Methylene blue for clinical anaphylaxis treatment: a case report.
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CONTEXT AND OBJECTIVE: Nitric oxide has a pathophysiological role in modulating systemic changes associated with anaphylaxis. Nitric oxide synthase inhibitors may exacerbate bronchospasm in anaphylaxis and worsen clinical conditions, with limited roles in anaphylactic shock treatment. The aim here was to report an anaphylaxis case (not anaphylactic shock), reversed by methylene blue (MB), a guanylyl cyclase inhibitor. CASE REPORT: A 23-year-old female suddenly presented urticaria and pruritus, initially on her face and arms, then over her whole body. Oral antihistamine was administered initially, but without improvement in symptoms and signs until intravenous methylprednisolone 500 mg. Recurrence occurred after two hours, plus vomiting. Associated upper respiratory distress, pulmonary sibilance, laryngeal stridor and facial angioedema (including erythema and lip edema) marked the evolution. At sites with severe pruritus, petechial lesions were observed. The clinical situation worsened, with dyspnea, tachypnea, peroral cyanosis, laryngeal edema with severe expiratory dyspnea and deepening unconsciousness. Conventional treatment was ineffective. Intubation and ventilatory support were then considered, because of severe hypoventilation. But, before doing that, based on our previous experience, 1.5 mg/kg (120 mg) bolus of 4% MB was infused, followed by one hour of continuous infusion of another 120 mg diluted in dextrose 5% in water. Following the initial intravenous MB dose, the clinical situation reversed completely in less than 20 minutes, thereby avoiding tracheal intubation. CONCLUSION: Although the nitric oxide hypothesis for MB effectiveness discussed here remains unproven, our intention was to share our accumulated cohort experience, which strongly suggests MB is a lifesaving treatment for anaphylactic shock and/or anaphylaxis and other vasoplegic conditions. (+info)
Pigmented purpuric dermatosis: classification by phenotypic and molecular profiles.
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The categorization of pigmented purpuric dermatosis (PPD) as a form of cutaneous lymphoid dyscrasia has been suggested. Phenotypic and molecular studies were done on 43 patients with PPD. The molecular studies used a capillary gel electrophoresis T-cell receptor beta multiplex polymerase chain reaction assay. There were 2 principal categories: polyclonal PPD represented by 22 cases and monoclonal variants comprising 21 cases. Monoclonal cases had extensive skin lesions. An identical restricted T-cell repertoire independent of time and location was observed. Approximately 40% of the monoclonal cases had clinical and pathologic features of mycosis fungoides (MF). In the polyclonal variant, disease outside the lower extremities was uncommon; there were no patients with MF. Striking reductions in CD7 and CD62L were seen in both groups. PPD is a form of cutaneous T-cell lymphoid dyscrasia, based on the frequency of monoclonality, the preservation of persistent T-cell clonotypes, and extent of pan-T-cell marker loss. Stratification of lesions of PPD according to the molecular profile may be of significant value prognostically and influence therapeutic intervention. (+info)
Improved survival of children with sepsis and purpura: effects of age, gender, and era.
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BACKGROUND: To gain insight into factors that might affect results of future case-control studies, we performed an analysis of children with sepsis and purpura admitted to the paediatric intensive care unit (PICU) of Erasmus MC-Sophia Children's Hospital (Rotterdam, The Netherlands). METHODS: Between 1988 and 2006, all 287 children consecutively admitted with sepsis and purpura were included in various sepsis studies. Data regarding age, gender, ethnicity, serogroup of Neisseria meningitidis, severity, therapy, and survival were collected prospectively. These data were pooled into one database and analyzed retrospectively. RESULTS: The case fatality rate (CFR) from sepsis and purpura was 15.7%. During the study period, survival improved significantly. Younger age was significantly associated with more severe disease and a higher CFR. Children under the median age of 3.0 years had an increased risk of case fatality (odds ratio 4.3, 95% confidence interval 2.1 to 9.2; p < 0.001). Gender was not associated with CFR. However, males did have higher Paediatric Risk of Mortality scores, fewer PICU-free days, and more presence of shock. The course of sepsis and purpura was not related to ethnic origin. A causative organism was isolated in 84.3% of cases. N. meningitidis was the major organism (97.5%). Although N. meningitidis serogroup B was observed more often in younger children, serogroups were not associated with severity or survival. During the study period, the use of inotropic agents and corticosteroids changed substantially (less dopamine and more dobutamine, norepinephrine, and corticosteroids). CONCLUSION: Age and gender are determinants of severity of paediatric sepsis and purpura. Survival rates have improved during the last two decades. (+info)
Improvements in the outcome of children with meningococcal disease.
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Recent years have seen a marked reduction in the mortality of children with meningococcal disease in paediatric intensive care units (PICU); the reasons for this improvement are multifactorial. The mortality rates for critically ill children overall have improved and reasons for this are probably increased centralisation of PICU services and that fewer critically ill children are now looked after on adult units. Specific treatment pathways for sepsis have improved with the publication of clinical guidelines for children and initiatives such as the Surviving Sepsis Campaign. There is a continuing need to focus on the care delivered to children before reaching PICU and to minimise the morbidity suffered by survivors of this disease. (+info)
Angioma serpiginosum: a simulator of purpura.
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We describe two patients with angioma serpiginosum who had both undergone a variety of haematological tests for investigation of purpura, but in whom careful examination of the skin demonstrated abnormal blood vessels rather than extravasated blood. Recognition of vascular disorders which simulate purpura may avoid unnecessary investigations. (+info)
A zebrafish (Danio rerio) model of infectious spleen and kidney necrosis virus (ISKNV) infection.
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Bleeding and bruising: a diagnostic work-up.
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Primary care physicians are often asked about easy bruising, excessive bleeding, or risk of bleeding before surgery. A thorough history, including a family history, will guide the appropriate work-up, and a physical examination may provide clues to diagnosis. A standardized bleeding score system can help physicians to organize the patient's bleeding history and to avoid overlooking the most common inherited bleeding disorder, von Willebrand's disease. In cases of suspected bleeding disorders, initial laboratory evaluations should include a complete blood count with platelet count, peripheral blood smear, prothrombin time, and partial thromboplastin time. More specialized yet relatively simple tests, such as the Platelet Function Analyzer-100, mixing studies, and inhibitor assays, may also be helpful. These tests can help diagnose platelet function disorders, quantitative platelet disorders, factor deficiencies, and factor inhibitors. (+info)