[Rhinosinusitis in Hurler syndrome patients requiring hematopoietic stem cells transplantation]. / Problema rinosinusita u patsientov s sindromom Gurler pri transplantatsii gemopoéticheskikh stvolovykh kletok.

INTRODUCTION: Allogenic transplantation of hemopoetic stem cells (allo-THSC) is one of the most effective treatment methods for Hurler syndrome, aimed at maximal correction of complications related to the genetic disorder. Presence of infection in the recipient is an adverse risk factor, affecting the possibility of starting the conditioning regimen and THSC peforming in general. AIM: To assess the condition of the nasal cavity and paranasal sinuses in Hurler syndrome patients before the allo-THSC, dynamics of these changes after the transplantation taking into account the correction of alpha-L-iduronidase enzyme level with donor blood cells. MATERIAL AND METHODS: From February 2012 to December 2017, In the Raisa Gorbacheva Research Institute of Child Oncology, Hematology and Transplantology of the Pavlov First Saint Petersburg State Medical University, eighteen Hurler syndrome patients (10 girls and 8 boys) received an allo-THSC. Median age at the time of the procedure was 23,5 months (min - 3,4; max - 24,8). Each patient with the shadowing of paranasal sinuses, rhinitis or nasal breathing difficulty received a standard rhinosinusitis treatment before the transplantation, effect of which was insignificant. Symptoms of rhinitis, condition of pharyngeal tonsil and paranasal sinuses were assessed before and auto the allo-THSC. RESULTS: In the post-allo-THSC, with the correction of alpha-L-iduronidase level each evaluated parameter has improved reliably (p-value < 0,05). Comparative analysis of the condition of the nasal cavity and pharyngeal tonsil before and after THSC was conducted on 14 patients out of 18. Rhinitis symptoms decreased in 9 (64,2%) patients; in 11 patients (78,5%) adenoids size reduced. Comparative analysis of the condition of paranasal sinuses was possible in 12 patients out of 18. Sinuses aeration improved in eight (66,6%) if patients. CONCLUSION: Nasal cavity and paranasal sinuses changes in Hurler syndrome patients before and after allo-THSC is poorly studied. Our experience demonstrates the normalization of nasal cavity, pharyngeal tonsila and paranasal sinuses symptoms in the majority of the patients receiving allo-THSC. These symptoms are, it seems a consequence of the underlying disease.

[Our experience with tracheotomy in the hematological patients in need of prolonged artificial lung ventilation].

The objective of the present work was to study the possibility and technical peculiarities of planned tracheotomy in the hematological patients with thrombocytopenia and coagulopathy suffering severe concomitant pathology. A total of 45 tracheotomies were performed in hematological patients during the period from 2009 till July 2012. The case histories of 32 patients were available for the retrospective analysis. At the time of surgical intervention, 81% of the patients presented with grade IV thrombocytopenia. Five of the patients (15.6%) suffered bleeding from the tracheostomic canal in the early postoperative period. In four of them, hemorhage was stopped by the placement of the hemostatic sponge. One patient had to be managed by means of cauterization . Two (6.25%) patients developed inflammation around tracheotsoma. It is concluded that thrombocytopenia and probable coagulopathy do not constitute an absolute contraindication for planned tracheostomy. However, such operation may have a favourable outcome only after preliminary transfusion, the application of cauterization, and delicate surgical intervention.