RESUMEN
Differential diagnosis of bone involvement in patients with Gaucher disease can be challenging. Other diseases with similar radiological signs should be ruled out. Here we present a clinical case of tuberculous sacroiliitis in the patient with type I Gaucher disease. Advanced radiological methods of examination are described. Our case report proves the necessity of an individual approach to the management of such cohort of patients. Keywords: Gaucher disease, tuberculosis of bones and joints, differential diagnosis, comprehensive treatment.
Asunto(s)
Enfermedad de Gaucher/diagnóstico por imagen , Radiografía/métodos , Sacroileítis/diagnóstico por imagen , Tuberculosis Osteoarticular/diagnóstico por imagen , Diagnóstico Diferencial , Enfermedad de Gaucher/complicaciones , Humanos , Sacroileítis/complicaciones , Tuberculosis Osteoarticular/complicacionesRESUMEN
The study presents comparative analysis of porous composites made of chitosan, alginate, fibrin with beta-tricalcium phosphate. Histological findings on Wistar rat condyles showed that fibrin-beta-TCP-based composite had the most effective positive biological response.
Asunto(s)
Alginatos/uso terapéutico , Cementos para Huesos/uso terapéutico , Sustitutos de Huesos/uso terapéutico , Fosfatos de Calcio/uso terapéutico , Cementoplastia , Quitosano/uso terapéutico , Fibrina/uso terapéutico , Alginatos/efectos adversos , Alginatos/química , Animales , Cementos para Huesos/efectos adversos , Cementos para Huesos/química , Sustitutos de Huesos/efectos adversos , Sustitutos de Huesos/química , Huesos/efectos de los fármacos , Huesos/lesiones , Fosfatos de Calcio/efectos adversos , Fosfatos de Calcio/química , Quitosano/efectos adversos , Quitosano/química , Fibrina/efectos adversos , Fibrina/química , Ácido Glucurónico/efectos adversos , Ácido Glucurónico/química , Ácido Glucurónico/uso terapéutico , Ácidos Hexurónicos/efectos adversos , Ácidos Hexurónicos/química , Ácidos Hexurónicos/uso terapéutico , Ensayo de Materiales , Porosidad , Ratas , Ratas WistarRESUMEN
Gaucher disease (GD) is an inherited enzymatic defect resulting from a deficiency of acid [3-glucosidase, a lysosomal enzyme involved in the degradation of cell metabolic products. The major clinical manifestations of GD are hepatosplenomegaly, cytopenia, and bony involvement varying from asymptomatic osteopenia to severest osteoporosis and ischemic necrosis to develop irreversible orthopedic defects. Timely enzyme replacement therapy with recombinant glucosidase makes it possible to arrest disease progression and to prevent damage to the vital organs. However, GD in adult patients is frequently diagnosed in the presence of occurring osteoarticular lesions (arthrosis deformans, abnormal fractures). In these instances, besides enzyme replacement therapy, high-quality orthopedic care is required. The description of the case history of a patient undergoing splenectomy in childhood is given as a clinical example of severe osteoarticular lesion in GD and complex differential diagnosis with the intercurrent disease extrapulmonary tuberculosis.
Asunto(s)
Enfermedad de Gaucher/complicaciones , Sacroileítis/complicaciones , Tuberculosis Osteoarticular/complicaciones , Adulto , Antituberculosos/administración & dosificación , Antituberculosos/uso terapéutico , Diagnóstico Diferencial , Terapia de Reemplazo Enzimático , Enfermedad de Gaucher/diagnóstico , Enfermedad de Gaucher/terapia , Glucosilceramidasa/uso terapéutico , Humanos , Masculino , Sacroileítis/diagnóstico , Sacroileítis/terapia , Resultado del Tratamiento , Tuberculosis Osteoarticular/diagnóstico , Tuberculosis Osteoarticular/terapiaRESUMEN
AIM: To evaluate the efficiency of intensive polychemotherapy (PCT) in adult patients having diffuse large B-cell lymphoma (DLBL) of bones and soft tissues with and without poor prognostic factors (PPF). SUBJECTS AND METHODS: Out of 58 enrolled patients, 51 were diagnosed as having DLBL. Burkitt's lymphoma (BL) was diagnosed in 6 patients. One patient had marginal zone B-cell lymphoma. Thirty-five patients with DLBL (10 patients with PPF and 25 without PPF) and 3 patients with BL were treated with the CHOP/R-CHOP regimen. The NHL-BFM-90 program was used in 3 patients with BL and 16 with DLBL (15 patients with PPF and 1 patient without PPF). RESULTS: After radiotherapy, the patient with marginal zone B-cell lymphoma achieved a 20-month remission; three BL patients receiving CHOP died. All the BL patients receiving NHL-BFM-90 achieved complete remissions of 48 to 72 months. In 9 patients having DLBL without PPF, who received CHOP, five-year overall and event-free survival rates were 100%; in 22 patients with PPF, these were 50 and 45%, respectively. In the patients with and without PPF, who received CHOP/R-CHOP, the survival rates differed statistically significantly (p = 0.01; logrank test). In the group of 15 patients having DLBL with PPF, who were treated with the NHL-BFM-90 protocol, 14 achieved an average remission of 17 months. In the patients having DLBL with PPF who used NHF-BFM-90, therapeutic efficiency was significantly higher (p = 0.05; Fisher's exact test). CONCLUSION: Differential therapy for primary lymphomas of bones and soft tissues indicated that the NHL-BFM-90 protocol used in the PPF group was significantly more effective than the CHOP regimens.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Óseas/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Neoplasias de los Tejidos Blandos/tratamiento farmacológico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias Óseas/patología , Supervivencia sin Enfermedad , Femenino , Humanos , Linfoma de Células B Grandes Difuso/patología , Masculino , Persona de Mediana Edad , Pronóstico , Inducción de Remisión/métodos , Neoplasias de los Tejidos Blandos/patología , Tasa de Supervivencia , Factores de Tiempo , Adulto JovenRESUMEN
The study was conducted in 102 patients with hematological disorders admitted to the Research Center of Hematology. It was concluded that more than 50% of them needed oral surgery procedures requiring multidisciplinary approach.
Asunto(s)
Enfermedades Hematológicas/epidemiología , Enfermedades Estomatognáticas/epidemiología , Adulto , Femenino , Enfermedades Hematológicas/terapia , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Federación de Rusia/epidemiología , Adulto JovenRESUMEN
Case presentation of radical maxillary sinusotomy in chronic sinusitis patient with severe haemophilia A, the paper contains detailed description of the surgical technique, postsurgical care, hemostatic and complementary treatment, as well as anesthesia options making this procedure safe in terms of hemorrhagic complications.
Asunto(s)
Hemofilia A/complicaciones , Sinusitis Maxilar/complicaciones , Sinusitis Maxilar/cirugía , Adulto , Pérdida de Sangre Quirúrgica/prevención & control , Técnicas Hemostáticas , Humanos , Masculino , Hemorragia Posoperatoria/prevención & control , Resultado del TratamientoRESUMEN
Intracranial hemorrhage in patients with chronic autoimmune thrombocytopenic purpura (CATP) is a rare and severe complication of the disease. By taking into account a concomitance of chronic subdural hematoma (CSH) and CATP and no generally accepted approaches to managing the patients with this concomitance, the authors describe a clinical case of mini-invasive CSH drainage in a patient with CATP.
Asunto(s)
Hematoma Subdural Crónico/cirugía , Procedimientos Neuroquirúrgicos , Púrpura Trombocitopénica Idiopática/complicaciones , Esplenectomía , Adulto , Enfermedad Crónica , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Hematoma Subdural Crónico/diagnóstico , Hematoma Subdural Crónico/etiología , Hematoma Subdural Crónico/inmunología , Humanos , Masculino , Recuento de Plaquetas , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Púrpura Trombocitopénica Idiopática/cirugía , Resultado del TratamientoRESUMEN
AIM: To evaluate efficacy of intensive modified program NHL-BFM-90 (mNHL-BFM-90) in adult poor-prognosis patients with diffuse large B-cell lymphosarcoma (DLBCL) of the bones and soft tissues. MATERIAL AND METHODS: The mNHL-BFM-90 program was used in the treatment of 3 male and 2 female patients aged 17-69 years (median 42 years). Four patients had DLBCL of the bones and one patient--DLBCL of the soft tissues. All the patients had tumors more than 10 cm in size. B-symptoms, a high concentration of lactate dehydrogenase (LDG) were registered in 3 patients. One patient had stage IE by Ann-Arbor, two--stage IIE (involvement of regional lymph nodes), two--stage ME (multiple bone lesions). A total of 4-6 blocks of polychemotherapy according to mNHL-BFM-90 program were performed. RESULTS: Complete remissions were achieved in all the patients. They had no recurrences after 6 to 20 month (median 13 months) follow-up. CONCLUSION: Positive results of the program mNHL-BFM-90 in poor-prognosis patients with DLBCL of the bones and tissues necessitate further studies of this therapy.