Management of late onset urea cycle disorders-a remaining challenge for the intensivist?

BACKGROUND: Hyperammonemia caused by a disorder of the urea cycle is a rare cause of metabolic encephalopathy that may be underdiagnosed by the adult intensivists because of its rarity. Urea cycle disorders are autosomal recessive diseases except for ornithine transcarbamylase deficiency (OTCD) that is X-linked. Optimal treatment is crucial to improve prognosis. Main body We systematically reviewed cases reported in the literature on hyperammonemia in adulthood. We used the US National Library of Medicine Pubmed search engine since 2009. The two main causes are ornithine transcarbamylase deficiency followed by type II citrullinemia. Diagnosis by the intensivist remains very challenging therefore delaying treatment and putting patients at risk of fatal cerebral edema. Treatment consists in adapted nutrition, scavenging agents and dialysis. As adults are more susceptible to hyperammonemia, emergent hemodialysis is mandatory before referral to a reference center if ammonia levels are above 200 µmol/l as the risk of cerebral edema is then above 55%. Definitive therapy in urea cycle abnormalities is liver transplantation. CONCLUSION: Awareness of urea cycle disorders in adults intensive care units can optimize early management and accordingly dramatically improve prognosis. By preventing hyperammonemia to induce brain edema and herniation leading to death.

Potassium disorders in pediatric emergency department: Clinical spectrum and management.

INTRODUCTION: Potassium abnormalities are frequent in intensive care but their incidence in the emergency department is unknown. AIM: We describe the spectrum of potassium abnormalities in our tertiary-level pediatric emergency department. METHODS: Retrospective case-control study of all the patients admitted to a single-center tertiary emergency department over a 2.5-year period. We compared patients with hypokalemia (<3.0mEq/L) and patients with hyperkalemia (>6.0mEq/L) against a normal randomized population recruited on a 3:1 ratio with potassium levels between 3.5 and 5mEq/L. RESULTS: Between January 1, 2013 and August 31, 2016 we admitted 108,209 patients to our emergency department. A total of 9342 blood samples were tested and the following potassium measurements were found: 60 cases of hypokalemia (2.8±0.2mEq/L) and 55 cases of hyperkalemia (6.4±0.6mEq/L). In total, 200 patients with normokalemia were recruited (4.1±0.3mEq/L). The main causes of the disorders were non-specific: lower respiratory tract infection (23%) and fracture (15%) for hypokalemia, lower respiratory tract (21.8%) and ear-nose-throat infections (20.0%) for hyperkalemia. Patients with hyperkalemia had an elevated creatinine level (0.72±1.6 vs. 0.40±0.16mg/dL, P<0.0001) with lower bicarbonate (19.4±3.8 vs. 21.8±2.8mmol/L, P=0.0001) and higher phosphorus levels (1.95±0.6 vs. 1.42±0.27mg/dL, P=0.0001). Patients with hypokalemia had an elevated creatinine level (0.66±0.71 vs. 0.40±0.16mg/dL, P<0.0001) and a lower phosphorus level (1.12±0.31 vs. 1.42±0.27mg/dL, P=0.0001). We did not observe significant differences in pH, PCO2, base excess and lactate, or in the mean duration of hospitalization in general wards and pediatric intensive care units according to the PIM and PRISM scores. DISCUSSION: Dyskalemia is rare in emergency department patients: 0.64% for hypokalemia and 0.58% for hyperkalemia. This condition could be explained by a degree of renal failure due to transient volume disturbance. The main mechanism is dehydration due to digestive losses, polypnea in young patients, and poor intake. In the case of hypokalemia, poor intake and digestive losses could be the main explanation. These disorders resolve easily with feeding or perfusion and do not impair development. CONCLUSION: Dyskalemia is rare in emergency department patients and is easily resolved with feeding or perfusion. A plausible etiological mechanism is a transient volume disturbance. Dyskalemia is not predictive of poor development in the emergency pediatric population.

[Infants poisoned with cannabis]. / L'intoxication au cannabis chez le nourrisson.

We reported three cases of infants poisoned with cannabis. These patients presented with acute neurological disorders such as drowsiness accompanied by hypotonia, mydriasis and seizure. Cannabis was found in all children either in the urine or in the blood. These cases illustrated that young age should not exclude toxicologic analysis in acute neurological disorders. Cannabis poisoning in infants is a rare reason for consultation. Clinical signs and symptoms are unspecific and severe manifesta- tions in pediatric age are not well known by emergency physicians and paediatricians.

Nous rapportons trois cas de nourrissons intoxiqués au cannabis. Ces patients s'étaient présentés avec des troubles aigus du comportement de type somnolence accompagnés d'hypotonie, de mydriase et de convulsions pour l'un d'eux. Du cannabis était présent soit dans les urines soit dans le sang dans les trois cas. Ces cas illustrent que le jeune âge ne doit pas exclure la recherche de toxiques dans les mises au point de troubles neurologiques aigus. L'intoxication au cannabis du nourrisson est un motif rare de consultation. La clinique est aspécifique et les manifestations sévères plus fréquentes à l'âge pédiatrique sont peu connues.

[When a multiple trauma complains about abdominal pains six weeks after the fall. . ]. / Quand un polytrauma se plaint de douleurs abdominales six semaines après la chute. . .

A 18-year old patient presents abdominal pains associated with nausea and vomiting six weeks after a multiple trauma leading to paraplegia. Esophagitis, urolithiasis, and acalculous cholecystitis were diagnosed. This report illustrates two rare abdominal complications of a multiple trauma with immobilisation: acalculous cholecystitis, hypercalcaemia, coralliform urolithiasis following urinary infection and bacteriurie with Enterobacter Cloacae. The interpretation of the abdominal semiology of a multiple trauma's patient presenting long-term immobilization may be misleading.