[Siblings in a pediatric intensive care unit]. / L'accueil des fratries dans un service de réanimation pédiatrique.

Welcoming visitors has long had difficulty finding its place in intensive care units, including pediatric units. This article describes the reflective approach adopted by a working group in the pediatric intensive care unit at the regional university hospital in Tours, with the aim of fully integrating the reception of siblings into the child's hospitalized care project. The momentum generated by this reflection has gradually enabled the family environment to be restructured around the child in care, reinvigorating a breath of life that extends far beyond the purely biological.

Intracranial Pressure Monitoring Demonstrates that Cerebral Edema Is Not Correlated to Hyperammonemia in a Child with Ornithine Transcarbamylase Deficiency.

BACKGROUND: Ornithine transcarbamylase deficiency (OTCD) is an inborn error of urea cycle resulting in increased plasma levels of ammonia and glutamine and cerebral edema. However, the underlying mechanism of brain cytotoxicity remains controversial. Our objective is to present an unusual acute hyperammonemic crisis suggesting a key role of brain glutamine to mediate ammonia neurotoxicity and the interest of intracerebral pressure (ICP) monitoring to maintain adequate cerebral perfusion pressure and to prevent neurological damages. PATIENT: A 6-year-old boy with OTCD was admitted for an acute hyperammonemic encephalopathy following viral infection. At admission, he presented vomiting, confusion, lethargy (Glasgow scale 7/15), and bilateral papilledema, suggesting cerebral edema. Plasma ammonia level was slightly increased (194 µmol/L, rr 25-50 µmol/L), contrasting with the severity of neurological deterioration and with high levels of glutamine in plasma (1,949 µmol/L, rr 335-666 µmol/L) and the brain (10-fold increase on in vivo MR spectroscopy). The patient was placed on neuroprotective treatments and respiratory support. MAIN RESULTS: With a hypercaloric protein-free diet and nitrogen scavenger drugs, plasma levels of ammonia and glutamine rapidly decreased without neurological improvement. Continuous ICP monitoring showed repetitive peaks of pressure up to 60 mmHg in the first four days and was helpful to manage neuroprotective treatments. After several days, the patient progressively recovered without cognitive or motor disability. CONCLUSION: This case report highlights the discrepancy between the severity of neurological impairment, presumably related to high level of brain glutamine, and plasma levels of ammonia or glutamine in a child with acute hyperammonemic encephalopathy related to OTCD. In this situation, continuous ICP monitoring was helpful to manage neuroprotective treatments and prevent brain damages.

Establishing a hypothermia service for infants with suspected hypoxic-ischemic encephalopathy.

The translation of new treatments based upon established evidence into clinical practice is often difficult. The establishment of a therapeutic hypothermia (TH) service and a related cooling register would provide the opportunity to examine how a new therapy becomes implemented in a country or region. The objectives of a TH program should be: to provide guidance to clinicians who are considering the introduction of this new therapy; to ensure standardized clinical practices; to audit the implementation and conduct of TH; to provide surveillance for cooling-related adverse effects; and to evaluate the subsequent neurodevelopmental outcome. Prior to the use of TH, the most important practices to prioritize during its implementation should be identified and include the following: ensure timely identification of infants with neonatal encephalopathy; develop a coordinated system with the local or regional referral cooling center; develop a transport team capable of performing cooling during transport; ensure that each participating unit has access to a national encephalopathy register, and have developmental follow-up arrangements in place that are appropriate and uniform for the region/country.