Since the publication of the Duchenne muscular dystrophy (DMD) care considerations in 2010, multidisciplinary care of this severe, progressive neuromuscular disease has evolved. In conjunction with improved patient survival, a shift to more anticipatory diagnostic and therapeutic strategies has occurred, with a renewed focus on patient quality of life. In 2014, a steering committee of experts from a wide range of disciplines was established to update the 2010 DMD care considerations, with the goal of improving patient care. The new care considerations aim to address the needs of patients with prolonged survival, to provide guidance on advances in assessments and interventions, and to consider the implications of emerging genetic and molecular therapies for DMD. The committee identified 11 topics to be included in the update, eight of which were addressed in the original care considerations. The three new topics are primary care and emergency management, endocrine management, and transitions of care across the lifespan. In part 1 of this three-part update, we present care considerations for diagnosis of DMD and neuromuscular, rehabilitation, endocrine (growth, puberty, and adrenal insufficiency), and gastrointestinal (including nutrition and dysphagia) management.
Objectives
To assess the precision magnetic resonance imaging (MRI) in the neonate and determine if there is an early maternal influence on the pattern of neonatal fat deposition in the offspring of mothers with gestational diabetes (GDM) and obesity compared with the offspring of normal weight women.
Study design
25 neonates, born to normal weight mothers (n=13) and to obese mothers with GDM (n=12), underwent MRI for measurement of subcutaneous and intra-abdominal fat and magnetic resonance spectroscopy for the measurement of intrahepatocellular (IHCL) fat at 1-3 weeks of age.
Results
Infants born to obese/GDM mothers had a mean 68% increase in IHCL compared with infants born to normal weight mothers. For all infants, IHCL correlated with maternal pre-pregnancy BMI but not with subcutaneous adiposity.
Conclusion
Deposition of liver fat in the neonate correlates highly with maternal BMI. This finding may have implications for understanding the developmental origins of childhood NAFLD.
Obese pregnant women may transmit their metabolic phenotype to offspring, leading to a cycle of obesity and diabetes over generations. Early childhood obesity predicts nonalcoholic fatty liver disease (NAFLD), the most common chronic human liver disease. The fetus may be vulnerable to steatosis because immature fetal adipose depots are not available to buffer the excess transplacental lipid delivery in maternal obesity. In animal models, in utero high-fat diet exposure results in an increase in the accumulation of liver triglycerides in offspring and increased hepatic oxidative stress and apoptosis, perhaps priming the liver for later development of NAFLD. Innate immune dysfunction and necroinflammatory changes have been observed in postnatal offspring liver of animals born to high-fat–fed dams. Postweaning, livers of offspring exposed to maternal high-fat feeding in utero share pathophysiologic features with human NAFLD, including increased de novo lipogenesis and decreased free fatty acid oxidation. Human studies using magnetic resonance imaging have shown that maternal BMI predicts infant intrahepatocellular lipid storage, as seen in animal models. The generational transfer of NAFLD may occur via epigenetic changes in offspring liver. Transmission of micro-biota from mother to infant may impact energy retention and immune function that contribute to a predisposition to NAFLD.
Pediatric care facilities must be prepared to act quickly and concertedly in the case of button battery-associated esophageal hemorrhage, which is most likely to present as a "sentinel bleed" in a toddler.
SUMMARY
Although the existence of national data on BBIs provides much-needed information on the epidemiology of these serious injuries, there are no prospective studies available to guide management decisions. As a result, treatment recommendations are primarily based on expert opinion from related clinical experience. The single-center experience of the serious esophageal BBI cases presented in this article is the largest published outside of the compiled national data and offers important lessons for the management of future cases. Additional study is needed to better define the role of acid blockade, endoscopy, and MRI in the postremoval management of these cases and determine optimal timing for initiation of enteral feeding. Advocacy efforts are ongoing and vital in order to minimize the occurrence of these potentially fatal injuries.
CS infants exhibited different microbiota in the oral inoculum, a chaotic pattern of bacterial succession, and a persistent deficit of intestinal Bacteroidetes. Pioneer OP bacteria transferred from maternal vaginal and intestinal communities were not prominent constituents of the early infant fecal microbiome. Oral inoculation at birth may impact the intestinal microenvironment, thereby modulating early succession of intestinal bacteria.
Despite ongoing efforts of the national Button Battery Task Force (BBTF), cases of major morbidity and mortality continue to be reported at a higher rate after ingestion of higher voltage and larger button batteries. Initiation of sucralfate or honey shortly after some button battery ingestions is crucial to prevent further injury while awaiting emergent removal. Endoscopic acetic acid irrigation post removal of button battery may halt deeper injury. Gastroenterologists need to be aware of the updates to the National Capital Poison Center algorithm for button battery ingestion.
This study demonstrates a concerning and related pattern of IMCL and visceral fat deposition in prepubertal children. Intramuscular fat deposition is linked to markers of insulin resistance and cardiovascular disease risk.
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