ABSTRACT
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Abstract Hemophilia is a hemorrhagic disorder with a sex-linked inherited pattern, characterized by an inability to amplify coagulation due to a deficiency in coagulation factor VIII (hemophilia A or classic) or factor IX (hemophilia B). Sequencing of the genes involved in hemophilia has provided a description and record of the main mutations, as well as a correlation with the various degrees of severity. Hemorrhagic manifestations are related to levels of circulating factor, mainly affecting the musculoskeletal system and specifically the large joints (knees, ankles and elbows). This document is a review and consensus of the main genetic aspects of hemophilia, from the inheritance pattern to the concept of women carriers, physiopathology and classification of the disorder, the basic and confirmation studies when hemophilia is suspected, the various treatment regimens based on infusion of the deficient coagulation factor as well as innovative factor-free therapies and recommendations for the management of complications associated with treatment (development of inhibitors and/or transfusion transmitted infections) or secondary to articular hemorrhagic events (hemophilic arthropathy). Finally, relevant reviews of clinical and treatment aspects of hemorrhagic pathology charachterized by acquired deficiency of FVIII secondary to neutralized antibodies named acquired hemophilia.
ABSTRACT
ABSTRACT Objective: To describe a case series of four (4) patients with hemolytic uremic syndrome due to Streptococcus pneumoniae in a level four complexity institution in the city of Bogotá, D.C., Colombia. Cases description: We describe cases of four patients who presented respiratory symptoms and fever. All four patients were in regular conditions on hospital admission, after which they required intensive care and ventilatory support. Upon admission, three cases showed evidence of pleuropulmonary complication. Penicillin-sensitive Streptococcus pneumoniae was isolated in all cases. All patients presented anemia, severe thrombocytopenia, schistocytes on peripheral blood smear, and hyperazotemia. They required blood transfusion and renal replacement therapy during their hospitalization. The patients were diagnosed with hemolytic uremic syndrome due to S. pneumoniae. Three of the four patients had a progressive recovery of the renal function and were discharged after an average of 36 days of hospital stay. The remaining patient had two amputations in the extremities due to thrombotic vascular complications and was discharged after 99 days of hospital stay, requiring hemodialysis every other day. Comments: Hemolytic uremic syndrome due to Streptococcus pneumoniae is a rare but severe complication of invasive pneumococcal disease. Complicated pneumonia is the main condition associated with this entity. It is noteworthy the short period in which these cases were presented, considering the low annual incidence of the disease.
RESUMO Objetivo: Descrever uma série de casos de quatro pacientes com síndrome hemolítico-urêmica por pneumococo em uma instituição de referência em Bogotá, Colômbia. Descrição dos casos: Descrevemos os casos de quatro pacientes que apresentaram sintomas respiratórios e febre. Todos estavam em estado geral regular à admissão hospitalar e necessitaram de cuidados intensivos e suporte ventilatório. Na admissão, em três dos casos foi evidenciada a complicação pleuropulmonar. Isolamento de Streptococcus pneumoniae sensível à penicilina foi realizado em todos os casos. Os quatro pacientes precisaram de transfusão sanguínea e terapia de reposição renal durante a hospitalização. Nos testes laboratoriais, observou-se anemia, trombocitopenia grave, presença de esquizócitos em esfregaço de sangue periférico e hiperazotemia. Com esse quadro, o diagnóstico foi de síndrome hemolítico-urêmica associada à infecção por S. pneumoniae. Houve recuperação progressiva da função renal em três dos quatro pacientes, que tiveram alta após 36 dias de internação hospitalar, em média. Um paciente teve complicações vasculares trombóticas, resultando em duas amputações nas extremidades, e teve alta após 99 dias de internação, com necessidade de hemodiálise em dias alternados. Comentários: A síndrome hemolítico-urêmica por Streptococcus pneumoniae é uma complicação rara, mas grave, da doença invasiva pneumocócica. A pneumonia complicada é a principal condição associada a essa entidade. Destaca-se o curto período em que esses casos foram apresentados, levando em conta a baixa incidência anual de síndrome hemolítico-urêmica.
Subject(s)
Humans , Male , Infant , Child, Preschool , Adolescent , Pneumococcal Infections/complications , Streptococcus pneumoniae/isolation & purification , Hemolytic-Uremic Syndrome/etiology , Hemolytic-Uremic Syndrome/therapy , Pneumococcal Infections/microbiology , Pneumococcal Infections/therapy , Pneumococcal Infections/diagnostic imaging , Pneumonia, Pneumococcal/diagnosis , Shock, Septic/etiology , Thrombosis/surgery , Blood Transfusion/methods , Treatment Outcome , Renal Replacement Therapy/methods , Renal Insufficiency/etiology , Renal Insufficiency/therapy , Hemolytic-Uremic Syndrome/diagnosis , Amputation, Surgical/methods , Length of Stay/statistics & numerical dataABSTRACT
Regional anesthesia and pain management have experienced advances in recent years, especially with the advent of fascial plane blocks. The erector spinae plane block is one of the newest techniques to be described. In the past two years, publications referring to ESP block have increased significantly. The objective of this review is to analyze the articles about ESP block that have been published to date. We performed a search in the main databases and identified 368 articles. After a selection of the relevant articles, 125 studies were found eligible and were included in the review. The ESP block is performed by depositing the local anesthetic in the fascial plane, deeper than the erector spinae muscle at the tip of the transverse process of the vertebra. Many cases of its use have been described with satisfactory results in the treatment of both acute pain and chronic pain. The applicability of the technique covers many clinical scenarios. Of the 98 case reports reviewed, 12 and 87 articles, respectively described the technique as a treatment for chronic pain and acute pain. The single-shot was the most frequently used technique. As described in the articles published to date, the technique is easy to perform and has a low rate of complications. However, despite the effectiveness of the technique, further studies are necessary to obtain more evidence of its actions.
ABSTRACT
Regional anesthesia and pain management have experienced advances in recent years, especially with the advent of fascial plane blocks. The erector spinae plane block is one of the newest techniques to be described. In the past two years, publications referring to ESP block have increased significantly. The objective of this review is to analyze the articles about ESP block that have been published to date. We performed a search in the main databases and identified 368 articles. After a selection of the relevant articles, 125 studies were found eligible and were included in the review. The ESP block is performed by depositing the local anesthetic in the fascial plane, deeper than the erector spinae muscle at the tip of the transverse process of the vertebra. Many cases of its use have been described with satisfactory results in the treatment of both acute pain and chronic pain. The applicability of the technique covers many clinical scenarios. Of the 98 case reports reviewed, 12 and 87 articles, respectively described the technique as a treatment for chronic pain and acute pain. The single-shot was the most frequently used technique. As described in the articles published to date, the technique is easy to perform and has a low rate of complications. However, despite the effectiveness of the technique, further studies are necessary to obtain more evidence of its actions.