Adolescents and School Asthma Knowledge and Attitudes

Introduction: bronchial asthma is a chronic disease that affects a high percentage of adolescents, with a significant restriction of daily activities, and is a cause of school absenteeism. The relationships between adolescents and asthma disease in school were assessed, with a view to improving knowledge about the asthmatic adolescent. Methods: a survey was conducted in the Lisbon metropolitan area, covering urban (Lisbon) and rural (Lourinh˜a ) zones and including 1879 students and 81 teachers from the 7th to 9th high school years. The study groups were asthmatic students, their peers, and teachers. A self-administered questionnaire was applied to collect information. The results were compared with a reference group of 91 asthmatic students attending our Department of Immunoallergy-Hospital Dona Estefânia. Cotinine urinary measurements were made in a sample of asthmatics and a control group. Results: the prevalence of current asthma among students was 10%. Estimates of asthma annual burden among 7th to 9th year students from Lisbon and Lourinh˜a high schools included 4,307 days missed from school, 4,148 medical consultations and a minimum of 351 hospital emergency care and 80 hospital admissions. Exposure to passive smoking was not significantly different between asthmatic students and theirs peers. Cotinine urinary measurements did not discriminate between exposed and non-exposed individuals. Cigarette smoking was almost as common among adolescent asthmatics (5.4%) as it was in non-asthmatic subjects (6.7%). However, 55% of asthmatics mentioned active and passive smoking as an asthma exacerbating factor. Asthmatic students, theirs peers and teachers showed a deficient knowledge about asthma (mean group scores: 17.6; 14.2 and 17.7 of a possible 30), particularly in the areas related to asthma recognition and its management. Asthmatics attending our Allergy Department had the highest scores. All groups showed tolerance in the sense of a positive and understanding attitude toward a person with asthma. However, traditional beliefs about asthma disease (dependence, inferiority...) were confirmed. A positive correlation between knowledge levels and tolerance attitudes was found. Conclusion: in view of the dimension of the asthma problem in adolescence and its social and economic impact, it is justifiable to assess the need for the implementation of asthma education programs in schools in order to improve asthma management by the adolescents and their schools.

Encerramento Percutâneo de Shunts Interauriculares: Experiência de uma Década de um Centro Terciário

INTRODUCTION: Atrial septal defects (ASD) are among the most common congenital anomalies and account for 10% of congenital heart disease in the pediatric age-group and 30% in adults. Closure is indicated when there is evidence of hemodynamic significance or after a paradoxical embolic event. Ten years ago, percutaneous closure became the treatment of choice in our center for all patients with a clear indication and favorable anatomy. In this paper we report the experience of this first decade. OBJECTIVE: To assess the short- and long-term results of our ten-year experience with percutaneous closure of atrial septal defects. METHODS: We studied retrospectively all patients with ASD treated with a percutaneous approach between November 1998 and December 2008. The pediatric age-group consisted of patients younger than 19 years old. Demographic data, clinical indications, minor and major complication rates, success rate and long-term outcome were assessed. RESULTS: In the first ten years of experience 510 patients, of whom 166 were in the pediatric group, were treated in our center by a team of adult and pediatric cardiologists. The overall success rate of the procedure was 98% (97.5% in ASD and 99.5% in patent foramen ovale (PFO). The minor complication rate was 3% (3.4% in ASD and 2% in PFO). The most frequent complication was supraventricular tachycardia. The major complication rate was 1.2% (0.6% in ASD and 2% in PFO). Two patients developed cardiac tamponade due to hemopericardium that was resolved by pericardiocentesis, without need for surgery. One patient had an arterial pseudoaneurysm corrected by vascular surgery. There was no device embolization and no need for urgent surgery in this population. During follow-up two patients had recurrence of ischemic stroke, one had a transient ischemic attack and another had a hemorrhagic stroke. Mortality was 0.6% (0.6% in ASD and 0.5% in PFO). There were no in-hospital deaths. During follow-up there were two deaths, both in the adult group. DISCUSSION AND CONCLUSION: In this population the success rate was high and most of the complications were minor. The results of this collaboration between adult and pediatric cardiologists in the first ten years of activity confirm the safety and efficacy of percutaneous closure of septal defects, when there is careful patient selection and a standardized technique.

Clinical and Bacteriological Survey of Diabetic Foot Infections in Lisbon

AIMS: An epidemiological survey of diabetic foot infections (DFIs) in Lisbon, stratifying the bacterial profile based on patient demographical data, diabetic foot characteristics (PEDIS classification), ulcer duration and antibiotic therapy. METHODS: A transversal observational multicenter study, with clinical data collection using a structured questionnaire and microbiological products (aspirates, biopsies or swabs collected using the Levine method) of clinically infected foot ulcers of patients with diabetes mellitus (DM). RESULTS: Forty-nine hospitalized and ambulatory patients were enrolled in this study, and 147 microbial isolates were cultured. Staphylococcus was the main genus identified, and methicillin-resistant Staphylococcus aureus (MRSA) was present in 24.5% of total cases. In the clinical samples collected from patients undergoing antibiotic therapy, 93% of the antibiotic regimens were considered inadequate based on the antibiotic susceptibility test results. The average duration of an ulcer with any isolated multi-drug resistant (MDR) organism was 29 days, and previous treatment with fluoroquinolones was statistically associated with multi-drug resistance. CONCLUSIONS: Staphylococcus aureus was the most common cause of DFIs in our area. Prevalence and precocity of MDR organisms, namely MRSA, were high and were probably related to previous indiscriminate antibiotic use. Clinicians should avoid fluoroquinolones and more frequently consider the use of empirical anti-MRSA therapy.

Terapêutica Endovascular de Pseudoaneurisma e Fístula Arteriovenosa em Enxerto Renal

A 31 year old male Caucasian received a renal cadaveric allograft. Reconstruction of an inferior polar artery was corrected pre-implantation. Delayed graft function occurred leading to dialysis support for one month. Graft biopsies(days 7, 15) showed acute tubular necrosis(ATN) and no rejection. Serial ultrasound (US), performed on average weekly, were compatible with ATN. On day 31, Doppler US and a CAT scan suggested for the first time a pseudoaneurysm adjacent to the implantation of the graft artery on the external iliac artery. For clinical and technical reasons, arteriography was only performed on day 67, when serum creatinine was 3.3 mg/dl. It showed a large pseudoaneurysm with an arteriovenous fistula to the right common iliac vein. Compression of the right external iliac artery was clear. In an attempt to close the arteriovenous fistula, the communication with the pseudoaneurysm was embolised with gelfoam and metallic coils with partial success. One week later, by right femoral approach a covered wallstent was placed immediately below the origin of the graft artery.Subsequent Doppler US and arteriography con-firmed closure of the communication with thepseudoaneurysm and of the arteriovenous fistula. The calibre of the right external iliac artery was then normal. By month 18, serum creatinine is stable at 2.1 mg/dl. We can only speculate on the origin of thepseudoaneurysm and of the AV fistula, whichwere not evident until one month post-transplantation. Backtable surgery was performed on thepolar not the main graft artery. Invasive angiography was irreplaceable in this unusual clinical situation.

Phototherapy in Childhood: a 17-Year Retrospective Study Regarding Effectiveness and Safety

Introduction: Skin diseases in paediatric age are often distressing conditions with significant impact in children’s psychosocial development. Additionally, systemic therapeutic options are often limited in childhood, due to its potential toxicity in this vulnerable group. Phototherapy is therefore an endorsed option for photo-responsive dermatological conditions. Objective and Methods:This observational retrospective study aims to access efficacy and safety of Phototherapy in our paediatric population. Relevant clinical data from 1996 to present concerning patients aged 18 years or less was collected. Results: 78 patients were included, of which64,1%was female. Mean age was 12,9 years (range 2-18). Distribution according to diagnosis was:47,4%psoriasis, 34,6% alopecia areata, 9,0% vitiligo, 9,0% other diagnosis. Mean number of cycles was 1,5 (range 1-7), with an average of 16,3 treatments per cycle and mean cumulative dose 134 J/cm2. 70,5% was treated with one single cycle. Topic and systemic PUVA were the first choice in 37,2% and 39,7%, respectively, while UVB TL01 and broadband UVB were used in 11,5% each. On the first cycle 67,5% improved, 14,3% showed no sustained clinical response and 19,5% were lost to follow-up. Psoriasis patients had the best response rates (81,8%), followed by alopecia areata (59,3%). Side effects occurred in 21%, being erythema the most common (12%). None led to therapeutic interruption. Discussion: Phototherapy is a safe and effective option in childhood, yet the withdraw rate might be an important limitation.

Blood Donation in the Hospital de S. José. A 11-Year Retrospective Analysis of Deferrals and Exclusions

Background: Approximately 5% of the population donates blood each year in developed countries. Recruiting and maintaining a pool of altruistic and healthy blood donors is a challenging task. Blood donation as a dynamic process must naturally co-exist with the arguably essential deferrals. Aims: To analyse a 11-year cohort of donors and blood donations in order to determine the profile of the average donor and the typical donation. Characterize the donor’s population in terms of gender, age, number of donations, most common causes for deferral and exclusion and the possible relationships between them. Establish the tendency flow of donations per year. Methods: Analysis of 95861 blood donations from 31550 donors collected between 2000 and 2010 (11 years) in the Immunohemotherapy Department of the ‘‘Centro Hospitalar Lisboa Central - Hospital de Sa˜o Jose´’’ (Lisboa, Portugal). Prior to blood donation, donors were required to fill out a form of informed consent, a questionnaire of 21 ‘‘yes or no’’ questions and were submitted to a clinical assessment and physical examination including: measurement of weight, blood pressure, pulse and capillary hemoglobin levels. Post-donation, the collected blood was tested for ALT elevation and blood-borne viral agents (HBV, HCV, HIV 1 and 2 and HTLV 1 and 2) and other infections (Treponema pallidum). Blood donors and donations were registered in a database and statistically studied in terms of: gender and age distribution, number of donations, most common causes for deferral and exclusion. The frequency of blood donations throughout the period of observation was analyzed and statistically significant relationships between the collected variables were investigated. Results: From the population of 31550 donors 61% were male and a mean age of 41.5 years (± 12.5 years) was found. From the total of 95682 blood donations collected 78% were successful while the most common causes for deferral were: donation incompatible hemoglobin levels (5% of the blood donations and 22% of deferrals), ALT elevation (3% and 14%), positive blood screening test for Treponema pallidum (1% and 6%), medication (1% and 4%), positive serological blood markers for HBV (1% and 4%), endoscopy in the previous 12 months (1% and 3%), arterial hypertension (1% and 3%), infectious conditions (1% and 3%), influenza or influenza-like symptoms (1% and 2%) and positive serological blood markers for HCV (1% and 2%). Summary/Conclusions: Several factors may have contributed to a limited number of new regular donors in the population, namely: ageing population, the alienation of the individual from the community induced by modern lifestyles and job precariousness. It is of the utmost importance to refine our blood donation campaigns according to the existing population of donors. The optimization of the blood donation potential of a population of donors must be achieved through the development of reliable and consistent screening methods. In order to appeal to new donors it is important to promote blood donations considering the profile of the regular and healthy blood donor of the existing population.

Alcohol Septal Ablation in Obstructive Hypertrophic Cardiomyopathy: Four Years of Experience at a Reference Center

INTRODUCTION: We describe our center's initial experience with alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy. The procedure, its indications, results and clinical outcomes will be addressed, as will its current position compared to surgical myectomy. OBJECTIVE: To assess the results of ASA in all patients treated in the first four years of activity at our center. METHODS: We retrospectively studied all consecutive and unselected patients treated by ASA between January 2009 and February 2013. RESULTS: In the first four years of experience 40 patients were treated in our center. In three patients (7.5%) the intervention was repeated. Procedural success was 84%. Minor complications occurred in 7.5%. Two patients received a permanent pacemaker for atrioventricular block (6% of those without previous pacemaker). The major complication rate was 5%. There were no in-hospital deaths; during clinical follow-up (22 ± 14 months) cardiovascular mortality was 2.5% and overall mortality was 5%. DISCUSSION AND CONCLUSION: The results presented reflect the initial experience of our center with ASA. The success rate was high and in line with published results, but with room to improve with better patient selection. ASA was shown to be safe, with a low complication rate and no procedure-related mortality. Our experience confirms ASA as a percutaneous alternative to myectomy for the treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy refractory to medical treatment.

Vascular Malformation and Common Keratinocytic Nevus of the Soft Type: Phacomatosis Pigmentovascularis Revisited

Phacomatosis pigmentovascularis is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation. We report a 5-year-old boy with all the typical findings of phacomatosis pigmentovascularis type Ia. Although its existence according to the traditional classification has been questioned, this case represents a very rare association of a capillary vascular malformation and a common keratinocytic nevus of the soft type.

Tumors of the Brachial Plexus in a Tertiary Referral Center: a Case Series and Literature Review

Introduction: Brachial plexus (BP) tumors are very rare tumors, with less than 800 cases been described in the literature worldwide since 1970. These tumors often present as local or radicular pain, with scant or no neurological deficits. These symptoms are shared by many other more common rheumatologic diseases, thus making their diagnosis difficult in most cases. Additionally, these tumors often present as lumps and are therefore biopsied, which carries a significant risk of iatrogenic nerve injury. Material and Methods: In this paper the authors describe their experience with the management of 5 patients with BP tumors followed up for at least 2 years. There were 4 males and 1 female. Median follow-up time was 41 ± 21 months. Average age at diagnosis was 40,0 ± 19,9 years. The most common complaints at presentation were pain and sensibility changes. All patients had a positive Tinel sign when the lesion was percussed. In all patients surgery was undertaken and the tumors removed. In 4 patients nerve integrity was maintained. In one patient with excruciating pain a segment of the nerve had to be excised and the nerve defect was bridged with sural nerve grafts. Results: Pathology examination of the resected specimens revealed a Schwannoma in 4 cases and a neurofibroma in the patient submitted to segmental nerve resection. Two years postoperatively, no recurrences were observed. All patients revealed clinical improvement. The patient submitted to nerve resection had improvement in pain, but presented diminished strength and sensibility in the involved nerve territory. Conclusion: Surgical excision of BP tumors is not a risk free procedure. Most authors suggest surgery if the lesion is symptomatic or progressing in size. If the tumor is stationary and not associated with neurological dysfunction a conservative approach should be taken.

The PROP1 2-Base Pair Deletion Is a Common Cause of Combined Pituitary Hormone Deficiency

Combined pituitary hormone deficiency (CPHD) has an incidence of approximately 1 in 8000 births. Although the proportion of familial CPHD cases is unknown, about 10% have an affected first degree relative. We have recently reported three mutations in the PROP1 gene that cause CPHD in human subjects. We report here the frequency of one of these mutations, a 301–302delAG deletion in exon 2 of PROP1, in 10 independently ascertained CPHD kindreds and 21 sporadic cases of CPHD from 8 different countries. Our results show that 55% (11 of 20) of PROP1 alleles have the 301–302delAG deletion in familial CPHD cases. Interestingly, although only 12% (5 of 42) of the PROP1 alleles of our 21 sporadic cases were 301–302delAG, the frequency of this allele (in 20 of 21 of the sporadic subjects given TRH stimulation tests) was 50% (3 of 6) and 0% (0 of 34) in the CPHD cases with pituitary and hypothalamic defects, respectively. Using whole genome radiation hybrid analysis, we localized the PROP1 gene to the distal end of chromosome 5q and identified a tightly linked polymorphic marker, D5S408, which can be used in segregation studies. Analysis of this marker in affected subjects with the 301–302delAG deletion suggests that rather than being inherited from a common founder, the 301–302delAG may be a recurring mutation.

Acute Otitis Media a Reliable Warning Sign for Primary Immunodeficiencies?- A Critical Appraisal

Acute otitis media (AOM) is the most common infection in childhood, resulting from both anatomic and immunologic specificities of this age group. Recurrent AOM has been defined as one of the warning signs for primary immunodeficiencies (PID), In this study we evaluated the strength of recurrent AOM as clinical predictor of PID. Methods: Retrospective study (August 2010 - December 2013) which included all patients referred to PID appointment because of recurrent AOM (= 8 AOM episodes/year). Syndromic patients or those presenting with another warning sign for PID were excluded. Clinical, demographic and laboratory results were analized and statistical analysis was made using SPSS 20. Results: Seventy-five patients were included (median age 37,8 months; 62,7% male gender), corresponding to 15% of all first appointments. Other comorbidities were present in 20% of the patients and 17% had ORL surgery prior to PID referral. In most patients, the immunologic screening consisted on the evaluation of humoral function, but in selected cases other studies were performed (namely complement and lymphocyte immunophenotyping). A PID was identified in 12 children (16,0%) and the majority of these patients had other distinctive feature (personal or familiar antecedent of infection or auto-immunity, 66,7%, p<0,05). Nine children (12,0%) underwent prophylactic cotrimoxazole. The average length of follow-up was 11,2 months. Conclusion: Despite being a very frequent cause of immunologic screening, in this study recurrent AOM was not found to be a good predictor of underlying PID, unless the patients presents other significant personal or family history.

The PROP1 2-Base Pair Deletion Is a Common Cause of Combined Pituitary Hormone Deficiency

Combined pituitary hormone deficiency (CPHD) has an incidence of approximately 1 in 8000 births. Although the proportion of familial CPHD cases is unknown, about 10% have an affected first degree relative. We have recently reported three mutations in the PROP1 gene that cause CPHD in human subjects. We report here the frequency of one of these mutations, a 301-302delAG deletion in exon 2 of PROP1, in 10 independently ascertained CPHD kindreds and 21 sporadic cases of CPHD from 8 different countries. Our results show that 55% (11 of 20) of PROP1 alleles have the 301-302delAG deletion in familial CPHD cases. Interestingly, although only 12% (5 of 42) of the PROP1 alleles of our 21 sporadic cases were 301-302delAG, the frequency of this allele (in 20 of 21 of the sporadic subjects given TRH stimulation tests) was 50% (3 of 6) and 0% (0 of 34) in the CPHD cases with pituitary and hypothalamic defects, respectively. Using whole genome radiation hybrid analysis, we localized the PROP1 gene to the distal end of chromosome 5q and identified a tightly linked polymorphic marker, D5S408, which can be used in segregation studies. Analysis of this marker in affected subjects with the 301-302delAG deletion suggests that rather than being inherited from a common founder, the 301-302delAG may be a recurring mutation.