RESUMO
To establish a biological profile and disease aetiologies for one of four burials recovered during a Time Team dig at the St. Mary Magdalen leprosarium, Winchester, UK in AD 2000. Osteological techniques were applied to estimate age at death, biological sex, stature and pathology. Visual assessment of the material was supplemented by radiographic examination. Evidence for leprosy DNA was sought using ancient DNA (aDNA) analysis. The remains are those of a male individual excavated from a west-east aligned grave. The skeleton shows signs of two pathologies. Remodelling of the rhino-maxillary area and degenerative changes to small bones of the feet and reactive bone on the distal lower limbs suggest a multibacillary form of leprosy, whereas the right tibia and fibula show the presence of a primary neoplasm identified as an osteosarcoma. The aDNA study confirmed presence of Mycobacterium leprae in several skeletal elements, and the strain was genotyped to the 3I lineage, one of two main SNP types present in mediaeval Britain and ancestral to extant strains in America. This is a rare documentation of leprosy in association with a primary neoplasm.
Assuntos
Hanseníase Virchowiana , Hanseníase , Osteossarcoma , Osso e Ossos , DNA Antigo , Humanos , Hanseníase/diagnóstico , Hanseníase Virchowiana/microbiologia , Masculino , Mycobacterium leprae/genética , Osteossarcoma/genética , Reino UnidoRESUMO
BACKGROUND: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. AIM: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. METHOD: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. RESULTS: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron's rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. LIMITATIONS: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. CONCLUSION: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.
Assuntos
Dermatomiosite/complicações , Adolescente , Artralgia/etiologia , Calcinose/complicações , Criança , Pré-Escolar , Fármacos Dermatológicos/uso terapêutico , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Exantema/etiologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Masculino , Metotrexato/uso terapêutico , Debilidade Muscular/etiologia , Mialgia/etiologia , Osteossarcoma/complicações , Prednisolona/uso terapêutico , Estudos Retrospectivos , Dermatoses do Couro Cabeludo/etiologia , Dermatopatias/complicações , Neoplasias de Tecidos Moles/complicações , Telangiectasia/etiologia , Centros de Atenção Terciária , TailândiaRESUMO
Osteosarcoma is the most common cancer in bone. Drug resistance is a challenge of current treatments that needs to be improved with novel treatment strategies. In this research, a new dual drug delivery system was developed with Gemcitabine (GEM) and Clofazimine (CLF) co-loaded liposome formulations. GEM is a well-known anticancer agent and CLF is a leprostatic and anti-inflammatory drug recently recognized as effective on cancer. GEM and CLF co-loaded liposomal formulation was achieved with compartmentalization as hydrophilic GEM being in core and lipophilic CLF sequestering in lipid-bilayer. Liposomes had high encapsulation efficiency (above 90%, GEM and above 80%, CLF). CLF release was enhanced while GEM release was slowed down in co-loaded liposomes compared to single cases. GEM/CLF co-loaded liposomes significantly enhanced cytotoxicity than GEM or CLF loaded liposomes on osteosarcoma cell line. CLF and GEM had synergistic effect (CIâ¯<â¯1). Results of flow cytometry showed higher apoptotic cell ratio, caspase-3 activity, mitochondrial membrane depolarized cells' ratio for GEM/CLF co-loaded liposome treatments than other liposomes. Cytotoxicity of CLF on bone cancer cells and also its synergistic effect with GEM on osteosarcoma is reported for the first time with this study. CLF's loading with GEM into liposome was also a new approach for enhancement of anticancer effect on Saos-2 cells. Therefore, GEM/CLF co-loaded liposomal delivery system is proposed as a novel approach for treatment of osteosarcoma.
Assuntos
Antineoplásicos/administração & dosagem , Neoplasias Ósseas/tratamento farmacológico , Clofazimina/administração & dosagem , Desoxicitidina/análogos & derivados , Osteossarcoma/tratamento farmacológico , Linhagem Celular Tumoral , Sobrevivência Celular/efeitos dos fármacos , Desoxicitidina/administração & dosagem , Combinação de Medicamentos , Sinergismo Farmacológico , Humanos , Lipossomos , GencitabinaRESUMO
La lepra es una enfermedad infecciosa, poco transmisible, de evolución crónica, causada por el Mycobacterium leprae, que se caracteriza por afectar la piel, los nervios periféricos, la mucosa de las vías respiratorias superiores además de otras estructuras. Se ha subestimado su prevalencia y permanece siendo un problema de salud pública, detectándose aún nuevos casos cada año. Después de la introducción de la multiterapia MDT tanto prevalencia como incidencia han disminuido. En la actual investigación se hace la presentación de un caso clínico, paciente femenina, de 49 años, con diagnóstico de Lepra Dimorfa, que tiene antecedentes de haber sido amputada hace tres años de miembro inferior izquierdo por osteosarcoma de tobillo
Leprosy is an infectious disease with a chronic evolution and its etiological agent is Mycobacterium leprae. The disease affects skin, peripheral nerves, upper respiratory tract and other structures. Its prevalence has been underestimated and continues to be a public health problem in many countries. After the introduction of MDT, both prevalence and incidence declined. This study is a presentation of a clinical case of a 49 year old woman diagnosed of dimorphic leprosy and a history of amputation three years earlier due to osteosarcoma of the ankle
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Osteossarcoma/complicações , Osteossarcoma/diagnóstico , Hanseníase Dimorfa/complicações , Hanseníase Dimorfa/diagnóstico , Hanseníase Dimorfa/patologia , Rifampina/uso terapêutico , Cefalexina/uso terapêutico , Tornozelo/patologia , Articulação do Tornozelo/patologia , Hanseníase Dimorfa/prevenção & controle , Hanseníase Dimorfa/fisiopatologia , Dapsona/uso terapêutico , Diagnóstico Precoce , Mycobacterium leprae/isolamento & purificação , Mycobacterium leprae/patogenicidadeRESUMO
This is a rare case report of osteosarcoma with lepromatous leprosy. A 15 year old male patient presented with swelling around the right knee joint. Imaging and biopsy were consistent with osteosarcoma. After his first cycle of adjuvant chemotherapy (ACT), the patient developed fever, erythematous nodules, perichondritis of ear lobe, and thickened nerves. His slit-skin smear examination showed acid-fast bacilli in clumps, and a diagnosis of multibacillary leprosy was made. He was treated with anti-leprosy medications with steroids, and once his condition stabilized, his ACT was continued. On follow-up, his skin lesions completely recovered.
Assuntos
Hanseníase Multibacilar/patologia , Osteossarcoma/microbiologia , Adolescente , Humanos , Hansenostáticos/uso terapêutico , Hanseníase Multibacilar/diagnóstico , Hanseníase Multibacilar/tratamento farmacológico , Masculino , Osteossarcoma/diagnóstico , Osteossarcoma/tratamento farmacológicoRESUMO
Adult CBA mice thymectomized, treated with antilymphocytic globulin (ALG) and inoculated with human leprosy organisms were accidentally infected with polyoma virus and all developed tumours. After cessation of ALG administration, some animals were given spleen cells from syngeneic donors immunized with polyoma virus; none developed tumours. Similar results were obtained in mice deliberately infected with polyoma virus but not with leprosy organisms. Passive transfer of antibody before but not after virus inoculation prevented tumour formation in immunosuppressed recipients. Virus infection in thymectomized, lethally irradiated and bone marrow reconstituted mice resulted in only a very low incidence of tumours. These results emphasize the role of immunological surveillance in preventing polyoma tumour formation under natural conditions.