Central catheter removal timing and growth patterns in preterm infants.

BACKGROUND: Early discontinuation of total parenteral nutrition (TPN) at 100 ml/kg/day of enteral feeds, compared with 140 ml/kg/day, led to significant delay in time to regain the birth weight in very low birth weight infants (birth weight < 1500 g, VLBW). Our aim was to compare the growth of infants in relation with timing of TPN discontinuation up to 2 years corrected gestational age (CGA). METHODS: Posttrial follow-up study using review of paper medical records. Participants of the randomized controlled trial studying effect of early parenteral nutrition discontinuation on time to regain birth weight in VLBW infants were included. Growth parameters inclusive of weight, length, and occipital-frontal circumference (OFC) were collected. Z-scores were calculated at five predefined time points-birth, 0-11 weeks CGA, 12-35 weeks CGA, 36-60 weeks CGA, and 61-96 weeks CGA and compared for control and intervention groups. RESULTS: Regarding weight, we found lower mean Z-score in the intervention group between 0 and 11 weeks CGA, with larger difference in extremely low birth weight infants (birth weight < 1000 g, ELBW), but this did not reach the statistical significance. Regarding length, the same difference, slightly delayed to 35 weeks CGA was observed and reached statistical significance for ELBW infants between 12 and 35 weeks CGA. There was no difference in OFC mean Z-scores at any timepoint. CONCLUSIONS: The discontinuation of TPN at 100 ml/kg/day showed significantly lower Z-score for length in ELBW infants between 12 and 35 weeks CGA. There were no differences in Z-scores by 2 years CGA.

An international survey of the nutrition management of chylothorax: a time for change.

INTRODUCTION: Although chylothorax is an uncommon complication following paediatric cardiothoracic surgery, it has significant associated morbidities and increased in-hospital mortality, as well as results in higher costs. A lack of prospective evidence or consensus guidelines for management of chylothorax further hinders optimal management. The aim of this survey was to characterise variations in practice in the management of chylothorax and to identify areas for future research. MATERIALS AND METHODS: A descriptive, observational survey investigating conservative management practices of chylothorax was distributed internationally to health-care professionals in paediatric intensive care and cardiology units. The survey investigated five domains: the first providing general information about health-care professionals and four domains focusing on clinical practice including diet composition and duration. RESULTS: In total, sixty-four health-care professionals completed the survey, representing 38 organisations from 16 countries. The respondents were dietitians (80%), physicians (19%), and nurses (1%). In Australia and New Zealand, management was most commonly directed by physicians' preference (67%) as compared to unit protocols in Europe (67%), United States of America (67%), and Other regions (55%). Dietitians in Australia/New Zealand, United Kingdom, and Ireland followed the most restrictive diet therapy recommending <5 g long chain triglyceride fat per day (p < 0.00001). The duration of diet therapy significantly varied between regions: Australia/New Zealand: 4 weeks (36%) and 6 weeks (43%); Europe: 4 weeks (25%) and 6 weeks (57%); and North America: 4 weeks (18%) and 6 weeks (75%) (p < 0.00001). CONCLUSIONS: This survey highlights international variations in practice in the management of chylothorax, particularly with respect to treatment duration and dietary fat restriction. Future research should include a multi-centre randomised controlled trial to inform evidence-based practice and reduce morbidity, particularly poor growth.

The relationship between early pregnancy dietary intakes and subsequent birthweight and neonatal adiposity.

Background: Maternal nutrition intakes may influence neonatal birthweight and adiposity; however, inconsistencies within the literature exist. The relationships between maternal dietary intakes in early pregnancy and both birthweight and neonatal adiposity requires elucidation. This study examined the relationship between early pregnancy dietary intakes and subsequent birthweight and neonatal adiposity. Methods: Women were recruited at their convenience after sonographic confirmation of a singleton pregnancy. Women completed a Willet food frequency questionnaire evaluating habitual food and nutrient intakes at their first antenatal visit. Neonatal body composition was measured using air-displacement plethysmography. Results: Of the 385 mother-neonate dyads, mean maternal age was 30.8 ± 5.3 years, mean Body Mass Index (BMI) was 24.5 ± 4.8 kg/m2 and 41.8% (n = 161) were nulliparous. There were no relationships between maternal food intakes and birthweight (P > 0.05) (n = 385). On multivariable analysis there was a positive relationship between polyunsaturated fat and neonatal fat mass index (FMI) (beta = 0.015, 95% CI = 0.002-0.028, P = 0.04) (n = 80). Conclusion: Dietary intakes of polyunsaturated fat in early pregnancy are positively associated with neonatal FMI at birth on multivariable analysis. Further longitudinal studies need to explore this association and the long-term implications for the neonate.

Preferences of Jehovah's Witnesses regarding haematological supports in an obstetric setting: experience of a single university teaching hospital.

BACKGROUND: Jehovah's Witnesses have been shown to be at increased risk of mortality and morbidity as a consequence of obstetric haemorrhage and refusal of blood products. Since 2004, however, Jehovah's Witnesses have been allowed to accept minor fractions of blood at their own discretion. We sought to determine the preferences of pregnant Jehovah's Witnesses regarding haematological supports since this policy change. METHODS: This is a retrospective observational study of consecutive Jehovah's Witnesses attending a university-affiliated tertiary referral centre between 1 January 2007 and 31 December 2013. The main outcome measure was the proportion of women who would be willing to accept blood products and other haematological supports in the event of life-threatening bleeding, should it occur. RESULTS: Seventy-six Jehovah's Witnesses attended for obstetric care during the study period. Major fractions of blood (red cells, plasma or platelets) were acceptable to 7.9% and 50% would accept some minor fractions. Some blood components were acceptable to 70.3% of nulliparous women compared to 48.9% of multiparous women. In women with advance directives some blood components were acceptable to 70.5% compared with 37.5% of those without. Recombinant factor VIIa was acceptable to 53.9%. Black African women had the lowest acceptance of any ethnic group of any blood products. CONCLUSION: The spectrum of acceptance of blood products is wide ranging within our obstetric Jehovah's Witnesses population. Recombinant factors are not universally acceptable despite their identification as non-blood products. A multidisciplinary approach with individualized consent is recommended.

Compound and double mutations in patients with hypertrophic cardiomyopathy: implications for genetic testing and counselling.

OBJECTIVE: To report the frequency of single and multiple gene mutations in an Australian cohort of patients with hypertrophic cardiomyopathy (HCM). METHODS: Genetic screening of seven HCM genes (beta-MHC, MyBP-C, cTnT, cTnI, ACTC, MYL2, and MYL3) was undertaken in 80 unrelated probands. Screening was by denaturing high performance liquid chromatography and direct DNA sequencing. Clinical data were collected on all patients and on genotyped family members. RESULTS: 26 mutations were identified in 23 families (29%). Nineteen probands (24%) had single mutations (11 beta-MHC, 4 MyBP-C, 3 cTnI, 1 cTnT). Multiple gene mutations were identified in four probands (5%): one had a double mutation and the others had compound mutations. Six of 14 affected individuals from multiple mutation families (43%) experienced a sudden cardiac death event, compared with 10 of 55 affected members (18%) from single mutation families (p = 0.05). There was an increase in septal wall thickness in patients with compound mutations (mean (SD): 30.7 (3.1) v 24.4 (7.4) mm; p<0.05). CONCLUSIONS: Multiple gene mutations occurring in HCM families may result in a more severe clinical phenotype because of a "double dose" effect. This highlights the importance of screening the entire panel of HCM genes even after a single mutation has been identified.