUCTION: Pneumocystis jiroveci (PJ) infection is rare in infants and is suggestive of primary or secondary immunodeficiency. We report on a case of severe PJ pneumonia in an immunocompetent infant after prolonged corticosteroid treatment. CASE REPORT: A 5 1/2 month-old girl presented with hypoxemic respiratory distress. Her medical record was remarkable only for a bulky parotid haemangioma, which was treated with prolonged oral corticosteroid therapy. The chest X-ray showed a mixed alveolar-interstitial pattern, and bronchoalveolar lavage revealed the presence of PJ. A favourable outcome was obtained after three weeks of intravenous trimethoprim-sulfamethoxazole treatment. CONCLUSION: PJ infection should be suspected in infants presenting with progressive respiratory distress associated with a mixed alveolar-interstitial pattern. Its potential seriousness justifies prophylactic therapy during prolonged immunosuppressive treatment (chemotherapy, corticosteroid treatment).

Recombination of nanometric vesicles during freeze-drying.

Concentrated dispersions of nanometric lipid vesicles (mean diameter 20 nm) in water/maltose solutions have been freeze-dried and then redispersed in water, yielding again dispersions of lipid vesicles. At each stage of the freeze-drying process, the organization of the vesicles in the dispersion and their size distribution were examined through small-angle neutron scattering and gel permeation chromatography. It was found that the osmotic deswelling of the vesicles caused them to recombine into larger vesicles. A single burst of recombination events occurred when the maltose concentration in the aqueous phase rose above 100 g/L. The final vesicle population was monopopulated, with a central diameter about twice as large as that of the original dispersion.

Power consumption profiles in high-shear wet granulation. II: Predicting the overwetting point from a spreading energy.

Granulation is an important process in the pharmaceutical industry for the preparation of solid dosage forms. For high-shear wet granulation, the process endpoint is monitored using the device power consumption. However, granulation is very sensitive to variations in the feed product physicochemical properties and, in some cases, power consumption profiles can not be used for process control. In this paper, a model is proposed to predict the granulation overwetting point from the spreading energy of the liquid binder on the powder. This energy is independently calculated from measurements of the powder true surface area, liquid binder surface tension, and liquid contact angle on the powder surface.

Power consumption profiles in high-shear wet granulation. I: Liquid distribution in relation to powder and binder properties.

Wet granulation processes frequently rely on the device power consumption profile to monitor the process endpoint. This work shows how the observed power consumption relates to the physical properties of the mass. The liquid binder was traced with a fluorescent marker and the liquid distribution could be followed during the process. It was found that primary agglomerates form in the reactor, the shape and composition of which depend on the wettability of the starting powders by the liquid binder. An increase of the device power consumption is conditional to the disruption of these primary agglomerates. Increasing the viscosity of liquid binders up to 250 mPa. s had no significant effect on the power consumption. Eventually, the lower surface tension liquid binders yielded greater energy consumption, indicating that interparticle friction forces had a great impact on the device power consumption.

Powder dynamic contact angle data in the pharmaceutical industry.

Formulation scientists generate physicochemical data that are used in process modelling and in the prediction of end-product quality. In this respect, contact angles are of particular importance as the surface energy of substances influences their processability and bioavailability. Dynamic contact angle (DCA) analysis represents a straightforward wettability assay. Now fully adapted to powders, DCA analysis also proves to be more efficient than other methods for wettability determination.

[Premature mortality in France: relative importance of the main causes of death according to the number of years of life lost]. / Mortalité prématurée en France: importance relative des principales causes de décès selon le nombre d'années de vie perdues.

The importance of major causes of deaths in France have been studied for 1972 and 1982, using years of potential life lost before age 65 and in relation to life expectancy. These indicators of premature mortality have been compared to death rates. Unintentional injuries and malignant neoplasms are the two main causes of mortality before age 65. There is an important excess mortality for males, particularly for unintentional injuries. Total premature mortality declined between 1972 and 1982, but cancer mortality for males and suicide and chronic bronchitis mortality for both sexes have increased. Cardiac mortality decreased only for females.