Visualizing water clearance in the lung with MRI.

Current indirect measurements of alveolar fluid clearance (AFC) suggest that the rate of fluid clearance correlates with morbidity and mortality in patients with pulmonary edema. In a traditional AFC-measurement, fluid laced with a tracer macromolecule is instilled into the lung and thereafter repeated samples of the instilled fluid are extracted from the lung's fluid-filled airspaces. The change in concentration of the tracer molecule indicates the AFC-rate.

The human papillomavirus vaccine in Canada.

Clinical studies have shown the human papillomavirus (HPV) vaccines to be very effective at preventing persistent infection by vaccine serotypes. The development of these new vaccines heralds a new era in cancer prevention. Gardasil, Merck's quadravalent HPV vaccine, has recently been licensed in Canada for women aged 9 to 26 years of age.

Single pass albumin dialysis (SPAD) in fulminant Wilsonian liver failure: a case report.

Since fulminant Wilsonian liver failure has an extremely poor prognosis, the use of a liver support system that can bridge patients to liver transplant is life saving. We report here the case of a 17-year-old female who presented with fulminant Wilsonian liver failure and intravascular hemolysis. With the subsequent development of encephalopathy and oliguria, single pass albumin dialysis (SPAD) was initiated for 5 days to augment copper removal.

Finding the fertile woman with phenylketonuria.

This review highlights two groups of women with phenylketonuria (PKU) who are at risk of producing offspring with maternal phenylketonuria (MPKU) embryopathy: (I) those not yet diagnosed; (II) those lost to follow-up. The world literature is reviewed, including that published from the International MPKU Collaborative Study (MPKUCS) and evidence is presented to support our hypothesis that at least 10% of subjects with untreated "classical PKU" will have relatively normal intellectual function and that a significantly higher percentage of the less severe "variants" (who make up approximately 50% of the total) will have IQ's measured within the normal range. The offspring of the females with these PKU variants, however, are not as fortunate-most suffering profound damage in-utero if the pregnant woman is not treated.

Abnormal timing delays in auditory brainstem responses evoked by bilateral cochlear implant use in children.

Hypothesis: A period of unilateral implant use before bilateral implantation affects timing of brainstem processes measured by the electrically evoked auditory brainstem response (EABR).

Background: EABR latencies decrease with unilateral implant use potentially disrupting binaural timing cues important in auditory brainstem processing of bilateral input.

Methods: EABRs were evoked by electrical pulses from the left, right, and both implants simultaneously in 3 groups of children.

Cardiac chamber hypertrophy following hematopoietic stem cell transplantation for primary immunodeficiency.

Children with primary immune deficiency (PID) who receive hematopoietic stem cell transplantation (HSCT) often suffer from graft-versus-host disease (GVHD), which is commonly treated with corticosteroids (CS). CS may cause hypertension, development of cardiac chamber hypertrophy (CCH), and left ventricular outflow tract obstruction (LVOTO). We followed the development of CCH and LVOTO by serial echocardiograms in 10 children with PID before and 6 to 12 weeks after HSCT, and correlated their development with age of transplant, GVHD, use of CS and hypertension.

Improved results with single-stage total correction of Taussig-Bing anomaly.

Objective: The arterial-switch operation (ASO) for management of Taussig-Bing anomaly is associated with important morbidity, mainly related to multiple associated cardiac anomalies. Our surgical management has evolved to suggest a single-stage total repair strategy tailored to address all abnormalities on an individual basis. We examine the efficacy of this treatment approach.

Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection.

Background: We explore early results and time-related morbidity after surgical repair of partial anomalous pulmonary venous connection (PAPVC) at our institution.

Methods: Between 1982 and 2006, 306 consecutive patients underwent surgery for PAPVC; of these, 236 (77%) were children with a median age of 5.3 years (range, 0.

Competing outcomes after neonatal and infant wait-listing for heart transplantation.

Background: Neonatal and infant heart transplantation is a therapeutic option for patients with end-stage cardiac lesions, yet infants continue to face a considerable shortage of donor organs. We sought to ascertain outcomes after listing for heart transplantation using a competing outcomes methodology, and to identify factors predicting each outcome.

Methods: Review of the Toronto cardiac transplant database was undertaken to identify infant patients ( View Article and Find Full Text PDF

Matched unrelated bone marrow transplant for severe combined immunodeficiency.

Severe combined immunodeficiency (SCID) is a lethal disease unless allogeneic bone marrow transplantation (BMT), preferably from a family related HLA identical donor (RID) is given. Previously, some patients received HLA-mismatched related donors (MMRD) BMT, which often resulted in slow immune reconstitution and variable survival. Alternatively, HLA-matched unrelated donors (MUD) BMT have been suggested.

Survival outcomes after rescue extracorporeal cardiopulmonary resuscitation in pediatric patients with refractory cardiac arrest.

Objectives: We report our experience with extracorporeal cardiopulmonary resuscitation with extracorporeal membrane oxygenation in children having cardiac arrest refractory to conventional cardiopulmonary resuscitation and explore predictors for favorable outcome (survival with grossly intact neurologic status).

Methods: We reviewed all patients who required extracorporeal cardiopulmonary resuscitation from 2000 to 2005. Multivariable regression analysis determined factors associated with favorable outcome and time-related survival.

Outcomes of different surgical strategies in the treatment of neonates with aortic coarctation and associated ventricular septal defects.

Background: We reviewed surgical results after treatment of aortic coarctation (CoA) associated with ventricular septal defect (VSD) in neonates. We examined morbidity associated with the two different therapeutic strategies of combined repair versus initial coarctation repair alone and attempted to identify preoperative predictors to guide optimal surgical management.

Methods: Between 1990 and 2006, 141 neonates with CoA and VSDs underwent operation using two management strategies.

Neurologic abnormalities in patients with adenosine deaminase deficiency.

Defects in adenosine deaminase enzyme cause severe immunodeficiency. Without enzyme replacement or allogeneic bone marrow transplantation, patients often suffer fatal infection in infancy. Adenosine deaminase is expressed ubiquitously; deficiency may affect various organs, including the brain.

The sounds of silence: language, cognition, and anxiety in selective mutism.

Objectives: To determine whether oral language, working memory, and social anxiety differentiate children with selective mutism (SM), children with anxiety disorders (ANX), and normal controls (NCs) and explore predictors of mutism severity.

Method: Children ages 6 to 10 years with SM (n = 44) were compared with children with ANX (n = 28) and NCs (n = 19) of similar age on standardized measures of language, nonverbal working memory, and social anxiety. Variables correlating with mutism severity were entered in stepwise regressions to determine predictors of mute behavior in SM.

Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase.

We describe a patient with severe combined immunodeficiency because of aberrations in adenosine deaminase (ADA) who despite adequate replacement with polyethylene glycol-linked ADA (PEG-ADA) for 13 years developed Burkitt's lymphoma. Although treatment corrected the metabolic abnormalities caused by ADA deficiency, it failed to fully restore cellular immunity.

Management options in neonates and infants with critical left ventricular outflow tract obstruction.

Despite being one of the most common congenital cardiac abnormalities, the treatment of critical left ventricular outflow tract obstruction (LVOTO) in the neonate and infant remains a significant challenge. Critical LVOTO in the neonate and infant includes continuous and diverse spectrum of anatomic diagnoses ranging from hypoplastic left heart complex to isolated aortic valvular stenosis with otherwise normally formed left heart structures. Depending on related anatomic features, there are multiple single ventricle and biventricular management strategies available for patients with critical LVOTO; all of which carry significant risk of death and requirement for future interventions.

Pseudotumor cerebri after allogeneic bone marrow transplant associated with cyclosporine a use for graft-versus-host disease prophylaxis.

Pseudotumor cerebri (PTC) is a syndrome of increased intracranial pressure for which several risk factors have been described. We report 2 patients who developed PTC after cyclosporine A (CsA) therapy for graft-versus-host disease (GvHD) prevention after bone marrow transplant. Both patients were obese which may have also contributed to the PTC.

Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve.

Objective: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20-60%). We aim to report our results and identify factors associated with survival and prolonged ventilation.

New developments in the treatment of hypoplastic left heart syndrome.

In the current decade, the prognosis of newborns with hypoplastic left heart syndrome, previously considered a uniformly fatal condition, has dramatically improved through refinement of rapidly evolving treatment strategies. These strategies include various modifications of staged surgical reconstruction, orthotopic heart transplantation, and hybrid palliation using ductal stenting and bilateral pulmonary artery banding. The variety of treatment approaches are based on different surgical philosophies, and each approach has its unique advantages and disadvantages.

Cytotoxic granule disruption is a late event in chemotherapy-induced apoptosis in natural killer YT cells.

Cytotoxic lymphocytes such as Natural Killer (NK) cells can result in leukemias and lymphomas with aggressive clinical course. The cytoplasmic granules of NK cells contain molecules that cause apoptosis of their target cells. In this study, we examined changes of the cytotoxic granules of the Natural Killer cell line YT during Etoposide-induced apoptosis.

Inner ear dysplasia is common in children with Down syndrome (trisomy 21).

Objectives/hypothesis: Middle and external ear anomalies are well recognized in Down syndrome (DS, trisomy 21). Inner ear anomalies are much less frequently described. This study reviews inner ear morphology on imaging to determine the prevalence of cochlear and vestibular anomalies in children with DS.

Incidence and characteristics of facial nerve stimulation in children with cochlear implants.

Objectives: Electrical stimulation from a cochlear implant can spread beyond the auditory nerve. The aims of this study were to accurately measure facial nerve stimulation in pediatric implant users and to determine the characteristics and incidence of this unwanted activity. Part A consisted of a prospective study of a randomized sample of 44 pediatric implant users.

Milk folate secretion is not impaired during iron deficiency in humans.

The purpose of this study was to examine whether maternal iron and/or folate status influences human milk folate secretion and is responsible for growth faltering of Otomi infants in Capulhuac, Mexico. Breast-feeding mothers (n = 71) were randomized at 22 +/- 13 d (baseline) postpartum to receive a daily multivitamin supplement containing folic acid (400 microg) with and without iron (18 mg). Mothers provided blood and milk samples at baseline, and at 82 +/- 15 and 138 +/- 18 d postpartum.