An Updated, Evidence-Based Clinician ’s Guide to the Evaluation and Treatment of West Syndrome
AbstractPurpose of ReviewWest syndrome (WS) is a rare disorder consisting of developmental regression, hypsarrhythmia, and infantile spasms. Over time, the clinical approach to this frequently debilitating epileptic encephalopathy has evolved considerably; however, the relative rarity of WS has made it challenging to study its evaluation, treatment, and neurodevelopmental outcome. The purpose of this review is to provide the practicing clinician with an evidence-based approach to WS.Recent FindingsWe focus on recent evidence that informs the clinician of the expected yield of diagnostic testing, including genetic evaluatio...
Source: Current Pediatrics Reports - February 28, 2017 Category: Pediatrics Source Type: research
Lennox-Gastaut Syndrome: a Current Review
AbstractPurpose of ReviewThe diagnosis of Lennox-Gastaut syndrome (LGS), a refractory epileptic encephalopathy, is often made late into disease progression based on the absence of classic EEG abnormalities or typical seizure types at disease onset. The goal of this article is to serve as a general review of LGS for the non-neurologist. As such, we hope to elucidate how a diagnosis is made, highlight novel treatment options, and encourage collaborative treatment decisions between care givers, primary care physicians, neurologists, and psychiatrists.Recent FindingsAlthough a relatively limited number of randomized clinical t...
Source: Current Pediatrics Reports - February 21, 2017 Category: Pediatrics Source Type: research
Pediatric Multiple Sclerosis
AbstractPurpose of ReviewPediatric multiple sclerosis (MS) is an under-diagnosed disease with profound lifelong morbidity. Early recognition is essential so that disease modifying therapies (DMT) can be started and can slow the rate of injury to the brain and spinal cord. This review aims to explore the emerging genetic and environmental risk factors for developing this multiphasic, demyelinating disease. We will also review the clinical and imaging findings of patients with MS, and we will discuss acute management of attacks as well as the newest options in chronic immunomodulatory therapies.Recent FindingsOur understandi...
Source: Current Pediatrics Reports - February 20, 2017 Category: Pediatrics Source Type: research
New Insights and Interventions for Short Bowel Syndrome
AbstractPurpose of ReviewThis review summarizes recent innovations in the treatment of patients with short bowel syndrome.Recent FindingsThe use of surgical procedures, growth factor stimulation, and bioengineering approaches to increase absorptive surface area of the intestine is examined. While the morphology of the intestine is clearly altered by these interventions, it is less clear that the overall function of the intestine is improved.SummaryContinued innovations will likely bring about new therapeutic options for patients with short bowel syndrome. Careful evaluations of the impact of these interventions await contr...
Source: Current Pediatrics Reports - February 1, 2017 Category: Pediatrics Source Type: research
Novel Treatments for Established Food Allergies
This article reviews recently published literature on this rapidly evolving field. (Source: Current Pediatrics Reports)
Source: Current Pediatrics Reports - November 7, 2016 Category: Pediatrics Source Type: research
Finding Treatments for Genetic Metabolic Disease
AbstractGenetic metabolic diseases are comprised of a vast array of single-gene disorders that affect critical cellular biochemical pathways. Various approaches have been taken to treating individual metabolic diseases, each aimed at mitigating specific biochemical pathway deficiencies through targeting facets of cellular function. These approaches include targeting at the gene, enzyme, and biochemical levels via methodologies such as direct gene modification, enzyme replacement therapy, substrate reduction therapy, cofactor administration, dietary management, and organ transplantation. In this review, an overview of each ...
Source: Current Pediatrics Reports - October 26, 2016 Category: Pediatrics Source Type: research
Adolescent Transgender Health: Awareness Does not Equal Resources
(Source: Current Pediatrics Reports)
Source: Current Pediatrics Reports - October 21, 2016 Category: Pediatrics Source Type: research
Testing the Complex Child: CGH Array, WES, Clinical Exome, WGS
AbstractPurpose of ReviewThe purpose of this review was to compare existing strategies for evaluation of complex paediatric patients with newer techniques. Comparative genomic hybridization (CGH) array is the currently accepted first tier genetic test in the evaluation of a pediatric patient with complex physical and developmental anomalies. CGH provides an answer in only 15 –20 % cases, and further genetic testing is required in the majority of cases. This has previously involved sequential single-gene tests, with low yield, significant costs and delay in diagnosis.Summary of Recent FindingsNew genetic techniques allow...
Source: Current Pediatrics Reports - October 12, 2016 Category: Pediatrics Source Type: research
The New Findings in the Genetics and Pathology of Structural Brain Diseases
AbstractPurpose of ReviewMany disorders involving the cerebral hemispheres have been identified and classified; however, in the last five years, significant progress has been made in the identification of genes involved in hindbrain development. Space constraints prohibit an exhaustive review of genetics of brain malformations encountered in the newborn. Rather, this review will address malformations of the corpus callosum, one of the more common malformations, and the hindbrain disorders, a less common entity.Recent FindingsNeuroimaging of the brain is often one of the first steps in the determination of etiology and prog...
Source: Current Pediatrics Reports - October 3, 2016 Category: Pediatrics Source Type: research
Treating Common Fungal Infections in Children
AbstractPurpose of ReviewThe purpose of this paper is to provide a framework for the paediatric practitioner to approach common superficial fungal infections and less frequent but difficult to manage allergic mycoses and invasive fungal infections. Important research findings are summarised and diagnostic and therapeutic guidance is provided. Best practice recommendations to guide the use of topical and systemic antifungals to treat superficial and invasive paediatric fungal infections are outlined here.Recent FindingsThe global burden of fungal infection is greater than 2 million cases per annum; the proportion represente...
Source: Current Pediatrics Reports - August 11, 2016 Category: Pediatrics Source Type: research
Remaining Challenges in Pediatric HIV-1 Infection
< h3 class= " a-plus-plus " > Abstract < /h3 > < span class= " a-plus-plus abstract-section id-a-sec1 " > < h3 class= " a-plus-plus " > Purpose of Review < /h3 > < p class= " a-plus-plus " > Challenges in treatment and prevention remain for pediatric human immunodeficiency virus type 1 (HIV-1) infection as well as increasing concerns for HIV-1 exposed but uninfected infants. < /p > < /span > < span class= " a-plus-plus abstract-section id-a-sec2 " > < h3 class= " a-plus-plus " > Recent Findings < /h3 > < p class= " a-plus-plus " > Current antiretroviral drugs have excellent potency. However, stigma, adherence barriers, ant...
Source: Current Pediatrics Reports - July 21, 2016 Category: Pediatrics Source Type: research
The Role of Hypoallergenic Formula and Dietary Supplements in the Prevention of Early Onset Allergic Disease
< h3 class= " a-plus-plus " > Abstract < /h3 > < span class= " a-plus-plus abstract-section id-a-sec1 " > < h3 class= " a-plus-plus " > Purpose of Review < /h3 > < p class= " a-plus-plus " > Allergic diseases have risen in prevalence in recent decades and placed a significant burden on individuals, their families and the health system. Identifying interventions that may prevent the development of allergies has been a significant focus of research, and the infant diet is often investigated as a potential target as it is readily modifiable. This includes the use of modified ( “hypoallergenic”) infant formulas and dietary...
Source: Current Pediatrics Reports - July 21, 2016 Category: Pediatrics Source Type: research
Natural History of Eosinophilic Oesophagitis in Childhood
Abstract
Purpose of Review
Paediatric eosinophilic oesophagitis (EoE), first described in the 1990s, has emerged as a distinctive chronic inflammatory disease which is now recognised as a major cause of feeding problems in children. Despite the recognition of the clinical importance of EoE, the natural history of this disorder remains unclear, as does understanding of its immunopathogenesis, which makes it difficult to make long-term treatment recommendations. The purpose of this paper is to review available information on the natural history of EoE, including the ...
Source: Current Pediatrics Reports - July 12, 2016 Category: Pediatrics Source Type: research
Infectious Diseases in Refugee Children: To Screen or Not to Screen
< h3 class= " a-plus-plus " > Abstract < /h3 > < span class= " a-plus-plus abstract-section id-a-sec1 " > < h3 class= " a-plus-plus " > Purpose of Review < /h3 > < p class= " a-plus-plus " > To review the evidence on cost-effectiveness of screening refugee children for
latent tuberculosis infection, soil-associated helminths, malaria, chronic
hepatitis B infection and human immunodeficiency virus infection. < /p > < /span > < span class= " a-plus-plus abstract-section id-a-sec2 " > < h3 class= " a-plus-plus " > Recent Findings < /h3 > < p class= " a-plus-plus " > There is strong epidemiologic evidence that it...
Source: Current Pediatrics Reports - July 11, 2016 Category: Pediatrics Source Type: research
Infectious Diseases in Refugee Children: To Screen or Not to Screen
Abstract
Purpose of Review
To review the evidence on cost-effectiveness of screening refugee children for
latent tuberculosis infection, soil-associated helminths, malaria, chronic
hepatitis B infection and human immunodeficiency virus infection.
Recent Findings
There is strong epidemiologic evidence that it is likely to be cost-effective
to screen refugee children from endemic areas for tuberculosis, hepatitis B,
HIV infection and schistosomiasis. The evidence...
Source: Current Pediatrics Reports - July 11, 2016 Category: Pediatrics Source Type: research
