Endothelial dysfunction and hypercoagulability in severe sickle cell acute chest syndrome

RationaleAcute pulmonary hypertension (PH) may develop during sickle-cell acute chest syndrome (ACS), and is associated with an increased mortality. Its mechanisms remain poorly known. The question was to assess if there is an endothelial dysfunction and a hypercoagulability in severe ACS, with and without acute PH.MethodsIn a prospective monocenter cohort follow-up study, all sickle-cell adult patients with ACS admitted to the intensive care unit underwent a trans-thoracic echography (TTE), and measurements of biomarkers of coagulation, endothelial activation, and platelet and erythrocyte activation. Acute PH was defined as a high echocardiographic probability of PH. The biological profiles of sickle-cell patients were analysed at the time of ACS, contrasting the existence of acute PH, and compared with steady state and with non-sickle-cell controls (healthy subjects and community-acquired pneumonia (CAP)).ResultsMost patients (36 patients with 39 ACS episodes; 23 males; 27 years old) had thoracic pain, dyspnea and CT scan lung consolidation. Acute PH was diagnosed in 7 patients (19%). Erythrocyte and platelet-derived microparticles (MPs) and the pro-coagulant activity of MPs were higher in ACS patients with acute PH, as compared with their counterparts. As compared with healthy controls, ACS patients had higher levels of tissue factor, fibrin monomers, D-dimer, release of pro-coagulant microparticles, and erythrocyte and platelet-derived MPs. As compared with CAP patients, ACS patients had increased levels of fibrin monomers, and erythrocyte and platelet-derived MPs.ConclusionsSevere ACS is characterised by endothelial dysfunction and hypercoagulability state, with a marked pro-coagulant profile in case of associated PH.

Nitroglycerin inhalation for acute treatment of pulmonary arterial hypertension in children with congenital heart disease

Objectives: Acute pulmonary hypertension and pulmonary hypertensive crisis may result in adverse clinical outcomes if unsuccessfully treated. Inhaled nitric oxide has long been considered as the standard pharmacotherapy for acute pulmonary hypertension, but lack of feasibility in some settings and evidences challenging its benefits lead to the use of alternative treatment, amongst which is nitroglycerin inhalation. The purpose of this review article is to discuss available data on the use of nitroglycerin inhalation for acute treatment of pulmonary hypertension in children with CHD and its potential benefit in post-operative setting. Data sources: Literatures included in this review were acquired by searching in PubMed online database. Keywords used were “Pulmonary Hypertension”, “Congenital heart defects”, “Pediatrics”, “Inhaled nitroglycerin”, and its synonyms. Study selection: Title and abstract were screened to select relevant literatures including the three paediatric clinical trials on nitroglycerin inhalation. Critical appraisal of the clinical trials was then done using the University of Oxford Centre of Evidence-Based Medicine Critical Appraisal Tools. Conclusions: Paediatric studies showed the benefit of nitroglycerin inhalation in uncorrected cases of CHD during catheterisation procedures. Until recently, there have been no studies conducted in paediatric post-operative CHD cases. Further study is required to provide evidence for inhaled nitroglycerin use in this setting including the appropriate dosing and potential side effects with repeated administration,

Thiamine-Responsive Acute Pulmonary Hypertension of Early Infancy (TRAPHEI)—A Case Series and Clinical Review

Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of high pulmonary vascular resistance (PVR) commonly seen all over the world in the immediate newborn period. Several case reports from India have recently described severe pulmonary hypertension among infants in the postneonatal period. These cases typically present with respiratory distress in 1–6-month-old infants, breastfed by mothers on a polished rice-based diet. Predisposing factors include respiratory tract infection such as acute laryngotracheobronchitis with change in voice, leading to pulmonary hypertension, right atrial and ventricular dilation, pulmonary edema and hepatomegaly. Mortality is high without specific therapy. Respiratory support, pulmonary vasodilator therapy, inotropes, diuretics and thiamine infusion have improved the outcome of these infants. This review outlines four typical patients with thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI) due to thiamine deficiency and discusses pathophysiology, clinical features, diagnostic criteria and therapeutic options.

Emboli Air Ketuban

Emboli cairan amnion (EAK) adalah komplikasi kehamilan yang jarang namun membawa angka mortalitas yang tinggi. Patogenesis yang tepat dari kondisi ini masih belum diketahui. Emboli air ketuban (EAK) atau amniotic fluid embolism (AFE) atau anaphylactoid syndrome of pregnancy adalah salah satu komplikasi kehamilan yang paling membahayakan. Cairan ketuban, debris fetal diduga menyebabkan kolaps kardiovaskular dengan cara memicu reaksi imun/anafilaktoid maternal. Patofisiologi EAK hingga kini masih belum jelas tetapi diduga melibatkan kaskade immunologis. Kematian maternal bisa terjadi karena cardiac arrest mendadak, perdarahan karena koagulopati, dan kegagalan organ multipel dengan acute respiratory distess syndrome (ARDS). Gejala dan tanda EAK antara lain dispnea akut, batuk, hipotensi, sianosis, bradikardia fetal, ensefalopati, hipertensi pulmoner akut, koagulopati, dan sebagainya. Diagnosis EAK adalah bersifat klinis dan ditegakkan setelah menyingkirkan kemungkinan penyebab lain. Penatalaksanaan bersifat suportif dan memerlukan persalinan janin jika diperlukan, support respiratorik, dan support hemodinamik. Prognosis maternal setelah EAK masih sangat buruk meski tingkat survival janin sekitar 70%. Pasien dengan EAK paling baik dikelola di unit perawatan kritis oleh tim multidisiplin dan dengan manajemen supportif. Amniotic Fluid Embolism Abstract Amniotic fluid embolism (AFE) is a rare complication of pregnancy carrying a high mortality rate. The exact pathogenesis of the condition is still not known. Amniotic fluid embolism (AFE) or anaphylactoid syndrome of pregnancy is one of the most dangerous pregnancy complications. Amniotic fluid, fetal debris is thought to cause cardiovascular collapse by triggering a maternal immune / maternal anaphylactoid reaction. The pathophysiology of AFE remains unclear but is thought to involve an immunological cascade. Maternal deaths may occur due to sudden cardiac arrest, bleeding due to coagulopathy, and multiple organ failure with ARDS. AFE symptoms and signs include acute dyspnea, cough, hypotension, cyanosis, fetal bradycardia, encephalopathy, acute pulmonary hypertension, coagulopathy. Management is supportive, respiratory support, and haemodynamic support. The maternal prognosis is very poor even though the survival rate of the fetus is about 70%. Patients with AFE are best managed in a critical care unit by a multidisciplinary team and management is largely supportive