La Revue de médecine interne
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The occurrence of acquired hemophilia during pregnancy or postpartum is rare (2 to 10 % in series). It is generally suspected in the presence of haemorrhagic manifestations (especially subcutataneous or mucosal bleeding) associated with an isolated prolongation of the activated partial thromboplastin time (APTT). The diagnosis is confirmed by the association of a low level of factor VIII (FVIII) and the presence of an anti-FVIII inhibitor. ⋯ The management of childbirth is particularly delicate in terms of haemorrhage, especially if the anti-FVIII inhibitor is still present, and must be prepared in a multidisciplinary manner. Finally, as with any acquired hemophilia, a relapse is possible, especially in the year following remission. During a subsequent pregnancy, the risk of recurrence is possible but should not be a contraindication to a new pregnancy.
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Cardiopulmonary complications are the leading cause of mortality in patients with systemic sclerosis (SSc) requiring an early identification. A global and comprehensive approach is needed due to the complexity of the overlapping aetiologies of dyspnoea in SSc. ⋯ CPET may be used to screen for pulmonary arterial hypertension, suspect interstitial lung disease and guide therapeutic strategies including exercise rehabilitation. This review focuses on the clinical value of CPET in the decision-making processes for a more personalised diagnostic approach to SSc-related complications.
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Postpartum ovarian vein thrombosis (POVT) is a rare but serious postpartum complication that can be life-threatening due to its embolic and septic risks. The clinical and paraclinical diagnosis is difficult because of the non-specific signs and the absence of a gold standard for imaging. There is no consensus in the literature on the treatment and follow-up of these patients. The primary objective was to specify the clinical and paraclinical signs suggestive of POVT in order to improve the diagnostic delay. The secondary objectives were to describe the extent of POVT and the proposed immediate therapeutic management. ⋯ The diagnosis of POVT is difficult and needs to be evoked in front of a painful symptomatology or a fever in postpartum. It can be made by ultrasound, but the injected CT scan specifying the specific search for a POVT remains the imaging examination of choice in order to confirm the diagnosis and eliminate differential diagnoses. Under curative anticoagulation and broad-spectrum antibiotic therapy, the clinical course is generally very favourable. A consultation with an internist makes it possible to define instructions for a subsequent pregnancy.
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Acute esophageal necrosis (AEN) is a rare medical disorder, which is characterized by a diffuse black esophageal mucosal during upper gastrointestinal endoscopy which is a highly recommended diagnostic tool. Its high mortality rate requires to be quickly evocated and an early management. ⋯ AEN has to be quickly evocated in a polyvascular and old patient with upper gastrointestinal bleeding. Our experience confirms that optimal and early management allow a esophageal complete healing at 6weeks.
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Chronic haemolysis exposes patients with sickle cell disease (SCD) to the development of black pigment gallstones, which can trigger biliary complications. In order to avoid these complications, elective cholecystectomy is recommended in France for all SCD patients with detected gallstones. ⋯ The conditions for avoiding transfusion for a simple and frequent surgery such as cholecystectomy are based on a benefit- risk balance, and must be discussed on a case-by-case basis by the SCD specialist. In particular, it seems fully justified to perform prophylactic preoperative transfusion in patients with a history of recent vaso-occlusive crisis or acute chest syndrome (within 6 months preoperatively), and those operated on in an emergency setting, who are particularly at risk of postoperative events.