The lower PUFA supply with the olive/soybean oil emulsion appears to enhance linoleic acid conversion. The reduced PUFA content, combined with a higher antioxidant intake in the olive oil group, results in an improved vitamin E status. The olive oil-based emulsion is a valuable alternative for parenteral feeding of preterm infants who are often exposed to oxidative stress, while their antioxidative defense is weak.

ZusammenfassungHintergrund: Pankreas-Agenesie ist eine seltene Ursache des neonatalen Diabetes mellitus. Pankreas-Agenesie wurde in einzelnen Fallberichten beschrieben, und Informationen zur Klinik und Prognose sind spärlich. Wir berichten über einen Patienten mit Pankreas-Agenesie und double outlet right ventricle, eine bisher nicht beschriebene Assoziation. Zusätzlich geben wir einen Überblick über insgesamt 14 Patienten mit Pankreas-Agenesie, die bisher berichtet wurden. Methode: Wir berichten über einen Patienten und fanden 13 weitere Patienten durch Medlineund OMIM-Recherche. Ergebnisse: Bei einem Kind wurde die Schwangerschaft in der 19. Woche abgebrochen. 31 % (4/13) der Kindern starben in der ersten Woche und 69 % (9/13) in den ersten sechs Lebenswochen, 17 % (2/12) waren Frühgeborene, 83 % (10/12) Reifgeborene, 93 % (13/14) hatten eine schwere intrauterine Wachstumsretardierung, Manifestation des Diabetes mellitus war bei 60 % (6/10) während der ersten zwei Lebenstage, Ketonurie ist selten und wurde nur einmal berichtet. 64 % (9/14) der Kinder mit Pankreas-Agenesie hatten zusätzliche Fehlbildungen, überwiegend des Gallengangssystems (50 %) und/oder des Herzens (36 %), 31 % (4/13) überlebten die Neonatalperiode und zeigten eine gute Entwicklung. Nach Substitution der Pankreasenzyme kam es zum Aufholwachstum. Schlussfolgerung: Pankreas-Agenesie ist eine klinische Entität, charakterisiert durch schwere intrauterine Wachstumsretardierung, frühe Manifestation eines persistierenden Diabetes mellitus ohne Ketoazidose, Gedeihstörung infolge exokriner Pankreasinsuffizienz und assoziierter Fehlbildungen, vorwiegend des Gallengangssystems Abstract Background: Pancreatic agenesis is a rare cause of neonatal diabetes mellitus and the knowledge about the clinical features is sparse. A patient with pancreatic agenesis and double outlet right ventricle is reported. This association has not previously been reported. In addition a synopsis of the patients (n = 14) with pancreatic agenesis who have hitherto been described is given. Method: We studied one patient and obtained information on 13 additional patients with pancreatic agenesis by reviewing literature. Results: Literature review: In one patient the pregnancy was terminated at 19 weeks. 31 % (4/13) of the infants died in the first week and 69 % (9/13) in the first six weeks of live, 17 % (2/12) were born preterm and 83 % (10/12) at term, 93 % (13/ 14) had severe intrauterine growth restriction, onset of diabetes was in 6 out of 10 infants during the first two days of live, ketonuria is rare and has been reported only once. 64 % (9/14) of the infants with pancreatic agenesis had additional malformations mainly of the biliary system (50 %) and/or the heart (36 %). 31 % (4/13) of the infants survived the neonatal period and developed normally. Failure to thrive was compensated by catch-up growth after replacement of pancreatic enzymes and surgical correction of the cardiac malformation. Conclusions: Pancreatic agenesis is a clinical entity characterized by severe intr...

Objective: As 80% of intrauterine bone mineralization takes place during the last trimester of pregnancy, preterm infants should be supplemented postnatally with optimal doses of calcium, phosphate and vitamin D. Calcium and phosphate excretion in the urine may be used to monitor individual mineral requirements, but are sometimes difficult to interpret. The objective of this study was to assess the value of quantitative ultrasound (QUS) for the analysis of bone status in neonates.Study Design: All admissions to three independent tertiary neonatal intensive care units were studied. In 172 preterm and term infants with a gestational age between 23 and 42 weeks (mean 33.8 ± 5.0) and a birth weight from 405 to 5130 g (mean 2132±1091 g) bone status was evaluated prospectively by quantitative ultrasound velocity using a standardized protocol. Infants were followed in regular intervals up to their first discharge home. While measurements were conducted in weekly intervals initially (n ¼ 55), 2-week intervals were regarded as sufficient thereafter due to limited changes in QUS values within the shorter period. Infants with a birth weight below 1500 g were followed during outpatient visits until up to 17 months of age.Result: The intra-individual day-to-day reproducibility was 0.62%. QUSvalues from the first week of life correlated significantly with gestational age and birth weight (r ¼ 0.5 and r ¼ 0.6; P<0.001). Small-forgestational-age infants showed lower values for QUS than appropriate-forgestational-age infants allowing for their gestational age. Follow-up measurements correlated positively with age and weight during the week of measurement (r ¼ 0.2 and r ¼ 0.4; P ¼ 0.001). Comparing bone quality at 40 weeks of age in infants born at term versus infants born at 24 to 28 weeks, preterm infants showed significantly lower QUS than term infants (P<.0001).There was a significant correlation of QUS with serum alkaline phosphatase (P ¼ 0.003), the supplementation with calcium, phosphate and vitamin D (P< 0.001 each), as well as risk factors for a reduced bone mineralization. No correlation was found between QUS and calcium or phosphate concentration in serum or urine.Conclusion: QUS is a highly reproducible, easily applicable and radiation-free technique that can be used to monitor bone quality in individual newborns. Further prospective randomized-trials are necessary to evaluate, if therapeutic interventions based on QUS are able to prevent osteopenia of prematurity.

Additional metformin therapy in T1DM is primarily used in obese females. Additional therapy with metformin was associated with minor benefits.

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