Demographic profile and outcome analysis of pediatric intensive care patients.

BACKGROUND: Demographic profile and outcome can vary in pediatric intensive care unit (PICU) patients. The aim of our study was to analyze demographic profile and outcome in a Greek PICU. METHODS: Prospective observational study. DATA COLLECTED: demographic profile; co morbidities; source and diagnosis at admission; Pediatric Risk of Mortality (PRISM III-24); Glasgow Coma Scale (GCS, pediatric); Injury Severity Score (ISS); procedures; treatment; mechanical ventilation (MV); MV days; length of stay (LOS) and the outcome at PICU discharge. STATISTICAL ANALYSIS: Student's t-test; Mann-Whitney U test; Kruskall-Wallis test; χ(2) criterion with relative risk (RR) estimation; Cox regression analysis; as appropriate. Values are mean ± SD, p < 0.05. RESULTS: 300 patients (196 boys/104 girls), aged 54.26 ± 49.93 months, were admitted due to respiratory failure (22.3%), head trauma (15.3%), seizures (13.7%), coma (9.7%), postoperative care (7.7%), polytrauma (7%), accidents (5.3%), sepsis-septic shock (5.3%), cardiovascular diseases (4.7%), metabolic diseases (3.3%), multiple organ failure syndrome (3%) and miscellaneous diseases (2.7%). PRISM III-24 score was 8.97 ± 7.79 and predicted mortality rate was 11.16% ± 18.65. MV rate was 67.3% (58.3% at admission) for 6.54 ± 14.45 days, LOS 8.85 ± 23.28 days and actual PICU mortality rate 9.7%. Patients who died had statistically worse severity scores. Significant mortality risk factors were inotropic use, PRISM III-24 > 8, MV, arterial and central venous catheterization, nosocomial infections, complications, and cancer. COX regression analysis showed that PRISM III-24 score and inotropic use were independent predictors of mortality. CONCLUSIONS: Demographic profile followed similar patterns to relevant studies while there were major differences in case mix and the severity of the disease. Mortality rate (9.7%) was relatively high but better than predicted and in accordance with the characteristics of our population.

Ethylmalonic encephalopathy: clinical and biochemical observations.

Ethylmalonic encephalopathy (EE) is a rare, recently defined inborn error of metabolism which affects the brain, gastrointestinal system and peripheral blood vessels and is characterized by a unique constellation of clinical and biochemical features. A 7-month-old male, who presented with psychomotor retardation, chronic diarrhea and relapsing petechiae is described with the objective of highlighting the biochemical and neuroradiological features of this disorder as well as the effect of high-dose riboflavin therapy. Urinary organic acid analysis revealed markedly increased excretion of ethylmalonic acid, isobutyrylglycine, 2-methylbutyrylglycine and isovalerylglycine. Acylcarnitine analysis in dried blood spots showed increased butyrylcarnitine. Short-chain acyl-CoA dehydrogenase (SCAD) activity in muscle was normal as were mitochondrial OXPHOS enzyme activities in cultured skin fibroblasts. In skeletal muscle the catalytic activity of complex II was decreased. Brain MRI revealed bilateral and symmetrical atrophy in the fronto-temporal areas, massive enlargement of the subarachnoid spaces and hyperdensities on T (2) sequences of the basal ganglia. Mutation analysis of the ETHE1 gene demonstrated homozygosity for the Arg163Gly mutation, confirming the diagnosis of EE at a molecular level. On repeat MRI, a significant deterioration was seen, correlating well with the clinical deterioration of the patient.

Prevention of complications related to traumatic brain injury in children and adolescents with creatine administration: an open label randomized pilot study.

BACKGROUND: There has been an enormous focus on the discovery and development of neuroprotective agents that might have clinical relevance after traumatic brain injury (TBI). Based on experimental facts, we studied administration of creatine to patients with TBI. METHODS: A prospective, randomized, comparative, open-labeled pilot study of the possible neuroprotective effect of creatine was performed on 39 children and adolescents, aged between 1 to 18 years old, with TBI. The creatine was administered for 6 months, at a dose of 0.4 gr/kg in an oral suspension form every day. For categorical variables, we used the chi test to identify differences between controls and cases. Statistical significance was defined as a p value <0.05 and not statistically significant if p value >0.1. RESULTS: The administration of creatine to children with TBI improved results in several parameters, including duration of post-traumatic amnesia (PTA), duration of intubation, intensive care unit (ICU) stay, disability, good recovery, self care, communication, locomotion, sociability, personality/behavior and neurophysical, and cognitive function. Significant improvement was recorded in the categories of Cognitive (p < 0.001), personality/behavior (p < 0.001), Self Care (p = 0.029), and communication (p = 0.018) aspects in all patients. No side effects were seen because of creatine administration. CONCLUSION: Preliminary data suggest that the administration of creatine may be beneficial to patients with traumatic brain injury.

Lamotrigine childhood overdose.

A 2-year-old male developed generalized tonic-clonic seizure activity, tremor of limbs, muscle weakness, ataxia, and hypertonia after he swallowed 16 50-mg tablets of lamotrigine. His vital signs were normal, as were electroencephalography and laboratory investigation tests. The urine toxicologic screen revealed no other drugs. Treatment included midazolam and gastric lavage followed by activated charcoal and fluid loads. Symptoms resolved within 24 hours, and the child was discharged without any further complications. Serial blood samples revealed plasma lamotrigine levels at the high adult therapeutic range (3.8 mg/L) but a slow elimination rate. This is the first report of seizure activity reported in a patient receiving an overdose of lamotrigine. However, no evident concentration-effect-side-effect relationship has been established in children. Interestingly in this child, lamotrigine overdose presented exclusively with treatment-emergent neurologic abnormalities, sparing all other systems.