Portopulmonary hypertension in children: a rare but potentially lethal and under-recognized disease.

Portopulmonary hypertension (PoPH) is defined by the combination of portal hypertension and precapillary pulmonary arterial hypertension (PAH). Very little is known about this process in pediatric patients but prognosis is generally poor. We review our institutional experience and report on five patients with pediatric PoPH.

Safety and Efficacy of Belimumab to Treat Systemic Lupus Erythematosus in Academic Clinical Practices.

Objective: To evaluate the use and efficacy of belimumab in academic practices. Belimumab is a human monoclonal antibody that inhibits soluble B lymphocyte stimulator and has been approved for the treatment of adults with systemic lupus erythematosus (SLE).

Methods: Invitations to participate and complete a 1-page questionnaire for each patient prescribed belimumab were sent to 16 physicians experienced in SLE phase III clinical trials.

Attitudes towards microbicide use for bacterial vaginosis in pregnancy.

Unlabelled: Background Bacterial vaginosis (BV) is the most common reproductive tract infection (RTI) and is a significant risk factor for preterm birth. Microbicides could be an option for the prevention and treatment of BV in pregnancy, and understanding use of the product will be crucial. The present study explored attitudes of women in the third trimester of pregnancy regarding topical microbicide use for the prevention and treatment of BV.

Triple outlet right ventricle: a previously unknown cardiac malformation.

We present the case of an infant with three distinct outflow tracts from the right ventricle. Three outlets from the heart have been previously named the "Tritruncal Heart". We review the two previously reported cases of tritruncal hearts and describe the anatomy, diagnosis, surgical management, and outcome of our case.

Cryoablation with an 8-mm tip catheter for pediatric atrioventricular nodal reentrant tachycardia is safe and efficacious with a low incidence of recurrence.

Background: Cryoablation with 4- and 6-mm tip ablation catheters has been demonstrated to be safe and effective in the treatment of atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients, albeit with a higher rate of clinical recurrence. Limited information is available regarding efficacy, mid-term outcomes, and complications related to the use of the 8-mm Freezor Max Cryoablation catheter (Medtronic, Minneapolis, MN, USA) in pediatric patients.

Methods: We performed a retrospective review of all pediatric patients with normal cardiac anatomy who underwent an ablation procedure for treatment of AVNRT using the 8-mm tip Cryoablation catheter at three large pediatric academic arrhythmia centers.

Transition from pediatric to adult health care for adolescents with congenital heart disease: a review of the literature and clinical implications.

As medical and surgical techniques advance in modern health care, children and adolescents with congenital heart disease (CHD) are living well into their adult years. Their complex cardiac anatomy, physiology, and medical histories present a challenge to adult health care providers who are not traditionally educated and trained in CHD. Growing evidence demonstrates that adolescents and young adults with CHD are at risk for extensive complications and sequelae from their heart condition and surgical treatments.

Characterization of natural killer and natural killer-like T cells derived from ex vivo expanded and activated cord blood mononuclear cells: implications for adoptive cellular immunotherapy.

Objective: Cord blood (CB) is limited by the absence of available donor effector cells for post-unrelated CB transplantation adoptive cellular immunotherapy. We reported the ability to ex vivo expand (EvE) CB mononuclear cells (MNC) after short-term incubation with anti-CD3, interleukin (IL)-2, IL-7, and IL-12 (antibody/cytokine [AB/CY]) into subpopulations of CD3(-)/56(+) natural killer (NK) cells with enhanced in vitro and in vivo tumor cytotoxicity.

Materials And Methods: We compared 2- vs 7-day EvE of rethawed CB MNCs in AB/CY and activation of NK and NK-like T (NKT) cell (CD3(+)/56(+)) subsets expressing specific NK-cell receptors along with IL-15, IL-18, and interferon-gamma production.

Variable morphology wide complex rhythm: what is the etiology?

We report a case of an infant who developed transient complete heart block following aortic and mitral valve surgery. In the course of his recovery, he developed a variable morphology wide complex rhythm with intermittent narrow complex beats. Review of his initial postoperative electrocardiogram (ECG), which demonstrated a right bundle branch block, helped to clarify the rhythm.

Update on the epidemiology and management of Staphylococcus aureus, including methicillin-resistant Staphylococcus aureus, in patients with cystic fibrosis.

Purpose Of Review: Staphylococcus aureus is one of the first and most common pathogens to be isolated from the respiratory tract of patients with cystic fibrosis. The prevalence of respiratory tract colonization/infection with both methicillin-susceptible and methicillin-resistant S. aureus has increased over the past decade.