Congenital Portosystemic Shunts: Current Diagnosis and Management.

Congenital portosystemic shunts are increasingly recognized in several settings and at any age. The following are some of the most common presentations: prenatal ultrasound, neonatal cholestasis, incidental finding on abdominal imaging, or systemic complications such as unexplained cardiopulmonary or neurological disease, or the presence of liver nodules in a noncirrhotic liver. The purpose of the present review is to summarize clinical presentation and current recommendations for management, and highlight areas of future research.

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management.

Paediatric pulmonary arterial hypertension (PAH) shares common features of adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for the care of children with PAH, as presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary Hypertension. We provide updates of the current definition, epidemiology, classification, diagnostics and treatment of paediatric PAH, and identify critical knowledge gaps.

Optimizing seroprotection against pneumococcus in children with nephrotic syndrome using the 13-valent pneumococcal conjugate vaccine.

Background: Infections are among the main life-threatening complications in patients with nephrotic syndrome (NS), in particular with Streptococcus pneumoniae, the first cause of bacterial peritonitis and sepsis in these patients. This study aims to evaluate the baseline seroprotection of NS patients against S. pneumoniae, and immunize them with the 13-valent pneumococcal conjugate vaccine (PCV13) regardless of disease activity and previous immunization history, in order to evaluate the immunogenicity, safety profile, and effect of NS treatment on vaccine responses.

Current practices in long-term video-EEG monitoring services: A survey among partners of the E-PILEPSY pilot network of reference for refractory epilepsy and epilepsy surgery.

Purpose: The European Union-funded E-PILEPSY network aims to improve awareness of, and accessibility to, epilepsy surgery across Europe. In this study we assessed current clinical practices in epilepsy monitoring units (EMUs) in the participating centers.

Method: A 60-item web-based survey was distributed to 25 centers (27 EMUs) of the E-PILEPSY network across 22 European countries.

Clinical uses of anti-Müllerian hormone assays: pitfalls and promises.

Objective: To investigate whether the controversy about fluctuations of anti-Müllerian hormone (AMH) levels during the menstrual cycle results from differences between the immunoassays currently available: the Beckman Coulter Immunotech kit (Fullerton, CA) and the Diagnostic Systems Laboratories kit (Webster, TX).

Design: Prospective trial.

Setting: Fertility clinics of two tertiary university hospitals.

Outlook: Roles of FSH and LH during the follicular phase: insight into natural cycle IVF.

A mounting interest in natural cycle IVF has challenged the medical community to better understand the mechanisms controlling the follicular phase and ovulation in particular, in an effort to optimize this procedure and its outcome. For practical reasons, the advancement of the follicular phase in the menstrual cycle is commonly timed according to the onset of last menses. However, this precludes knowing when the follicular phase truly begins and hampers the possibility of optimizing timing of late follicular-phase events, notably, the triggering of ovulation.

Serum antimüllerian hormone levels remain stable throughout the menstrual cycle and after oral or vaginal administration of synthetic sex steroids.

Objective: To investigate whether oral or vaginal administration of contraceptive hormones might affect antimüllerian hormone (AMH) levels.

Design: Prospective trial with women recruited by advertisement. Women who wished contraception were randomized between oral or vaginal estroprogestative contraception, and those who did not choose contraception were included in the control group.

CRIT is expressed on podocytes in normal human kidney and upregulated in membranous nephropathy.

Complement C2 receptor inhibitor trispanning (CRIT) is a novel human complement regulatory cell surface receptor. It binds the human complement protein C2 and blocks the classical pathway of complement activation, thus protecting the cell against complement attack. CRIT expression in the kidney was analyzed by immunohistochemistry and in situ hybridization.