3D printed soft surgical planning prototype for a biliary tract rhabdomyosarcoma.

Biliary tract rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor which is located in the biliary tract. Although this tumor represents less than 1% of the total amount of childhood cancers, when localized, a >70% overall 5-year survival rate, the resection is clinically challenging and complications might exist during the biliary obstruction. Although surgery remains a mainstay, complete tumor resection is generally difficult to achieve without mutilation and severe long-term sequelae. Therefore, manufacturing multi-material 3D surgical planning prototypes of the case provides a great opportunity for surgeons to learn beforehand what they can expect. Additionally, practicing before the operation enhances the probability of success. That is why different compositions of materials have been characterized to match the mechanical properties of the liver. To do this, Dynamic Mechanical Analysis (DMA) tests and Shore hardness tests have been carried out. Amongst the material samples produced, 6%wt PVA (poly vinyl alcohol)/1%wt PHY (Phytagel)-1FT (Freeze-Thaw cycles) and 1%wt agarose appear as the best options for mimicking the liver tissue in terms of viscoelasticity. Regarding the Shore hardness, the best solution is 1%wt agarose. Additionally, a surgical planning prototype using this last material mentioned was manufactured and validated using a CT (Computed Tomography) scanner. In most of the structures the difference between the 3D model and the organ in terms of dimensions is less than 3.35 mm, which represents a low dimensional error, around 1%. On the other hand, the total manufacturing cost of the 3D physical model was €513 which is relatively low in comparison with other technologies.

Associations between neural injury markers of intrauterine growth-restricted infants and neurodevelopment at 2 years of age.

Objectives: The aim of this study was to evaluate the relationships between brain injury biomarkers in intrauterine growth-restricted (IUGR) infants (S100B and neuron-specific enolase (NSE)) and neurodevelopment at 2 years of age. Methods: This prospective case-control study was a cooperative effort among Spanish Maternal and Child Health Network (Retic SAMID) hospitals. At inclusion, biometry for estimated fetal weight and feto-placental Doppler variables were measured for each infant. Maternal venous blood and fetal umbilical arterial blood samples were collected at the time of delivery and neural injury markers S100B and NSE concentrations were measured. Neurodevelopment was evaluated at 2 years of age using the Bayley Scales of Infant and Toddler Development, third edition (Bayley-III). Results: Fifty six pregnancies were included. Thirty-one infants were classified as IUGR and 25 as non-IUGR. Neurodevelopmental evaluation at 2 years of age indicated that there were no between-group differences for any of the tests. For all patients in both groups, we found statistically significant inverse relationships between the concentrations of NSE in the cord blood and the results of the cognitive test (r = -271, p = .042), fine motor subtest (r = -280, p = .036), and social-emotional test (r = -349, p = .015). We also found statistically significant differences between the concentrations of S100B in the cord blood and the results of the cognitive test (r = -306, p = .022) and expressive communication subtest (r = -304, p = .023). For the IUGR group, we found a significant inverse relationship between the concentrations of S100B in the maternal serum and the results of adaptive behavior test (p < .05). In the non-IUGR group, we found statistically significant inverse relationships between the concentration of NSE in the cord blood and the results of the fine motor subtest (r = -446, p = .025) and social-emotional test (r = -489, p = .021). The difference between the concentration of S100B in the cord blood and the language composite score was also statistically significant (p = .038). Conclusions: At 2 years of age, the concentrations of NSE and S100B were higher in the non-IUGR and IUGR groups with the worst scores for some areas of neurodevelopmental evaluation. The value of these biomarkers for prognostic neurodevelopmental use requires further investigation for both non-IUGR and IUGR infants.

Lupus eritematoso con afectación genital: una localización inusual / Lupus Erythematosus Affecting the Genitalia: An Unusual Site

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Múltiples pápulas en ambas manos / Múltiple Paules on Both Hands

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Neural injury markers in intrauterine growth restriction and their relation to perinatal outcomes.

BACKGROUNDThe aims of this study were to (i) compare the concentrations of two neural injury markers, S100B protein and neuron-specific enolase (NSE), in intrauterine growth-restricted (IUGR) fetuses and in fetuses with appropriate growth-for-gestational-age (AGA), and (ii) investigate potential relationships between concentrations of these markers, Doppler abnormalities, and adverse perinatal or neonatal outcomes.METHODSThis was a case-controlled, cooperative, prospective study among Spanish Maternal and Child Health Network (Retic SAMID) hospitals. At inclusion, biometry for estimated fetal weight and feto-placental Doppler were measured. At the time of delivery, maternal venous blood and fetal umbilical arterial blood samples were collected. S100B and NSE concentrations were determined from these samples.RESULTSIn total, 254 pregnancies were included. Among these, 147 were classified as IUGR and 107 as AGA. There were no differences between the groups in S100B concentrations. However, levels of NSE in maternal and umbilical cord serum differed significantly between these groups (2.31 in AGA vs. 2.51 in IUGR in (P<0.05); and 2.89 in AGA vs. 3.25 in IUGR (P<0.05), respectively). No differences were observed in these neurological markers when stratified by perinatal or neonatal complications.CONCLUSIONAlthough some variations exist in these neurological markers, they did not correlate with perinatal or neonatal complications.

Evaluation of the bedside Quikread go® CRP test in the management of febrile infants at the emergency department.

Recently C-reactive protein (CRP) point-of-care tests have been developed. We aimed to validate a bedside CRP test (QuikRead go® CRP), to compare it with the laboratory CRP (ARCHITECT c8000 Abbott, Germany) test in children with fever without source (FWS), and to evaluate the optimal CRP cut-off value to identify those patients at a high risk for serious bacterial infection (SBI). The CRP bedside test was prospectively performed in capillary blood samples concurrently with the laboratory CRP testing for 283 well-appearing infants aged 1 to 24 months with FWS attending the emergency department (ED) between May 2013 and August 2015. The mean difference between the laboratory CRP and the QuikRead go CRP values was 0.71 mg/L (p = 0.444). Pearson's correlation coefficient between the CRPs was r = 0.929 (p < 0.001). SBI was diagnosed in 34 patients (12.0%). The area under the receiver operating characteristics (ROC) curve obtained was 0.87 (95%CI: 0.82-0.90) for an optimal CRP cut-off value of > 10 mg/L (sensitivity: 94.1%, specificity: 49.0%, positive predictive value: 20.1%, negative predictive value: 98.4%), as a predictor of SBI. Nearly 45% of the patients were at a low risk for SBI according to CRP value; thus, additional laboratory tests would have been hypothetically avoided. There was a very strong, positive correlation between the QuikRead go CRP test and laboratory CRP determination. The QuikRead go CRP test provides reliable results to rule out SBI. Its implementation at the ED would improve the management of infants with FWS.

Alopecia frontal fibrosante en el varón: presentación de 12 casos y revisión de la literatura / Frontal fibrosing alopecia in men: presentations in 12 cases and a review of the literature

INTRODUCCIÓN: La alopecia frontal fibrosante (AFF) es una alopecia cicatricial caracterizada por el retroceso de la línea de implantación del pelo, asociada a alopecia de cejas. Habitualmente afecta a mujeres en edad posmenopáusica, siendo mucho menos prevalente en varones. OBJETIVO: Describir las características clínicas de la AFF en los hombres estudiados y compararlos con los datos recogidos en la literatura. MATERIAL Y MÉTODOS: Se realizó un estudio descriptivo de los varones diagnosticados de AFF en nuestro Servicio, desde enero del 2010 hasta diciembre del 2015. Se recogieron los datos demográficos, las características clínicas y los tratamientos realizados. RESULTADOS: Se reclutó a 12 pacientes. La edad media fue de 75 años. La alopecia fue el motivo de consulta únicamente en 4 pacientes. El retroceso medio de la línea de implantación del pelo fue de 3cm. Las pápulas faciales estaban presentes en el 50% de los hombres, el 83% presentaba alopecia de cejas, extremidades y alopecia androgenética (AGA). El eritema y la hiperqueratosis folicular se veían en el 66% de los casos y solo el 25% refería prurito. El tratamiento más frecuentemente utilizado consistió en corticoide tópico en 8 pacientes (66%), asociado a minoxidil tópico en 4 de ellos (33%). CONCLUSIONES: Según los datos obtenidos en nuestra serie, las pápulas faciales, la AGA y la afectación del vello corporal son más frecuentes en los hombres con AFF que en las mujeres. Por otra parte, a diferencia de los casos de AFF en varones descritos en la literatura, la edad media es mayor en nuestra serie, lo que podría explicar la mayor incidencia de AGA asociada y que la mayoría consulte por otro motivo

BACKGROUND: Frontal fibrosing alopecia (FFA) is a scarring disease in which the hairline recedes and the eyebrows can be affected. Usually seen in postmenopausal women, FFA is much less common in men. OBJECTIVE: To describe the clinical characteristics of FFA in a case series of men and compare this series to those reported in the literature. MATERIAL AND METHODS: Men with FFA being treated in our dermatology department from January 2010 to December 2015 were included prospectively for this descriptive study. We collected patient information and clincal and treatment characteristics. RESULTS: Twelve men (mean age, 75 years) were recruited. Alopecia was the reason for seeking medical care in only 4 cases. The hairline had receded 3cm on average. Half the patients had facial papules, and 83% had androgenetic alopecia or hair loss on eyebrows or extremities. Follicular hyperkeratosis and erythema were present in 66%, and only 25% of the men reported pruritus. The most commonly prescribed treatments were topical: corticosteroids in 8 patients (66%) and minoxidil in 4 (33%). CONCLUSIONS: Facial papules, androgenetic alopecia, and loss of body hair are more often observed in men with FFA than in women. The men in this series were older on average than in other FFA case series in the literature, possibly accounting for the higher prevalence of associated androgenetic alopecia and the fact that most of these men were seeking care for conditions other than hair loss

Calidad de los informes de alta hospitalaria por embolia pulmonar / Quality of pulmonary embolism's hospital discharge reports

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Frontal Fibrosing Alopecia in Men: Presentations in 12 Cases and a Review of the Literature. / Alopecia frontal fibrosante en el varón: presentación de 12 casos y revisión de la literatura.

BACKGROUND: Frontal fibrosing alopecia (FFA) is a scarring disease in which the hairline recedes and the eyebrows can be affected. Usually seen in postmenopausal women, FFA is much less common in men. OBJECTIVE: To describe the clinical characteristics of FFA in a case series of men and compare this series to those reported in the literature. MATERIAL AND METHODS: Men with FFA being treated in our dermatology department from January 2010 to December 2015 were included prospectively for this descriptive study. We collected patient information and clinical and treatment characteristics. RESULTS: Twelve men (mean age, 75 years) were recruited. Alopecia was the reason for seeking medical care in only 4 cases. The hairline had receded 3cm on average. Half the patients had facial papules, and 83% had androgenetic alopecia or hair loss on eyebrows or extremities. Follicular hyperkeratosis and erythema were present in 66%, and only 25% of the men reported pruritus. The most commonly prescribed treatments were topical: corticosteroids in 8 patients (66%) and minoxidil in 4 (33%). CONCLUSIONS: Facial papules, androgenetic alopecia, and loss of body hair are more often observed in men with FFA than in women. The men in this series were older on average than in other FFA case series in the literature, possibly accounting for the higher prevalence of associated androgenetic alopecia and the fact that most of these men were seeking care for conditions other than hair loss.

Outcomes and infectious etiologies of febrile neutropenia in non-immunocompromised children who present in an emergency department.

There are no unified protocols governing the management of healthy children with febrile neutropenia in the emergency department (ED). Conservative management is the norm, with admission and empirical broad-spectrum antibiotics prescribed, although viral infections are considered the most frequent etiology. The aim of this study was to describe the clinical outcomes and identified etiologies of unsuspected neutropenia in febrile immunocompetent children assessed in the ED. This was a retrospective study: well-appearing healthy children <18 years old with febrile moderate [absolute neutrophil count (ANC) 500-999 neutrophils ×10(9)/l] or severe (ANC <500 neutrophils ×10(9)/l) neutropenia diagnosed in ED between 2005 and 2013 were included. Patients newly diagnosed with hematologic or oncologic disease were excluded. We included 190 patients: 158 (83.2 %) with moderate and 32(16.8 %) with severe neutropenia. One hundred and one (53.2 %) were admitted; 48(47.5 %) with broad-spectrum antibiotics. The median length of stay was 3 days (IQR 3-5) and the median duration of neutropenia was 6 days (IQR 3-12). An infectious agent was identified in 23(12.1 %); 21 (91.3 %) were viruses. Four (2.1 %) children had a serious bacterial infection (SBI): urinary tract infection and lobar pneumonia (two cases each). All blood cultures performed (144; 75.8 %) were negative. Over the 1-year follow-up, one or several blood tests were performed on 167 patients (87.9 %); two (1.2 %) were diagnosed with autoimmune chronic neutropenia. Previously healthy children with moderate or severe febrile neutropenia have a low risk of SBI and a favorable clinical outcome. Less aggressive management could be carried out in most of them. Although chronic hematological diseases are infrequently diagnosed, serial ANC are necessary to detect them.

Larva migrans cutánea de origen autóctono en Guipúzcoa / Autochthonous cutaneous larva migrans infection in Guipúzcoa

INTRODUCCIÓN: Larva migrans cutánea (LM) es una erupción serpiginosa causada por helmintos nematodos que circulan por la epidermis. Se adquiere cuando la piel entra en contacto con tierra contaminada por heces de animales infestados por estos nematodos. Hasta ahora se consideraba como enfermedad importada de zonas tropicales y subtropicales. El objetivo fue estudiar los casos de LM diagnosticados como autóctonos por no haber salido de la provincia de Guipúzcoa recientemente. MATERIAL Y MÉTODOS: Estudio observacional transversal retrospectivo de los casos diagnosticados de LM en el Hospital Universitario Donostia de 2011 a 2015, sin viaje previo a ninguna zona endémica de este cuadro. El diagnóstico fue clínico ante las lesiones características. Las variables estudiadas fueron: edad, género, localización de las lesiones, fecha de inicio de los síntomas, posible fuente de contagio, datos anatomo-patológicos, tratamiento y evolución. RESULTADOS: Se han recogido 4 casos, todos varones, con una media de edad de 60 años. Tres casos presentaron lesiones en las extremidades inferiores, mientras que uno lo hizo en el tronco. Todos nuestros pacientes habían estado en contacto con tierra que pudiera estar contaminada por heces, siendo este el mecanismo de transmisión más probable. Se instauró tratamiento con albendazol oral, con resolución de las lesiones. CONCLUSIONES: La aparición de nuevos casos de LM de origen autóctono en Europa obliga al estudio de la/s especie/s causal/es, a una revisión epidemiológica de esta infestación y a planificar qué medidas se deberían tomar para evitar que una enfermedad considerada hasta ahora como importada, se convierta en autóctona

INTRODUCTION: Cutaneous larva migrans (LM) infection forms a serpiginous eruption caused by the migration of nematode helminths through the epidermis. The parasites are acquired when the skin comes into contact with soil contaminated by the feces of infected animals. Until now, infections have been believed to be imported from tropical and subtropical regions. Our aim was to study cases of cutaneous LM diagnosed in residents of the Spanish province of Guipúzcoa who had not recently traveled to such regions. Material and methods; Cross-sectional observational study of LM cases diagnosed in Hospital Universitario Donostia from 2011 to 2015 in patients who had not visited a region where this nematode infection is endemic. Clinical diagnoses were based on characteristic lesions. We studied the following variables: age, sex, site of lesions, date of onset of symptoms, possible source of contagion, pathologic findings, treatment, and clinical course. RESULTS: We found 4 cases, all in men (mean age, 60 years). Lesions were on the lower extremities in 3 patients and on the trunk in 1 patient. All had been in contact with soil that could have been contaminated by feces and was the most likely source of the parasite. The lesions disappeared after treatment with oral albendazole. CONCLUSIONS: The appearance of cases of autochthonous LM in Europe requires investigation of the culprit species, a review of the epidemiology of this infection, which was once considered imported, and the planning of public health measures to prevent it from becoming endemic