Humanistic burden of haemophilia A without inhibitors: A cross-sectional analysis of the HemoLIFE study.

AIM: To evaluate the impact of haemophilia A without inhibitors on humanistic outcomes in patients and caregivers. Herein, we report a cross-sectional analysis of the baseline data of persons with haemophilia (PWH) participating in the prospective study HEMOLIFE. METHODS: These data are part of a prospective, observational, and multicentre study currently being conducted in 20 hospitals in Spain by haematologists. We included subjects 12 years or older diagnosed with haemophilia. The evaluations included the Maladjustment Scale, Haemophilia-Specific Quality of Life Questionnaire for Adults (HaemoQol)/HaemoQol Short Form (Children), haemophilia-specific version of the Work Productivity and Impairment Questionnaire plus the Classroom Impairment Questionnaire (WPAI+CIQ:HS), Haemophilia Activity List (HAL)/Paediatric Haemophilia Activities List (pedHAL), visual analogue scale (VAS) for evaluating pain, Coping Pain Questionnaire-Reduced (CAD-R), and Hospital Anxiety and Depression Scale (HADS). RESULTS: A total of 81 PWH were recruited at 18 centres; 66 PWH were ≥18 years (i.e., adults), and PWH 15 were <18 years (i.e., paediatric patients). Out of the 79 evaluable subjects, 16 (20%) showed an impact of haemophilia on daily life, and the areas most affected were "leisure time" (58% showed maladjustment) and "work/studies" (47% showed maladjustment). Patients reported a higher impact of haemophilia on quality of life (mean [SD] of the transformed score) in the dimensions of "sport" (49.4 [28.6]), "physical health" (40.5 [25.8]) and "future" (37.7 [28.9]). In adults, according to HAL scores, greater impairment of function was observed in "lying/sitting/kneeling/standing," "function of legs" and "leisure activities and sports," with mean normalized scores of 64.7, 65.1 and 69.0, respectively. Productivity was mostly impacted by presenteeism. The pain was infrequent and moderate. According to the HADS scores, nine (11.5%) patients had clinical anxiety and depression. CONCLUSION: PWH without inhibitors exhibited impairments in adjustment, quality of life and functionality, especially related to leisure and sports activities, and exhibit relevant levels of anxiety and depression.

Spatial and Temporal Dynamics of the Vegetation Cover from the Bijagual Massif, Boyacá, Colombia, during the 1986-2021 Period.

Landscape changes based on spectral responses allow showing plant cover changes through diversity, composition, and ecological connectivity. The spatial and temporal vegetation dynamics of the Bijagual Massif from 1986 to 2021 were analyzed as a measure of ecological integrity, conservation, and territory. The covers identified were high open forest (Hof), dense grassland of non-wooded mainland (Dgnm), a mosaic of pastures and crops (Mpc), lagoons (Lag), and bare and degraded lands (Bdl). The Bijagual Massif has 8574.1 ha. In 1986, Dgnm occupied 42.6% of the total area, followed by Mpc (32.8%) and Hof (24.5%); by 2000, Mpc and Hof increased (43.7 and 28.1%, respectively), while Dgnm decreased (28%); by 2021, Dgnm was restricted to the northeastern zone and continued to decrease (25.2%), Mpc occupied 52.9%, Hof 21.7% and Bdl 0.1%. Of the three fractions of the connectivity probability index, only dPCintra and dPCflux contribute to ecological connectivity. Hof and Dgnm show patches with biota habitat quality and availability. Between 1986 and 2021, Dgnm lost 1489 ha (41%) and Hof 239.5 ha (11%). Mpc replaced various covers (1722.2 ha; 38%) in 2021. Bijagual has a valuable biodiversity potential limited by Mpc. Territorial planning and sustainable agroecological and ecotourism proposals are required due to the context of the ecosystems.

EVALUATION OF MACULAR ATROPHY IN PATIENTS TREATED WITH MACULAR BUCKLE FOR MYOPIC TRACTION MACULOPATHY: Mid- and Long-Term Follow-Up.

PURPOSE: To evaluate the incidence, rate, and pattern of progression of myopic maculopathy in eyes operated with macular buckle (MB) for myopic traction maculopathy versus a control group without surgery to find out whether the progression varies due to the MB's indentation and to evaluate possible MB-related pigmentary changes or atrophy. METHODS: Eyes operated with MB with two good quality fundus images: one preoperative or early postoperative image and a second image at least 12 months apart; the control group comprised the contralateral eyes. Demographics, axial length, follow-up, stage of myopic traction maculopathy, and myopic maculopathy were reported. Progression results of groups and subgroups (mid- and long-term follow-up) were reported and compared. RESULTS: Overall, 116 eyes of 66 patients were included. Progression was found in 29 eyes (41.4%) and 23 eyes (50%) in the MB group and control group, respectively. The progression rate was 73 per 1,000 eye-years and 88.9 per 1,000 eye-years in the MB group and the control group, respectively. Axial length was found to predict progression (odds ratio [OR], 2.59; P = 0.02). CONCLUSION: Progression of myopic maculopathy was similar in both groups and was mildly greater in the control group. No MB-related pigmentary changes or atrophy was detected.

FRONTIER1: a partially randomized phase 2 study assessing the safety, pharmacokinetics, and pharmacodynamics of Mim8, a factor VIIIa mimetic.

BACKGROUND: Mim8 (denecimig) is a factor VIII (FVIII) mimetic bispecific antibody in development for the treatment of hemophilia. Data from the phase 1 part of FRONTIER1 (EudraCT: 2019-000465-20, NCT04204408, and NN7769-4513) suggested that Mim8 was well tolerated in healthy participants and exhibited pharmacokinetic (PK) properties consistent with dose proportionality. OBJECTIVES: The partially randomized, phase 2, multiple ascending dose (MAD) part of FRONTIER1 aimed to evaluate the safety, PK, pharmacodynamics (PD), and exploratory efficacy of Mim8 in participants with hemophilia A with or without FVIII inhibitors. METHODS: The MAD part of FRONTIER1 consisted of 42 participants, assigned to 5 cohorts, with participants in cohorts 3 and 4 randomized 1:1 to dosing weekly or every 4 weeks, respectively. Four of the 42 participants (9.5%) had FVIII inhibitors prior to study enrolment. The primary endpoint was treatment-emergent adverse events (TEAEs). PK and PD were evaluated by Mim8 plasma concentration and thrombin generation, respectively. Exploratory efficacy was assessed via the number of treated bleeds. Safety and PD parameters were also evaluated from an exploratory cohort treated with emicizumab. RESULTS: Mim8 was well tolerated, with 1 serious TEAE (anxiety-related chest pain) deemed unrelated to Mim8. There was no dose dependency on the number, causality, type, or severity of TEAEs. PK/PD properties supported weekly to monthly dosing approaches, and few participants experienced treated bleeds beyond the lowest dose cohort (1 in cohorts 2 and 3, and 3 in cohort 5). CONCLUSION: These data support the continued clinical development of Mim8, and FRONTIER1 has proceeded onto an extension phase.

Strengthening Privacy and Data Security in Biomedical Microelectromechanical Systems by IoT Communication Security and Protection in Smart Healthcare.

Biomedical Microelectromechanical Systems (BioMEMS) serve as a crucial catalyst in enhancing IoT communication security and safeguarding smart healthcare systems. Situated at the nexus of advanced technology and healthcare, BioMEMS are instrumental in pioneering personalized diagnostics, monitoring, and therapeutic applications. Nonetheless, this integration brings forth a complex array of security and privacy challenges intrinsic to IoT communications within smart healthcare ecosystems, demanding comprehensive scrutiny. In this manuscript, we embark on an extensive analysis of the intricate security terrain associated with IoT communications in the realm of BioMEMS, addressing a spectrum of vulnerabilities that spans cyber threats, data manipulation, and interception of communications. The integration of real-world case studies serves to illuminate the direct repercussions of security breaches within smart healthcare systems, highlighting the imperative to safeguard both patient safety and the integrity of medical data. We delve into a suite of security solutions, encompassing rigorous authentication processes, data encryption, designs resistant to attacks, and continuous monitoring mechanisms, all tailored to fortify BioMEMS in the face of ever-evolving threats within smart healthcare environments. Furthermore, the paper underscores the vital role of ethical and regulatory considerations, emphasizing the need to uphold patient autonomy, ensure the confidentiality of data, and maintain equitable access to healthcare in the context of IoT communication security. Looking forward, we explore the impending landscape of BioMEMS security as it intertwines with emerging technologies such as AI-driven diagnostics, quantum computing, and genomic integration, anticipating potential challenges and strategizing for the future. In doing so, this paper highlights the paramount importance of adopting an integrated approach that seamlessly blends technological innovation, ethical foresight, and collaborative ingenuity, thereby steering BioMEMS towards a secure and resilient future within smart healthcare systems, in the ambit of IoT communication security and protection.

Rationale for combined therapies in severe-to-critical COVID-19 patients.

An unprecedented global social and economic impact as well as a significant number of fatalities have been brought on by the coronavirus disease 2019 (COVID-19), produced by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Acute SARS-CoV-2 infection can, in certain situations, cause immunological abnormalities, leading to an anomalous innate and adaptive immune response. While most patients only experience mild symptoms and recover without the need for mechanical ventilation, a substantial percentage of those who are affected develop severe respiratory illness, which can be fatal. The absence of effective therapies when disease progresses to a very severe condition coupled with the incomplete understanding of COVID-19's pathogenesis triggers the need to develop innovative therapeutic approaches for patients at high risk of mortality. As a result, we investigate the potential contribution of promising combinatorial cell therapy to prevent death in critical patients.

Excellent hemostatic control during cardiac surgery in a patient with hemophilia B treated with albutrepenonacog alfa (rIX-FP): A case report.

Key Clinical Message: In hemophilia patients undergoing cardiac surgery, ROTEM® and Quantra® viscoelastic tests are useful to monitor perioperative hemostatic status and a single dose of rIX-FP is safe and avoids any hemorrhagic or thrombotic complication. Abstract: Cardiac surgery poses a high hemostatic risk in patients with hemophilia. We present the first case of an adult patient with hemophilia B on treatment with albutrepenonacog alfa (rIX-FP) who underwent surgery for acute coronary syndrome. Treatment with rIX-FP made it possible to perform the surgery safely.

Expert opinion paper on the treatment of hemophilia a with emicizumab.

INTRODUCTION: Prophylaxis with emicizumab, a bispecific monoclonal antibody that mimics FVIII function, has shown encouraging results in clinical trials in terms of efficacy and safety. However, current experience is limited, and many areas of concern to clinicians have yet to be reviewed. AREAS COVERED: This paper reviews the experience of hemophilia A patients treated with emicizumab based on the results of clinical trials and real-life studies. The authors place special emphasis on issues such as the management of these patients in situations of hemorrhage and/or surgical interventions, joint health or laboratory monitoring. EXPERT OPINION: Treatment with emicizumab has been shown to improve joint health and reduce bleeding, of particular interest to patients with inhibitors and high bleeding rates. However, there are still concerns about its administration in neonates and previously untreated patients due to limited reported experience. Laboratory monitoring is not strictly necessary due to the stable pharmacokinetics emicizumab has been shown to exhibit, however, tests that globally assess hemostasis may be useful especially in cases of bleeding or surgery. The authors are also of the opinion that prophylaxis before minor surgery is not necessary and that major surgeries can be safely performed with additional prophylactic coagulation factor.Trial registration ClinicalTrials.gov identifier: NCT04431726..

Clot Stiffness Measured By Seer Sonorheometry As a Marker Of Poor Prognosis In Hospitalized COVID-19 Patients.

A high risk of thrombotic complications has been observed among severely ill COVID-19 patients. Viscoelastic tests (VET) have shown a hypercoagulable profile in these patients, although so far there is no clear evidence on the use of these tools as predictors of risk in the clinical course of patients. In this study we aimed to evaluate the association between Quantra® sonorheometry VET parameters, standard coagulation tests and inflammatory markers in 69 patients with COVID-19 on hospital admission with disease severity and outcome. Inflammatory markers were elevated in a high percentage of patients, as were coagulation-related parameters such as fibrinogen and D-dimer levels. Quantra® sonorheometry analysis revealed increased clot stiffness (CS), especially due to increased fibrinogen contribution (FCS) in 63.7%. Analysis of clot stability to lysis (CSL) on the Quantra showed a value of 100%, suggesting hypofibrinolysis, in 32.4%. Age > 65 years, elevated values of fibrinogen, D-dimer, LDH, increased CS and CSL were significantly associated with worsening disease. The combination of elevated FCS and D-dimer values showed a particularly high prognostic value in distinguishing patients with severe symptomatology. In conclusion, FCS measured by Quantra® system and its combination with D-dimer could be established as a powerful tool to identify poor prognosis in COVID-19 patients on hospital admission.

Normal myocardial perfusion despite a very high coronary calcium score.

An exceptionally high coronary calcium score, greater than 10,000 UA, superior to any other found in the literature reviewed, was reported in an asymptomatic, adult man with hypertension, obesity and dyslipidemia, without myocardial ischemia and no significative coronary stenosis, associated to Glagov's phenomenon in the left coronary artery and an abdominal aortic aneurysm.

Expert opinion paper on the treatment of hemophilia B with albutrepenonacog alfa.

Introduction: Current guidelines recommend prophylactic treatment of hemophilia B with the missing coagulation factor IX, either with standard half-life or extended half-life products. Extended half-life products have half-lives three to six times longer than the former, allowing a reduction in the number of weekly injections and therefore, potentially impacting on treatment adherence and quality of life. Albutrepenonacog alfa is an extended half-life fusion protein of coagulation factor IX with recombinant human albumin, indicated for both on-demand and prophylactic treatment for bleeding in patients with hemophilia B of all ages.Areas covered: The authors review the clinical and pharmacokinetic characteristics of albutrepenonacog alfa, as well as the available information regarding trough levels and real-world evidence. Given the availability of other factor IX products in the market, indirect comparisons of clinical and pharmacokinetic characteristics are presented.Expert opinion: The authors exhibit their expert opinion on which patient profiles are candidates for prophylactic treatment with albutrepenonacog alfa, and on the management of patients in terms of dosing, regimens of administration and protocols for switching the treatment.

Incidence, characteristics and clinical profile of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in patients with pre-existing primary immune thrombocytopenia (ITP) in Spain.

Infections are one of the well-known precipitating factors for relapses in patients with immune thrombocytopenia (ITP). Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection can sometimes lead to or be associated with thrombocytopenia due to an increase in peripheral platelet destruction from inflammatory hyperactivation. Currently, we do not know if SARS-CoV-2 infection modifies the natural evolution of chronic or persistent ITP or if previous immunosuppression of patients with ITP influences the incidence and severity of coronavirus disease 2019 (COVID-19) in this group. The present study was an observational, multicentre, national series of 32 adult patients with pre-existing ITP and subsequent SARS-CoV-2 infection, collected by the Spanish ITP Group [Grupo Español de Trombocitopenia Inmune (GEPTI)].

Pulpotomía utilizando extracto de orégano como alternativa en el tratamiento de dientes temporales / Pulpotomy using oregan extract as an alternative in the treatment of temporary teeth

El formocresol es uno de las sustancias más empleadas para la terapia dental de la pulpotomía en la Odontpediatría debido a sus efectos tóxicos, genotóxicos y mutagénicos; por dicha razón es que se está constantemente buscando nuevas alternativas para dicho tratamiento con el uso de distintas sustancias o medicamentos. Este es un estudio experimental básico en donde se recolectaron 20 muestras de órganos dentarios de pacientes con edades que oscilaban entre los tres y nueve años de la Clínica de Odontopediatría de Pregrado de la Facultad de Estomatología de la U. A. S. L. P. que cumplieron con los criterios de inclusión para el tratamiento de pulpotomías. El tratamiento se realizó de acuerdo con los lineamientos de la AAPD, con excepción de que se utilizó extracto hidroalcohólico 70:30 de orégano y se midió en el laboratorio su capacidad para eliminar microorganismos.

Formocresol is one of the most widely used drugs for the treatment of pulpotomy for the reason in Pediatric Dentistry; due to its toxic, genotoxic and mutagenic effects, it is constantly searching for alternatives with other substances or drugs. This is a basic experimental study in twenty samples collected from dental organs of patients with ages ranging from three to nine years of the Pediatric Dentistry Clinic of the Faculty of Stomatology of the UASLP met the inclusion criteria for the treatment of pulpotomies. The treatment was performed according to those established by AAPD with the exception that hydroalcoholic extract 70:30 of oregano was used and its ability to eliminate microorganisms was measured in the laboratory.

Risk of Recurrent Venous Thromboembolism in Autoimmune Diseases: A Systematic Review of the Literature.

Despite an abundance of literature on the risk of a first venous thromboembolic event (VTE) in autoimmune diseases, specific recommendations about managing VTE in autoimmune diseases are lacking. This article aimed to collect evidence on the risk of recurrent VTE in patients with autoimmune diseases. The authors searched PubMed/Embase for studies including patients with VTE and autoimmune diseases as an exposure or studies including patients with autoimmune diseases in which recurrent VTE was one of the outcomes. Eleven articles were selected from 4,739 unique abstracts. Of the 11 studies, 3 reported time-dependent rates. Two studies collected rates of recurrence in Behcet's disease, reporting a 5-year recurrence risk between 35 and 40%. However, the 5-year recurrence risk was lower than 10% in patients treated with immunosuppressant medication, while two studies suggested frequent recurrence in patients on only anticoagulant therapy. The other study reporting time-dependent incidence concerned patients with inflammatory bowel disease and index VTE. The 5-year risk of recurrent VTE was 33.4%, yielding a hazard ratio of 1.7 versus controls. All studies were retrospective and therefore risk may overestimate recurrence risk in comparison with known prospective cohort studies. There are insufficient data to make confident recommendations about the management of recurrent VTE prevention in patients with autoimmune diseases in general. The overall VTE risk profile, lower effectiveness of anticoagulants, and the observation that immunosuppression lowered risk of recurrence in patients with Behcet's disease seem to warrant immunosuppressant therapy over anticoagulation as a first consideration when preventing VTE recurrence in these patients.

Risk factors for postobstructive diuresis in pediatric patients with ureteropelvic junction obstruction, following open pyeloplasty in three high complexity institutions.

INTRODUCTION: Postobstructive diuresis (POD) is a polyuric state in which large quantities of salt and water are eliminated after solving a urinary tract obstruction. These patients are at increased risk of severe dehydration, electrolytic disturbances, hypovolemic shock, and death. Ureteropelvic junction obstruction (UPJO) is the most common etiology of collecting system dilatation in the fetal kidney, and a significant number of patients require pyeloplasty. There are limited data regarding prognostic risk factors for POD in this scenario. OBJECTIVE: To describe possible clinical risk factors for POD in the pediatric population after open pyeloplasty. STUDY DESIGN: This was a retrospective case series study of consecutive patients diagnosed with UPJO at three high complexity centers, managed with open pyeloplasty from 2006 to 2016. Multiple qualitative and quantitative variables possibly associated with POD were included according to the literature review. They were statistically analyzed with STATA 14 software. RESULTS: A total of 88 patients with UPJO following open pyeloplasty were analyzed. Twenty-seven patients (30%) had POD. A tendency to present POD in younger patients was found, with a mean age of 20.2 months vs. 72.3 months. There was also an increased risk of POD in patients with previous diagnosis of tubular acidosis. CONCLUSIONS: There are no data about prognostic clinical risk factors for POD after open pyeloplasty in the pediatric population. Our study corresponds to one of the larger series reported so far. It suggests that younger patients and patients with a previous diagnosis of tubular acidosis could be at greater risk of POD. Consequently, prospective studies are required for validation of our results, and possible impact on patient follow-up.

Pene no Conspicuo (Oculto) / Inconspicuous Penis

El término pene no conspicuo (oculto), agrupa las patologías en las que el pene adopta un tamaño aparentemente más pequeño, ya sea porque se oculta bajo el tejido cicatricial en caso del pene atrapado, cuando presenta un pliegue de piel que reduce el ángulo penoescrotal tratándose del pene en vela, o en los casos en los que el pene es cubierto por prepucio redundante produciendo un pene enterrado. Se realiza una revisión de la definición y terminología utilizada a través de la historia, clasificación actual, etiología, diagnóstico y técnica quirúrgica empleada para la corrección del pene no conspicuo (oculto).

Inconspicuous penis is referred conditions where the penis looks apparently small but is truely hidden under scarring tissue or under redundant mucosa or prepubic fat. These are all different pathologies that are surgically treated. Multiple techniques have been described. The aim of the present article is to review the state of the art in management throughout history, current classification, etiology, diagnosis and surgical techniques for correcting this pathology.

Experiencia quirúrgica en el manejo de litiasis urinaria en la población pediátrica: estudio multicéntrico / Surgical Experience in Management of Urinary Lithiasis in Pediatric Population: Multicentric Study

La incidencia de la enfermedad litiásica ha aumentado globalmente en los últimos 25 años. A pesar de que la incidencia de litiasis es baja en la población pediátrica,1 ese aumento no ha sido ajeno a esa población. Se ha visto un incremento importante en su incidencia y prevalencia en los últimos años, alcanzado en países como Estados Unidos de 18,5 por cada 100.000 habitantes.2 La edad de presentación se encuentra entre los 7 y los 8 años de edad.

The incidence of lithium disease has increased globally over the past 25 years. Although the incidence of lithiasis is low in the paediatric population,1 this increase has not been alien to the paediatric population. There has been a significant increase in its incidence and prevalence in recent years, reached in countries such as the United States of 18.5 per 100,000 inhabitants.2 The age of presentation is between 7 and 8 years of age

Descripción de factores clínicos asociados a la torsión testicular / Description of Clinical Factors Associated with Testicular Torsion

El escroto agudo es una de las pocas condiciones urológicas que requiere intervención inmediata.1 La principal causa de escroto agudo es la torsión testicular y afecta a 4,5 de cada 100.000 hombres menores de 25 años.2 Se presenta por una predisposición dada por un defecto congénito de la inserción del testículo al escroto mediante un gubernaculum testis largo y/o con una implantación posterior que le permite al testículo girar libremente sobre su propio eje

The acute scrotum is one of the few urological conditions requiring immediate intervention.1 The main cause of acute scrotum is testicular torsion and affects 4.5 out of every 100,000 men under the age of 25.2 It arises from a predisposition given by a congenital defect of insertion of the testicle into the scrotum by means of a long gubernaculum testis and/or with a subsequent implantation that allows the testicle to rotate freely on its own axis.

Experiencia en el abordaje de pacientes con complejo extrofia-epispadias en un centro de alto nivel de complejidad en Colombia, 10 años / 10-years experience in the management of bladder exstrophy-epispadias complex in a tertiary care health centre in Colombia

Objetivos El complejo extrofia-epispadias (CEE) se considera una de las malformaciones más severas de la línea media de compromiso multisistémico. La extrofia vesical es la presentación más frecuente en el espectro del complejo. Esta patología tiene un alto impacto en la calidad de vida. A pesar de la relación entre un cierre primario temprano y mejores resultados, en nuestro medio la remisión es tardía y la experiencia es escasa. El objetivo del siguiente estudio es mostrar la experiencia en el abordaje de CEE en los últimos 10 años en una institución de alto nivel de complejidad y remisión en Colombia. Materiales y métodos Se realiza un estudio observacional descriptivo, con una serie de casos del 2006 al 2016. Resultados En 10 años, se presentaron 5 casos de CEE en un centro de alta complejidad y remisión en Colombia. La mayoría de los pacientes han tenido múltiples intervenciones; la edad del primer procedimiento fue 829 días en promedio (27,6 meses). Se ha tenido un seguimiento postoperatorio promedio de 2,8 años. No se han presentado neoplasias en el seguimiento. Las comorbilidades más frecuentes son infección y litiasis. Ninguno de los pacientes contactados reportó inicio de vida sexual. La escala International Consultation on Incontinence Questionnaire-Urinary Incontinence-Short Form (ICIQ-UI-SF) tuvo un promedio de 9 puntos. Existen factores sociales asociados en nuestro medio. Conclusión El CEE requiere un abordaje temprano y multidisciplinario en instituciones con experiencia; los resultados en continencia urinaria, función sexual, desarrollo psicosocial y calidad de vida están sujetos a tratamiento oportuno de la patología.

Objective Exstrophy-epispadias complex (ECC) is considered one of the most severe midline abnormalities. Exstrophy of the bladder is the most frequent presentation of the exstrophy-epispadias spectrum. The disease has high impact on the quality of life. In developing countries, patient referral to experienced centres is often delayed. The experience in the disease is generally poor. The aim of this study is to describe the experience in the approach to EEC in the past 10 years in an institution of high level of complexity in Colombia. Materials and methods An observational descriptive study of a case series. Results A total of 5 cases of ECC were identified in the last 10 years in an institution of high level of complexity in Colombia. All the patients had multiple interventions. The mean follow-up time was 2.8 years. No neoplasms were recognised. The most common comorbidities were lithiasis and urinary tract infections. None of the patients contacted reported starting a sex life. The International Consultation on Incontinence Questionnaire-Urinary Incontinence-Short Form (ICIQ-UC-SF) gave a mean of 9 points. Conclusion The EEC requires an early and cross-disciplinary assessment in experienced centres. There are additional factors associated with the prognosis in our area.

Using 3D Physical Modeling to Plan Surgical Corrections of Complex Congenital Heart Defects.

Background Understanding the anatomy and physiology of congenital heart defects is crucial for planning interventions in these patients. Congenital heart procedures often involve complex three-dimensional (3D) reconstructions. Excellent imaging techniques are required to depict all anatomical details. We have used and evaluated fast 3D prototyping technology for reconstruction and planning of corrections of complex congenital heart defects. Materials and Methods 3D physical models were constructed from contrast-enhanced computed tomography (CT) datasets of patients with complex congenital heart defect. Two different commercially available printing technologies were used and their clinical application compared. Results Physical models of three different patients were used for preoperative surgical planning. All models showed good correspondence to patient anatomy. Both printing technologies gave excellent results. Conclusion Physical models could be easily constructed with the use of CT datasets. The printing process could be done efficiently, quite rapidly, and cost effectively. Surgical corrections could be planned based on these models.