Nebulized aerosols are commonly used to deliver drugs into the lungs of patients with cystic fibrosis (CF). The aim of this study was to assess the effectiveness of pressure-support (PS) ventilation ...in increasing aerosol deposition within the lungs of children with CF. An in vitro study demonstrated the feasibility of coupling a breath-actuated nebulizer to a PS device. An in vivo study was done with 18 children (ages 6 to 21 yr) with clinically stable CF, each of whom underwent both a standard and a PS-driven ventilation scan (control session and PS session, respectively). In addition, a perfusion scan was used to determine lung outlines and to construct a geometric model for quantifying aerosol deposition by radioactivity counting in MBq. Homogeneity of nebulization was evaluated from the four first-order moments of aerosol distribution in the peripheral and central lung regions. The time-activity nebulization curve was linear in all patients, with higher slopes during the PS than during the control session (0.43 +/- 0.07 mean +/- SD MBq/min and 0.32 +/- 0.23 MBq/min, respectively; p < 0.018). Quantitatively, aerosol deposition was about 30% greater after the PS session (4.4 +/- 2.7 MBq) than after the control session (3.4 +/- 2.1 MBq; p < 0.05). Similarly, deposition efficacy (as a percentage of nebulizer output) was significantly better during the PS session than during the control session (15.3 +/- 8.3% versus 11.5 +/- 5.7%, p < 0.05). No differences in the regional deposition pattern or in homogeneity of uptake were observed. In conclusion, our data show that driving the delivery of a nebulized aerosol by noninvasive PS ventilation enhances total lung aerosol deposition without increasing particle impaction in the proximal airways.
At present, it is conceivable that gene therapy of the cystic fibrosis airway epithelium is possible using the direct transfer of a functional human cystic fibrosis transmembrane conductance ...regulator (CFTR) gene to a wide variety of patients' tracheo-bronchial cells. Here we describe a novel approach (aerosolization) to deliver a replication-deficient adenovirus carrying the CFTR gene (Ad.CFTR) to the airways. Results obtained in vitro and in Rhesus monkeys suggest that the delivery of recombinant adenovirus as an aerosol is feasible and is not associated with severe toxicity after single or double administration depending on the Ad.CFTR dose. This study supports the concept of aerosolization as a delivery method for adenovirus-mediated lung gene therapy.
Purpose
To present a case of recurrent bilateral optic oedema in a patient with tubulo‐interstitial nephritis and uveitis syndrome (TINU) with a limited response to corticosteroids, immunosuppressive ...drugs and tumor necrosis factor inhibitor (TNFi). Plasmapheresis was added as rescue therapy.
Methods
Observational case report about a 13‐year‐old boy diagnosed with TINU and bilateral optic oedema. An extensive general and ophthalmological work‐up confirmed the diagnosis of TINU.
Results
At referral the patient presented with bilateral anterior uveitis and nephritis. Biomicroscopy showed a trace of anterior chamber cells. Funduscopy revealed bilateral optic oedema with neither haemorrhages nor exudates. Initially the patient responded well to systemic corticosteroids but he relapsed while tapering. Consecutive treatment consisted of azathioprine, tacrolimus and methotrexate. Due to limited response, infliximab, a TNFi, was associated. After 7 months disease‐free, the TNFi treatment failed. Finally, he responded well to plasmapheresis.
Conclusions
TINU may rarely manifest with optic oedema. Although nephritis typically resolves, ocular inflammation often becomes chronic and can be therapy‐resistant. This case suggests that plasmapheresis can be an alternative in case of a therapy‐resistant TINU. A multidisciplinary approach is essential to confirm the diagnosis and initiate appropriate treatment.
The first reference to an attempt at functional surgery of the nose which we have discovered in France concerns Blandin (Paris 1798-1849) who "corrected" septal deviations with a punch; one arm is ...introduced into each nostril. Heylen performed a submucous resection in 1847, Chassaignac in 1851 and Demarquay in 1859 through the external median columellar route. In 1876 Richet carried out a resection of the deviated septum after having elevated the whole cartilaginous pyramid, achieved by a horizontal incision of the base of the columella. Paul Berger recommended, in 1883, a subperichondrial chisel resection of the salient part of the septum. In 1888, Miot approached septal thickening with galvano-caustic chemicals using metal plates or through a method called galvano-puncture. In 1892 Escat resected the cartilaginous arch and its corresponding mucosa after having separated the contralateral mucosa with injected water. In 1903, Caboche referred to both operations used at that time to correct cartilaginous septal deviations, e.g. Petersen's operations (submucous resection) and Asch's operation (fracture with repositioning). In 1905, Blanc distinguished between three types of operations: 1. Procedures based upon the fracturing the septum or its straightening and its maintenance with a splint. 2. Procedures designed in order to overcome the elasticity of the cartilage by making incisions followed by its retention in place. 3. Submucous resection of the cartilage based upon the principle that the septum is too big for its surroundings. In 1917, Dangouloff and Woyatchek developed a septoplasty technique, many modern operations being only pale copies of theirs. It consisted of four possibilities: mobilization, straightening, circular resection and partial resection.
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