Extremely low birthweight neonates with phenylketonuria require special dietary management.

Aim: Extremely low birthweight (ELBW) neonates require a high protein intake, but this can be challenging in the very rare cases when they also have phenylketonuria (PKU). This is due to a lack of suitable parenteral nutrition or enteral formula. Our aim was to analyse tolerance to phenylalanine in these infants.

Paediatric Home Parenteral Nutrition in the Czech Republic and Its Development: Multicentre Retrospective Study 1995-2011.

Background: Treatment quality and outcomes of paediatric home parenteral nutrition (HPN) program during its development in the Czech Republic.

Methods: A retrospective study of patients receiving HPN from May 1995 till June 2011.

Results: Sixty-six patients were treated in 8 centres.

Post-mortem magnetic resonance imaging and its irreplaceable role in determining CNS malformation (hydranencephaly)--case report.

Post-mortem magnetic resonance appears to be a method supplementary to classic pathological-anatomical autopsy in determining foetal abnormalities. Frequently, it plays a key role, primarily where autopsy options are in some way limited (developed autolysis, dilatation of the ventricular system). This case report demonstrates that post-mortem magnetic resonance imaging can precisely determine the type of congenital malformation (hydranencephaly), by contrast to ultrasound, with which alobar holoprosencephaly has been described, often presenting a differential diagnosis problem.

High-frequency jet ventilation improves gas exchange in extremely immature infants with evolving chronic lung disease.

Extremely preterm infants often develop chronic lung disease (CLD) characterized by heterogeneous aeration; poorly supported, floppy airways; and air trapping. High-frequency jet ventilation (HFJV) with high end-expiratory pressure (optimal lung volume strategy [OLVS]) may improve airway patency, lead to better gas distribution, improve gas exchange, and facilitate extubation. In a pilot trial, this study sought to explore the effect of HFJV on oxygenation, ventilation, and ease of extubation in preterm infants with evolving CLD and refractory respiratory failure (RRF).

Induction of uncoupling protein 3 gene expression in skeletal muscle of preterm newborns.

Prematurity is associated with delayed postnatal activation of mitochondrial oxidative phosphorylation and impaired switch from glycolytic to oxidative metabolism. Fatty acids (FA), which represent a major energy substrate in mature muscle cells, are engaged in the postnatal activation of genes of energy metabolism and lipid oxidation. To understand the mechanism activating mitochondria in human newborns, expression of the genes for mitochondrial uncoupling proteins (UCP) was characterized in autopsy samples of skeletal (n = 28) and cardiac (n = 13) muscles of preterm neonates, who mostly died during the first postnatal month, and two aborted fetuses.

Early versus delayed surfactant administration in extremely premature neonates with respiratory distress syndrome ventilated by high-frequency oscillatory ventilation.

Objective: To determine whether early surfactant administration is superior to selective delayed treatment in terms of improving survival and/or reducing chronic lung disease in extremely premature neonates with respiratory distress syndrome (RDS) treated by high-frequency oscillatory ventilation (HFOV).

Design: Prospective randomized clinical trial.

Setting: Tertiary neonatal intensive care unit (NICU) in the Perinatology Center of Prague.

Fetal anemia, thrombocytopenia, dilated umbilical vein, and cardiomegaly due to a voluminous placental chorioangioma. A case report.

We report a case of voluminous placental chorioangioma diagnosed by ultrasound and color Doppler imaging during the 20th week of pregnancy. The size of the tumor was enlarging progressively (up to 10 cm in the 32nd week), and during this time the signs of fetal intrauterine volume overload and blood cell consumption, such as cardiomegaly, umbilical vein dilation, hydramnios, anemia, and thrombocytopenia, were observed. In the 32nd week of pregnancy, the signs of uteroplacental insufficiency and fetal hypoxia appeared; therefore, the pregnancy was terminated by cesarean section, and a female infant weighing 1,870 g was delivered.

[Effect of combined administration of TRH and dexamethasone in pregnant women and the course of their pregnancy].

Unlabelled: Extremely immature neonates are threatened during the first days after delivery by many conditions which are due to incomplete development.--A key role is played during the first days of extrauterine life by the incidence and degree of the respiratory distress syndrome (RDS). Its incidence in neonates born before the completed 32nd week of gestation is very common.

A prospective randomized comparison of conventional mechanical ventilation and very early high frequency oscillatory ventilation in extremely premature newborns with respiratory distress syndrome.

Objective: To compare the effectiveness and safety of very early high-frequency oscillatory ventilation (HFOV) with conventional mechanical ventilation (CMV) in treatment of the respiratory distress syndrome (RDS) and to evaluate their impact on the incidence of chronic pulmonary disease and early and late morbidity of very low-birthweight neonates.

Design: A prospective randomized clinical trial.

Setting: Tertiary neonatal intensive care unit in the Perinatology Center in Prague.

[An approach to the differential diagnosis of dilatation of the caliceal-pelvic system in children].

The authors submit suggestions for the differential diagnostic procedure in dilatation of the calico-pelvic system in children involving consecutive application of non-invasive methods to methods pretentious for the patient. In the second part they present a review of recent findings regarding visualization techniques of the hollow renal system.