Erythrocytosis in children and adolescents-classification, characterization, and consensus recommendations for the diagnostic approach

During recent years, the increasing knowledge of genetic and physiological changes in polycythemia vera (PV) and of different types of congenital erythrocytosis has led to fundamental changes in recommendations for the diagnostic approach to patients with erythrocytosis. Although widely accepted for adult patients this approach may not be appropriate with regard to children and adolescents affected by erythrocytosis. The "congenital erythrocytosis" working group established within the framework of the MPN&MPNr-EuroNet (COST action BM0902) addressed this question in a consensus finding process and developed a specific algorithm for the diagnosis of erythrocytosis in childhood and adolescence which is presented here. Pediatr Blood Cancer 2013;9999:XX-XX. © 2013 Wiley Periodicals, Inc.

Fetal Umbilical Artery Doppler Pulsatility Index as a Predictor of Cardiovascular Risk Factors in Children - A Long-Term Follow Up Study

Abstract Objective To determine if high umbilical artery Doppler (UAD) pulsatility index (PI) is associated with cardio-vascular (CV) risk-factors in children at age 12 years. Methods We studied 195 children at age 12 years who had had in-utero UAD studies performed at 28 weeks gestation. The children were grouped according to whether their umbilical Doppler PI was high (indicating poor feto-placental circulation) or normal. At age 12 years we assessed CV risk factors, including anthropometric measures, blood pressure, pulse wave velocity (a measure of arterial compliance), cardio-respiratory fitness and homocysteine and cholesterol serum levels. Results Compared with children with a normal UAD PI (N=88), the children (N=107) with high UAD PI had higher resting pulse rate (p=0.04), higher pulse wave velocity (p=0.046), higher serum homocysteine levels (p=0.032) and reduced arterial compliance (7.58 v 8.50 m/sec, p=0.029) using univariate analysis. These differences were not present when adjusting for cofounders was modelled. Conclusion High PI on UAD testing in-utero may be associated with increased likelihood of some cardio-vascular risk factors at age 12-years but confounding variables may be as important. Our study raises possible long-term benefits of in-utero UAD measurements.

Blocking in children's causal learning depends on working memory and reasoning abilities.

A sample of 99 children completed a causal learning task that was an analogue of the food allergy paradigm used with adults. The cue competition effects of blocking and unovershadowing were assessed under forward and backward presentation conditions. Children also answered questions probing their ability to make the inference posited to be necessary for blocking by a reasoning account of cue competition. For the first time, children's working memory and general verbal ability were also measured alongside their causal learning. The magnitude of blocking and unovershadowing effects increased with age. However, analyses showed that the best predictor of both blocking and unovershadowing effects was children's performance on the reasoning questions. The magnitude of the blocking effect was also predicted by children's working memory abilities. These findings provide new evidence that cue competition effects such as blocking are underpinned by effortful reasoning processes. 

Attention during social interaction in children with autism: Comparison to specific language impairment, typical development, and links to cognition

Eye-tracking studies have shown how people with autism spend significantly less time looking at socially relevant information on-screen compared to those developing typically. This has been suggested to impact on the development of socio-cognitive skills in autism. We present novel evidence of how attention atypicalities in children with autism extend to real-life interaction, in comparison to typically developing (TD) children and children with specific language impairment (SLI). We explored the allocation of attention during social interaction with an interlocutor, and how aspects of attention (awareness checking) related to traditional measures of social cognition (false belief attribution). We found divergent attention allocation patterns across the groups in relation to social cognition ability. Even though children with autism and SLI performed similarly on the socio- cognitive tasks, there were syndrome-specific atypicalities of their attention patterns. Children with SLI were most similar to TD children in terms of prioritising attention to socially pertinent information (eyes, face, awareness checking). Children with autism showed reduced attention to the eyes and face, and slower awareness checking. This study provides unique and timely insight into real-world social gaze (a)typicality in autism, SLI and typical development, its relationship to socio-cognitive ability, and raises important issues for intervention.

A Novel Dried Blood Spot-LCMS Method for the Quantification of Methotrexate Polyglutamates as a Potential Marker for Methotrexate Use in Children

Objective: Development and validation of a selective and sensitive LCMS method for the determination of methotrexate polyglutamates in dried blood spots (DBS).

Methods: DBS samples [spiked or patient samples] were prepared by applying blood to Guthrie cards which was then dried at room temperature. The method utilised 6-mm disks punched from the DBS samples (equivalent to approximately 12 μl of whole blood). The simple treatment procedure was based on protein precipitation using perchloric acid followed by solid phase extraction using MAX cartridges. The extracted sample was chromatographed using a reversed phase system involving an Atlantis T3-C18 column (3 μm, 2.1x150 mm) preceded by Atlantis guard column of matching chemistry. Analytes were subjected to LCMS analysis using positive electrospray ionization.

Key Results: The method was linear over the range 5-400 nmol/L. The limits of detection and quantification were 1.6 and 5 nmol/L for individual polyglutamates and 1.5 and 4.5 nmol/L for total polyglutamates, respectively. The method has been applied successfully to the determination of DBS finger-prick samples from 47 paediatric patients and results confirmed with concentrations measured in matched RBC samples using conventional HPLC-UV technique.

Conclusions and Clinical Relevance: The methodology has a potential for application in a range of clinical studies (e.g. pharmacokinetic evaluations or medication adherence assessment) since it is minimally invasive and easy to perform, potentially allowing parents to take blood samples at home. The feasibility of using DBS sampling can be of major value for future clinical trials or clinical care in paediatric rheumatology. © 2014 Hawwa et al.

Fabricated or Induced Illness in Children: A Narrative Review of the Literature

Although child maltreatment due to abuse or neglect is pervasive within our society, less
is known about fabricated or induced illness by carers (FII), which is considered to be a
rare form of child abuse. FII occurs when a caregiver (in 93% of cases, the mother)
misrepresents the child as ill either by fabricating, or much more rarely, producing
symptoms and then presenting the child for medical care, disclaiming knowledge of the
cause of the problem. The growing body of literature on FII reflects the lack of clarity
amongst professionals as to what constitutes FII, the difficulties involved in diagnosis,
and the lack of research into psychotherapeutic intervention with perpetrators. This lack
of clarity further complicates the identification, management and treatment of children
suffering from FII and may result in many cases going undetected, with potentially lifethreatening
consequences for children. It has been suggested that there is a national
under-reporting of fabricated or induced illness. In practice these cases are encountered
more frequently due to the chronic nature of the presentations, the large number of
professionals who may be involved and the broad spectrum including milder cases that
may not all require a formal child protection response. Diagnosis of fabricated disease
can be especially difficult, because the reported signs and symptoms cannot be confirmed
(when they are being exaggerated or imagined) or may be inconsistent (when they are
induced or fabricated). This paper highlights and discusses the controversies and
complexities of this condition, the risks to the child and how it affects children; the
paucity of systematic research regarding what motivates mothers to harm their children
by means of illness falsification; how the condition should be managed and treated for
both mother and child; and implications for policy and practice.

Interventions to build resilience in children of problem drinkers

This is the protocol for a review and there is no abstract. The objectives are as follows:
-To assess the effects of interventions for building resilience in children or young people living with parents/carers who are problem drinkers.

Thinking about the past, present and future in adolescents growing up in Children's Homes

The present study tested the hypothesis that adolescents growing up in Children's Homes differ from adolescents growing up in a family environment in how they think about their past, present and future, in the way they make decisions about future events and rewards, and in their levels of empathy and perspective taking. The participants were 40 adolescents from Children's Homes in Budapest, Hungary, and 40 age- and gender-matched controls. Group differences were found in participants' past and present time perspectives, and girls from Children's Homes showed reduced consistency in their plans for the future. Additionally, gender differences emerged in empathy, perspective taking, and in participants' present and future time perspectives. We discuss the implications of our findings for interventions to improve the future prospects of adolescents in Children's Homes.