Vitamin d deficiency with high parathyroid hormone levels is related to late onset SEPSIS among preterm infants.

Preterm infants (PTs) are at greater risk for vitamin D deficiency, which relates to the possibility of a higher incidence of comorbidities. Our goal was twofold, 1) to monitor vitamin D, calcium, phosphorus, parathyroid hormone (PTH), matrix metalloproteinase-8 (MMP-8) serum levels at three-time points during hospitalization, and 2) to assess the association between 25-hydroxyvitamin D (25OHD) levels and outcomes in PTs. METHODS: We carried out a follow-up on 50 Caucasian PTs ≤ 32 weeks of gestational age (GA) and/or ≤ 1500 g birth weight at 28 days and at 4 months. PTs were divided into two subgroups for tests of association with clinical outcomes based on vitamin D deficient infants 25(OH) D cord blood levels: ≤ 20 ng/ml). At an initial stage, 25(OH) D levels were determined in maternal/preterm blood samples and were compared to full term delivery infants. RESULTS: There were no differences in 25(OH) D serum levels at birth when comparing PTs to term infants, or regarding maternal levels. A strong positive correlation was detected between maternal and neonatal 25(OH) D serum levels among PTs and term infants (r: 0.466; p < 0.001). Neonates with vitamin D deficiency did not present a higher incidence of comorbidities. PTs were classified in two subgroups based on vitamin D and PTH (group 1: vitamin D < 20 ng/mL and PTH > 60 pg/mL; group 2: vitamin D > 20 and PTH < 60 pg/mL). The PTs in group 1 showed a higher incidence of LOS (RR: 2; 95% CI: 1.31-3.55). No relationship was observed between MMP-8 serum levels and the incidence of sepsis. CONCLUSIONS: This study did not find any evidence of an increase in preterm birth risk related to vitamin D level at birth. Vitamin D deficiency by itself is not associated with a higher incidence of comorbidities. However, the binomial vitamin D-PTH must be taken into consideration.

Comorbidities in childhood associated with extrauterine growth restriction in preterm infants: a scoping review.

Extrauterine growth restriction (EUGR) is a frequent morbidity of preterm infants that can affect short- and long-term prognosis as it involves different EUGR-related alterations in growth and neurological development, as well as cardiometabolic risk. However, knowledge about the prognosis of EUGR is scarce. Thus, the objective of this study is to review the evidence regarding EUGR-related comorbidities in childhood by a systematic approach. This review was carried out using the Joanna Briggs Institute Reviewers' Manual Methodology and the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analyses)-Search Extension for scoping review. The MEDLINE and EMBASE databases were used to identify papers published until September 2017. Twenty-four publications were included and 19 examined cohort studies. EUGR is mainly associated with (1) lower weight, length, and head circumference measures in childhood; (2) poor neurodevelopment; and (3) alterations in cardiometabolic risk markers. The definition for EUGR and the populations studied differ among authors.Conclusion: EUGR is mainly associated with poor growth and neurodevelopment, as well as with cardiometabolic alterations in childhood. Evidence is based on observational studies with variability in the included populations due to the lack of consensus regarding the definition for EUGR. Finding a gold standard definition becomes paramount in order to select phenotypes at risk later in life. What is known? • EUGR is a frequent condition of preterm infants. Up to date little is known about the effect of the metabolic programming on prognosis. What is new? • The available evidence, which is based on observational studies with variability in the population and the existing different definitions for EUGR, do not enable appropriate data collection. EUGR is mainly associated with poor growth and neurodevelopment, as well as with cardiometabolic alterations in childhood.

Metabolic profiling in children with autism spectrum disorder with and without mental regression: preliminary results from a cross-sectional case-control study.

INTRODUCTION: It is challenging to establish the mechanisms involved in the variety of well-defined clinical phenotypes in autism spectrum disorder (ASD) and the pathways involved in their pathogeneses. OBJECTIVES: The aim of the present study was to evaluate the metabolomic profiles of children with ASD subclassified by mental regression (AR) phenotype and with no regression (ANR). METHODS: The present study was a cross-sectional case-control study. Thirty children aged 2-6 years with ASD were included: 15 with ANR and 15 with AR. In addition, a control group of 30 normally developing children was selected and matched to the ASD group by sex and age. Plasma samples were analyzed with a metabolomics single platform methodology based on liquid chromatography-mass spectrometry. Univariate and multivariate analysis, including orthogonal partial least squares-discriminant analysis modeling and Shared-and-Unique-Structures plots, were performed using MetaboAnalyst 4.0 and SIMCA-P 15. The primary endpoint was the metabolic signature profiling among healthy children and autistic children and their subgroups. RESULTS: Metabolomic profiles of 30 healthy children, 15 ANR and 15 AR were compared. Several differences between healthy children and children with ASD were detected, involving mainly amino acid, lipid and nicotinamide metabolism. Furthermore, we report subtle differences between the ANR and AR groups. CONCLUSIONS: In this study, we report, for the first time, the plasmatic metabolomic profiles of children with ASD, including two different phenotypes based on mental regression status. The use of a liquid chromatography-mass spectrometry platform approach for metabolomics in ASD children using plasma appears to be very efficient and adds further support to previous findings in urine. Furthermore, the present study documents several changes related to amino acid, NAD+ and lipid metabolism that, in some cases, such as arginine and glutamate pathway alterations, seem to be associated with the AR phenotype. Further targeted analyses are needed in a larger cohort to validate the results presented herein.

Analysis of the local and systemic inflammatory response in hospitalized infants with respiratory syncitial virus bronchiolitis.

BACKGROUND: Respiratory syncytial virus acute bronchiolitis (RSV-AB) is a major cause of hospital admission among our infants. The immune and inflammatory mechanisms involved in the RSV-AB and factors influencing severity have not been clearly established, although an imbalanced Th1 and Th2 response seems to be crucial. OBJECTIVES: To assess the local and systemic inflammatory response in RSV-AB. To find a possible marker of clinical severity and/or oxygen requirements. PATIENTS AND METHODS: Levels of nine cytokines were measured in nasopharyngeal aspirate (NPA) and peripheral blood (PB) of 45 infants with RSV-AB and 27 peer controls, including IFNγ, TNFα, VEGF, interleukins 4, 6 and 10, and chemokines (IL-8 and macrophage inflammatory proteins 1-α and 1-ß). RESULTS: The levels of the analyzed cytokines and chemokines were significantly higher in the NPA of RSV-AB group, with a decrease in IL-4/IFNγ ratio. IL-6 and MIP-1ß levels in NPA were directly correlated to oxygen therapy. PB showed an increase in IL-8 and a decrease in MIP-1α and MIP-1ß in the RSV-AB group (only MIP-1ß associated to the need for oxygen therapy). No correlation was found between cytokines and chemokines levels in NPA and PB. CONCLUSIONS: This study shows that RSV triggers an inflammatory response fundamentally at the respiratory level, with scant systemic repercussion. This local response is characterized by an increase in Th1 and Th2 cytokines, although with a relative predominance of Th1. The determination upon patient admission of IL-6 and MIP-1ß levels in NPA, and of MIP-1ß in PB could help predict severe forms and the need for oxygenotherapy.

Alterations in the antioxidant defense system in prepubertal children with a history of extrauterine growth restriction.

PURPOSE: The role of oxidative stress is well known in the pathogenesis of acquired malnutrition. Intrauterine growth restriction has been associated with an imbalance in oxidative stress/antioxidant system. Therefore, early postnatal environment and, consequently, extrauterine growth restriction might be associated with alterations in the antioxidant defense system, even in the prepubertal stage. METHODS: This is a descriptive, analytical, and observational case-control study. The study included two groups; 38 Caucasian prepubertal children born prematurely and with a history of extrauterine growth restriction as the case group, and 123 gender- and age-matched controls. Plasma exogenous antioxidant (retinol, ß-carotene, and α-tocopherol) concentrations were measured by HPLC; antioxidant enzyme activities of catalase, glutathione reductase, glutathione peroxidase, and superoxide dismutase were determined in lysed erythrocytes by spectrophotometric techniques. RESULTS: Catalase and glutathione peroxidase concentrations were significantly lower in extrauterine growth restriction children than in controls (P < 0.001). Lower plasma retinol concentrations were found in the case group (P = 0.029), while concentrations of ß-carotene and α-tocopherol were higher (P < 0.001) in extrauterine growth restriction prepubertal children as compared with controls. After correction by gestational age, birth weight, and length, statistically significant differences were also found, except for retinol. CONCLUSIONS: Prepubertal children with a history of extrauterine growth restriction present alterations in their antioxidant defense system. Knowing these alterations may be important in establishing pharmacological and nutritional treatments as this situation might be associated with higher metabolic disorders in adulthood.

Effects of parenteral nutrition formulas on plasma lipid profile in children with bone marrow transplantation.

BACKGROUND/AIMS: Children undergoing bone marrow transplantation (BMT) often require parenteral nutrition (PN). This is a comparative study of plasma lipid profiles in BMT children after fish oil or soybean PN. METHODS: A total of 14 children with BMT requiring PN for at least 10 days were recruited during 24 months. They were randomized to receive a lipid emulsion enriched with ω3 polyunsaturated fatty acid, or soybean oil. Clinical monitoring was performed. Blood samples were collected before and after administration of PN to analyze the lipid profile. RESULTS: There were no complications associated with PN. The increase in TG levels was more pronounced after administration of an enriched ω3 emulsion and the decrease in cHDL and apo A was greater after administration of soybean. The ω3 group showed an increase in eicosapentaenoic and a decrease in arachidonic acids compared to the soybean group. Both groups showed similar levels of linolenic acid. CONCLUSION: PN with soybean or ω3 emulsions for 10 days is safe in children. However, research in children are necessary in order to examine the impact of the duration of PN and the type of lipid formula used, and determine their health benefits in relation to the fatty acid profile.

A pleural ultrasound image of a collapsed lung surrounded by pleural fluid ("jellyfish sign") may correspond to an intrapericardial mass.

Lung ultrasound has been shown to be a valuable diagnostic tool. It has become the main way to get to the diagnosis of pleural effusion with much more specificity and sensibility than the x-ray. The diagnosis of pleural effusion with ultrasound is easily obtained after the visualization of hypoechoic fluid surrounding the lung. Sometimes it appears as an image of a collapsed lung moving with the surrounded pleural fluid ("jellyfish sign"). Until now this sign was almost pathognomonic of pleural effusion, but we explore a case in which this sign could have led to a misleading diagnosis. We present the case of a child admitted to intensive care with respiratory distress. In the point of care lung ultrasound we believed to see a pleural effusion with a collapsed lung moving into the effusion. Due to the enlargement of the pericardial sac, we did not realize that what we thought to be the pleural space was in fact the pericardial space. Unfortunately, there was a more echogenic area inside the pericardial effusion which led to a misleading fake lung atelectasis with pleural effusion ("jellyfish sign"). The correct diagnosis was properly obtained after assessing a cardiac point of care ultrasound using a four chambers view. The left side of the thorax is more difficult to be sonographed than the right due to the presence of the heart fossa that occupies a significant part of that side. Obtaining the diagnosis of pleural effusion on that side is more difficult for this reason and can sometimes be misleading with a pericardial effusion. The presence of the "jellyfish sign" is not pathognomonic and may lead to an error if we are guided only by the presence of that sign. To avoid such a misleading diagnosis, we highly recommend performing a point of care cardiac ultrasound if a pleural effusion is primarily seen in the lung ultrasound.

Cardiorespiratory fitness and oxidative stress: effect of acute maximal aerobic exercise in children and adolescents.

AIM: Evaluate the effects of oxidative stress in saliva in young males, according to their cardiorespiratory fitness and taking acute maximal aerobic exercise into consideration. An incremental exercise test (20 meter shuttle run) was used. METHODS: Seventy healthy male subjects, aged 10 to 14 years, were included in the study and were classified into two groups according to fitness parameters. Subjects were expected to take the 20 meter shuttle run test. RESULTS: Group I had high cardiorespiratory fitness while group II had low cardiorespiratory fitness below the mean for their age. Saliva samples were taken before and immediately after exercise in order to measure levels of reduced glutathione, lipoperoxides, glutathione/lipoperoxides ratio and catalase. The values of reduced glutathione were significantly diminished regardless the subjects' cardiorespiratory fitness. The glutathione/lipoperoxides ratio was significantly diminished in group I. In addition, positive correlations were observed between lipoperoxides values after the 20 meter shuttle run test. CONCLUSION: High cardiorespiratory fitness does not seem to be an essential factor effecting in the oxidative stress values before exercise. However, oxidative stress could be greater with more intensity and duration after and acute maximal physical exercise.

Experience in neuromuscular diseases in children and adolescents and their comorbidities in a tertiary hospital.

INTRODUCTION: Neuromuscular diseases include a large group of heterogeneous and rare pathologies that affect different components of the motor unit. It is essential to optimize resources to know the prevalence of comorbidities in the most frequent groups to establish an early multidisciplinary approach in a specialized setting. PATIENTS AND METHODS: Retrospective descriptive study of pediatric and adolescent patients with neuromuscular diseases (NMDs). The Inclusion criteria were NMDs patients with motor neuron involvement divided into three groups, depending on the affected component of the motor unit. Group I: involvement of the motor neuron; Group II: peripheral neuropathies; Group III: myopathies. Demographic variables, association with comorbidities, need for respiratory support, and rehabilitative treatment were collected in each group. RESULTS: Ninety-six patients who met the inclusion criteria were studied. In group I, when compared to the other two groups, a higher incidence of scoliosis (68.3%, p = 0.011), deformity of the rib cage (31.3%, p = 0.0001), chronic respiratory insufficiency (62.5%, p = 0.001) and bronchial aspiration (12.5%, p = 0.03) was detected. In this group, 50%of the patients required non-invasive mechanical ventilation (p = 0.0001). The in-hospital requirement for respiratory physiotherapy was higher in group I (75%, p = 0.001). We observed a higher incidence of scoliosis in Group III compared to Group II. CONCLUSIONS: Neuromuscular diseases with motor neuron involvement present more comorbidities and require an early approach after diagnosis to improve prognosis.

[Changes in oxidative stress biomarkers induced by puberty]. / Cambios en biomarcadores del estrés oxidativo inducidos por la pubertad.

AIM: Evaluate the influence of puberty in oxidative stress. SUBJECTS AND METHODS: The study included 38 prepubescent males with Tanner G(1)-P(1) and 32 healthy pubescent boys with Tanner G(3-4)-P(3-4). Weight, height and body mass index, heart rate, blood pressure values were within the 50 percentile 50+/-1SD for their age. The biomarkers were measured in saliva, as a good correlation between saliva and plasma levels has been reported in lipoperoxidation products, reduced glutathione and catalase. RESULTS: Pubescent boys had significantly higher levels of lipoperoxidation products (P<0.001) compared with the prepubertal group, with no significant differences in the other parameters measured. There was a significant positive correlation between lipoperoxides and reduced glutathione in these children. CONCLUSION: It is the first time that an increase of lipoperoxidation products has been reported in pubertal boys and this biomarker could play a role in the development of oxidative stress in this stage of life.

[Infection-triggered familial or recurrent acute necrotizing encephalopathy]. / Encefalopatía aguda necrosante familiar o recurrente desencadenada por infecciones.

Acute necrotizing encephalopathy (ANE) presents in children after common viral infections. Most cases of ANE are non-familial and non-recurrent and have been mainly reported in Asian patients, although ANE affects children worldwide. Recently, missense mutations in the gene encoding the nuclear pore protein Ran Binding Protein 2 (RANBP2) have been found in several families with familial or recurrent cases of ANE. We describe a Spanish family with familial and recurrent ANE without mutations in RANBP2. Mutations in RANBP2 are not the sole susceptibility alleles for familial or recurrent ANE.

[Fatal poisoning by accidental inhalation of aluminium phosphide]. / Intoxicación letal por inhalación accidental de fosfuro alumínico.

The cases of two 6 and 9-year-old sisters with lethal poisoning by inhalation of aluminium phosphide, after its inadequate use in a rural environment, are described. The clinical symptoms consisted of sudden vomiting, cardiac arrhythmias, shock, dyspnea, pulmonary edema/acute respiratory distress, metabolic acidosis and hepatic dysfunction, and the patients died in spite of advanced life support. Although an early diagnosis might theoretically improve the poisoning outcome, its high lethality rate and the absence of a specific antidote, efforts must be directed towards prevention and restricting its use as pesticide and being aware of its toxicity.

Evaluation of the vasoactive-inotropic score, mid-regional pro-adrenomedullin and cardiac troponin I as predictors of low cardiac output syndrome in children after congenital heart disease surgery.

OBJECTIVE: To determine the predictive value of the inotropic score (IS) and vasoactive-inotropic score (VIS) in low cardiac output syndrome (LCOS) in children after congenital heart disease surgery involving cardiopulmonary bypass (CPB), and to establish whether mid-regional pro-adrenomedullin (MR-proADM) and cardiac troponin I (cTn-I), associated to the IS and VIS scores, increases the predictive capacity in LCOS. DESIGN: A prospective observational study was carried out. SETTING: A Paediatric Intensive Care Unit. PATIENTS: A total of 117children with congenital heart disease underwent CPB. Patients were divided into two groups: LCOS and non-LCOS. INTERVENTIONS: The clinical and analytical data were recorded at 2, 12, 24 and 48h post-CPB. Logistic regression was used to develop a risk prediction model using LCOS as dependent variable. MAIN OUTCOME MEASURES: LCOS, IS, VIS, MR-proADM, cTn-I, age, sex, CPB time, PIM-2, Aristotle score. RESULTS: While statistical significance was not recorded for IS in the multivariate analysis, VIS was seen to be independently associated to LCOS. On the other hand, VIS>15.5 at 2h post-CPB, adjusted for age and CPB timepoints, showed high specificity (92.87%; 95%CI: 86.75-98.96) and increased negative predictive value (75.59%, 95%CI: 71.1-88.08) for the diagnosis of LCOS at 48h post-CPB. The predictive power for LCOS did not increase when VIS was combined with cTn-I >14ng/ml at 2h and MR-proADM >1.5nmol/l at 24h post-CPB. CONCLUSIONS: The VIS score at 2h post-CPB was identified as an independent early predictor of LCOS. This predictive value was not increased when associated with LCOS cardiac biomarkers. The VIS score was more useful than IS post-CPB in making early therapeutic decisions in clinical practice post-CPB.

[Infant botulism]. / Botulismo del lactante.

We report a case of botulism in a 40 day old infant. The patient presented a descending flaccid paralysis requiring mechanical ventilation for 12 days. She is the first European patient treated with Human Botulism Immune Globulin. A few weeks later a second case of infant botulism was detected in our geographical region in Southern Spain. We emphasise the importance of an early diagnosis and treatment with Human Botulism Immune Globulin to decrease morbidity.

[Artificial nutrition in pediatric intensive care units]. / Nutrición artificial en las unidades de cuidados intensivos pediátricos.

OBJECTIVE: To perform an epidemiologic study of artificial nutrition in critically-ill pediatric patients. PATIENTS AND METHODS: A multicenter, prospective and descriptive study was conducted in 23 Spanish intensive care units (ICU) (18 pediatric ICUs and five pediatric/neonatal ICUs) over a 1-month period. Artificial nutrition (AN) was required by 165 critically-ill patients (21.4 %). Data on diagnosis, severity, treatment, type of nutrition administered and complications were analyzed. RESULTS: A total of 54.4 % of the participants were younger than 1 year, 19.4 % were aged between 1 and 5 years old, 15.7 % between 5 and 10 years old and 13.4 % were older than 10 years. ICU mean length stay was 11 days. One hundred six patients were administered enteral nutrition (EN): 67.9 % continuous nasogastric EN, 27.4 intermittent nasogastric EN, 16 % nasojejunal EN, 2.8 % gastrostomy EN. Eighty patients required parenteral nutrition (PN): 86.3 % central PN, 20 % peripheral PN. No significant differences were found between patients with EN and PN in mean energy intake, days receiving AN, diagnosis at admission to the ICU, disease severity (measured by PRISM III) or intensive support techniques. The EN group required greater inotropic support. Patients undergoing mechanical ventilation had equal mortality independent of the type of AN. The most common complications in EN were: 17.9 % emesis, 13.2 % abdominal distension, 11.3 % diarrhea, 4.7 % gastric residual volumes, and 6.6 % hypokalemia. In PN complications consisted of: 5 % catheter related infection, 1.3 % thrombophlebitis, 7.5 % hyponatremia, 3.8 % hypoglycemia, 6.3 % hypophosphatemia and 3.8 % hypertriglyceridemia. CONCLUSIONS: EN provides critically-ill children with adequate energy intake and is well tolerated. Therefore, if there are no contraindications, EN should be the system of choice in the critically-ill patient requiring AN.

[Möbius syndrome and an apparently life-threatening event]. / Síndrome de Möbius y episodio aparentemente letal.

Möbius syndrome is characterized by congenital facial weakness with impairment of ocular abduction. A subgroup of these patients have associated apneas because of involvement of brainstem respiratory centers located slightly lateral to the abducens nuclei. We report a 7-month old infant admitted to the pediatric intensive care unit because of an episode of cyanosis, hypotonia and unresponsiveness. The patient then became respirator dependent afterwards. On examination, facial diplegia, impairment of ocular abduction and hypotonia were evident. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in brainstem tegmentum. At the age of 11 months he was discharged but required a home ventilator. He died 5 months later due to an infection. Möbius syndrome is associated with central respiratory dysfunction. The finding of abnormal signal intensity in brainstem tegmentum on MRI is a possible predictor of apnea in these patients.

Role of culture of postoperative drainage fluid in the prediction of infection of the surgical site after major oncological operations of the head and neck.

Infection of the surgical site after major oncological operations of the head and neck increases mortality and morbidity. The aim of this prospective pilot study was to assess the efficacy of culturing the exudate from the drain after cervical neck dissection to see if it predicted such infection. We studied 40/112 patients with squamous cell cancer of the head and neck who were treated during the last two years and met our inclusion criteria. Six patients developed infections (15%). Reconstruction with pedicled rather than local or microvascular flaps, duration of operation of over 7 hours, the presence of a tracheostomy, and bilateral neck dissection were considered risk factors (p=0.01). Culture of drainage fluid on postoperative day 3 that grew no pathogens predicted that the site would not become infected, with a negative predictive value of 96%.

[Aortic valve replacement with pulmonary autograft (the Ross procedure) in adult and pediatric patients]. / Sustitución valvular aórtica con autoinjerto pulmonar (operación de Ross) en pacientes adultos y pediátricos.

Aortic valve replacement with pulmonary autograft was first performed by Donald Ross in 1967. Initially, the procedure was not widely accepted, by Cardiologists and Cardiac surgeons fundamentally due to its complexity and demanding surgical technique, and because innumerous series two cardiac valves were at risk. The results published in the last 10-15 years established the pulmonary autograft as one of the best methods of aortic valve replacement, especially in pediatric patients and young adults. In the present article, we reviewed present indications and contraindications, and our clinical experience with 26 patients (pediatrics and adults). Analysis of the first 22 the patients with a minimum of 6 months of follow-up (180-620 days) was performed. Follow-up is complete (100%). Mean age was 31.4 +/- 12.6 years. Five patients were pediatrics (<= 14 years). Three patients (11%) with previous percutaneous procedures and 4 patients (14%) with previous surgical procedures. There was no early or late mortality. In the last follow-up, 19 of 22 (86.36%) had no autograft insufficiency (>= grade 1), and in one patient it was moderate (grade 2). The 2 remaining patients developed severe autograft insufficiency (grade 4) and were reoperated on, with satisfactory postoperative outcome. Mean maximal gradient was 7.85 +/- 5 mmHg at 18 months (3-29). Patients with preoperative aortic stenosis showed a significant reduction in myocardial mass index (208.7 +/- 32 a 95.8 +/- 28.8 g/m2) at 18 months. In these patients, septal and posterior wall thickness decreased significantly, in the first month. Two pediatric patients have developed transpulmonar gradient > 50 mmHg. One of them underwent successful stent implantation. We have not observed significant homograft insufficiency in any of our patients. All our patients remain asymptomatic (functional class I) without medical treatment. We have not observed either thromboembolic or haemorrhagic episodes, nor endocarditis. No patient is receiving anticoagulants. Clinical and echocardiographic mid term results in pulmonary autograft and homograft in our series, are excellent after the Ross procedure.

[Fatal outcome after severe cerebellar infarction due to spontaneous dissection of the vertebral artery]. / Evolución fatal tras infarto cerebeloso masivo por disección espontánea de arteria vertebral.

Cerebellar infarction due to vertebrobasilar ischemia in spontaneous vertebrobasilar dissection is infrequent in children and adolescents. The commonest pathogenic mechanism of cerebellar infarction is arterial occlusion. Although usually favorable, outcome can be fatal, as in the present case. A previously healthy 4-year-old girl was admitted to the emergency room with sensory failure and trembling. The previous week she had suffered from a transitory ischemic attack that resolved spontaneously. On admission to the emergency department, the Glasgow Coma Scale score was 10/15 and she presented irregular polypneic breathing, anisocoric reactive pupils, enhanced reflexes, lower limb clonus and bilateral pyramidal signs. Cranial computed tomography revealed marked hypodensity in both cerebellar hemispheres and white supratentorial substance with obliteration of the quadrigeminus cisterns. Cerebral angiography showed left vertebral artery dissection with distal pseudoaneurysm and marked basilar occlusion. Despite aggressive antiedemic treatment and early instauration of intravenous continuous sodium heparin infusion, the patient developed a bilateral cerebellar infarction and supratentorial hydrocephalus. Brain death was diagnosed after 48 hours. Postmortem pathologic examination revealed previous thrombus the in superior left cerebellar artery and basilar artery occlusion. We discuss the absence of predisposing factors and the transitory ischemic attack in this patient 1 week before she developed massive cerebellar infarction.