The Self-Inflicted Burns - Typology and its Prognostic Relevance in a 14-Year Review of Self-Inflicted Burns in a Tertiary Referral Centre

Self-inflicted burns (SIB) are responsible for 2-6% of admissions to Burn Units in Europe and North America, and for as many as 25% of admissions in developing nations. Recently, a promising new tool was proposed to stratify SIB patients in the following subgroups: "typical", "delirious", and "reactive". However, as far as the authors know, the clinical usefulness of this instrument has not yet been validated by others. We retrospectively reviewed the clinical records of 56 patients admitted to our Burn Unit with the diagnosis of SIB injury in the past 14 years. The following parameters were evaluated: demographic features; psychiatric illness; substance abuse; mechanism of injury; burn depth, total body surface area (TBSA) involved, Abbreviated Burn Severity Index (ABSI); length of hospital stay, and mortality. All patients were followed up by a psychologist and a psychiatrist, and were classified according to the SIB-Typology Tool, into three classes: "typical", "delirious" and "reactive". There was a slight predominance of the "typical" type (44.6%), followed by the "delirious" type (30.4%), and, finally the "reactive" type (25.0%). Mortality was significantly higher in the "typical" subgroup. In conclusion, the SIB-Typology Tool appears to be a valuable instrument in the clinical management of SIB patients.

Prevalence of Asthma in a Portuguese Countryside Town:Repercussions on Absenteeism and Self-Concept

Background: Childhood asthma represents an increasing health problem and is the leading cause of hospital admission and absenteeism in children with chronic disease. It also compromises quality of life, eventually contributing to disturbances in self-concept. Self-concept is a recent and global perspective of “the self” and relates to skills, self-image and self-esteem. Little information is available on this topic and there are no data from Portuguese countryside towns. Objective: The aim of this study was to determine the prevalence of asthma among all school children in the 5th and 6th grades in a Portuguese countryside town and to establish its possible correlation with absenteeism and self-concept. Methods: In April 2002, two questionnaires were administered in the presence of the researcher to a group of 950 children attending different schools. The children completed the internationally renowned questionnaires: ISAAC and the Self-Concept Scale by Susan Harter. Results: Our sample (n = 818) had a mean age of 11 years (10-15 years) and a male-to-female ratio of 1/1. The cumulative prevalence of asthma was11.9% and that of active asthma was 8.8 %; 63.9% of asthmatics were male and 36.1 % were female. The mean age of asthmatics was 11.34 years and 74 % had active symptoms. Comparison of this group of 97 asthmatic children with the remaining children revealed a statistically significant correlation between the presence of asthma and school absenteeism (global: p = 0.04; gymnastics: 0.05). Regarding the Self-Concept Scale a statistically significant association was found between the presence of asthma and school achievement (p = 0.027), physical appearance (p = 0.015), behavior (p < 0.000) and self-esteem (p < 0.000). No statistically significant correlations were found in social acceptance (p = 0.289) or athletic competence (p = 0.085). Asthmatic boys had higher self-concept scores than girls, except in the domain of behavior. Conclusions: Twelve percent of the population studied was asthmatic. In asthmatic children, absenteeism was higher and self-concept was lower for almost all domains, except social acceptance and athletic achievement, probably due to overprotection.

Primary Antiphospholipid Syndrome: Pregnancy Outcome in a Portuguese Population

Introduction:Women with antiphospholipid syndrome(APS) may suffer from recurrent miscarriage, fetal death, fetal growth restriction (FGR), pre-eclampsia, placental abruption, premature delivery and thrombosis. Treatment with aspirin and low molecular weight heparin (LMWH) combined with close maternal-fetal surveillance can change these outcomes. Objective: To assess maternal and perinatal outcome in a cohort of Portuguese women with primary APS. Patients and Methods: A retrospective analysis of 51 women with primary APS followed in our institution (January 1994 to December 2007). Forty one(80.4%) had past pregnancy morbidity and 35.3%(n=18) suffered previous thrombotic events. In their past they had a total of 116 pregnancies of which only 13.79 % resulted in live births. Forty four patients had positive anticardiolipin antibodies and 33 lupus anticoagulant. All women received treatment with low dose aspirin and LMWH. Results: There were a total of 67 gestations (66 single and one multiple). The live birth rate was 85.1%(57/67) with 10 pregnancy failures: seven in the first and second trimesters, one late fetal death and two medical terminations of pregnancy (one APS related). Mean (± SD) birth weight was 2837 ± 812 g and mean gestational age 37 ± 3.3 weeks. There were nine cases of FGR and 13 hypertensive complications(4 HELLP syndromes). 54.4% of the patients delivered by caesarean section. Conclusions: In our cohort, early treatment with aspirin and LMWH combined with close maternal-fetal surveillance was associated with a very high chance of a live newborn.

Impact of Donor and Recipient Cytokine Genotypes on Renal Allograft Outcome

Allelic differences in gene promoter or codifying regions have been described to affect regulation of gene expression, consequently increasing or decreasing cytokine production and signal transduction responses to a given stimulus. This observation has been reported for interleukin (IL)-10 (-1082 A/G; -819/-592 CT/CA), transforming growth factor (TGF)-beta (codon 10 C/T, codon 25 G/C), tumor necrosis factor (TNF)-alpha (-308 G/A), TNF-beta (+252 A/G), interferon (IFN)-gamma (+874 T/A), IL-6 (-174 G/C), and IL-4R alpha (+1902 G/A). To evaluate the influence of these cytokine genotypes on the development of acute or chronic rejection, we correlated the genotypes of both kidney graft recipients and cadaver donors with the clinical outcome. Kidney recipients had 5 years follow-up, at least 2 HLA-DRB compatibilities, and a maximum of 25% anti-HLA pretransplantation sensitization. The clinical outcomes were grouped as follows: stable functioning graft (NR, n = 35); acute rejection episodes (AR, n = 31); and chronic rejection (CR, n = 31). The cytokine genotype polymorphisms were defined using PCR-SSP typing. A statistical analysis showed a significant prevalence of recipient IL-10 -819/-592 genotype among CR individuals; whereas among donors, the TGF-beta codon 10 CT genotype was significantly associated with the AR cohort and the IL-6 -174 CC genotype with CR. Other albeit not significant observations included a strong predisposition of recipient TGF-beta codon 10 CT genotype with CR, and TNF-beta 252 AA with AR. A low frequency of TNF-alpha -308 AA genotype also was observed among recipients and donors who showed poor allograft outcomes.

Clinical Outcome and Morphologic Analysis after Endovascular Aneurysm Repair Using the Excluder Endograft

OBJECTIVE: Long-term follow-up after endovascular aneurysm repair (EVAR) is very scarce, and doubt remains regarding the durability of these procedures. We designed a retrospective cohort study to assess long-term clinical outcome and morphologic changes in patients with abdominal aortic aneurysms (AAAs) treated by EVAR using the Excluder endoprosthesis (W. L. Gore and Associates, Flagstaff, Ariz). METHODS: From 2000 to 2007, 179 patients underwent EVAR in a tertiary institution. Clinical data were retrieved from a prospective database. All patients treated with the Excluder endoprosthesis were included. Computed tomography angiography (CTA) scans were retrospectively analyzed preoperatively, at 30 days, and at the last follow-up using dedicated tridimensional reconstruction software. For patients with complications, all remaining CTAs were also analyzed. The primary end point was clinical success. Secondary end points were freedom from reintervention, sac growth, types I and III endoleak, migration, conversion to open repair, and AAA-related death or rupture. Neck dilatation, renal function, and overall survival were also analyzed. RESULTS: Included were 144 patients (88.2% men; mean age, 71.6 years). Aneurysms were ruptured in 4.9%. American Society of Anesthesiologists classification was III/IV in 61.8%. No patients were lost during a median follow-up of 5.0 years (interquartile range, 3.1-6.4; maximum, 11.2 years). Two patients died of medical complications ≤ 30 days after EVAR. The estimated primary clinical success rates at 5 and 10 years were 63.5% and 41.1%, and secondary clinical success rates were 78.3% and 58.3%, respectively. Sac growth was observed in 37 of 142 patients (26.1%). Cox regression showed type I endoleak during follow-up (hazard ratio, 3.74; P = .008), original design model (hazard ratio, 3.85; P = .001), and preoperative neck diameter (1.27 per mm increase, P = .006) were determinants of sac growth. Secondary interventions were required in 32 patients (22.5%). The estimated 10-year rate of AAA-related death or rupture was 2.1%. Overall life expectancy after AAA repair was 6.8 years. CONCLUSIONS: EVAR using the Excluder endoprosthesis provides a safe and lasting treatment for AAA, despite the need for maintained surveillance and secondary interventions. At up to 11 years, the risk of AAA-related death or postimplantation rupture is remarkably low. The incidences of postimplantation sac growth and secondary intervention were greatly reduced after the introduction of the low-permeability design in 2004.

Norovírus Associated Encephalopathy

clinical presentation is self limited. It is classified into five groups (genogroups I through V). There are numerous reports of neurologic complications, namely afebrile seizures, but only two reports of associated encephalopathy. Case Report: A 12 month old girl with previous history of a pneumonia treated with amoxicillin-clavulanic acid and clarythromycin, presented in our emergency department with strabismus, ataxia for 3 days, later associated with vomiting and diarrhea. On admission she had ataxia and an episode of strabismus, but her later neurologic exam was normal. Laboratory data revealed: 10,9 g/dL hemoglobin, 11.200/μL leukocytes, 29,1% neutrophils and 65,2% lymphocytes, 488.000/μL platelets and negative CRP. The brain MRI showed middle ear, maxillary sinus and ethmoidal opacification, with no other abnormalities. During the first day of admission she had a tonic (?) seizure for 20 minutes. CSF analysis showed 5,6 cells/μL, 100% lymphocytes, 80 mg/dL glucose and 154,1 mg/dL protein. The EEG revealed short duration paroxystic activity located to the vertex. She was treated with acyclovir, ciprofloxacin, cefthriaxone and phenytoin. Her symptoms resolved by the third day of admission. Blood samples were tested for numerous pathogens, including serology for Borrelia, which was positive for IgG but negative for IgM. Fecal sample analysis revealed positive PCR for norovirus, although it was negative in CSF samples. IL-6 was measured in the CSF and was negative (5,8 pg/mL). She had a history of recurrent otitis media and pernieal candidiasis, which led to a detailed immune function study, which showed Immunology tests revealed diminished IgA (< 0,244 g/L) and absent antibody response to vaccinations. Since she was only 13 months old when she was tested, only follow up will determine the relevance of these values. Follow up at two years of age showed no delays and a normal development. Conclusion: Norovirus encephalitis is a rare entity, although gastrointestinal infection with this agent is relatively common. Here we present a case of a probable norovirus associated encephalopathy, although PCR for norovirus was negative in CSF samples and there was no CSF cytokine increase. It was not associated with adverse neurologic outcome and so far her development is normal, unlike the evolution described in previous case reports.

Cat-Scratch Disease With Bone Involvemnet

Background: Bartonella henselae infection typically presents as a self-limiting regional lymphadenopathy. Bone involvement is a very rare form of the disease. Aims: To describe bone infection associated to cat-scratch disease (CSD) in a portuguese pediatric hospital. Methods: Clinical records of children admitted at the hospital with the diagnosis of CSD associated bone infection, during 2010, were reviewed. Diagnosis was confirmed by serology using indirect fluorescence assay and nucleic acid amplification from lymph node biopsy. Results: Two boys, 2 and 7 years old, were identified. One had prolonged fever and neck pain. MRI suggested D6-D9 osteomyelitis. Cultures were negative and Mycobacterium tuberculosis and Brucella infection were excluded. He was treated with gentamicin and cotrimoxazol, with clinical, but no significant image, improvement. The second child presented subacute sternoclavicular swelling and mildly enlarged axillary lymph nodes. Image studies revealed an osteolytic lesion of the clavicle and hypoechogenic splenic lesions. Histopathology of lymph node showed granulomatous adenitis and excluded malignancy. Therapy with azythromicin and rifampicin was successful. Both had contact with cats. Primary and secondary immunodeficiency was excluded. Conclusion: The optimal therapy for atypical Bartonella henselae infection is unknown and the role of antibiotics uncertain. Several combinations of antibiotics have been proposed for bone disease treatment, but recommendations are lacking. The different outcome in the presented cases could be related with the distinct therapeutic regimens used. Although atypical infection has classically been associated with immunodeficiency, this has not been the rule in bone disease and the need for extensive evaluation must be reviewed.

A Rare Obstetric Outcome in Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis

Background: The unique clinical syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly is very rare and can be quite difficult to recognize because of the enormous heterogeneity in its clinical presentation. There are few long-term reports of the reproductive performance of women with this syndrome following treatment, or about the location of subsequent pregnancies. Case: A case in which two spontaneous pregnancies occurred alternatively in both hemiuteri: one despite a previous ipsilateral large hematometra and hematocolpos and the other, 8 years after, simultaneously with contralateral hematometra and hematocolpos(because of vaginal restenosis), is reported. Drainage of hematocolpos was performed at 14 weeks of pregnancy with immediate pain relief. Results: Pregnancy proceeded without complications. Eight month after delivery, a vaginoplasty was performed by excising the longitudinal vaginal septum, and marsupializing the vaginal cuff. Conclusions: This case highlights the importance of a correct and early diagnosis of developmental anomalies of the urogenital tract, as well as how a conservative approach in a Mullerian anomaly with unilateral obstruction led to two successful pregnancies occurring alternatively in the unaffected and in the previously blocked side. This is additional information supporting that every effort should be made to preserve the obstructed uterus.

The Risks of Self - Medication: Case Report of Familial Misuse of AM3 (Immunoferon®)

Over the last decades extended medical knowledge has been an important health care benefit in terms of disease prevention and management. However, probably with no exception, most pharmaceutical products are not devoid of adverse consequences. Immunomodulators are commonly considered a “benign” drug whose advantages bypass consequences. The immunomodulator AM3 (Immunoferon®) is a clinically used, orally administered compound whose active principle is stabilised in an inorganic matrix of calcium. We report the misuse of AM3 in three members of a family; father and two children. The drug was prescribed to the father who subsequently administered it to the children without seeking medical advice. Two months later, all subjects developed abdominal and/or flank colicky pain. Hypercalciuria was diagnosed in the children with different degrees of severity. It is likely that the calcium content of the inorganic matrix played an important role in the onset of symptoms. No adverse side effects related to the inorganic matrix of calcium of immunoferon® have been documented so far. This family case report calls attention to the risks of self -medication in a susceptible family. Paediatric patients are vulnerable as they rely on adults for the supply of medications. Concerning the use of drugs in family, especially nonprescription drugs, the quality of health care provided to the children depends on the health literacy of their parents.

Perinatal Outcome and Change in Body Mass Index in Mothers of Dichorionic Twins: a Longitudinal Cohort Study

We used a prospective cohort to analyze the effect of change in BMI rather than change in weight, in mothers carrying dichorionic twins from a population that did not receive any dietary intervention. A total of 269 mothers (150 nulliparas and 119 multiparas) were evaluated. The average change (%) from the pre-gravid BMI was 7.2+/-6.1, 17.4+/-8.2, and 28.7+/-10.8, at 12-14, 22-25, and 30-34 weeks, respectively, without difference between nulliparas and multiparas. The comparison between maternities below or above the average change from the pregravid BMI failed to demonstrate an advantage (in terms of total twin birthweight and gestational age) of an above average change from the pregravid BMI, even when the lower versus upper quartiles were compared. Our observations reached different conclusions regarding the recommended universal dietary intervention in twin gestations. A cautious approach is advocated towards seemingly harmless excess weight gain, as normal weight women may turn overweight, or even obese, by the end of pregnancy, and be exposed to the untoward effects of obesity on future health and body image.

Predicting Outcome after Cardiopulmonary Arrest in Therapeutic Hypothermia Patients: Clinical, Electrophysiological and Imaging Prognosticators

INTRODUCTION: Predicting outcome in comatose survivors of cardiac arrest is based on data validated by guidelines that were established before the era of therapeutic hypothermia. We sought to evaluate the predictive value of clinical, electrophysiological and imaging data on patients submitted to therapeutic hypothermia. MATERIALS AND METHODS: A retrospective analysis of consecutive patients receiving therapeutic hypothermia during years 2010 and 2011 was made. Neurological examination, somatosensory evoked potentials, auditory evoked potentials, electroencephalography and brain magnetic resonance imaging were obtained during the first 72 hours. Glasgow Outcome Scale at 6 months, dichotomized into bad outcome (grades 1 and 2) and good outcome (grades 3, 4 and 5), was defined as the primary outcome. RESULTS: A total of 26 patients were studied. Absent pupillary light reflex, absent corneal and oculocephalic reflexes, absent N20 responses on evoked potentials and myoclonic status epilepticus showed no false-positives in predicting bad outcome. A malignant electroencephalographic pattern was also associated with a bad outcome (p = 0.05), with no false-positives. Two patients with a good outcome showed motor responses no better than extension (false-positive rate of 25%, p = 0.008) within 72 hours, both of them requiring prolonged sedation. Imaging findings of brain ischemia did not correlate with outcome. DISCUSSION: Absent pupillary, corneal and oculocephalic reflexes, absent N20 responses and a malignant electroencephalographic pattern all remain accurate predictors of poor outcome in cardiac arrest patients submitted to therapeutic hypothermia. CONCLUSION: Prolonged sedation beyond the hypothermia period may confound prediction strength of motor responses.

Psychiatric and Psychosocial Predictors of Medical Outcome After Liver Transplantation: a Prospective, Single-Center Study

OBJECTIVE: Recognizing the potential impact of psychiatric and psychosocial factors on liver transplant patient outcomes is essential to apply special follow-up for more vulnerable patients. The aim of this article was to investigate the psychiatric and psychosocial factors predicted medical outcomes of liver transplanted patients. METHODS: We studied 150 consecutive transplant candidates, attending our outpatient transplantation clinic, including 84 who had been grafted 11 of whom died and 3 retransplanted. RESULTS: We observed that active coping was an important predictor of length of stay after liver transplantation. Neuroticism and social support were important predictors of mortality after liver transplantation. CONCLUSION: It may be useful to identify patients with low scores for active coping and for social support and high scores for neuroticism to design special modes of follow-up to improve their medical outcomes.

When Does Quality of Life Improve After Liver Transplantation? A Longitudinal Prospective Study

OBJECTIVES: We sought to investigate the improvement in quality of life (mental and physical components) at 1 and 6 months after liver transplantation. METHODS: A sample of liver transplant candidates (n = 60), comprising consecutive patients attending outpatient clinics of a liver transplantation central unit (25% of the patients had familial amyloid polyneuropathy [FAP] and the remaining patents had chronic liver diseases), was assessed by means of the Short Form (SF)-36, Portuguese-validated version, a self-rating questionnaire developed by the Medical Outcome Trust, to investigate certain primary aspects of quality of life, at 3 times: before, and at 1 and 6 months after transplantation. RESULTS: We observed a significant improvement in quality of life (both mental and physical components) by 1 month after transplantation. Between the first month and the sixth month after transplantation, there also was an improvement in the quality of life (both mental and physical components), although only the physical components of quality of life was significantly improved. CONCLUSIONS: Our findings suggested that quality of life improved early after liver transplantation (1 month). Between the first and the sixth months, there only was a significant improvement in the physical quality of life.

Effect of C282Y Genotype on Self-Reported Musculoskeletal Complications in Hereditary Hemochromatosis

OBJECTIVE: Arthropathy that mimics osteoarthritis (OA) and osteoporosis (OP) is considered a complication of hereditary hemochromatosis (HH). We have limited data comparing OA and OP prevalence among HH patients with different hemochromatosis type 1 (HFE) genotypes. We investigated the prevalence of OA and OP in patients with HH by C282Y homozygosity and compound heterozygosity (C282Y/H63D) genotype. METHODS: A total of 306 patients with HH completed a questionnaire. Clinical and demographic characteristics and presence of OA, OP and related complications were compared by genotype, adjusting for age, sex, body mass index (BMI), current smoking and menopausal status. RESULTS: In total, 266 of the 306 patients (87%) were homozygous for C282Y, and 40 (13%) were compound heterozygous. The 2 groups did not differ by median age [60 (interquartile range [IQR] 53 to 68) vs. 61 (55 to 67) years, P=0.8], sex (female: 48.8% vs. 37.5%, P=0.18) or current smoking habits (12.4% vs. 10%, P=0.3). As compared with compound heterozygous patients, C282Y homozygous patients had higher median serum ferritin concentration at diagnosis [1090 (IQR 610 to 2210) vs. 603 (362 to 950) µg/L, P<0.001], higher median transferrin saturation [80% (IQR 66 to 91%) vs. 63% (55 to 72%), P<0.001]) and lower median BMI [24.8 (22.1 to 26.9) vs. 26.2 (23.5 to 30.3) kg/m2, P<0.003]. The overall prevalence of self-reported OA was significantly higher with C282Y homozygosity than compound heterozygosity (53.4% vs. 32.5%; adjusted odds ratio [aOR] 2.4 [95% confidence interval 1.2-5.0]), as was self-reported OP (25.6% vs. 7.5%; aOR 3.5 [1.1-12.1]). CONCLUSION: Patients with C282Y homozygosity may be at increased risk of musculoskeletal complications of HH.

Clinical Outcome and Morphologic Determinants of Mural Thrombus in Abdominal Aortic Endografts

OBJECTIVE:Endograft mural thrombus has been associated with stent graft or limb thrombosis after endovascular aneurysm repair (EVAR). This study aimed to identify clinical and morphologic determinants of endograft mural thrombus accumulation and its influence on thromboembolic events after EVAR. METHODS: A prospectively maintained database of patients treated by EVAR at a tertiary institution from 2000 to 2012 was analyzed. Patients treated for degenerative infrarenal abdominal aortic aneurysms and with available imaging for thrombus analysis were considered. All measurements were performed on three-dimensional center-lumen line computed tomography angiography (CTA) reconstructions. Patients with thrombus accumulation within the endograft's main body with a thickness >2 mm and an extension >25% of the main body's circumference were included in the study group and compared with a control group that included all remaining patients. Clinical and morphologic variables were assessed for association with significant thrombus accumulation within the endograft's main body by multivariate regression analysis. Estimates for freedom from thromboembolic events were obtained by Kaplan-Meier plots. RESULTS: Sixty-eight patients (16.4%) presented with endograft mural thrombus. Median follow-up time was 3.54 years (interquartile range, 1.99-5.47 years). In-graft mural thrombus was identified on 30-day CTA in 22 patients (32.4% of the study group), on 6-month CTA in 8 patients (11.8%), and on 1-year CTA in 17 patients (25%). Intraprosthetic thrombus progressively accumulated during the study period in 40 patients of the study group (55.8%). Overall, 17 patients (4.1%) presented with endograft or limb occlusions, 3 (4.4%) in the thrombus group and 14 (4.1%) in the control group (P = .89). Thirty-one patients (7.5%) received an aortouni-iliac (AUI) endograft. Two endograft occlusions were identified among AUI devices (6.5%; overall, 0.5%). None of these patients showed thrombotic deposits in the main body, nor were any outflow abnormalities identified on the immediately preceding CTA. Estimated freedom from thromboembolic events at 5 years was 95% in both groups (P = .97). Endograft thrombus accumulation was associated with >25% proximal aneurysm neck thrombus coverage at baseline (odds ratio [OR], 1.9; 95% confidence interval [CI], 1.1-3.3), neck length ≤ 15 mm (OR, 2.4; 95% CI, 1.3-4.2), proximal neck diameter ≥ 30 mm (OR, 2.4; 95% CI, 1.3-4.6), AUI (OR, 2.2; 95% CI, 1.8-5.5), or polyester-covered stent grafts (OR, 4.0; 95% CI, 2.2-7.3) and with main component "barrel-like" configuration (OR, 6.9; 95% CI, 1.7-28.3). CONCLUSIONS: Mural thrombus formation within the main body of the endograft is related to different endograft configurations, main body geometry, and device fabric but appears to have no association with the occurrence of thromboembolic events over time.