Hemoptysis from bronchial varices associated with pulmonary vein stenosis: Role of surgical repair

We present the case of a 6‐year‐old child who presented with an episode of life threatening hemoptysis. Investigations revealed multiple areas of endobronchial varices and abnormal pleural vessels as well as severe left pulmonary vein stenosis and an atrial septal defect (ASD). After extensive work up and consultation he underwent repair of the left pulmonary vein using a sutureless technique and ASD closure. This resulted in a marked improvement in the appearances of the left lung. The bronchial varices in the right lung remain unchanged. No further hemoptysis has occurred and the child continues to be monitored. Pediatr Pulmonol. 2013; 48:838–840. © 2012 Wiley Periodicals, Inc.     

Immunopathogenic mechanisms in tourette syndrome: A critical review

Tourette syndrome (TS) has a multifactorial etiology, in which genetic, environmental, immunological and hormonal factors interact to establish vulnerability. This review: (i) summarizes research exploring the exposure of TS patients to immune‐activating environmental factors, and (ii) focuses on recent findings supporting a role of the innate and adaptive immune systems in the pathogenesis of TS and related disorders. A higher exposure prior to disease onset to group A β‐haemolytic streptococcal (GABHS) infections in children with tics and obsessive‐compulsive (OC) symptoms has been documented, although their influence upon the course of disease remains uncertain. Increased activation of immune responses in TS is suggested by changes in gene expression profiles of peripheral immune cells, relative frequency of lymphocyte subpopulations, and synthesis of immune effector molecules. Increased activity of cell‐mediated mechanisms is suggested by the increased expression of genes controlling natural killer and cytotoxic T cells, increased plasma levels of some pro‐inflammatory cytokines which correlate with disease severity, and increased synthesis of antineuronal antibodies. Important methodological differences might account for some inconsistency among results of studies addressing autoantibodies in TS. Finally, a general predisposition to autoimmune responses in TS patients is indicated by the reduced frequency of regulatory T cells, which induce tolerance towards self‐antigens. Although the pathogenic role of immune activation in TS has not been definitively proven, a pathophysiological model is proposed to explain the possible effect of immunity upon dopamine transmission regulation and the generation of tics. © 2009 Movement Disorder Society     

Development of a selection and monitoring protocol for safe sedation of children

A protocol was developed for children undergoing diagnostic and therapeutic procedures under sedation to try to raise the standards of patient selection, preparation, monitoring and management to those of children undergoing general anaesthesia. The principles underlying the protocol design were that informed consent should be obtained; fasting guidelines as for general anaesthesia should be implemented; contraindications to sedation should be actively sought; monitoring should be commenced from the time of administration of sedation until recovery and should be maintained during patient transport; personnel trained in monitoring and resuscitation of children should have as their sole duty the monitoring of the sedated child and should not take part in the procedure; the minimum monitoring standard should comprise regular assessments of the level of sedation, oxygen saturation by pulse oximetry, respiratory rate and pulse rate supplemented by temperature, ECG and blood pressure for infants, for prolonged procedures or where verbal contact with the child is lost.     

Stent implantation to relieve native obstructed left partial anomalous pulmonary venous connections

Obstructed partial anomalous pulmonary venous connections (APVC) are rare but may be associated with severe pulmonary hypertension (PHTN) and warrant urgent relief. There are a number of case reports of successful catheter intervention for obstructed total APVC. We present the first reported case of catheter intervention to relieve obstructed, left sided PAPVC in a neonate with Turner syndrome. © 2016 Wiley Periodicals, Inc.     

Doubly committed subarterial ventricular septal defect: A risk factor for aortic distortion in patients considered for percutaneous pulmonary valve implantation?

Percutaneous pulmonary valve implantation has gradually become the first line strategy for re‐intervention for right ventricular outflow tract dysfunction during long‐term follow‐up after congenital cardiac surgery in many centers. We describe a case of a patient with double outlet right ventricle (Fallot's type) with a doubly committed subarterial ventricular septal defect, where the unique anatomy precluded percutaneous pulmonary valve implantation. © 2016 Wiley Periodicals, Inc.     

A rare case of cutaneous Trichosporon asahii infection in an immunocompromised child

We present an 11-year-old girl who developed neutropenic fever and papulonodular lesions over her limbs and back following chemotherapy for relapsed acute myeloid leukemia. She was found to have cutaneous Trichosporon asahii infection, with no evidence of systemic infection, and responded to treatment with voriconazole. T. asahii infection is rare but often fatal and usually causes disseminated infections in immunocompromised patients. Cutaneous infection is relatively uncommon and rarely occurs in the pediatric population.