Capillary leak syndrome: State of the art in 2021.

Capillary leak syndrome (CLS) is an increasingly acknowledged multifaceted and potentially lethal disease. Initial nonspecific symptoms are followed by the intriguing CLS hallmark: the double paradox associating diffuse severe edema and hypovolemia, along with hemoconcentration and hypoalbuminemia. Spontaneous resolutive phase is often associated with poor outcome due to iatrogenic fluid overload during leak phase. CLS is mainly triggered by drugs (anti-tumoral therapies), malignancy, infections (mostly viruses) and inflammatory diseases. Its idiopathic form is named after its eponymous finder: Clarkson's disease. CLS pathophysiology involves a severe, transient and multifactorial endothelial disruption which mechanisms are still unclear. Empirical and based-on-experience treatment implies symptomatic care during the acute phase (with the eventual addition of drugs amplifying cAMP levels in the severest cases), and the prophylactic use of monthly polyvalent immunoglobulins to prevent relapses. As CLS literature is scattered, we aimed to collect and summarize the current knowledge on CLS to facilitate its diagnosis, understanding and management.

[Idiopathic systemic capillary leak syndrome: 2 cases with misleading presentation]. / Syndrome de fuite capillaire idiopathique : 2 cas cliniques de présentation trompeuse.

INTRODUCTION: Idiopathic systemic capillary leak syndrome (ISCLS) also known as Clarkson syndrome is a rare and sudden life-threatening entity. Three consecutive phases are described. A first non-specific prodromal phase often manifests as "flu-like" symptoms and precedes capillary leak phase with major hypovolemic and distributive shock leading to serious and frequent multiorgan dysfunction syndrome (MODS). Severe hypovolemia contrasts with edema, and hemoconcentration with hypoalbuminemia. ISCLS is characterized by these two clinical and biological paradoxes. Subsequent recovery phase exhibits organ function restoration along with interstitial/intravascular volumes normalization. The latter occurs spontaneously and systematically in patients surviving from leak phase. OBSERVATIONS: We report here two ISCLS cases admitted in intensive care unit (ICU) both enhancing initial misdiagnosis possibly lowering prognosis and outcome. Our first 28-year-old female patient was admitted for « polycythemia vera ¼ although hemoconcentration was attributable to hypovolemia. She presented circulatory arrest during the second bloodletting session and complicated with MODS. In and out ICU favorable outcome was noted on intravenous immunoglobulin therapy. A second 57-year-old male patient was admitted in ICU for severe "myositis" (myalgia and rhabdomyolysis) although rectified diagnosis retained compartment syndrome (muscular severe edema following capillary leak). Rapid and refractory hypovolemic shock appeared with subsequent MODS leading to death. CONCLUSION: ISCLS pathophysiology remains unknown but certainly implies transitory endothelial dysfunction. Impossibility of randomized controlled trial for this exceptional disease led to based-on-experience therapeutic guidelines implying symptomatic care (cardiac output surveillance, nephroprotection, prudent fluid intake, prudent vasoactive amine use) and specific therapies (intravenous aminophylline during severe flares). Although enhancing controversial and even deleterious effects during the acute phase, polyvalent immunoglobulins are effective for relapse prevention. Syndromic diagnosis is difficult, but its precocious finding constitutes a key-element in better outcome before organ failure.

Sources of interrater variability and prognostic value of standardized EEG features in post-anoxic coma after resuscitated cardiac arrest.

OBJECTIVES: To assess interrater variability and prognostic value of simple EEG features based on the recent American Clinical Neurophysiology Society (ACNS) classification in post cardiac arrest comatose patients. METHODS: All patients admitted for a resuscitated cardiac arrest in a university hospital were prospectively included in the study. EEG interpretation was made by 3 independent neurophysiologists (2 senior and 1 junior) blind to the outcome. Kappa score and prognostic performances were estimated for each EEG pattern and discrepancies were analyzed. RESULTS: 122 cardiac arrest patients were admitted of whom 48 went through a full neurologic evaluation. Eighty-one percent had an unfavorable outcome. Burst suppression, paroxystic seizure activity, and non-reactive EEG were strongly associated with an unfavorable evolution. Kappa score between the senior neurophysiologists was excellent or substantial while it was only fair or slight between the junior and senior neurophysiologists. Reactivity, discontinuity and electrographic seizure were patterns particularly subject to discrepancy. CONCLUSIONS: Bedside EEG is an excellent tool for predicting outcome of post-anoxic coma through simple EEG features. However, the interrater variability emphasizes the need to be well trained for the standardized methods of evaluating EEG parameters. SIGNIFICANCE: A second look at complex patterns seems mandatory. The development of automated tools could help to improve the reliability of EEG interpretation.

Tropheryma whipplei DNA in bronchoalveolar lavage samples: a case control study.

Tropheryma whipplei, the causative bacterium of Whipple's disease, can cause acute pneumonia. We performed a case-control study including patients with T. whipplei in bronchoalveolar lavages (BALs) and controls in order to compare patients' clinical statuses. We tested T. whipplei PCR from January 2013 to December 2014, in all the 1438 BALs in Marseille, France. Controls were hospitalized in the same unit during the same period and were comparable in age and sex. Eighty-eight BALs (6.1%) were positive for T. whipplei and 58 patients had pneumonia. Sixty-four patients were male with a mean age of 50.5 years. T. whipplei was commonly associated with aspiration pneumonia (18/88 patients compared with 6/88 controls, p 0.01) and was detected as a unique pathogen in nine cases. Overall, no difference was observed regarding immunocompromised status. Nevertheless, the six AIDS-infected patients in the T. whipplei group had a significantly lower CD4 level than the five AIDS-infected patients in the control group (49 vs. 320/mm3, p 0.01); in addition, five patients were treated with tumour necrosis factor alpha inhibitors (including three treated by monocolonal antibodies and two with soluble receptor) compared with none of the controls (p 0.03). Pneumocystis jirovecii was frequently associated with the T. whipplei group (7/88 vs. 0/88 in control group), Pseudomonas aeruginosa was only detected in the control group (8/88). This study adds evidence for a causative role of T. whipplei in pneumonia. In the future, an experimental model of pneumonia induced by T. whipplei will prove its role in pneumonia.

[Ischemic heart failure making the diagnosis of a Vaquez disease: a rare event]. / Choc cardiogénique ischémique révélateur d'une maladie de Vaquez, un événement rare.

We present the case of a 46-year-old patient without any past medical history, admitted to our ICU for cardiogenic shock complicating acute coronary syndrome. The blood tests found polycethemia, a polycethemia vera was suspected and confirmed by genetic analysis. Ischemic heart failure as an initial symptom of polycethemia vera and its treatment by arterial bleeding is a rare event that we describe in this article.

[Severe air embolism after surgical irrigation with hydrogen peroxide]. / Embolie gazeuse grave après une irrigation chirurgicale à l'eau oxygénée.

BACKGROUND: Hydrogen peroxide is widely used for its antiseptic properties. In certain circumstances, however the risk of air embolism can create a life-threatening situation. CASE REPORT: A 16-year-old adolescent required surgical treatment for femorotibial trauma. During the surgical procedure, cardiac arrest suddenly occurred when hydrogen peroxide was being used to irrigate the wound. A central catheter was inserted and aspiration of air bubbles in the line led to the diagnosis of air embolism. Outcome was unfavorable despite successful resuscitation. A chronic neurovegatative state ensued and the patient died 8 months later. DISCUSSION: Several cases of air embolism have been described due to hydrogen peroxide in surgical, medical and accidental circumstances. Our case emphasizes the potential danger of using hydrogen peroxide in certain situations, including orthopedic surgery. Clinicians should be aware that hydrogen peroxide is not a perfectly safe product.

[Fat embolism after intrapenile injection of sweet almond oil]. / Embolie graisseuse après injection intrapénienne d'huile d'amande douce.

We report a case of fat embolism following self injection of vegetable oil in the penis, to treat an impotence. The patient developed respiratory failure and neurological disorders as confusion. A chest roentgenogram revealed diffuse alveolar infiltrate. Initially, he presented an hemoconcentration, and fat globules in his urine. The outcome was favourable in 72 hours, with oxygen and hydration. The diagnosis of fat embolism was made after neurological improvement: the patient admitted to injection his penis with vegetable oil, in his corpus cavernosum.