Acquired acrodermatitis enteropathica due to zinc‐depleted parenteral nutrition

Well‐known causes of zinc deficiency, also referred to as acrodermatitis enteropathica (AE), include defects in intestinal zinc transporters and inadequate intake, but a rare cause of acquired zinc deficiency discussed here is an iatrogenic nutritional deficiency caused by parenteral nutrition administered without trace elements. While zinc‐depleted parenteral nutrition causing dermatosis of acquired zinc deficiency was first reported in the 1990s, it is now again relevant due to a national vitamin and trace element shortage. A high index of suspicion may be necessary to diagnose zinc deficiency, particularly because early clinical findings are nonspecific. We present this case of acquired zinc deficiency in a patient admitted to a pediatric intensive care unit for respiratory distress and atypical pneumonia, who subsequently developed a severe bullous eruption due to iatrogenic zinc deficiency but was treated effectively with enteral and parenteral zinc supplementation, allowing for rapid re‐epithelialization of previously denuded skin.     

Communication Regulatory Science: Mapping a New Field

ABSTRACT

Communication regulatory science is an emerging field that uses validated techniques, tools, and models to inform regulatory actions that promote optimal communication outcomes and benefit the public. In the opening article to this special issue on communication and tobacco regulatory science, we 1) describe Food and Drug Administration (FDA) regulation of tobacco products in the US; 2) introduce communication regulatory science and provide examples in the tobacco regulatory science realm; and 3) describe the special issue process and final set of articles. Communication research on tobacco regulatory science is a burgeoning area of inquiry, and this work advances communication science, informs and potentially guides the FDA, and may help to withstand legal challenges brought by the tobacco industry. This research has the potential to have a major impact on the tobacco epidemic and population health by helping implement the most effective communications to prevent tobacco initiation and increase cessation. This special issue provides an example of 10 studies that exemplify tobacco regulatory science and demonstrate how the health communication field can affect regulation and benefit public health.     

The microbiome in pediatric oncology

The human microbiome comprises a diverse set of microorganisms, which play a mostly cooperative role in processes such as metabolism and host defense. Next-generation genomic sequencing of bacterial nucleic acids now can contribute a much broader understanding of the diverse organisms composing the microbiome. Emerging evidence has suggested several roles of the microbiome in pediatric hematology/oncology, including susceptibility to infectious diseases, immune response to neoplasia, and contributions to the tumor microenvironment as well as changes to the microbiome from chemotherapy and antibiotics with unclear consequences. In this review, the authors have examined the evidence of the role of the microbiome in pediatric hematology/oncology, discussed how the microbiome may be modulated, and suggested key questions in need of further exploration.     

Inherited paediatric neurometabolic disorders,can brain magnetic resonance imaging predict?

Objectives:To evaluate diagnostic capability of brain magnetic resonance imaging (MRI) in detection of inherited neurometabolic disorders.Methods:This retrospective observational study was performed in Radiology Department at our Hospital in Dhahran, from January 2013 to January 2020. We evaluated brain MRIs of children (under 5) who were referred to pediatric neurology for clinical suspicion of neuro-developmental delay and metabolic disease. Known perinatal ischemia and birth trauma cases were excluded. Imaging criteria included: (i) bilateral symmetric white matter signal abnormality, (ii) diffusion restriction affecting bilateral deep grey nuclei with or without brainstem involvement, (iii) brain atrophy or edema with abnormal white matter signal, (iv) characteristic MR spectroscopic finding. Presence of any one of these findings was considered positive for neurometabolic disease. Two neuroradiologists interpreted MRIs with substantial interobserver agreement. Diagnoses were confirmed on biochemical/ metabolic screening and genetic testing. A 2 × 2 contingency table was used for results. Chi square test was used to determine association.Results:Out of 133 cases, 72 (49 males, 90% AR) were found to have neurometabolic disorders. Sensitivity, specificity, positive and negative predictive values were calculated as 81.94% (CI, 71.11-90.02), 67.21% (CI, 54.00-78.69), 74.68% (CI, 66.96-81.11) and 75.93% (CI, 65.16-84.17) respectively. Findings were found significant (p-value=0.0001).Conclusion:Brain MRI can help to predict inherited neurometabolic disorders considering certain findings.

Metabolic diseases can be either inherited (inborn errors of metabolism) or acquired. Inborn errors of metabolism (IEMs) that primarily affect the central nervous system are referred to as neurometabolic diseases, and usually occur in neonates and infants.1 These diseases involve genetic defects that result in certain enzyme deficiency leading to deficiency of essential metabolite or toxic accumulation of others with specific biochemical and molecular abnormalities. Clinical presentations may be confusing and potentially lead to delay in diagnosis and treatment.2 Overall incidence of this group of disorders may vary from region to region, being much higher in communities with consanguineous marriages, ranging from 1.2 to 2 per 100,000 live births.3 Most exhibit autosomal recessive (AR) mode of inheritance. Neurometabolic disorders can be classified by various methods based on clinical and biochemical characteristics, area of brain involvement, or cellular organelle.4 Imaging based classification includes leukodystrophy (primary involvement of white matter due to genetic abnormality), leukoencephalopathy (secondary involvement of white matter either due to genetic or acquired systemic disorder), poliodystrophy (predominant involvement of grey matter), and pandydystrophy (mixed involvement of both white and grey matter).5 Central nervous system white matter is usually affected,6 and can result from various pathologic process like delayed myelination (myelin maturation delayed for expected age), hypomyelination (scarcity of myelin or arrest in myelination process), dysmyelination (deposition of abnormally composed fragile myelin), demyelination (secondary loss of myelin that may have been previously normal) and myelinopathy (vacuolating due to deranged brain iron and water hemostasis).Magnetic resonance imaging is the modality of choice for evaluation of neurometabolic disorders.7 Analyzing pattern recognition in MR imaging and clinical clues help to narrow the differential, tailor subsequent laboratory (targeted metabolomics) or genetic investigations (either requiring single gene testing or broad-spectrum genetic testing i.e., whole exome sequencing/ WES).4 The MRI can be helpful in diagnostic workup of various diseases and may be decisive for early management even before arrival of costly and time-consuming biochemical or genetic testing results.8 We therefore sought to highlight ability of MRI in predicting diagnoses of inherited neurometabolic disorders in neonates and young children considering certain MRI findings.     

ASO Author Reflections: Perioperative Antibiotic Prophylaxis for Mastectomy—Too Much or Too Little

Annals of Surgical Oncology -