Unveiling the strong positive relationship: Maternal characteristics and neonatal outcomes in the Better Outcomes in Labour Difficulty (BOLD) study - a secondary analysis validating neonatal near miss classification.

Background: The near miss concept, denoting near collisions between aircraft, originated in aeronautics, but has recently been transferred to the neonatal context as a way of evaluating the quality of health services for newborns, especially in settings with reduced child mortality. However, there is yet no consensus regarding the underlying criteria. The most common indicators used to assess health care quality include mortality (maternal and neonatal) and life-threatening conditions.

A research agenda to improve incidence and outcomes of assisted vaginal birth.

Access to emergency obstetric care, including assisted vaginal birth and caesarean birth, is crucial for improving maternal and childbirth outcomes. However, although the proportion of births by caesarean section has increased during the last few decades, the use of assisted vaginal birth has declined. This is particularly the case in low- and middle-income countries, despite an assisted vaginal birth often being less risky than caesarean birth.

The International Collaborative Gaucher Group GRAF (Gaucher Risk Assessment for Fracture) score: a composite risk score for assessing adult fracture risk in imiglucerase-treated Gaucher disease type 1 patients.

Background: Fractures in Gaucher disease type 1 (GD1) patients cause significant morbidity. Fracture risk may be decreased by enzyme replacement therapy (ERT) but not eliminated. When considering initiation of treatment, it is useful to know to what extent fixed patient-specific factors determine risk for future fractures beyond standard risk factors that change with time and treatment, such as decreased bone mineral density.

Gaucher disease type 1 patients from the ICGG Gaucher Registry sustain initial clinical improvements during twenty years of imiglucerase treatment.

Background: Alglucerase enzyme replacement therapy was approved for Gaucher disease (GD) in the United States in 1991; imiglucerase in 1994. We report hematologic, visceral, bone pain, bone crisis, height, weight, and Body Mass Index (BMI) outcomes in patients treated for 20 (±3) years with subset analyses based on pre-treatment severity, genotype, and age at treatment initiation.

Methods: GD type 1 (GD1) patients in the ICGG Gaucher Registry with complete sets of baseline, 10-year, and 20-year data are included (N = 475).

Maple syrup urine disease in Brazilian patients: variants and clinical phenotype heterogeneity.

Background: Maple syrup urine disease (MSUD) is an autosomal recessive inherited metabolic disease caused by deficient activity of the branched-chain α-keto acid dehydrogenase (BCKD) enzymatic complex. BCKD is a mitochondrial complex encoded by BCKDHA, BCKDHB, DBT, and DLD genes. MSUD is predominantly caused by Variants in BCKDHA, BCKDHB, and DBT genes encoding the E1α, E1β, and E2 subunits of BCKD complex, respectively.

Essential and toxic elements in human milk concentrate with human milk lyophilizate: A preclinical study.

Concentrated human milk (HM-concentrate) can be obtained from the simple and inexpensive method of donated breast milk direct lyophilization. A previous study reported that HM-concentrate contains the adequate amount of main macro- and micronutrients for use as a nutritional resource for preterm infants with very low birth weight admitted to neonatal intensive care units. However, further details need to be elucidated about HM-concentrate composition, particularly its content of essential and potentially toxic trace elements.

DNA damage is inversely associated to blood levels of DHA and EPA fatty acids in Brazilian children and adolescents.

This study aimed to investigate the association between DNA damage and blood levels of docosahexaenoic acid (DHA), eicosapentaenoic acid (EPA), retinol, beta-carotene and riboflavin in Brazilian children and adolescents. Subjects (n = 140) were healthy boys and girls aged 9 to 13 years in Ribeirão Preto (SP, Brazil). Data collection included anthropometry, assessment of energy intake and blood sampling.

The Role of Rehabilitation Specialists in Canadian NICUs: A 21st Century Perspective.

Unlabelled: Rehabilitation specialists are an integral part of the team in the neonatal intensive care unit (NICU). New approaches to rehabilitation practice in the NICU have evolved over the past decade that aim to promote child health and development.

Aims: The aim of this study was to describe the current roles of the occupational therapist (OT), physical therapist (PT) and speech-language pathologist (SLP) in Canadian NICUs as compared to the roles documented in an earlier national survey conducted 15 years ago.

Human milk enriched with human milk lyophilisate for feeding very low birth weight preterm infants: A preclinical experimental study focusing on fatty acid profile.

Background: Human milk, with essential nutrients and long chain polyunsaturated fatty acids (LC-PUFAs) such as the omega 3 and 6 fatty acids is important for development of the central nervous system and the retina in very low birth weight infants (<1,500 g). However, breast milk may not be sufficient to meet these needs. The possibility of supplementing breast milk with a lyophilisate of human milk was explored in this study.

Gestational, perinatal, and postnatal factors that interfere with practice of exclusive breastfeeding by six months after birth.

Background: Despite evidences indicating the superiority of breastfeeding and recent advances in the indicators of breastfeeding in Brazil, exclusive breastfeeding (EBF) during the first six months after birth continues to be an infrequent practice in the country. The objective of the present study was to determine which gestational, perinatal, and postnatal factors of the mother-baby dyad might be associated with the cessation of EBF by six months after birth.

Methods: Data were collected at the rooming-in facility of the Reference Center of Women's Health of Ribeirão Preto-Mater (CRSM-Mater) during the postpartum period (24 to 72 h after birth) from December 2012 to April 2013 and by telephone contact between the researcher and participating mothers by six months after birth.

Living related versus deceased donor liver transplantation for maple syrup urine disease.

Maple syrup urine disease (MSUD) is an inherited disorder of branched chain ketoacid (BCKA) oxidation associated with episodic and chronic brain disease. Transplantation of liver from an unrelated deceased donor restores 9-13% whole-body BCKA oxidation capacity and stabilizes MSUD. Recent reports document encouraging short-term outcomes for MSUD patients who received a liver segment from mutation heterozygous living related donors (LRDT).

Bioelectrical impedance vector analysis in obese women before and after bariatric surgery: changes in body composition.

Objective: Because of the inefficacy of standard methods for the evaluation of body composition of grade III obese individuals, it is difficult to analyze the quality of weight loss after bariatric surgery in these patients. Electrical bioimpedance vector analysis and the RXc graph uses crude resistance (R) and reactance (Xc) values, like components of the Z vector, to monitor variations in body fluid and the nutritional status of obese individuals. Using bioelectrical impedance vector analysis (BIVA) and the RXc graph, the objective of the present study was to evaluate long-term changes in weight and body composition of obese women after Roux-en-Y bariatric surgery.

Bioelectrical impedance vector analysis (BIVA) in stable preterm newborns.

Objective: To observe the behavior of the plotted vectors on the RXc (R - resistance - and Xc - reactance corrected for body height/length) graph through bioelectrical impedance analysis (BIVA) and phase angle (PA) values in stable premature infants, considering the hypothesis that preterm infants present vector behavior on BIVA suggestive of less total body water and soft tissues, compared to reference data for term infants.

Methods: Cross-sectional study, including preterm neonates of both genders, in-patients admitted to an intermediate care unit at a tertiary care hospital. Data on delivery, diet and bioelectrical impedance (800 mA, 50 kHz) were collected.

Deuterium equilibrium time in saliva of newborn infants.

Unlabelled: There is an increasing interest about the use of stable isotopes for body composition analysis in pediatrics. To ensure the success of total body water analysis by the deuterium dilution method, it is fundamental to determine the equilibrium time (plateau) of deuterium in the body fluid studied.

Objectives: We report here the equilibration time of deuterium oxide in the saliva of newborns after oral intake of the isotope.

The role of angiotensin II receptor-1 blockade in the hypoxic pulmonary vasoconstriction response in newborn piglets.

Background: Angiotensin-converting enzyme activity is increased in newborn infants with respiratory distress syndrome and in animals with alveolar hypoxia.

Objective: To test whether angiotensin II (Ang II) mediates the pulmonary vasoconstriction induced by acute hypoxia in newborn piglets.

Methods: Eight unanesthetized chronically instrumented newborn piglets (mean +/- SEM; age 6.

Assessment of vitamin A nutritional status in newborn preterm infants.

Objective: This study assessed the vitamin A nutritional status of preterm infants determined by the vitamin A relative dose-response test (RDR) compared with serum levels of vitamin A, retinol-binding protein (RBP), transthyretin (TTR), and retinol relations with carrier proteins.

Methods: Serum levels of retinol, RBP, and TTR and retinol/RBP, retinol/TTR, and RBP/TTR molar ratios were determined in 120 infants at 7 d and in 92 at 28 d. For the determination of the performance of the tests, the RDR was considered the reference method.

[Nutritional dilemmas in extremely low birth weight infants and their effects on childhood, adolescence and adulthood].

Objective: To review the recent medical literature on nutrition of extremely low birth weight infants, focusing on nutritional disorders and their effects on childhood, adolescence and adulthood.

Sources Of Data: An extensive review of the related literature was performed using MEDLINE, the Cochrane Database of Systematic Reviews and the Best Evidence database.

Summary Of The Findings: There is a growing body of evidence that early nutritional practices may affect short-term growth and development outcome.