The literature describing tuberculosis (TB) in hematopoietic stem cell transplant (HSCT) recipients is scant, even in countries where TB is common. We describe a case of pulmonary TB in a patient who underwent HSCT and review the English language literature on this subject. An extensive PubMed and Ovid search was undertaken for the period January 1980 to March 2009; the search terms used were 'Mycobacterium tuberculosis' or 'tuberculosis', in combination with 'hematopoietic stem cell transplantation' or 'bone marrow transplantation'. The patient in the present case report underwent allogeneic transplantation and developed TB 8 days after his HSCT. The patient had received vaccination against TB in childhood. During the year prior to the HSCT he had had contact with a relative who had pulmonary TB. On day 3 of anti-TB treatment he developed pericarditis. The patient received anti-TB treatment for 6 months without major problems. From the literature review, we found 34 related studies, 25 on the clinical manifestations of TB. Most of the reports were from Asia (48%), and the incidence of TB varied from 0.0014% in the USA to 16% in Pakistan. TB occurred at between +21 and +1410 days post-HSCT (257.2 days the median), and the lung was the organ most frequently involved. Mortality varied from 0% to 50% and was higher in allogeneic HSCT. There is no consensus regarding screening with the tuberculin skin test or primary prophylaxis for latent TB, and further research into this is necessary in developing countries with a high prevalence of TB.
SummaryHaemoglobin (Hb) SC disease is the second most common subtype of sickle cell disease and is potentially fatal. This study aimed to determine the clinical characteristics, outcome and predictors of mortality in HbSC disease patients, and to compare these findings with patients followed-up in different centres. Clinical, laboratory and outcome data were collected from a cohort of adult patients with HbSC disease followed between 1991 and 2103. Cox regression multivariate analysis was used to determine predictors of mortality. One hundred and fifty-five patients were followed-up over 20 years: 9% died and 70Á8% had at least one complication. The most common complications were: painful crises (38Á3%), retinopathy (33Á8%), cholelithiasis (30Á3%), osteonecrosis (24Á8%) and sensorineural hearing disorders (9Á7%). Frequency of chronic complications was similar in most studies. In multivariate analysis, hearing disorders remained an independent predictor of mortality (Odds Ratio 9Á26, 95% confidence interval 1Á1-74Á8; P = 0Á03). It was concluded that patients with HbSC disease receive a late diagnosis and there is remarkable similarity between the studies conducted in different centres around the world. Sensorineural hearing disorders were an independent predictor of mortality, suggesting that it may be useful to implement routine diagnostic screening.
As porfirias são doenças incomuns e de herança genética na maior parte dos casos. As porfirias são divididas em eritropoiéticas, hepáticas agudas e hepáticas crônicas. Os subtipos de maior relevância clínica são a porfiria cutânea tarda e a porfiria intermitente aguda. O diagnóstico das porfirias pode ser bastante difícil, dada a sobreposição de quadros clínicos e achados bioquímicos. A precisão do diagnóstico depende da dosagem de porfirinas urinárias e fecais, da análise da atividade enzimática de eritrócitos e, eventualmente, da pesquisa de mutações. O objetivo do presente artigo é realizar revisão literária das porfirias, com ênfase no diagnóstico e tratamento de seus diversos subtipos.
INTRODUCTION: Head trauma is an important consequence of child abuse. Specific pathophysiological mechanisms in child abuse are responsible for the ''whiplash shaken-baby syndrome'', which would favour the occurrence of intracranial hemorrhages. CASE REPORT: We report the case of a child who developed epidural hematoma following minor-intensity head trauma. Initial diagnosis of child abuse was made, but subsequent investigation led to the diagnosis of hemophilia A. CONCLUSION: Even though epidural hematoma is not closely associated with child abuse, this aethiology must always be considered when the reported trauma mechanism is out of proportion to the magnitude of the encountered lesions.
We report a rare case of aseptic arthritis in the temporomandibular joint of a patient with sickle cell anemia. A 22-year-old woman with sickle cell disease, in the 18th week of gestation, was referred by her hematologist to investigate a sudden mouth opening limitation and severe pain on her left cheek. The patient received a standard pain assessment protocol, clinical examination, and complementary exams (complete blood count, hemoglobin electrophoresis, blood solubility test, panoramic radiograph, and magnetic resonance imaging [MRI]). The blood results were consistent with a sickle cell crisis and the MRI showed an inflammatory process around the left temporomandibular joint. Treatment with opioid analgesics and blood transfusion provided good results. Sickle cell anemia is a disease that can cause arthritis of the temporomandibular joint, and although it is rare, clinicians should be attentive to the differential diagnosis in patients with this disease.
A anemia ferropriva grave secundária à hemorragia digestiva por angiodisplasiasintestinais representa um grande desafio terapêutico. Comumente, as ectasias vasculares sãomúltiplas e dispersas ao longo do intestino, limitando a eficácia do tratamento hemostáticolocal. Nos últimos anos houve significativo avanço no tratamento anti-angiogênico sistêmico dasangiodisplasias intestinais, sendo talidomida a droga mais empregada para tal fim. Relatamoso caso de uma paciente de 49 anos com angiodisplasias intestinais secundárias a síndromeCREST (Calcinose, Raynaud, Dismotilidade Esofágica, Esclerodactilia e Telangiectasias). Apaciente apresentava quadro de melena recorrente e alta necessidade transfusional, e nãoobteve resposta clínica após realização de enteroscopia e eletro-coagulação das lesões complasma de argônio. Após a introdução de talidomida 100mg ao dia, a paciente evoluiu deforma bastante satisfatória. O caso apresentado neste texto, além de demonstrar sucesso datalidomida no tratamento de angiodisplasias intestinais refratárias à eletro-coagulação complasma de argônio, também revela eficácia da droga na situação específica da síndromeCREST. Tal fato pode ser de grande valia quando da abordagem de hemorragia intestinal porangiodisplasias nesses pacientes, representando nova opção terapêutica
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