Establishing Normative Values for Healthy Term Infant Feeding Performance: Neonatal Eating Assessment Tool-Mixed, Oral Feeding Scale, and Early Feeding Skills Assessment.

PURPOSE: Infants with perceived feeding problems are frequently referred for assessment of their feeding abilities. However, little is known regarding how healthy nondysphagic infants perform on commonly used assessments, making determination of impairment difficult. The aim of this investigation was to elucidate the characteristics of healthy term infant feeding performance using three commonly employed clinical assessments: Neonatal Eating Assessment Tool-Mixed (NeoEat-Mixed), Oral Feeding Scale, and Early Feeding Skills (EFS). METHOD: In this prospective case-control study, we recruited 30 infants without feeding impairments to undergo video-monitored bottle feeds under their normal feeding conditions. Caregiver perception of infant feeding was evaluated using the NeoEat-Mixed. Milk ingestion was monitored real time using the Oral Feeding Scale for rate of milk transfer and modified proficiency as characterized by the total volume consumed out of the total volume the caregiver provided. Videos were analyzed by two speech pathologists using the EFS assessment. Descriptive statistics were used to characterize performance. RESULTS: Participants underwent feeding monitoring at an average chronological age of 4 ± 2 months. Caregivers primarily reported normal, nonconcerning feeding patterns across all of the NeoEAT-Mixed outcomes. Infants consumed milk at an average rate of transfer of 7 ± 3 ml/min, a modified proficiency of 50 ± 21%, and achieved the highest OFS score of 4 (93%, n = 28). The majority of infants scored the best EFS score (mature-3) as it related to the absence of color changes during the feed (97%, n = 29), although commonly scored in the worst EFS score (immature-1) in their presentation of one or more compelling stress cues (63%, n = 19). CONCLUSION: Establishing healthy term infant normative values for commonly used feeding assessments is critical in accurately distinguishing infants with feeding impairments from those with normal developmental variants.

Changes in Food Security Status During Undergraduate Enrollment.

OBJECTIVE: To explore changes in student food security status (FSS) during college enrollment and correlates of those changes. DESIGN: Two cross-sectional surveys (2016 and 2019). PARTICIPANTS: Undergraduates (n = 338) at a public university who completed both surveys. VARIABLES MEASURED: Food security status (US Department of Agriculture 10- and 18-item measures), demographics, and grade point average (GPA). ANALYSIS: Chi-square tests of goodness of fit for representativeness, McNemar-Bowker and paired samples Wilcoxon signed rank test for within-student FSS differences over time, logistic regression for the relationship between demographics and FSS over time, and ANCOVA for the relationship between FSS and GPA. Critical P ≤ 0.05 unless otherwise noted. RESULTS: The prevalence of food insecurity was 22.4% in 2016 and 41.0% in 2019, and the McNemar-Bowker test indicated a significant asymmetrical relationship in FSS over time (P < 0.001). Almost half of the respondents (47.0%) reported experiencing food insecurity at least once. In multivariate analyses controlling for other demographic variables, first-generation students had the most consistent risk of food insecurity with lower FSS in 2016 (P < 0.001) and 2019 (P < 0.001), and they were more likely to have worsened FSS over time (P = 0.05). Food insecurity at either time was related to a lower 2019 GPA (P < 0.001). CONCLUSIONS AND IMPLICATIONS: Food security status measured cross-sectionally cannot capture changes in student FSS, which many students may experience. Longitudinal research is needed to better understand the role of demographic and student factors in FSS changes over time. Food resources should be offered to all students, as many will need them at some point.

Imaging of pediatric neuroblastoma: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Neuroblastoma is the most common extracranial solid neoplasm in children. This manuscript provides consensus-based imaging recommendations for pediatric neuroblastoma patients at diagnosis and during follow-up.

Imaging of pediatric thyroid tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Pediatric thyroid cancer is rare in children; however, incidence is increasing. Papillary thyroid cancer and follicular thyroid cancer are the most common subtypes, comprising about 90% and 10% of cases, respectively. This paper provides consensus imaging recommendations for evaluation of pediatric patients with thyroid cancer at diagnosis and during follow-up.

Magnetic resonance imaging glossary of findings of pediatric pancreatitis and the revised Atlanta classification.

While still uncommon, the incidence of acute pancreatitis in children has been increasing over the last two decades. The Atlanta classification for acute pancreatitis, developed for adults, stratifies cases of acute pancreatitis based on imaging and clinical criteria. This classification scheme allows for standardized use of terminology to facilitate treatment and prognostication. Although US and CT should be used in critical or unstable patients, MRI is an ideal imaging modality in pediatric patients with acute pancreatitis because of its ability to characterize tissue without ionizing radiation. We review MRI examples specific to Atlanta classification terminology in pediatric patients. Chronic pancreatitis has also been increasingly diagnosed in children, and imaging plays a key role in the diagnosis and management of this insidious disease. MRI with magnetic resonance cholangiopancreatography is the optimal modality for assessing the pancreas in a child with known or suspected chronic pancreatitis because it provides tissue characterization and high-contrast imaging of the pancreatic duct without the use of invasive instrumentation or ionizing radiation. We also review and demonstrate accepted MRI findings of chronic pancreatitis.

Anti-MDA5 juvenile idiopathic inflammatory myopathy with second-degree heart block but no skin or lung involvement: a case report.

BACKGROUND: Patients with idiopathic inflammatory myopathy and autoantibodies directed against melanoma differentiation-associated protein 5 (MDA5) characteristically have interstitial lung disease, severe cutaneous involvement, arthritis, and relatively mild myositis. Cardiac involvement in idiopathic inflammatory myopathy can occur and has been associated with anti-signal recognition particle and anti-polymyositis-scleroderma autoantibodies, but not with anti-MDA5 autoantibodies. CASE PRESENTATION: A 14-year-old male presented with weakness, second-degree heart block, arthritis, and hematologic cytopenias. Imaging and biopsies confirmed the diagnosis of juvenile idiopathic inflammatory myopathy, and he had high titer anti-MDA5 autoantibodies. There were no cutaneous or pulmonary abnormalities. While on prednisone and methotrexate, the patient's heart block improved from second- to first-degree and the cytopenias resolved. Persistent myositis prompted the addition of intravenous immunoglobulin. Seven months into the disease course, the arthritis and myositis are in remission and the patient is no longer taking corticosteroids. CONCLUSIONS: We report a novel case of a patient with juvenile idiopathic myositis who lacked the typical cutaneous and pulmonary findings associated with anti-MDA5 positivity, but who had cardiac conduction defects. This report broadens the clinical spectrum of anti-MDA5-associated inflammatory myopathy.

A 9-Month-Old with Skeletal Abnormalities and a Consanguineous Sibling with Mucopolysaccharidosis IVA: The Role of Urinary Glycosaminoglycan Testing in Disease Diagnosis and Treatment Monitoring.

Mucopolysaccharidosis IVA (MPS IVA) is a rare autosomal recessive lysosomal storage disorder resulting from N-acetylgalactosamine-6-sulfatase (GALNS) deficiency that occurs in approximately 1 in 76 000 to 1 in 640 000 live births. Given that the diagnosis of MPS IVA relies heavily on the results of initial urine glycosaminoglycan (GAG) screening, cases that present with falsely normal urine GAG concentrations can delay the diagnosis and follow-up care for patients. This case study follows a patient diagnosed with MPS IVA at 9 months of age based on relation to a consanguineous 3-year-old sibling with MPS IVA and the use of direct enzyme activity analysis. Details regarding skeletal presentation and identification of genetic variants are presented along with data on follow-up urinary GAG monitoring during treatment with enzyme replacement therapy and treatment for a growth hormone disorder.

Mixed epithelial and stromal tumor after pediatric kidney transplant.

MEST of the kidney are a benign group of tumors with very rare incidence of malignant transformation. First described in 1998, this tumor has never been reported in a transplanted organ before. We present a unique case of de novo MEST in a donor kidney 4 years after transplant into a pediatric patient. Although removal of the lesions is curative without risking malignant transformation, in this case, surgical removal was not attempted to prevent reduction in transplant longevity. In this unique report of MEST in a transplanted kidney, we describe the patient/transplant outcomes without MEST resection.

Liver Doppler Findings in Pediatric Patients After Total Pancreatectomy and Islet Autotransplantation.

OBJECTIVES: This study was intended to establish normal values for velocities in the hepatic artery and portal veins in pediatric patients after total pancreatectomy and islet autotransplantation (TPIAT). METHODS: A retrospective review was performed of liver Doppler studies in pediatric patients after TPIAT over 6 years at an academic children's hospital. Doppler velocities in the liver vasculature and the hepatic artery resistive index were recorded. RESULTS: Sixty-five pediatric patients were evaluated. There were no cases of portal vein thrombosis or other hepatic vascular complications. The mean hepatic artery peak systolic velocity was 157.6 cm/s (SE, 60.9 cm/s), with a mean resistive index of 0.57 (SE, 0.09). The mean main portal vein velocity was 31.9 cm/s (SE, 12.9 cm/s). CONCLUSIONS: Portal vein thrombosis is rare in pediatric patients after TPIAT. A wide range of hepatic artery and portal vein velocities are encountered in children immediately after TPIAT without hepatic vascular complications and can be considered normal.

Fetal MRI: incidental findings in the mother.

BACKGROUND: Fetal magnetic resonance imaging (MRI) is a routinely used tool in prenatal diagnosis; however, there is a lack of studies evaluating incidental findings observed in the mother. OBJECTIVE: This study describes and quantifies incidental findings observed in the mother during fetal MRI. MATERIALS AND METHODS: We reviewed all fetal MRI studies at the University of Minnesota Medical Center from February 2008 to September 2014. Two pediatric radiologists retrospectively conducted a consensus evaluation. The maternal findings were categorized into neurologic, gynecologic, urinary, gastrointestinal and musculoskeletal. Hydronephrosis consistent with the stage of pregnancy was recorded but was not included as an abnormal finding. Abnormal findings were classified into three groups, depending on their clinical significance: level I (low), level II (medium) and level III (high). RESULTS: We evaluated 332 pregnant patients with a mean age of 29.3 years and a mean gestational age of 29 weeks. Of these, 55.4% had at least 1 incidental finding, for a total of 262 incidental maternal findings. Of the 262 abnormalities, 113 (43.1%) were neurologic, 69 were gynecologic (26.3%), 36 (13.7%) urinary, 24 (9.2%) gastrointestinal and 20 (7.6%) musculoskeletal. Of the 262 incidental findings, 237 (90.5%) were level I, 24 (9.2%) were level II and 1 (0.4%) was level III. CONCLUSION: Our results suggest that although the vast majority of incidental maternal findings are benign, more significant findings are still encountered and should be expected.

The transverse diameter of the chest on routine radiographs reliably estimates gestational age and weight in premature infants.

BACKGROUND: Prior to digital radiography it was possible for a radiologist to easily estimate the size of a patient on an analog film. Because variable magnification may be applied at the time of processing an image, it is now more difficult to visually estimate an infant's size on the monitor. Since gestational age and weight significantly impact the differential diagnosis of neonatal diseases and determine the expected size of kidneys or appearance of the brain by MRI or US, this information is useful to a pediatric radiologist. Although this information may be present in the electronic medical record, it is frequently not readily available to the pediatric radiologist at the time of image interpretation. OBJECTIVE: To determine if there was a correlation between gestational age and weight of a premature infant with their transverse chest diameter (rib to rib) on admission chest radiographs. MATERIALS AND METHODS: This retrospective study was approved by the institutional review board, which waived informed consent. The maximum transverse chest diameter outer rib to outer rib was measured on admission portable chest radiographs of 464 patients admitted to the neonatal intensive care unit (NICU) during the 2010 calendar year. Regression analysis was used to investigate the association between chest diameter and gestational age/birth weight. Quadratic term of chest diameter was used in the regression model. RESULTS: Chest diameter was statistically significantly associated with both gestational age (P < 0.0001) and birth weight (P < 0.0001). CONCLUSION: An infant's gestational age and birth weight can be reliably estimated by comparing a simple measurement of the transverse chest diameter on digital chest radiograph with the tables and graphs in our study.

Beyond acute appendicitis: imaging of additional pathologies of the pediatric appendix.

Primary acute appendicitis is by far the most common pathological condition affecting the appendix. There are differential diagnoses, however, when an abnormal appendix is found by imaging. The purpose of this paper is to highlight the imaging and clinical manifestations of less common appendiceal abnormalities in children. Familiarity with these alternative diagnoses might be particularly helpful in guiding management of the child whose clinical presentation is not typical for primary acute appendicitis.

Increased insulin sensitivity in paternal Gnas knockout mice is associated with increased lipid clearance.

The G protein alpha-subunit Gsalpha is required for hormone-stimulated cAMP generation. The Gsalpha gene Gnas is a complex gene with multiple imprinted gene products. Mice with heterozygous disruption of the Gnas paternal allele (+/p-) are partially Gsalpha deficient and totally deficient in XLalphas, a neuroendocrine-specific Gsalpha isoform that is expressed only from the paternal Gnas allele. We previously showed that these mice are hypermetabolic and lean and have increased insulin sensitivity. We now performed hyperinsulinemic-euglycemic clamp studies, which confirmed the markedly increased whole body insulin sensitivity in +/p- mice. +/p- mice had 1.4-, 7- and 3.8-fold increases in insulin-stimulated glucose uptake in muscle and white and brown adipose tissue, respectively, and markedly suppressed endogenous glucose production from the liver. This was associated with increased phosphorylation of insulin receptor and a downstream effector (Akt kinase) in both liver and muscle in response to insulin. Triglycerides cleared more rapidly in +/p- mice after a bolus administered by gavage. This was associated with decreased liver and muscle triglyceride content and increased muscle acyl-CoA oxidase mRNA expression. Resistin and adiponectin were overexpressed in white adipose tissue of +/p- mice, although there was no difference in serum adiponectin levels. The lean phenotype and increased insulin sensitivity observed in +/p- mice is likely a consequence of increased lipid oxidation in muscle and possibly other tissues. Further studies will clarify whether XLalphas deficiency is responsible for these effects and if so, the mechanism by which XLalphas deficiency leads to this metabolic phenotype.

Genetic background (C57BL/6J versus FVB/N) strongly influences the severity of diabetes and insulin resistance in ob/ob mice.

We studied the effects of genetic background on the phenotype of ob/ob mice, a model of severe obesity, insulin resistance, and diabetes caused by leptin deficiency. Despite a comparable degree of obesity and hyperinsulinemia, C57BL/6J ob/ob mice had much milder hyperglycemia and, surprisingly, normal circulating adiponectin levels despite still-prominent signs of insulin resistance. Hyperinsulinemic-euglycemic clamp revealed relatively less whole-body and muscle insulin resistance in C57BL/6J ob/ob mice, whereas liver insulin resistance tended to be more severe than in FVB/N ob/ob mice. C57BL/6J ob/ob mice had also more rapid clearance of circulating triglycerides and more severe hepatic steatosis. We suggest that strain-related distinction in lipid handling is the most important player in the differences in diabetic phenotype and insulin sensitivity, whereas the impact of circulating adiponectin levels on the overall phenotype of ob/ob mice is less important.

Liver peroxisome proliferator-activated receptor gamma contributes to hepatic steatosis, triglyceride clearance, and regulation of body fat mass.

Peroxisome proliferator-activated receptor gamma (PPAR gamma) is a nuclear receptor that mediates the antidiabetic effects of thiazolidinediones. PPAR gamma is present in adipose tissue and becomes elevated in fatty livers, but the roles of specific tissues in thiazolidinedione actions are unclear. We studied the function of liver PPAR gamma in both lipoatrophic A-ZIP/F-1 (AZIP) and wild type mice. In AZIP mice, ablation of liver PPAR gamma reduced the hepatic steatosis but worsened the hyperlipidemia, triglyceride clearance, and muscle insulin resistance. Inactivation of AZIP liver PPAR gamma also abolished the hypoglycemic and hypolipidemic effects of rosiglitazone, demonstrating that, in the absence of adipose tissue, the liver is a primary and major site of thiazolidinedione action. In contrast, rosiglitazone remained effective in non-lipoatrophic mice lacking liver PPAR gamma, suggesting that adipose tissue is the major site of thiazolidinedione action in typical mice with adipose tissue. Interestingly, mice without liver PPAR gamma, but with adipose tissue, developed relative fat intolerance, increased adiposity, hyperlipidemia, and insulin resistance. Thus, liver PPAR gamma regulates triglyceride homeostasis, contributing to hepatic steatosis, but protecting other tissues from triglyceride accumulation and insulin resistance.

Risk factors for diabetes in familial partial lipodystrophy, Dunnigan variety.

OBJECTIVES: Familial partial lipodystrophy, Dunnigan variety (FPLD), is an autosomal dominant disorder due to missense mutations in the lamin A/C (LMNA) gene encoding nuclear lamina proteins. It is characterized by loss of subcutaneous fat from the extremities and trunk and accumulation of fat in the head and neck region beginning at puberty. Patients with FPLD are predisposed to metabolic complications of insulin resistance such as diabetes. We sought to identify risk factors for diabetes in patients with FPLD. RESEARCH DESIGN AND METHODS: A cross-sectional study comparing clinical, biochemical, and anthropometric variables and LMNA genotypes in FPLD patients with and without diabetes. RESULTS: We studied 52 women and 24 men with FPLD from 18 different families. Twenty-eight women (54%) but only four men (17%) had diabetes (P < 0.001); therefore further comparisons were mostly limited to women. Compared with women without diabetes, those with diabetes had higher BMI (median values 23 vs. 24 kg/m(2), respectively; P = 0.03), increased chin skinfold thickness (10 vs. 20 mm; P = 0.001), lower rates of nulliparity (60% vs. 28%; P = 0.04), and higher levels of fasting serum triglycerides (2.4 vs. 3.5 mmol/l; P < 0.001) but similar serum leptin levels (3.4 vs. 3.6 ng/ml; P = 0.9). The prevalence of diabetes was not related to age, menopausal status, family history of type 2 diabetes in unaffected relatives, or LMNA genotype. CONCLUSIONS: We conclude that increased adiposity as reflected by excess subcutaneous fat accumulation in the chin region and parity may predispose women with FPLD to develop diabetes.