Hematopoietic stem cell transplantation for adolescents and adults with inborn errors of immunity: an EBMT IEWP study.

peer reviewedAllogeneic hematopoietic stem cell transplantation (HSCT) is the gold standard curative therapy for infants and children with many inborn errors of immunity (IEI), but adolescents and adults with IEI are rarely referred for transplant. Lack of published HSCT outcome data outside small, single-center studies and perceived high risk of transplant-related mortality have delayed the adoption of HSCT for IEI patients presenting or developing significant organ damage later in life. This large retrospective, multicenter HSCT outcome study reports on 329 IEI patients (age range, 15-62.5 years at HSCT). Patients underwent first HSCT between 2000 and 2019. Primary endpoints were overall survival (OS) and event-free survival (EFS). We also evaluated the influence of IEI-subgroup and IEI-specific risk factors at HSCT, including infections, bronchiectasis, colitis, malignancy, inflammatory lung disease, splenectomy, hepatic dysfunction, and systemic immunosuppression. At a median follow-up of 44.3 months, the estimated OS at 1 and 5 years post-HSCT for all patients was 78% and 71%, and EFS was 65% and 62%, respectively, with low rates of severe acute (8%) or extensive chronic (7%) graft-versus-host disease. On univariate analysis, OS and EFS were inferior in patients with primary antibody deficiency, bronchiectasis, prior splenectomy, hepatic comorbidity, and higher hematopoietic cell transplant comorbidity index scores. On multivariable analysis, EFS was inferior in those with a higher number of IEI-associated complications. Neither age nor donor had a significant effect on OS or EFS. We have identified age-independent risk factors for adverse outcome, providing much needed evidence to identify which patients are most likely to benefit from HSCT

Ligneous Periodontitis in a Child with Plasminogen Deficiency

Ligneous periodontitis (LP), a rare periodontal disease, is seen secondary to plasminogen deficiency and fibrin deposition. It is characterized by nodular gingival enlargements and progressive destructive membranous periodontal disease. It generally ends with the early loss of teeth. Defective fibrinolysis and abnormal wound healing is the main pathogenesis of this rare disease and should be considered different from other mucosal systemic disorders. In this case report, we describe the management of ligneous periodontitis and ligneous conjunctivitis developing secondary to plasminogen deficiency in a 6-year-old girl.Keywords: Ligneous periodontitis, ligneous conjuctivitis, plasminogen deficienc

A new set of deschampes for arytenoidopexy [PRESENTATION D'UN NOUVEAU JEU D'AIGUILLES POUR L'ARYTENOIDOPEXIE]

PubMed ID: 734270[No abstract available

Endoscopic sinus surgery in the isolated paranasal sinus aspergilloma [La chirurgie endoscopique pour l'aspergillome d'un sinus paranasal]

PubMed ID: 10769567Fungal infections of the paranasal sinuses are in four clinical forms. Beside the acute fulminating form, chronic invasive form, allergic fungal sinusitis and fungus ball. Fungus ball is mostly encountered in only one paranasal sinus of an otherwise healthy person. Ten fungus balls of the paranasal sinuses are presented with their management and results

MIB-1 expression in odontogenic epithelial rests, epithelium of healthy oral mucosa and epithelium of selected odontogenic cysts - An immunohistochemical study

WOS: 000229449100017PubMed: 16053855The aim of this study is to investigate the proliferative potential of rests of odontogenic epithelium found in follicles of unerupted teeth, epithelium of oral mucosa and epithelial linings of various odontogenic cysts. MIB-1 expression was studied in the rests of odontogenic epithelium (n = 10), healthy oral mucosa (17 = 10), odontogenic keratocysts (n = 10) and other odontogenic cysts (n = 10) using an avidin-biotin peroxidase technique on paraffin sections. The number of positively stained cells was counted on 10 representative areas of epithelium using a x 40 objective. The average number of MIB-1 positive cells in each group was calculated. No MIB-1 positive cells were seen in the rests of odontogenic epithelium. The mean numbers of MIB-1 positive cells detected within the epithelium of oral mucosa, and of radicular and dentigerous cysts were similar. The number of MIB-1 positive cells was found to be increased in the presence of marked inflammatory cell infiltration. The highest number of MIB-1 positive cells was seen in the keratocysts. These findings suggest that removal of an unerupted tooth to prevent the possibility of neoplastic transformation of rests of odontogenic epithelium is not a justifiable rationale

A series of sonographically standardized test material for audiometric screening

[No abstract available

Mental substitution investigation test

[No abstract available