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Background: Neurocognitive functioning is an integral phenotype of 22q11.2 deletion syndrome relating to severity of psychopathology and outcomes. A neurocognitive battery that could be administered remotely to assess multiple cognitive domains would be especially beneficial to research on rare genetic variants, where in‐person assessment can be unavailable or burdensome. The current study compares in‐person and remote assessments of the Penn computerised neurocognitive battery (CNB). Methods: Participants (mean age = 17.82, SD = 6.94 years; 48% female) completed the CNB either in‐person at a laboratory (n = 222) or remotely (n = 162). Results: Results show that accuracy of CNB performance was equivalent across the two testing locations, while slight differences in speed were detected in 3 of the 11 tasks. Conclusions: These findings suggest that the CNB can be used in remote settings to assess multiple neurocognitive domains. [ABSTRACT FROM AUTHOR]

Objectives: A Kommerell''s diverticulum in patients with a right aortic arch may become aneurysmal and be an independent cause of tracheoesophageal compression, even after ligation and division of a left ligamentum. We review the indications for and results of Kommerell''s diverticulum resection and left subclavian artery transfer in children with a right aortic arch who previously underwent vascular ring (ligamentum) division. Methods: From 1998 through 2001, eight children have been referred with recurrent respiratory symptoms (n=8) and/or recurrent dysphagia (n=4) after vascular ring division. Each child had a right aortic arch with a left ligamentum and had undergone division of the ligamentum elsewhere. All had a Kommerell''s diverticulum that was not addressed at the initial operation. All patients had a repeat left thoracotomy with resection of the diverticulum. Five patients had division and reimplantation of the left subclavian artery into the left carotid artery to relieve the sling-like effect of the retroesophageal left subclavian artery on the right aortic arch. One other patient had primary Kommerell''s diverticulum resection and transfer of the left subclavian artery to the left carotid artery. Results: The mean age at the initial operation was 1.7±0.9 years, and the mean age at reoperation was 8.0±3.7 years. In all patients postoperative bronchoscopy confirmed relief of the tracheal compression. There were no complications related to the subclavian artery transfer. Two patients developed postoperative chylothorax, one requiring thoracic duct ligation. The median hospital stay was 5 days. All patients had dramatic resolution of their preoperative symptoms. Conclusions: Kommerell''s diverticulum is an important anatomic structure that can cause recurrent symptoms in patients with a right aortic arch after ligamentum division. In selected patients, reoperation with resection of the Kommerell''s diverticulum and transfer of a retroesophageal left subclavian artery results in relief of symptoms. This technique has become our procedure of choice as a primary operation for children with a right aortic arch and a significant Kommerell''s diverticulum. [Copyright &y& Elsevier]

Aims To evaluate the relative contribution of blood glucose levels at different time points of the day to HbA1c in Type 1 diabetes. Methods Consecutive home blood glucose records (n = 146) from 71 Type 1 diabetic patients who were on an intensive diabetes therapy programme were examined. Each home blood glucose record included six daily blood glucose profiles over 2 months. The relationship between glycaemic values at each time point and HbA1c measured at the end of each record period was analysed. Results Significant linear correlations were found between HbA1c and glycaemia at each time point of the day (ranged from 0.413 to 0.593), the strongest being with predinner glycaemia (r = 0.593; P = 0.000). Total daily glycaemia, mean preprandial and mean postprandial glycaemia were also significant and linearly correlated with HbA1c (r = 0.701; r = 0.686; r = 0.620, respectively; P < 0.0001). Multiple linear regression analysis showed that predinner, prebreakfast and post-breakfast glycaemia correlated significantly and independently with HbA1c. The model accounted for 47.8% of the variance in HbA1c. Conclusions Our study shows that among individual time points, prebreakfast and predinner are those with more influence on HbA1c in Type 1 diabetes and, to a lesser extent, post breakfast. It also confirms that preprandial glycaemia better predicts overall glycaemic control in Type 1 diabetes than postprandial glycaemia. [ABSTRACT FROM AUTHOR]

Objective: The PREDG trial was designed to study the influence of an educative program on gestational weight gain in women with pregestational obesity., Methods: Randomized controlled clinical trial (https://www.isrctn.com/ISRCTN61793947) in 169 women with pregestational obesity (BMI ≥30 kg/m 2 ). Women were randomized to participate in a group education program in nutrition and physical activity or conventional follow-up in a specialized Unit of Obesity and Pregnancy. The nutritional intervention was adjusted to prepregnancy BMI and to the physical activity intensity. Quality was based on the Mediterranean diet. Macronutrients were distributed as follows: 50% carbohydrates, 20% protein and 30% fat. Adequate gestational weight gain was defined between 5 and 9 kg (IOM 2009). Mean gestational weight gain was compared between groups by using the T Student test and frequencies of adequate gestational weight gain were compared by using ꓫ 2 ., Results: Gestational weight gain was lower in the intervention group: 8 (4-11) vs 9.2 (6-13) kg, p 0.026. Gestational weight gain was below 9 kg in 24 of 39 (61.5%) women of the intervention vs 10 of 41 (24.4%) of the control group (p 0.001). Regarding obstetric complications, there were 15 (8.3%) cases of gestational diabetes with no differences between the groups. There were 14 of 85 (18.2%) cases of gestational hypertension or preeclampsia in the intervention group compared with 26 of 84 (32.5%) in the control group (p 0.040). With reference to neonatal weight, there were 7 of 82 (8.5%) large for gestational age neonates in the intervention group compared with 15 of 79 (19.2%) in the control group (p 0.050)., Conclusions: A group-based educative and structured intervention results in an adequate weight gain and lower rates of gestational hypertension, preeclampsia and large for gestational age neonates in pregnant women with obesity., Competing Interests: Declaration of competing interest The authors declare that there is no conflict of interest regarding the publication of this paper., (Copyright © 2023 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

Background: Neuropsychiatric disorders are common in 22q11.2 Deletion Syndrome (22q11DS) with about 25% of affected individuals developing schizophrenia spectrum disorders by young adulthood. Longitudinal evaluation of psychosis spectrum features and neurocognition can establish developmental trajectories and impact on functional outcome., Methods: 157 youth with 22q11DS were assessed longitudinally for psychopathology focusing on psychosis spectrum symptoms, neurocognitive performance and global functioning. We contrasted the pattern of positive and negative psychosis spectrum symptoms and neurocognitive performance differentiating those with more prominent Psychosis Spectrum symptoms (PS+) to those without prominent psychosis symptoms (PS-)., Results: We identified differences in the trajectories of psychosis symptoms and neurocognitive performance between the groups. The PS+ group showed age associated increase in symptom severity, especially negative symptoms and general nonspecific symptoms. Correspondingly, their level of functioning was worse and deteriorated more steeply than the PS- group. Neurocognitive performance was generally comparable in PS+ and PS- groups and demonstrated a similar age-related trajectory. However, worsening executive functioning distinguished the PS+ group from PS- counterparts. Notably, of the three executive function measures examined, only working memory showed a significant difference between the groups in rate of change. Finally, structural equation modeling showed that neurocognitive decline drove the clinical change., Conclusions: Youth with 22q11DS and more prominent psychosis features show worsening of symptoms and functional decline driven by neurocognitive decline, most related to executive functions and specifically working memory. The results underscore the importance of working memory in the developmental progression of psychosis.

Background and Aims: Postpartum glucose metabolism disorders are a common problem in women with gestational diabetes mellitus (GDM). They are often underdiagnosed since many patients do not attend the postpartum screening. This study aims to assess predictors of postpartum glucose metabolism disorders and type 2 diabetes mellitus (T2DM) after GDM., Material and Methods: Retrospective study in women with GMD who underwent postpartum screening for glucose metabolism disorders (n = 2688). Logistic regression was used in the statistical analysis., Results: 24.6% of women had postpartum glucose metabolism disorder. In multivariate analysis, pre-pregnancy body mass index (BMI) 25-30 kg/m 2 (OR 1.46, 95%CI 1.05 to 2.02) or BMI ≥30 kg/m 2 (OR 2.62, 95%CI 1.72 to 3.96), diagnosis of GDM before 20 weeks of pregnancy (OR 2.33, 95%CI 1.57 to 3.46), fasting plasma glucose after diagnosis of GDM ≥90 mg/dl (OR 2.12, 95%CI 1.50 to 2.98), postprandial glucose ≥100 mg/dl (OR 1.47, 95%CI 1.09 to 2.99), and HbA1c in the third trimester of pregnancy ≥5.3% (2.04, 95%CI, 1.52 to 2.75) were independent predictors for any postpartum glucose metabolism disorder., Conclusion: postpartum screening for T2DM should be performed in all women with GDM, and it is especially important not to lose follow-up in those with one or more predictive factors., Competing Interests: Declaration of competing interest The authors declare that there is no conflict of interest regarding the publication of this paper., (Copyright © 2022 Diabetes India. Published by Elsevier Ltd. All rights reserved.)

Aims: This systematic review aims to evaluate the effect of continuous glucose monitoring (CGM) on maternal and neonatal outcomes in gestational diabetes mellitus (GDM)., Methods: Two authors conducted a systematic search using PubMed, Embase, CENTRAL, CINAHL, Scopus, Web of Science, ClinicalTrials.gov and the World Health Organization International Clinical Trials Registry Platform. The inclusion criteria for the systematic review were randomized clinical trials that compared the effects of CGM and blood glucose monitoring (BGM) in women with GDM. A restricted maximum likelihood random-effects model was used for the meta-analysis. The measures of effect were risk ratios for categorical data and mean differences for continuous data., Results: Of the 457 studies reviewed, six randomized clinical trials met the inclusion criteria. A total of 482 patients were included in the meta-analysis. The use of CGM was associated with lower HbA1c levels at the end of pregnancy (mean difference: -0.22; 95%CI -0.42 to -0.03) compared to BGM. Women using CGM also had less gestational weight gain (mean difference: -1.17, 95%CI -2.15 to -0.19), and their children had lower birth weight (mean difference: -116.26, 95%CI -224.70 to -7.81). No differences were observed in the other outcomes evaluated., Conclusion: Women with GDM using CGM may achieve lower average blood glucose levels, lower maternal weight gain and infant birth weight than women using BGM. Nevertheless, current evidence is limited by the low number of studies and the small sample sizes of these studies. Larger clinical trials are needed to better understand the effects of CGM in GDM., Registration: PROSPERO registration ID CRD42021225651., (© 2021 Diabetes UK.)

Objective: The devastation of pharmaceutical production facilities from Hurricane Maria caused a national shortage of parenteral amino acids in October 2017. Our institution decreased trophamine in very low birth weight (VLBW) infants and initiated human milk fortification at a lower feeding volume to increase enteral protein intake more quickly. The objective of this study was to assess how protein management during the shortage period affected the incidence of malnutrition., Methods: This was a retrospective cohort study of infants admitted to 2 neonatal intensive care units from June 1, 2017 to May 31, 2018. Infants between 23 and 32 weeks' gestation were included in this study. The primary outcome was the incidence of malnutrition at 14 days, defined as a z score decline of ≥0.8 SDs, in the pre-shortage period compared with the shortage period. Clinical data regarding adverse effects associated with early fortification and pharmacy costs were recorded., Results: There were 68 infants prior to and 65 during the shortage who met inclusion criteria. There was no difference in malnutrition between the pre-shortage and shortage groups; however, a significant increase in malnutrition was observed in infants who did not receive early fortification during the shortage. No difference in time to full enteral feeds or necrotizing enterocolitis was observed with early fortification., Conclusions: Early fortification in VLBW infants receiving less trophamine during the shortage was not associated with an increase in malnutrition. Restricting trophamine in neonates during the shortage allowed for distribution to other critically ill patients., Competing Interests: Disclosures. The authors declare no conflicts or financial interest in any product or service mentioned in the manuscript, including grants, equipment, medications, employment, gifts, and honoraria., (Copyright. Pediatric Pharmacy Association. All rights reserved. For permissions, email: mhelms@pediatricpharmacy.org 2022.)

Aims: To evaluate if neonatal complications or death were poorer for neonates born small for gestational age (SGA) than for those born with adequate weight or large for gestation age (LGA) to women with gestational diabetes mellitus (GDM)., Methods: Retrospective analysis of the clinical outcomes of neonates born to 3413 women with GDM. The prevalence of neonatal hypoglycaemia, hypocalcaemia, hyperbilirubinemia, polycythaemia, and death was compared among three birthweight groups: SGA, adequate, and LGA. A two-sided chi-squared or Fisher's exact test was used for between-group comparisons. A forward multiple logistic regression was performed to determine the odds ratio (OR) associated with SGA., Results: Neonatal complications were more frequent in the SGA group (20.1%) than in the adequate (9.9%) or LGA (15.2%) groups. There were four deaths (1.6%) in the SGA group compared to one in the LGA (0.4%) and six in the adequate (0.2%) groups (P = 0.002). SGA was a risk factor for neonatal complications or death (OR. 2.122; 95% confidence interval, 1.552-2.899), independent of maternal age, weight gain, fasting glucose, glycaemic control, gestational hypertension, pre-eclampsia, smoking, or neonatal prematurity., Conclusion: SGA birthweight is an important risk factor for neonatal complications or death among neonates born to mothers with GDM., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objectives: Our aim was to investigate the greatest gestational weight gain (GWG) without adverse pregnancy complications in women with gestational diabetes mellitus (GDM) and morbid obesity., Methods: An observational retrospective study including 3284 patients with single pregnancies and GDM was completed. Of the patients, 131 (4.0%) were classified as having pre-pregnancy morbid obesity (BMI ≥ 35 kg/m 2 ). Perinatal complications were compared among BMI groups. In the group with morbid obesity, GWG threshold values to predict outcomes were examined based on sensitivity and specificity values under the receiver operating characteristic curve., Results: GWG was higher in mothers with morbid obesity and macrosomic neonates: 11.3 (4.4-15.7) versus 4.8 (1.5-8.2) kg (p = 0.033). The GWG and neonatal ponderal index were positively correlated (r = 0.305, p = 0.001). The GWG was 7.0 (2.9-11.6) kg in women with hypertensive disorder versus 4.5 (1.0-7.5) kg in normotensive women (p = 0.017). A GWG above 5 kg was a risk factor for macrosomia (87.8% sensitivity, 54.7% specificity) and hypertensive disorder (70.0% sensitivity, 48.4% specificity). GWG associations were maintained after controlling for glycemic control, maternal and gestational age, parity, smoking and neonatal sex., Conclusions for Practice: A GWG below 5 kg is recommended for women with GDM and morbid obesity. In these women, adequate GWG may prevent macrosomia, fetal overgrowth and hypertensive disorder.

Objective: To assess the impact of glycemic control in gestational on neonatal weight and metabolic complications of twin and singleton pregnancies., Methods: An observational, retrospective study to monitor 120 twin and 240 singleton pregnancies in women with GDM. Maternal glycemic parameters during pregnancy (oral glucose tolerance test results, treatment, insulinization rate, mean HbA1c in the third trimester), and neonatal complications and weight were recorded., Results: A higher infant birth weight ratio (IBWR 1.02±0.12 vs. 0.88±0.12, P<.001) and a lower rate of newborns small for gestational age (severe SGA 2.5% vs. 8.3%, P=.012) were seen after singleton pregnancies as compared to twin pregnancies. The rates of newborns large for gestational age (LGA 12.6% vs. 12.5%, P=.989); macrosomic (6.7% vs. 7.5%, P=.777); or small for gestational age (SGA 6.7% vs. 10.8%, P=.175) were similar in both groups. Neonates from twin pregnancies had a higher risk of hypoglycemia (adjusted OR 4.71; 1.38-16.07, P=.013) and polycythemia (adjusted OR 10.05; 1.82-55.42, P=0.008). A linear relationship was seen between third trimester HbA1c levels and IBWR in singleton (r=.199, P=.003), but not in twin pregnancies (r=0.049, P=0.610)., Conclusions: Risk of severe SGA, hypoglycemia, and polycythemia was significantly higher in twin pregnancies of women with GDM. Neonatal weight outcomes and metabolic complications in twin pregnancies of women with GDM were not related to glycemic control. Moreover, in our study population, fasting glucose at diagnosis and mean HbA1c in the third trimester showed a linear relationship with higher birth weights in singleton, but not in twin pregnancies., (Copyright © 2018 SEEN y SED. Publicado por Elsevier España, S.L.U. All rights reserved.)

This study describes the development of a new questionnaire to measure health-related quality of life (HRQoL) in patients with type 1 diabetes (the ViDa1 questionnaire) and provides information on its psychometric properties. For its development, open interviews with patients took place and topics relevant to patients' HRQoL were identified and items were generated. Qualitative analysis of items, expert review, and refinement of the questionnaire followed. A pilot study ( N = 150) was conducted to explore the underlying structure of the 40-item ViDa1 questionnaire. A Principal Component Analysis (PCA) was performed and six of the items that did not load on any of the factors were eliminated. The results supported a four-dimensional structure for ViDa1, the dimensions being Interference of diabetes in everyday life, Self-care, Well-being, and Worry about the disease. Subsequently, the PCA was repeated in a larger sample ( N = 578) with the reduced 34-item version of the questionnaire, and a Confirmatory Factor Analysis (CFA) was performed ( N = 428). Overall fit indices obtained presented adequate values which supported the four-factor model initially proposed [([Formula: see text] 2601.93) ( p < 0.001); Root Mean Square Error of Approximation = 0.060 (CI = 0.056 -0.064)]. As regards reliability, the four dimensions of the ViDa1 demonstrated good internal consistency, with Cronbach's alphas ranging between 0.71 and 0.86. Evidence of convergent-discriminant validity in the form of high correlations with another specific HRQoL questionnaire for diabetes and low correlations with other constructs such as self-efficacy, anxiety, and depression were presented. The ViDa1 also discriminated between different aspects of clinical interest such as type of insulin treatment, presence of chronic complications, and glycemic control, temporal stability, and sensitivity to change after an intervention. In conclusion, the ViDa1 questionnaire presents adequate psychometric properties and may represent a good alternative for the evaluation of HRQoL in type 1 diabetes.

Background: Maternal glucose and weight gain are related to neonatal outcome in women with gestational diabetes mellitus (GDM). The aim of this study was to explore the influence of average third-trimester HbA1c and excess gestational weight gain on GDM neonatal complications., Materials and Methods: This observational study included 2037 Spanish singleton pregnant women with GDM followed in our Diabetes and Pregnancy Unit. The maternal HbA1c level was measured monthly from GDM diagnosis to delivery. Women were compared by average HbA1c level and weight gain categorized into ≤ or > the current Institute of Medicine (IOM) recommendations for body mass index. The differential effects of these factors on large-for-gestational-age birth weight and a composite of neonatal complications were assessed., Results: Women with an average third-trimester HbA1c ≥5.0% (n = 1319) gave birth to 7.3% versus 3.8% (p = 0.005) of large-for-gestational-age neonates and 22.0% versus 16.0% (p = 0.006) of neonates with complications. Women with excess gestational weight gain (n = 299) delivered 12.5% versus 5.2% (p < 0.001) of large-for-gestational-age neonates and 24.7% versus 19.0% (p = 0.022) of neonates with complications. In an adjusted multiple logistic regression analysis among mothers exposed to the respective risk factors, ∼47% and 52% of large-for-gestational-age neonates and 32% and 37% of neonatal complications were potentially preventable by attaining an average third-trimester HbA1c level <5.0% and optimizing gestational weight gain., Conclusions: Average third-trimester HbA1c level ≥5% and gestational weight gain above the IOM recommendation are relevant risk factors for neonatal complications in mothers with gestational diabetes.

Background and Objective: To determine frequency of women with type 1 diabetes showing menstrual cyclic changes in glycemia, analyze their clinical characteristics, and assess the pattern of glycemic changes., Patients and Methods: We analyzed glucose meter readings along 168 menstrual cycles of 26 women with type 1 diabetes. We evaluated mean glucose, mean glucose standard deviation, mean fasting glucose, percentage of glucose readings>7.8 mmol/L and<3.1 mmol/L, and mean insulin dose in 4 periods for each cycle. A woman was identified as having cyclic changes when mean glucose rose from early follicular to late luteal in two-thirds of her menstrual cycles., Results: A percentage of 65.4 of the women had cyclic changes. Characteristics of women with and without cyclic changes, including self-perception of glycemic changes, were similar with exception of age at diabetes diagnosis (22.5 [7.5] vs. 14.4 [9.5] years; P=.039). In women with cyclic changes mean percentage of glucose readings>7.8 mmol/L rose from early follicular (52.2 [16.3] %) to early and late luteal (58.4 [16.0] %, P=.0269; 61.0 [16.9] %, P=.000)., Conclusion: Almost two-thirds of women with type 1 diabetes experience a menstrual cycle phenomenon, attributable to an increase in hyperglycemic excursions during the luteal phase. Enabling women to evaluate their weekly mean glucose from their meter and exploring the causes of hyperglycemic excursions during luteal phase should ensure more accuracy when giving instructions for diabetes management in women with premenstrual hyperglycemia., (Copyright © 2016 Elsevier España, S.L.U. All rights reserved.)

Background: Gestational diabetes mellitus (GDM) may be an expression of early metabolic syndrome. It is unknown whether weight and/or glucose parameters assessed at GDM pregnancies predict the risk of metabolic syndrome at the early postpartum period., Methods: A group of women with GDM (N=1512) was evaluated at 3-11 months postpartum. Incident cases of diabetes were excluded. Antenatal measurements of GDM severity, third-trimester average glycated hemoglobin levels, prepregnancy body mass index (BMI), and increased gestational weight gain were considered. The predictive capability of these factors for postpartum metabolic syndrome was estimated., Results: The prevalence of postpartum metabolic syndrome was 10.9%. The three most common features of metabolic syndrome were low levels of high-density lipoprotein cholesterol (31.2%), high fasting glucose values (23.5%), and a high waist circumference (22.8%). The main predictors of metabolic syndrome were overweight or obesity prepregnancy and high antenatal fasting glycemia. This analysis was adjusted for family history of diabetes, prior GDM, dyslipidemia before pregnancy, chronic arterial hypertension, age, and smoking. The model area 95% confidence interval under the receiver operating characteristic curve was 0.87 (0.84-0.90) for metabolic syndrome presence. The risk for metabolic syndrome was progressively increased as risk factors were added (P<0.001 for trend). When obesity and high fasting glycemia were combined, a multiplied effect ensued., Conclusions: Women having GDM are at threat of early postpartum metabolic syndrome. This risk can be easily identified by assessing prepregnancy BMI and antenatal fasting glycemia in the first pregnancy visit.