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Lionel Donato

Publications and source records attributed to Lionel Donato.

5 recordsLinked to original sources
PubMed2006

Epidemiology of juvenile idiopathic arthritis in Alsace, France.

OBJECTIVE: To determine the incidence, prevalence, and principal characteristics of the different forms of juvenile idiopathic arthritis (JIA) in the region of Alsace, northeastern France, using the new classification of the International League of Associations for Rheumatology (ILAR). METHODS: In 2002 we performed a retrospective epidemiologic study pertaining to the year 2001. The pediatricians, rheumatologists, ophthalmologists, orthopedic surgeons, and physicians involved in functional reeducation in the Alsace region were interviewed, and all patients were classified according to the new ILAR classification using the criteria revised in Durban in 1997. RESULTS: Among the 361 clinicians contacted, the participation rate was 97.8%. The study identified 67 children followed for JIA in Alsace in 2001, from a total population of 1.8 million inhabitants including 339,095 children under age 16 years. The incidence was calculated to be 3.2 cases/100,000/year and the prevalence 19.8 cases/100,000 children under age 16 years. Among these 67 cases of JIA, the most frequent forms were oligoarthritis (n = 27, 40.3%), polyarthritis without rheumatoid factor (RF; n = 15, 22.4%), and enthesitis related arthritis (n = 12, 17.9%). Other forms, notably systemic arthritis (n = 6, 8.9%) and psoriatic arthritis (n = 3, 4.5%), were more rare and in this study there was no case of polyarthritis with RF. Only 4 patients (6%) were classified in the undifferentiated arthritis group using the new classification. Antinuclear antibodies (ANA; by indirect immunofluorescence, HEp >/= 1/80) were detected in patients with oligoarthritis (81%) and polyarthritis without RF (79%). Uveitis occurred in 41% of children with oligoarthritis and in 14% of those with polyarthritis without RF. CONCLUSION: Our results are comparable to those of other studies carried out in Caucasian populations with regard to incidence and prevalence. This work also highlights the frequent presence of ANA and uveitis in patients with oligoarthritis or polyarthritis without RF.

Arthritis, Juvenile↗
PubMed2005

French experience of silicone tracheobronchial stenting in children.

Silicone stents were inserted into the trachea or left main-stem bronchus in 14 children aged 2-69 months (median, 7 months). Indications were as follows: tracheomalacia or airway kinking (7 cases), vascular compression (5 cases), and surgically corrected congenital tracheal stenoses (2 cases). The best results were obtained in tracheomalacia. Overall, 6 cases out of 14 (43%) were considered successful, with a stent placement duration of 3-15 months (median, 7 months). Two cases were considered a technical success, although they were clinical failures. Five cases were considered failures primarily due to stent migration. A retrospective analysis of failures suggests that most of these could have been avoided by the use of larger stents. One patient died of stent obstruction. No wall erosion was observed, and the development of granulation tissue was infrequent. Endoscopic removal of the prostheses was uneventful. The biocompatibility of silicone stents appears to be better than what is reported for metal ones, although the stability of the former is less satisfactory. The present study shows the feasibility of silicone stent placement in infants. These stents should be considered as a possible therapeutic option in certain types of childhood airway disorders, although further studies are required.

Airway Obstruction↗
PubMed2004

Neonatal necrotizing tracheobronchitis: three case reports.

Necrotizing tracheobronchitis is a serious affection observed in ventilated newborns, frequently infants with instable hemodynamic state. It is characterized by acute episodes of airway obstruction. The treatment consists of the desobstruction by rigid bronchoscopy. The vascular theory seems to be of utmost importance in the physiopathology. Three cases are reported.

Airway Obstruction↗
PubMed2002

Analysis of natural killer cells in TAP2-deficient patients: expression of functional triggering receptors and evidence for the existence of inhibitory receptor(s) that prevent lysis of normal autologous cells.

Natural killer (NK) cells are characterized by the ability to kill cells that lack HLA class I molecules while sparing autologous normal (HLA class I(+)) cells. However, patients with transporter-associated antigen processing (TAP) deficiency, though displaying strong reductions of HLA class I surface expression, in most instances do not experience NK-mediated autoimmune phenomena. A possible mechanism by which TAP(-/-) NK cells avoid autoreactivity against autologous HLA class I-deficient cells could be based on either quantitative or qualitative defects of surface receptors involved in NK cell triggering. In this study we show that NK cells derived from 2 patients with TAP2(-/-) express normal levels of all known triggering receptors. As revealed by the analysis of polyclonal and clonal NK cells, these receptors display normal functional capabilities and allow the killing of a panel of NK-susceptible targets, including autologous B-LCLs. On the other hand, TAP2(-/-) NK cells were unable to kill either allogeneic (HLA class I(+)) or autologous (HLA class I(-) ) phytohemagglutinin (PHA) blasts even in the presence of anti-HLA class I monoclonal antibody. These data suggest that TAP2(-/-) NK cells express still unknown inhibitory receptor(s) capable of down-regulating the NK cell cytotoxicity on binding to surface ligand(s) expressed by T cell blasts. Functional analyses, both at the polyclonal and at the clonal level, are consistent with the concept that the putative inhibitory receptor is expressed by virtually all TAP2(-/-) NK cells, whereas it is present only in rare NK cells from healthy persons. Another possibility would be that TAP2(-/-) NK cells are missing a still unidentified triggering receptor involved in NK cell-mediated killing of PHA blasts.

ATP Binding Cassette Transporter, Subfamily B, Mem↗