RESUMO
Osteochondroma is called a benign cartilage-forming tumor arising from an aberrant subperiosteal cartilage. Multiple osteochondromas syndrome (MOS) is an autosomal dominant disease, the basis for which is mutations in the EXT (EXT1 or EXT2) genes. Osteochondroma is one of the most common benign bone tumors. According to the WHO data, it is detectable in 35% of benign bone tumors and 8% of all surgically removed bone tumors. A total of 491 cases of bone tumors were analyzed in the children and adolescents diagnosed at the Department of Pathoanatomy, Russian Children's Clinical Hospital, Moscow, in 2009 to 2014. All the patients with osteochondroma were divided into 2 groups: 1) sporadic cases (n = 63) and 2) tumors included in MOS (n = 33). Both groups showed a preponderance of boys (39 boys and 24 girls in Group 1 and 21 boys and 12 girls in Group 2). Clinical, radiological, and morphological criteria for the diagnosis and differential diagnosis of osteochondromas in children and adolescents are given.
Assuntos
Exostose Múltipla Hereditária/diagnóstico , Exostose Múltipla Hereditária/patologia , Exostose Múltipla Hereditária/cirurgia , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
DESIGN: A randomized prospective study. PATIENTS AND METHODS: The study included 87 children aged from 5 to 18, ASA I-II. The patients were divided into two groups. We assessed the impact of reamberin 1.5% (succinate-containing infusion solution) on the level of metabolism and recovery after surgery in patients of main group (n = 44) and control group (n = 43) during different surgeries. All patients received general anaesthesia with sevoflurane, fentanyl, and rocuronium. RESULTS: Reamberin 1.5% promotes to increase the level of basal metabolism in the early postoperative period, decreases the duration of awakening periods, improves recovery of motor activity and adequate breathing.
Assuntos
Período de Recuperação da Anestesia , Anestesia Geral/métodos , Metabolismo Basal/efeitos dos fármacos , Meglumina/análogos & derivados , Assistência Perioperatória/métodos , Succinatos/farmacologia , Adolescente , Criança , Pré-Escolar , Humanos , Infusões Intravenosas , Meglumina/administração & dosagem , Meglumina/farmacologia , Estudos Prospectivos , Soluções , Succinatos/administração & dosagem , Fatores de TempoRESUMO
Production of citokines of tumor-necrosis factor (TNF)-alpha and interferon (IFN)-alpha by peripheral blood mononuclear cells (PBMC) from healthy men and from patients with common variable immunodeficiency (CVID) after stimulation with zymosan, lypopolysaccharides, flagellin and CpG, which are ligands of TLR 2/6, TLR 4, TLR 5, TLR 9 respectively, was studied in vitro. In healthy men production of TNF-alpha varied between individuals, whereas synthesis of IFN-alpha was similar. Spontaneous production of TNF-alpha by PBMC in patients with CVID was increased and accompanied by decrease in TNF-alpha production stimulated by each analyzed ligands except CpG. Observed changes in TLR-dependent TNF-alpha production can play important role in pathogenesis of CVID.
Assuntos
Imunodeficiência de Variável Comum/imunologia , Citocinas/biossíntese , Imunocompetência/imunologia , Receptores Toll-Like/imunologia , Adolescente , Adulto , Células Cultivadas , Criança , Feminino , Humanos , Interferon-alfa/biossíntese , Leucócitos Mononucleares/imunologia , Ligantes , Masculino , Fator de Necrose Tumoral alfa/biossínteseRESUMO
The production of TNF-alpha and IFN-alpha cytokines by peripheral blood mononuclears in response to stimulation by TLR2/6, TLR4, TLR5, TLR9 ligands (zymosan, LPS, flagellin, and CpG-oligodeoxynucleotide, respectively) was studied in donors and patients with common variable immunodeficiency. Individual characteristics of TNF-alpha production by mononuclears were revealed in donors. Reduced stimulated production of TNF-alpha in response to stimulation with TLR4 and TLR5 ligands in vitro was detected in patients with common variable immunodeficiency.
Assuntos
Doadores de Sangue , Imunodeficiência de Variável Comum/fisiopatologia , Interferon-alfa/biossíntese , Receptores Toll-Like/fisiologia , Fator de Necrose Tumoral alfa/biossíntese , Adolescente , Adulto , Criança , Imunodeficiência de Variável Comum/sangue , Feminino , Flagelina/farmacologia , Humanos , Leucócitos Mononucleares , Lipopolissacarídeos/farmacologia , Masculino , Oligodesoxirribonucleotídeos/farmacologia , Poli I-C/farmacologia , Receptores Toll-Like/efeitos dos fármacos , Zimosan/farmacologiaRESUMO
To study clinical peculiarities of parainfectious opsoclonus-myoclonus syndrome (OMS) in children and to elaborate approaches to its pharmacotherapeutic correction, 20 children, including 12 girls and 8 boys, have been examined using neurological, neurophysiological, immunological and virological methods along with magnetic resonance tomography (MRT). Age-at-disease-onset was from 8 months to 3 years old. The development of neurological symptoms was related to a virus infection (55% of cases) or vaccination (15%). Marked disease seasonality (autumn, spring) was observed. In some cases, a possible role of infectious factor in the disease development was confirmed by virological and immunological studies. OMS was featured by the absence of specific brain MRT changes and low frequency of significant abnormalities of cerebrospinal fluid. A positive experience of the use of hormonal therapy and immunomodulators is presented.