Clarkson’s disease: a rare capillary leak syndrome

Lilas Al Zein1, Cécile Yelnik2, Juliette Woessner2, Angélique Lemaire-Olivier2, Marc Lambert2, Pierre-Yves Hatron2 Published in the journal : January 2019 Category : Internal Medicine

Idiopathic capillary leak syndrome or Clarkson’s disease is a rare and potentially life-threatening condition. This condition presents with recurrent crises, characterized by the abrupt onset of generalized edema with relative hypovolemia associated with paradoxical hypoalbuminemia and elevated hematocrit levels. This almost pathognomonic clinical and biological presentation should remind us of the diagnosis, after excluding secondary causes of capillary leaks, and lead to initiating prompt supportive treatment in a specialized department, in an effort to avoid critical care and associated complications. This article describes the clinical characteristics of the disease and current knowledge about its physiopathology, natural history, and long-term prognosis, while highlighting the efficacy of first-line prophylactic therapy using intravenous immunoglobulins (IVIg) for relapse prevention, designed to avoid progression into a more severe form with gloomy prognosis.

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Cirrhosis management in general medical practice

Sophie Paternostre (1), Jean-Cyr Yombi (1), Peter Stärkel (2) Published in the journal : January 2019 Category : Hépato-gastroentérologie

Liver cirrhosis is commonly encountered in general practice. Although cirrhosis can result from various causes, it most often has a toxic (alcohol), metabolic (nonalcoholic steatohepatitis [NASH]), or infectious (hepatitis B and C) origin. The initial evaluation includes a blood test and an abdominal ultrasound. The diagnosis can be confirmed with non-invasive quantification of fibrosis, using either biological markers (FibroTest®) or transient elastography (FibroScan®). A liver biopsy is only rarely required. Therapeutic management of cirrhosis involves the treatment of the underlying disease, along with the management of complications. Complete alcohol abstinence, weight loss, and the control of risk factors, such as the metabolic syndrome, are some examples. In case of confirmed cirrhosis, abdominal ultrasound and alpha-fetoprotein measurements should be performed every 6 months to screen for hepatocarcinoma. It is also recommended to conduct a gastroscopy every 2-3 years to assess the presence of esophageal varices.

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Suliqua®, a fixed-ratio combination of basal insulin glargine and lixisenatide: role in the management of type 2 diabetes

Martin Buysschaert, Vanessa Preumont (1) Published in the journal : January 2019 Category : Diabétologie

Suliqua® is a new fixed-ratio combination product of the basal insulin glargine 100U/mL and the GLP-1 receptor agonist lixisenatide. This article sought to analyze the recent literature to better identify the product’s potential advantages in general and more particularly, in comparison to basal insulin, and to clarify its precise place in the treatment algorithm for Type 2 diabetes in Belgium.

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Dapagliflozin and cardiovascular events in type 2 diabetes : the model of the DECLARE-TIMI 58 trial

Martin Buysschaert Published in the journal : January 2019 Category : Diabétologie

This paper aimed to review the clinical data on dapagliflozin (Forxiga®) treatment in the light of the recent DECLARE-TIMI 58 trial results. This study involving Type 2 diabetic patients, either with or without prior macroangiopathy, has demonstrated cardiovascular benefits in terms of the primary efficacy outcome, namely a composite of cardiovascular death and hospitalization for heart failure. Moreover, the study has revealed a lower risk for renal disease progression.

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Bullous skin disease

Pamela El Nemnom (1), Marie Baeck (1), Claire Dachelet (2), Liliane Marot (1) Published in the journal : December 2018 Category : Dermatology

We have herein reported on the “PEAU’se dermatologique” meeting conducted at the Cliniques universitaires Saint- Luc on November 13, 2017, while being focused on bullous skin disease. After a brief reminder of the clinical lesions for each condition, the anatomopathological characteristics were specified, with the latest updates about their treatment provided. The newest update concerned rituximab that will now be reimbursed in the context of complicated bullous skin disease.

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Traumatic bipolar dislocation of the clavicle

Alain Colette (1), Arnaud Toussaint, Guillaume Vandemeulebroecke (2) Published in the journal : December 2018 Category : Chirurgie orthopédique et traumatologie

While acromio-clavicular dislocations are common lesions, the association with sterno-clavicular joint dislocation proves to be very rare, with less than fifty cases reported worldwide.

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An uncommon rhabdomyolysis: a case report on a Mycoplasma pneumoniae pneumonia

Laurent Truffaut(1), Caroline Vincent(1), Yves Coyette(2), Pierre Hanotier(2) Published in the journal : December 2018 Category : Infectiologie

We have herein reported on the case of a 97-year-old patient suffering from hypoxemic pneumonia caused by Mycoplasma pneumoniae, complicated by severe rhabdomyolysis. The diagnosis was based on the clinical picture associated with an equivocal serology, after excluding other obvious rhabdomyolysis-based etiologies. The clinical and biological progression proved to be rapidly favorable following targeted antibiotic therapy. After clarifying the unusual rhabdomyolysis etiology within a Geriatric Unit, we have further insisted on both the search and treatment of atypical bacteria when the clinical and radiological presentations point towards these conditions.

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Incidental finding of a nasopharyngeal mass in an anterior pituitary insufficiency setting

Lara Delcour (1), Marie-Anne Labaisse (2), Carine Hinkeltz (3) Published in the journal : December 2018 Category : Endocrinology, Otorhinolaryngology

The differential diagnosis of nasopharyngeal masses is vast and mainly comprises three categories of lesions: benign masses corresponding to benign tumors and inflammatory lesions, malignant tumors, and congenital lesions. The age of the patient, clinical context, symptoms associated with the mass, as well as iconographic data are essential to orientate professionals towards the various possible etiologies. In a context of hypothalamic-pituitary axis malformation, and with reference to the embryological origins of the pituitary gland, the hypothesis of extracranial ectopic pituitary tissue is, although rare, an etiology that must be evoked when confronted to a nasopharyngeal mass. However, biopsy with an anatomopathological examination proved to be the only technique able to confirm the lesion’s histological origin.

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Anomalous right coronary artery from the pulmonary artery

Morgane Slaedts(1), MD, Alain Poncelet(2), PhD, Stéphane Moniotte(1), PhD Published in the journal : December 2018 Category : Cardiovasculaire

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital heart disease, with an incidence of 0.002%. Often asymptomatic upon childhood, the diagnosis is easily missed, with the anomaly often diagnosed incidentally. Nevertheless, ARCAPA may lead to myocardial ischemia or sudden cardiac death, even in early childhood.

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“Autoresuscitation” after cardiac arrest: the Lazarus phenomenon

Michèle J. Yerna (1), Cristian R. Tarta (1), Juline A. Verjans (2), Renaud A. Verjans (2), Amir S. Aouachria (3) Published in the journal : December 2018 Category : Cardiovasculaire

The Lazarus phenomenon is characterized by spontaneous recovery of circulation after cessation of cardiopulmonary resuscitation. The pathophysiological mechanism is still unknown. We have reported the case of a 61-year-old man who collapsed owing to cardiac arrest after anaphylactic shock in relation to hymenoptera bites. Advanced life support was initiated within 10 minutes, and an alternation of shockable and non-shockable rhythms was observed over 60 minutes. Following intravenous administration of 14mg epinephrine and six external electric shocks, treatment was stopped. Monitoring system was maintained, and 5 minutes later, a spontaneous return of electrical activity with palpable pulse was noted. Despite an aggressive treatment, the patient died 12 hours later from refractory shock.

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