Augusto Ribeiro

Systemic polyarteritis nodosa associated with acute Epstein-Barr virus infection.

Polyarteritis nodosa (PAN) is a rare cause of systemic vasculitis in children, affecting medium and small-sized arteries. We report on a patient who presented with prolonged fever, shock, acute renal failure with nephrotic range proteinuria, hypertension, and sudden deterioration of consciousness. Cranial tomography revealed a left extensive hemorrhagic lesion. Renal biopsy revealed a large fibrinoid necrosis lesion consistent with PAN. Epstein-Barr virus (EBV) infection was confirmed by real-time polymerase chain reaction (RT-PCR) detection of EBV DNA. The patient was successfully treated with oral prednisolone and intravenous pulse of cyclophosphamide. To our knowledge, this is the first reported case of c-PAN related to active EBV infection.

Ischemic subglottic damage following a short-time intubation.

The objective of this study is to report a case of ischemic subglottic damage after a short-time intubation with a large, overinflated endotracheal tube cuff in a child. The study uses individual case report. A 6-year-old boy was admitted to the pediatric intensive care unit after a head trauma intubated with a 5.5-mm inner diameter cuffed endotracheal tube overinflated with 16 ml of air that produced a pressure of more than 120 cm H2O. The endotracheal tube cuff pressure produced by inflation was reduced after 4 h. The child presented postextubation stridor with subglottic edema. Inappropriate handling of tracheal intubation without accurate measurement of endotracheal tube size and intracuff pressures of endotracheal tubes, can cause airway trauma and place patients at risk.

Thermal Properties of Ionic Liquids and Ionanofluids

The current world economy and energy situation demands the search for alternative energies to conventional fuels, the optimization of current energy technologies, and the search for new and clean working fluids. In the field of heat transfer, all current liquid coolants used at low and moderate temperatures exhibit very poor thermal conductivity and heat storage capacities, as the classical equipments for heat transfer use working fluids that were developed, tested and applied in a world of positive economical growth. In contrast, the uses of chemical technologies today are considered unsustainable. Although increased heat transfer can be achieved creating turbulence, increasing surface area and so on, ultimately the transfer will still be limited by the low thermal conductivity of the conventional fluids. Therefore, there is a need for new and efficient heat transfer liquids that can meet the cooling challenges for advanced devices as well as energy conversion for domestic and industrial applications. Ionic liquids (IL) have proven to be safe and sustainable alternatives for many applications in industry and chemical manufacturing. Their success arises mainly from their thermophysical and phase-equilibria properties, and the versatility of their synthesis, manageable to be tailored for a given application. Their solvent properties as well as their heat transfer or heat storage and surface properties make this class of fluids possible to use in a high plethora of applications (Earle & Seddon, 2007; Nieto de Castro & Santos, 2007). Other advantages of ionic liquids include high ionic conductivity, high chemical and thermal stabilities, negligible vapor pressure and an ability to dissolve a wide range of inorganic and organic compounds. Due to all of these fascinating characteristics they have been investigated extensively as alternatives to molecular solvents for liquid phase reactions (Wasserscheid & Welton,2007; Pârvulescu & Hardacre, 2007). Ionic liquids are of great interest to scientists as well as chemical companies, not only because of their interesting properties, but also for their actual and potential applications in the chemical process industries. In the past, the values of their thermophysical properties found to have significant effect on the design of physico-chemical processing and reaction units, influencing directly the design parameters and performance of equipments like heat exchangers, distillation columns and reactors (Franca et al., 2009). Their thermophysical properties justify the replacement of several of the chemical processes now under exploitation, and some of the solvents used, because they can, in certain conditions, be

SHAKEN BABY SYNDROME – 10 YEARS EXPERIENCE IN A PAEDIATRIC INTENSIVE CARE SERVICE

Introduction: Shaken baby syndrome is one of the causes of non-accidental injury most difficult to diagnose. Objectives: To characterize the patients hospitalized in the intensive care unit with shaken baby syndrome. Material and Methods: Retrospective study of patients admitted at the Paediatric Intensive Care Unit with shaken baby syndrome from 1 January 1999 to 31 December 2009. Results: There were eight children with shaken baby syndrome. The mean age was 4.1 months and five patients were male. One patient had a history of trauma. The most frequent cause of admission were epileptic seizures with cardiorespiratory/ respiratory arrest (50%). Bilateral retinal haemorrhages were present in six patients (75%). The CT scan showed subdural haematomas in all patients. Seven patients (87.5%) required mechanical ventilation, and five (62.5%) required inotropic support. The mean length of hospitalization was 5.25 days (1 -11 days). One patient died. Three patients showed no sequelae. There was a transient hemiparesis in one patient. Three patients presented epilepsy and deficits in visual acuity or hearing and in one case there was loss of prior acquisitions with a moderate to severe retardation of psychomotor development. Conclusion: In our study Shaken Baby Syndrome resulted in high morbidity with long-term sequelae in five patients (62,5%), consistent with data published in other series. The mortality rate was lower than in previous studies (12.5%). The presence of traumatic history was mentioned only in one case and, although all of them presented subdural haematomas, 25% of cases had no retinal haemorrhages. The clinical presentation was very severe and in most cases a high degree of suspicion is necessary in the diagnosis.

Heart Rate Variability in Children Submitted to Surgery

Objective: Heart Rate Variability (HRV) is known to reflect the sympathetic/parasympathetic interaction on several physiological and pathological conditions such as the cardiac activity responsiveness to physiological and environmental stimuli. Therefore, it has been widely used to assess nervous autonomic fluctuations and their influence on sinus node. Studies on children have demonstrated the correlation between HRV and different parameters like age, gender, physical activity and autonomic diseases whereas little evidence exists of the effects of surgery and residual anesthesic drugs on HRV. The aim of this study was to define the possible role of minor surgery in HRV in healthy children. Design: Observational prospective cohort study. Setting: Pediatric Surgery Department of a tertiary, university-affiliated hospital. Patients: 47 healthy children who were scheduled for elective minor surgery. Measurements and main results: HRV measurements of 10 to 15 minutes were obtained before and after surgery, using a Holter recorder and the BioSigBrowser® software. Results showed significant differences in HRV time domain indices before and after surgery in younger patients and in frequency domain in the older ones. They also demonstrated decreased HRV indices until one hour after being submitted to surgery in both time domain and LF parameters, reflecting a parasympathetic withdrawal and sympathetic predominance. Differences were also found when analyzing other variables such as anesthetic drugs. Conclusion: This study demonstrated that surgery has impact on Autonomic Nervous System function in healthy children but, independently of anesthetic drugs, type of surgery, age and existence of pain 60 minutes after surgery no difference in HRV measures was found and autonomic homeostasis was re-established. However, further investigation in this area is required in order to a better support of the role of HRV monitoring on early prognosis prediction and risk stratification in the surgical context.

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.

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.

AB0009 Association of an HLA-G 3’UTR haplotype with susceptibility to rheumatoid arthritis: A north-south analysis in two independent brazilian cohorts: Table 1.

Background HLA-G may exert long-term immunotolerogenic effects through the generation of suppressor cells [reviewed in [1]]. Such features render HLA-G an attractive candidate gene for susceptibility to immune mediated diseases. Indeed, our group has observed a positive association of a 3’UTR haplotype encompassing the 14bp locus and the +3142C/G (rs1063320) and disease susceptibility in patients with systemic lupus erythematosus [2]. Objectives To investigate the genetic influence of the two HLA-G 3’UTR polymorphisms – the 14 bp insertion/deletion (rs1704) and the +3142C>G (rs1063320) – in the susceptibility to rheumatoid arthritis in a double-center study comprising a Southern-Brazilian cohort from Porto Alegre and Northern-Brazilian cohort from the city of Belém. Methods A total number of 546 RA patients and 531 controls was PCR genotyped for the two polymorphisms. Results Haplotype frequencies differed significantly between control groups from the Northern and Southern cohorts. After adjusting for city of origin and gender, we observed that female patients presented a higher frequency of the deletion-G (D/G) haplotype (OR=1.634, 95% CI=1.128–2.368, P=0.009). After stratifying for RF positivity, only the female RF+ group of patients presented statistical significance (OR=1.829, 95%C.I =1.243–2.690, P=0.002) - Table 1. Table 1. HLA-G D/G carrier frequencies in patients and comparison with controls All Females Males N/Total (freq) N/Total (freq) N/Total (freq) Porto Alegre (South)  Controls 0.206 (59/287) 0.133 (16/120)a,b 0.257 (43/167)  Patients (all) 0.235 (80/340) 0.242 (67/277)a 0.206 (13/63)  RF+ 0.238 (67/281) 0.247 (56/227)b 0.204 (11/54)  RF– 0.232 (13/56) 0.234 (11/47) 0.222 (2/9) Belém (North)  Controls 0.338 (75/222) 0.302 (38/126)c 0.385 (37/96)  Patients (all) 0.360 (71/197) 0.374 (68/182) 0.200 (3/15)  RF+ 0.405 (60/148) 0.414 (58/140)c 0.250 (2/8)  RF– 0.242 (8/33) 0.286 (8/28) 0.0 (0/5) aP=0.015, bP=0.017, cP=0.072. Conclusions Our results suggest a differential influence of HLA-G 3’UTR polymorphisms in disease susceptibility, with the D/G haplotype as a risk factor for RA in RF+ females. References Carosella ED, HoWangYin K-Y, Favier B, LeMaoult J. Hla-g-dependent suppressor cells: Diverse by nature, function, and significance. Human immunology 2008;69:700-7. Consiglio CR, Veit TD, Monticielo OA, et al. Association of the hla-g gene +3142c>g polymorphism with systemic lupus erythematosus. Tissue Antigens 2011;77:540-5. Disclosure of Interest None Declared