The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
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J. Matern. Fetal. Neonatal. Med. · Oct 2010
ReviewTreatment of organic acidurias and urea cycle disorders.
Three steps are extremely important in emergency management (awaiting diagnosis) of organic acidurias and urea cycle disorders. The first is to obtain adequate samples (blood and urine) before any treatment, parenteral and/or enteral nutrition with protein-free solution and toxin removal procedures are the second step. Additional therapies with cofactors and vitamins are the last step. It is very important to quickly carry out this strategy every time we suspect an inborn error of metabolism.
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Shock defines a complex dysfunction of organ perfusion, that produces a status of cellular energy failure, resulting from an insufficient supply of oxygen and nutrients to tissues. The diagnosis of shock is very difficult because of the lack of sufficiently sensitive and specific clinical criteria, and is substantially based on the demonstration of an arterial hypotension, an indicator unfit to detect the organ hypoperfusion. It determines the necessity of firmly introducing in the diagnostic run the functional echocardiography, the near infrared spectroscopy and the amplitude – integrated electroencephalography, etc., in the monitoring of the critical newborn. In order to simplify the problem, the authors identify the clinical scenarios of the newborn's shock to enhance the different pathogenetic moments and to build up appropriate therapeutic algorithms, without forgetting that at present there is no evidence that treatment of shock improves outcomes, despite the large amount of the studies conducted on this topic.
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J. Matern. Fetal. Neonatal. Med. · Oct 2010
ReviewCongenital cytomegalovirus infection: treatment, sequelae and follow-up.
Cytomegalovirus (CMV) is the most common cause of congenital infection affecting about 1% of all the live births worldwide. Its prevalence in the developed world seems to be slightly lower, ranging between 0.6 and 0.7%. Symptoms can be detected at birth in 10-15% of the congenitally infected of which 50-90% will develop sequelae, the most frequent being sensorineural hearing loss (SNHL), visual defect, psychomotor impairment, mental retardation, cerebral palsy and seizures. ⋯ Infants born with symptomatic congenital infection have a worse prognosis than those with no evidence of clinical disease, and ganciclovir (GCV) intravenous 6 mg/kg every 12 h for 6 weeks is the most used therapy for symptomatic newborns. Valganciclovir (V-GCV) syrup is a pro-drug of GCV and presents high oral bioavailability. To date, it is possible to administer this drug at home, and the tolerability profile may allow for wider indications and longer treatments.