[Anti-meningococcal vaccines: diversity of vaccination policies and recommendations].

In European Country, Canada, Australia and Brazil immunization program with conjugate meningococcal C, including universal vaccination of infants or toddlers, with a catch-up program up to 19 y in several areas, have been successful in reducing disease incidence through direct and indirect protection. In USA, quadrivalent conjugate vaccines targeting serogroups ACYW135 are used in programs of adolescent immunization at 10 and 15 years because serotype Y is frequent. A mass immunization campaign against serogroupe A disease with a conjugate vaccine is beginning in African belt of meningitis.

[A medical-pharmaceutical partnership model as a contributor to the success in conditioning regimen for allogenic hematopoietic stem cell transplantation in adults: a cross-reflection on our organizations].

Allogeneic hematopoietic stem-cell transplant (allo-SCT) remains the only cure for many hematological malignancies and some benign and congenital diseases. Busulfan, proposed in its injectable form, has quickly become a mainstay of pharmacological and myeloablative (or non-myeloablative) conditioning. This is following the outbreak in 2010 of a multicenter international clinical phase II trial, we tested the robustness and reliability of our organization in a complex model of organization and multifactorial partnership.

[Transposition of the great arteries: a curable congenital heart defect?].

Transposition of the great arteries (TGA) is a common congenital heart malformation, involving the inversion of both great vessels (aorta and pulmonary artery). It is not compatible with life in the absence of surgical treatment. The prognosis of this malformation has been transformed by the development of neonatal cardiac surgery.

[Anatomic correction of transposition of the great arteries: a model of surgical "cure" in pediatric cardiology?].

Transposition of the great arteries (TGA) is a common congenital heart malformation, involving inversion of the two great vessels (aorta and pulmonary artery). Without surgical treatment, TGA is not compatible with life. The prognosis of this malformation has been dramatically changed by the development of neonatal cardiac surgery.

[New developments in ultrasound imaging for chronic liver diseases: from anatomic imaging to structural and functional imaging].

Conventional US imaging is playing a key role for the diagnosis and the therapeutic management of chronic liver diseases. Nevertheless, conventional US imaging is facing many limitations: operator-dependency, subjective assessment, variable detectability of nodules depending on accessibility to the US beam and spontaneous contrast to surrounding normal parenchyma, limited characterization capabilities. Conventional US imaging is taking advantage of major technological improvements including contrast-enhanced US (CEUS), elastography and volume and fusion imaging techniques.

Contrast enhanced ultrasound in the detection of liver metastases: a prospective multi-centre dose testing study using a perfluorobutane microbubble contrast agent (NC100100).

Objective: To conduct a dose testing analysis of perfluorobutane microbubble (NC100100) contrast-enhanced ultrasound (CEUS) to determine the optimal dose for detection of liver metastases in patients with extra-hepatic primary malignancy.

Methods: 157 patients were investigated with conventional US and CEUS. CEUS was performed following intravenous administration of perfluorobutane microbubbles (using one dose of either 0.

First experience of autologous peripheral blood stem cell mobilization with biosimilar granulocyte colony-stimulating factor.

Introduction: Mobilization techniques for autologous peripheral blood stem cell (PBSC) collection include chemotherapy followed by hematopoietic growth factors, such as granulocyte colony-stimulating factor (G-CSF). Biosimilar versions of G-CSF are now available in Europe.

Methods: In this study, 40 patients with a hematological malignancy scheduled to receive biosimilar G-CSF (Zarzio(®) Sandoz Biopharmaceuticals, Paris, France) following first-cycle chemotherapy for treatment and autologous PBSC mobilization were prospectively included at a single center.

Scalp nodules as a presenting sign of fibrodysplasia ossificans progressiva: a register-based study.

Background: Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive ossification of soft tissues. Clinical diagnosis is important because trauma from lesional biopsies can exacerbate the disease.

Objective: We sought to evaluate the frequency of scalp nodules as the presenting manifestation of FOP.

The French national registry of primary immunodeficiency diseases.

The French National Reference Center of Primary Immunodeficiencies (CEREDIH) was established in 2005 and now constitutes a nationwide network of pediatric and adult medicine departments in university medical centers. The registry comprises a total of 3,083 patients (mainly children), with an overall prevalence of 4.4 cases per 100,000 inhabitants.

[Anidulafungin: a new therapeutic option in systemic candidiasis].

The increasing incidence of invasive fungal infections, epidemiological changes in these infections, and safety or interactions issues that might limit the use of traditional antifungal therapies explain the need for new antifungal agents. Anidulafungin is a new echinocandin with excellent activity on most Candida as well as on Aspergillus species. Its pharmacokinetic properties allow its administration without dosage adjustments in patients with hepatic and renal impairment and account for the absence of drug interactions.

[Allergic and non-allergic hypersensitivity reactions to toxoid-containing vaccines].

Most allergic (like) reactions to vaccines are reported in patients immunized with diphtheria and tetanus toxoid-containing vaccines. Local inflammatory reactions are the most frequent, but most of them are non-specific. Diagnosis of Arthus-type reactions is based on clinical history and specific IgM/IgG anti-toxoid determination.

[Transition from childhood to adulthood and management of spasticity].

The authors report specific concerns on the transition to adulthood in cerebral palsy patients, with particular attention devoted to spasticity treatment follow-up.

[Transition from childhood to adulthood and lumbosacral dysraphism].

The diagnosis of lumbosacral dysraphism is now made during the first year of life. Although problems due to filum lipomas are solved when the patient becomes an adult, the lipomas of the lumbar spinal cord can induce late complications. It is therefore necessary to organize a neurological, urological, and obstetrical follow-up to: screen for late retethering and/or deterioration ; preserve kidney function and monitor the consequences of the different procedures performed during childhood ; support pregnancies (delivery, prenatal screening for Currarino syndromes).

[Passage from childhood to adulthood and craniovertebral malformations].

Craniovertebral malformations are infrequent in children. Their causes are numerous (Chiari, congenital bone diseases, metabolic diseases, and genetic anomalies). When symptomatic (seldom), these malformations require surgical decompression and fixation.

[Characteristics of encrustation of ureteric stents in patients with urinary stones].

Introduction: The goal of this prospective study was to characterize ureteral stents encrustation in stone formers.

Material And Methods: We report the results of a study based on 658 double-J stents (412 men and 246 women) collected from patients with in situ urinary calculi. The mean age was 48.

Primary cutaneous Epstein-Barr virus-related lymphoproliferative disorders in 4 immunosuppressed children.

Primary cutaneous Epstein-Barr virus-related lymphoproliferative disorders are rare. We describe 4 cases in children: two with acquired immunodeficiencies (HIV infection, heart transplantation) and two with congenital immunodeficiencies (ataxia-telangiectasia and an undetermined disease affecting the T lymphocytes). Two of the lymphoproliferative disorders were T-cell types and two were B-cell types.

[Anhidrotic ectodermal dysplasia: "congenital ameibomia"].

Anhidrotic ectodermal dysplasia is a congenital, generally X-linked dermatosis that associates facial dysmorphy, short stature, and severe blepharitis. The anomalies of the skin are epidermic abnormalities; reduction of the glands of the derm, particularly the sweat glands, explaining the hypohidrosis; onychodysplasia; trichodysplasia; and abnormal dentition. The ophthalmologic manifestations are palpebral anomalies with a reduction in or an absence of Meibomian glands, dysfunction of the Moll and Zeis glands, leading to chronic squamous blepharitis and lacrimal punctal atresia.

Experimental ischemic cardiomyopathy: insights into remodeling, physiological adaptation, and humoral response.

Ligation of the left anterior descending coronary artery (LAD) is used to induce experimental myocardial infarction (MI). Most previous studies have focused on the early postoperative period, while data on mid-term follow-up are scanty. This study examined the mid-term effects of LAD ligation in 95 MI rats and 28 controls.

Prenatal diagnosis of some metabolic diseases using early amniotic fluid samples: report of a 15 years, experience.

Background: In the present study, we report the results of 132 prenatal diagnoses performed on chorionic villi and cell-free amniotic fluid obtained simultaneously at 12-13 weeks of gestation. In addition, we report the result of 59 prenatal diagnoses performed at 12-13th week using amniotic fluid only.

Methods And Results: A total of one fetal loss (1/191) was observed when a sample of amniotic fluid was obtained at around 12-13 weeks, whereas three losses (3/82) were observed after midtrimester amniocentesis.

Orthotopic heart transplantation for congenital heart defects: situs inversus.

Although rare, visceroatrial situs inversus is not exceptional in patients with complex congenital heart defects. Achieving orthotopic heart transplantation using a graft harvested in a donor with situs solitus is surgically demanding. Technical problems can, however, be overcome by adequate harvesting of donor heart and by the use of innovative reimplantation techniques.

Orthotopic heart transplantation for congenital heart defects: anomalies of the systemic venous return.

Anomalies of the systemic venous return are frequently associated with other congenital heart defects. Some anomalies do not complicate really orthotopic heart transplantation (such as azygos continuation of the inferior vena cava). Other anomalies raise more difficulties; the most frequent one is persistent left superior vena cava draining into either the coronary sinus or the left atrium.

Social and psycho-intellectual outcome following radical removal of craniopharyngiomas in childhood. A prospective series.

Background: A prospective study on childhood craniopharyngiomas (CPs) was conducted from 1994 to 1998 to appreciate the pre- and postoperative clinical, endocrine, mental, and intellectual status of the patients and to determine the incidence and severity of the postoperative hypothalamic syndrome.

Methods: The series included 14 consecutive CPs. Twelve were retrochiasmatic and intraventricular, and two were partly prechiasmatic and extraventricular.