Ruptured Intracranial Aneurysm in a 60-Day-Old Infant: An Extreme Case.

The prevalence of aneurysms in children is low when compared to adults, being even rarer in the first year of life. They can be secondary to infections, traumatic brain injury, autoimmune diseases, or connective tissue diseases. Dissecting etiology is rare. A 60-day-old female infant, previously healthy, presented to the emergency department (ED) with irritability and loss of appetite since the preceding day, a fever of one-hour duration, and vomiting. Laboratory analysis revealed a hemoglobin level of 6.5 g/dL, without elevation of inflammatory markers. In the ED, she experienced two episodes, with a one-hour interval, of clonic movements of the upper eyelid and right upper limb, along with conjugate gaze deviation to the same side, which resolved after intravenous diazepam. Levetiracetam was initiated after the second episode. The anterior fontanelle became progressively tense. Brain computed tomography (CT) showed a voluminous intraparenchymal and subarachnoid hemorrhage with an aneurysm at the bifurcation of the left middle cerebral artery (MCA). Initially, an endovascular approach was tried but was not successful due to technical problems. Consequently, a Vaso-CT scan was performed that confirmed a dissecting aneurysm/pseudoaneurysm (8 mm × 10 mm × 10 mm) of the left MCA, originating from the upper wall of the M1 segment. Next, she underwent microsurgical exclusion of the aneurysm using microclips. Post-surgery brain CT showed acute ischemia in the entire MCA region. Follow-up angiography showed complete exclusion of the aneurysm. She evolved to grade 3 monoparesis of the upper limb at the six-month interval follow-up, which has been gradually improving with physical rehabilitation. The next-generation sequencing (NGS) panel for aneurysms and arterial dissections did not detect any pathogenic variants. Clinical presentation of cerebral aneurysms in infants can be subtle, and a high index of suspicion is required in cases of irritability, altered consciousness, seizures, bulging fontanelle, and motor deficits. Early detection is of utmost importance as it is associated with moderate mortality. Surgical treatment with the use of clips proved to be effective in this case.

Acute Encephalopathy in a 10-Year-Old Patient With Maple Syrup Urine Disease: A Challenging Diagnosis.

Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder characterized by a deficiency in the branched-chain alpha-keto acid dehydrogenase complex, leading to the toxic accumulation of leucine, isoleucine and valine. Acute encephalopathy (AE) is a severe neurological disorder with diverse etiologies, demanding prompt identification and intervention. We present a unique case of a previously healthy teenage patient who developed AE during an influenza infection. Despite initial inconclusive investigations, the patient's condition rapidly deteriorated, requiring pediatric intensive care unit (PICU) admission. Diagnostic challenges included fluctuating mental status and refractory intracranial hypertension, ultimately necessitating decompressive craniectomy. Empirical treatments, including corticosteroids, tocilizumab, and plasmapheresis, were administered. Finally, clinical exome analysis revealed a pathogenic variant in homozygosity in the BCKDHA gene associated with MSUD type Ia. Her adult sister, experiencing similar symptoms in the same time period, did not survive. This case underscores the importance of considering metabolic disorders in AE etiology, even accounting for its various associated syndromes and usual prolonged diagnostic investigation, as prompt treatment initiation is vital for improved outcomes. Management of AE involves addressing seizures, systemic support and neuromonitoring, namely, intracranial pressure monitoring. Inborn errors of metabolism, like MSUD, should be considered, even if universally screened, as delayed diagnosis can result in prolonged hospitalization and significant morbidity.

Methotrexate-Induced Stroke-Like Syndrome: A Typical Presentation of a Rare Complication.

Methotrexate (MTX) is one of the mainstay drugs used in acute lymphoblastic leukemia (ALL) management; however, it can cause damage to the central nervous system (CNS), typically to the subcortical white matter. Stroke-like syndrome is one particular form of MTX-related neurotoxicity that occurs within 21 days of methotrexate administration (intrathecal or high-dose intravenous treatment). The clinical picture comprehends fluctuating neurological symptoms evoking acute cerebral ischemia or hemorrhage (paresis or paralysis, speech disorders - aphasia and/or dysarthria, altered mental status, and occasionally seizures), with spontaneous resolution in the majority of cases, without other identifiable cause. The typical neuroimage includes areas of restricted diffusion on diffusion-weighted imaging and non-enhancing T2 hyper-intense lesions in the white matter, on brain MRI. We report a 12-year-old boy with low-risk B-ALL without CNS involvement, who presented to the emergency department with complaints of sudden paresis of the four limbs (more severe on the right side), aphasia, and confusion. He had received one dose of intrathecal MTX 11 days prior to this episode. An angio-MRI of the brain revealed bilateral restricted diffusion areas in the centrum semiovale, and symptoms fluctuated until complete neurological recovery without any medical intervention, which is very suggestive of MTX-related neurotoxicity. This case illustrates a rare complication of MTX administration that presented with typical clinical and radiological characteristics, in an adolescent with hematological malignancy who experienced swift and full neurological recovery.

Neuroimaging of central nervous system tuberculosis.

A 20-month-old female, not immunized with Bacillus Calmette-Guérin (BCG) vaccine, was admitted due to a four-day history of fever and cough. In the past three months, she presented respiratory infections, weight loss and enlarged cervical lymph nodes. On day two of admission, she displayed drowsiness and positive Romberg's sign; cerebrospinal fluid (CSF) workout revealed 107/ul cells, low glucose and high protein levels. Ceftriaxone and acyclovir were initiated, and she was transferred to our tertiary hospital. Brain magnetic resonance imaging showed punctiform focal areas of restricted diffusion in left capsular lenticular region suggestive of vasculitis secondary to infection. Tuberculin skin test and interferon-gamma release assay were positive. She started tuberculostatic therapy, but two days later she presented tonic-clonic seizures and impaired consciousness. Cerebral computed tomography (CT) revealed tetrahydrocephalus (Figure 1), needing external ventricular derivation. She had a slow clinical improvement, requiring several neurosurgical interventions and developing a syndrome of inappropriate antidiuretic secretion alternating with cerebral salt wasting. Positive results for Mycobacterium tuberculosis were obtained by CSF culture and by polymerase chain reaction in CSF, bronchoalveolar lavage and gastric aspirate specimens. Repeated brain CT showed a large-vessel vasculitis with basal meningeal enhancement, typical of central nervous system (CNS) tuberculosis (Figure 2). She completed one month of corticosteroids and maintained antituberculosis treatment. At two years of age, she has spastic paraparesis and no language skills. Portugal had 1836 cases of tuberculosis (17.8 per 100000) in 2016 and was considered a low-incidence country; consequently, BCG vaccination is not universal (1). We present a severe case of CNS tuberculosis with intracranial hypertension, vasculitis and hyponatremia, associated with poorer outcomes (2). A high index of suspicion allowed prompt start of antituberculosis treatment. Diagnosis was corroborated by microbiological positivity and a typical triad in neuroimaging (hydrocephalus, vasculitis and basal meningeal enhancement) (3), which we wish to emphasize.

Challenging Diagnosis of a Congenital Tracheal Malformation: Considerations From an Intensive Care Perspective.

Congenital tracheal stenosis is a rare airway malformation. A high index of suspicion is fundamental. The authors report a case of congenital tracheal stenosis in a 13-month-old male infant, with a challenging diagnosis from the intensive care perspective. At birth, the patient presented an anorectal malformation with a recto-urethral fistula so a colostomy with mucous fistula was performed in the neonatal period. At the age of seven months, he was admitted due to a respiratory infection, treated with steroids and bronchodilators, and discharged after three days without any complications. He underwent complete repair of tetralogy of Fallot when he was 11 months old, which was performed without any reported perioperative complications. However, at the age of 13 months, due to another respiratory infection, he presented more severe symptoms and required admission to the pediatric intensive care unit (PICU) for invasive mechanical ventilation. He was intubated on the first attempt. While monitoring the difference between peak inspiratory and plateau pressures, we observed a sustained elevated difference between pressures suggestive of increased airway resistance, thus raising the possibility of an anatomical obstruction. Laryngotracheoscopy confirmed distal tracheal stenosis (grade II) with four complete tracheal rings. In our case, the absences of perioperative challenges or complications in previous respiratory infections were not suggestive of a tracheal malformation. Furthermore, no difficulties were encountered during intubation due to the distal location of the tracheal stenosis. A careful appreciation of respiratory mechanics on the ventilator at rest and during tracheal aspirations was essential to suspect an anatomical defect.

Correlating blast vibrations and geomechanical properties to determine damage profiles and improve wall conditions in open pit mining.

Slope stability is one of the biggest concerns for mining practices and to consider the rock mass response over blasting is fundamental to achieve pit geometry. This study consists in developing a methodology which connects the dynamic behavior of one lithological domain to blast designs applied at a copper mine. The central element of this study was the construction of vibration attenuation and seed wave model which, in conjunction with geomechanical properties, has allowed the characterization of this particular rock mass and the vibration attenuation phenomena. The new blast design was developed from the model simulations, once it was possible to recognize which parameters of the blast design affect most of the damage induced by blasting. To guarantee model representativeness, two blast tests were conducted: one with the usual blast design and another using the new one. Furthermore, holes were drilled behind the blasts, which were inspected before and after each blast to compare the produced fracturing with the fracturing expected by the model. The results obtained in these blast tests show a strong correlation between the modeled and the real. The modeling proved to be a useful tool providing manners to stablish a blast design, which generates stable walls.

ALCAPA syndrome: A rare etiology of cardiac arrest in a teenager.

ALCAPA syndrome is a rare congenital heart disease and a cause of myocardial ischemia in pediatric population. The authors present the case of a 10-year-old girl admitted to the emergency room after experiencing cardiac arrest at school. In the echocardiogram, the inability to identify the origin of the left coronary artery raised the hypothesis of abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA). A CT-scan and a cardiac catheterization were performed confirming the diagnosis. Infants with the syndrome may have myocardial infarction and congestive heart failure. She successfully underwent cardiac surgery. Most undiagnosed patients die within the first year of life, and it is necessary to develop an extensive network of collaterals to ensure survival. ALCAPA syndrome rarely manifests in late childhood, teenagers, and adults and may be an important cause of sudden cardiac arrest.

Assessment of the renal angina index for the prediction of acute kidney injury in patients admitted to a European pediatric intensive care unit.

BACKGROUND: Acute kidney injury (AKI) is associated with worse outcomes and increased morbidity and mortality in pediatric intensive care unit (PICU) patients. The renal angina index (RAI) has been proposed as an early prediction tool for AKI development. OBJECTIVES: The objective was to evaluate outcomes of RAI-positive patients and to compare RAI performance with traditional AKI markers across different patient groups (medical/post-surgical). This was an observational retrospective study. All children admitted to a tertiary hospital PICU over a 3-year period were included. Electronic medical records were reviewed. Day 1 RAI was calculated, as was the presence and staging of day 3 AKI. RESULTS: A total of 593 patients were included; 56% were male, the mean age was 55 months, and 17% had a positive RAI. This was associated with day 3 AKI development and worse outcomes, such as greater need for kidney replacement therapy, longer duration of mechanical ventilation, vasoactive support and PICU stay, and higher mortality. For all-stage kidney injury, RAI presented a sensitivity of 87.5% and a specificity of 88.1%. Prediction of day 3 all-stage AKI by RAI had an AUC=0.878; its performance increased for severe AKI (AUC = 0.93). RAI was superior to serum creatinine increase and KDIGO AKI staging on day 1 in predicting severe AKI development. The performance remained high irrespective of the type of admission. CONCLUSIONS: The RAI is a simple and inexpensive tool that can be used with medical and post-surgical PICU patients to predict AKI development and anticipate complications, allowing for the adoption of preventive measures.

Pulmonary embolism in pediatric age: A retrospective study from a tertiary center.

INTRODUCTION: Pediatric pulmonary embolism (PE) is rare but associated with adverse outcomes. We aimed to characterize PE cases admitted in a tertiary hospital and to evaluate sensitivity of selected PE diagnostic prediction tools. METHODS: Retrospective, descriptive study of PE cases admitted from 2008 to 2020 using data collected from hospital records. Patients were grouped according to PE severity and setting (outpatients vs. inpatients). Links and correlation with demographic characteristics, risk factors, clinical presentation, management, and outcomes were analyzed. PE diagnostic prediction tools were applied. RESULTS: Twenty-nine PE episodes occurred in 27 patients, 62.9% female, mean age 14.1 years. Most PE were central and split between massive or submassive. One was diagnosed in autopsy. Twenty outpatients, all adolescents, were admitted for classic PE symptoms; in half of them the diagnosis had been previously missed. Risk factors included contraceptives (65%), thrombophilia (35%), obesity (20%) and auto-immunity (20%). Eight inpatients, diagnosed during cardiorespiratory deterioration (n = 5), or through incidental radiological findings (n = 3), were younger and had immobilization (87.5%), complex chronic diseases (75%), infections (75%) and central venous catheter (62.5%) as risk factors. Retrospectively, d -dimer testing and adult scores performed better than pediatric scores (sensitivity 92.9%-96% vs. 85.7%-92.9%). Both pediatric scores missed a case with a positive family history. DISCUSSION: Pediatric PE diagnosis is often delayed or missed. Development of pediatric prediction tools from validated adult scores merits being explored. We argue clinical presentation and risk factors may be different in inpatients and outpatients and propose broader reliance on family history.

Somatic Embryogenesis from Mature Embryos of Olea europaea L. cv. 'Galega Vulgar' and Long-Term Management of Calli Morphogenic Capacity.

Several olive cultivars, characterized by high-quality olive oil show agronomical issues such as excessive vigor, high susceptibility to biotic and abiotic stresses, and low propagation ability. They are strong candidates for breeding based on new technologies to improve their performance in a short period of time. For this reason, the first step is developing efficient somatic embryogenesis (SE) protocols. Somatic embryogenesis in olive is highly genotype-dependent for both adult tissues and mature embryos as initial explants, requiring the development of specific protocols for each genotype. Trials using cotyledons and radicles as initial explants, isolated from ripe seeds from the Portuguese olive cv. 'Galega vulgar', gave more than 95% calli development. Radicles proved to be the most responsive tissue for SE induction, with an average of 2 embryos per callus after callus transfer to expression medium, and 14 embryos per callus after subculture on the olive cyclic embryogenesis medium (ECO). Embryogenic competence could be recovered after several subcultures on ECO medium that maintained cyclic embryogenesis for an indeterminate period of time. Embryo conversion and plant acclimatization were also attained with high success rates. Media management for cyclic embryogenesis maintenance is of general importance for SE protocols in any olive genotype. Somatic embryogenesis was thus attained for the first time in embryo-derived explants of cv. 'Galega vulgar'.

Productive performance of soybean cultivars grown in different plant densities / Desempenho produtivo de cultivares de soja cultivadas em diferentes densidades de plantas

ABSTRACT: Plants density in soybean cultivation is an important management practice to achieve high grain yield. In this way, the objective was to evaluate the agronomic traits and grain yield in soybean in different plant densities, in two locations in the south of Minas Gerais. The experimental design was in randomized blocks, arranged in a split plot design, with three replications. Plots were composed of four population densities (300, 400, 500 and 600 thousand plants per hectare) and the subplots were composed of six cultivars (‘BMX Força RR’, ‘CD 250 RR’, ‘FMT 08 - 60.346/1’, ‘NA 5909 RR’, ‘TMG 7161 RR’ and ‘V - TOP RR’) grown in Lavras and Inconfidentes, both in Minas Gerais. At the time of harvest was determined the plant height, lodging, insertion of the first pod, harvest index, number of pods per plant, number of grains, number of grains per pod and yield. Regardless of the soybean cultivar, the plant density of up to 600,000 per ha does not affect grain yield, plant height, lodging, harvest index, and number of grains per pod. The cultivars ‘V-TOP RR’ and ‘BMX FORÇA RR’ showed high grain yield and good agronomic traits in Lavras and Incofidentes.

RESUMO: A densidade de plantas no cultivo de soja é uma prática de manejo relevante para o alcance de alta produtividade de grãos. Dessa forma, objetivou-se avaliar os caracteres agronômicos e produtividade dos grãos na soja em diferentes densidades de plantas. O delineamento experimental utilizado foi o de blocos casualizados, dispostos em esquema de parcelas subdivididas, com três repetições. As parcelas foram compostas por quatro densidades populacionais (300, 400, 500 e 600 mil plantas por hectare) e, as subparcelas foram compostas por seis cultivares (‘BMX Força RR’, ‘CD 250 RR’, ‘FMT 08 - 60.346/1’, ‘NA 5909 RR’, ‘TMG 7161 RR’ e ‘V - TOP RR’) cultivados em Lavras e Incofidentes, ambos em Minas Gerais. Na ocasião da colheita determinou-se a altura de plantas, o acamamento, a inserção do primeiro legume, o índice de colheita, o número de vagens por planta, o número de grãos, o número de grãos por vagem e a produtividade. Independentemente da cultivar de soja, a densidade de plantas de até 600.000 por ha, não afeta o rendimento de grãos, altura da planta, acamamento, índice de colheita e número de grãos por vagem. As cultivares ‘V-TOP RR’ e ‘BMX FORÇA RR’ apresentaram alto rendimento de grãos e boas características agronômicas em Lavras e Incofidentes.

Hyponatremia in a Teenager: A Rare Diagnosis.

INTRODUCTION: Hyponatremia is a common electrolyte alteration which has the potential for significant morbidity and mortality. Endocrine disorders, such as primary hypothyroidism and adrenal insufficiency are uncommon causes of hyponatremia. We present the case of a teenager with symptomatic hyponatremia caused by a rare disorder. CASE: A 17-year-old boy was admitted to the emergency department with abdominal pain, nausea and vomiting, asthenia, and weight loss. He was in poor general condition, hypotensive, and he had dry mucous membranes and skin as well as mucosa hyperpigmentation. The laboratory findings showed severe hyponatremia, hyperkalemia, and renal dysfunction. The patient started inotropic support and antibiotics. Plasma cortisol and corticotropin levels allowed the diagnosis of primary adrenal insufficiency. He began replacement therapy with hydrocortisone and fludrocortisone, with gradual symptom resolution. An abdominal computed tomography scan showed adrenal hypoplasia. Findings for antiadrenal and antithyroid antibodies were positive, allowing the diagnosis of autoimmune polyglandular syndrome type II. DISCUSSION: Adrenal insufficiency is a rare disease, especially in children, and its clinical manifestations are due to glucocorticoid and mineralocorticoid deficiency. In most of the cases, symptoms are nonspecific, requiring a high index of clinical suspicion. If the diagnosis and treatment are delayed, acute adrenal insufficiency carries a high morbidity and mortality.

Performance of PRISM III and PELOD-2 scores in a pediatric intensive care unit.

UNLABELLED: The study aims were to compare two models (The Pediatric Risk of Mortality III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD-2)) for prediction of mortality in a pediatric intensive care unit (PICU) and recalibrate PELOD-2 in a Portuguese population. To achieve the previous goal, a prospective cohort study to evaluate score performance (standardized mortality ratio, discrimination, and calibration) for both models was performed. A total of 556 patients consecutively admitted to our PICU between January 2011 and December 2012 were included in the analysis. The median age was 65 months, with an interquartile range of 1 month to 17 years. The male-to-female ratio was 1.5. The median length of PICU stay was 3 days. The overall predicted number of deaths using PRISM III score was 30.8 patients whereas that by PELOD-2 was 22.1 patients. The observed mortality was 29 patients. The area under the receiver operating characteristics curve for the two models was 0.92 and 0.94, respectively. The Hosmer and Lemeshow goodness-of-fit test showed a good calibration only for PRISM III (PRISM III: χ (2) = 3.820, p = 0.282; PELOD-2: χ (2) = 9.576, p = 0.022). CONCLUSIONS: Both scores had good discrimination. PELOD-2 needs recalibration to be a better reliable prediction tool. WHAT IS KNOWN: • PRISM III (Pediatric Risk of Mortality III) and PELOD (Pediatric Logistic Organ Dysfunction) scores are frequently used to assess the performance of intensive care units and also for mortality prediction in the pediatric population. • Pediatric Logistic Organ Dysfunction 2 is the newer version of PELOD and has recently been validated with good discrimination and calibration. What is New: • In our population, both scores had good discrimination. • PELOD-2 needs recalibration to be a better reliable prediction tool.

Neuroborreliosis presenting as acute disseminated encephalomyelitis.

We report a case of a 5-year-old boy with acute disseminated encephalomyelitis as the initial presentation of neuroborreliosis. Parents report an upper-airway infection a few days before the development of acute encephalopathy, mild facial palsy, and seizures. The patient needed mechanical ventilation for 10 days, and after extubation, he presented hypotonia, ataxia, dysarthria, as well as weak gag and cough reflexes. Brain magnetic resonance imaging showed hyperintense lesions on T2- and fluid-attenuated inversion recovery sequences on the right subcortical occipital and parietal region, left posterior arm of the internal capsule, and in the medulla oblongata. Borrelia burgdorferi was identified in the plasma and cerebrospinal fluid by polymerase chain reaction and in the plasma by Western blotting. He was treated with ceftriaxone, methylprednisolone, and human immunoglobulin. Recovery was partial.

Pulmonary artery sling: An uncommon cause of stridor.

Pulmonary artery sling is a rare congenital condition often associated with congenital tracheal stenosis. Untreated pulmonary sling carries a high morbidity and mortality, most of which is due to the airway and other associated anomalies rather than the aberrant artery itself. We report the case of an infant presenting progressive respiratory distress after a viral infection. The workup revealed a left pulmonary sling with concomitant tracheal stenosis. This anomaly was successfully corrected by slide tracheoplasty and left pulmonary artery reinsertion. Conclusion pulmonary artery sling is a rare entity with a nonspecific clinical presentation, a high degree of clinical suspicion is needed in order to obtain the correct diagnosis.

Análise da prática de automedicação nos pacientes atendidos na Unidade de Referência Especializada Demétrio Medrado / Analysis of self-medication practice among patients taken care of in the Unidade de Referência Especializada Demétrio Medrado

Os medicamentos são, desde 2000, o principal agente de intoxicação humana registrado pelo SUS no Brasil. A automedicação é um procedimento caracterizado fundamentalmente pela iniciativa de um doente, ou de seu responsável, em obter ou produzir e utilizar um produto que ele acredita trará benefícios no tratamento de doenças ou alívio de sintomas. O objetivo deste trabalho é analisar os pacientes atendidos na Unidade de Referência Especializada Demétrio Medrado, no período de setembro a dezembro de 2007, que se automedicam. Em relação ao método, trata-se de um estudo prospectivo, transversal e de coorte, envolvendo 117 pacientes atendidos na URE Demétrio Medrado, em Belém/PA, por meio da aplicação de protocolos aos pacientes, contendo perguntas relacionadas ao tema. Os resultados foram os seguintes: 52% (117) dos pacientes são do sexo feminino; concentram-se na faixa etária de 31-40 anos (40,18%); 87 (74%) pacientes praticam automedicação; sendo que 29 (33,33%) o fazem devido a cefaleia. Dentre os medicamentos utilizados sem recomendação médica, 35 (40,23%) pacientes utilizam analgésicos; 51 (58,63%) se automedicam até cessarem os sintomas, sendo que 40 (46%) pacientes apresentaram alguma reação adversa aos medicamentos utilizados. Portanto, conclui-se que dos pacientes entrevistados na Unidade de Referência Especializada Demétrio Medrado, a maioria é do sexo feminino, concentram-se na faixa etária de 31 a 40 anos, automedicam-se na maioria, devido a cefaleia, utilizando principalmente analgésicos até que se cessem os sintomas.

[Plasma hypertonicity in children]. / Hipertonia plasmática na criança.

The plasma hypertonicity is a severe and quite frequent disorder in children. The most frequent causes are hypernatremia related conditions, even though other causes of hyperosmolarity, such as hyperglycaemia and exogenous solutes accumulation also occur. The management and treatment of this condition is delicate and requires a thorough understanding of the underlying hydro electrolytic disorder. The authors perform a theoretical review of plasma hypertonicity in children, focusing on the three most frequent associated conditions: hypernatremic dehydration, salt poisoning and hyperosmolar coma.