Outcome improvement with chemotherapy and radiotherapy in primary, localized, radiation-associated angiosarcoma of the breast region: a retrospective case series analysis.

BACKGROUND: We report on a series of consecutive patients with localized radiation-associated angiosarcoma (RAAS) of the breast region (BR) treated at two Italian sarcoma reference centers. MATERIALS AND METHODS: We retrospectively reviewed all cases of primary, localized, resectable RAAS of the BR, treated at one of the two participating institutions from 2000 to 2019. Relapse-free survival (RFS) and overall survival (OS) were calculated. The prognostic role of several variables was investigated. A propensity score matched (PSM) analysis was carried out. RESULTS: Eighty-four patients were retrospectively identified. Nineteen out of 84 patients (22.6%) were pretreated with an anthracycline-based regimen for previous cancer. All patients but one underwent surgery, with 37/84 (44.1%) receiving surgery alone and 46/84 (54.8%) a multimodal approach: 18/84 (21.4%) received radiation therapy (RT) and 46/84 (54.9%) received chemotherapy. An anthracycline-based regimen was used in 10/84 patients (11.9%), while a gemcitabine-based regimen was used in 33/84 (39.3%). With a median follow-up of 51 months (interquartile range: 30-126 months), 36/84 patients (42.9%) relapsed and 35/84 patients (41.7%) died (8/84, 9.5% in the lack of metastatic disease). Five-year OS and 5-year RFS were 57% [95% confidence interval (CI) 43% to 68%] and 52% (95% CI 39% to 63%), respectively. Both (neo)adjuvant RT and chemotherapy were associated with better RFS [hazard ratio (HR) 0.25, 95% CI 0.08-0.83; HR 0.45, 95% CI 0.23-0.89] with a trend towards a better OS (HR 0.51, 95% CI 0.18-1.46; HR 0.60, 95% CI 0.29-1.24). Gemcitabine-based regimens seemed to perform better (HR 4.28, 95% CI 1.29-14.14). PSM analysis retained the above results. CONCLUSIONS: This retrospective study supports the use of (neo)adjuvant RT and chemotherapy, in primary, localized resectable RAAS of the BR. An effort to prospectively validate the role of (neo)adjuvant RT and chemotherapy is warranted.

Role of inflammatory markers in the diagnosis of vascular contributions to cognitive impairment and dementia: a systematic review and meta-analysis.

Vascular contribution to cognitive impairment and dementia (VCID) is a clinical label encompassing a wide range of cognitive disorders progressing from mild to major vascular cognitive impairment (VCI), which is also defined as vascular dementia (VaD). VaD diagnosis is mainly based on clinical and imaging findings. Earlier biomarkers are needed to identify subjects at risk to develop mild VCI and VaD. In the present meta-analysis, we comprehensively evaluated the role of inflammatory biomarkers in differential diagnosis between VaD and Alzheimer's disease (AD), and assessed their prognostic value on predicting VaD incidence. We collected literature until January 31, 2021, assessing three inflammatory markers [interleukin(IL)-6, C-reactive protein (CRP), tumor necrosis factor (TNF)-α] from blood or cerebrospinal fluid (CSF) samples. Thirteen cross-sectional and seven prospective studies were included. Blood IL-6 levels were cross-sectionally significantly higher in people with VaD compared to AD patients (SMD: 0.40, 95% CI: 0.18 to 0.62) with low heterogeneity (I2: 41%, p = 0.13). Higher IL-6 levels were also associated to higher risk of incident VaD (relative risk: 1.28, 95% CI: 1.03 to 1.59, I2: 0%). IL-6 in CSF was significantly higher in people with VaD compared to healthy subjects (SMD: 0.77, 95% CI: 0.17 to 1.37, I2: 70%), and not compared to AD patients, but due to limited evidence and high inconsistency across studies, we could not draw definite conclusion. Higher blood IL-6 levels might represent a useful biomarker able to differentiate people with VaD from those with AD and might be correlated with higher risk of future VaD.

Systemic treatment in advanced phyllodes tumor of the breast: a multi-institutional European retrospective case-series analyses.

BACKGROUND: We aimed at investigating outcome of systemic treatments in advanced breast PT. METHODS: All cases of advanced breast PT treated with systemic treatments from 1999 to 2019, in one of the referral sarcoma centers involved in the study, were retrospectively reviewed. RESULTS: 56 female patients were identified. Median age was 52 (range of 25-76) years. Patients received a median number of 2 systemic treatments (range of 1-4). Best responses according to RECIST were 1 (3.7%) CR, 11 (40.7%) PR, 6 (22.2%) SD, 9 (33.3%) PD with anthracyclines plus ifosfamide (AI); 2 (16.7%) PR, 4 (33.3%) SD, 6 (50.0%) PD with anthracycline alone; 3 (18.8%) PR, 4 (25.0%) SD, 9 (56.3%) PD with high-dose ifosfamide given as a continuous infusion (HD-IFX); 3 (20.0%) SD, 12 (80.0%) PD with a gemcitabine-based regimen (with 2 patients not evaluable); 1 (8.3%) PR, 2 (16.7%) SD, 9 (75.0%) PD with trabectedin (with 1 patient not evaluable); 1 (16.7%) PR, 1 (16.7%) SD, 4 (66.7%) PD with tyrosine-kinase inhibitors (TKI). The median PFS were 5.7 (IQR 2.5-9.1) months with AI; 3.2 (IQR 2.2-5.0) months with anthracycline alone; 3.4 (IQR 1.4-6.7) months with HD-IFX; 2.1 (IQR 1.4-5.2) months with gemcitabine-based chemotherapy; 1.8 (IQR 0.7-6.6) months with trabectedin; 3.4 (IQR 3.1-3.8) months with TKI. With a median follow-up of 35.3 (IQR 17.6-66.9) months, OS from the start of first-line systemic treatment was 15.2 (IQR 7.6-39.6) months. CONCLUSION: In this series of advanced PT (to our knowledge, the largest reported so far), AI was associated with a high rate of responses, however, with a median PFS of 5.7 months. Other systemic treatments were poorly active.

Mammographic density to predict response to neoadjuvant systemic breast cancer therapy.

BACKGROUND: Mammographic density (MD) is a risk factor for breast cancer (BC) development, and recurrence. However, its predictive value has been less studied. Herein, we challenged MD as a biomarker associated with response in patients treated with neoadjuvant therapy (NAT). METHODS: Data on all NAT treated BC patients prospectively collected in the registry of Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy (2009-2019) were identified. Diagnostic mammograms were used to evaluate and score MD as categorized by the Breast Imaging-Reporting and Data System (BI-RADS), which identifies 4 levels of MD in keeping with relative increase of fibro-glandular over fat tissue. Each case was classified according to the following categories a (MD < 25%), b (26-50%), c (51-75%), and d (> 75%). The association between MD and pathological complete response (pCR), i.e., absence of BC cells in surgical specimens, was analyzed in multivariable setting used logistic regression models with adjustment for clinical and pathological variables. RESULTS: A total of 442 patients were analyzed, 120 of which (27.1%) attained a pCR. BI-RADS categories a, b, c, and d accounted for 10.0%, 37.8%, 37.1% and 15.2% of cases. Corresponding pCR were 20.5%, 26.9%, 30.5%, 23.9%, respectively. At multivariable analysis, when compared to cases classified as BI-RADS a, those with denser breast showed an increased likelihood of pCR with odds ratio (OR) of 1.70, 2.79, and 1.47 for b, c and d categories, respectively (p = 0.0996), independently of age, BMI [OR underweight versus (vs) normal = 3.76], clinical nodal and tumor status (OR T1/Tx vs T4 = 3.87), molecular subtype (HER2-positive vs luminal = 10.74; triple-negative vs luminal = 8.19). In subgroup analyses, the association of MD with pCR was remarkable in triple-negative (ORs of b, c and d versus a: 1.85, 2.49 and 1.55, respectively) and HER2-positive BC cases (ORs 2.70, 3.23, and 1.16). CONCLUSION: Patients with dense breast are more likely to attain a pCR at net of other predictive factors. The potential of MD to assist decisions on BC management and as a stratification factor in neoadjuvant clinical trials should be considered.

European Reference Network for Rare Vascular Diseases (VASCERN) position statement on cerebral screening in adults and children with hereditary haemorrhagic telangiectasia (HHT).

Hereditary haemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia inherited as an autosomal dominant trait. Approximately 10 % of patients have cerebral vascular malformations, a proportion being cerebral arteriovenous malformations (AVMs) and fistulae that may lead to potentially devastating consequences in case of rupture. On the other hand, detection and treatment related-risks are not negligible, and immediate. While successful treatment can be undertaken in individual cases, current data do not support the treatment of unruptured AVMs, which also present a low risk of bleeding in HHT patients. Screening for these AVMs is therefore controversial.Structured discussions, distinctions of different cerebrovascular abnormalities commonly grouped into an "AVM" bracket, and clear guidance by neurosurgical and neurointerventional radiology colleagues enabled the European Reference Network for Rare Vascular Disorders (VASCERN-HHT) to develop the following agreed Position Statement on cerebral screening:1) First, we emphasise that neurological symptoms suggestive of cerebral AVMs in HHT patients should be investigated as in general neurological and emergency care practice. Similarly, if an AVM is found accidentally, management approaches should rely on expert discussions on a case-by-case basis and individual risk-benefit evaluation of all therapeutic possibilities for a specific lesion.2) The current evidence base does not favour the treatment of unruptured cerebral AVMs, and therefore cannot be used to support widespread screening of asymptomatic HHT patients.3) Individual situations encompass a wide range of personal, cultural and clinical states. In order to enable informed patient choice, and avoid conflicting advice, particularly arising from non-neurovascular interpretations of the evidence base, we suggest that all HHT patients should have the opportunity to discuss knowingly brain screening issues with their healthcare provider.4) Any screening discussions in asymptomatic individuals should be preceded by informed pre-test review of the latest evidence regarding preventative and therapeutic efficacies of any interventions. The possibility of harm due to detection of, or intervention on, a vascular malformation that would not have necessarily caused any consequence in later life should be stated explicitly.We consider this nuanced Position Statement provides a helpful, evidence-based framework for informed discussions between healthcare providers and patients in an emotionally charged area.

Older age and comorbidity in breast cancer: is RT alone the new therapeutic frontier?

AIM: To assess the impact of age, comorbidities and endocrine therapy (ET) in older breast cancer (BC) patients treated with hypofractionated radiotherapy (Hypo-RT). METHODS: From June 2009 to December 2017, we enrolled in this study 735 ER-positive BC patients (stage pT1-T2, pNx-1, M0 and age ≥ 65 years) receiving hypo-RT and followed them until September 2019. Baseline comorbidities included in the hypertension-augmented Charlson Comorbidity Index were retrospectively retrieved. Logistic regression model estimated adjusted-odds ratios (ORs) of ET prescription in relation to baseline patient and tumor characteristics. Competing risk analysis estimated 5-year cumulative incidence function (CIF) of ET discontinuation due to side effects (with BC progression or death as competing events), and its effect on locoregional recurrence (LRR) and distant metastasis (DM) (with death as competing event). RESULTS: ET has been prescribed in 89% patients. In multivariable analysis, the odds of ET prescription was significantly reduced in older patients (≥ 80 years, OR 0.08, 95% CI 0.03-0.20) and significantly increased in patients with moderate comorbidity. Patients ≥ 80 years discontinued the prescribed therapy earlier and more frequently than younger (65-69 years) patients (p = 0.060). Five-year CIF of LLR, DM and death from causes other that BC were 1.7%, 2.2% and 7.5%, respectively. Patients who discontinued ET had higher chance of LRR (p = 0.004). ET use did not impact on OS in any of the analyzed groups. CONCLUSIONS: In older patients, ET did not show a benefit in terms of overall survival. Further studies focusing on tailored treatment approaches are warranted to offer the best care in terms of adjuvant treatment to these patients.

Axillary nodal involvement by primary tumor features in early breast cancer: an analysis of 2600 patients.

BACKGROUND: Primary tumor characteristics, which are readily available to all clinicians, may aid in selecting the optimal adjuvant therapy for patients with breast cancer (BC). Herein, we investigated the relationship between tumor size, hormone receptor and HER2 status, Ki67 and age with axillary lymph node metastases (ALNM) in early-BC patients. METHODS: We analyzed data on consecutive 2600 early-BC cases collected in the registry of Fondazione IRCC Istituto Nazionale dei Tumori, Milano, Italy. Correlation between Ki67 and primary tumor size (T-size) was calculated by Spearman's rank correlation coefficient. Association of ALNM with Ki67 and other tumor characteristics was investigated by logistic regression. Adjusted odds ratios (ORs) with 95% confidence intervals (95% CIs) were estimated in all cases, and separately analyzed according to age, T-size and BC subtype. RESULTS: Large tumor size strongly associated to ALNM, with an adjusted odds ratio (OR) for each 5-mm increase of 1.32 (95% CI 1.24-1.41), except for triple-negative BC (TNBC) cases. In tumors =10 mm, without lymphovascular invasion, representing the strongest predictor of ALNM (OR 6.09, 95% CI 4.93-7.53), Ki67 resulted particularly informative, with a fourfold increased odds of ALNM for values > 30%. CONCLUSIONS: These results raise the question whether axillary node status is redundant in cases with exceptionally good features, i.e., small tumors with low Ki67, or in those candidate to adjuvant systemic treatment/radiotherapy anyway including TNBC, and support the incorporation of primary BC tumor characteristics as stratification factors in ongoing trials aiming at de-escalating axillary surgical procedures.

PROMs in post-mastectomy care: Patient self-reports (BREAST-Q™) as a powerful instrument to personalize medical services.

One of the goals of immediate breast reconstruction (IBR) is to satisfy the patient's outcome. Recent studies therefore tended to focus on the patient's perception of the care and on the impact on quality of life using patients-reported-outcome-measures (PROMs), able to measure the health status directly without the clinician's interposition. We present a preliminary prospective study on 333 patients who underwent mastectomy with IBR in a two-year period, in a single Italian centre, using a dedicated PROMs, the BREAST-Q™, to determine the patient's satisfaction. We studied two groups of IBR: Group A (two-step with tissue-expander) and Group B (one-step: prosthesis/mesh) and conducted a pre- and post-operative comparison for each group to evaluate score-gain over time, and a group-score comparison to determine whether differences were significant between reconstruction types. Two-hundred-and-nine were actually enrolled and 132 completed all the questionnaires. The response rate was 62.8% and the compliance rate (completion of all the questionnaires) was 63.1%. In both groups all the analyzed domains worsened comparing the pre and post-operative period; the differences were statistically significant only for physical and sexual-wellbeing. In the comparison between the two groups, none of the detected differences reached the statistical significance. According to our experience, we can state that PROMs could improve the health concept redefining the variables to be monitored even if data is still insufficient to draw any definitive conclusion. PROMs can help surgeons and patients decide the most appropriate surgery for a particular patient-profile and to identify those who require further support.

El impacto de los espacios formativos acerca de las competencias sociales y laborales en la carrera de medicina veterinaria

El presente trabajo da cuenta de una propuesta de espacios formativos destinados a la adquisición de habilidades sociales en los estudiantes próximos a finalizar la carrera de Medicina Veterinaria de la Facultad de Ciencias Veterinarias de la Universidad Nacional del Litoral, Argentina. Dichos espacios, brindados desde el Servicio de Orientación Educativa, se organizaron en cuatro talleres inspirados en la teoría de las habilidades sociales y laborales. Los cuatro ejes principales fueron autoconocimiento, inserción laboral, habilidades sociales y competencias laborales. Los cuatros ejes principales fueron: autoconocimiento, inserción laboral, habilidades sociales y competencias laborales. El valor pedagógico de la propuesta se evaluó a través de la aplicación de encuestas de opinión y valoraciones de frases significativas. La metodología de abordaje se caracterizó por ser cualitativa, consistente en una estrategia de intervención dentro del ámbito educativo. Algunos resultados preliminares dan cuenta de la necesidad de articular talleres de habilidades sociales a la estructura curricular de la formación universitaria. Los protagonistas de este proceso enfatizan la significatividad que aportan estos espacios como oportunidades válidas para su formación, a la vez que se transforman en instancias de aprendizaje compartido.

The present work accounts for a proposal of training spaces aimed at the acquisition of social skills in students close to finishing the career of Veterinary Medicine of the Faculty of Veterinary Sciences of Universidad Nacional del Litoral, Argentina. These spaces, provided by the Educational Guidance Service, were organized in four workshops inspired by the theory of social and work skills. The four main axes were self-knowledge, labor insertion, social skills and labor competencies. The four main axes were: self-knowledge, labor insertion, social skills and labor competencies. The pedagogical value of the proposal was evaluated through the application of opinion surveys and judgments of significant sentences. The approach methodology was characterized as being qualitative, consisting of an intervention strategy within the educational environment. Some preliminary results show the need to articulate social skills workshops to the curricular structure of university education. The protagonists of this process emphasize the significance that these spaces provide as valid opportunities for their formation, while they are transformed into instances of shared learning.

Management of breast cancer after Hodgkin's lymphoma and paediatric cancer.

RATIONALE: The risk of women developing a breast cancer (BC) after receiving chest radiotherapy for paediatric cancers and Hodgkin lymphomas is well established. The aim of this study was to assess these patients' clinical characteristics and clinical outcomes. METHODS: The study concerns women with a history of primary neoplasms treated with chest irradiation ± chemotherapy and subsequently diagnosed with BC. RESULTS: We identified 78 women who developed BC (invasive in 68 cases, 87%). They were a median 18 and 38 years of age when their first neoplasm and BC were diagnosed, respectively. Breast-conserving surgery was performed in 39 patients, and 32 underwent breast irradiation. Twenty of the 41 patients (49%) treated with chemotherapy received an anthracycline-containing regimen. The 5- and 11-year event free survival (EFS) and overall survival (OS) rates were 69% and 42%, respectively. Nine patients (12%) developed a third cancer and 18 (23%) a cardiovascular event. Of the 68 women with invasive BC, the first event involved contralateral BC in 55% of cases: time to progression (TTP) rates were 70% and 47% at 5 and 11 years. The 5- and 11-year BC-specific survival rates (BCSS) were 84% and 68%, respectively. CONCLUSIONS: Judging from our experience, survival rates after BC developing in women previously given chest radiotherapy are not dissimilar to those observed in other women with primary BC. Given the far from negligible risk of subsequent cancers and cardiovascular events, it is mandatory to discuss the best choice of treatment for such patients in terms of their chances of cure and quality of life, and also the risks of late sequelae.

Epidydimo-orchitis and anorectal malformations: when and in whom?

PURPOSE: Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. METHODS: A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. RESULTS: A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with recto-urethral (RU) fistula after reconstruction. The patients' age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). CONCLUSIONS: EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject.

Pulmonary Arteriovenous Malformations and Cerebral Abscess Recurrence in a Child With Hereditary Hemorrhagic Telangiectasia.

BACKGROUND: A 17-year-old boy was referred to our center with a history of brain abscess (BA) recurring after 9 years. The patient reported 2 previous treatments for pulmonary arteriovenous malformations, sporadic nosebleeds, and familial history for epistaxis. Clinical investigations revealed arteriovenous malformations in lung, brain, and liver, as well as mucocutaneous telangiectases. A definite diagnosis of hereditary hemorrhagic telangiectasia was made based on clinical criteria and confirmed by genetic analysis. This is the first report of BA recurrence at the end of pediatric age. CONCLUSIONS: The present case and the literature review of all cases of BA thus far reported highlight the need to raise the suspicion of a pulmonary arteriovenous malformations, both isolated and in the context of a possible hereditary hemorrhagic telangiectasia, for any case of BA of unexplained etiology, in children as well as in adults.

Selective axillary dissection after axillary reverse mapping to prevent breast-cancer-related lymphoedema.

BACKGROUND: It has recently been reported that, using axillary reverse mapping (ARM), the lymphatics from the arm can be spared to reduce the incidence of breast-cancer-related lymphoedema (BCRL). The aim of this study was to assess the feasibility of selective axillary dissection (SAD) after using ARM and partially preserving arm drainage, and to assess the occurrence of BCRL. METHODS: Using a radioisotope and lymphoscintigraphy, ARM was performed in 60 patients scheduled for SAD, who were subsequently divided for the purpose of comparing the BCRL rates into: group A, comprising 45 patients who successfully underwent SAD with a residual lymphatic hot spot; and group B with 15 whose hot nodes were removed as is normally the case during complete axillary lymph node dissection (ALND). RESULTS: SAD was feasible in 75% of the 60 patients. SAD was completed successfully in 19 of the first 30 patients, and in 26 of the second 30 patients (p = 0.072). The median follow-up was 16 months (6-36), during which 9 patients developed a BCRL, 4 in group A (9%) and 5 in group B (33%); p = 0.035. None of the patients had nodal relapses during the follow-up. CONCLUSIONS: Using a radioisotope enables an effective and safe SAD in a large proportion of patients. There was evidence of a trend to suggest a learning curve. The rate of BCRL after SAD was less than one third of the rate recorded after ALND, a result that should encourage the development of the former technique.

Use of a high-risk human papillomavirus DNA test as the primary test in a cervical cancer screening programme: a population-based cohort study.

OBJECTIVE: To present the results of the first 2 years of a human papillomavirus (HPV) test-based screening programme outside the research context. DESIGN: Population-based cohort study. SETTING: A cervical service screening programme in Italy. POPULATION: Women aged 25-64 years invited to screening from April 2009 to April 2011. METHODS: Eligible women were invited to undergo an HPV test: those with a negative HPV test went on to the next screening episode; those with a positive HPV went on to triage with a Pap smear. Women with positive cytology (i.e. positive for atypical squamous cells of undetermined significance or worse, ASC-US+) were referred to colposcopy, whereas those with negative cytology were referred to repeat HPV testing 1 year later. MAIN OUTCOME MEASURES: Participation rate, positivity at HPV and at triage, referral rate to colposcopy, positive predictive value for cervical intraepithelial neoplasia grade 2+ (CIN2+) at colposcopy, and detection rate for CIN2+. RESULTS: Participation increased compared with the previous Pap programme (60.6 versus 43.9%). The HPV positivity rate was 7.0; 39.6% of Pap smears were scored as positive, and therefore 2.8% of the women screened were referred for immediate colposcopy. The compliance of women who scored positive for HPV and negative for Pap for repeat HPV testing at 12 months was 78.6%, and the HPV positivity rate was 56.6%. The overall referral rate to colposcopy was 4.6%. The overall detection rate for CIN2+ was 4.5 versus 1.5% of the Pap programme (25-34 years, 8.2%; 35+ years, 3.6%). CONCLUSIONS: Compared with the traditional Pap test, the HPV programme recorded a higher response to invitation and an increased DR for CIN2+. The most critical aspects were the reading of cytology in women that were positive for HPV and the increased workload at colposcopy.

A long diagnostic delay in patients with Hereditary Haemorrhagic Telangiectasia: a questionnaire-based retrospective study.

BACKGROUND: The difficulty in establishing a timely correct diagnosis is a relevant matter of concern for several rare diseases. Many rare-disease-affected patients suffer from considerable diagnostic delay, mainly due to their poor knowledge among healthcare professionals, insufficient disease awareness among patients' families, and lack of promptly available diagnostic tools. Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal-dominantly inherited vascular dysplasia, affecting 1:5,000-10,000 patients. HHT is characterized by high variability of clinical manifestations, which show remarkable overlapping with several common diseases. AIM: To perform a detailed analysis concerning the diagnostic time lag occurring in patients with HHT, defined as the time period spanning from the first clinical manifestation to the attainment of a definite, correct diagnosis. METHODS: A questionnaire was administered to the HHT patients previously recruited from 2000 and 2009. Clinical onset, first referral to a physician for disease manifestations, and first correct diagnosis of definite HHT were collected. Eventual misdiagnosis at first referral and serious complications occurring throughout the time elapsing between disease onset and definite diagnosis were also addressed. RESULTS: In the 233 respondents, the clinical onset of disease occurred at an age of 14.1 yrs, while the age of first referral and the age of first definite diagnosis of HHT were 29.2 yrs and 40.1 yrs, respectively. Only 88/233 patients received a correct diagnosis at first counseling. Thus, the diagnostic time lag, represented by the time elapsing from disease onset and first definite diagnosis of HHT, proved to be 25.7 yrs. Twenty-two patients suffered from severe complications during this time interval. The diagnostic delay was significantly longer (p < 0.001) in index patients (first patients who attained definite HHT diagnosis in a given family) than in non-index patients (relative of index patients). The diagnostic time lag was also significantly associated with education grade (p < 0.001). CONCLUSIONS: Our data report for the first time a systematic inquiry of diagnostic delay in HHT showing that patients receive a definite diagnosis only after nearly three decades from disease onset. Concerted efforts are still to be made to increase awareness of this disease among both families and physicians.

Alergia al huevo en Pediatría. Documento del Comité de Pediatría de la Asociación Argentina de Alergia e Inmunología Clínica / Egg allergy in pediatrics. Pediatric Committee document of the Argentina Association of Allergy and Clinical Immunology

El huevo de gallina es una fuente de proteínas de alto valor biológico de bajo costo y de vitaminas del complejo B, importantes para la alimentación del niño. Culturalmente es uno de los alimentos básicos de nuestra dieta y, debido a esto, la alergia a sus proteínas es una de las más frecuentes en la infancia y tiene su mayor impacto en niños en edad preescolar. Estos niños representan una población vulnerable debido a que se encuentran en una etapa importante de su crecimiento y desarrollo, y el tratamiento de esta patología genera la adopción de dietas restrictivas que pueden impactar en forma negativa en su salud y calidad de vida. Este impacto está dado en parte por la ubicuidad de sus proteínas, que limita ampliamente la dieta y genera riesgos de reacciones alérgicas que se incrementan a medida que el niño crece y alcanza una mayor independencia. Teniendo en cuenta la importancia de esta patología, el Comité de Pediatría realizó una revisión actualizada con el fin de proveer herramientas útiles para el manejo adecuado de la misma. (AU)

Eggs are a source of low cost high biological value protein and complex B vitamins important for the child's nutrition. Culturally it is one of the staples of our diet and because of this, egg allergy is one of the most common food allergies in childhood and has its greatest impact on preschool children. These children represent a vulnerable population because they are at an important stage in their growth and development and the treatment of this condition generates the adoption of restrictive diets that may impact negatively on their health and quality of life. This impact is given in part by the ubiquity of their proteins that largely restrict the diet and generates risks of allergic reactions that increase as the child grows and earns greater independence. Given the importance of this issue the Pediatrics Committee conducted an updated review to provide useful tools to manage this condition.(AU)

[Inguinal hernia repair: an experimental study on mesh migration with the tension-free suturless technique. 10 year follow-up]. / Ernioplastica inguinale: studio sperimentale sulla mobilità protesica con tecnica tension-free suturless. Follow-up a dieci anni.

AIM: Can the tension-free suturless technique, used in the surgical treatment of inguinal hernia, to be the gold standard for treatment of inguinal hernia? METHODS: The tension-free suturless technique is often criticized as a fundamental principle: do not have suture. The criticism stems from concern that the mesh can migrate and cause damage to important anatomical structures. We conducted a study on the mobility of prosthesis on 33 patients, by using titanium clips that we have fixed on the meshes corner, X-rays over time, done at last, a follow-up of ten years. RESULTS: The study shows that the prosthesis moves together with the anatomical space in which there is the forces present in the inguinal canal: gravity, intra-abdominal pressure, reactive force ascending gait. Across thirty-three patients have relapsed in the first six months and two recurrences in ten years, in the reconstitution of the neo-orifice, through which passes the cord. In the remaining patients the mesh were relocated upward and medially (as identified by the clips of the increase of 10-15%). CONCLUSION: Our study shows that the mesh migrates upwards and medially. Migration is more or less, depending on the patient's age and quality of its tissue. Fix the prosthesis is good practice to secure at the flag on the inguinal ligament leads to two advantages: not to frustrate the principle tension-free, since the fixed prosthesis on one side does not create moments of tension, and prevent the prosthesis returns to the opening road to relapse.

VURD Syndrome in a Female.

VURD syndrome has been repeatedly described as unilateral reflux into a nonfunctioning renal moiety. This syndrome is considered a pop-off mechanism dissipating pressure in lower urinary tract obstruction: it may be found in association with other protective mechanisms occurring in utero, such as ascites and/or urinomas, and has been exclusively described in male patients. A premature female baby with signs and symptoms of outflow obstruction underwent diagnostic workup revealing congenital urethral hypoplasia with unilateral reflux into a dysplastic kidney. Obstetrical history was positive for early onset, serologically negative ascites without cardiomegaly, which required serial aspirations. Reconstructive surgery was carried out with good results: ascites and VURD syndrome were both deemed to be perinatal protective mechanism against excess pressure in the urinary tract. Although rare, lower urinary tract obstruction in the female can lead to the same protective mechanisms seen in male fetuses/newborns. VURD syndrome and ascites should be interpreted as such and require perinatal specialist counselling.

Estudio multicéntrico de evaluación del conocimiento de los pediatras acerca de reacciones alérgicas a vacunas para enfermedades infecciosas / A multicenter study assessing pediatrician's knowledge about allergic reactions to infectious disease vaccines

Resumen. Las reacciones alérgicas a las vacunas contra agentes infecciosos han generado preocupación entre los pediatras. Sin embargo, se desconoce el grado de información que tienen estos especialistas de nuestro país sobre este tema. Objetivo. Contar con datos estadísticos acerca de este problema. Población. Trescientos veinte pediatras encuestados. Método. Estudio multicéntrico descriptivo prospectivo de corte transversal realizado con encuestas estandarizadas Resultados. El 12,5% de los encuestados reconoció la presencia de síntomas de aparición rápida como reacción de hipersensibilidad inmediata. (61,6%) consideró a estas reacciones como infrecuentes. El 72,6% reconoció a la neomicina como causa de alergia, el 51,6 % al timerosal, el 73% a los conservantes, un 30,4% a la gelatina y la mitad de los encuestados al componente activo. El 62,3% reconoció a la proteína del huevo como componente de la vacuna MMR. Ante antecedentes de alergia al huevo, el 35% de los médicos contestó que contraindica siempre las vacunas que contienen proteína del huevo, el 14% no las contraindica nunca y el 9% no sabe. Los médicos de menos de 5 años de recibidos reconocieron con mayor frecuencia la presencia de una reacción alérgica a vacunas (p = 0,004). Los médicos de más de 10 años de recibidos solicitan más frecuentemente interconsulta con el especialista ante casos de vacunación de pacientes con alergia a la proteína del huevo (p = 0,01). Conclusiones. Existe un grado importante de desconocimiento acerca de las reacciones alérgicas a vacunas, los componentes de las vacunas involucrados en dichas reacciones y las conductas a tomar frente a pacientes con alergia al huevo.(AU)

Background: Allergic reactions to infectious disease vaccines have generated concern among pediatricians. It is unknown the level of pediatrician's knowledge about this issue. The aim of this study is to obtain statistical data about this issue in our country. Population: 320 pediatricians. Methods: A transversal prospective descriptive multicenter study by means of a survey. Results: 12.5% of participants were capable to identify symptoms of immediate hypersensitivity reactions and 61.6% considered that these reactions are not frequent. The pediatricians pointed out as the most commonly allergen components the following ones: Neomicine (72.6%), thymerosal (51.6 %), preservatives (73%), gelatin (30.4%) and active component (nearly 50%). 62.3% knew that eggs proteins are part of MMR vaccine. In the case of patient with history of egg allergy, 35% answered that they always contraindicate vaccination with egg protein vaccines while14% do not contraindicate and 9% do not know what to do. Physicians less than 5 years of graduation recognized more frequently the presence of allergic reactions (p: 0.004). Physicians with 10 or more years of graduation asked for specialist opinion more frequently in the case of patients with egg allergy (p: 0.01). Conclusions: It was found an important lack of information about allergic vaccine reactions, the involved vaccine constituents and the correct management of situations related to egg allergy.(AU)