Remodelling of the superior caval vein after angioplasty in an infant with superior caval vein syndrome

An 8-month old girl was presented with superior caval vein syndrome early after cardiac surgery. Angiography showed severe stenosis of the superior caval vein with 50 mmHg pressure gradient. Following balloon angioplasty, the pressure gradient was reduced to 7 mmHg with some residual stenosis of the superior caval vein. When the patient was reevaluated 5 months after the procedure, angiography revealed a normal diameter of the superior caval vein without a pressure gradient

Subacute sclerosing panencephalitis and immune thrombocytopenia: More than a coincidence?

Subacute sclerosing panencephalitis (SSPE) is a progressive disease caused by persistent measles virus (MV). It has an incidence of 0.4-2.0/million in Turkey. Immune thrombocytopenia (ITP) is a bleeding disorder whose estimated incidence is 4.2/100.000 person/years in the pediatric age group. We observed three cases with ITP in our cohort of 315 pediatric SSPE cases, an incidence higher than coincidentally expected in the general population. We hypothesize an association between SSPE and ITP. Our three cases had measles 1-2 years before the onset of ITP and 8-10 years before first symptoms of SSPE. A common immunogenetic background creating susceptibility to infection and autoimmunity might play a role. Alternatively, chronic antigenic stimulation by the MV leading to synthesis of cross-reacting antibodies against platelets, or treatment of ITP with immunoglobulins or steroids might affect or alter the development and manifestation of SSPE. The co-occurrence of these two disorders of viral and immune pathogenesis may draw attention to similar observations and provide clues for their mechanisms

The megacystis-microcolon-intestinal hypoperistalsis syndrome - Report of a case and review of the literature

The megacystis-microcolon-intestinal hypoperistalsis syndrome is part of a spectrum of intestinal motility disorders and is characterized by abdominal distension, lax abdominal musculature, incomplete intestinal rotation, microcolon, megacystis, bilious vomiting and decreased or absent intestinal peristalsis. In this report a newborn girl with megacystis-microcolon-intestinal hypoperistalsis syndrome is reported

Arterial switch operations for single coronary artery ostium or intramural coronary artery

Background Successful transfer of the coronary arteries is the most critical step during arterial switch operations for patients with transposition of the great arteries with a single coronary artery ostium and/or intramural coronary arteries. Various techniques have been reported and the present study was an evaluation of them in 10 neonatal patients

Retrospective analysis of children with myelin oligodendrocyte glycoprotein antibody-related disorders

PubMedID: 30212767Background: Knowledge has been expanding on myelin oligodendrocyte glycoprotein (MOG) antibody-associated central nervous system disorders. We delineate the clinical and paraclinical findings and outcome of our pediatric patients with MOG antibody seropositive disease. Methods: We retrospectively analyzed the clinical presentation, cerebrospinal fluid findings, magnetic resonance imaging (MRI) studies, course and outcome of children seropositive for anti-MOG IgG. Results: Total 20 children with neurological symptoms and serum anti-MOG IgG were identified from six centers in Turkey. Median age at onset was 9 years (mean 8.8 ± 5.0 years, range: 1.5–16.5 years). Final diagnoses were acute disseminated encephalomyelitis (ADEM) (n = 5), ADEM + optic neuritis (n = 4), neuromyelitis optica spectrum disorder (NMOSD) (n = 3), myelitis (n = 2), relapsing optic neuritis (n = 2), multiphasic DEM (n = 3), and unclassified relapsing demyelinating disease (n = 1). Seven/20 (35%) children experienced a single episode while 13/20 (65%) had a least one relapse during follow-up. On MRI, subcortical white matter, brainstem, and corpus callosum were preferentially involved regions. Full recovery was observed in 15/20 (75%) children. Conclusion: MOG autoimmunity in children has a wide clinical spectrum, tendency to relapse, and a favourable outcome compared with other relapsing demyelinating diseases. © 201

Polyethylenimine (PEI) is a simple, inexpensive and effective reagent for condensing and linking plasmid DNA to adenovirus for gene delivery

A simple and inexpensive method of condensing and linking plasmid DNA to carrier adenovirus particles is described. The synthetic polycation polyethylenimine is used to condense plasmid DNA into positively charged 100 nm complexes. These PEI–DNA complexes are then bound to adenovirus particles through charge interactions with negative domains on the viral hexon. The resulting transfection complexes deliver plasmid DNA to cells by the adenovirus infectious route without interference from virus gene expression because psoralen-inactivated virus is employed. The PEI–DNA–adenovirus complexes display DNA delivery comparable to more sophisticated DNA virus complexes employing streptavidin/biotin linkage, but require no special reagents and are much easier to prepare