Myopathie inflammatoire post-méningococcémie aiguë chez un patient présentant un déficit en properdine : à propos d’un cas

IntroductionMyalgia is a classical sign in invasive meningococcal diseases (IMD), but severe and persistent myalgia following an IMD have never been reported to date.Case reportA 20-year-old man presented with purpura fulminans and meningitis caused by Neisseria meningitidis serogroup Y, revealing properdin deficiency. Although meningitis symptoms improved after antibiotherapy, initial myalgia of the lower limbs increased, associated with mild rhabdomyolysis. Magnetic resonance imaging (MRI) revealed an increased STIR (Short TI inversion recovery) signal of both quadriceps muscles, without abscess. After exclusion of other causes of myopathy, a post-infectious myositis was diagnosed. A four-week course of corticosteroids led to dramatic improvement.ConclusionPost-infectious inflammatory myopathy should be suspected in case of severe and persistent myalgia associated with rhabdomyolysis following an IMD, after exclusion of pyomyositis especially. A short course of corticosteroids seems to be effective.     

Tamponnade révélant un kyste hydatique intra-péricardique – un cas

Introduction

Hydatid disease is a parasitic infection caused by the development of the larval form of the Teania of Echinococcus granulosus. It is endemic in many regions of the world such as the Mediterranean basin. Location without pericardial cardiac involvement is extremely rare. In this case study, we will elaborate the case of an intra-pericardial hydatidosis disease without cardiac location revealed by a tamponade.

Observation

AA is a 60-year-old man with no pathological history and who was admitted for a tamponade assessment. Indeed, the cardiovascular examination showed a muffling of the heart sound and signs of a right heart failure. Besides, the ECG shows a microvoltage, and the chest radiography shows cardiomegaly. Moreover, the transthoracic echocardiogram confirmed the presence of an abundant pericardial effusion along with signs of a tamponade. It also reifies the presence of, at the intra-pericardial level, a multiple vesicular formation giving a cluster of grapes highly suggestive of an intra-pericardial hydatid disease. An emergency surgical drainage allowed removing about two liters of suspicious fluid along with cysts and white membranous. The pathological examination of the membranous confirmed the diagnosis of a pericardial hydatid cyst. The performance of a chest CT and an abdominal ultrasound's scan to identify other locations shows no further anomalies. The patient was put under medical treatment (Albendazole^®) and is showing a good clinical improvement.

Conclusion

The intra-pericardial hydatid disease is another cause of tamponade not to fail despite its rareness, as it is endemic to North African countries.     

Infection des abords vasculaires et interventions cardiaques percutanées : un risque significatif ?

Vascular access site infections are infrequent and rarely reported as a potential complication of percutaneous cardiac intervention. A case of access site infection is reported with a literature review. Femoral access is mainly concerned in some circumstances: delayed sheath withdrawal, vascular complications (hematoma, false-aneurysm, arteriovenous fistula), or use of hemostatic closure device. These infectious complications are always serious requiring medical and surgical treatment and potentially associated with life-threatening complications. Preventive measures should be applied in order to reduce the risks: optimisation of femoral punctures with the support of echography guidance, avoid a new puncture in a area with hematoma, femoral angiographic evaluation and strict aseptic precautions with vascular closure devices, and obviously preferential choice of radial access.     

Dysfonction endothéliale au cours du Purpura Thrombotique Thrombocytopénique : un nouvel axe de prise en charge ?

Immune Thrombotic Thrombocytopenic Purpura (iTTP) is a rare but severe disease with a mortality rate of almost 100 % in the absence of adequate treatment. iTTP is caused by a severe deficiency in ADAMTS13 activity due to the production of inhibitory antibodies. Age has been shown to be a major prognostic factor. iTTP patients in the elderly (60yo and over) have more frequent organ involvement, especially heart and kidney failures compared with younger patients. They also have non-specific neurologic symptoms leading to a delayed diagnosis. Factors influencing this impaired survival among older patients remain unknown so far. Alteration of the functional capacity of involved organs could be part of the explanation as could be the consequences of vascular aging. In fact, severe ADAMTS13 deficiency is necessary but likely not sufficient for iTTP physiopathology. A second hit leading to endothelial activation is thought to play a central role in iTTP. Interestingly, the mechanisms involved in endothelial activation may share common features with those involved in vascular aging, potentially leading to endothelial dysfunction. It could thus be interesting to better investigate the causes of mid- and long-term mortality among older iTTP patients to confirm whether inflammation and endothelial activation really impact vascular aging and long-term mortality in those patients, in addition to their presumed role at iTTP acute phase. If so, further insights into the mechanisms involved could lead to new therapeutic targets.     

Histiocytose langerhansienne révélée par un désordre de l’appareil manducateur : rapport d’un cas et revue de la littérature des atteintes crâniofaciales

IntroductionLangerhans cell histiocytosis (HL) is a rare disease that can affect all tissues. Oral manifestations such as mucosal ulcer and tooth mobility are often the first signs of the disease. We report a rare case of mandibular condyle unifocal HL mimicking a temporomandibular joint disorder.Case reportA 44-year-old patient presented with a left temporomandibular disorder with painful left preauricular swelling. The imaging assessment found a bone lesion of the left mandibular condyle. A curettage with biopsy was used to diagnose HL. Six months later, the patient had no more pain.DiscussionThe craniofacial clinical expressions of HL mainly concern the bones, which can cause: pain, swelling, fracture, compression of noble organs. The other sites are: oral cavity, skin, lymph nodes, or eyes. Isolated forms are generally benign, and their treatment is discussed between abstention and non-aggressive surgery.     

Réaction transfusionnelle hémolytique retardée due à un anti-U

Delayed hemolytic transfusion reactions due to anti-U are rare, only two (2) cases having been reported in the literature. We now report a third case : a multiparous black woman without any transfusion history was admitted to hospital for severe microcytic anemia (31 g/l). The patient was group AB negative, the direct antiglobuline test was negative and an anti P₁ cold allo-antibody was present in her serum. Five A, Rh negative, P₂ packed red cells were cross-marched with the sample obtained at admission on January 8, 1988. She was transfused on January 8, 9, 10, 11 and 12. On the 12th of January her hemoglobin level reached 125 g/l. On January 13, the patient presented clinical signs of hemolysis and her hemoglobin fell to 60 g/l within 24 hours. On january 15, the direct antiglobulin test was positive and an antibody found in her serum was reactive with all the red cells of the commercial panel. The sample was referred to our red cell serology reference laboratory. The phenotype of the pre-transfusion sample was found to be Fy(a-b-) M, N, S-s-U-. An anti-U was detected in the eluate and the serum. The patient was transfused with two (2) units of O-P₂, U-red cells obtained from the American Red Cross, Syracuse, and her hemoglobin reached 90 g/l within 48 hours. This is the third reported case of a delayed hemolytic transfusion reaction due to anti-U. This case illustrates the need to perform cross-matches with samples obtained within 48 hours of the scheduled transfusion for patients who have been transfused with blood in the preceding 3 months. Also, this case emphasises the need to recruit U negative blood donors for the Canadian rare donor file.