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1.
J Assoc Physicians India ; 72(1): 99-102, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38736082

RESUMO

BACKGROUND: Peripheral T cell lymphoma (PTCL), not otherwise specified (NOS) is a heterogenous group of predominantly nodal T cell lymphomas that generally presents with lymphadenopathy with or without extra nodal involvement. Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and acral cyanosis. Although, this condition is commonly associated with connective tissue disorder, smoking and vasculitis, its association with lymphoid malignancy is very rare. Here, we present a case report of a patient with digital gangrene of all toes and fingers as a presenting symptom of PTCL-NOS. CASE DESCRIPTION: A 62 year old male presented with digital ischemia associated with pain, low grade fever, loss of appetite and significant weight loss of 6 kilograms over a period of 3 months. On examination, he was found to have bilateral inguinal and axillary lymph nodes with gangrenous changes over toes and fingers but peripheral pulses were palpable. On evaluation he had anemia, elevated ESR and CRP. CT angiogram revealed thinned out digital arteries with multifocal areas of narrowing. Patient was screened for other causes of digital gangrene and was tested negative for ANCA, ANA, cryoglobulins and viral markers. Lymph node biopsy with IHC was suggestive of peripheral T-cell lymphoma-NOS and was started on CHOP regimen. Lymph nodes size decreased and gangrenous changes resolved. CONCLUSION: Though digital ischemia is a rare paraneoplastic presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevent permanent disability. In our patient, progression of gangrene was prevented even though it was an aggressive variant of T cell lymphoma.


Assuntos
Dedos , Gangrena , Linfoma de Células T Periférico , Síndromes Paraneoplásicas , Dedos do Pé , Humanos , Masculino , Gangrena/etiologia , Gangrena/diagnóstico , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/complicações , Pessoa de Meia-Idade , Dedos/patologia , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/etiologia , Dedos do Pé/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Vincristina/uso terapêutico , Prednisona/uso terapêutico
2.
Br J Dermatol ; 186(4): 609-619, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-34862606

RESUMO

Epidermolysis bullosa (EB) encompasses a heterogeneous group of inherited skin fragility disorders, with mutations in genes encoding the basement membrane zone (BMZ) proteins that normally ensure dermal-epidermal integrity. Of the four main EB types, recessive dystrophic EB (RDEB), especially the severe variant, represents one of the most debilitating clinical entities, with recurrent mucocutaneous blistering and ulceration leading to chronic wounds, infections, inflammation, scarring and ultimately cutaneous squamous cell carcinoma, which leads to premature death. Improved understanding of the molecular genetics of EB over the past three decades and advances in biotechnology have led to rapid progress in developing gene and cell-based regenerative therapies for EB. In particular, RDEB is at the vanguard of advances in human clinical trials of advanced therapeutics. Furthermore, the past decade has witnessed the emergence of a real collective, global effort involving academia and industry, supported by international EB patient organizations such as the Dystrophic Epidermolysis Bullosa Research Association (DEBRA), among others, to develop clinically relevant and marketable targeted therapeutics for EB. Thus, there is an increasing need for the practising dermatologist to become familiar with the concept of gene therapy, fundamental differences between various approaches, and their human applications. This review explains the principles of different approaches of gene therapy, summarizes its journey, and discusses its current and future impact in RDEB.


Assuntos
Carcinoma de Células Escamosas , Epidermólise Bolhosa Distrófica , Epidermólise Bolhosa , Neoplasias Cutâneas , Carcinoma de Células Escamosas/terapia , Epidermólise Bolhosa/genética , Epidermólise Bolhosa/patologia , Epidermólise Bolhosa/terapia , Epidermólise Bolhosa Distrófica/genética , Epidermólise Bolhosa Distrófica/patologia , Epidermólise Bolhosa Distrófica/terapia , Terapia Genética , Humanos , Neoplasias Cutâneas/terapia
3.
J Postgrad Med ; 67(4): 238-240, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34341225

RESUMO

Congenital hypothyroidism (CH) occurs due to thyroid dysgenesis, thyroid ectopy, and dyshormonogenesis. A proportion of CH is transient which might be due to iodine deficiency/excess or maternal antibody-mediated. Certain forms of dyshormonogenetic defects may cause transient hypothyroidism. Here is a report of a neonate with overt clinical and biochemical hypothyroidism, who on evaluation was found to have dyshormonogenesis with a homozygous mutation in dual oxidase 2 (DUOX2) gene. During infancy, she became euthyroid. Severe in utero deficiency of thyroid hormone, very short duration of hypothyroidism and first-reported mutation of the DUOX2 gene in the Indian subcontinent were interesting features in this infant.


Assuntos
Hipotireoidismo Congênito , Disgenesia da Tireoide , Hipotireoidismo Congênito/diagnóstico , Hipotireoidismo Congênito/genética , Oxidases Duais/genética , Feminino , Humanos , Lactente , Recém-Nascido , Mutação
4.
J Postgrad Med ; 67(1): 29-32, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33380594

RESUMO

Coronary heart disease (CHD) is the most important cause of cardiovascular death and when premature, it affects the most productive population of the community. Premature CHD usually has a specific etiology, which on diagnosis, might help in the secondary prevention in that individual. We report a case of young adult with recurrent myocardial infarction, who on evaluation had mildly reduced HDL and Protein C levels with elevated serum homocysteine. Clinical exome identified a possibly pathogenic variant of ABCA1 gene, associated with Tangier disease.


Assuntos
Transportador 1 de Cassete de Ligação de ATP/genética , Infarto do Miocárdio/diagnóstico , Doença de Tangier/complicações , Adulto , Predisposição Genética para Doença , Humanos , Masculino , Infarto do Miocárdio/genética , Doença de Tangier/sangue , Doença de Tangier/genética
5.
Malays J Pathol ; 43(2): 291-301, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34448793

RESUMO

BACKGROUND: Bloodstream infection (BSI) is a major cause of morbidity and mortality. The classification of infection into community-acquired, hospital-acquired, and healthcare-associated infection provides an educated guess on the possible aetiological agents and appropriate empirical antimicrobial therapy to be instituted. This study aims to determine the aetiological agents, the antimicrobial susceptibility patterns, and the classification of infections among the paediatric population. MATERIALS & METHODS: This study was conducted in Hospital Kuala Lumpur, Malaysia from January 2016 to December 2017. A total of 303 isolates were included in this study which was obtained from 238 patients. The patients' microbiological worksheets and medical notes were reviewed to determine the antimicrobial susceptibility patterns, demographic data, classification of infection, and outcome (survival versus death). RESULTS: Most of the patients were in the age group of one to less than five years old (41%) with 58% male and 85% Malay patients. Common causes of BSI were Staphylococcus aureus (17%), followed by Klebsiella pneumoniae (15%), Acinetobacter baumanii (10%), Pseudomonas aeruginosa (10%), and Escherichia coli (6%). Sixty percent of BSI episodes were caused by gram-negative bacteria, 34% by gram-positive bacteria, and 6% by fungi. Most of the infections were classified as hospital-acquired infections (72%), followed by healthcareassociated (20%) and community-acquired infections (8%). There were 33% of methicillin-resistant Staphylococcus aureus, 53% of extended-spectrum beta-lactamase (ESBL) producing Klebsiella pneumoniae, and 33% ESBL producing Escherichia coli. The overall case fatality rate (CFR) was 27% with the highest CFR caused by Serratia marcescens (53.3%). CONCLUSIONS: The majority of paediatric bloodstream infections are hospital-acquired. Improvement in prevention strategies and revisions in antibiotic policies are important to overcome it.


Assuntos
Bacteriemia , Sepse , Antibacterianos/uso terapêutico , Bacteriemia/tratamento farmacológico , Bacteriemia/epidemiologia , Pré-Escolar , Infecção Hospitalar/tratamento farmacológico , Infecção Hospitalar/epidemiologia , Escherichia coli , Feminino , Hospitais Gerais , Humanos , Lactente , Masculino , Staphylococcus aureus Resistente à Meticilina , Testes de Sensibilidade Microbiana , Estudos Retrospectivos
6.
Med J Malaysia ; 74(5): 454-455, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31649231

RESUMO

Neonatal death due to inborn error of metabolism (IEM) is rare in Malaysia. We report a sudden neonate death just a few hours after being discharged from the hospital. The deceased was a two-day-old baby boy and was asymptomatic until his demise. He was fed with expressed breast milk and formula milk. Autopsy revealed fatty changes of the liver and an enlarged heart. Laboratory investigation confirmed very long chain Acyl-CoA dehydrogenase deficiency which resulted in his death. Autopsy of sudden unexpected death in neonate should include investigation for inborn error of metabolism. Fatty liver and enlarged heart could give a clue for the diagnosis.


Assuntos
Acil-CoA Desidrogenase de Cadeia Longa/deficiência , Acil-CoA Desidrogenase/deficiência , Síndrome Congênita de Insuficiência da Medula Óssea/complicações , Fígado Gorduroso/diagnóstico , Erros Inatos do Metabolismo Lipídico/complicações , Doenças Mitocondriais/complicações , Doenças Musculares/complicações , Acil-CoA Desidrogenase/metabolismo , Acil-CoA Desidrogenase de Cadeia Longa/metabolismo , Síndrome Congênita de Insuficiência da Medula Óssea/diagnóstico , Síndrome Congênita de Insuficiência da Medula Óssea/metabolismo , Evolução Fatal , Fígado Gorduroso/etiologia , Fígado Gorduroso/metabolismo , Humanos , Recém-Nascido , Erros Inatos do Metabolismo Lipídico/diagnóstico , Erros Inatos do Metabolismo Lipídico/metabolismo , Masculino , Doenças Mitocondriais/diagnóstico , Doenças Mitocondriais/metabolismo , Doenças Musculares/diagnóstico , Doenças Musculares/metabolismo
7.
Malays J Pathol ; 41(3): 359-364, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31901923

RESUMO

INTRODUCTION: Brucellosis is a zoonotic disease with variable clinical manifestations and atypical presentation in humans. Human brucellosis cases are not seen often in Malaysia. CASE REPORT: This is a case report of 19 years old gentleman who presented with fever, lower limb redness, pain and swelling. He was initially treated as cellulitis. However, based on the recovery of Brucella melitensis from his blood culture, he was later diagnosed to have brucellosis. He had a history of consumption of fresh goat's milk and uncooked meat which could have been the possible modes of transmission. Brucella serology IgM and IgG were both positive, and anti-Brucella immunocapture agglutination test (BrucellaCapt) was also positive with a titer of 1:2560. He was treated with six weeks of oral doxycycline 100 mg twice daily and oral rifampin 450 mg twice daily. DISCUSSION: This is a case of human brucellosis with atypical cutaneous involvement.


Assuntos
Brucella melitensis/patogenicidade , Brucelose/patologia , Celulite (Flegmão)/microbiologia , Celulite (Flegmão)/patologia , Adulto , Testes de Aglutinação , Anticorpos Antibacterianos/uso terapêutico , Brucelose/diagnóstico , Celulite (Flegmão)/diagnóstico , Humanos , Malásia , Masculino , Adulto Jovem
8.
Malays J Pathol ; 41(1): 51-54, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31025638

RESUMO

Spontaneous coronary artery dissection is a rare event and commonly associated with pregnancy and female gender. This condition can reduce or completely obstruct the blood flow to the heart, causing a myocardial ischaemia, abnormalities in heart rhythm or sudden death. We present a case of a 28-year-old Indian male with no previous medical illness who complained sudden onset of chest pain prior to his death. Autopsy revealed a left anterior descending coronary artery dissection associated with plaque rupture. The anterior wall of left ventricle showed contraction band necrosis. There was also atheroma present in the right coronary artery which was insignificant. Histologically, dissection was associated with atherosclerosis. There was no evidence of vasculitis. The cause of death was given as coronary artery dissection due to coronary artery atherosclerosis.


Assuntos
Doença da Artéria Coronariana/complicações , Anomalias dos Vasos Coronários/etiologia , Morte Súbita Cardíaca/etiologia , Doenças Vasculares/congênito , Adulto , Doença da Artéria Coronariana/mortalidade , Doença da Artéria Coronariana/patologia , Anomalias dos Vasos Coronários/mortalidade , Anomalias dos Vasos Coronários/patologia , Morte Súbita Cardíaca/patologia , Humanos , Masculino , Doenças Vasculares/etiologia , Doenças Vasculares/mortalidade , Doenças Vasculares/patologia
9.
Parasitology ; 145(14): 1896-1906, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-29692277

RESUMO

GLP-1 (abnormal germline proliferation) is a Notch-like receptor protein that plays an essential role in pharyngeal development. In this study, an orthologue of Caenorhabditis elegans glp-1 was identified in Meloidogyne incognita. A computational analysis revealed that the orthologue contained almost all the domains present in the C. elegans gene: specifically, the LIN-12/Notch repeat, the ankyrin repeat, a transmembrane domain and different ligand-binding motifs were present in orthologue, but the epidermal growth factor-like motif was not observed. An expression analysis showed differential expression of glp-1 throughout the life cycle of M. incognita, with relatively higher expression in the egg stage. To evaluate the silencing efficacy of Mi-glp-1, transgenic Arabidopsis plants carrying double-stranded RNA constructs of glp-1 were generated, and infection of these plants with M. incognita resulted in a 47-50% reduction in the numbers of galls, females and egg masses. Females obtained from the transgenic RNAi lines exhibited 40-60% reductions in the transcript levels of the targeted glp-1 gene compared with females isolated from the control plants. Second-generation juveniles (J2s), which were descendants of the infected females from the transgenic lines, showed aberrant phenotypes. These J2s exhibited a significant decrease in the overall distance from the stylet to the metacorpus region, and this effect was accompanied by disruption around the metacorporeal bulb of the pharynx. The present study suggests a role for this gene in organ (pharynx) development during embryogenesis in M. incognita and its potential use as a target in the management of nematode infestations in plants.


Assuntos
Arabidopsis/parasitologia , Proteínas de Helminto/genética , Doenças das Plantas/parasitologia , Interferência de RNA , Receptores Notch/genética , Tylenchoidea/genética , Animais , Repetição de Anquirina/genética , Caenorhabditis elegans/genética , Proteínas de Caenorhabditis elegans/genética , Resistência à Doença , Família de Proteínas EGF/genética , Desenvolvimento Embrionário , Feminino , Estágios do Ciclo de Vida , Plantas Geneticamente Modificadas/parasitologia , Tylenchoidea/parasitologia
10.
Med J Malaysia ; 73(5): 272-275, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30350803

RESUMO

INTRODUCTION: The role of pathologist not only confined in performing post mortem but also can assist in prevention. The aim of this study to determine the prevalence and association of drug of abuse (DoA) in road traffic collision (RTC) at Hospital Kuala Lumpur. METHODS: This is a retrospective study of post mortem cases at Hospital Kuala Lumpur from 2014 to 2016. Deaths from RTC were included while decomposed and homicide cases were excluded. We performed Spearman Correlation statistical test to relate RTC and positive DoA results. RESULTS: A total of 523 RTC cases were identified in which either blood or urine or both samples were taken for toxicology. 93 cases were positive for both DoA and therapeutic drugs. A total of 37 cases were positive for DoA. Alcohol was present in 5 out of 37 DoA positive cases. Most of the cases seen among 16 to 45 years old (69%) and predominantly in males (93.1%). 29 out of 37 were motorcyclist and the rest were pillion rider and pedestrian. Spearman Correlation statistical test showed a negative relationship between RTC and positive DoA results. DISCUSSION AND CONCLUSION: Majority of the DoA cases in RTC were identified in the younger age group and among the motorcyclist. Spearman Correlation statistical test showed that more cases of DoA died in natural or suicidal manner compared to RTC. However, this doesn't reflect the true association of DoA in RTC. This is because of mainly two factors which the delayed effect of DoA that gives negative toxicology test and also the influence of other road users on DoA.


Assuntos
Acidentes de Trânsito/mortalidade , Transtornos Relacionados ao Uso de Substâncias/complicações , Adolescente , Adulto , Feminino , Humanos , Drogas Ilícitas/efeitos adversos , Malásia/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
11.
Vox Sang ; 112(5): 443-452, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28466601

RESUMO

BACKGROUND AND OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) is a method of life support for either isolated cardiac failure or respiratory failure, with or without cardiac failure. When used for hemodynamic support, the ECMO circuit presents a non-endothelialized, artificial surface to blood inciting an inflammatory response which activates haemostatic pathways. Anticoagulation may complicate a pre-existing coagulopathy and/or inadequate surgical hemostasis of varying severity. There is no standardized method to achieve and monitor anticoagulation or guide transfusion therapy during ECMO. We tested the hypothesis that institutions across the world conduct similar management of anticoagulation and transfusion during adult ECMO support. METHODS: This is a descriptive, self-reporting cross-sectional survey of anticoagulation and transfusion practice for patients age 18 or older on ECMO. This 38 multiple-choice question survey was sent to 166 institutions, internationally, utilizing adult ECMO. About 32·4% (54) of institutions responded. Responses were anonymously collected. Descriptive analyses were calculated. RESULTS: Our findings indicate there appears to be a significant practice variation among institutions regarding anticoagulation and transfusion during adult ECMO support. DISCUSSION: The lack of standard practices among institutions may reflect a paucity of data regarding optimal anticoagulation and transfusion for patients requiring ECMO. Standardized protocols for anticoagulation and transfusion may help increase quality of care for and reduce morbidity, mortality and cost to patients and healthcare centres. Further study is required for standardized, high quality care.


Assuntos
Coagulação Sanguínea , Transfusão de Sangue/métodos , Oxigenação por Membrana Extracorpórea/métodos , Anticoagulantes/farmacologia , Estudos Transversais , Pesquisas sobre Atenção à Saúde , Heparina/farmacologia , Humanos , Tempo de Coagulação do Sangue Total
12.
Intern Med J ; 46(5): 616-9, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-27170239

RESUMO

Biological therapy, particularly the anti-tumour necrosis factor (TNF) antibodies, infliximab and adalimumab, are used for the maintenance of remission for patients with inflammatory bowel diseases (IBD). We present 21 pregnancies in IBD patients exposed to anti-TNF agents between 2007 and 2014. Our study demonstrates that anti-TNF therapy is safe and effective in pregnancy. Rates of foetal complications are similar to IBD cohorts from the pre anti-TNF era.


Assuntos
Adalimumab/administração & dosagem , Anti-Inflamatórios/administração & dosagem , Doenças Inflamatórias Intestinais/tratamento farmacológico , Infliximab/administração & dosagem , Complicações na Gravidez/tratamento farmacológico , Adalimumab/efeitos adversos , Adulto , Anti-Inflamatórios/efeitos adversos , Austrália , Feminino , Humanos , Imunoterapia/efeitos adversos , Infliximab/efeitos adversos , Gravidez , Resultado da Gravidez , Estudos Prospectivos , Fator de Necrose Tumoral alfa/antagonistas & inibidores
14.
Dis Aquat Organ ; 122(2): 105-123, 2016 12 21.
Artigo em Inglês | MEDLINE | ID: mdl-28000602

RESUMO

Although infections caused by megalocytiviruses have been reported from a wide range of finfish species for several decades, molecular characterisation of the viruses involved has been undertaken only on more recent cases. Sequence analysis of the major capsid protein and adenosine triphosphatase genes is reported here from formalin-fixed, paraffin-embedded material from 2 archival ornamental fish cases from 1986 and 1988 in conjunction with data for a range of genes from fresh frozen tissues from 5 cases obtained from 1991 through to 2010. Turbot reddish body iridovirus (TRBIV) genotype megalocytiviruses, previously not documented in ornamental fish, were detected in samples from 1986, 1988 and 1991. In contrast, megalocytiviruses from 1996 onwards, including those characterised from 2002, 2006 and 2010 in this study, were almost indistinguishable from infectious spleen and kidney necrosis virus (ISKNV). Three of the species infected with TRBIV-like megalocytiviruses from 1986 to 1991, viz. dwarf gourami Trichogaster lalius (formerly Colisa lalia), freshwater angelfish Pterophyllum scalare and oscar Astronotus ocellatus, were infected with ISKNV genotype megalocytiviruses from 2002 to 2010. The detection of a TRBIV genotype isolate in ornamental fish from 1986 represents the index case, confirmed by molecular sequence data, for the genus Megalocytivirus.


Assuntos
Peixes/virologia , Iridoviridae/genética , Iridoviridae/isolamento & purificação , Animais , Bancos de Espécimes Biológicos , DNA Viral/genética , DNA Viral/isolamento & purificação , Genótipo , Filogenia
15.
Intern Med J ; 45(11): 1154-60, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26337851

RESUMO

BACKGROUND: Musculoskeletal symptoms are the most common extra-intestinal manifestation associated with inflammatory bowel disease (IBD). Spondyloarthritis (SpA) is an umbrella term applied to a group of rheumatic diseases with some features in common and others distinct from other inflammatory arthritides. AIM: To determine self-reported prevalence of SpA associated musculoskeletal manifestations in an IBD cohort on tumour necrosis factor (TNF) inhibitors using a questionnaire incorporating Assessment of SpondyloArthritis International Society (ASAS) criteria. METHODS: Consecutive IBD patients on TNF inhibitors attending a single IBD centre (May-September 2011) were asked to complete a SpA questionnaire. Data collected included SpA and IBD variables, demographics, concurrent medications, co-morbidities and autoimmune serology. RESULTS: The 140-patient cohort included 96 suffering from Crohn disease and 44 from ulcerative colitis. The mean age of disease onset was 29.3 years and 45% were men. Concurrent or past history of inflammatory back pain was reported by 29% subjects. Using the imaging and clinical arms of the ASAS criteria, 30% and 14% subjects respectively had axial SpA. Arthritis was reported by 34%, enthesitis 17%, dactylitis 4%, uveitis 6%, psoriasis 6% and a family history of SpA in 39%. Peripheral SpA was present in 41% by the ASAS criteria. There were no differences in these frequencies between Crohn disease and ulcerative colitis. A positive antinuclear antibodies (>1:80) was found in 19% before commencement of TNF inhibitor therapy and increased to 78% on therapy. Clinical drug-induced lupus erythematosus was uncommon (4%) and was characterised by new clinical signs and symptoms, including arthralgia, rash with elevated dsDNA titres and positive antinuclear antibodies. CONCLUSIONS: Inflammatory bowel disease patients on TNF inhibitors frequently reported musculoskeletal manifestations. Increased recognition of SpA occurred with use of an SpA self-reported questionnaire in IBD patients: this could alter management and improve patient outcomes. Clinical drug-induced lupus erythematosus was uncommon.


Assuntos
Doenças Inflamatórias Intestinais/tratamento farmacológico , Doenças Inflamatórias Intestinais/epidemiologia , Espondiloartropatias/tratamento farmacológico , Espondiloartropatias/epidemiologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adolescente , Adulto , Estudos de Coortes , Feminino , Humanos , Doenças Inflamatórias Intestinais/diagnóstico , Infliximab/farmacologia , Infliximab/uso terapêutico , Masculino , Pessoa de Meia-Idade , Autorrelato , Espondiloartropatias/diagnóstico , Adulto Jovem
16.
Intern Med J ; 44(5): 464-70, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24612209

RESUMO

BACKGROUND: Anti-tumour necrosis factor (TNF) agents are used as induction and maintenance therapy in ulcerative colitis (UC) refractory to standard therapy and as rescue therapy in acute severe UC (ASUC). AIMS: To determine long-term outcomes including colectomy rates, predictors of maintenance of response and remission, risk of serious adverse events by reviewing 12-year clinical experience from a single centre in Australia. METHODS: Seventy-one patients with moderate-severe UC (Mayo score ≥6) (n = 52) and ASUC (n = 19) treated with anti-TNF agents were included. Primary end-points were colectomy at 12 weeks and colectomy-free survival at last follow up. Secondary endpoints included clinical response (decrease in Mayo score of ≥3) and remission (Mayo score ≤2). RESULTS: Colectomy at 12 weeks was 1%, and colectomy-free survival was 69%. Using full Mayo score, at 3 months, 32/37 (87%) refractory and 9/12 (75%) ASUC patients responded to anti-TNF therapy; 19/37 (51%) refractory and 8/12 (67%) ASUC patients were in remission. Long-term response rates (mean follow up 37.4 months) were 24/44 (55%) and 11/15 (73%) in refractory and ASUC groups respectively. Long-term remission rates were 43% in refractory and 60% in ASUC patients. Twenty two of 71 (31%) underwent colectomy (mean time 50.4 months). Clinical non-response at 3 months was a predictor of colectomy (hazard ratio = 9.346; P = 0.001). ASUC predicted long-term maintenance of response (odds ratio 19.4; P = 0.013) and remission (odds ratio 6.13; P = 0.037). Two of 71 patients had serious infections. CONCLUSIONS: Anti-TNF therapy is effective in both refractory and ASUC. We argue that early anti-TNF therapy may improve outcome in UC.


Assuntos
Anti-Inflamatórios/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Colectomia/estatística & dados numéricos , Colite Ulcerativa/cirurgia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adalimumab , Corticosteroides/farmacologia , Corticosteroides/uso terapêutico , Adulto , Anti-Inflamatórios/efeitos adversos , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais/efeitos adversos , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/efeitos adversos , Colite Ulcerativa/tratamento farmacológico , Intervalo Livre de Doença , Resistência a Medicamentos , Quimioterapia Combinada , Feminino , Seguimentos , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Infliximab , Masculino , Mesalamina/administração & dosagem , Mesalamina/uso terapêutico , Pessoa de Meia-Idade , Estudos Observacionais como Assunto , Prognóstico , Modelos de Riscos Proporcionais , Ensaios Clínicos Controlados Aleatórios como Assunto , Recidiva , Indução de Remissão , Fatores de Risco , Índice de Gravidade de Doença , Resultado do Tratamento
17.
Intern Med J ; 44(3): 287-90, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24621284

RESUMO

We present three cases of the rare hepatosplenic T-cell lymphoma (HSTCL); two patients suffering from Crohn disease who developed HSTCL on azathioprine without exposure to biologicals, and a third patient who had psoriasis treated using etanercept, cyclosporine and methotrexate. The evidence for an association between HSTCL and immunosuppressive drugs and biologicals is reviewed. We argue for improved pharmacovigilance processes to help determine the benefit to risk ratios for the use of these and other new agents.


Assuntos
Produtos Biológicos/efeitos adversos , Imunossupressores/efeitos adversos , Neoplasias Hepáticas/diagnóstico , Linfoma de Células T/diagnóstico , Neoplasias Esplênicas/diagnóstico , Adulto , Humanos , Neoplasias Hepáticas/induzido quimicamente , Linfoma de Células T/induzido quimicamente , Masculino , Fatores de Risco , Neoplasias Esplênicas/induzido quimicamente
18.
Childs Nerv Syst ; 30(9): 1543-7, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25038841

RESUMO

PURPOSE: Postoperative CSF leak is a known complication of spinal surgery especially after surgery for neural tube defects (NTD). The problem can metamorphose into a severe infection. This article hopes to shed some light on the management of these problems and suggests precautions so as to reduce their occurrence. MATERIALS AND METHODS: A retrospective analysis of 102 children, between the ages of 1 day and 12 years, operated for various spinal pathologies, over the past 2.5 years by the same surgeon (CB) was done. The various methods of dural closure were noted. The methods of management of postoperative CSF leaks were analysed, and the patients were followed till discharge. RESULTS: The incidence of CSF leak was found to be 12.7%. The methods of management included lumbar drain only (n = 7), lumbar drain with re-exploration (n = 3), lumbar drain followed by lumboperitoneal shunt (n = 2) and only lumboperitoneal shunt (n = 1). The use of fibrin glue did not seem to significantly prevent the incidence of CSF leak in cases. CONCLUSIONS: Primary and meticulous dural closure is sine qua non in preventing postoperative CSF leak. A lumbar drain is a convenient and economical method of managing the problem initially failing which more invasive methods like re-exploration may be employed.


Assuntos
Rinorreia de Líquido Cefalorraquidiano/etiologia , Rinorreia de Líquido Cefalorraquidiano/cirurgia , Derivações do Líquido Cefalorraquidiano/métodos , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/cirurgia , Rinorreia de Líquido Cefalorraquidiano/diagnóstico , Criança , Pré-Escolar , Feminino , Adesivo Tecidual de Fibrina , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/diagnóstico , Estudos Retrospectivos , Doenças da Medula Espinal/cirurgia
19.
J Fish Dis ; 37(7): 609-18, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23952914

RESUMO

'Gold standard' OIE reference PCR assay was utilized to detect the presence of infectious spleen and kidney necrosis virus (ISKNV) in freshwater ornamental fish from Malaysia. From total of 210 ornamental fish samples representing 14 species, ISKNV was detected in 36 samples representing 5 fish species. All positive cases did not show any clinical signs of ISKNV. Three restriction enzymes analyses showed that the fish were infected by identical strains of the same virus species within Megalocytivirus genus. Major capsid protein (MCP) genes of 10 ISKNV strains were sequenced and compared with 9 other reference nucleotide sequences acquired from GenBank. Sequence analysis of MCP gene showed that all strains detected in this study were closely related to the reference ISKNV with nucleotide sequence identity that was ranging from 99.8% to 100%. In addition, phylogenetic analysis of MCP gene revealed that viruses from genus Megalocytivirus can be divided into three genotypes: genotype 1 include reference ISKNV and all other strains that were detected in this study, genotype 2 include viruses closely related to red sea bream iridovirus (RSIV), and genotype 3 include viruses closely related turbot reddish body iridovirus (TRBIV).


Assuntos
Proteínas do Capsídeo/genética , Infecções por Vírus de DNA/veterinária , Doenças dos Peixes/virologia , Peixes , Iridoviridae/genética , Iridoviridae/isolamento & purificação , Animais , Infecções por Vírus de DNA/epidemiologia , Infecções por Vírus de DNA/virologia , Doenças dos Peixes/epidemiologia , Iridoviridae/classificação , Malásia/epidemiologia , Dados de Sequência Molecular , Filogenia , Análise de Sequência de DNA/veterinária
20.
Malays J Pathol ; 36(3): 213-6, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25500522

RESUMO

Ruptured dissecting aortic aneurysm more commonly occur in men in the 40 to 70 age group, and most commonly is associated with atherosclerosis. Uncommon causes are previous heart surgery, connective tissue disorders and aortitis. Despite its rarity, Clostridium spp aortitis progresses very rapidly with a mortality rate of approximately 79% in adults, typically occurring within 48 hours of infection. We present a case of sudden death due to clostridial aortitis causing ruptured aortic dissection in an apparently healthy adult female, 7 weeks post-spontaneous vaginal delivery. This case highlights the pathology of aortic dissection and cystic media necrosis as presentations of clostridium spp infection in young female adult.


Assuntos
Dissecção Aórtica/microbiologia , Ruptura Aórtica/microbiologia , Aortite/microbiologia , Infecções por Clostridium/complicações , Adolescente , Morte Súbita , Evolução Fatal , Feminino , Humanos , Período Pós-Parto
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