Effect of single-unit transfusion in patients treated for haematological disease including acute leukemia: A multicenter randomized controlled clinical trial.

BACKGROUND: Retrospective studies in hematological unit have suggested that single red blood cell (1-RBC) unit transfusion policy may reduce the number of RBC used without negative clinical impact. METHOD: Acute leukemia patients requiring intensive chemotherapy or patients receiving autologous or allogeneic transplantation were randomly assigned to receive either single RBC (1-RBC arm) or double RBC (2-RBC arm) per transfusion with a hemoglobin trigger of 8 g/dL. The primary composite endpoint was the percentage of patients experiencing serious complications, such as a non-hematological adverse event grade ≥ 3 or intensive care admission or death. FINDINGS: A total of 981 and 592 RBC transfusions were required in the 1-RBC arm (n = 125) and the 2-RBC arm (n = 120), respectively. The mean pre-transfusion hemoglobin levels were 7.49 ± 0.83 g/dL in the 1-RBC arm and 7.46 ± 0.67 g/dL in the 2-RBC arm (p = 0.275). The predefined non-inferiority criteria was achieved with 28/125 patients reaching the primary endpoint in the 1-RBC arm (22.4 %) and 28/120 patients in the 2-RBC arm (23.3 %) (Risk difference 0.009; 95 %, Confidence interval [-0.0791 to 0.0978], p = 0.021). The median (IQR) of RBC units transfused per patient was 7 (4-12) in the 1-RBC arm and 8 (4-12) in 2-RBC arm. Hemoglobin levels at discharge were also comparable in both arms. INTERPRETATION: The results of this trial indicate that a single RBC transfusion policy is not inferior to a double RBC transfusion policy for patients receiving a bone marrow transplant or intensive chemotherapy in a hematological intensive care unit. However, the single RBC transfusion policy did not reduce the number of RBC units transfused per stay. FUNDING: This trial was funded by a grant from the French Ministry of Health.

Efficacy of microsurgical vascular decompression in trigeminal neuralgia.

OBJECTIVES: Analysis of the long-term efficacy of microvascular decompression surgery in trigeminal neuralgia. MATERIAL AND METHODS: A single-center retrospective study included patients undergoing microvascular decompression surgery for trigeminal neuralgia after failure of well-conducted medical or complementary therapy, with visualization of nerve compression syndrome on MRI. RESULTS: Eighty-seven patients were included. Nerve compression was alleviated without interposition of polytetrafluoroethylene in 79.3% of cases. Postoperative efficacy on pain was immediate in 97.7% of cases. There were no postoperative deaths, and the rate of severe complications was low (2.3%). The efficacy of microvascular decompression surgery was total at 2 years in 90.8% of cases and at 10 years in 92.3%, without resumption of medical treatment. The failure rate was 10.3%; 26.3% of these patients had been previously treated by a lesional technique (P: 0.043) and 33.3% by interposition of polytetrafluoroethylene (P: 0.003). CONCLUSIONS: With confirmed clinical and radiological diagnosis, microvascular decompression surgery for trigeminal nerve compression was safe, with total effectiveness in the immediate, short and long terms. It should be considered in first line in case of failure or intolerance of well-conducted medical treatment.

Drilling and control of the internal auditory canal by fixed endoscope.

Preservation of vestibular structures, particularly the posterior semicircular canal, is essential to ensure hearing preservation in addition to complete tumour resection during retrosigmoid surgical resection of a vestibular schwannoma. Drilling of the internal auditory canal (IAC) is a delicate step, during which these structures can be accidentally perforated. The orientation of the IAC results in the formation of poorly visible zones that can predispose to perforation of these structures when drilling is performed with a microscope. Hand-held endoscopy exposes all of the operative field, but immobilizes one of the surgeon's hands, making this surgery even more delicate. Fixed endoscopy is a solution that gives the surgeon greater freedom of movement, while ensuring precise control of the surgical procedure. It allows identification and avoidance of vestibular structures, while allowing resection as close as possible to the tumour. The schwannoma can be entirely cleaved when the fundus of the IAC is correctly controlled, while sparing the facial and cochlear nerves.

High-speed Si/GeSi hetero-structure Electro Absorption Modulator.

The ever-increasing demand for integrated, low power interconnect systems is pushing the bandwidth density of CMOS photonic devices. Taking advantage of the strong Franz-Keldysh effect in the C and L communication bands, electro-absorption modulators in Ge and GeSi are setting a new standard in terms of device footprint and power consumption for next generation photonics interconnect arrays. In this paper, we present a compact, low power electro-absorption modulator (EAM) Si/GeSi hetero-structure based on an 800 nm SOI overlayer with a modulation bandwidth of 56 GHz. The device design and fabrication tolerant process are presented, followed by the measurement analysis. Eye diagram measurements show a dynamic ER of 5.2 dB at a data rate of 56 Gb/s at 1566 nm, and calculated modulator power is 44 fJ/bit.

[Is the research of posttransfusional alloantibodies still relevant?] / Est-ce que la réalisation de la recherche d'anticorps antiérythrocytaires post­transfusionnelle est toujours pertinente ?

The decision of November 6th, 2006 defining the principles of best practices recommends that posttransfusional red cell alloantibodies research is performed after one to three months after. In the University hospital of Brest, the haemovigilance unit takes charge of sending the medical prescription within the required time and centralizing the results. We wished to estimate if the realization of this research still remains relevant. METHODS: A prospective analysis was performed in 2015. We evaluated the realization rate, the red cell alloantibodies rate and the recipient adverse reactions with the diagnostic category: alloimmunization (delayed serological transfusion reaction, DSTR). RESULTS: In 2015, 2162 prescriptions were sent to the 3271 transfused patients. One thousand and eighteen red cell alloantibodies research were done, i.e. a return rate of 61%. Among them, 12 alloantibodies appeared (0.9%) within an average of 56 days. Thirty-three other alloantibodies appeared and were discovered most frequently before a new transfusion. In 10 cases, a posttransfusional research was done that was negative. A survey was conducted among GHCOH members to describe the practices in these health institutions. Twelve questionnaires were analysed. Ten institutions performed a posttransfusional alloantibodies research by issuing a prescription at the patient's exit with a return rate between 0.14 and 16%; 1 institution has a centralized organization with a return rate of 68.3%; 1566 red cell alloantibodies research were performed and among them, 24 alloantibodies appeared (1.53%). CONCLUSION: These results indicate that to be effective, the management of this biological test must be centralized. Despite this, the red cell alloantibodies rate remains very low (0.9 and 1.53%) and raises the question of the relevance of this systematic testing after transfusion, which is in any case mandatory before a new transfusion of red blood cells.

[The tumors of the ear]. / Les tumeurs de l'oreille.

Any cutaneous lesion of the outer ear must be managed jointly by a dermatologist and an ENT, regardless of the age of the patient. The presence of a malignant cutaneous carcinoma (Squamous cell carcinoma or melanoma) of the pavilion requires a minimum extension assessment by a cervical ultrasound, CT-scan and MRI will be prescribed according to the degree of infiltration and the presence of clinics signs (lymphadenopathy, facial paralysis, cognitive impairment). A polyp of the external auditory meatus must be systematically biopsied in consultation and, if necessary, in the operating room with fresh anatomopathological analysis. Any "otitis externa", which does not progress favorably under local treatment, must lead to eliminate a tumoral pathology of the external acoustic meatus or of the middle ear. Any suspicion of cholesteatoma should lead to an ENT consultation to confirm the diagnosis and consider its treatment to limit the auditory dysfunction. Any unilateral neurosensorial hearing loss or unilateral vestibular involvement with normal otoscopy should lead to eliminate a inner ear tumor by an MRI of the inner ear and the ponto-cerebellar angle in millimeter sections.

Ultra-compact III‒V-on-Si photonic crystal memory for flip-flop operation at 5 Gb/s.

We report on a photonic crystal (PhC) nanolaser based on the heterogeneous integration of a III-V PhC nanocavity on SOI, configured to operate as a Set-Reset Flip-Flop (SR-FF). The active layer is a nanobeam cavity made of a 650 nm × 285 nm InP-based wire waveguide evanescently coupled to 500 nm × 220 nm SOI wire waveguides, demonstrating a record-low footprint of only 6.2 µm2. Injection locking enables optical bistability allowing for memory operation with only 6.4 fJ/bit switching energies and <50 ps response times. Bit-level SR-FF memory operation was evaluated at 5 Gb/s with PRBS-resembling data patterns, revealing error free operation with a negative power penalty.

A series of 38 novel germline and somatic mutations of NIPBL in Cornelia de Lange syndrome.

Cornelia de Lange syndrome is a multisystemic developmental disorder mainly related to de novo heterozygous NIPBL mutation. Recently, NIPBL somatic mosaicism has been highlighted through buccal cell DNA study in some patients with a negative molecular analysis on leukocyte DNA. Here, we present a series of 38 patients with a Cornelia de Lange syndrome related to a heterozygous NIPBL mutation identified by Sanger sequencing. The diagnosis was based on the following criteria: (i) intrauterine growth retardation and postnatal short stature, (ii) feeding difficulties and/or gastro-oesophageal reflux, (iii) microcephaly, (iv) intellectual disability, and (v) characteristic facial features. We identified 37 novel NIPBL mutations including 34 in leukocytes and 3 in buccal cells only. All mutations shown to have arisen de novo when parent blood samples were available. The present series confirms the difficulty in predicting the phenotype according to the NIPBL mutation. Until now, somatic mosaicism has been observed for 20 cases which do not seem to be consistently associated with a milder phenotype. Besides, several reports support a postzygotic event for those cases. Considering these elements, we recommend a first-line buccal cell DNA analysis in order to improve gene testing sensitivity in Cornelia de Lange syndrome and genetic counselling.

Modulation contrast optimization for wavelength conversion of a 20 Gbit/s data signal in hybrid InP/SOI photonic crystal nanocavity.

Photonic crystal cavity-based switching is studied both theoretically and experimentally in order to identify the best configuration to maximize "wavelength conversion" efficiency. In particular, it is shown that an enhanced contrast can be reached when the probe is blueshifted with respect to the resonance. The use of an InP/SOI hybrid photonic crystal nanocavity is reported for the first time for all-optical error-free "wavelength conversion" at 20 Gbit/s with a nonreturn to zero on-off keying signal.

A French collaborative survey of 272 fetuses with 22q11.2 deletion: ultrasound findings, fetal autopsies and pregnancy outcomes.

OBJECTIVE: The 22q11.2 deletion (del22q11.2) is one of the most common microdeletions. We performed a collaborative, retrospective analysis in France of prenatal diagnoses and outcomes of fetuses carrying the del22q11.2. METHODS: A total of 272 fetuses were included. Data on prenatal diagnosis, ultrasound findings, pathological features, outcomes and inheritance were analyzed. RESULTS: The mean time of prenatal diagnosis was 25.6 ± 6 weeks of gestation. Most of the diagnoses (86.8%) were prompted by abnormal ultrasound findings [heart defects (HDs), in 83.8% of cases]. On fetal autopsy, HDs were again the most common disease feature, but thymus, kidney abnormalities and facial dysmorphism were also described. The deletion was inherited in 27% of cases. Termination of pregnancy (TOP) occurred in 68.9% of cases and did not appear to depend on the inheritance status. However, early diagnosis was associated with a higher TOP rate. CONCLUSION: This is the largest cohort of prenatal del22q11.2 diagnoses. As in postnatally diagnosed cases, HDs were the most frequently observed abnormalities. However, thymus and kidney abnormalities and polyhydramnios should also be screened for in the prenatal diagnosis of del22q11.2. Only the time of diagnosis appeared to be strongly associated with the pregnancy outcome: the earlier the diagnosis, the higher the TOP rate.

[Facial nerve outcome after vestibular schwannoma surgery: About 89 patients]. / Fonction du nerf facial après chirurgie du schwannome vestibulaire: àpropos de 89 patients.

OBJECTIVES: We evaluated the postoperative facial nerve function after vestibular schwannoma (VS) surgery and analyzed factors causing it. MATERIAL AND METHODS: We included 89 consecutive patients undergoing surgical excision of unilateral VS. Patient and tumor characteristics, surgical approaches, facial nerve function, extent of tumor resection and complications were analyzed. RESULTS: Complete tumor resection was achieved in 85% of cases. Anatomic preservation of the facial nerve was achieved in 96% of patients. In all tumor stages, 88.2% of patients, have a normal or subnormal facial function within one year of the intervention, the rate was 100% in patients carrying a VS stage I or II. No severe complication or death was reported. CONCLUSION: Short- and long-term facial nerve outcome was comparable with results of other recent series reported in literature. The facial nerve function after surgery was better with small VS compared to large VS. The intraoperative decision of near-total excision of the tumor followed by adjuvant radiotherapy, in some cases, can prove to be a good option in the interest of better preservation of the facial nerve function. The adhesion of the facial nerve and its complex relationship with the tumor remain mysterious; RMI can bring significant refinements, helping to get the best preservation offacial function rate, in the coming years.

Molecular characterization of 39 de novo sSMC: contribution to prognosis and genetic counselling, a prospective study.

Small supernumerary marker chromosomes (sSMCs) are structurally abnormal chromosomes that cannot be characterized by karyotype. In many prenatal cases of de novo sSMC, the outcome of pregnancy is difficult to predict because the euchromatin content is unclear. This study aimed to determine the presence or absence of euchromatin material of 39 de novo prenatally ascertained sSMC by array-comparative genomic hybridization (array-CGH) or single nucleotide polymorphism (SNP) array. Cases were prospectively ascertained from the study of 65,000 prenatal samples [0.060%; 95% confidence interval (CI), 0.042-0.082]. Array-CGH showed that 22 markers were derived from non-acrocentric markers (56.4%) and 7 from acrocentic markers (18%). The 10 additional cases remained unidentified (25.6%), but 7 of 10 could be further identified using fluorescence in situ hybridization; 69% of de novo sSMC contained euchromatin material, 95.4% of which for non-acrocentric markers. Some sSMC containing euchromatin had a normal phenotype (31% for non-acrocentric and 75% for acrocentric markers). Statistical differences between normal and abnormal phenotypes were shown for the size of the euchromatin material (more or less than 1 Mb, p = 0.0006) and number of genes (more or less than 10, p = 0.0009). This study is the largest to date and shows the utility of array-CGH or SNP array in the detection and characterization of de novo sSMC in a prenatal context.

Uniformity of the lasing wavelength of heterogeneously integrated InP microdisk lasers on SOI.

We report a high lasing wavelength uniformity of optically pumped InP-based microdisk lasers processed with electron-beam lithography, heterogeneously integrated with adhesive bonding on silicon-on-insulator (SOI) waveguide circuits and evanescently coupled to an underlying waveguide. We study the continuous wave laser emission coupling out of the SOI via a grating coupler etched at one side of the waveguide, and demonstrate a standard deviation in lasing wavelength of nominally identical devices on the same chip lower than 500 pm. The deviation in the diameter of the microdisks as low as a few nanometers makes all-optical signal processing applications requiring cascadability possible.

Maternal transmission of interstitial 8p23.1 deletion detected during prenatal diagnosis.

We report on the first prenatally diagnosed interstitial 8p23.1 maternally inherited deletion. At 20 weeks of gestation (WG) the fetus was diagnosed with a complete atrioventricular canal. In infancy, the mother underwent a two-step cardiac surgery for an interrupted aortic arch type A associated to an inlet ventricular septal defect (VSD). A straddling of the tricuspid valve type B was confirmed during surgery. The outcome showed no cardiac failure or conduction anomalies. However, she presented with moderate intellectual disability. Classical and molecular cytogenetic studies on amniotic and maternal lymphocytes cells showed a nearly identical interstitial deletion of the 8p23.1 region encompassing the GATA4 gene locus (Mother: nt 6,913,337-12,580,828, fetus: nt 7,074,449-12,580,828) with no modification of the telomeric region. The relevance of our report is not only the maternal syndromic interstitial 8p23.1 deletion, but also maternal transmission which has never been reported before. The maternal and fetal phenotypes were not identical, however, even though they had the same cellular and molecular background: an alteration of the epithelial mesenchymal transition of the atrioventricular valvulo-septal complex where GATA4 plays a positive role in the regulation. We reviewed all cases of interstitial 8p23.1 deletions diagnosed either prenatally or postnatally.

High quality beaming and efficient free-space coupling in L3 photonic crystal active nanocavities.

We report on far-field measurements of L3 photonic crystal (PhC) cavities with high quality beaming. This is achieved by means of the so-called "band folding" technique, in which a modulation of the radius of specific holes surrounding the cavity is introduced. Far-field patterns are measured from photoluminescence of quantum wells embedded in the PhC. A very good agreement between experimental results and simulated radiation patterns has been found. Laser effect is demonstrated in the beaming cavity with a threshold comparable to the regular one. In addition, free-space input coupling to this cavity has been achieved. In order to fully analyze the coupling efficiency, we generalize the approach developed in S. Fan, et al., [J. Opt. Soc. Am. A 20, 569 (2003)], relaxing the hypothesis of mirror symmetry. The obtained coupling efficiencies are about 15% with quality factors (Q) exceeding 10(4). These results further validate the "folding" technique on L3 cavities for nanocavity realization with efficient free-space coupling and high Q factors.

[Hospital haemovigilance and adverse events or reactions: who notifies and what?]. / Gestion des évènements indésirables receveurs: le point de vue de l'hémovigilant d'un établissement de santé.

The French haemovigilance system designates a physician in charge of haemovigilance in each hospital. She/he collects the adverse reactions and events reported by medical staff or nurses. In 2010, 7360 (2.42/1000 units) adverse reactions and 518 adverse events (excepted donors) were reported. These results mean that the system is particularly effective in hospitals. This study collected the opinion of physicians in charge of haemovigilance about this organization.

[Factsheets: support in the analysis of recipients' adverse reactions]. / Fiches techniques: aide à l'analyse des effets indésirables receveurs.

In order to help the analysis of adverse effects of transfusion, factsheets have been written by working groups of the French agency for the safety of health products ANSM. Each factsheet deals with a blood transfusion side effect and is composed of five parts, including pathophysiological mechanisms, diagnostic criteria, management recommendations, etiologic investigations and rules for filing the notification form to ANSM. Since 2006, 11 factsheets have been published on the French haemovigilance network website. The major characteristics of the two last sheets published "post-transfusion purpura" and "non erythrocyte incompatibility reaction" are presented, followed by the updated card for "allergy". These factsheets give relevant guidelines allowing better evaluation of recipients' adverse reactions, particularly their diagnosis, severity and accountability. They also could initiate studies among European and international haemovigilance networks.

Occupational hepatitis C seroconversions in a Brazilian hospital.

BACKGROUND: There are six known cases of occupational human immunodeficiency virus (HIV) seroconversion in Brazil. However, there are neither published cases of occupational hepatitis C virus (HCV) seroconversion nor systematic studies of blood and body fluid exposures (BBFE) that could estimate the risk of HCV or HIV occupational seroconversion in Brazil. AIMS: To describe the outcomes of BBFEs in a Brazilian hospital over 12 years and 2 months. METHODS: Statistical analysis of a computerized database of exposure events recorded on printed forms. Incidence rates (IR) were calculated as the number of BBFE per 100 full-time equivalent worker-years. RESULTS: There were 1457 BBFE, 87% being percutaneous and 561 (38%) recurring in health care workers (HCWs) who reported having previous exposures. The highest IRs occurred in laboratory technicians (9.7), medical students (9.5), cleaning staff (9.5) and nursing aids (9.2). The IR in temporary employees was 13.0. Two HCWs, a nursing aid and a surgeon, seroconverted to hepatitis C after HCV exposures involving 13 G catheter needles. The risk of acquiring a HCV infection was 2 in 38 percutaneous HCV exposures, i.e. 5% (95% CI: 0.89-16.3). There were no seroconversions to HIV despite 80 percutaneous HIV exposures. CONCLUSIONS: HCV has a higher potential for occupational transmission than HIV. Measures to reduce the risks of BBFE and occupational transmission of blood-borne viral infections should be improved in Brazil.