Idiopathic pulmonary arterial hypertension in childhood.

The introduction of new medicines to treat pulmonary vascular disease has renewed interest in the management of children with idiopathic pulmonary arterial hypertension. We now have a practical classification of pulmonary hypertension, improved diagnostic techniques, and internationally recognized diagnostic and management guidelines. In 2009 the UK Pulmonary Hypertension Service for Children reported survival figures for idiopathic pulmonary arterial hypertension of 85.

Donor site reconstitution for ear reconstruction.

Background: Current techniques of autologous ear reconstruction involve the soft tissue coverage of a carved costal cartilage framework. However, assessment of the morbidity associated with this donor site has been little documented. This study describes a method to reconstruct the defect and analyses the outcomes with or without donor site reconstitution.

Vasculitis in children.

Primary systemic vasculitides of the young are relatively rare diseases, but can have a significant morbidity and mortality. The purpose of this review is to provide an overview of the paediatric vasculitides. Vasculitides that predominantly affect children will be considered in more detail than vasculitic diseases that although are seen in children affect adults more commonly, such as the ANCA associated vasculitides.

Advances in childhood vasculitis.

Purpose Of Review: To provide an update on new developments in paediatric vasculitis.

Recent Findings: New classification criteria for childhood vasculitis have recently been proposed and are currently undergoing validation. Infectious triggers are still implicated in the aetiopathogenesis of Kawasaki disease and Henoch-Schonlein purpura.

Sensory function in severe semilobar holoprosencephaly.

We report a 4-year-old child with severe semi-lobar holoprosencephaly (HPE) not expected to survive after birth. Magnetic resonance imaging (MRI) revealed agenesis of the corpus callosum, absence of the third ventricle, fused thalami and basal ganglia. To investigate sensory function, visual, auditory and somatosensory evoked potential and imaging studies were carried out.

Cidofovir: to use or not to use?

Purpose Of Review: Cidofovir is an antiviral agent that has been used increasingly in the last decade as an adjuvant therapy for recurrent respiratory papillomatosis. It has been used in patients with moderate to severe recurrent respiratory papillomatosis requiring frequent surgical intervention or if there is evidence of distal spread. Intralesional administration after surgical debulking delivers the medication directly to the site of disease and is thought to have fewer systemic side effects than intravenous infusion.

T cell activation profiles in Kawasaki syndrome.

Superantigens (SAgs) are potent stimulators of T cells bearing specific Vbeta T cell receptors (TCR) and may play a role in the pathogenesis of Kawasaki syndrome (KS), although despite 15 years of intense study this area remains controversial. Because SAgs can cause Vbeta restricted T cell activation in the absence of Vbeta skewing the aims of this study were to describe a flow cytometric protocol to study both CD4 and CD8 Vbeta repertoires, and CD69 expression across the CD4 and CD8 Vbeta repertoire in children with KS. Sixteen children with KS were studied.

Congenital corneal opacities - a surgical approach to nomenclature and classification.

The classification system of congenital corneal opacification (CCO) may be better considered from a perspective of pathogenesis, surgical intervention, and prognosis. The author feels that CCO is best considered as being primary and secondary. Primary CCO includes corneal dystrophies and choristomas presenting at birth.

Angiographic diagnosis of inflammatory bowel disease in patients presenting with gastrointestinal bleeding.

Objective: The objective of our study was to review the final diagnosis and outcome in patients undergoing visceral angiography for gastrointestinal hemorrhage in whom a diagnosis of inflammatory bowel disease (IBD) had been suspected on the basis of angiographic findings.

Materials And Methods: A retrospective review was performed of all visceral angiography referrals over a 10-year period. During that time, 522 visceral angiograms were obtained for the investigation of acute or chronic gastrointestinal bleeding.

From coagulation to enucleation: the use of lasers in surgery for benign prostatic hyperplasia.

The application of lasers for the treatment of benign prostatic hyperplasia has evolved over the past 15 years. Early-generation neodymium:yttrium aluminum garnet lasers were used to coagulate and ablate prostatic tissue, but significant postoperative irritative symptoms and high reoperation rates meant that this approach did not seriously challenge the status quo for long. Ablative techniques have recently become popular again with the marketing of the newer-generation, higher-power potassium titanyl phosphate and holmium lasers.

Advanced therapy for juvenile arthritis.

The management of juvenile idiopathic arthritis (JIA) has undergone dramatic changes in the last decade with undoubtedly great benefit for many patients. In particular, more effective use of available drugs and the application of newly discovered drugs have been responsible for much of this improvement. Methotrexate is the gold standard for management of moderate to severe polyarthritis.

Mobile thoracolumbar gibbus in Morquio type A: the cause of paraparesis and its management.

Four children with Morquio A syndrome had neurologic deterioration related to a progressive thoracolumbar gibbus. Findings on the magnetic resonance imaging scan did not suggest a significant canal compromise, but X-rays taken in the erect position showed a much larger kyphosis, and thus more canal compromise. All patients recovered neurologically after anterior decompression and correction of kyphosis.

Impact of low birth weight and cardiovascular risk factors on endothelial function in early adult life.

Background: Low birth weight is related to increased risk of coronary heart disease in adults and recently has been associated with vascular endothelial dysfunction in children. We investigated whether the relation between birth weight and endothelial function was still present in early adult life and whether there was an interaction with emerging risk factors.

Methods And Results: In 315 adults (165 women, 150 men, aged 20 to 28 years), high-resolution ultrasound was used to determine endothelium-dependent and -independent vascular responses of the brachial artery.

Autistic spectrum disorders in childhood epilepsy surgery candidates.

One third of children with autistic spectrum disorders (or pervasive developmental disorders) enter that state by regression from a more normal prior development at the onset of epilepsy or epileptiform abnormality in the electroencephalogram. In a very small proportion structural lesions of the temporal lobes are discovered. These form part of the sample of children coming to a surgical treatment programme.

Biodistribution, radiation dosimetry and pharmacokinetics of 111In-antimyosin in idiopathic inflammatory myopathies.

Unlabelled: In view of the established role of 111In-antimyosin in the detection of heart muscle pathology, radiation dose estimates were made for this substance. Biodistribution and biokinetic data were obtained from our studies, which failed to show abnormal uptake of 111In-antimyosin in localized sites of skeletal muscle involvement in patients with idiopathic inflammatory myopathies.

Methods: After intravenous administration of 74 MBq (2 mCi) 111In-antimyosin, gamma camera scintigraphy was performed in 12 adult patients with inflammatory muscle disease and in 2 control patients.

Three patients with the osteochondrodysplasia and hypertrichosis syndrome--Cantu syndrome.

Cantu syndrome is a rare condition whose main features are hypertrichosis, cardiac anomalies and wide ribs. Four children have been described and we now present details of a further three. The parents of one of these are first cousins, adding weight to Cantu's theory that the condition is an autosomal recessive disease.