[Appropriate use of red blood cell transfusion in the emergency department before and after a specific protocol]. / Pertinence des prescriptions de concentrés de globules rouges par le médecin urgentiste avant et après la mise en place d'un protocole.

The purpose of this study was to assess appropriateness of red blood cell (RBC) transfusion in the emergency department before and after a specific protocol. METHODS: A retrospective monocenter study was realized during two six-month periods at the emergency unit. A protocol based on the French national guidelines (HAS) in 2014 was set up between the two periods. All the adult patients, except in a context of massive transfusion, were included in the study. The principal criteria analysed was the rate of appropriate transfusion. RESULTS: Ninety-five patients were included during the first period (group 1) and 98 during the second one (group 2). The rate of appropriateness was the same in the two groups (81% for the group 1 and 81.6% for the group 2). The inappropriate indications were more frequent with older patients (74.6 ± 15.2 years in the group 1 and 79.5 ± 8.5 in the group 2, P=0.01) and patients suffering from a stable coronary artery disease (18.5% in the group 1 and 38.9% in the group 2, P=0.008). CONCLUSION: The rate of appropriate red blood cells transfusion, already high at the beginning of the study, could not be significantly improved with the institution of a specific protocol. It is necessary to continue medical training.

Oxybutynin as a treatment for generalized hyperhidrosis: a randomized, placebo-controlled trial.

BACKGROUND: Hyperhidrosis is a disorder that can impair quality of life. Localized treatments may be cumbersome and ineffective, and no systemic treatments have proven to be significantly beneficial. OBJECTIVES: To evaluate the effectiveness and tolerance of low-dose oxybutynin for hyperhidrosis. METHODS: We conducted a prospective, randomized, placebo-controlled trial. From June 2013 to January 2014, 62 patients with localized or generalized hyperhidrosis were enrolled. Oxybutynin was started at a dose of 2·5 mg per day and increased gradually to 7·5 mg per day. The primary outcome was defined as improvement of at least one point on the Hyperhidrosis Disease Severity Scale (HDSS). Dermatology Life Quality Index (DLQI) and tolerance were also reported. RESULTS: Most patients (83%) in our study had generalized hyperhidrosis. Oxybutynin was superior to placebo in improving the HDSS: 60% of patients treated with oxybutynin, compared with 27% of patients treated with placebo, improved at least one point on the HDSS (P = 0·009). The mean improvement in quality of life measured by DLQI was significantly better in the oxybutynin arm (6·9) than in the placebo arm (2·3). The most frequent side-effect was dry mouth, which was observed in 43% of the patients in the oxybutynin arm, compared with 11% in the placebo arm. CONCLUSIONS: Treatment with low-dose oxybutynin is effective in reducing symptoms of hyperhidrosis in generalized or localized forms. Side-effects were frequent but minor and mainly involved dry mouth.

[Sorafenib-induced multiple eruptive keratoacanthomas]. / Kératoacanthomes multiples éruptifs induits par le sorafénib.

BACKGROUND: Sorafenib has been approved for use in the treatment of metastatic renal cell carcinoma. Cutaneous side-effects are common, including rash, hand-foot syndrome, alopecia, pruritus, dry skin and erythema. We report an original unexpected cutaneous effect: multiple keratoacanthomas. In the light of a literature review of drug-induced keratoacanthomas, we discuss the potential underlying physiopathological mechanism. CASE REPORT: Three weeks after starting treatment with sorafenib for metastatic renal cell carcinoma, a 64-year-old man developed skin lesions on the face, ears, forearms and thighs having the appearance of dome-shaped nodules with central keratotic cores. Eruptive keratoacanthomas were suspected and were in fact confirmed by histology. Thanks to effective antiangiogenic treatment and the mild discomfort of the keratoacanthomas, sorafenib could be continued. Three weeks after the end of treatment, all lesions had regressed and the patient's skin returned to normal. DISCUSSION: Although the precise aetiology is unknown, the development of eruptive keratoacanthomas is associated with impaired immunity, sun exposure, viral infection, genetic predisposition, radiation therapy and exposure to chemical carcinogens. A few cases of drug-induced keratoacanthomas have been described in the literature and certain immunosuppressant drugs have been implicated. This case suggests that the novel antineoplastic agent sorafenib has a complex activity that, in addition to tyrosine kinases inhibition, includes an immunosuppressant mechanism that can occasionally cause skin lesions.

[Brachioradial pruritus revealing an ependymoma]. / Prurit brachioradial révélant un épendymome.

BACKGROUND: Brachioradial pruritus is a rare form of pruritus localised to one or more brachioradial dermatomes, initially classified as a photodermatosis but which in fact is generally brought on by nervous compression. We report a case of a brachioradial pruritus revealing an intramedullary tumour. PATIENTS AND METHODS: A 53-year-old man had presented pruritus for seven years under the left clavicle, then on the left forearm followed by the right forearm. Finally cervicodynia appeared associated with dysaesthesia of the two upper limbs, fulgurating pains and paresis of the left cubital region. The examination revealed suspended bilateral hypoaesthesia (C4, C5, C6, C7), proprioceptive disorders of the left upper limb, mild motor deficit in the left C8 area and tetrapyramidal syndrome. Cervical radiography did not show cervical osteoarthritis. The MRI revealed a bulky cervical intramedullary tumour extending from C2 to C6. After ruling out cavernoma by medullary angiography, surgery was performed and histopathological analysis of the complete lesion revealed a benign ependymoma. Four months later, this patient complained about residual pains requiring treatment with gabapentin and class 2 analgesics. DISCUSSION: The case presented underlines the possibility of a brachioradial pruritus revealing an intramedullary tumour. Ependymomas are usually seen in children and are frequently evoked in the presence of dysaesthesia. We report the third case of brachioradial pruritus revealing a medullary tumour. The two other cases involved syringomyelia revealed by pruritus in C5 and ependymoma revealed by pruritus in C5-C6. The patient with ependymoma had refused surgical treatment. CONCLUSION: Atypical brachioradial pruritus complicated by neuropathic pains and disorders should prompt screening for a medullary tumour.

[Neonatal haemangiomatosis in identical twins with twin-twin transfusion syndrome]. / Hémangiomatose néonatale chez des jumeaux ayant un syndrome transfuseur-transfusé.

INTRODUCTION: Benign natal haemangiomatosis is characterised by the presence of multiple congenital haemangiomas restricted to the skin. It is differentiated from diffuse neonatal haemangiomatosis in which there is both cutaneous and visceral involvement, with higher morbidity and mortality. PATIENTS AND METHODS: Two identical twins, I and II (monochorionic placenta, biamniotic), born prematurely at 30 weeks' amenorrhoea, presented twin-transfusion syndrome resulting in retarded intrauterine growth in twin I, the donor, and incipient anasarca in twin II, the recipient. Twin I weighed 960 g while twin II weighed 1 200 g. At birth, miliary haemangiomatosis was observed in both infants (16 haemangiomas in I, 19 in II). Abdominal ultrasound and whole-body MRI performed in the two children revealed multiple angiomatous hepatic nodular lesions in I. Subsequent routine clinical and ultrasound monitoring (hepatic and cardiac) showed increased size of the haemangiomatous lesions over the first 4 months followed by stabilisation and gradual regression. No systemic therapy was required. In twin I an episode of ulceration of a neck haemangioma occurred at 5 months and a favourable outcome was obtained on administration of topical hydrocolloid therapy. DISCUSSION: Twin-transfusion syndrome affects 15 to 30% of monochorionic biamniotic pregnancies. It is a serious complication of twin pregnancies resulting from a dynamic process of interfoetal blood transfusion as a result of venous-venous or arteriovenous vascular anastomoses. In the present case, which appears to be the first reported case, it seems that these monochorionic twins, who shared the same placenta, presented haemangiomatosis simultaneously in utero, if we accept the hypothesis of grafting of emboli of placental microvessels in the formation of congenital haemangiomas.

[A guide for education programs in atopic dermatitis]. / Dermatite atopique: un référentiel d'éducation du malade.

BACKGROUND: Education about therapy applies to many chronic diseases. The aim is to improve patient management through the development of certain skills by patients themselves. Atopic dermatitis is an area amenable to the development of therapeutic education. The purpose of this study was to define the skills required for management of atopic dermatitis suitable for therapeutic education and to bring together these skills in a handbook suitable for use. MATERIALS AND METHODS: Thirty caregivers were involved in the drafting of the handbook (dermatologists, a doctor specialising in therapeutic education, a psychologist and nurses), each of whom has experience of therapeutic education in atopic dermatitis. RESULTS: Four age groups were selected (under 5 years, 6 to 10 years, pre-teens/adults, parents of children aged under 5 years). For each age group, different levels of skill were identified for patients or parents of children and suitable learning methods were selected. Skills were classed according to 3 levels: (i) knowledge about the disease, treatments, triggering factors, (ii) knowledge about provision of care by patients or their parents, (iii) knowledge in terms of explaining the disease and treatment methods to family, and knowing who to contact and when. Finally, a 10-question evaluation guide was drawn up. DISCUSSION: In this paper we report the method of production and content of the handbook of skills for atopic dermatitis patients. The aim is not to impose all skills listed in this work on patients but rather to provide caregivers with a complete handbook covering therapeutic education. The book is intended for patients with moderate to severe forms of atopic dermatitis currently in therapeutic failure. It may be used by anyone treating such patients, whether doctors, nurses or psychologists, depending on the items chosen. It is intended for use as a support for the elaboration, diffusion and evaluation of a therapeutic education programme for atopic dermatitis.

[DRESS syndrome related to Hexaquine (quinine and thiamine)]. / Syndrome d'hypersensibilité médicamenteuse à l'Hexaquine (quinine et thiamine).

BACKGROUND: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS syndrome) is a severe drug eruption defined as an association of a drug reaction, mostly cutaneous, laboratory abnormalities with the presence of hypereosinophilia and/or atypical lymphocytes, and involvement of one or more organs. We report a case of DRESS syndrome associated with quinine or thiamine (which in France are combined in Hexaquine with serious hepatitis and renal failure and associated viral reactivation of Human Herpes Virus 6 (HHV6). OBSERVATION: A twenty-three year-old woman was hospitalized for asthenia, fever, erythroderma, facial edema and painful cervical lymph nodes. Laboratory results showed inflammatory syndrome, hypogammaglobulinemia and the presence of activated atypical lymphocytes. Broad screening for infectious serology showed HHV6 reactivation with marked increase in specific IgG levels within two weeks and detection of circulating DNA in blood by PCR (polymerase chain reaction). Two severe complications subsequently appeared: cytolytic hepatitis and interstitial nephritis. The outcome was favorable with oral corticosteroid therapy. Repeated history-taking did not initially identify any causal agent, but several weeks later, the patient remembered taking Hexaquine (quinine and thiamine) for essential muscular cramps daily for 27 days before the onset of symptoms. The patient had taken no other medication during the previous weeks. One year later, at the end of an enteroviral infection, a skin rash similar to the first episode was seen but in isolation on this occasion, suggesting minor reactivation of DRESS syndrome. DISCUSSION: To our knowledge, ours is the first reported case of DRESS syndrome associated with this drug. Because of the difficulty of identifying the causative drug, even though taken alone, our case once again illustrates the importance of spending adequate time on history taking. Although the physiopathology of DRESS syndrome has not yet been fully elucidated, it is nevertheless probable that it is a multi-factor phenomenon (involving medication and viruses among others).

[Evaluation of a new method for detection of obstructive disease in children asthma: the negative expiratory pressure (NEP)]. / Intérêt d'une nouvelle méthode de détection du trouble obstructif dans l'asthme de l'enfant: la pression négative expiratoire (NEP).

INTRODUCTION: To take in charge of an asthmatic child it is necessary to evaluate the lung function. METHODS: In this study, the Negative Expiratory Pressure (NEP) has been used for the first time in children with asthma. After lung spirometry by plethysmography, we have used the NEP to assess the prevalence of expiratory flow limitation (FL) during resting breath in 27 asthmatic children (mean age: 11 +/- 2,5 years) 3-4 days after a crisis in both sitting and supine positions. RESULTS: All the children presented an obstructive defect (FEV 1: 63 +/- 13% med) and a dynamic hyperinflation (FRC: 128 +/- 25% med). According to the NEP, 11 children presented an expiratory flow limitation (FL). Asthma was more severe in the FL than in non-FL children (GINA 2002 classification). Among the 11 FL children, 5 were FL in both sitting and supine position and 6 only in supine. Nine of the 27 children were FL with the conventional method. NEP seems a more accurate method to assess the clinical gravity of asthma than FEV 1. The reduction of FRC in the supine position probably explains the greater incidence of FL in supine position. CONCLUSION: Because of its easy execution, NEP seems to be well adapted for children. Links between FL detected by NEP and clinical signs of asthma has to be assessed by furthers studies including more patients.

Tidal expiratory flow limitation and chronic dyspnoea in patients with cystic fibrosis.

Cystic fibrosis (CF) eventually leads to hyperinflation linked to tidal expiratory flow limitation (FL) and ventilatory failure. Presence of FL was assessed at rest in 22 seated children and adults with CF (forced expiratory volume in one second (FEV1) range: 16-92% predicted), using both the negative expiratory pressure (NEP) technique and the "conventional" method based on comparison of tidal and maximal expiratory flow/volume curves. In addition, chronic dyspnoea was scored with the modified Medical Research Council (MRC) scale. Measurements were made before and 15 min after inhalation of salbutamol. With NEP, FL was present in only three malnourished patients, who had the lowest FEV1 values (16-27% pred) and claimed very severe dyspnoea (MRC score 5). By contrast, an additional seven patients were classified as FL with the conventional method. Six of these patients had little or no dyspnoea (MRC scores 0-1). Salbutamol administration had no effect on the extent of FL, and the concomitant decrease in functional residual capacity (FRC) was too small to play any clinically significant role. This study concluded that in seated patients with cystic fibrosis, expiratory flow limitation is absent at rest, unless the forced expiratory volume in one second is <30% predicted. If present, expiratory flow limitation is associated with severe chronic dyspnoea. The conventional method for assessing expiratory flow limitation is not reliable and bronchodilator administration has little effect on expiratory flow limitation.