Showing papers by "Hospital for Sick Children published in 2006"

Myelin basic protein: a multifunctional protein

Abstract: Myelin basic protein (MBP), the second most abundant protein in central nervous system myelin, is responsible for adhesion of the cytosolic surfaces of multilayered compact myelin. A member of the ‘intrinsically disordered’ or conformationally adaptable protein family, it also appears to have several other functions. It can interact with a number of polyanionic proteins including actin, tubulin, Ca2+-calmodulin, and clathrin, and negatively charged lipids, and acquires structure on binding to them. It may act as a membrane actin-binding protein, which might allow it to participate in transmission of extracellular signals to the cytoskeleton in oligodendrocytes and tight junctions in myelin. Some size isoforms of MBP are transported into the nucleus and thus they may also bind polynucleotides. Extracellular signals received by myelin or cultured oligodendrocytes cause changes in phosphorylation of MBP, suggesting that MBP is also involved in signaling. Further study of this very abundant protein will reveal how it is utilized by the oligodendrocyte and myelin for different purposes.

Photo Elicitation Interview (PEI): Using Photos to Elicit Children’s Perspectives

Abstract: When conducting photo elicitation interviews (PEI), researchers introduce photographs into the interview context. Although PEI has been employed across a wide variety of disciplines and participants, little has been written about the use of photographs in interviews with children. In this article, the authors review the use of PEI in a research study that explored the perspectives on camp of children with cancer. In particular, they review some of the methodological and ethical challenges, including (a) who should take the photographs and (b) how the photographs should be integrated into the interview. Although some limitations exist, PEI in its various forms can challenge participants, trigger memory, lead to new perspectives, and assist with building trust and rapport.

Iodine supplementation for pregnancy and lactation-United States and Canada: recommendations of the American Thyroid Association.

Abstract: The fetus is totally dependent in early pregnancy on maternal thyroxine for normal brain development. Adequate maternal dietary intake of iodine during pregnancy is essential for maternal thyroxine production and later for thyroid function in the fetus. If iodine insufficiency leads to inadequate production of thyroid hormones and hypothyroidism during pregnancy, then irreversible fetal brain damage can result. In the United States, the median urinary iodine (UI) was 168 µg/L in 2001–2002, well within the range of normal established by the World Health Organization (WHO), but whereas the UI of pregnant women (173 µg/L; 95% CI 75–229 µg/L) was within the range recommended by WHO (150–249 µg/L), the lower 95% CI was less than 150 µg/L. Therefore, until additional physiologic data are available to make a better judgment, the American Thyroid Association recommends that women receive 150 µg iodine supplements daily during pregnancy and lactation and that all prenatal vitamin/mineral preparations contain 150 µ...

Essential role for collectrin in renal amino acid transport

Abstract: Angiotensin -converting enzyme 2 (ACE2) is a regulator of the renin angiotensin system involved in acute lung failure, cardiovascular functions and severe acute respiratory syndrome (SARS) infections in mammals. A gene encoding a homologue to ACE2, termed collectrin (Tmem27), has been identified in immediate proximity to the ace2 locus. The in vivo function of collectrin was unclear. Here we report that targeted disruption of collectrin in mice results in a severe defect in renal amino acid uptake owing to downregulation of apical amino acid transporters in the kidney. Collectrin associates with multiple apical transporters and defines a novel group of renal amino acid transporters. Expression of collectrin in Xenopus oocytes and Madin-Darby canine kidney (MDCK) cells enhances amino acid transport by the transporter B(0)AT1. These data identify collectrin as a key regulator of renal amino acid uptake.

Atelectasis Causes Alveolar Injury in Nonatelectatic Lung Regions

Abstract: Rationale: Many authors have suggested that the mechanism by which atelectasis contributes to injury is through the repetitive opening and closing of distal airways in lung regions that are atelectatic. However, neither the topographic nor mechanistic relationships between atelectasis and distribution of lung injury are known.Objectives: To investigate how atelectasis contributes to ventilator-induced lung injury.Methods: Surfactant depletion was performed in anesthetized rats that were then allocated to noninjurious or injurious ventilation for 90 min.Measurements: Lung injury was quantified by gas exchange, compliance, histology, wet-to-dry weight, and cytokine expression, and its distribution by histology, stereology, cytokine mRNA expression, in situ hybridization, and immunohistochemistry. Functional residual capacity, percent atelectasis, and injury-induced lung water accumulation were measured using gravimetric and volumetric techniques.Main Results: Atelectasis occurred in the dependent lung regio...

Assessment of radiation dose awareness among pediatricians

Abstract: There is increasing awareness among pediatric radiologists of the potential risks associated with ionizing radiation in medical imaging. However, it is not known whether there has been a corresponding increase in awareness among pediatricians. To establish the level of awareness among pediatricians of the recent publicity on radiation risks in children, knowledge of the relative doses of radiological investigations, current practice regarding parent/patient discussions, and the sources of educational input. Multiple-choice survey. Of 220 respondents, 105 (48%) were aware of the 2001 American Journal of Roentgenology articles on pediatric CT and radiation, though only 6% were correct in their estimate of the quoted lifetime excess cancer risk associated with radiation doses equivalent to pediatric CT. A sustained or transient increase in parent questioning regarding radiation doses had been noticed by 31%. When estimating the effective doses of various pediatric radiological investigations in chest radiograph (CXR) equivalents, 87% of all responses (and 94% of CT estimates) were underestimates. Only 15% of respondents were familiar with the ALARA principle. Only 14% of pediatricians recalled any relevant formal teaching during their specialty training. The survey response rate was 40%. Awareness of radiation protection issues among pediatricians is generally low, with widespread underestimation of relative doses and risks.

Reading fluency: the whole is more than the parts.

Abstract: This study examined the relative contributions of phonological awareness, orthographic pattern recognition, and rapid letter naming to fluent word and connected-text reading within a dyslexic sample of 123 children in second and third grades. Participants were assessed on a variety of fluency measures and reading subskills. Correlations and hierarchical multiple regression analyses were carried out to explore these relationships. The results demonstrate that phonological awareness, rapid letter naming, and orthographic pattern recognition contribute to word-reading skills. Furthermore, rapid naming, orthographic pattern recognition, and word reading fluency moderately predict different dimensions of connected-text reading (i.e., rate, accuracy, and comprehension) whereas phonological awareness contributes only to the comprehension dimension of connected-text reading. The findings support the multidimensional nature of fluency in which the whole is more than its parts.

Health-related quality of life and treatment satisfaction in North American patients with primary immunedeficiency diseases receiving subcutaneous IgG self-infusions at home

Abstract: The lifelong IgG replacement therapy for patients with primary immunedeficiencies (PIDD) may be provided by intravenous (IVIG) or by subcutaneous IgG (SCIG) infusions. We investigated the impact of weekly SCIG self-infusions at home on the health-related quality of life, treatment satisfaction, and preferences in patients treated with IVIG at the hospital/doctor's office (Group A) or at home (Group B) before the study started. Forty-four adult North American PIDD patients were included in the study, 28 patients in Group A and 16 in Group B. Patients in Group A reported significantly less limitations with their work/daily activities, a significantly improved vitality, and better general health. Treatment satisfaction was significantly improved in Group A. The preference for the subcutaneous route and for home therapy was respectively 81% and 90% in Group A. In Group B, 69% preferred the subcutaneous route and 92% home therapy.

Mutations in the Cystic Fibrosis Transmembrane Regulator Gene and In Vivo Transepithelial Potentials

Abstract: Aim: To examine the relationship between cystic fibrosis transmembrane regulator gene mutations (CFTR) and in vivo transepithelial potentials.Methods: We prospectively evaluated 162 men including 31 healthy subjects, 21 obligate heterozygotes, 60 with congenital bilateral absence of the vas deferens (CBAVD) and 50 with CF by extensive CFTR genotyping, sweat chloride and nasal potential difference testing.Results: Six (10%) men with CBAVD carried no CFTR mutations, 18 (30%) carried one mutation, including the 5T variant, and 36 (60%) carried mutations on both alleles, for a significantly higher rate carrying one or more mutations than healthy controls (90% versus 19%, p < 0.001). There was an overlapping spectrum of ion channel measurements among the men with CBAVD, ranging from values in the control and obligate heterozygote range at one extreme, to values in the CF range at the other. All pancreatic-sufficient patients with CF and 34 of 36 patients with CBAVD with mutations on both alleles carried at lea...

Second malignant neoplasms following childhood Hodgkin's disease: treatment and splenectomy as risk factors.

Abstract: The risk of second malignant neoplasm (SMN) was evaluated in 979 children with Hodgkin's disease. This cohort was diagnosed between 1955 and 1979 at one of the institutions of the Late Effects Study Group. Solid tumors, non-lymphocytic leukemia, and non-Hodgkin's lymphoma (NHL) developed in 18, 17, and 3 patients, respectively. The estimated cumulative probability of developing any SMN was 2% at 5 years from diagnosis, 5% at 10 years, and 9% at 15 years. The incidence is ninefold greater than the risk of acquiring cancer in 19 year-olds, the median age at which the diagnosis of SMN was made in this study population. For leukemia and NHL the corresponding probabilities were 1%, 3%, and 4% for the group as a whole but were increased (2%, 6%, and 8%) in patients who had suffered one or more recurrences. In order to analyze the risk of leukemia and NHL associated with alkylating agent chemotherapy, each patient was assigned a score of one for each alkylating agent administered for a 6-month period. Scores of 2, 4, 6, and 8 were associated with probabilities of leukemia or NHL of 2%, 3%, 6%, and 10%, respectively. In a multivariate analysis for leukemia/lymphoma that included AAD score, stage, and splenectomy, the effect of AAD score and splenectomy did not change substantially compared to the univariate results. AAD score remained statistically significant (P = .0001), and splenectomy was of borderline significance (P = .09). Ot the 18 solid tumor SMNs, 15 developed within the field of radiation, and one other developed in tissue irradiated 34 years earlier for hemangioma. This study of a large and unselected group of children with Hodgkin's disease who received a variety of therapies demonstrates that children are as likely as adults to develop acute leukemia after alkylating agents and solid tumors in the field of radiation therapy.

Identifying the behavioural phenotype in fetal alcohol spectrum disorder: sensitivity, specificity and screening potential

Abstract: Background: In most cases of Fetal Alcohol Spectrum Disorder (FASD), the pathognomonic facial features are absent making diagnosis challenging, if not impossible, particularly when no history of maternal drinking is available. Also because FASD is often comorbid with Attention Deficit Hyperactivity Disorder (ADHD), children with FASD are frequently improperly diagnosed and receive the wrong treatment. Since access to psychological testing is typically limited or non-existent in remote areas, other diagnostic methods are needed to provide necessary interventions.

Craniocervical Arterial Dissection in Children: Clinical and Radiographic Presentation and Outcome:

Abstract: Craniocervical arterial dissection is a recognized cause of arterial ischemic stroke in children. Whether children with craniocervical arterial dissection have dissection characteristics different from those of adults is unclear. A retrospective review of children, 1 month to 18 years of age, with dissection from two Canadian pediatric ischemic stroke registry centers was conducted. From 213 patients with arterial ischemic stroke, 16 (7.5%) were identified with dissection, 37.5% had warning symptoms, and 50% had a history of head or neck trauma. The clinical presentation included headache (44%), altered consciousness (25%), seizures (12.5%), and focal deficits (87.5%). Dissection involved extracranial vessels in 75% and anterior circulation in 56%. Follow-up included complete recovery in 43%, mild to moderate deficits in 44%, and severe deficits in 13%. Fourteen (87.5%) children received antithrombotic treatment. Follow-up angiography showed resolution of abnormalities in 60% of vessels. Total occlusion had the worst outcome for recanalization. In conclusion, the etiology of arterial dissection in the majority of children appears to be either trauma or idiopathic. Long-term angiography shows variable outcomes, depending on the initial findings. The relationship of angiographic outcomes with recurrent strokes requires further study in pediatric dissection. (J Child Neurol 2006;21:8-16).

Ontogeny of drug elimination by the human kidney

Abstract: Renal clearance is an important route of drug elimination While during the neonatal period there is minimal glomerular filtration and active tubular secretion of drugs, there is a well-described rapid development in these processes in the post-neonatal period A less appreciated fact is that during toddlerhood, there is an "overshoot" of the glomerular filtration rate (GFR) well above the levels encountered in older children and adults, and there is an early achievement of adult levels in active drug secretion, which stays at a plateau throughout childhood and adulthood with an "overshoot" in toddlers due to specific transport mechanisms This phenomenon leads to dose requirements for renally excreted drugs in this age group being, on a per-kilogram basis, much larger than in adults This review discusses the mechanisms related to renal ontogeny in drug handling

Hereditary haemorrhagic telangiectasia: mutation detection, test sensitivity and novel mutations

Abstract: Background: Hereditary haemorrhagic telangiectasia (HHT) is a genetic disorder present in 1 in 8000 people and associated with arteriovenous malformations. Genetic testing can identify individuals at risk of developing the disease and is a useful diagnostic tool. Objective: To present a strategy for mutation detection in families clinically diagnosed with HHT. Methods: An optimised strategy for detecting mutations that predispose to HHT is presented. The strategy includes quantitative multiplex polymerase chain reaction, sequence analysis, RNA analysis, validation of missense mutations by amino acid conservation analysis for the ENG ( endoglin ) and ACVRL1 ( ALK1 ) genes, and analysis of an ACVRL1 protein structural model. If no causative ENG or ACVRL1 mutation is found, proband samples are referred for sequence analysis of MADH4 (associated with a combined syndrome of juvenile polyposis and HHT). Results: Data obtained over the past eight years were summarised and 16 novel mutations described. Mutations were identified in 155 of 194 families with a confirmed clinical diagnosis (80% sensitivity). Of 155 mutations identified, 94 were in ENG (61%), 58 in ACVRL1 (37%), and three in MADH4 (2%). Conclusions: For most missense variants of ENG and ACVRL1 reported to date, study of amino acid conservation showed good concordance between prediction of altered protein function and disease occurrence. The 39 families (20%) yet to be resolved may carry ENG , ACVRL1 , or MADH4 mutations too complex or difficult to detect, or mutations in genes yet to be identified.

Assessment of fetal atrioventricular time intervals by tissue Doppler and pulse Doppler echocardiography: normal values and correlation with fetal electrocardiography

Abstract: Objective: To establish gestational age-specific reference values of normal fetal atrioventricular (AV) time interval by spectral tissue Doppler imaging (TDI) and pulse-wave Doppler (PD) methods, and to assess their correlation with signal-averaged fetal PR intervals (ECG). Design: Cohort study. Setting: Tertiary centre for fetal cardiology. Patients and measures: 131 pregnant women between 14 and 42 weeks’ gestation underwent 196 fetal echocardiograms and 158 fetal ECG studies. TDI-derived AV intervals were measured as the intervals from atrial contraction (Aa) to isovolumic contraction (IV) and from Aa to ventricular systole (Sa) at the right ventricular free wall. PD-derived AV intervals were measured from simultaneous left ventricular inflow/outflow (in/out) and superior vena cava/aorta (V/AO) recordings. Results: Measurements were possible by ECG in 61%, by TDI in 100%, by in/out in 100% and by V/AO in 97% of examinations. Aa-IV correlated significantly better with PR intervals (y = 0.67x + 38.29, R 2 = 0.15, p 2 = 0.10, p = 0.002, bias 18.7 ms) and V/AO (R 2 = 0.06, p = 0.02, bias 12.4 ms). Gestational age and AV intervals were positively correlated with all imaging modalities (R 2 = 0.19–0.31, p Conclusion: This study showed the feasibility of fetal AV interval measurements by TDI, and established gestational age-specific reference data. TDI-derived Aa-IV intervals track ECG PR intervals more closely than PD-derived AV intervals and thus should be used as the ultrasound method of choice in assessing fetal AV conduction.

Hippocampal contributions to recollection in retrograde and anterograde amnesia

Abstract: Lesions restricted to the hippocampal formation and/or extended hippocampal system (hippocampal formation, fornix, mammil- lary bodies, and anterior thalamic nuclei) can disrupt conscious recol- lection in anterograde amnesia, while leaving familiarity-based memory relatively intact. Familiarity may be supported by extra-hippocampal medial temporal lobe (MTL) structures. Within-task dissociations in rec- ognition memory best exemplify this distinction in anterograde amnesia. The authors report for the first time comparable dissociations within recognition memory in retrograde amnesia. An amnesic patient (A.D.) with bilateral fornix and septal nuclei lesions failed to recognize details pertaining to personal past events only when recollection was required, during recognition of episodic details. His intact recognition of generic and semantic details pertaining to the same events was ascribed to intact familiarity processes. Recollective processes in the controls were reflected by asymmetrical Receiver's Operating Characteristic curves, whereas the patient's Receiver's Operating Characteristic was symmetri- cal, suggesting that his inferior recognition performance on episodic details was reliant on familiarity processes. Anterograde and retrograde memories were equally affected, with no temporal gradient for retro- grade memories. By comparison, another amnesic person (K.C.) with extensive MTL damage (involving extra-hippocampal MTL structures in addition to hippocampal and fornix lesions) had very poor recognition and no recollection of either episodic or generic/semantic details. These data suggest that the extended hippocampal system is required to sup- port recollection for both anterograde and retrograde memories, regard- less of their age. V V C 2006 Wiley-Liss, Inc.

Understanding help seeking delay in the prodrome to first episode psychosis: a secondary analysis of the perspectives of young people.

Abstract: First episode psychosis represents a critical period for intervention to prevent future impairments and to initiate optimal interventions. Using an interpretive interactionist framework, a secondary analysis of interview transcripts was conducted based on the narratives of youth experiences of psychosis. Our goal was to better understand the factors involved in the decision to seek help (or not) from the mental health system. Findings suggest that help seeking is a social process involving a wide range of influences; two such influences are highlighted in this paper, an individual avoidant strategy of ignoring and hiding early symptoms, and the persuasive influence of significant others in the social network.

Detection of acetaminophen protein adducts in children with acute liver failure of indeterminate cause.

Abstract: OBJECTIVE. Acetaminophen cysteine protein adducts are a widely recognized correlate of acetaminophen-mediated hepatic injury in laboratory animals. The objective of this study was to use a new assay for the detection of acetaminophen cysteine protein adducts in children with acute liver failure to determine the role of acetaminophen toxicity in acute liver failure of unknown cause. METHODS. Serum samples from children with acute liver failure were measured for acetaminophen cysteine protein adducts using high-performance liquid chromatography with electrochemical detection. For comparison, samples from children with well-characterized acetaminophen toxicity and children with known other causes of acute liver failure also were measured for acetaminophen cysteine protein adducts. The analytical laboratory was blinded to patient diagnoses. RESULTS. Acetaminophen cysteine protein adduct was detected in 90% of samples from children with acute liver failure that was attributed to acetaminophen toxicity, 12.5% of samples from children with acute liver failure of indeterminate cause, and 9.6% of samples from children with acute liver failure that was attributed to other causes. Adduct-positive patients from the indeterminate cause subgroup had higher levels of serum aspartate aminotransferase and alanine aminotransferase and lower levels of bilirubin. Adduct-positive patients also had lower rates of transplantation and higher rates of spontaneous remission. CONCLUSIONS. A small but significant percentage of children with acute liver failure of indeterminate cause tested positive for acetaminophen cysteine protein adducts, strongly suggesting acetaminophen toxicity as the cause of acute liver failure. An assay for the detection of acetaminophen cysteine protein adducts can aid the diagnosis of acetaminophen-related liver injury in children.

Long-term effects of transient cerebellar mutism after cerebellar astrocytoma or medulloblastoma tumor resection in childhood.

Abstract: Following cerebellar tumor resection, some patients develop transient cerebellar mutism (TCM). Although the mutism resolves, it is not known whether there are long-term motor speech deficits in patients with TCM that are in excess of those in individuals with cerebellar tumors who had not developed postoperative TCM. Long-term survivors of cerebellar tumors resected in childhood who developed TCM were matched to survivors without TCM and to controls. Speech samples were formally analyzed by two speech pathologists. Tumor survivors who had TCM had significantly more ataxic dysarthric speech and slower speech than either those without TCM or controls and were more dysfluent than controls. Tumor survivors without TCM did not differ from controls on ataxic dysarthria or speech rate. Survivors who had TCM showed more speech deficits than controls or survivors without TCM. The data suggest that speech deficits are chronic if not permanent sequelae of TCM.

Purinoceptors in microglia and neuropathic pain

Abstract: Emerging evidence indicates that microglia play a critical role in the pathogenesis of neuropathic pain, a debilitating chronic pain condition that can occur after peripheral nerve damage caused by disease, infection, or physical injury. Microglia are immunocompetent cells of the central nervous system and express various ionotropic P2X and metabotropic P2Y purinoceptors. After injury to a peripheral nerve, microglia in the spinal cord become activated and upregulate expression of the P2X4 receptor. Recent findings suggest that activation of P2X4 receptors evokes release of brain-derived neurotrophic factor from microglia and that this mediates microglia-neuron signaling leading to pain hypersensitivity. Thus, P2X4 receptors and the intracellular signaling mediators in microglia are promising therapeutic targets for the development of novel pharmacological agents in the management of neuropathic pain.

The Pediatric Heart Network: a primer for the conduct of multicenter studies in children with congenital and acquired heart disease.

Abstract: Most contemporary diagnostic and treatment strategies for pediatric patients with cardiovascular disease are not supported by evidence from clinical trials but instead are based on expert opinion, single-institution observational studies, or extrapolated from adult cardiovascular medicine. In response to this concern, the National Heart, Lung, and Blood Institute established the Pediatric Heart Disease Clinical Research Network (PHN) in 2001. The purposes of this article are to describe the initiation, structure, and function of the PHN; to review the ongoing studies; and to address current and future challenges. To date, four randomized clinical trials and two observational studies have been launched. Design and conduct of complex, multicenter studies in children with congenital and acquired heart disease must address numerous challenges, including identification of an appropriate clinically relevant primary endpoint, lack of preliminary data on which to base sample size calculations, and recruitment of an adequate number of subjects. The infrastructure is now well developed and capable of implementing complex, multicenter protocols efficiently and recruiting subjects effectively. The PHN is uniquely positioned to contribute to providing evidence-based medicine for and improving the outcomes of pediatric patients with cardiovascular disease.

A radial basis classifier for the automatic detection of aspiration in children with dysphagia

Abstract: Background Silent aspiration or the inhalation of foodstuffs without overt physiological signs presents a serious health issue for children with dysphagia. To date, there are no reliable means of detecting aspiration in the home or community. An assistive technology that performs in these environments could inform caregivers of adverse events and potentially reduce the morbidity and anxiety of the feeding experience for the child and caregiver, respectively. This paper proposes a classifier for automatic classification of aspiration and swallow vibration signals non-invasively recorded on the neck of children with dysphagia.

Age at onset of multiple sclerosis may be influenced by place of residence during childhood rather than ancestry.

Abstract: Multiple sclerosis (MS) most commonly affects individuals of Northern European descent who live in countries at high latitude. The relative contributions of ancestry, country of birth and residence as

The Risks and Safety of Clopidogrel in Pediatric Arterial Ischemic Stroke

Abstract: Background and Purpose— The purpose of this study was to determine safety and tolerability of clopidogrel in children with arterial ischemic stroke (AIS). Clopidogrel is the alternative antiplatele...

Colour Perception in ADHD.

Abstract: Attention-deficit/hyperactivity disorder (ADHD) is associated with unexplained impairments on speeded naming of coloured stimuli. These deficits may reflect hypofunctioning retinal dopaminergic mechanisms impairing particularly blue-yellow colour discrimination. Colour perception and rapid colour naming ability were investigated in 14 children with ADHD and 13 healthy peers matched for age, gender, and IQ, using the Farnsworth-Munsell 100 Hue Test (FMT) and the Stroop-Colour-Word test. Children with ADHD committed more errors on the FMT, particularly on discrimination of colours along the blue-yellow axis, and were slower on Stroop subtests involving colour naming. However, the latter deficit was accounted for similarly by blue-yellow and red-green discrimination abilities. Blue-yellow colour perception problems in ADHD contribute to but do not fully explain the observed slowed colour naming.

The Relationship of Spelling Recognition, RAN, and Phonological Awareness to Reading Skills in Older Poor Readers and Younger Reading-Matched Controls

Abstract: The role of spelling recognition was examined in word reading skills and reading comprehension for dyslexic and nondyslexic children. Dyslexic and nondyslexic children were matched on their raw word reading proficiency. Relationships between spelling recognition and the following were examined for both groups of children: verbal ability, working memory, phonological measures, rapid naming, word reading, and reading comprehension. Children’s performance in spelling recognition was significantly associated with their skills in word reading and reading comprehension regardless of their reading disability status. Furthermore, spelling recognition contributed significant variance to reading comprehension for both dyslexic and nondyslexic children after the effects of phonological awareness, rapid naming, and word reading proficiency had been accounted for. The results support the role of spelling recognition in reading development for both groups of children and they are discussed using a componential reading fluency framework.

Basal ganglia germinoma in children with associated ipsilateral cerebral and brain stem hemiatrophy

Abstract: Germinoma is the most common and least-malignant intracranial germ cell tumor, usually found in the midline. Germinoma that arises in the basal ganglia, called ectopic germinoma, is a rare and well-documented entity representing 5% to 10% of all intracranial germinomas. The association of cerebral and/or brain stem atrophy with basal ganglia germinoma on CT and MRI is found in 33% of the cases. To review the literature and describe the CT and MRI findings of basal ganglia germinoma in children, known as ectopic germinoma, with associated ipsilateral cerebral and brain stem hemiatrophy. Three brain CT and six brain MRI studies performed in four children at two institutions were retrospectively reviewed. All patients were male (case 1, 14 years; case 2, 13 years; case 3, 9 years; case 4, 13 years), with pathologically proved germinoma arising in the basal ganglia, and associated ipsilateral cerebral and/or brain stem hemiatrophy on the first imaging study. It is important to note that three of these children presented with cognitive decline, psychosis and slowly progressive hemiparesis as their indication for imaging. Imaging results on initial scans were varied. In all patients, the initial study showed ipsilateral cerebral and/or brain stem hemiatrophy, representing Wallerian degeneration. All patients who underwent CT imaging presented with a hyperdense or calcified lesion in the basal ganglia on unenhanced scans. Only one of these lesions had a mass effect on the surrounding structures. In one of these patients a large, complex, heterogeneous mass appeared 15 months later. Initial MR showed focal or diffusely increased T2 signal in two cases and heterogeneous signal in the other two. The association of a focal lesion in the basal ganglia of children with progressive hemiparesis, neuropsychiatric symptoms and ipsilateral cerebral and/or brain stem hemiatrophy should prompt the diagnosis of ectopic germinoma, avoiding delay in the diagnosis and further irreversible clinical deterioration, in a malignancy with an otherwise favorable prognosis. In these patients, hemiatrophy preceding or accompanying the imaging depiction of a basal ganglia mass lesion is thought to be caused by a paraneoplastic process.

When Family-Centered Care Is Challenged by Infectious Disease: Pediatric Health Care Delivery During the SARS Outbreaks:

Abstract: In this ethnographic study, the authors examined the experiences and perspectives of children hospitalized because of SARS (severe acute respiratory syndrome), their parents, and pediatric health care providers. The sample included 5 children, 10 parents, and 8 health care providers who were directly affected by SARS during the time of the outbreaks and extreme infection control procedures. The data analyses illuminated a range of perceived experiences for this triadic sample. Issues related to social isolation due to infection control precautions were predominant. Themes included emotional upheaval, communication challenges, and changes in parental and professional roles. These findings reveal the cogent effects of SARS on family-centered care. The notion of providing family-centered care within an environment plagued by an infectious outbreak suggests an omniously difficult task. Efforts must be made to optimize family-centered care despite obstacles. The authors suggest effective clinical approaches in the event of future outbreaks.