Hyperammonemia From Ureaplasma Infection in an Immunocompromised Child.

Idiopathic hyperammonemia is a rare, poorly understood, and often lethal condition that has been described in immunocompromised patients. This report describes an immunocompromised patient with acute myelogenous leukemia who developed persistent hyperammonemia up to 705 µmol/L (normal, 0 to 47 µmol/L) refractory to multiple different therapies. However, after beginning azithromycin and then doxycycline therapy for Ureaplasma species infection, the patient showed immediate and sustained clinical improvement and resolution of ammonia levels. Recognizing disseminated Ureaplasma species infection as a potential cause of idiopathic hyperammonemia, an unexplained, often fatal condition in immunocompromised patients, and empirically treating for this infection could potentially be lifesaving.

Clinical Practice Approach to Nonalcoholic Fatty Liver Disease by Pediatric Gastroenterologists in the United States.

OBJECTIVES: Nonalcoholic fatty liver disease (NAFLD) is common; however, no information is available on how pediatric gastroenterologists in the United States manage NAFLD. Therefore, study objectives were to understand how pediatric gastroenterologists in the US approach the management of NAFLD, and to identify barriers to care for children with NAFLD. METHODS: We performed structured one-on-one interviews to ascertain each individual pediatric gastroenterologist's approach to the management of NAFLD in children. Responses were recorded from open-ended questions regarding screening for comorbidities, recommendations regarding nutrition, physical activity, medications, and perceived barriers to care. RESULTS: Response rate was 72.0% (486/675). Mean number of patients examined per week was 3 (standard deviation [SD] 3.5). Dietary intervention was recommended by 98.4% of pediatric gastroenterologists. Notably, 18 different dietary recommendations were reported. A majority of physicians provided targets for exercise frequency (72.6%, mean 5.6 days/wk, SD 1.6) and duration (69.9%, mean 40.2 minutes/session, SD 16.4). Medications were prescribed by 50.6%. Almost one-half of physicians (47.5%) screened for type 2 diabetes, dyslipidemia, and hypertension. Providers who spent more than 25 minutes at the initial visit were more likely to screen for comorbidities (P = 0.003). Barriers to care were reported by 92.8% with 29.0% reporting ≥3 barriers. CONCLUSIONS: The majority of US pediatric gastroenterologists regularly encounter children with NAFLD. Varied recommendations regarding diet and exercise highlight the need for prospective clinical trials. NAFLD requires a multidimensional approach with adequate resources in the home, community, and clinical setting.

Magnetic resonance elastography measured shear stiffness as a biomarker of fibrosis in pediatric nonalcoholic fatty liver disease.

Magnetic resonance elastography (MRE) is a promising technique for noninvasive assessment of fibrosis, a major determinant of outcome in nonalcoholic fatty liver disease (NAFLD). However, data in children are limited. The purpose of this study was to determine the accuracy of MRE for the detection of fibrosis and advanced fibrosis in children with NAFLD and to assess agreement between manual and novel automated reading methods. We performed a prospective, multicenter study of two-dimensional (2D) MRE in children with NAFLD. MR elastograms were analyzed manually at two reading centers, and using a new automated technique. Analysis using each approach was done independently. Correlations were determined between MRE analysis methods and fibrosis stage. Thresholds for classifying the presence of fibrosis and of advanced fibrosis were computed and cross-validated. In 90 children with a mean age of 13.1 ± 2.4 years, median hepatic stiffness was 2.35 kPa. Stiffness values derived by each reading center were strongly correlated with each other (r = 0.83). All three analyses were significantly correlated with fibrosis stage (center 1, ρ = 0.53; center 2, ρ = 0.55; and automated analysis, ρ = 0.52; P < 0.001). Overall cross-validated accuracy for detecting any fibrosis was 72.2% for all methods (95% confidence interval [CI], 61.8%-81.1%). Overall cross-validated accuracy for assessing advanced fibrosis was 88.9% (95% CI, 80.5%-94.5%) for center 1, 90.0% (95% CI, 81.9%-95.3%) for center 2, and 86.7% (95% CI, 77.9%-92.9%) for automated analysis. CONCLUSION: 2D MRE can estimate hepatic stiffness in children with NAFLD. Further refinement and validation of automated analysis techniques will be an important step in standardizing MRE. How to best integrate MRE into clinical protocols for the assessment of NAFLD in children will require prospective evaluation. (Hepatology 2017;66:1474-1485).

An evaluation of veterinary allergen extract content and resultant canine intradermal threshold concentrations.

BACKGROUND: Limited information is available for dogs on threshold concentrations (TCs), and the protein composition of common allergenic extracts produced by different manufacturers. HYPOTHESIS/OBJECTIVES: To characterize the protein heterogeneity of tree, grass, weed and mite allergens from different lots of allergenic extracts, and to determine intradermal TCs for healthy dogs using extracts from two manufacturers. ANIMALS: Twenty five privately owned, clinically healthy dogs and ten purpose-bred beagle dogs. METHODS AND MATERIALS: Protein concentration and heterogeneity of 11 allergens from two manufacturers were evaluated using a Bradford-style assay and SDS-PAGE. Intradermal testing was performed with 11 allergens from each company at four dilutions. Immediate reactions were subjectively scored (0 to 4+), and objectively measured (mm) and their percentage concordance evaluated. Model-based TCs were determined by fitting positive reactions (≥2+) at 15 min to generalized estimating equations. RESULTS: Allergen extract protein quantity and composition varied within and between manufacturers despite sharing the same PNU/mL values. Model-based TCs of one weed, five trees, two grasses and a house dust mite were determined for extracts from Manufacturer 1 (M1), and for extracts of three weeds, three trees and two grasses from Manufacturer 2 (M2). Receiver operating characteristic curve analyses determined a percentage concordance of the objective and subjective measurements of 77.3% for M1 and 75% for M2 allergens. CONCLUSIONS AND CLINICAL IMPORTANCE: Veterinary allergen extracts labelled as the same species and PNU/mL are not standardized; they show heterogeneity in composition and potency within and between manufacturers. Variability in extract content may require adjustment of intradermal testing concentrations.

NASPGHAN Clinical Practice Guideline for the Diagnosis and Treatment of Nonalcoholic Fatty Liver Disease in Children: Recommendations from the Expert Committee on NAFLD (ECON) and the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN).

Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It has rapidly evolved into the most common liver disease seen in the pediatric population and is a management challenge for general pediatric practitioners, subspecialists, and for health systems. In this guideline, the expert committee on NAFLD reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD.

Kamp K'aana, a 2-Week Residential Weight Management Summer Camp, Shows Long-Term Improvement in Body Mass Index z Scores.

Long-term effects of Kamp K'aana, a 2-week residential weight management camp, on body mass index (BMI) measures were evaluated on 71 of 108 (66%) obese youth 10 to 14 years of age. Measures were obtained at 11-month study follow-up (n = 38) or extracted from medical record (n = 33). Compared with baseline, BMI increased (P < 0.001), but both BMI percentile and BMI z score decreased (98.7 ±â€Š1.0 to 97.3 ±â€Š6.7 and 2.34 ±â€Š0.30 to 2.23 ±â€Š0.34, P < 0.001). A decrease in BMI z score of ≥0.2 units was seen in 27% of the participants (P < 0.001). The short program has sustained effect.

Relation between vitamin D status and nonalcoholic fatty liver disease in children.

OBJECTIVES: In adults, vitamin D deficiency is common in patients with nonalcoholic fatty liver disease (NAFLD) and has been associated with the severity of histology. There are known differences between adult and pediatric NAFLD, with little data regarding the relation between vitamin D and pediatric NAFLD. The aim of the present study was to examine the relation between vitamin D levels and NAFLD in children. METHODS: Clinical and histological data were used from children ages 2 to 18 years with biopsy-proven NAFLD enrolled in the Nonalcoholic Steatohepatitis Clinical Research Network studies. 25(OH) vitamin D levels were measured from serum. Data examined included demographics, anthropometrics, laboratory markers, and liver histology. Data were analyzed using 3 categories of vitamin D level: deficient (≤ 20 ng/mL), insufficient (21-29 ng/mL), and sufficient (≥ 30 ng/mL). RESULTS: A total of 102 children were studied. There was a high prevalence (80/102, 78%) of vitamin D deficiency or insufficiency; however, there were no significant associations between vitamin D level and the histological characteristics or severity of NAFLD. Significantly higher levels of triglycerides were found in those with vitamin D deficiency (P = 0.004), but there was no association with other features of the metabolic syndrome. CONCLUSIONS: There is a high prevalence of vitamin D deficiency and insufficiency in children with biopsy-proven NAFLD; however, no association was found between vitamin D deficiency and the severity of disease on biopsies. This differs from adult NAFLD studies in which vitamin D deficiency correlates with histological severity, suggesting differences in the risk factors for or consequences of pediatric NAFLD.

A residential summer camp can reduce body fat and improve health-related quality of life in obese children.

In an earlier report, we showed that a 2-week, residential summer camp (Kamp K'aana) led to improved body weight, body mass index, body mass index z score, and self-esteem among obese children. To assess whether improvements in body weight and self-esteem translate into improvement in body fat and weight-related quality of life, we measured the changes in body fat by bioimpedance and quality of life by Impact of Weight on Quality of Life instrument on 42 multiethnic obese children who took part in our Kamp K'aana program. Significant reduction in body fat was detected with significant improvements in the weight-related quality of life scores.

Persons With Spinal Cord Injury Report Peripherally Dominant Serotonin-Like Syndrome After Use of Serotonergic Psychedelics.

Psychedelic-assisted therapy (PAT) may treat various mental health conditions. Despite its promising therapeutic signal across mental health outcomes, less attention is paid on its potential to provide therapeutic benefits across complex medical situations within rehabilitation medicine. Persons with spinal cord injury (SCI) have a high prevalence of treatment-resistant mental health comorbidities that compound the extent of their physical disability. Reports from online discussion forums suggest that those living with SCI are using psychedelics, though the motivation for their use is unknown. These anecdotal reports describe a consistent phenomenon of neuromuscular and autonomic hypersensitivity to classical serotonergic psychedelics, such as psilocybin and lysergic acid diethylamide (LSD). Persons describe intense muscle spasms, sweating, and tremors, with an eventual return to baseline and no reports of worsening of their baseline neurological deficits. The discomfort experienced interferes with the subjective beneficial effects self-reported. This phenomenon has not been described previously in the academic literature. We aim to provide a descriptive review and explanatory theoretical framework hypothesizing this phenomenon as a peripherally dominant serotonin syndrome-like clinical picture-that should be considered as such when persons with SCI are exposed to classical psychedelics. Raising awareness of this syndrome may help our mechanistic understanding of serotonergic psychedelics and stimulate development of treatment protocols permitting persons with SCI to safely tolerate their adverse effects. As PAT transitions from research trials into accepted clinical and decriminalized use, efforts must be made from a harm reduction perspective to understand these adverse events, while also serving as an informed consent process aid if such therapeutic approaches are to be considered for use in persons living with SCI.

Nutritional strategy for adolescents undergoing bariatric surgery: report of a working group of the Nutrition Committee of NASPGHAN/NACHRI.

Surgical options for the treatment of adolescent obesity have been gaining popularity. Adolescent patients present a particular challenge to clinicians, secondary to age-related issues, revolving around both mental and physical growth. These age-related issues require a unique approach to nutritional intervention for adolescents undergoing bariatric surgery as opposed to standardized approaches for adults. Despite the increasing numbers of adolescents undergoing obesity surgery, evidence-based nutritional guidelines have yet to be published. The goal of this document is to provide the clinician with recommendations on how to assess, educate, nourish, and monitor the adolescent who has undergone obesity surgery. A multidisciplinary panel composed of 3 pediatric gastroenterologists, 1 psychologist, and 3 registered dietitians from the Nutrition Committee for the North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition and National Association of Children's Hospitals and Related Institutions, with experience in nutrition and adolescent weight loss surgery, reviewed the medical literature for evidence-based practice for nutritional strategies for patients undergoing bariatric surgery. In addition to this group, an adolescent medicine physician was consulted for matters related to reproductive health. The present article presents a consensus of recommendations based on a review of the literature. In areas for which there was a lack of evidence to support the recommendations, best-practice guidelines were used. The present article provides the clinician with an overview of the nutritional concerns for adolescent patients undergoing obesity surgery. These guidelines address the preoperative educational pathway, the postoperative diet progression, recognition of disordered eating, guidelines for female reproductive issues, and assistance for the adolescent in a school/college environment.

Multicomponent school-initiated obesity intervention in a high-risk, Hispanic elementary school.

The aim of the study was to determine the effectiveness of a school-initiated cognitive and behavioral program to reduce childhood obesity. Height, weight, body mass index (BMI), and BMI z scores were obtained at the beginning and end of the school year at an intervention school (n = 1022) and at a control school (n = 692). The prevalence of overweight and obesity was 18.9% and 30.4% versus 19% and 30.2%, respectively, in the intervention and control schools. The incidence of overweight increased in the control school, but the incidence of obesity, weight, and BMI z scores increased significantly in the intervention school, suggesting that implementation of any school-based obesity intervention programs requires careful planning to achieve goals.

The effect of obesity on the ED(95) of propofol for loss of consciousness in children and adolescents.

INTRODUCTION: Anesthesiologists face a dilemma in determining appropriate dosing of anesthetic drugs in obese children. In this study we determined the dose of propofol that caused loss of consciousness in 95% (ED(95)) of obese and nonobese children as determined by loss of eye lash reflex. METHODS: Forty obese (body mass index [BMI] > 95th percentile for age and gender) and 40 normal weight (BMI 25th to 84th percentile) healthy ASA 1 to 2 children ages 3 to 17 years presenting for surgical procedures were studied using a biased coin design. The primary endpoint was loss of lash reflex at 20 seconds after propofol administration. The first patient in each group received 1.0 mg/kg of IV propofol, and subsequent patients received predetermined propofol doses based on the lash reflex response in the previous patient. If the lash reflex was present, the next patient received a dose increment of 0.25 mg/kg. If the lash reflex was absent, the next patient was randomized to receive either the same dose (95% probability) or a dose decrement of 0.25 mg/kg (5% probability). The ED(95) and 95% confidence intervals (CI) were calculated using isotonic regression and bootstrapping methods respectively. RESULTS: The ED(95) of propofol for loss of lash reflex was significantly lower in obese pediatric patients (2.0 mg/kg, approximate 95% CI, 1.8 to 2.2 mg/kg) in comparison with nonobese patients (3.2 mg/kg, approximate 95% CI, 2.7 to 3.2 mg/kg), P ≤ 0.05. DISCUSSION: A simple approach to deciding what dose of propofol should be used for induction of anesthesia in children ages 3 to 17 years is to first establish the child's BMI on readily available gender-specific charts. Obese children (BMI >95th percentile for age and gender) require a lower weight-based dose of propofol for induction of anesthesia, than do normal-weight children.

Effects of temperature and salt concentration on Francisella noatunensis subsp. orientalis infections in Nile tilapia Oreochromis niloticus.

Little is known about the environmental conditions that allow Francisella noatunensis subsp. orientalis, a worldwide emergent bacterial fish pathogen, to colonize and infect wild and cultured fish. We evaluated the effect of temperature and salinity on the infectivity of F. noatunensis subsp. orientalis in Nile tilapia Oreochromis niloticus (L). Immersion challenges of tilapia with F. noatunensis subsp. orientalis at water temperatures of 25 and 30°C in both sea and fresh water were conducted for 14 d. Morbidity and mortality were recorded daily, and at the completion of the study, a quantitative assessment of the splenic bacterial burden was performed in surviving fish. Fish maintained at 25°C developed francisellosis and had considerably higher mortality and splenic bacterial concentrations compared to control fish and fish maintained at 30°C. Moreover, increasing the water temperature from 25 to 30°C prevented the development of clinical signs and mortality in Francisella-challenged fish. In conclusion, temperature significantly influenced the development of francisellosis in tilapia, whereas salinity had no effect. Our findings may be useful in the establishment of improved prophylactic practices and in the management of outbreaks of francisellosis in the aquaculture industry.

Effect of vitamin E or metformin for treatment of nonalcoholic fatty liver disease in children and adolescents: the TONIC randomized controlled trial.

CONTEXT: Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in US children and adolescents and can present with advanced fibrosis or nonalcoholic steatohepatitis (NASH). No treatment has been established. OBJECTIVE: To determine whether children with NAFLD would improve from therapeutic intervention with vitamin E or metformin. DESIGN, SETTING, AND PATIENTS: Randomized, double-blind, double-dummy, placebo-controlled clinical trial conducted at 10 university clinical research centers in 173 patients (aged 8-17 years) with biopsy-confirmed NAFLD conducted between September 2005 and March 2010. Interventions Daily dosing of 800 IU of vitamin E (58 patients), 1000 mg of metformin (57 patients), or placebo (58 patients) for 96 weeks. MAIN OUTCOME MEASURES: The primary outcome was sustained reduction in alanine aminotransferase (ALT) defined as 50% or less of the baseline level or 40 U/L or less at visits every 12 weeks from 48 to 96 weeks of treatment. Improvements in histological features of NAFLD and resolution of NASH were secondary outcome measures. RESULTS: Sustained reduction in ALT level was similar to placebo (10/58; 17%; 95% CI, 9% to 29%) in both the vitamin E (15/58; 26%; 95% CI, 15% to 39%; P = .26) and metformin treatment groups (9/57; 16%; 95% CI, 7% to 28%; P = .83). The mean change in ALT level from baseline to 96 weeks was -35.2 U/L (95% CI, -56.9 to -13.5) with placebo vs -48.3 U/L (95% CI, -66.8 to -29.8) with vitamin E (P = .07) and -41.7 U/L (95% CI, -62.9 to -20.5) with metformin (P = .40). The mean change at 96 weeks in hepatocellular ballooning scores was 0.1 with placebo (95% CI, -0.2 to 0.3) vs -0.5 with vitamin E (95% CI, -0.8 to -0.3; P = .006) and -0.3 with metformin (95% CI, -0.6 to -0.0; P = .04); and in NAFLD activity score, -0.7 with placebo (95% CI, -1.3 to -0.2) vs -1.8 with vitamin E (95% CI, -2.4 to -1.2; P = .02) and -1.1 with metformin (95% CI, -1.7 to -0.5; P = .25). Among children with NASH, the proportion who resolved at 96 weeks was 28% with placebo (95% CI, 15% to 45%; 11/39) vs 58% with vitamin E (95% CI, 42% to 73%; 25/43; P = .006) and 41% with metformin (95% CI, 26% to 58%; 16/39; P = .23). Compared with placebo, neither therapy demonstrated significant improvements in other histological features. CONCLUSION: Neither vitamin E nor metformin was superior to placebo in attaining the primary outcome of sustained reduction in ALT level in patients with pediatric NAFLD. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00063635.

An innovative summer camp program improves weight and self-esteem in obese children.

To determine the potential benefits of a residential summer camp to treat childhood obesity, 21 obese, multiethnic children (aged 11.4+/-1.4 years; body mass index [BMI] percentile 98.5+/-1.4; BMI z score 2.30+/-0.33) from a diverse socioeconomic background were enrolled in a 2-week summer camp program. Significant improvements (P<0.04) were observed in self-esteem (+0.27+/-0.33 point), body weight (-3.7+/-1.2 kg), BMI (-1.60+/-0.48 kg/m), BMI z score (-0.12+/-0.06), number of curl ups (+10.9+/-21.5), systolic and diastolic blood pressure (-10.8+/-13.4 and -9.4+/-5.5 mmHg, respectively), and heart rate (-8.2+/-12.7 bpm).

Analogs of human genetic skin disease in domesticated animals.

Genetic skin diseases encompass a vast, complex, and ever expanding field. Recognition of the features of these diseases is important to ascertain a correct diagnosis, initiate treatment, consider genetic counseling, and refer patients to specialists when the disease may impact other areas. Because genodermatoses may present with a vast array of features, it can be bewildering to memorize them. This manuscript will explain and depict some genetic skin diseases that occur in both humans and domestic animals and offer a connection and memorization aid for physicians. In addition, we will explore how animal diseases serve as a model to uncover the mechanisms of human disease. The genetic skin diseases we will review are pigmentary mosaicism, piebaldism, albinism, Griscelli syndrome, ectodermal dysplasias, Waardenburg syndrome, and mucinosis in both humans and domesticated animals.

A cross-sectional study of osteocalcin and body fat measures among obese adolescents.

UNLABELLED: Osteocalcin (OCN), a marker of osteoblast activity, has been implicated in the regulation of energy metabolism by the skeleton and thus may affect body fat measures. OBJECTIVE: To examine the relationships of OCN to body fat measures and whether they vary according to markers of energy and vitamin D metabolism. DESIGN AND METHODS: Data were obtained from 58 obese adolescents aged 13-17.9 years (38 females, 8 black or African-American). Total fat mass (FM) [dual X-ray absorptiometry (DXA)] and visceral adipose tissue (VAT) [computerized axial tomography (CT)] were calculated. Blood tests included leptin, OCN, 25-hydroxyvitamin D [25(OH)D], parathyroid hormone (PTH), thyroid function tests, and triglycerides. Markers of glucose metabolism were obtained from fasting and OGTT samples. RESULTS AND CONCLUSIONS: Adolescents with 25(OH)D <20 ng mL(-1) were considered deficient (n = 17/58); none had high PTH (PTH ≥ 65 pg mL(-1) ). OCN was associated with lower VAT (-84.27 ± 33.89 mm(2) ) and BMI (-0.10 ± 0.05 kg m(-2) ), not FM (P = 0.597) in a core model including age, sex, race, geographic latitude, summer, height z-score, and tanner stage. Adding 25(OH)D deficiency and PTH attenuated the inverse association of OCN to VAT. There was a significant interaction of OCN and 25(OH)D deficiency on FM (0.37 ± 0.18 kg, P = 0.041) and BMI (0.28 ± 0.10 kg m(-2) , P = 0.007) in this adjusted model, which was further explained by leptin. Adding A1C to the core model modified the relationship of OCN to VAT (-93.08 ± 35.05 mm(2) , P = 0.011), which was further explained by HOMA-IR. In summary, these findings provide initial evidence for a relationship between OCN and body fat measures that is dependent on energy metabolism and vitamin D status among obese adolescents.

Physical activity and nutrition attitudes in obese Hispanic children with non-alcoholic steatohepatitis.

AIM: To assess nutrition, physical activity and healthful knowledge in obese children with biopsy-proven non-alcoholic steatohepatitis (NASH or NA) compared to children without liver disease. METHODS: Children with biopsy-proven NASH comprised the NASH group. Age, sex and ethnicity matched control groups consisted of obese (OB) and lean (CO) children with no liver disease. Subjects were administered the School Physical Activity and Nutrition Survey and one blood draw was obtained. RESULTS: Fifty-seven patients were enrolled with a mean age of 12.1 ± 2.1 years, and all were Hispanic. Even though the OB and NA had a similar increased body mass index (%), 35% of the NA group always read nutrition labels compared to none in the OB (P < 0.05), and more NA children felt their diet is "less healthy". NA consumed the least amount of fruits with only 25% having ≥ 1 fruit/d vs 45% in OB and 64.7% in CO (P < 0.05 NA vs CO). Only 15% of NA subjects performed light exercise vs 35% and 59% of OB and CO groups, respectively (P = 0.02). The mean physical activity score was lowest in the NA group (P < 0.05). Amongst the subjects with NASH, we found that 100% of patients with grade 2 or 3 fibrosis had a sedentary score > 2 compared to only 63.6% of those with grade 1 or no fibrosis (P < 0.05). CONCLUSION: Children with NASH had increased se-dentary behavior, decreased activity, and fruit intake. Larger studies may determine the benefit of changing these behaviors as treatment for NASH.